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 Merle Reeseman ~ Support Group Leader
2011 Meeting Notes

DECEMBER 3RD, 2011 

Welcome and introduction of pharma reps and clinicians - Thank you to Actelion for providing lunch.
A moment of silence to remember those who have departed this life, for those who are breathing easier and are no longer suffering
Special announcements -- birthdays, wedding anniversaries etc.
5th Annual Walk & Roll was on September 10th. PHAssociation presented our group an award and Dr. Raed Dweik spoke to us on: The Past, Present and Phuture of PH. Next year’s walk will be either Aug 4th or Aug 25th
Drawing for Afghan will be delayed -- we need to get more tickets sold - $5 ea or 3 for $10
PHA support line 1-800-748-72741-800-748-7274 If you need to talk with someone who understands
Don't forget HR-1810 – call reps and senators for S 775 to co-sponsor
On November 6th, CCF had their 8th Annual Summit: Translating Discoveries In to Patient Care. 
March 10th – next meeting 

Presentation of the day: EXERCISE AND PAH - Robert Schilz, D.O., Ph.D., 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. 

What's New - Freedom M (Oral Remodulin Study) – results have been rather disappointing. Oral is not the efficacy of IV
Imatinib (Gleevac) – has been shown to be effective but not near what was expected in the beginning.

Exercise has been shown to be beneficial in PAH patients. See PHA website for other general recommendations.
Benefits: 
1. Better conditioning
2. Psychologically feel better
3. Weight control
4. Strengthening and endurance
5. Osteoporosis prevention
6. Improves core strength and balance
7. Increased mobility
8. Improves HDL cholesterol
9. Improves diabetes
Benefits of Cardiopulmonary Rehab
1. Supervised
2. Regular schedule
3. Education
4. Aerobic and weight training
5. Individualized program
6. Support of patients with similar problems

PAH patients are usually sob due to more than one factor. Other reasons for exercise intolerance include: deconditioning, weight, hypoxia, PAH, other lung disease. Some things you can change and some you can’t. But if you modify some of these, for example deconditioning, you will feel better. 

Study in Germany in 2006 – Exercise and PAH: Studied 25 patients over a 15 wk period. At the end of the 15 wks., patients who exercised walked 220 meters farther than those who did not exercise.
Amount of exercise – do not over exercise. Know the signs and symptoms. Stop if you experience any of the following: lightheadedness or dizziness, chest pain, severe SOB, or severe fatigue. ALWAYS talk with your PAH specialist

Type of exercise: 
1. Light resistance – low weights and high repetitions (no heavy lifting)
2. Light to moderate aerobic (walking, swimming)
3. Inadvisable to do upper and lower extremity training (i.e. elliptical) at the same
Time unless the patient has little or no symptoms
4. If you have severe symptoms, may do seated exercise with arm bands, etc.
5. Avoid outdoor exercise in hot/humid weather.

Exercise induced PAH in patients with systemic disease such as scleroderma - may need to do exercise testing to be diagnosed.

Exercise can create a feeling of well being. Questions were during the presentation.
This Holiday Season remember Red, Green and Gold – foods red food (strawberries, raspberries, tomatoes, pomegranates contain antioxidants), green foods (also contain antioxidants-help immune system and both help blood flow) , and golden (nuts which are brain and mood food).

Happy Holiday’s Every One ~ every one of them :o)
Remember there is always hope and remember to smile, it’s contagious.
  % % % % % % % % 

July 10, 2011 

Merle is still recouping from her extended stay in the hospital and David had to work; so Janice was gracious enough to run the meeting. Welcome and confidentiality statement was read and a moment of silence was held for those in the PH community who now have their wings.

Introduction of clinicians and those from industry. A thank you to LungRx for providing lunch for the day. 
Announcements: 

•On the Road – June 11th in Pittsburgh, was a great success and I’m so glad many of you were able to attend.
•Rick had his double lung transplant in May and is doing well
•Tickets are now available for the Afghan of Hope $3 each 2 $5 – 6 for $10 If you can get phriends and phamily and write their name down on a piece of paper for the drawing in Nov (if I don’t make the meeting with the tickets)
•Walk for Ellie raised almost $32,000. Leah raised about $5000. in memory of her Mom, Amy, who was a member of our group. Leah and Jon spoke at our last meeting.
•On August 16th, UT will sponsor a dinner meeting from 6:30 to 8:30 at the Blue Canyon Kitchen and Tavern (The Lodge) 8960 Wilcox Dr., Twinsburg, OH 44087 Complimentary valet parking is available. Space is limit so make your reservation early. Call Jennifer Roe at 866-495-5053866-495-5053
•5th Annual Walk & Roll this September 10th - to raise funds for Research/Awareness. Save your loose change and put it in a jar or a dollar or two a week. Ask a friend/phriend for a donation. Ask10 people for $10 = $100 or 20 friends for $5. = $100 
•Our September meeting which was scheduled for the 24th had to be cancelled because of a commitment the Library has. It is rescheduled for October 8th 
•The November meeting at the Cleveland Clinic Summit will be November 6th thru the 8th, at this time I’m not sure which day is scheduled for patients.
•The PH Research and Education Act of 2011 is now out in both the Senate as S 775 and in the House as HR 1810 (the HR bill was introduced on May 10th) Please call your Senators and Congress folks to co-sponsor these bills
• Our next meeting – note date and time: October 8th, back on Saturday and at 1 p.m.

