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         Merle Reeseman ~ Support Group 
2015 Meetings

Sorry pholks, my computer is acting up... I will post asap.

September 19, 2015
Update on various insurance issues - Denisha Washington from Medical Mutual of Ohio

August 15,WALK & ROLL -- fund raiserALLCC Twinsburg

July 18,  
Emergency Preparedness for PAH - Debra Hudock RNGilead CC Twinsburg

May 16, 2015
CARING FOR THE CAREGIVER -- Landra Slaughter, RN from University Hospital

March 21, 2015
Pulmonary Hypertension due to Blood Clots (CTEPH) -- Dr. G. Heresi-Davila from the Cleveland Clinic
                 ^    ^     ^    ^    ^    
2014 Meetings

December 12, 2014

​The meeting was opened by Merle with a disclaimer that the support information shared is for support and not medical advice. She reminded participates to keep shared information private.

A moment was taken to remember those who have departed this life, those who are breathing easier and are no longer suffering. Pam Johnston passed away in October.

The PH bill did not pass so we will start again next year.

Introdction of Supporters/providers a BIG thank you to Lung Bio-Tech for the feast of the day. TY Todd

Our presentation for the day.

Lung Transplantation and Pulmonary ThrombEndarterectomy

Benjamin Medalion, MD ~ Case University Hospitals

Chronic Thrombo-Embolic PH
Latency from symptom onset to diagnosis:
oGradual onset of SOBOE
oSubtlety of clinical signs in early phase
oLack of CTEPH awareness amongst medics
(“asthma”, “LVF”……)
o2 to 3 YEAR delay in diagnosis is common

Histology
NOT blood clot!
Combination of
1. Fibrous organization incorporated in vessel wall
2. Remodeling of small vessels
Mechanical problem -> Mechanical solution

Prognosis of CTEPH
Stratified by mean PAP (n=147)
^^Predicted survival of 40-50 y.o.male

100% *, **, ***, ****, *****
*<20 mmHg (64)
  80%
** 21-30 mmHg (19)
  60%
*** 31-40 mmHg (10)
  40%
**** 41-50 mmHg (19)
  20%
  0%***** >50 mmHg (35)
  From : 0 to 10 years

Conditional Survival Post - PTE

92.5% after 5 years and 88.5% after 10 years

Investigations
Referral
Exercise testing (6 minute walk)
Echocardiogram
Pulmonary function tests
V/Q scan
Right heart catheterization
Phase II coronary angio, carotid dopplers
(Insertion of IVC filter pre-op)

Surgical Classification of CTEPH
Lungs showing clots and how they would impair breathing.
Various types of clots that have been removed

Drawing showing procedure for removal of blood clots

View inside pulmonary artery
Before: Airway clogged
After: room for blood to circulate

Lung Transplantation
1963 -James Hardy 1st lung transplantation
1963 – 198540 cases most of them unsuccessful
1982 – Shumway 1st heart-lung transplant Stanford
1985 – Cooper Toronto Single/Double lung
2014 -> 300 centers worldwide

A chart showing Adult and Pediatric Lung Transplants with number of Transplants by year and procedure type
1985 there were 6. Each year the number increased, from single lung transplant to double lung transplant. In 2011 there were 3747 double lung transplants.  

Adult Lung Transplants
Indications for single Lung Transplants
(Transplants: January 1995 – June 2014)
5%Alpha-1
44%COPD
2%CF
34%IPF
1%IPAH
3%Re-Tx
10%Other which includes:
4%Pulmonary Fibrosis
0.4%Bronchiectasis
1.9%Sarcoidosis
1.1%ConnectiveTissue Disease
0.7%OB (non-Re TX)
1.0%LAM
0.4%Congenital Heart Disease
1.1%Miscellaneous

Pictures showing procedure
A.Main airway and vessels are cut to remove the diseased right lung.  
  Recipient: Main airway – Pulmonary artery – Pulmonary veins
B. Healthy donor (transplanted) lung in place.
Stitches used to connect donor (transplanted) lung to recipient’s main airway and blood vessels.

The NOVEL Lung Trial One-Year Outcomes – P.G. Sanchez
From August 2011 to May 2013, 76 EVLPs were performed and 42 were transplanted (55% utilization). Sonors in the EVLP group had a significantly lower Pa02/Fi02 ratio (p 0.001)… Early outcomes after lung transplantation and one-year survival were not significantly different between patients that received EVLP or standard criteria lungs.

The EXPAND Lung International Trial to Evaluate the Safety and Effectiveness of the Portable Organ Care System (OCS) Lung for Recruiting, Preserving and Assessing Expanded Criteria Donor Lungs for Transplantation.  
D.Van Raemdonck, G. Warnecke

Results: To-date 224 patients were transplanted in 20 transplant centers in the US, Europe, Australia and Canada


Lung Allocation Score – LAS
The lung allocation score calculates:
oThe probability of a patient’s survival in thenext year without a transplant
oThe probability of a patient’s survival in the first year post transplant
The difference between those values is the raw allocation score
LAS = normalized raw score (0 to 100)

LAS parameters
DiagnosisPCWP
Age02 requirement
BMIsix minute walk
DMmechanical ventilation
NYHA classcreatinine
FVC
Pulmonary pressure

Adult Lung Transplants
Kaplan-Meier Survival by Diagnosis Conditional on Survival to 1 year
(Transplants: January 1990 to June 2011)

100% - survival one to two years with a slow decline after 15 years
Median survival (years): Alpha 1 = 8.7 years; CF = 10.5 year; COPD = 6.9 years; IPF = 7.0 years; IPAH = 10.0 years; Sarcoidosis = 8.5 years

ADULT LUNG RECIPIENTS
Functional Status of Surviving Recipients – (follow-ups: April 1994 to June 2006)
Very few require total assistance
About ¼ perform with some assistance
¾ have no activity limitations

Complications after lung transplantations
Technical / cardiac1 month to a year
Acute rejection possibility the first 6 months
Infectionspossibility the first 6 months
Chronic rejection6 months and thereafter

Other: Drug related side effects; renal insufficiency; Osteoporosis; Hypertension; Diabetes; Malignancy

Questions and answers during and following the presentation

“Remember, the future of lung transplantation is no transplantation.”



September 13, 2014

The meeting was opened by Merle with a disclaimer that the support information shared is for support and not medical advice. She reminded participates to keep shared information private.

A moment was taken to remember those who have departed this life, those who are breathing easier and are no longer suffering. Since our July meeting, Merle has not been notified of any of our members passing. 

Introduce Supporters/providers a BIG thank you to Gilead Sciences for the feast of the day. TY Gia

Announcements: Birthdays, Anniversaries (wedding or PH) any other special event.
I have an announcement… Marilyn O is having brain surgery on September 18th… let’s keep her in our thoughts and prayers… she has a brain leakage which is giving her severe headahes. This procedure will be done at UH.
Last month PHA had a webinar concerning Oxygen Access and PH: Getting the Oxygen You Need. It is now on line at https://www.phassociation.org/Classroom/Recording/LivingWithPH/O2AccessandPH 
September is Sickle Cell Awareness Month
Every Breath Counts: Idiopathic Pulmonary Fibrosis, a documentary to mark Pulmonary Fibrosis Awareness Month, will air on the Discovery Channel on September 13th and 27th at 8:00 am ET/PT. Be an early bird and watch this :D http://www.everybreathcountsfilm.com/
8th Annual Walk & Roll–was on August 16th we moved our location which is an indoor facility with ac, a kitchen and indoor plumbing. Yeah! Brushwood Pavilion Townsend Rd., 4955 Townsend Rd., Richfield, OH Still taking donations.  
In June, Nicole had her 2nd Annual golf outing and in July Maria celebrated 10 years having PAH by having an antique CAR SHOW and I think she’s still celebrating… she’s in NYC this weekend.
Our PH bills --  HR 2073 and S 1453 – The Pulmonary Hypertension Research and Diagnosis Act of 2013. Call your politician and tell a little about how long it took you to be diagnosed… I’m sure it took a while – this bill does not ask for money but is pushing for early diagnosis and treatment. 
November 5th the Cleveland Clinic will be holding their one day 8th Summit. It is for clinicians and CME course but we are welcome to attend, you need to register before October 1 by calling 216-445-5763216-445-5763.  
To see an overview, click here
Our next meeting is December 6 when Dr. Robert Schliz will be our guest speaker.

Our presentation for the day:  Have you ever wondered what to do in an emergency situation? What do I need, what should I know?????? Who do I contact?????

Deb Hudock, RN, MSN, CNS from Akron General Medical Center advised us of what we will need and she reviewed having the correct supplies when you travel out of the home, do you have back up medicines, medication lists, etc. Gia supplied emergency refrigerator packs and other patient education information.

 Emergency Preparedness for PAH
Goals for this presentation
Recognize a medical emergency
Prepare yourself for an emergency
Educate your local emergency medical services

Recognizing a medical emergency
Understanding some basic emergency terminology

ER – Emergency Room: Area of a hospital designated for rapid treatment of people experiencing sudden illness or trauma (the term Emergency Department or ED, is also used) 
EMS – Emergency Medical Technician: A certified healthcare provider who is trained to treat and transport people in need of emergency care.
Paramedic: A certified healthcare provider who has more advanced training than an EMT
9-1-1:  Telephone number to report an emergency or request emergency assistance from police fire department, or ambulance. (Assistance can vary by state).  
When calling from a cell phone you may be directed to the State Police, you may want to ask for the specific service needed. In some rural communities, 911 may not be available. 

What is a PAH emergency?
Any situation that is serious or life-threatening and demands immediate medical attention
PAH emergencies may include:
  Severe bleeding
  Sudden worsening of PAH symptoms
  Malfunctioning IV pump or loss of a central line
  Fainting or near fainting
  Fever
Any medical emergency can become a PAH emergency
  Other PAH emergencies can include:
  Coughing up blood abrupt discontinuation of PAH medication, excessive bleeding if you are taking anticoagulants (blood thinners), new-onset or worsening chest pain or pressure, unusually rapid or irregular heartbeat, fainting or near-fainting, unusually severe shortness of breath, high or prolonged fever,, significant swelling in the arms and legs, taking the incorrect dosage of your PAH medication.
Potential emergencies for those receiving medications through a pump or catheter:
Equipment problems: catheter falls out or is pulled out, catheter is leaking or is cracked, or pump stops working
Infection can be a serious concern. Signs of infection include redness, tenderness, warmth, firmness, draining or “oozing” from the skin around the catheter, and/or discharge of any color at or near the exit site of your catheter; feeling unusually weak or tired; having fever/chills 
PAH can have significant effects on other medical emergencies, so medical emergencies not related to PAH – such as being in a car accident or developing an infection – can quickly become PAH emergencies
Calling 9-1-1 does NOT mean you are bothering the fire department/EMS. Their job is to serve the public 24 hours a day. (Have you notified them of your condition?)
When in doubt, call 9-1-1

Preparing Yourself for an Emergency
EMS and ER staff may be unfamiliar with PAH. You can help them by.
Wearing a medical alert bracelet
Knowing your infusion dose if you are on therapy that requires an infusion pump (this does not mean the rate of the pump, this means the medication (Flolan, Remodulin, Veletri), how much you draw – the dosage)
oIf you are on an oral or inhaled, what medication and how much
Keeping extra supplies, if you can, and bringing then with you to the hospital.
Have the following written information available in case of an emergency
Current list of your medications (some clip to driver’s license or in their pouch)
A list of any medications you are allergic to
Contact number for your PAH physician – a 24 hour a day number
Written instructions for emergency personnel from your PAH physician (ie: if you are on an IV or subcu – not to be disconnected)
List of people you wish emergency personnel to contact in case of an emergency
Written advance directive, if one exists
oAn advance directive is a legal document that informs others about your specific wishes regarding medical care, should you become unable to communicate. An advanced directive may include a living will or a “do not resuscitate” (DNR) directive. Be certain your advance directive form is a signed, original copy—photocopies are typically not valid. Your doctor or an attorney can help you with the proper forms.
Prepare a fridge kit and program “ICE” into your phone
Fridge kit
Emergency personnel are trained to look on your refrigerator for important medical information
Clearly nark your fridge kit and affix it to the outer door of your refrigerator
A fridge kit contains
oWritten emergency information
oExtra medical supplies, if possible or where they may be located
ICE – in case of emergency
Program emergency numbers into your mobile phone
Provide your emergency contacts with the information they will need to give to emergency personnel
Prepare your emergency information before an emergency occurs
Every additional piece of information can improve your chance for successful treatment
A medical alert bracelet let EMS/ER know that you have PAH
A fridge kit should be attached to the refrigerator door and clearly marked
oThrough the Insights program, Gilead has a fridge kit that a patient can request through their healthcare provider or that an HCP can request from their Gilead representative
oEMS personnel are trained to look on the refrigerator for important medical information
oA sticker or note on the front door of your house can ehlp alert EMS to a fridge kit
Your fridge kit should include:
oInformation on PAH
oList of medications and medication allergies
oExtra medical supplies (if possible or where they can be located)
oList of people to contact in case of emergency
oYour advance directive, if one exists
oContact information and instructions fro hyour PAH physician and/or nurse coordinator
When traveling, keep a copy of your emergency documentation or fridge kit in your car or carry-on baggage
If you use an infusion pump
It is unlikely that EMS personnel will know how to operate your pump – do not assume they know more than you do about your pump and how it works
oRemind EMS staff that your central catheter is not to be used for administering fluids or medications other than your PAH medication
oREMIND EMS STAFF THAT YOUR PUMP IS NOT TO BE STOPPED OR DISCONNECTED FOR ANY REASON
The hospital may not have the type of pump you require
oAlways bring your backup pump with you
oIf possible, have someone familiar with your pump meet you at the hospital and stay with you.
Bring additional supplies, including your backup medication to the hospital
Bring contact information for your specialty pharmacy
What happens when you call 9-1-1
Activating 9-1-1 sets off a chain of events. Each town, city, or county has a different set of rules and protocols for who reacts when—depending on what resources are available
If the accident or illness is life-threatening, the ambulance will take you to the closest available hospital: if it is not life-threatening, you may be able to choose (within reasonable distance) the hospital you wish to be transported to.
Home – 
1. An emergency is recognized  
2. 9-1-1 is called  
3. Dispatcher sends EMS  
4. Fire department/ambulance arrives
Transport –
5. EMS assessed your condition and prepares you for transport
6. Ambulance transports you to the hospital
7. Treatment (oxygen, IV, medications) may be initiated in the ambulance
Hospital –
8. ER staff assesses your condition upon arrival
9. ER doctor examines you and orders tests
10. You are treated
11. You may be admitted to the hospital for further evaluation and treatment, or you may be discharged
12. Be sure your PAH specialist is contacted before you are discharged

Educate Your Local Emergency Medical Services
Speak with personnel from your local EMS and ER before an emergency occurs.
The size of your city or town may determine who you talk to. Larger cities may have more “red tape than smaller towns and may have numerous EMS/ER staff and facilities, making it harder to predict where and by who you will be treated in the event of an emergency

Do not show up unannounced – make an appointment by looking in your phone book for the non-emergency number of your fire department or 9-1-1 ask for fire chief or person in charge; for the hospital ask for the nurse manager or nurse educator in ER department. 
Plan to be brief – they may be busy and you may have to reschedule
Speak with your nurse coordinator about the best way to educate emergency personnel
Specific thing to explain to EMS
Advise EMS if you have a front-door sticker and/or a fridge kit
Ask to be put on the “Be On the LookOut” (BOLO)
oThis system works with your telephone number; so if 9-1-1 is called from your phone number, the EMS dispatcher will already have your name and information – including you having PAH – and can instruct EMS personnel to look for your fridge kit
oIf the ER receives your fridge kit and calls your PAH specialist, you are more likely to get proper care.
Inform staff never to turn off your pump or change your medication without speaking to your PAH physician or nurse coordinator

Specific things to explain to ER staff
Give a brief description of your PAH medication, as it may not be familiar to the ER staff
Emphasize the importance of contacting your PAH specialist immediately
Ask if your medication is stocked in the hospital’s inpatient pharmacy
oDiscuss your PAH medication with the ER—especially if there are certain dos and don’ts regarding your treatment
oMention that your medication may be available only through a specialty pharmacy
If it is not, speak with your pharmacy about providing information to the hospital’s inpatient pharmacy staff
Inform staff never to turn off your pump or change your medication (including those or orals or in haled medications) without speaking to your PAH physician or nurse coordinator
Inform staff not to use your central line to administer other medications or solutions (including saline) or to draw blood.