Janice introduced Nancy Bair a ph nurse specialist at the Cleveland Clinic
CAREGIVER: Novice to Expert 
Nancy D. Bair, RN, CNS-BC - Cleveland Clinic – Respiratory Institute 

Understanding your role
Not everyone is a nurse 
Role reversal 
Determine who is taking care of what and whom 
Recognize that these roles may change in time
Take the ball and run with it

Recognizing Signs of Stress
Fatigue
Feeling overwhelmed and irritable 
Feeling sad, resentful 
Physical signs—weight loss, change in sleep habits 
Losing interest in activities you used to enjoy

Anxiety and Depression
In PH patients: 35% of PH patients are in this category 
Nearly 16% have major depressive disorders
Over 10% have panic attacks
Less than 25% of patients are treated

Mental and Emotional Effects of Caregiving
Depression appears to be the most common psychological disorders
20% to 50% of caregivers report depressive disorder or symptoms
The higher levels of depression are mostly attributed to people caring for individual with dementia 
Studies show that 30% to 40% of dementia caregivers suffer from depression and emotional stress
Selected Caregiver Statistics – http://www.caregiver.org/caregiver 

Normal Reaction
Don’t be ashamed of your feelings
Don’t feel as if you have to do everything
Don’t be too hard on yourself
Think what you have accomplished – not what you haven’t

Support
Accept help
Information is a valuable tool
Be proactive
Join a support group

Stay connected
PCP/Counselor – talk with them 
Talk to your loved one’s physicians/ph nurse
Journaling – can be a comfort
Do Something for “YOU”
Tell Yourself: There will be better days
Celebrate – however you can
Bask in the sunshine 
Think of beautiful places 
Build a sandcastle
Take a walk with a friend
Marvel at the miracles in front of us everyday

Questions and answers followed along with our chat and snack time. 

Do to a conflict with the Library the September meeting was cancelled -- and renovations during October cancelled that meeting 

  DON'T FORGET THE CCF SUMMIT 11-6-11 ~~~ SEE YOU DECEMBER 1ST in Twinsburg  
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May 14th meeting notes to follow

Merle is still in the hospital, David Grady did the business part of our meeting and introduction of our guest speaker. The presentation for the day was Asthma or PH, Dr. Robert Schliz of University Hospital did the presentation -- he helped us learn what the difference is and how it applies to our lives. 
* * * * * * * * * * * * * * * * * * * * * * * * * * * * * * 
March 12, 2011- Cleveland Area PH Support Group Meeting

Welcome by Merle, the confidentiality statement was read; and a moment of silence was held for those who now have their wings.
Introduction of clinicians and those in industry. A thank you to United Therapeutics for providing lunch.

Announcements: 

April 9, 2011 the Ellie Godina Memorial Walk 10 a.m. Euclid High School – a fund raiser
May 15, 2011 Running Phor Amy – her daughter and son and others – a fund raiser
June 4, 2011 – HEART Ballet at the Byham Theater in PGH – a fund raiser
June 11, 2011 – PHA On the Road; a mini conference – it’s free; at PGH Airport, registration required. Go to PHAssociation.org to register
Envelope of Hope Questionnaire was handed out to be returned to PHA
Tickets available for the Lapghan of Hope
5th Annual Walk & Roll September 10th at Akron State Park
FDA has approved a new drug for Lupus – Bealysta
HR 1030 did not pass; as soon as a new bill is out we will need to start again contacting our Reps
Next meeting: May 14, 2011 with Dr. Robert Schilz – PH, Asthma or Both…