Useful resources
Consider reviewing these resources before speaking with EMS and ER personnel; you may wish to share then during your meeting.

Insights at www.InsightsOnPAH.com
The Pulmonary Hypertension Association at www.phassociation.org
PHCentral at www.phcentral.org
The National Institutes of Health at www.nih.gov
The Scleroderma Foundation at www.scleroerma.org
Know Your PH at www.knowyourph.org 
The Cleveland Area PH Support Group at www.ClevelandAreaPH.com 

Questions and answers followed. Then our chat and snack time.
Dr Charles Roach also updated us on PATCH and donated a PATCH coffee mug filled with goodies to add to our door prize drawings

&  &  &  &  &  &  &  


July 12, 2014

The meeting was opened by Merle with a disclaimer that the support information shared is for support and not medical advice. She reminded participates to keep shared information private.

A moment was taken to remember those who have departed this life, those who are breathing easier and are no longer suffering. It is with a saddened heart that she let the members know on the morning of April 17th, Zahi Kakish took his last earthly breath and on May 28th, Tom Risko, also lost his battle to PH.  Zahi has been a member of our group since he was first diagnosed.  He and Janice attended all our meetings and they have been a great support for us and especially with our Walk & Roll each year. Zahi and I spoke often on the phone, first to make sure all our ducks were in a row for the W&R, and then just because.. a how are you doing type thing. Tom was a newer member to our group but we also talked often. He wanted to learn more so we talked; since March he spent an exceptional amount of time in the hospital and he was awaiting a new set of lungs.

Introduce Supporters/providers I want to thank Accredo for our feast today.  TY Beth

Announcements: Birthdays, Anniversaries (wedding or PH) any other special event.
I have an announcement… Sherry Warrington has offered to be a co-leader :D stand up and take a bow :D
Envelope of Hope for new comers – on the table up front – an info packet will be mailed out to with info about PH and PHA. Or just give me your name and address and I will send in the info
8th Annual Walk & Roll–will be on August 16th and we will be moving our location which is an indoor facility with ac, a kitchen and indoor plumbing. It is on a level so no little hill to walk. Brushwood Pavilion Townsend Rd., 4955 Townsend Rd., Richfield, OH 44286, still part of the Metro Park system (Furnace Run). Zahi’s family will still cook for us this year as Janice and family want to honor Zahi. This is our special event to raise funds for R/A. I hope you have been saving your change or a $1 a week. Our Walk & Roll is a fund raiser as well as a social. You can also ask you friends and family for donations.
July 26th Maria will be celebrating 10 years having PAH and is having an antique CAR SHOW – DJ, Chinese Auction, 50/phifty raffle Quaker Steak & Lube 5800 Interstate Blvd, in Austintown.
Our PH bills --  HR 2073 and S 1453 – The Pulmonary Hypertension Research and Diagnosis Act of 2013. Tell a little about how long it took you to be diagnosed… I’m sure it took a while – this bill does not ask for money but is pushing for early diagnosis and treatment. 
Our next meeting is September 13 and Deb Hudock, RN will do our presentation on Emergency Preparedness.

Our speaker for the day:  Beth Patrick, Accredo Customer Relations Specialist who talked to us about:  
Traveling with Pulmonary Hypertension

We will discuss:
Planning your trip
Altitude
Weather 
Tips on hydration
Pollution
Traveling with oxygen
Mediations
Documentation and information for your trip

Consider high pollution areas
      High pollution areas can trigger allergic reactions
http://www.airnow.gov/indes.cfm?action=airnow.local_state
The AIR Now Web site: Can provide for over 300 cities in the USA
The Air Quality Index (AQI) is an index for reporting daily air quality. It tells you how clean or polluted your outdoor air is, and what associated health effects might be a concern for you.
The web site provides detailed information on the pollution levels and numerous other lings on air quality.
The site provides a sign up for e-mail notifications.

Choosing a destination
      When planning your trip
Consider the effects of altitude and weather on your body.
Consider climate changes
   oLow humidity can worsen PH Symptoms
   oCold weather can precipitate bronchospasm

Higher altitudes can make it difficult to breathe.
Everyone breathes faster and deeper (hyperventilates) at high altitudes – it is necessary to do this in order to survive. The function of the lungs is to expose blood to fresh air, and breathing faster essentially increases the flow of fresh air past the blood. This means that whenever an oxygen molecule is taken away by the blood, it is quickly replaced by a fresh one. This means that there is always more oxygen available to be taken up into the blood.

Tips for traveling in cold weather
Wear a mask or a long, warm scarf to protect your face and warm the air around your mouth
Prevent hypothermia and chills by wearing multiple layers and a warm hat. Use heat packs inside of mittens to keep your hands warm
Use a can to keep steady, especially in slick conditions. Try to avoid icy or slippery situations if at all possible
Oxygen users should carry an extra cannula with them
If driving in cold weather, store extra blankets, warm clothing, food and water in your car; always let someone know where you’re going and when you’ll return; and keep a charged cell phone on you at all times.

Tip for traveling in warm weather
           Adequate hydration is a must
Warm weather means you’ll need to plan to stay in an air conditioned environment and pay close attention to your body’s reaction to warm weather, especially while enjoying the outdoors.
Getting enough liquids and getting the right type of liquids is key to heart, brain, kidney and bowel/digestive health.
If you notice your urine look darker than lemonade it is a safe bet you need to up your fluid intake*

Tips on Beverages
Caffeinated beverages such as tea, coffee, colas, chocolate and energy drinks deplete fluid, and do not replenish it. Even many root beers and orange sodas add caffeine. It is safest to read the label of any sodas you purchase.
Alcohol also causes dehydration and requires you to increase hydration*
Know your salt restrictions.
Water and sport drinks are best sources for hydration. Sports drinks replace necessary salts in addition to the water lost in sweating and urination.
Know the best diet for your health. Keep regular meal times and sleep patterns

CONSULT YOUR PULMONARY HYPERTENSION DOCTOR!!!
Discuss your travel plans with your PH specialist well in advance.
Tell him/er where you are planning to go, what you plan to do and how long you plan to stay.
Obtain a list of local PAH treating physicians and medical facilities in the area of your destination in case of an emergency.
Obtain a copy of your recent medical history.
Have your doctor write a letter giving you permission to travel and have them list your medications, including oxygen, medically necessary supplies and other assistive devices.

High Altitude Simulation Test
Discuss the possibility of undergoing a high altitude simulation test (HAST) with your doctor, and you are considering a trip by airplane, or a trip to a higher elevation, you may need this test.  
High Altitude Simulation Test (HAST is a test that can determine the need for supplemental oxygen in patients who are going to be traveling by air or at high altitude. If you have chronic lung disease (COPD, emphysema, chronic bronchitis, severe asthma), and you are considering a trip by airplane, or a trip to a higher elevation, you may need this test. If you are already on supplemental oxygen you may still need testing. This is simply the safest and most accurate way to determine if you will need oxygen at higher altitudes, and exactly what level of oxygen keeps your oxygen saturation in the safe range.*

Notify your Specialty Pharmacy
Notify your pharmacy of your destination, and ask for assistance with your travel plans.
Consider having your medications shipped directly to your destination within the US.
Always carry a back-up supply for several days with you when you travel overnight.
Carry your medical information, list of medications, your doctor’s prescriptions and Medical Clearance to Travel Letter – in your carry on with your medications.
Request insurance coverage for extra medication for your trip. Many insurance groups will require prior authorization. Allow adequate time for approval.
Flolan, Veletri & Remodulin patients should consider requesting an extra infusion pump when traveling out of country.
Speak with the Cardiopulmonary Nurse directly and have them identify a contact person in your destination area to provide you assistance in case of emergencies: Especially important for patient on Continuous IV or Subcutaneous therapy

Before you travel
Consult with your doctor about having routine blood test done before your trip.
Refill your prescriptions. Double check the amount needed for your trip
Make up a brief medical history sheet and detailed medication list.
Try not to travel alone.
Inform and provide your traveling partners of your emergency contact information provide them with a copy.
Consider purchasing travel insurance.
Consider booking non-stop flights
Have a plan in place for how you will get to from your car to the gate, to your destination when you travel.
Pack well in advance to avoid a last minute rush and extra stress.
Call the airline, cruise line, tour company, hotel, and oxygen supplies 48 hours before leaving for confirmation of plans.
Don’t be afraid to ask for assistance.
Know your limitations, give yourself extra time.

Packing tips
Keep MD name, coordinator, and phone number on hand.
Wear medical alert jewelry.
Carry emergency instructions; pump manual; EMS letter.
Pack your Medications in your carry-on, do not check-in your medication bag!!! Take at least one week of additional supplies for “just in case”!
Carry your meds in their original pharmacy bottles.
Carry your medical information, list of medications, your doctor’s prescriptions and Medical Clearance to Travel Letter in your carry-on with your medications.
If your medication needs to be on ice: have an ice chest with at least six to eight ice packs and a premixed medication cassette. Keep at least one week supply of all vials, supplies, back-up pump in your carry-on container.
Place new batteries in your medication pump prior to your trip and you can pack these extra batteries in checked luggage.
When traveling with a portable oxygen concentrator your extra batteries need to be in your carry-on luggage.
Take your Emergency Kit!!!

Air travel with Oxygen
For safety reasons Federal Aviation Administration regulations do not allow you to carry your own oxygen canisters on board. Liquid oxygen is easily combustible and is therefore prohibited. FAA has an approved portable oxygen concentrators (POC) list and will allow empty oxygen tanks to be checked as luggage. Airline personnel will need to verify that they are oxygen tanks are empty before checking them.
Always contact the individual airline well in advance for their current policies and charges regarding traveling with oxygen.
You must carry your prescription for oxygen with you at all times. It must specify the need for a portable oxygen concentrator during flight and required liters per minute (LPM) flow rate. Many of the airlines have their own airline specific medical form for your doctor to fill out. You will need your oxygen prescription to be written with 10 days of your 1st let of your trip.*

TSA Guidelines
Official website of the Department of Homeland Security
http://www.tsa.gov/traveler-information/travelers-diabilities-and-medical-conditions

Airline Oxygen Council of America (AOCA) AOCA is a coalition of individuals and organizations whose objective is to ease air travel for those who require supplemental oxygen.

Check the web site: http://www.tsa.gov/ For links and information on: Airline Policies – Travelling Tips – A list of DOT Approved POCs – How to Report a Problem with an Airline

Tips for traveling with POC and batteries
When traveling with a POC, keep batteries and extra supplies for the POC in your carry-on baggage – not in your checked baggage! Flight crew confirms you have enough batteries for your POC for the length of the flight. You will be required to carry enough POC batteries to last 150% of predicted length of your flight.
DOT’s rule on carrying lithium batteries during air travel, which took effect January 1, 2008, prohibits loose (spare) lithium batteries in checked baggage, i.e., large suitcases handed to the airline. Portable electronic devices packed with checked baggage, may contain correctly packaged batteries.
In carry-on baggage, certain type of lithium batteries may be packed, such as those used in cell phones and most laptop computers, provided you take measures to protect (cover) terminals.
* http://safetravel.dot.gov/ 

Special considerations when traveling with 02
Book a direct flight to your destination whenever possible. If not possible consider the layover destination related to altitudes and durations. Be prepared for delays.
Airlines that provide supplemental oxygen in-flight do not provide the same service during layovers. Passengers are responsible to arrange for their own oxygen during the time on the ground.
If oxygen is needed during flight, options include renting or purchasing an approved portable concentrator, or making arrangements with the airline to provide oxygen in-flight.
Cost and availability of in-flight oxygen for purchase varies by carrier. Remember that aircraft electrical power is generally not available for portable oxygen concentrators. In the cabin, flight crew can better monitor conditions, and have access to the batteries or device if a fire does occur.


                                                <   <   <   <     >   >   >   >   

May 3, 2014

The meeting was opened by Merle with a disclaimer that the support information shared is for support and not medical advice. She reminded participates to keep shared information private.

A moment was taken to remember those who have departed this life, those who are breathing easier and are no longer suffering. It is with a saddened heart that she let the members know on the morning of April 17th, Zahi Kakish took his last earthly breath.

Introduction of supporters/providers and to thank Bayer Healthcare LLC for our lunch.

Announcements: Birthdays, Anniversaries (wedding or PH) any other special event.
Early registration for the PHA International Conference June 20th thru the 22nd has now closed but you can still register. It will be held in Indianapolis, IN
Envelope of Hope for new comers – on the table up front – an info packet will be mailed out to with info about PH and PHA. Or just give me your name and address and I will send in the info
8th Annual Walk & Roll–will be on August 16th and we will be moving our location which is an indoor facility with ac, a kitchen and indoor plumbing. It is on a level so no little hill to walk. Brushwood Pavilion Townsend Rd., Richfield, OH 44286, still part of the Metro Park system. Zahi’s family will still cook for us this year and Janice mentioned, in honor of Zahi there will be a belly dancer once again. This is our special event to raise funds for R/A. Don’t forget, if we get a jar and put a $1 in it a week or $5 a month; or our loose change daily we will have some funds to donate. Our Walk & Roll is a fund raiser as well as a social. 
May 5th is World PH Awareness Day… if you didn’t call your Congressperson on April 2nd or even if you did…. Do call again. Congressman Ryan has co-sponsored this bill
Our PH bills --  HR 2073 and S 1453 – The Pulmonary Hypertension Research and Diagnosis Act of 2013. Tell a little about how long it took you to be diagnoised… I’m sure it took a while – this bill does not ask for money but is pushing for early diagnosis and treatment. 
Nicole was unable to attend the meeting so Merle mentioned about her upcoming 2nd golf outing on June 28th
After our presentation Dr. Charles Roach will give us an update on the research he is working on.
Our next meeting is July 12 – a Saturday the library has changed their hours so we can now meet on Saturday in July..

Our speaker for the day: Nancy Bair, RN CNS a nurse specialist at the Cleveland Clinic - ‎Department of Pulmonary Medicine and Critical Care will help us understand “the basics” and what really goes on with having pulmonary hypertension.  
Pulmonary Hypertension – Understanding the Basics
Bayer HealthCare LLC

Pulmonary Hypertension is a disease of the Lung and heart
Pulmonary hypertension (PH) is high blood pressure in your lungs’ arteries
PH has many potential causes
High blood pressure in your lungs makes the right side of your heart work harder
Over time the right side of your heart enlarges and is less able to pump blood

Different kinds of Blood Pressures
The blood pressure you measure with a cuff is called “systemic” blood pressure
    •It’s the pressure in your body’s arteries as your heart pumps blood out to the rest of your body
Blood vessels in your lungs, called pulmonary arteries, have their own pressures, and usually this pressure is very low
Even if you have normal systemic blood pressure you can have high blood pressure in : the lungs
    •This can happen because of an imbalance in the chemical that relax and constrict blood vessels
    •The walls of the arteries can thicken
    •The narrowed space in the pulmonary arteries restricts blood flow

Pulmonary Arterial Hypertension (PAH) Is One Kind of Pulmonary Hypertension
Normal pressure in your lungs: 8 to 20 mmHg at rest  
            High pressure in your lungs (PH) >25 mmHg at rest

Pulmonary Hypertension can be related to many conditions --> WHO
WHO GROUP 1 PAH - Right Side of Heart
    •Unknown causes
    •Passed down in families ie: Heritable or Familial 
    •Certain drugs
    •Certain diseases: ie scleroderma, lupus, HIV infection
    •Heart defects such as ASD or VSD; a hole in the heart
WHO GROUP 2 PH - Left Side of Heart
    •Left heart weakness or valve diseases
    •Common heart diseases
WHO GROUP 3 PH – Lung low oxygen
    •Certain lung diseases, like chronic obstructive pulmonary disease (COPD)
    •Airway diseases or scarring of lung
    •Sleep disorders and low oxygen levels
        Sleep apnea
        High altitude
        Emphysema 
WHO Group 4 -- Chronic thromboembolic pulmonary hypertension (CTEPH)
    •Blockage inside the lung vessels (blood clots)
    •Possible correction by operation (more later in presentation)
WHO Group 5 – Unclear
    •Multiple causes that are unclear (eg: thyroid diseases, kidney disease, some blood diseases, and some metabolic diseases)
    •ie: sarcoidosis, splenectomy disorders

Symptoms you may have if you have PAH
As PAH worsens you may have some of these symptoms
Feeling short of breath with activity and fatigue are two of the most common
You may also have a depressed mood, frustration, or anxiety
    •Shortness of breath with activity
    •Edema – swelling in your legs or feet
    •Fatigue weakness
    •Palpitations or racing heart beat
    •Chest pain
    •Lightheaded or dizzy
    •Bloating

Medical History and Physical Exam
A good history and a through physical exam by our healthcare provider are key to diagnosing and helping to determine the cause of your symptoms
Your healthcare provider may ask you:
    •Does PAH run in your family
    •Have you used diet drugs or stimulant drugs
    •Your healthcare provider may ask you:
    •Do you have any disease that may be associated with PAH
        Systemic sclerosis, lupus, 
        Infection with HIV
        Liver disease
Your healthcare provider will look to see if you have:
    •Abnormal heart sounds, such as a murmur
    •Neck veins larger than normal
    •Swelling in the legs or belly

If PH May Be a Possibility…
Your healthcare provider will order an echocardiogram
    •An echocardiogram uses sound waves to create a moving picture of your heart
        •It will show signs of changes in your heart related to PH
        •Enlarge right heart chambers
        •Backflow of blood when the heart pumps
    •Echocardiography can estimate pressures in the lung’s blood vessels

Other Tests Your Healthcare Provider May Order
Electrocardiogram
    •To see if there are signs that the right ventricle is thickening
Chest x-ray
    •To help exclude other lung diseases
Blood tests
    •Including measuring how much oxygen is in your blood
Pulmonary function tests
    •Measure how well your lungs take in and release air
    •To see if any underlying lung disease
6MW hopefully will improve over time
        •Don’t wear flip-flops or stiletto’s :D

To Identify a form of Pulmonary Hypertension
Ventilation/perfusion (V/Q) Scan
    •Special markers show flow of air (ventilation, “V”) and flow of blood (perfusion, “Q”) into the lungs
    •The “Q” of the scan can show parts of the lungs that may not be getting blood flow because of blockages
A V/Q scan can help physicians differentiate types of pulmonary hypertension and may help determine if you’re a good candidate for surgical treatment
Every patient is whom PH is suspected should get a V/Q scan

Right Heart Catheterization is Used by Your Healthcare Provider to Confirm Pulmonary Hypertension
A special tube called a catheter is threated into a big vessel in the neck, leg, or arm that takes blood to your lungs
Once in place, the catheter measures pressure in the heart and lungs
It also measurers how much blood your heart is pumping to your body
A left heart catheterization looks at blood vessels that goes to the muscles

6 Minute Walk Test
Measurers the distance a patient can walk in 6 minutes
    •Changes in distance can be compared from one test date to another
    •Easy to perform -> Can be repeated to compare results over time
Done in a straight hallway at least 100 foot long
Helps your healthcare provider understand:
    •How your heart rate, blood pressure and oxygen levels respond to activity
    •How severe your disease is
    •Whether you disease is worsening or getting better

Disease Progression
There is no cure for PAH
There are medical treatments for PAH that can improve some symptoms and slow its progression
With accurate, timely diagnosis a patient who has PAH can manage and minimize the impact of PAH

The World Health Organization’s Functional Classes Help Providers Describe PAH Symptoms
Definition
Class 1: No limitation of usual activity. Ordinary physical activity does not cause shortness of breath, fatigue, chest pain, or feeling faint
Class 2: Mild limitation of physical activity. No discomfort at rest, but normal physical activity cause symptoms
Class 3: Market limitation of physical activity. No Discomfort at rest, but even a little activity causes symptoms 
Class 4: Severe limitations with any physical activity. Shortness of breath and/or fatigue may be present at rest, and symptoms get worse with almost any physical activity. May have signs of heart failure
    •This is when you may pass out
Functional class helps you and your healthcare provider decide which treatment is best for you

How Will PAH Affect My Life?
Symptoms of PAH will have an impact on what you can do
    •Feeling tired may make you not want to do thing you like to do
    •Shortness of breath may limit how far you can walk or how well you can climb stair
    •Dizziness or syncope (fainting) may make it unsafe to drive
Some days will be better, and some days will be worse
Ask Family and friends for help when you need it
A RHC may only be required if and when there is a change in your symptoms

Monitoring Your Condition
After diagnosis, you and your healthcare provider need to understand how your are doing
    •What does the echocardiogram show
    •How are the pressures in your heart and lungs
    •How much physical activity can your body tolerate
    •Is it getting worse, staying the same, or getting better
    •Are your symptoms changing quickly or gradually
    •Are there signs of problems in the right side of the heart (i.e., right ventricular failure)
Answers to these questions help you and your provider create a treatment plan that is best for you.
Discussing these same questions over time helps you know if your treatment needs adjustment

Chronic Thromboembolic Pulmonary Hypertension Group 4
A POTENTIALLY SURGICALLY CURABLE FORM OF PH

CTEPH is Different From PAH
In PAH the narrowed pulmonary artery resists blood flow
In CTEPH a thrombus – a clotlike mass – gets stuck to the lung’s blood vessel wall and blocks blood flow
Like PAH, CTEPH results in high pressures in the lungs’ blood vessels
    •Over time, CTEPH can cause the heart to fail
    •But unlike PAH, in some cases, a surgeon can remove the thrombi and cure the patient’s CTEPH
This surgery to remove thrombi can be called PEA (pulmonary endarterectomy) or PTE by your healthcare provider

In CTEPH, a History of Pulmonary Embolism (PE) is Common
A pulmonary embolism (PE) is a clotlike blockage that get suck in an artery in your lungs
    •Often, a PE is caused by a blood clot that traveled into your lungs from another part of your body
PE can happen in healthy people
As many as 4 in 100 patients who have had a PE may develop CTEPH
    •But just because you have a blood clot doesn’t mean you will get PH
    •Many patients who have CTEPH had a PE in their past
    •Sometimes, there can be a long time between having a PE and developing CTEPH

Why Did I Get CTEPH? Risk Factors
Factors specific to pulmonary embolism (PE)
    •More than one PE
    •Young age when the PE was found
    •Very high blood pressure in your lung’s arteries
    •High pressures that last for at least 6 months after the PE was treated
Some of the associated medical conditions
    •Having your spleen removed
    •Infected surgical cardiac shunts, pacemakers, or defibrillator leads
    •Chronic inflammatory disorders
    •Thyroid replacement therapy
    •Cancer
    •High risk for clotting of blood 
    •Genetic factors

CTEPH Operability Assessment by an Experienced PH Center
Pulmonary anglography and CT scanning are useful tools for your healthcare provider to determine if surgery might help you
Pulmonary anglography (preferred)
    •An x-ray image of the blood vessels of the lungs
        This allows for detailed picture that can be used to assess blood flow to the lungs
CT Scan
    •Contrast dye “lights up” the blood vessels to produce a cross-sectional of the pulmonary arteries
Talk to your healthcare provider to learn more about PH centers that are experienced in managing CTEPH

Surgical Treatment of CTEPH
The treatment of choice for patients with CTEPH is PEA surgery to remove the blockages in the lung
Patients with CTEPH should receive life-long blood thinners usually warfarin
Surgery should not be delayed by treatment with a medication

Survival Rates Following Pulmonary Endarterectomy (PEA) Are Hightst at Centers With the Most Experience
A study of 27 centers showed that as the number of surgeries performed at a center went up, so did short and longer term survival rates
In this study as many as 97% of patients survived they surgery
95% of patient lived at least 1 year after surgery in the most experienced centers
With successful surgery, pulmonary arterial pressures should go down, and symptoms should improve or go away

Questions to Ask Your Healthcare Provider
Are there any more tests we need to do
Are there any activities I shouldn’t try to do
Can I exercise
Do I have to change my diet
What kind of treatments are available
Where can I get help

SUMMARY
Pulmonary Hypertension is a life threatening disease of the heart and lungs
Though there is no cure for PAH, medical treatments can minimize its impact
Your doctor may do a V/Q scan to see if CTEPH is the cause
Pulmonary endarterectomy is the only potentially curative treatment for CTEPH
There are a lot of sources of help available to you – you don’t have to go it alone
For more information, talk with your doctor

Normal Values for Common Right Heart Catheterization Measurements
Right atrial pressurenormal range 0-5mmHg
Right ventricular pressure, systolicnormal range15-25 mmHg
Right ventricular pressure, diastolicnormal range 0-10 mmHg
Pulmonary artery pressure, systolicnormal range15-25 mmHg
Pulmonary artery pressure, diastolicnormal range 6-12 mmHg
Mean pulmonary artery pressurenormal range < 25 mmHg
Pulmonary artery wedge pressurenormal range ≤ 12 mmHg
Cardiac outputnormal range > 5 L/min
Cardiac indexnormal range > 2.4 L/min/m²
Transpulmonary gradientnormal range ≤ 12 mmHg
Pulmonary vascular resistancenormal range ≤240 dyn-sec-cm⁵

Reliable sources of high-quality information
•Pulmonary Hypertension Association (PHA), http://www.phassociation.org/
Founded by a group of patients who had pulmonary hypertension and nurses and doctors who treat it
An invaluable resource for information, including videos and other materials, on the different forms of PH, including PAH and CTEPH
The PHA’s Pulmonary Hypertension: A Patient’s Survival Guide—with contributions from patients, nurses, and doctors—is the go-to manual on living with PH
•American Thoracic Society (ATS), http://www.thoracic.org/
Professional association that also includes information for patients about heart and lung diseases
•National Organization of Rare Diseases (NORD), http://www.rarediseases.org/
The National Organization for Rare Disorders (NORD) provides advocacy, education and other services to improve the lives of all people affected by rare diseases, including interactive maps to help explore insurance options
•Scleroderma Foundation, http://www.scleroderma.org: Scleroderma is a chronic connective tissue disease that is commonly associated with pulmonary arterial hypertension
Sources of personal support
•The PHA has a wealth of support resources, including community blogs, patient-to-patient help lines, and mentoring programs for caregivers
•Caring Voice Coalition, http://www.caringvoice.org/
Caring Voice Coalition empowers patients who live with a life threatening chronic disease through 
comprehensive outreach programs and services aimed at financial, emotional, and educational support

Abbreviations and Definitions
Arterial—something to do with the blood vessels that carry blood from the heart
Blood tests—blood is checked for oxygen content and other factors, including a chemical that indicates heart failure
Catheter—a long, narrow, flexible tube that can be threaded into the heart and lungs to measure pressures
Chest x-ray—an x-ray of the lungs and heart that’s used to help rule out other lung disease in a patient who may have PH
Chronic—something that happens over a long period of time
CTEPH, chronic thromboembolic pulmonary hypertension—pulmonary hypertension caused by potentially removable obstructions in the pulmonary arteries
CT (computed tomography) scan—a 3D scan of the lungs that can help assess operability of blockages in CTEPH
Dyspnea—shortness of breath
Echocardiogram—a moving picture of your heart used to detect signs of PH and to estimate pulmonary artery pressures
Electrocardiogram—a test that measures the electrical signals your heart sends. It can uncover signs that point to PH
Emboli—blood clots
Endothelial—the inner lining of the blood vessels
Hypertension—high blood pressure
Hypoxia—not enough oxygen in the blood
Left side of the heart—the part of your heart that pumps blood to the rest of your body
Pulmonary—something to do with the lungs
Pulmonary angiogram—a detailed picture of pulmonary arteries that is the preferred tool to assess operability of lesions in patients with CTEPH
Pulmonary arterial hypertension (PAH)—high pressure in the pulmonary arteries, defined as mean pulmonary artery pressure ≥25 mmHg with pulmonary arterial wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) >3 Woods units
Pulmonary embolism (PE)—a blood clot that forms in a leg vein that breaks off and gets stuck in a blood vessel in the lungs
Pulmonary endarterectomy (PEA)—a surgical operation to remove blockages in the lungs
Pulmonary function tests (PFT)—a battery of tests, including spirometry, that evaluates how well your lungs breathe in and release air
Pulmonary hypertension (PH)—any disease that causes high blood pressure in the lungs
Pulmonary vascular resistance (PVR)—a measure of the resistance in your lungs arteries against the flow of blood. Very high PVR suggests a worse prognosis
Right heart catheterization (RHC)—a test in which a tube is placed in a lung blood vessel to confirm if you have pulmonary arterial hypertension
Right side of the heart—the part of your heart that pumps blood to the lungs
Six-minute walk test (6MWT)—a test to determine how far you can walk in six minutes. It may be given throughout your treatment to record your progress
Syncope—fainting
Thromboembolic—something to do with a blood clot that travels through the blood and gets stuck in a smaller blood vessel
Thrombus—a blockage stuck to a blood vessel wall that makes it harder for blood to flow through the vessel
V/Q (ventilation/perfusion) scan—a test that measures flow of air (V) and blood (Q) in the lungs. A normal V/Q scan can rule out CTEPH
Warfarin—a commonly used blood thinner (or anticoagulant) that is often used in patient who have CTEPH
WHO Functional Class—a scale developed by the World Health Organization (WHO) for doctors to rate the severity of pulmonary hypertension symptoms
WHO Groups—the World Health Organization (WHO) classified 5 groups of PH by their causes

          < < < < > > > > 
March 8, 2014

The meeting was opened by Merle with a disclaimer that the support information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away. She was not notified of anyone passing on.​The pharmaceutical reps and clinicians introduced themselves and a thank you to United Therapeutics for providing lunch today.