Merle introduced Dr. Michaela Aldred who is an Assistant Professor in the Genomic Medicine Institute, an Assistant Professor in the Department of Genetics at Case Western Reserve University School of Medicine and a member of the Taussig Cancer Center. Her research interests are in pulmonary hypertension and cancer. 
  PH and Genetics – What’s the Connection and What’s New
DNA is in all the cells of our bodies except the red blood cells. We can see chromosomes but can’t see the DNA. Chromosomes are how DNA is organized or the genetic code for DNA is organized into chromosomes – 46 total or 23 from each parent
The DNA code is made up of 4 letters (A, G, T and C) that are read in groups of 3 such as AGC, CTC, TTC, GAT etc – together, these 3-letter “words” make up the instructions for a protein. And each set of instructions is called a gene.
Genome – the Book of Life; is a recipe book of instructions to make the protein our bodies need. Chromosomes are like a chapter of a book. Genes are the recipe for one protein. DNA – the letters on the page, each group of three letters makes a “word”.
A mutation is a mistake in the genetic code. There are 3 ways it may occur.
1. A letter may be changed (spelling error). The end result is when you cook you end up with a product but it isn’t the same and as if you use a different ingredient: ie if you use malt for salt; or pea for tea – only one letter is changed but they are very different  AGC, CTC, TTC, GAT mutated to AGC, TCT, TCG, AT
2. A letter may be lost or missing (nonsense). It wouldn’t make any sense so it doesn’t know what to do. Such as the directions say “mix egg and milk then add to flour” and it’s changed to “mix ega ndm ilkt hena ddt of lour”.
3. There are no letters – the whole gene is missing. It is like a page fell out of the recipe book or the dog ate that recipe. So you have nothing to follow and you make the wrong thing. No gene – no protein.
Familial PH 
• In a small proportion of cases, more than one member of a family has PH.
• Genetic studies of PH families identified two genes: BMPR2 and SMAD9
• In families where PH occurs with another rare disease – hereditary hemorrhagic telangiectasia (HHT) – the genes ALK1 or endoglin are altered
They (Chromosome 2  BMPR2 and SMAD9  Chromosome 13) are like members of a relay team
• They receive a signal (the baton), then pass it on within the cell or to the next member.
• The end result is that the cell knows whether or not to proliferate.
• If a member of the team is injured (a mutation) this process stalls, leading to changes in the lung vessels. Possibly lung cell growth is too fast in PH. 
Non - inherited genetic changes. 
Now studying genetic changes in lung cells of patients who have had a lung transplant; those that do and do not have PH. They are finding there are some genetic changes that are not inherited but occur only in the lungs. They are finding changes happen in 1 of 3 cases and are looking to see how this might contribute to PH. 
Questions and Answers followed
Endothelial cells are wider in PH patients – touch blood in vessel
Someone may not have PH but have changes in their lungs that are leading up to PH.
She showed the chromosomes of a patient who had lost chromosome 13 and 20% of those cells had lost chromosome 13 completely.
Dr. Aldred showed another patient’s chromosomes where the middle of chromosome 13 was lost so it was shorter in length. It still had the end parts but the middle was lower and was omitted and the two end parts were joined together. 
It was noted that woman are more likely to get PH than men and it is not understood why at this time. BMPR2 mutations are identified in up to one fourth (25%) of idiopathic cases.
Each child has a 50% chance of inheriting the mutation. If they do have the mutation, their chance of developing PH averages about 20%. If they didn’t inherit the mutation, their risk of getting PH is the same as the general population (i.e. very low). 
  ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~ ~
2010 Meeting Notes 

The meeting for December 4, 2010 ~ Merle read the confidentiality statement and a moment of silence was held for those who are no longer suffering. She read all those who have passed on this past year: December of last year: Beverly Staltari; in January: Marlene Kersman; in June: Daniel Terriaco; July: Cheryl Arnett and in September: Mary Saum. Yesterday, Dave Stamp called to let her know that Amy passed away this past Wednesday. Amy and her daughter Leah were at our summer meeting – she has been in the hospital most of this school year. Amy was a biology teacher and about 18 months ago she received her Masters degree. Dave mentioned he would ask Amy to slow down some and finally realized she needed to keep on going. Dave mentioned donations can be made in her name to PHA
Introduction of clinicians and pharmaceutical reps and a thank you to CuraScript for sponsoring our luncheon of the day.
Announcements: Les Pollack is still in the nursing home; PHA has a new name for their Help Line -- now called the PHA Support Line 1 800 748-72741 800 748-7274. The Support Line volunteers provide information; contact point of a nearby PH clinician or support group, they really want patients and caregivers to know that a Phriend is only a phone call away. • On November 19th, CCF had their 6th Annual Summit, A practical approach to evaluation and management ~~ Zahi Kakish won the afghan • And Dorothy gave me a good pointer – she has one of those life alarms (around your neck or wrist) she also wears a coaches whistle… for at home, when at the other end of the house if you should fall and you have a caregiver/spouse – restaurant etc. •It was mentioned that HR 1030 and S 2803 may not make it this time around :o(
At our September meeting Dr. Thomas Gildea spoke to us on: “Lung Transplantation: the process –> the procedure – the Why, When and How” 
There are several criteria that determine the need to consider lung transplants. 
The transplant patient must be sick enough to require a transplant but well enough to survive one. Notes to be posted soon. 

Our presentation for the day: 

  What do our doctors expect from us?

Dr. Robert Schilz ~ Medical Director of Lung and Pulmonary Vascular Disease - University Hospitals Case Med
ical Center – told us what our doctors expect.  