Merle also mentioned: • 
Team Phenomenal Hope. 7 Ladies riding their bicycles across America in 8 days. An awesome event and they will be done in time for the PHA Conference in June of this year. “Racing Toward a Cure” June 20 – 22 J W Marriott, Indianapolis, IN. Registration opens January 15, 2014
• Let’s not forget our new Pulmonary Hypertension Research and Diagnosis Act of 2013 HR 2073 and S 1453. Please call your respective politicians and ask to have them co-sponsor these much needed bills. Same name for the House as the Senate.
8th Annual Walk & Roll–will be on August 16th and we will be moving our location which is an indoor facility with ac and a kitchen. On a level so no little hill to walk. Brushwood Pavilion Townsend Rd., Richfield, OH 44286, still part of the Metro Park system. Maybe we can twist Zahi’s arm and still have some Mediterranean food. This is our special event to raise funds for R/A; I was going to send everyone a note but you know how that goes… if we get a jar and put a $1 in it a week or $5 a month; or our loose change daily we will have some funds to donate.  Our Walk & Roll is a fund raiser as well as a social.
​•Don’t forget the time change tonight… that must be a sign of spring. The change from standard time to daylight saving time is at 2:00 a.m. – or before you go to bed. Clocks should be set FORWARD 1 hour. Spring forward – Fall back :D

Our presentation for the day:

Updates from the 5th World Symposium for
Pulmonary Hypertension – Nice, France
Robert Schilz DO, PhD, FCCP
Interim Chief of the Division of Pulmonary, Critical Care and Sleep Medicine
Director of Pulmonary Vascular Disease and Lung Transplantation
University Hospitals – Case Medical Center

TOPICS DISCUSSED AT THE SYMPOSIUM

Pulmonary Arterial Hypertension: Epidemiology and Registries
Chronic Thromboembolic Pulmonary Hypertension
Relevant Issues in the Pathology and Pathobiology of Pulmonary Hypertension
New Trial Designs and Potential Therapies from Pulmonary arterial Hypertension
Right Heart Adaption in Pulmonary Arterial Hypertension
Pediatric Pulmonary Hypertension
Updated Clinical Classification of Pulmonary Arterial Hypertension
Updated Treatment Algorithm of Pulmonary Arterial Hypertension
Definitions and Diagnosis of Pulmonary Arterial Hypertension
Pulmonary Hypertension Due to Left Heart Disease
Genetics and Genomic of Pulmonary Arterial Hypertension
Pulmonary Hypertension in Chronic Lung Diseases

Demographic, Clinical, and Hemodynamic Characteristics of PAH
Registries From Different Countries and Time Periods
Several years ago the average age in the US as 36 ±
Survival Date of PAH Registries continues to increase
Multivariate Predictors of Survival – various testing shows positive indications

TOPIC: Relevant Issues in the Pathology and Pathobiology of PH

Does the Pulmonary Venous System Play and Important Role in PAH and to What Extent Are PAH and PVOD Part of the Same Spectrum of Disease?
Are There Distinct Pathways in Vascular Cells in Mild Versus Severe PH?
What Are the Differences and Similarities Between Cell Proliferations in PAH When Compared with Traditional Neoplastic Disease?
What Is the Role of Inflammation in the Initiation and Progression of Different PAH Types?

Slide: Relevant Issues in the Pathology and Pathobiology of PH: Proposed Multifactorial Factors Influencing Progression of PH
SEVERITY OF PULMONARY VASCULAR DYSFUNCTION
oVirus - Inflammation – Hypoxia – Drugs/toxins etc (low to stable)
oGenetically Susceptible (low to increasing)
oGenetically Non-susceptible (low to slight increase)
oEpigenetic changes (stable)

Emerging Paradigms in PH Research
Research involving the broad effects of metabolic programming of intima and media pulmonary vascular cells (endothelial and smooth muscle cells) and the immediate perivascular microenvironment.
The perivascular region is dominated by fibroblasts and migrating circulating cells, including inflammatory and progenitor cells.
Shown in the center is the impact of these factors in the intima and media of pulmonary arteries
The metabolic plasticity involves all cells involved in the pulmonary hypertension (PH) panvasculopathy and is itself modified by inflammation and infiltrating progenitor cells

TOPIC: New Trial Designs and Potential Therapies for PAH

Designs and Endpoints for PAH Trials
Future Targets for Therapeutic
Ethical/Global Issues in Drug Development for an Orphan Disease

Future Therapeutics
  Pathways/Targets Therapy
VasodilationNitric Oxide, Nitrite
Sympathetic nervous system Selective > Nonselective beta-adrenergic blockade
Renin-angiotensin-aldosterone Aldosterone antagonist, vasopressin receptor
systemantagonis, catheter guided ablation
Vascular remodeling metabolicDichloroacetate, ranolazine
alterations
Selective and multikinase inhibitorsTyrosine kinase inhibitors
Stem cells, Gene therapyEndothelial, mesenchymal and gene enhancing cells
DevicesCardiac resynchronization, extracorporal life support (venoarterial, venovenous and pulseless arteriovenous lung assist

TOPIC: Updated Clinical Classification of PAH

See first page of web site for the updated classifications

Updated Classification for Drug and Toxin Induced PAH
Definite Possible
Likely Unlikely
oAminorex Cocaine
oFenfluramine Phenylpropanolamine
oDexfenfluramine St. John’s Wort
oToxic repeseed oilChemotherapeutic agents
oBenflurexInterferon a and β
oSSRistAmphetamin-like drugs
oAmphetaminesOral contraceptives
oL-TryptophanEstrogen
oMethamphetaminesCigarette smoking
oDasatinib

Clinical Classification of Congenital Heart Disease Associated with PAH
Eisenmenger Syndrome
Left to right shunts
oOperable
oInoperable
PAH with co-incidental CHD
Post-operative PAH

TOPIC: Updated Treatment Algorithm of PAH

Classes of Recommendations
Class 1Evidence and/or general agreement that a given treatment or procedure is beneficial, useful, effective.
oIs recommended or indicated
Class 2Conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of the given treatment or procedure.
o
Class 2aWeight of evidence/opinion is in favor of usefulness/efficacy
oShould be considered
Class 2bUsefulness/efficacy is less well established by evidence/opinion
oMay be considered
Class 3Evidence or general agreement that the given treatment or procedure is not useful/effective, and in some cases may be harmful.
oIs not recommended

Levels of Evidence
AData derived from multiple randomized clinical trials or meta-analyses
BDate derived from a single randomized clinical trial or large nonrandomized studies
CConsensus of opinion of the experts and/or small studies, retrospective studies, registries

Updated Treatment Algorithm of PAH
One of those slides unable to view

TOPIC: Treatment Goals of PH

Variables Used in Clinical Practice to Determine Response of Therapy and Prognosis in Patients with PAH
Functional Class…. 1 or 2
Echocardiography/CMR
Normal/near-normal RV size and function
Hemodynamics
Normalization of RV function (RAP <8 mm Hg and CI >2.5 to 3.0 l/min/m2)
6-min walk distance
>380 to 440m; may not be aggressive enough in young individuals
Cardiopulmonary exercise testing
Peak VO2 > 15 ml/min/kg and EqC02 <45 l/min/l/min
B-type natriuretic peptide level
Normal

Recommendations for Treatment Goals of PH
Although the primarily observational studies discussed here do not allow for definitive conclusion, reasonable goals of therapy include the following:
o1) modified NYHA FC 1 or 2;
o2) echocardiography/CMR of normal/near-normal RV size and function
o3) hemodynamic parameters showing normalization of RV function (RAP <8 mm Hg and CI >2.5 to 3.0 l/min/m2)
o4) 6MWD of >380 to 440 m (which may not be aggressive enough)
o5) cardiopulmonary exercise testing, including peak oxygen consumption ml/min/kg and EqCO2 <45 l/min/l/min
o6) normal BNP levels
Patients who achieve these goals, no matter which specific therapy of approach is used, seem to have a better prognosis than those who do not.
A more aggressive approach to goal-oriented therapy may help us shift the survival curves farther to the right

TOPIC: Genetics and Genomics of PAH

Recommendations
oIn addition, the authors of these guidelines have recommended that patients with IPAH be advised about the availability of genetic testing and counseling because of the strong possibility that they carry a disease-causing mutation
oThe guidelines recommend that professionals offer counseling and testing to the affected IPAH patient before approaching other family members.
oThe identification of a disease-causing mutation in an affected family member allows less expensive testing of other family members, if they want such testing

TOPIC: Pulmonary Hypertension in Chronic Lung Diseases

oEpidemiology and Clinical Relevance of PH in Lung Disease
oTreatment of PH Caused by Chronic Lung Disease (Group 3); Evidence for Appropriate Benefit/Risk Ratio of PAH-Focused Drugs
oRecommendations for COPD and Lung Fibrosis
oSpecific Aspects of Sarcoidosis, Systemic Sclerosis, and Rare Lung Diseases

Recommendations
oIt is suggested that the term “out of proportion” be abandoned and that the following definitions for COPD, IPF, and CPFE (measurements undertaken at rest with supplemental oxygen if needed) be used:
COPD/IPF/CPFE with PH (mPAP <25 mmHg)
COPD/IPF/CPFE with PH (PAP ≥ 25 mm Hg? PH-COPD, PH-IPF, and PH-CPFE)
COPD/IPF/CPFE with severe PH (mPAP≥ 35 mm Hg or m PAP ≥25 mm Hg with low CI (<2.01/min/m2); severe PH-COPD, severe PH-IPF, and severe PH-CPFE).
oThe choice of mPAP ≥35 mm Hg as a cutoff for severe PH is based on the following findings/assumptions, which should be further addressed and putatively revised in future studies:
The “severe PH group” includes only a minority of chronic lung disease patients suspected of having significant/severe vascular abnormalities (remodeling) accompanying the parenchymal disease 40. For COPD, this corresponds to ~ 1% of the entire population included in the NETT (National Emphysema Treatment Trial).
This degree of PH in COPD/IPF is assumed to cause circulatory impairment that substantially worsens the reduced exercise capacity caused by obstructive/restrictive ventilator impairment.

Management of PH in the Setting of Chronic Lung Disease
COPD with FEV1 ≥ 60% of predicted IPF with FVC ≥ 70% of predicted CT: absence of or only very modest airway or parenchymal abnormalities
A. mPAP <25 mm Hg at rest ~  
oNo PH – NO PAH treatment recommended
B. mPAP ≥25 and <35 mm Hg at rest ~ 
oPH classification uncertain. No data currently support treatment with PAH approved drugs
C. mPAP ≥35 mmHg at rest
oPH classification uncertain: discrimination between PAH (group 1) with concomitant lung disease or PH caused by lung disease (group 3) Refer to a center with expertise in both PH and chronic lung disease
COPD with FEV2 <60% of predicted IPF with FVC <70% of predicted. Combined pulmonary fibrosis and emphysema on CT (define A, B, C see above)
A. No PH No PAH treatment recommended
B. PH-COPD, PH-IPF, PH-CPFE No date currently support treatment with PAH approved drugs
C. Severe PH-COPD severe PH-IPF, severe PH-CPFE refer to a center with expertise in both PH and chronic lung disease for individualized patient care because of poor prognosis, randomized controlled trials required.

Questions and Answers followed.

                                                     < << < < <  <  > > > >>> >>> 

2013 Meetings 

December 14, 2013

The meeting was opened by Merle with a disclaimer that the support information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away. She was not notified of anyone passing on.​The pharmaceutical reps and clinicians introduced themselves and a thank you to United Therapeutics for providing lunch today.

Merle also mentioned:  • Team Phenomenal Hope. 7 Ladies riding their bicycles across America in 8 days. An awesome event and they will be done in time for the PHA Conference in June of next year. “Racing Toward a Cure” June 20 – 22 JW Marriott, Indianapolis, IN. Registration opens January 15, 2014
Let’s not forget our new Pulmonary Hypertension Research and Diagnosis Act of 2013 HR 2073 and S 1453. Please call your respective politicians and ask to have them co-sponsor these much needed bills. Same name for the House as the Senate.
Our very own Nicole along with Julia Feitner and others went to the November luncheon in DC. Julia (a member of the Pittsburgh Group) shared her thoughts about why she was excited to attend the Luncheon:
“PH research has saved my life, and I wanted to do what I could to help further research and promote early diagnosis to help other patients. For me the best part of the Congressional Luncheon was ‘we the people’ participating in our government the way it was intended and speaking with our representatives about what’s important to us."
Seven Representatives were so moved by the stories shared by PHers during their visits, they agreed to co-sponsor the PH Research and Diagnosis Act. One being Congressman Tim Ryan, once again.
November 15 and 16th, the Cleveland Clinic had their PH Summit – Translating Discoveries Into Patient Care. The afternoon of the16th is specifically for patients and caregivers

The days program:

Pulmonary Hypertension (PH) vs Pulmonary Arterial Hypertension (PAH)
What’s the Difference?