Many of us know what we want to ask or expect from our doctors ~ 
What do they expect from us?
• It’s not always what we think the doctors or nurses want to know or hear from us, it’s also how we feel. Those “numbers” are important but how we feel is more important
 o Have an honest communication – tell the doctor (health care team) what is going on not necessarily what we think they want to hear
 o What are our expectations
 o Are there challenges – ask and tell
 o Trust and needs go both ways – Beliefs, Ethical Standards, Trust and Expectations
   We need to trust our health care team – they also need to be able to trust us
• In 1990 a law called the Self Determination Act influenced how doctors could act. This act gave patients the right to turn down care, participate in a plan for their healthcare, and determine in advance health care directives (i.e. Life support). This has led to increased consumerism and increasing insurance involvement. 
• There is increasing pressure on physicians to make decisions about time spent with patients as it influences the amount the doctor is reimbursed per hour.
• There are national guidelines for doctors.

Outcome
 o Are we satisfied, do we trust the interaction between the doctor and the patient
 o Are we both adhering to the program
Legal Issues
 o Access to medical information
 o What if any restrictions apply
 o Blood tests and others (echo, PFT’s) – prior to appointment
• Self determination of healthcare
 o Increasing consumerism – is it creating a problem
 o Increasing insurance involvement – managed health care
 o Increasing pressure on physicians and reimbursement to them
 o Increasing physician distrust – sensctioclization of medical errors

What involvement do we each expect?
 • What physician involvement do you expect
  o Who makes the decisions – do you have a parent/child relationship
  o Who has/is the authority figure
   Years ago the doctor told you – you did it
 • Remember those TV Drs. Kildare, Ben Casey and Marcus Welby
 • Now think about Grey’s Anatomy and HOUSE
 • These were all portrayed differently as time evolved to represent how patient physician relationships have as well.
   We were dictated by our physicians
• Collaborative relationship ~ preferred by most doctors
 o Now the patient knows more – asks more – is involved more
 o The patient and the doctor listen to and are talking to each other
 o Sometimes the doctor needs to “re-phrase” to understand what the patient is saying and v/v

Soooo, we are expected to show up at our appointments with
o Honest reporting of how we are ~ honest communication
 o We need to be involved with our health care
 o Let the doctor/nurse know if our medicine isn’t working as expected
 o Families need to be involved with your care and be familiar with the health care team.  
 o Utilize the health care team to assist in reaching your goals to obtain optimal health care
 o Are we willing to help with research and are we eligible
 o Do we smoke and if so how much – and we all know we shouldn’t
o Are we having any side effects ~ we might need to keep a journal
 o Too much fluid retention
   Is our fluid intake restricted
 o Are we following a low sodium diet
 o Too large a meal (holiday) – could we, should we use smaller plate size
   It might take an extra day or two to lose those extra holiday pounds
o Are we taking our medicines according to plan ~ adherence to therapy – follow advice given
 o Could the kidneys, heart or ph be compromised
o Did we have our pre-testings before doctors appointment
 o Ask what the tests are for and what are the results
 o Do you understand the test results and do you know why you are having them
 o Call and ask for results if the doctor doesn’t call you (phone works both ways)
 o Some doctors do not use email – again phone works both ways
o We need to tell about other medical problems and other medicines
 o Bring a complete list of all meds, dosage, times taken, frequency
 o Know what you are taking and what it is for
   Do not refer to medications as “that little yellow pill”
o Do we need to have other doctors involved – pap smear, colonoscopy, etc.
 o Ability to work with ancillary physicians
  Cardiologist; Rheumatologist; PCP for colds, flu, shots
o Be sure to ask questions
 o The doctor sets the course we are to follow
 o Tell the doctor when something changes – do not wait for the next appointment
 o Let the ph team be the judge of how serious your symptoms/questions are
 o Ask the doctor if he/she uses email – and the phone works both ways
 oBring a list of questions to the appointment and wait for answers
o Are we willing to be involved 
 o Education – we need to learn as much as possible about PAH
 o Appointment – be prepared – have a complete list of medicines
  Any needed refills required
 Are you will to help with research if you are eligible
o Mirror the kind of relationship you wish to establish with your PAH team
o Report all – have honest communication

Do you understand what the doctor is saying – does the doctor or nurse understand what you are saying. This is where it becomes a two way communication. Are we sending and receiving the same message 

Talking to your health care providers will
o Provide information -- not only to the doctor but also to the patient
o Make sure you let the doctor/nurse know of any major event or problem
 oDo not wait until next appoint and don’t make it an “oh by the way”
o Be sure to ask questions
o Take the information home with you
 o Best to bring someone along with you to appointments
o Follow up if needed
 o Interaction can be by phone and between visits
 o Report symptoms or problems in a timely manner
 o The phone works both ways – you do not always have to wait for the doctor to call you
o Mirror the kind of relationship you wish to establish with your health care team
 o What do you expect from them
 o What do they expect from you

After the presentation Merle had additional announcements: This Holiday Season remember Red, Green and Gold – about foods - red foods (strawberries, raspberries, tomatoes, pomegranates contain antioxidants), green foods (also contain antioxidants-help immune system and both help blood flow) , and golden (nuts which are brain and mood food).
UT has a trial for those who are NOT on IV meds. The Freedom Study. To let you know about the recent launch of www.pahstudy.com/advocacy , an online resource for patients with pulmonary arterial hypertension (PAH).
She also read her poem -- The Dastardly Disease which she hopes to make into a mini book.
Thanks to Janice, Roberta and Merle for these notes.
Our next meeting is March 12, 2011 ~ HAPPY HOLIDAYS everyone -- see you all next year

* * * * * * * * * * * * * * * * * * 
The meeting for September 11, 2010 opened with Merle welcoming all, reading the confidentiaity statement and having a moment of silence for those who have passed on. Since the last meeting Daniel Terriaco, Cheryl Arnett, and Mary Saum are now breathing easier. Merle also had a moment for those who lost their lives 9 years ago on 9/11 in NYC, western PA and Washington DC  
An introduction of all clinicians and those pharmaceutical representatives present with a special thank you to United Therapeutics for providing the luncheon for the day.
Announcements: 4th Annual Walk & Roll is next weekend. *PHA has a new Support Line (formerly the Help Line) it does have the same phone number and it is still a patient to patient call 1800 748-72741800 748-7274 -*Blue Lips Campaign will be now thru October to raise awareness and make a Guiness World Record -- * November 19th will be the Cleveland Clinic Summit, A practical approach to evaluation and management -- *Merle mentioned the donut hole rebae of $250. -- *Don't forget your flu shot, it's that time of year once again.
Merle mentioned how she spoke briefly before the UAW in May about PAH and also requested co-sponsorship for HR 1030 and S 2803. With that speech she was able to mail letters to 5 Congresspeople and 200 signatures to each Sen. Voinovich and Sen Brown -- time is running out so don't forget to call and ask friends and family to call or write.
Out next meeting will be December 4th when Dr. Robert Schilz will be our guest speaker
Prior to the speaker, Roger Towle, a member of the PHA Board of Directors made a special presentation. Roger became involved volunteering with PHA when his daughter Jocelyn was diagnosed with PH in 2002. He has served on the PHA Board of Directors since 2003 and as the PHA Treasurer since 2004. He and his wife Deb live in Grove City, PA and are a country mile or so neighbors of Merle. He says his real job is the Vice President for Financial Affairs at Grove City College. Roger was gracious enough to accept the Outstanding PH CItizen Award for Merle this past June while at the 9th International Conference in Garden Grove, CA. Merle was unable to attend. She asked Roger to say upon that acceptance: There is hope and remember to smile, it's contagious.
  Lung Transplantation: Why, When and How
Speaker for the day, Dr. Thomas Gildea a member of the transplant team of the Cleveland Clinic, where his clinical interests span the realm of advanced lung diseases, including lung volume reduction, lung transplantation, pulmonary hypertension and he is focused on advanced diagnostic and therapeutic bronchoscopy. Dr. Gildea was the first American doctor to do an electromagnetic navigational bronchoscopy. This new technology uses images from a CT scan of a patient to feed into a computer. This information dictates where electrode sensors should be attached to the patient’s chest wall. A bronchoscopy is then done with the ability to navigate the sensors to biopsy lung cancer.
He explained the reason we may need a lung transplant, when it would be necessary and the process involved. There are several criteria that determine the need to consider lung transplantation.
1. Declining 6 minute walk test results
2. Failing therapy with intravenous Flolan or equivalent (failing quality of life)
3. Lower extremity edema
4. Cardiac index <2 liters/minutes/square meter
5. Right arterial pressure > 15 mmHg
And there is the old transplant window – you must be sick enough to need a transplant and healthy enough to survive. The policy has changed within the last 3 or 4 years about who qualifies for a transplant. About 1/3 die while on the waiting list for a transplant.
 Disease Die Waiting
Emphysema 13.8%
PH 33%
PPH 30%
Cystic Fibrosis 2%
The Lung Allocation Score (LAS) is important – criteria noted on the web site www.OPTN.transplant.hrsa.gov (Organ Procurement and Transplant Network)
Now they accept donors who were not considered previously as there is a shortage. There has been an increase in using marginal donors and also lung volume reduction surgery for COPD. So, sicker patients and risker organs.
Donors need not to have smoked or used illegal drugs within 6 months or active malignancy within the past 2 years with the exception of basal cell carcinoma or squamous cell of the skin with EFS of 5 years. The higher the LAS score, the higher you are on the transplant list. Factors for the score include height, blood type and region of the country. The Cleveland Clinic patients have a better chance as The Clinic have a jet that will go 2000 miles to retrieve organs.
Patients are living longer due to much better therapy so there is not always a need for transplant until later in life. Shut off for transplant use to be 65 now it is well into the mid to upper 70’s.
Survival rate for those with Idiopathic Pulmonary Hypertension are:
 1 year 71%
 2 years 60%
 3 years 51%
 4 years 44%
 5 years 32%
Dr. Gildea also mentioned a patient survived 18 years
Doctors have learned two lungs are better for transplants as one lung or the new lung overworks as it is in better condition. Heart and lung transplants are uncommon as many types of heart problems or the right ventricle can repair itself after a lung transplant.
More than 80% have no problems after the first year of transplantation. Rejection is common and 40% is treatable. Anti-rejection medication is necessary. Pre-surgical counseling motivates every patient to take these necessary medications. Infection is a more serious problem and one type of possible infection is pneumonia. Other types of physical problems appear after transplantation. One half have high blood pressure after one year and renal dysfunction is another type. The risks are mentioned in pre-surgical counseling.
Transplantation is harder in the United States as we have many nationalities so our bodies are more diverse.
Questions and answers followed:
Living donors are not considered as there is too much long term risk for the donor. California had done one though. The donor could need a lung themselves later in life as a result of organ failure or a bad infection. 
If someone is on a ventilator, the Clinic will still do heart or lung transplants. 
A patient can be on more than one list. Michigan and Boston are popular one... 
Sometimes heart surgery is needed at the time of lung surgery, such as tightening a valve. Not all hospitals do that but Duke and the Clinic can. 
Lungs don’t repair themselves so stem cells are not being studied much for lungs. There is no money for stem cell research. 
The typical cutoff age for 2 lungs is 60 years. One lung is 65 years. The Clinic did a transplant on a 75 year old man. Pittsburgh did a transplant on an 81 year old man. 
Scarring will always be there but inflammation in lungs can improve. 
The average wait time for lungs at the Clinic is 90 days. 
Body Mass Index is an important consideration. The upper limit is a BMI of 30 and if an adult is less than 100 pounds they would not be considered. 
It is difficult for Pulmonary Hypertension patients to reduce their body mass index as they 
can’t exercise to lose weight- they have to control their diet. 
Pulmonary hypertension patients can donate eyes, kidneys, and their liver, assuming they are not damaged. Donors and patients are typed for many types of diagnoses. Hepatitis C donors may donate organs for Hepatitis C patients if that patient is close to death as there are variations of Hepatitis C. They are also talking about giving HIV patients organs now as their life span has increased. 
A DONOR SHOULD TELL THEIR FAMILY OF THEIR WISHES AND HAVE IT NOTED ON THEIR LICENSE. 
Thank you Roberta and Janice for taking notes of the presentation
Remember our next meeting is December 4th