Robert Schilz DO, PhD, FCCP
Director of Pulmonary Vascular Disease and Lung Transplantation
Interim Chief Division of Pulmonary, Critical Care and Sleep Medicine
University Hospitals Case Medical Center
Cleveland, OH

2013 Recap
New Therapies
oRiociguat (Adempas®)
oMacitentan (Opsumit®)
New Research Therapies
oBeraprost
oBardoxolone Methyl
oNitric Oxide (NO)
New Gene
oKCNK3
oPotassium Channel
oMay have drug to “Cure” Defect Early on
New Conference – WSPAH5 (Clinicians)
New Initiatives PHA
oPHCC
oEarly Diagnosis (HR 2073 and S 1453)

PH vs PAH – Simply Defined
1 PAH 
oIdiopathic or Primary (old term
oHeritable
oAssociated
oConnective Tissue Disorders
oLiver Disease
oHIV
oPVOD, PCH
oDrug Induced
2 PH Due to Left Heart Disease
oSystolic Dysfunction
oDiastolic Dysfunction
oValvular Disease
3 PH Due to Lung Diseases and/or Hypoxia
oCOPD
oInterstital Lung Disease
oOther pulmonary diseases with mixed restrictive and obstructive pattern
oSleep Disordered Breathing
oAlvolar Hypoventilation Syndromes
oChronic Exposure to High Altitude
oDevelopmental Abnormalities
4. Chronic Thromboembolic Pulmonary Hypertension (blood clots)
5. PH with unclear and/or multifactorial mechanisms

PH vs PAH: How Doctors Tell the Difference

Normal Pulmonary Pressures 8-20 mm Hg
PH Pulmonary Pressures >25 mm mmHg
PAH Pulmonary Pressures 25 mm HG AND Exclusion of Other Causes of PH
oPCWP < 15 mm Hg
oNo significant lung damage
oNo chronic thromboembolism

What distinguishes PAH from other elevations of pressures in the lungs (PH)?
oPathology
oProgressive, serious course if untreated
oMagnitude of Pressure elevations in symptomatic patients
oHemodynamic limitation of exercise
oTreatment

Pathophysiological Characteristics
oNormal artery – blood flows easily no constriction
oVasoconstriction
oSmooth muscle hypertrophy
oEarly intimal proliferation (walls starting to thicken)
oAdvanced Vascular Lesion
oSmooth muscle hypertrophy
oAdventital and intimal proliferation (walls thickening)
oInsitu thrombosis (showing signs of extra thickening)
oPlexiform lesions (guck and stuff)

Idiopathic PAH: Survival if Untreated
Slide show a 25 to 30% chance of survival 4.5 to 5 years

“Typical” Pulmonary Pressures in Patients with Various Disorders
oPAP = 25mm Hg (slide shows)
oNormal – below 25mm Hg
oCOPD about 25mm Hg
oOSA about 25mm Hg
oSCD a little above 25mm Hg
oIPF a little more above 25 mm Hg
oPAH (CTEPH way above 25mm Hg

Hemodynamic Progression of PAH
  Pre-symptomatic -- Symptomatic/Stable -- Progressive/Decline
oExercise Cardiac Output (at a high level to stable to decline)
oPulmonary Pressure (at a low level to stable and remains there)
oCardiac output (at a medium level to progressive/decline)
oIncreasing PVR (just as it states – low level to increasing)

FDA-approved Therapies for PAH
oProstacyclin Derivatives
oEpoprostenol: IV
oTreprostinil: Subcutaneous, IV, inhaled
oIloprost: inhaled
oEndothelin Receptor Antagonists
oBosentan: Oral
oAmbrisentan: Oral
oMacitentan: Oral
oPhosphodiesterase Type-5 Inhibitors
oSildenafil: Oral
oTadalafil: Oral
oSoluble Guanylate Cyclase Stimulators
oRiociguat: Oral

Questions and answers followed

HAPPY HOLIDAY EVERY ONE - SEE YOU NEXT YEAR
                                < < < < < > > > > > 
September 14, 2013

The meeting was opened by Merle with a disclaimer that the support the information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away. Unfortunately several in the PH Community have passed on.
Introduction of clinicians/providers.  A thank you to Lung LLC for providing lunch for the day

Announcements: Birthdays, Anniversaries (wedding or PH) any other special event.
Envelope of Hope for new comers – an info packet will be mailed out to with info about PH and PHA. If you have a computer, you can also register on line.
With awareness month coming up Merle asked if any have plans for a media connection ie: Pulmonary hypertension can develop from diseases that affect millions of people including sleep apnea, scleroderma, sickle cell disease, HIV, chronic obstructive pulmonary disease (COPD), schistosomiasis, hereditary hemorrhagic telangiectasia (HHT), liver disease, lupus, mixed connective-tissue disease, congenital heart disease (CHD). Or there can be no known cause.
7th Annual Walk & Roll was July 13th – next year we will be moving our location and will have an indoor facility with ac and a kitchen. Maybe we can twist Zahi’s arm and still have some mediterranean food. BTW we done good Gross: $27,688.11 Expenses: $1,269.61 Final Net: $26,418.50
We do have a new Research and Diagnosis bill out HR 2073 and S 1453. Please call your respective politicians and ask to have them co-sponsor these much needed bills
November 15 and 16th, the Cleveland Clinic is having their PH Summit – Translating Discoveries Into Patient Care. The afternoon of the16th is specifically for patients and caregivers… you do not have to be a patient at the clinic to attend… and it’s free – lunch included. Call Meredith Fisher at 216 445-5763216 445-5763 for registration. You can attend both days if you would like to.

Have you ever wondered why some medicines work for some of us while another may work differently for someone else…. Join us when Dr. Charles Roach will explain the ins and outs of trials; the whys, duration and what would be needed from a patient. And an update on what is currently being worked on:
“ON GOING TRIALS”

Our presentation for the day was on research and how to get involved with a current program and helping our PH needs. Our guest speaker is Dr. (Emir) Charles Roach – from the Department of Pathobiology at the Cleveland Clinic. Dr. Roach has received several medical school awards. He has been involved with several research trials mostly involving vascular surgery or cardiovascular disease. He has many “published” articles and his accomplishments go on and on. He will talk to us today about:
  Various PH meds in trial. We’ve heard the buzz on: laboratory experiments, researchers removed inflammation-producing cells called macrophages from the lung tissue of rats dying of pulmonary hypertension and put the cells in cultures with healthy rat endothelial cells. The trial done at Stanford also the Macitentan trial and Riociguat.

Carvedilol is an FDA Approved Drug used by millions of people for blood pressure and left heart failure. For this trial it will use for the right side! Carvedilol/Coreg has proven to prolong life in heart failure patients significantly!

Katie Zak also did a brief presentation on research she is involved with: She is a research coordinator in the field of Pulmonary Hypertension at Cleveland Clinic in Cleveland, OH. Her focus is on clinical trials investigating innovative therapies for PH patients. She joined the Respiratory Institute in 2008 after graduating with honors from Xavier University.  

​ = = = = = = = 
MAY 4, 2013
The meeting was opened by Merle with a disclaimer that the support the information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away. She was not notified of anyone passing on.​The pharmaceutical reps and clinicians introduced themselves and a thank you to Accredo for providing lunch today.
Announcements: Birthdays, Anniversaries (wedding or PH) any other special event.
Envelope of Hope for new comers – an info packet will be mailed out to with info about PH and PHA. If you have a computer, you can also do that on line.
May 5th is World PH Day, What are you doing? Light a Flame of Hope – make a donation in honor or memory of a loved one and share a message of hope with your Flame to show the world we have hope for better treatments, a cure and better quality of life. You can go to the PHA site for more suggestions to post a tid bit on FB, Twitter or other social networks (whatever they may be) A sample: Pulmonary hypertension can develop from diseases that affect millions of people including sleep apnea, scleroderma, sickle cell disease, HIV, chronic obstructive pulmonary disease (COPD), schistosomiasis, hereditary hemorrhagic telangiectasia (HHT), liver disease, lupus, mixed connective-tissue disease, congenital heart disease (CHD). 
Tickets are still available for the Afghan of Hope we need to raise money for research.
7th Annual Walk & Roll will be this July 13th – still in the park and parking just for us. This is also to raise funds for Research and Awareness; I was going to send everyone a note but you know how that goes… if we get a jar and put a $1 in it a week or $5 a month; or our loose change daily we will have some funds to donate. Our Walk & Roll is a fund raiser as well as a social. Zahi? This will replace our July meeting here which would have to start at 11 a.m.
Hopefully, a new R/A bill will be coming out soon. When it does, I’ll let you know the #
Our next meeting is July 13th our 7th Annual Walk & Roll and the meeting following will be September 14th 

Pulmonary Arterial Hypertension Treatment with Carvedilol for Heart Failure – PAHTCH
Emir Charles Roach, M.D – Department of Pathobiology

Carvedilol is an FDA Approved Drug used by millions of people for blood pressure and left heart failure. We will use if for the right side! :D
Carvedilol prolongs life in heart failure significantly!

It is a very common drug used in many countries under different names
Coreg – being one of them

It is used by millions of patients from every race and age for heart failure and high blood pressure

What would be involved with the trial?
The Study spans over 6 months – total of 7 visits
Blood draws 6 Minute walk test
Urine SamplingQuestionnaire
EKGSaliva sapling
EchoChest x-ray
PET scanBreath tests

No invasive procedures!
Other than occasional blood draws
Breath Tests! – Keep Blowing!
A closer look with special imaging studies (PET Scan)
Private room for the one overnite stay.

Where would the testing be held? At the Cleveland Clinic Research Unit

How to learn more…
Call 216 445-7706216 445-7706 or email roache@ccf.org stating your interest.
Handouts are available and consent forms, containing detailed information will be mailed.

​                                                                      ** * * * * * * * * * * * * **                       
March 9, 2013
The meeting was opened by Merle with a disclaimer that the support the information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away.  She was not notified of anyone passing on.The pharmaceutical reps and clinicians introduced themselves and a thank you to Gilead Sciences for providing our lunch and program.
Announcements:  Birthdays, Anniversaries (wedding or PH) any other special event.• I have some good news, Matt who doesn’t usually attend our meetings but always attends our Walk & Roll, had a double lung transplant in February, he is doing well and I hope he will continue to attend our Walk.  He’s in high school, and was on 20 red caps of Flolan.• Envelope of Hope for new comers – on the table up front – an info packet will be mailed out to with info about PH and PHA.• Tickets are still available for the Afghan of Hope we need to raise money for research.• 7th Annual Walk & Roll will be this July 13th – still in the park and parking just for us. This is also to raise funds for R/A; I was going to send everyone a note but you know how that goes… if we get a jar and put a $1 in it a week or $5 a month; or our loose change daily we will have some funds to donate.  Our Walk & Roll is a fund raiser as well as a social. Zahi? This will replace our July meeting here which would have to start at 11 a.m. and would be difficult for most of us to get up and about that early.• Hopefully, a new R/A bill will be coming out soon.  When it does, I’ll let you know the #• Don’t forget the time change tonight… that must be a sign of spring. The change from standard time to daylight saving time is at 2:00 a.m. – or before you go to bed.  Clocks should be set FORWARD 1 hour.  Spring forward – Fall back  :D
OUR PRESENTATION FOR THE DAY:  
 – offered by Gilead Sciences
Insights on health insurance options
Presented by: Landra Slaughter, RN - University Hospital = Cleveland

Goals for this presentationIn this presentation you will learn about: The basics of health insurance and the different options available to youo Managed care planso Medicaid and Medicareo Medicare Part D planso Additional financial assistance options
What is health insurance? Health insurance helps pay for medical expenseso Plan offerings typically include coverage for some or all of the costs of care received from or associated with: Doctors Hospitals Emergency careo Most Americans receive health insurance from: Group private insurance Individual private insurance Government-funded programs (e.g. Medicare and Medicaid)
MANAGED CARE PLANS Typically offered by private insurance companieso A fixed fee (called premium) may need to be paid each montho There are four common type of plans: Health maintenance organization (HMO) plans Preferred provider organization (PPO) plans Exclusive provider organization (EPO) plans Point-of-service (POS) plans
Health maintenance organization (HMO) plans Provide reduced-cost coverageo You must select a primary care physician (PCP) from the HMO’s networko You must see a PCP first (and receive referral) before seeing a specialisto You must pay a co-payment (flat fee) each time you receive a medical service
Preferred provider organization (PPO) plans Allow you to choose any doctor inside or outside the PPO network Allow you to see a specialist without a referral from your PCPo Offer the flexibility to see doctors of your choiceo You must pay an annual deductible (a set out-of-pocket amount) before coverage startso You may be required to pay a percentage of the cost for each healthcare visit (known as co-insurance)
Exclusive provide organization (EPO) plans Low-cost version of a PPO plano Offer you the flexibility to see in-network doctors of your choiceo You must meet an annual deductible requiremento You may be required to pay a percentage of the cost for each healthcare visit (co-insurance)o Doctors you see or services you receive outside of the EPO network will not be coveredo May require a higher co-pay
Point-of service (POS) plans Allow you to choose providers or specialists within the plan’s network if referred by your PCPo You can minimize costs by obtaining referrals beforehand and seeing doctors inside the networko No deductible is required for in-network serviceso You must pay a co-payment for medical serviceso You may see a doctor or receive medical services outside of the network at additional cost
Prior authorizations Certain tests, procedures, or medications require prior authorization from your insurance provifder You are responsible for obtaining the prior authorization (through your physician) to ensure the care is coveredo Your physician’s office may need to contact your insurance to obtain authorization on your behalf
Comparing managed care plans                    HMO PPO EPO POSMust choose a primary care physician                 x                             xNeed a referral to see a specialist                        x                   xNeed prior authorization for some services         x           x      x        xMust see in-network healthcare providers            x          xCan see out-of-network healthcare providers      x          xMust pay a deductible                                                         x       x       <>Must pay a percentage of cost of visit                               x       x       <>Monthly premium cost to your                              $$       $$$$    $     $$$
<>May be subject to increased out-of-pocket costs when using an out-of-network provider
Please be aware that each person’s insurance needs vary and every health plan is different.  Be sure to talk with your plan administrator before making any changes to your coverage.
Denied claims Common reasons for denied claimso Treatment was sought without prior authorizationo Claim was not filed properly (missing or illegible information)o Claim was not filed within proper time limito Procedure or medication was not deemed medically necessaryo Treatment or medication was not covered by the insurance policy What you can do when your claim is deniedo Let your physician and nurse know that your received a denialo Call your insurance company to make sure the denial was not due to an erroro Request a formal review by your insurance providero Contact your state’s insurance commission
GOVERNMENT- FUNDED INSURANCE PROGRAMS
Medicaid Provides healthcare coverage for certain people with financial difficulties, including:o Seniors (age varies by state)o People who are blindo People who are disabled Varies by stateo Each state will decide who is eligible and the scope of health services that will be offered
Medicare Federal health insurance program for people in the United States aged 65 years or older and for people with certain disabilities Divided into four parts:o Medicare Part A – helps cover hospital care, typically at no costo Medicare Part B – provides payments for doctors, services, and medical supplies. Most people pay a monthly premiumo Medicare Part C (Medicare Advantage Plans) – offers additional coverage for both medical and prescriptions costso Medicare Part D – a prescription drug plan offered to individuals who have Medicare Visit www.medicare.gov  for more information
Medicare Part Do Is a prescription drug programo Is administered by private insurance companieso May be able to help cover costs for many PAH medications
Who is eligible for Medicare Part D?o Eligibility includes people who are: Aged 65 years and older Under age 65 with certain disabilities Any age with end-stage renal diseaseo You are eligible to enroll starting 3 months before and ending 3 months after you turn 65 If you do not enroll at age 65 you may be subject to a penaltyo You may make adjustments without penalty during the open enrollment period November 15 to December 31 of each year
Medicare Part D Payment responsibility An example of how Medicare Part D plans help pay for the cost of prescriptions per year – this is an example.  Your plan may have different costs.
Prescription costs per year•   What you pay   **  What Medicare pays
0-$295   100%$296 - $2,700    25%     75%$2,701 - $6,154     Donut hole   100%Over $6,154       5%     95%
Reflects Medicare Part D coverage costs for 2009
Low-income subsidy Medicare beneficiaries with limited income and resources may qualify for additional financial assistance with:o Monthly premiumso Yearly deductibleso Prescription co-insuranceo Co-payments More information, including eligibility requirements, is available at:  www.cms.hhs.gov 
Supplemental insurance policies These are additional policies that can help pay some of the healthcare costs that Medicare may not cover 12 different plans are offered by private insurance companies Each plan offers a different set of basic and extra benefits
OTHER OPTIONS FOR COVERING HEALTHCARE COSTs COBRAo If you lose your insurance, COBRA continues your coverage for a limited amount of time HIGH-RISK INSURANCEo Coverage for people with certain medical conditions, lifestyles, and professions that carry a greater risk of injury and illness FEE-FOR-SERVICE PLANSo Insurance plans that allow you to visit any doctor or hospital you choose; however, you must pay for the services up front and you will be reimbursed at a later date DISABILITY INSURANCEo Helps you recover lost income if you have a long-term illness or injury and are unable to worko Does not cover health insurance
Supplemental health accountso Flexible spending accounts Use pretax dollars to pay for certain eligible medical expenseso Health savings accounts Tax advantage savings accounts For people with plans that have much higher deductibles than a traditional health plan Funds roll over and accumulate year after year if they are not spent
PAP and manufacturer programs Many pharmaceutical companies offer special services that may help pay for certain medical expenses, including costs associated with:o Medicationso Monthly premiumso Insurance deductibleso Medicare Part D coverage gap
Patient assistance programs for common PAH medications There may be a patient assistance program to help cover the costs of your PAH medications Several manufacturers offer patient assistance, including”o Gileado United Therapeuticso Pfizero Actelion For more information, visit:  www.rxassist.org
Assistance from non-profit organizations Independent non-profit organizations help underinsured patients with:o Financial assistanceo Covering insurance costso Obtaining medications Two industry-supported non-profit organizations are:o Chronic Disease Fund – non-profit organization that helps underinsured patients obtain the expensive medications they need (www.cdfund.org)o Caring Voice Coalition – non-profit organization offering financial assistance, insurance education, and patient support (www.caringvoice.org)
Useful resourcesFind more information about insurance and PAH from the following resources:
Insurance resources: Medicare – www.medicare.gov (1 800-633-42271 800-633-4227) Centers for Medicare & Medicaid Services – www.cms.hhs.gov National Institutes of Health – www.nih.gov (301-496-4000301-496-4000)
Financial assistance resources RxAssist – www.rxassist.org (401-729-3284401-729-3284) Caring Voice Coalition – www.caringvoice.org (1800-267-1440) PSI – A.C.C.E.S.S. Program – www.uneedpsi.org (1 888-700-70101 888-700-7010) Chronic Disease Fund – www.cdfund.org (1 877-968-72331 877-968-7233)
Visit your state government’s website for more information on public assistance programs offered in your state.
Additional PAH resources Pulmonary Hypertension Association (PHA) – www.phassociation.org  (301-565-3004301-565-3004) PHCentral – www.phcentral.org
Questions and answers were during and aftern the presentation. A chat and snack time followed along wth introduction of a few new members.  Dr. Charles Roach of the Cleveland Clinic gave a brief presentation on an upcoming trial: Pulmonary Arterial Hypertension Treatment with Carvedilol for Heart Failure – PAHTCH – he is from the Department of Pathobiology.

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2012 Meeting Notes 

December 1, 2012

The meeting was opened by Merle with a disclaimer that the support the information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away.  She was not notified of anyone passing on.
The pharmaceutical reps and clinicians introduced themselves and a Thank You to United Therapeutics for our lunch.

Announcements:  Birthdays, Anniversaries (wedding or PH) any other special event.
Dorothy G. is doing well but she did have to have an operation on her arm and her birthday is on Monday.  She and Paul have plans for the weekend and cannot be here – also wishing everyone a Happy Holiday Season
Sheila and Rick also have plans today, Rick is doing well, no complications with his lung transplant and they also wish everyone Happy Holidays
We had several new members so everyone introduced themselves.

•Envelope of Hope for new comers – an info packet will be mailed out to you from PHA
•Our 6th Annual Walk & Roll was another success and held August 4th – Merle received a note from PHA saying we raised $12,421.59   
•We don’t have much time left for the PH Research and Education Bill HR 1810 – and the similar Senate bill S 775.  Reps. Ryan and LaTourette have co-sponsored the bill. A need to contact other reps and our Senators.
•BTW In late September Merle contacted her local Representative Dick Stevenson, about making November PH Awareness Month in the Commonwealth of PA.  In mid October at his local Expo/Health Fair, he presented her with the proclamation. Woo Hoo.  Merle’s brother happened to be visiting at the time and wanted to do it for MA.  Copies and other information were given and he presented it to his local Representative Carolyn Dykema. He was notified mid-November that November is  PH Awareness Month in the Commonwealth of Massachusetts.  Nicole Stafford was at the Mercer Area PH SG meeting when Merle showed them the proclamation and she asked for info to do the same in Ohio.  We haven’t heard as yet on that one.
•It was decided at the July meeting that July seems to be a difficult meeting time (basically because of the Library hours closing at 1 p.m. and a Sunday date).  We will go from May to September with our Walk & Roll in July.
•Our next meeting will be March 9th 2013; suggestions for meetings are welcomed.

Ever have an echo or a heart cath and can’t figure out what all those words mean. Dr. Robert Schilz, PhD of University Hospitals, Associate Professor of Medicine, Case Western Reserve University ~ Medical Director of Lung Transplantation and Pulmonary Vascular Disease ~ University Hospitals of Cleveland, and Associate Editor of the PHA Journal Editorial Committee; did a presentation on: 

                                               TESTS: WHAT DO THOSE TERMS MEAN? 
 Dr. Schilz began his presentation by telling us WHAT’s NEW?
Drugs (In Development)• Imatinib withdrawn• Oral Remodulin declined – still in trial, more information was required• Macitentan and Riociguat to FDA  o Initial studies with Riociguat and left heart failure not as encouraging• Inhaled nitric oxide (NO) now in active trial.• Oral Selexipag - selective prostacyclin receptor - phase III-IV

New from PHA• Centers of Excellence Initiative• Pediatric Research and Education Fund

Terms
Right Heart CatheterizationA small tube called a “catheter” is inserted into a blood vessel and passed toward the heart.  It measures important pressures in the heart and blood vessels of the lungs; thus enabling the doctor to know which treatment would be most beneficial to the patient. Right heart catheterization is the “gold standard” of diagnosis. It gives physicians a lot of information about how both sides of the heart are functioning. It is performed in the hospital. Some of the more common information obtained during a right heart cath is described here.• Cardiac output - (CO) average range 4.0 – 8.0 L/minute (5.6 L/min male & 4.9 L/min female)• Stroke Volume - The amount of blood pumped by the left ventricle of the heart in one contraction; the stroke volume determines the output of blood by the heart per minute (cardiac output).• Cardiac index - 2.4 to 4.2 liters per minute per square meter (of body surface area)• Pulmonary Artery Pressure  o Mean PAP - 9 to 17 mmHg  o Systolic/Diastolic – 30/15 mm Hg is an example of normal  o Mean PAP > 25 is abnormal and considered having PH (Remember PH ≠ PAH)• Pulmonary Capillary Wedge (PCW) pressure is 5 to 15 mm Hg. This is a measure of back pressure from the left side of the heart either from a malfunctioning valve or abnormal left sided pressures. Significantly elevated PCW suggests pulmonary venous hypertension.• Right Atrial Pressure or CVP is 0 to 8 mm Hg• PVR - Pulmonary Vascular Resistance – is a measure of how constricted or narrowed the total blood vessels in the lung are. Higher numbers typically indicate more burden of disease. This value is calculated from other numbers obtained during the right heart catheterization ( 80 * (PAP - PCW) / C.O ) or PAP = Mean Pulm Art Press; PCW = Pulm Cap Wedge Press;  C.O = Cardiac Output

EchocardiographyComputer records sound wave (ultra sound) echoes and displays picture while patient lies on bed on left side.  Sonographer moves transducer on patient’s chest, viewing heart at different angles.  Electrode patches attached to chest (for EKG). The test can assess the chambers and valves of your heart and how well your heart muscle and heart valves are working. It's useful in diagnosing and evaluating several types of heart disease, as well as evaluating the effectiveness of treatments.

See the figure for the location of the structures described below.• Chambers of the heart  o Atria / Intra-atrial septum  o Ventricles  / Intra-ventricular septum• Valves (Regurgitation) also see Heart Anatomy below  o Tricuspid  o Pulmonic  o Mitral  o Aortic
• Vena Cava –  o Inferior Vena Cava - large vein that carries de-oxygenated blood from the 
lower half of the body into the right atrium of the heart.  o Superior Vena Cava - receives de-oxygenated blood from the head and arms and chest and empties into the right atrium of the heart• Pericardium - the fibroserous sac enclosing the heart• Function  o Ejection Fraction - measurement of how well your heart is pumping  o Hypertrophy - abnormal enlargement of organ (as heart); excessive growth, not excessive cellsPressure Estimates (RVSP) - right ventricular systolic pressure is non-invasively measured by echocardiography; the pressure gradient between the right ventricle and the right atrium, to the pressure in the right atrium. 
Heart AnatomyThe heart weighs between 7 and 15 ounces (200 to 425 grams) and is a little larger than the size of your fist. By the end of a long life, a person's heart may have beat (expanded and contracted) more than 3.5 billion times. In fact, each day, the average heart beats 100,000 times, pumping about 2,000 gallons (7,571 liters) of blood.Your heart is located between your lungs in the middle of your chest, behind and slightly to the left of your breastbone (sternum). A double-layered membrane called the pericardium surrounds your heart like a sac. The outer layer of the pericardium surrounds the roots of your heart's major blood vessels and is attached by ligaments to your spinal column, diaphragm, and other parts of your body. The inner layer of the pericardium is attached to the heart muscle. A coating of fluid separates the two layers of membrane, letting the heart move as it beats, yet still be attached to your body.
The Heart ValvesFour types of valves regulate blood flow through your heart:• The tricuspid valve regulates blood flow between the right atrium and right ventricle. • The pulmonary valve controls blood flow from the right ventricle into the pulmonary arteries, which carry blood to your lungs to pick up oxygen. • The mitral valve lets oxygen-rich blood from your lungs pass from the left atrium into the left ventricle. • The aortic valve opens the way for oxygen-rich blood to pass from the left ventricle into the aorta, your body's largest artery, where it is delivered to the rest of your body.

                                                     Additional Tests:  Spirometry  Measures lung function and volumes and is helpful if additional lung problems are being looked at such as:  Asthma, COPD, Fibrosis.  Also noted:  FVC, FEV1, DLCO
CT or CAT ScanningCardiac Computerized Axial Tomography – a form of x-rays and beneficial to see detailed images of the lungs, heart and blood vessels i.e. Pulmonary Fibrosis or Vascular Disease
 Exercise Testing6 Minute Walk Test – wired for heart rate and pulse ox for oxygen level.  The test measures the distance a patient can walk quickly on a flat, hard surface in 6 minutes and reflects an individual's ability to perform daily physical activities. Cardiopulmonary Exercise Test – usually done on a stationary bike with electrode patches and an oxygen mask. The stress test measures how your heart responds to exertion. If you have an exercise stress test, you'll either walk on a treadmill or ride a stationary bike while the level of difficulty increases. At the same time, your EKG, heart rate, and blood pressure will be monitored as your heart works harder. Doctors use a stress test to evaluate whether there is an adequate supply of blood to the heart muscle.
Additional Information
Testing: Electrocardiogram (EKG)An EKG (also ECG) is a painless test that uses electrodes placed on the skin to record the heart's electrical activity. The test provides information about your heart rhythm and damage to the heart muscle. An EKG can help your doctor diagnose a heart attack and evaluate abnormalities such as an enlarged heart. The results can be compared to future EKGs to track changes in the condition of your heart.
The Conduction SystemElectrical impulses from your heart muscle (the myocardium) cause your heart to contract. This electrical signal begins in the sinoatrial (SA) node, located at the top of the right atrium. The SA node is sometimes called the heart's "natural pacemaker." An electrical impulse from this natural pacemaker travels through the muscle fibers of the atria and ventricles, causing them to contract. Although the SA node sends electrical impulses at a certain rate, your heart rate may still change depending on physical demands, stress, or hormonal factors.

The Circulatory SystemYour heart and circulatory system make up your cardiovascular system. Your heart works as a pump that pushes blood to the organs, tissues, and cells of your body. Blood delivers oxygen and nutrients to every cell and removes the carbon dioxide and waste products made by those cells. Blood is carried from your heart to the rest of your body through a complex network of arteries, arterioles, and capillaries. Blood is returned to your heart through venules and veins. If all the vessels of this network in your body were laid end-to-end, they would extend for about 60,000 miles (more than 96,500 kilometers), which is far enough to circle the earth more than twice!

Questions and answered followed and then we had a chat and meet and greet time.

                                   HAPPY HOLIDAYS EVERY ONE -- SEE YOU NEXT YEAR

September 22, 2012

​The meeting was opened by Merle Reeseman, Support Group Leader, with a disclaimer that the information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away.
The pharmaceutical reps and clinicians introduced themselves and a Thank You to Acetelion for our lunch.

•Envelope of Hope for new comers – an info packet will be mailed out to you
•Tickets are still available for the Afghan of Hope we need to raise money for research.
•Our 6th Annual Walk & Roll was another success and held August 4th – also to raise funds for R/A; We’re still accepting donations.
•As a reminder, we only have until November for the PH Research and Education Bill HR 1810 – and the similar Senate bill S 775. August is the month the politicians are in their home turf so give them a call…Reps. Ryan and LaTourette have co-sponsored the bill.
•PHA had their 10th Annual International Conference this June 22nd to the 24th in Orlando, FL; I do have some info on it. I will share that in a bit.