  | \ / | \ / |

The meeting for June 13th 2010 started a little late - a quick setup was required  
The confidentiality statement was read and a moment of silence for those who are no longer suffering.
Introduction of clinicians and pharmaceutical reps and a thank you to United Therapeutics for sponsoring our luncheon of the day.
Announcements included:  
• A Woo Hoo moment – PHA has notified Merle she is the recipient for the Outstanding PH Citizen Award **see below
• Afghan – any more tickets needed – any tickets or funds to turn in
• Envelope of Hope for new comers – on the table up front – PHA will mail an info packet 
• The PHA 9th Annual Biennial International Conference ~ in Garden Grove, CA this coming June 25th thru the 27th 2010 “Riding the Wave to a Cure” **The OUTSTANDING PH CITIZEN award (a national recognition) will be presented at the conference. Merle will be unable to attend; a member of the PHA Board of Directors will accept it on her behalf. 
• Our 4th Annual Walk & Roll has been changed to September 18th - to raise funds for Research and Awareness – links are now on the site for donations and registration
• As of right now, November 19th will be a one day event for the CCF Summit
• Starting this week the health reform law gradually phases out the doughnut hole. This year, people who enter the coverage gap will receive the one-time $250 rebate check. Beginning next year, consumers in the doughnut hole will receive a 50 percent discount on brand-name drugs and a 7 percent discount on generics. The share consumers pay for both brand-name and generic drugs will decrease until the gap is eliminated in 2020, when consumers will pay the standard 25 percent of the costs for drugs while in the doughnut hole.
• Don't forget HR 1030 and S 2803 we need to call our politicians to co-sponsor these bills
• Our next meeting will be September 11th back to Saturdays and Dr. Gilda will speak to us about 
  "Lung Transplantation ~ Why, When and How."

Our presentation for the day: Dr. Richard Krasuski, Director of Adult Congenital Heart Disease Services, a staff cardiologist in the Department of Cardiovascular Medicine at the Cleveland Clinic also an Assistant Professor of Medicine at the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University and is certified by the American Board of Internal Medicine as Diplomat in Cardiovascular Disease. Dr. Krasuski received his medical degree from Harvard Medical School and completed a residency in internal medicine at Brigham & Women’s Hospital in Boston, Massachusetts and a fellowship in cardiology at Duke University Medical Center in Durham, North Carolina, where he received additional subspecialty training in adult congenital heart disease and pulmonary hypertension.  
In 2004 Dr. Krasuski was voted Researcher of the Year at Wilford Hall Air Force Medical Center in San Antonio, Texas and in 2005 he was the youngest finalist ever for the “Gold Cane Award” for academic, clinical and research excellence. In the fall of 2005 he joined the Cleveland Clinic and has since orchestrated a multidisciplinary program in adult congenital heart disease and collaborated on a multidisciplinary program in pulmonary hypertension.  

Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart disease (CHD) and greater numbers of patients with PAH associated with CHD (PAH-CHD) are now surviving into adulthood, many with increasingly complex cardiac defects. Let’s learn more when Dr. Krasuski will explain: 
  "Congenital Heart Disease and Pulmonary Hypertension ~ Plumbing and Beyond."  

Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart disease (CHD) and greater numbers of patients with PAH associated with CHD (PAH-CHD) are now surviving into adulthood, many with increasingly complex cardiac defects. 
Complications at birth:
Atrial Septal Defects
Tetralogy of Fallot
8/1,000 are complicated by heart disease, not including Atrial valve and mitral valve prolapsed.
800,000 adults with CHD are reported in the US and this increases ~ by 9,000 each year due to children surviving. 
Objectives:
1) Recognized and be treated
2) Newly diagnosed as adults are mostly with Atrial Septal Defects
Majority face a life time of problems such as:
  Rhythm disturbances
  Heart failure
  Need for re-operation
Adults think they are cured after surgery, as palliative methods can cause problems. 
RV (right ventricle) and TV (tricuspid valve) are ill equipped to handle systemic pressures. Shunt holes can result in Pulmonary Hypertension years later.
Right ventricle – Holy Grail of adult congenital heart disease.
It is shaped ellipsoidal- pyramid – crescent shaped- prism shaped – like a baseball mitt 
Left ventricle is oblong shaped – like a football.
Right ventricle can have problems with volume as associated with atrial septal defects- tetralogy of fallot – known as “Blue Baby”. Tetralogy of Fallot is classified as a cyanotic heart defect because the condition causes low oxygen levels in the blood, which leads to cyanosis (a bluish-purple coloration to the skin).  
Ventricular septal defect (hole between the right and left ventricles) as the valve leaks right ventricle becomes enlarged and volume issue and questionable to replace.
Narrowing of the pulmonary outflow tract (tube that connects the heart with lungs)
as a hole is present and narrowing under pulmonic valve.
Patients with Pulmonary Hypertension - 14% congenital population with congenital heart disease.
Patent Ductus Arteriosis: a vascular structure between the pulmonary artery and the aorta that normally closes shortly after birth, if it remains open will cause decrease in blood oxygenation; blood bypasses lungs – mixes with aorta and causes low saturations.
Eisenmenger syndrome (usually in infants and young children) is a left to right heart shunt caused by ventricular septal defect (a hole in the heart) and can lead to kidney failure, erythrocytosis (an abnormal increase in the number of circulating red blood cells), proteinuria (an excess of serum proteins in the urine) and elevated uric acid. This hole in your heart causes blood to flow or circulate abnormally in your heart. Oxygen-carrying blood (red blood) mixes with oxygen-poor blood (blue blood). The blood then returns to your lungs instead of going to the rest of your body, causing high blood pressure in your lungs. High blood pressure in your lungs (pulmonary hypertension), when significant, can cause serious medical conditions. Symptoms increase with increased pressures. 25% of children use to die at 5 years, now the median survival is 37 or longer. 
Catherization in Congenital Heart Disease patients outweighs the risk. 
Two cases were discussed and reviewed. One case involved an 85 yr old male with a history of Atrial fibrillation and moderate PAH. He had reactive pulmonary vasculature. A plug was implanted across the patent ductus. His functional capacity improved from WHO III to a WHO I to II with great improvement. 
Diuretics are used to assist to decrease the load on the right ventricle. There are now several medications used to treat PH. 
Cardiac catherization measures the cardiac index which is the amount of blood the left ventricle is ejecting out into the body which is a measure of the heart function. 
Questions and answers followed the presentation.
We then had our door prize drawing and our chat and snack time for sharing our thoughts, concerns and helpful hints.