​Our  presentation for the day:                   Chronic Illness and Anger    
Pat Turnbough Actelion

•May have loss of some relationships
•Family doesn’t under stand
•Loss of job and feeling of normal

Passport to new chronic illness – which can be a result of grieving

Definition of anger – what we need to know
•Recognizing that anger is a normal emotion
•Can affect both patient and caregiver
•We need to learn how anger affects our mood and our body
•Learn various types of anger and what triggers set it off

ANGER
Is a natural response to a threat or strong feeling of displaced hostility. Turned inward can also be described as depression; which is a normal reaction but if it is out of control it becomes destructive and destruction can destroy relationships, communication with anyone and think of road rage.

D in front of anger spells what……

Our Bodies and Anger
Mind – body – reactions
•It effects the nervous system
•Muscles tighten up
•There is a release of adrenaline
oSkin flushing
oMuscles tighten up
oHigh pulse rate and bp rises

Variety of Anger (types)
•Behavioral – aggressive both physical and emotional
•Passive – ignoring others
•Verbal – always or can be sarcastic
•Volatile – comes and goes
•Chronic and ongoing and can compromise the immune system
•Overwhelmed – not being able to handle “it” anymore
•Constructive – positive type of anger – taking the next step

Chronic illness and anger triggers….
•Why me
•My life has been interrupted with a chronic illness
•It isn’t fair
•Loss of physical abilities
•Fear may set in
•Feeling of loss of control
•Unmet needs
•Comments from others can be hurtful

Know your anger
•View anger as an opportunity
•Know what actions you take when you are angry
•Choose a good form of expressing yourself
•What is it you want
•You may need help and do not be afraid or ashamed to ask
•Do you stay angry for long
•Does my level of anger outweigh the issue
•Do I hold my anger in until I explode and if so does it affect my health
•Are you misdirecting your priorities

A practical approach to managing anger
•Release anger constructively
•Better personal expression
•Be realistic
•Change your environment
•Forgiveness – easier said than done at times
•Clarify the situation
•Stop and think before you speak
•Seek professional help

Thoughts, reflections, summary
•Do not defeat yourself – you can only change yourself
•The more you are at peace with yourself the more you will be with those around you.
•Anger is a normal emotion and there are many types of anger
•Recognize what anger may be and usually is a major problem
•Practice anger management strategies
•Seek professional help if needed

Questions and answers followed.

  * * * * * * * * * * * * * * * *  

May 5, 2012

The meeting was opened by Merle Reeseman, Support Group Leader, with a disclaimer that the information shared is for support and not medical advice. She reminded participates to keep shared information private. A moment of silence was held for those who have passed away. Clinicians and pharmaceutical representatives introduced themeselves.

Announcements• April 28th was the 2nd Annual Ellie Godina Memorial Walk in Euclid, OH.• Tickets for the Afghan of Hope still available: tickets are $5 each or 3 tickets for $10• The 10th PHA International Conference is June 22 – 24 in Orlando, FL. • Our 6th Annual Walk & Roll at Akron State Park: August 4tho Registration is $25/individual; $40/familyo It is a fundraiser for RESEARCH and AWARENESS of the Dastardly Disease. o Walk for 6 minutes, followed by a great picnic, entertainment as well as a PH presentationo Table of information -- pharmaceutical companies, PHAo Zahi and family will be doing the cooking (ribs, kabobs, & more)• With our walk in August we will be able to have the support meeting on September 22nd.• Lunch was provided by Actelion Pharmaceutical Other BusinessTom Lantos Pulmonary Hypertension Research and Education Act of 2011 could save thousands of lives. We onlyhave until November to get the bill passed. Congressman LaTourette and Congressman Ryan have co-sponsored this bill, 
HR 1810. Please call and thank them. Urge your representatives to support and sign the bill. We also need to contact our Senator to co-sponsor S775.May 5th (meeting day) PH is celebrating “World PH Day,” started by the Nationwide Hypertension Association of Spain. They are on Facebook, please make a comment on FB.Next Support Meeting - July 21st is our next meeting. It begins at 11 a.m. to accomdate the library’s summer hours. The guest speaker will be a PH doctor

 :Featured Presentation
Caring Voices Coalition
with speakers Jean Lua, Director of Patient Services and Rachel Kelly, Business Analyst
1. Caring Voice Coalition: Caring Voice Coalition is a national charitable organization that provides the following patient services to those who are insured and underinsured.
2. Financial Assistance Program: provides financial assistance to eligible patients in order to help cover the cost of high insurance premiums or expensive prescription therapies.
3. Insurance Education and Counseling Program: provides specialized insurance assistance and guidance to patients.
4. Patient Support Program: assists patients in managing changes that impact their emotional, financial, and social well being. We understand that coping with a chronic illness can often become overwhelming.
Caring Voices Coalition started with a video about the new health care plan run by the federal government. The video is available for viewing on line at the Kaiser Family Foundation: http://healthreform.kff.org.
Caring Voices Coalition offers an online magazine. For more information about the organization, visit their website at http://www.caringvoice.org/. 

Thank you Marilyn for doing these notes.
                                                            ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ 
March 10, 2012 

Welcome to all and introduction of pharma reps and clinicians - A big thank you to Accredo for providing our lunch for the day.
A moment of silence was given to remember those who have departed this life, for those who are breathing easier and are no longer suffering. To all in the PH Community.
Special announcements -- birthdays, wedding anniversaries etc. 2 birthday wishes
Merle announced • on April 28th The Ellie Godina Memorial Walk Starting at 9 a.m. ‘til noon. - Lakefront Community Center
PHA is having their 10th Annual International Conference this June 22nd to the 24th in Orland, FL, Merle has info you are interested in attending.
• Tickets are still available for the Afghan of Hope we need to raise money for research and awareness. $5 each - 3 for $10.
• 6th Annual Walk & Roll will be this August 4th – still in the park and parking just for us. This is also to raise funds for R/A; Merle mentioned if we get a jar and put a $1 in it a week or $5 a month; or our loose change daily we will have some funds to donate. Our Walk & Roll is a fund raiser as well as a social.
• We only have until November for the PH Research and Education Bill -- HR 1810 – and the similar Senate bill S 775. Call your Reps and Senators – have friends and family do the same. Reps. Ryan and LaTourette have co-sponsored, be sure to thank them.
• Don’t forget the time change tonight… that must be a sign of spring. The change from standard time to daylight saving time is at 2:00 a.m. – or before you go to bed. Clocks should be set FORWARD 1 hour.

Merle then introduced: Rosalia Rozsahegyi, ATR-BC, Art Therapist
Linda Merriam, Business Development Specialist

ACCESSING THE SELF THROUGH CREATIVE EXPLORATION
THE HEALING POWER OF ART

Receiving a diagnosis of pulmonary hypertension is a life-altering event. The normalcy of everyday life is shattered, and there may be a heightened sense of uncertainty and failure. It can seem as if one’s whole world has fallen to pieces. Words may be inadequate, or unavailable.
Making art can help.
Why? Because making art encourages individuals to ask questions, seek answers, and figure out how to solve problems. It encourages them to make small and large decisions, and express ideas and opinions. It encourages them to create something that is meaningful and, maybe share it with others. It encourages them to have fun! In short, making art helps people be involved in living!

WHAT IS ART THERAPY?

The American Art Therapy Association states: Art Therapy is a mental health profession that uses the creative process of making art to improve and enhance the physical, mental and emotional well-being of individuals. 
It is based on the belief that the creative process involved in artistic self-expression is central to healing and wellness. 

ART THERAPY IS A HIGHLY CREDENTIALED PROFESSION

Art therapists are trained in counseling as well as fine art. Registered Art Therapists (ATR) receive a master’s degree in Art Therapy and obtain 1,000 hours of supervised clinical work. Board certified art therapists (BC) also sit for and pass a national exam.

WHY ART THERAPY?

Individuals who are diagnosed with a very serious illness that will affect the way they live are often not able to process what they are feeling. Art therapy provides a way for individuals to express feelings, channel anger, and relieve depression. It can help a person sort through pain and confusion, replace loss with growth, and strengthen self-esteem.

More than just “arts and crafts”

     Art therapists are trained in counseling as well as fine art. They invite, guide, and encourage individuals to rediscover themselves through the art-making process. They take into account each client’s needs to provide a positive experience that supports each individual’s creativity. The emphasis is on process of making art, and control is left in the hands of the artist.

    Art therapy can be an especially rewarding means of expression for those with physical, cognitive, and emotional special needs. And for those for whom words are inadequate or unavailable.

    Art therapy provides an expressive outlet for feelings that may be too difficult to put into words.

    Art can be about expressing feelings through color or lines, or expressing pain through images, or expressing love and caring. Or it can be about wishing you were somewhere else.
The art therapist nurtures a trusting relationship with the individual so that he or she can process his or her feelings and express them.

    The benefits of Art Therapy are many. It can provide a refuge from intense emotions associated with illness and treatment, divert attention away from diagnosis, treatment, pain, and disability, reduce stress, provide an outlet for emotional exploration and expression, help individuals regain a sense of control, provide a non-threatening medium through which an individual can confront and integrate the injury or illness into their life story, assist in the emotional adjustment to a life-changing injury and illness, help build a positive identity that is not defined by the illness or disability, provide a sense of accomplishment, help build self-esteem, and provide pure enjoyment, relaxation, and enrichment, thus enhancing the quality of life. 

   Art Therapy also supports cognitive and physical rehabilitation goals including increasing attention span, concentration, and memory, improving problem solving skills and decision making, increasing ability to learn new information, and improving fine and gross motor skills. 

Recent studies demonstrate the benefits of Art Therapy.

1. A recent study at Chelsea & Westminster Hospital, UK, (Staricoff) showed that patients who received art therapy had: 
better vital signs, 
diminished cortisol related to stress, 
less medication needed to induce sleep and relieve pain, and 
reduced hospital stays. 

2. A survey of patients receiving art therapy at the Cleveland Clinic Taussig Cancer Center in 2009 showed that: 

• 54% of the patients said they were less aware of their physical pain while involved in art therapy.
• 100% said they felt less stress after art therapy.
• 100% of patients said art therapy offered them an emotional outlet.
• 100% of patients enjoyed the experience.
• 100% of patients would participate again.

3. A “Patient Satisfaction Survey” conducted in 2011 at the Louis Stokes Cleveland Veterans Affairs Medical Center Spinal Cord Injury unit indicated high program satisfaction: 
• 84% reported relief of distress, anxiety and tension.
• 72% said AT helped them discover new creativity or new ability.
• 70% indicated increased the use of their hands and fingers.
• 50% indicated improved overall coping.
• 90% would do it again.
• 95% would recommend AT to other veterans.

ABOUT ART THERAPY STUDIO

As the oldest program of its kind in the United States, Art Therapy Studio has been providing therapeutic art programs for individuals and agencies throughout the greater Cleveland area for over 45 years. 

Art Therapy Studio was founded in 1967 by George Streeter, MD, a psychiatrist/artist, and Mickie McGraw, ATR-BC, an artist/therapist, in collaboration with Highland View Rehabilitation Hospital (now MetroHealth Medical Center) as a creative arts program to help patients and their families cope with the life changing effects of trauma, chronic illness, medical treatment, and permanent disability.

Both founders had used art as a way to cope with serious physical illness, he with tuberculosis and she with polio, so they had a personal understanding of the healing power of art.
Today we specialize in working with children and adults with special needs including physical disabilities, chronic illnesses, and developmental disabilities. Our mission is to enhance the quality of life for children and adults affected by spinal cord injury, brain trauma, stroke and MS through the therapeutic use of art.

We served 1,954 individuals in 2011.

Our Programs

Today we provide 
• inpatient hospital services, 
• on-site programs for community agencies, 
• professional education workshops,
• corporate wellness programs, and 
• “Discover the Artist Within You” community classes.

Inpatient Hospital Services

We provide art therapy bedside or in small groups to reduce patient anxiety, increase self-expression, and help the patient begin to cope with change after traumatic injury or illness.

MetroHealth Medical Center – brain injury, spinal cord injury, stroke, psych, sub-acute, other inpatient units; cancer outpatient unit 

Cleveland VA Medical Center –spinal cord injury inpatients, spinal cord injury outpatients at high risk for psych problems, palliative care, hospice 

Cleveland Clinic – Pediatric Hematology and Oncology Unit 

Onsite Programs at Community Agencies

We send art therapists to provide direct service anywhere from 2-10 hours per week on location at community agencies. We currently provide services for

Cuyahoga Board of Developmental Disabilities 

Brooklyn Adult Activity Center 
Southwest Adult Activity Center 
United Cerebral Palsy 
autistic and multi-handicapped school children at 
Mentor High School 
Westlake High School 

Professional Education Workshops

Once or twice a year we offer Continuing Education Units (CEUs) workshops for social workers, counselors, psychologists, occupational therapists, physical therapists, MR/DD facilitators, case worker, and activity professionals to learn about using art with clients.
Last April we presented a workshop on professional ethics titled “Professional Ethics: Creating Healthy Boundaries.”

•Corporate Wellness Programs 
Corporations are finding the arts to be a cost-efficient way to supplement their preventative health initiatives. Art therapy can reduce workplace stress, improve productivity, spark personal creativity, help employees become more self-aware, 

•social interaction between employees, and develop deeper connections between employees. 

Art Therapy Studio can also serve as an adjunct to Employee Assistance Programs by providing art therapy services for employees dealing with personal issues such as divorce, separation, personal loss, bereavement, and addiction. 

Current clients include Acumen Solutions, Calfee Halter & Griswold, and Medical Mutual.

“Discover the Artist Within You” Community Classes

We offer weekly classes, usually 10 classes per quarter, at four community studios located at

Fairhill Center 
Ursuline College ArtSpace 
River’s Edge – St. Joseph Center 
MetroHealth 

  The classes work with a variety of media including watercolors, acrylics, clay, collage, drawing, mixed media, and more.

  Participants include anyone with special needs (physical, emotional, cognitive), caregivers, retirees, and people looking for art instruction in a supportive environment.

NO experience in art is necessary!

  Some classes are specifically designed for special populations such as individuals with multiple sclerosis and stroke survivors. Class members in our MS class enjoy socializing with each other, exchanging information about doctors and treatment, and knowing that the others in the class share the disease even though they are at different stages

HOW CAN WE HELP YOU?

Take a class!
Beginners are welcome! 
NO experience is needed 
All supplies are included 
Scholarships available for those in need 
We can design a class specifically for those affected by pulmonary hypertension.

HOW YOU CAN HELP US?

Spread the word 
Refer us to a health care professional or community agency decision maker 
Invite us to do an employee wellness workshop at your company 
Volunteer on a committee 
Donate cash, art supplies, or equipment 
Attend our annual fundraiser 
ArtLoan showcases our circulating collection of over 250 works by prominent Cleveland artists as well as some of our clients. The art is available to rent for your office or home. 
Dessert Competition features sweat delights created by 35 competitors. 
You can learn more about Art Therapy by visiting their website at www.arttherapystudio.org .

During and after the above presentation we not only had phun being creative but we had an additional sharing time -- an emotional time in expressing what our creations meant to us -- how this dastardly disease has impacted our lives and how it has impacted our loved ones lives as well. Thank you ladies for giving us a creative and beneficial outlet.

Our next meeting is May 5th.
                                                                   % % % % % % % % % % %

2017 Meetings

July 8, 2017

Larry mentioned the PHA upcoming On The Road in Pittsburgh which will be on September 9th, the same day as our next meeting
Merle told of Nicole's Golf Outing, the 5th and final.  Her friends/phriends and family raised $50,000.
The speaker for the day, Stacey Martin of UH.

LUNG TRANSPLANTATION
Stacey Martin, RN, BSN
University Hospitals Case Medical Center

Outline of today's discussion

University Hospitals Lung Transplant Program
Lung Transplant team members and roles
Lung transplant phases
Common indications for lung transplant 
Lung transplant exclusion criteria
How lungs are allocated 
Donors
Post transplant course

University Hospitals Lung Transplant Program
First lung transplant performed at UHCMC in 1999
Period of inactivation due to loss of surgical team
Acquired Benjamin Medalion and Basar Sareyyupoglu both experienced thoracic transplant surgeons. 
Program re-opened in 2015
Our lung transplant program was granted CMS certification on March 8th 2017

Lung Transplant Team
Transplant Surgeons:
Soon Park, MD
Salil Deo, MD
Benjamin Medalion, MD
Basar Sareyyupoglu, MD
Yakov Elgudin, MD

Transplant Coordinators:
Rachel Kayatin, RN
Alexandria Bojansky, RN
Stacey Martin, RN
Barbara Demagall, RN, Nurse Manager
Lisa Willis, RN, Asst. Nurse Manager
Transplant Pulmonologists:
Robert Schilz, DO, PhD, Director
Maroun Matta, MD
Financial Coordinators:
Cynthia Porter 
Tom Barrett
Social Workers:
Paul Bate, LISW
Dietitians:
Jessica Benavides, MS, RD, LD
Jennifer Kerner, MS, RD, LD
Pharmacist:
Rob Barcelona, PharmD
Two more coming in August 2017
Program Assistant: 
Shenee Dantzler

The Transplant Team
During evaluation the patient will meet:
Transplant Surgeon
Physician who performs the transplant surgery
Determines if the surgery can be performed
Transplant Pulmonologist
Physician who is an expert in treating advanced lung disease
Will review your overall health and test results
Social worker
Professional who helps patients and families understand and cope with a variety of challenges including social support, insurance, transportation, housing, emotional issues, etc.
Transplant Coordinator
Registered nurse who helps you throughout the pre and post transplant process. They will monitor your labs, tests, and medications 
Transplant Dietician
Professional that will provide you with resources on what kinds of food you can or cannot eat. They will assist you in reaching and maintaining a healthy weight  
Financial Counselor 
Professional who works with patients and families to understand and coordinate the financial/insurance aspects of transplant

Lung Transplant Phases
Referral
Referral can be made by :
Pulmonologist
Cardiologist
Primary Care Physician
Self Referral
Must have a disease process that warrants transplant but be physically and mentally well enough to undergo the procedure

We never see anyone too early!
Unfortunately some people are referred too late in their disease process

Pulmonary Arterial Hypertension
Cystic Fibrosis
Idiopathic Pulmonary Fibrosis
Emphysema/ Chronic Obstructive Pulmonary Disease (COPD)
Bronchiectasis 
Alpha 1 antitrypsin Deficiency
Eisenmenger’s Syndrome  
Occupational lung disease
Other end stage lung diseases may be evaluated on a case to case basis

Evaluation and testing
Blood / Urine Tests
V/Q Scan
Heart Catheterization
Left to assess for blockages
Right to assess pressures and fluid status
Other testing
Mammogram, Pap test
Prostate test
Dental examination
Chest CT
Sinus CT (CF patients)
Echocardiogram
Gallbladder U/S (CF patients)
Pulmonary Function Testing
Spirometry, total lung volumes, DLCO and 6MWT
Infection prevention
Vaccines may be necessary

Exclusion Criteria 
Lack of adequate medical insurance
Inadequate coverage for prescription medications
Inadequate coverage for transplant care
Multiple system organ dysfunction and/or failure
Severe weakness, frailty, osteoporosis
Uncontrolled diabetes
BMI greater than 30 or less than 18 (relative contraindication) 
Non-compliance with healthcare recommendations
Non-prescribed drug and/or alcohol: use or dependence
Tobacco use: all forms of nicotine (cigarettes, gum, patches, chew)
Psychosocial issues, lack of support system, major mental illness
Recent history of cancers
History of communicable disease such as HIV and hepatitis

The Evaluation Process
Patient Selection Committee
Committee members are all of the members of the Transplant Team 
Each patient’s suitability for transplant is discussed in full detail
All test results and consults are presented
Possible outcomes of the Selection Committee meeting
Approved: should be placed on the waiting list
Not Approved: will not be placed on the waiting list
Deferred: evaluation will continue because we need more information before approval can be granted 

The Listing Process
The Ohio Solid Organ Transplant Consortium (OSOTC)
After approval by UH Selection Committee
Prior to placement on the waiting list
Candidate must be approved by OSOTC
Transplant Coordinator will submit candidate’s information to the OSOTC Clinical Review Board for Lung Transplant

Listing and Waiting for Transplant
Waiting List
Duration of wait time is difficult to predict
Days to years 

Allocation of lungs based on several factors
Blood type compatibility
Lung size between you and the donor
Lung Allocation Score (LAS) 
Disease process
Test results

The Waiting List UNOS: United Network for Organ Sharing

UNOS manages the nation’s organ transplant system
The UNOS Waitlist does not discriminate based on race or financial status
Information needed to place you on the list
Name, date of birth, height, weight
Blood and tissue type
Medical urgency, Lung Allocation Score (LAS)
Clinical data
Duration of wait time is difficult to predict
Days to years


The Waiting List: Lung Allocation Score (LAS)

Uses your individual medical information to 
estimate severity of need for transplant 
and your chance of success after transplant surgery
LAS 0-100
Higher score is given higher priority
Updated every 6 months, or more often if your condition changes

Medical Information needed for the Lung Allocation Score
Type of lung disease
Age, BMI (height and weight)
Pulmonary Function Tests (PFTs)
Blood tests
Oxygen need (at rest)
Arterial Blood Gas results
Need for ventilator (breathing machine)
6 Minute Walk Distance
Right heart catheterization test results

Pulmonary Hypertension Exception

Severity of disease in PAH patients may be underrepresented by traditional LAS
Exception can be requested to Lung Review Board for candidates who meet the following criteria:
Patient that is deteriorating on optimal therapy and
Must have right atrial (RA) pressure greater than 15mmHg or cardiac index (CI) less than 1.8

Multiple Listing Option
Patients have the right to be listed at more than one transplant center
This may increase chances of getting a transplant
Transplant Coordinator will give you information about multiple listing
UH and Cleveland Clinic share in the local donor pool
Candidates must be within a 4 hour drive time of UHCMC
Candidates must notify us if you decide to list with another transplant center

Waiting for a Lung Transplant

Patients must complete regular and routine testing in order to remain active on the transplant list
Appointments with lung transplant physicians
Lab tests
Nicotine and/or drug screens, if necessary
Pulmonary function tests (PFTs)
6 Minute Walk Tests
X-rays, CT scans
Meetings with your transplant team
Routine tests and wellness visits

Waiting for a Lung Transplant
Be prepared
Make sure your transplant coordinator is able to reach you at all times, day and night
Notify your nurse coordinator if you are traveling more than 2 hours from UH Cleveland Medical Center
Make sure you always have enough oxygen supply for a round trip visit to Cleveland
Bring medications
A family member or friend should always be on call to drive you to Cleveland

The Transplantation Process
Lungs may become available at any time…
day or night
365 days / year
We will try to contact you for at least 1 hour before moving on to another patient
If we can’t reach you, we can’t transplant you!

Receiving the Call!
When you are offered an organ, the Coordinator will call you 
Assess your current health status
Nothing to eat or drink
Instructions for taking any medications, blood thinners, etc.
When to come to the hospital- you must have a driver
Remember this is a long process and you may have to wait based on many factors
Travel distance for the organ
Time spent recovering the organ
Schedules may change; be patient and flexible
“DRY RUN”: If at any time the lung(s) does not look good enough for you, we will cancel your transplant.
You will not lose your place on the transplant waiting list

The Transplant Event: Donors
Matching of a lung (or lungs) is based on several factors
Blood type compatibility
Location, distance from UHCMC
Body size (height and weight) compatibility
Other specifics that the doctor may consider at the time of selecting a donor that best meets your needs
Donors will undergo testing to make sure their lungs are healthy
chest x-ray, CT scan
blood gases
bronchoscopy
blood and sputum testing

Types of Lung Donors
Brain death (BD)
Donation after circulatory death (DCD)
Public Health Service (PHS) Increased risk donors 
Each type of donor may be offered to you
*You have the right to refuse transplant at any time.

PHS Increased Risk Donors

Increased Risk Donors - Behaviors indicating increased risk
People who have had sex with a person known or suspected to have HIV, HBV, or HCV infections in the preceding 12 months 
Men who have had sex with men (MSM) in the preceding 12 months 
Women who have had sex with a man with a history of MSM behavior in the preceding 12 months 
People who have had sex in exchange for money or drugs in the preceding 12 months 
People who have had sex with a person who had sex in exchange for money or drugs in the preceding 12 months
People who have had sex with a person that has injected drugs by intravenous, intramuscular, or subcutaneous route for nonmedical reasons in the preceding 12 months 
People who have injected drugs by IV, IM, or subQ route for nonmedical reasons in the preceding 12 months
People who have been in lockup, jail, prison, or a juvenile correctional facility for more than 72 hours in the preceding 12 months 
People who have been newly diagnosed with or have been treated for syphilis, gonorrhea, chlamydia, or genital ulcers in the preceding 12 months 
People who have been on dialysis in the preceding 12 months

Expands the pool of donors available to you
Voluntary

Surgery
Single lung
the surgeon will make a thoracotomy incision on that side
Double lung
the surgeon will make a clam shell incision 
Patients may be placed on a cardiopulmonary bypass machine which will mechanically take over your heart and lungs for a short time
Native lung(s) will be removed and your new lung(s) will be placed and connected to your airways and pulmonary blood vessels

Post Transplant Complications
Potential Complications of Lung Transplant Surgery
Bleeding 
Blood Clots
Infections
Rejection
Stroke
Respiratory failure with need for mechanical support
Side effects from anti-rejection medication

Complications can occur during or after the surgery 
May requiring re-operation or re-admission to the hospital 

Post-Transplant Hospital Stay
SICU
Breathing Tube/ Ventilator
Chest Drainage Tubes
Intravenous lines (IVs)
You will be in the SICU for as long as it takes you to recover
You will then be moved to a cardiothoracic surgery floor
Home-going Education
Medication – you will fill your pill organizer and learn the side effects of your immunosuppression medication
Physical therapy will assist you in building and regaining your strength
Dietitian – Healthy eating habits and choices for optimal recovery and wound healing
You will be seen by the transplant team daily
Usual length of stay is 14-21 days

Post-Transplant Medications
Rejection
Most likely 3-6 months after transplant
Can happen any time
Can be treated and reversed when caught in time
Anti-rejection Medications 
Prevents your immune system from attacking your new lung (“immunosuppression”)
Must be taken every day for the rest of your life
Your body can reject your new lung even with anti-rejection medication

Anti-rejection medications increase risk for infection
Wash your hands
Avoid crowds
Avoid sick people
Practice food safety
Keep up with all your immunizations
Other medications that may be prescribed:
Antibiotics
Antivirals
Antifungals
Blood Pressure 
Cholesterol
Diabetes 
Medications for other medical conditions such as depression

Post-Transplant Preventative Care
Malignancy (Cancer)
Increased risk of developing malignancies due to the effect of immunosuppressive medications.
1/100 people will get a cancer or cancer-like condition
Skins cancers and cancer of the lymph system are the most common malignancies 
Skin cancer prevention
START NOW!!
Sunscreen SPF 50 on all exposed skin
Sun hat, long sleeves, avoid “heat of the day”

Post-Transplant Going Home
Things to Remember
Avoid crowded places for the first 3 months
Avoid people with colds and infections
Apply sunscreen when outside
Avoid gardening and mowing the lawn
Wash all fresh vegetables and fruit before eating
Cook all meats thoroughly 
Wash all cuts with soap and water – watch for infections
If you have a cat – someone else should change the litter box
Avoid having as pets
Birds, snakes, lizards, frogs
Every morning record your vital signs. Bring your diary to clinic for follow-up appointments
DO NOT let your medications run out

Follow-up Appointments
Once or twice a week for 4-8 weeks

Questions and Answers followed
Thank you to the PHA Central Fund for providing our lunch.
************

March 18

Our Scheduled speaker was unable to attend our meeting but he did send on the information.  We had an awesome Q's and A's just among ourselves


PFT's and What They Mean
Jose Ramos, BSRC, RRT, Manager at Respiratory 

Spirometry (Hard blowing out test)
This test helps us determine if some may have an obstructed component to their breathing (like asthma or COPD). I also lets us know how well the lungs are functioning. Very patient effort dependent.

DLCO (Gas Exchange)
This is the test where you hold your breath for 10 seconds. We are giving a small amount of mixed gases in order to evaluate how well your lungs exchange gas "oxygen"

Lung Volumes/Body Box
The chamber that everyone fears. We use this to measure the air that doesn't leave your lungs called Residual Volume, the box helps us segment lung and determine someone's true Total Lung capacity. The panting part is what tells us how much air is in the lungs after a normal breath is exhaled. There is a different method but it's not as accurate especially if the lungs are diseased and it takes between 4-7 minutes vs the 45 seconds of the chamber. 

6 Minute walk test
This test helps the doctors determined not only what kind of shape and work a patient can do but it helps to titrate medication effectively as well. We also record the post heart which correlates with heart disease if the heart rate doesn't recover in a timely manner.







2016 Meetings

September 10, 2016

Some of us have been dealing with Pulmonary Hypertension for several years and then they are those of us who are newly diagnosed and have questions. 
Let's learn and share when Dr. Neal Chaisson from the Respiratory Institute; who deals with Pulmonary & Critical Care Medicine at the Cleveland Clinic; he will do a presentation on:

Pulmonary Hypertension – Understanding the basics.​

Questions and answers followed

Thank you Actelion for sponsoring our lunch of the day and to Bayer Cardiopulmonary Division for sponsoring our speaker

July 16, 2016

Join us when Dr. Thomas Gildea of the Cleveland Clinic, will do a presentation on:

PULMONARY DIAGNOSTICS

After each presentation we will have an informal chat and snack time to share our stories or some other aspects of living with PH and how we deal with these issues. If you can, bring a helpful hint on what you do to make your PH life a little easier or bring a question or concern. 

Thank you Bayer, Inc. for sponsoring our lunch of the day. 




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