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The meeting for May 15, 2010 opened approximately 1:00 pm. The confidentiality statement was read and Merle welcomed all. A moment of silence was held for those who were no longer suffering. 
A thank you to United Therapeutics for providing the lunch for the day 
Presentation on March 13, 2010 was given by Erin Dweik, a licensed dietician with a BS in Dietetics and MEd in Nutrition. Getting the Most Out of Your Food: Eating for Pulmonary Hypertension notes posted below on the web site.
Any Birthdays, Anniversaries (wedding or PH) any other special event. - Les and Marion Pollock celebrated 62 years of wedded bliss.  
• Afghan – any more tickets needed – any tickets or funds to turn in
• Envelope of Hope for new comers – on the table up front – an info packet will be mailed out by PHA
• The PHA 9th Annual Biennial International Conference ~ in Garden Grove, CA this coming June 25th thru the 27th 2010 “Riding the Wave to a Cure” forms/cards available
• Our 4th Annual Walk & Roll has been changed to September 18th - to raise funds for Research and Awareness. Same place Sand Run Park in Akron.
• As of right now, November 19th will be the one day event for the CCF Summit
• Pfizer acquired from Ergonex Pharma a new Phase 2 trial for the treatment of PAH. Terguride is an oral, potent antagonist of 5-HT2B and 5-HT2A (serotonin) receptors. Due to the potential anti-proliferative and anti-fibrotic activity of terguride, this potential medicine could offer the hope of achieving reversal of pulmonary artery vascular remodeling and attenuation of disease progression
• Copies of letters to be mailed to your politicians, Federal Representatives and Congressman regarding the HR 1030 – The Tom Lantos Pulmonary Hypertension Research and Education Act of 2009 are available.  
Merle asked that we each call our Senators this week asking them for co-sponsorship
• Our next meeting has been changed from July to Sunday, June 13 at 1:00 PM due to cut backs at the Library. 

Presentation of the day: Dr. Stefani Parrisbalogun from the Department of Psychiatry at University Hospitals Case Medical Center will help us to understand and how to deal with acceptance.

  “How to deal with acceptance of living with a chronic disease”

Ever say: I do not have a life threatening disease, I can still do as I want. Or: I just have a cold, I’ll be better soon. Or maybe you’ve said I’m a little out of shape (sound familiar)…. You are not alone in those thoughts. Living and accepting having a chronic disease can be a challenge.

  Pulmonary Hypertension fits into the definition of chronic illness of a lifelong progressive disease. Psychosocial aspects of wellness and family are all involved. The person & illness lose sense of self and become the illness. The difficulties of conquering the ability to cope with the illness and implications of other areas ones life were discussed as well as the perception both objective & subjective are different with male, female, age, and past experiences with the illness and whether or not one has had a good relationship with their physician. The cause of PAH as it labels one with the disease, cause, duration, consequences and controlled it is as well as how you deal with symptoms. Coping in a healthy way is very important in dealing with the changes that occur day to day. The overall psychosocial and support systems need to be addressed by the treating MD as well as the patient being honest and expressing the problems he/she is encountering in all aspects of their life. A person may have a sense of relief in having a diagnosis but is not sure how it will affect them personally. Some may lose their sense of self. 
  Emotions of anxiety, anger, fear, guilt, denial, shame, envy are all encountered by everyone the patient and family with PH. Anger and acceptance. Look at underlying reasons behind anger at yourself or the world. One’s personality has a great deal of how coping with PH is managed. All have different emotions at different times and many have problems dealing with the emotions and are unable to move into a healthier level of acceptance. Personality is a big factor in dealing with the illness. Underlying issues of abandonment, loss of control, loss of love, attractiveness, less loved and being a burden on the family are a few of those felt. 
  Different coping skills were discussed as individuals fall back on how they have dealt with problems in the past. Untreated symptoms of anxiety/depression needs taken care of and seeking support may be needed. Individual support from a therapist will help one to maintain a sense of self, productivity, need to continue. Re-examine priorities, health and family/friends. Try stress management techniques, meditation, breathing exercises, and yoga all decrease the levels of stress hormones. Try humor and laughter as a positive coping mechanism. Try pulmonary rehabilitation to improve your muscle strength, decrease anxiety, depression and distress. 
  Caregivers need to take care of themselves so they will maintain their health and sanity to be able to help the affected family/friend with PH. Learn your coping skill and keep doing it to maintain a positive energy, concentration. Talk with your family member/friend the importance of maintaining a positive attitude and strengthen your relationship. Acceptance takes time. If there is anger or denial, look for a reason why. 

MUSIC THEARPY -- Erika Svitak of Hospice of the Western Reserve talked about the different services provided. Different areas of services offered by Palliative services were discussed as well a packet listing summer 2010 community support groups & activities. Palliative care has a goal of keeping the patient out of the hospital. 
  Virginia Kallay, Music therapist discussed the history of music therapy. Research has shown that music therapy increases IGA in saliva which is very good for the immune system. We should have a song library of music for our different moods. Music carries memories- possibly of smells and taste when you hear a song. Restaurants and elevators use music to stimulate the mood. Songs can and are played hopefully to relax the patient - different people interpret various songs differently. So listen to music you enjoy…choosing a song we enjoy gives us a sense of some control.
  Music is used to stimulate exercise, gait training in individuals with Parkinson’s disease; individuals who stutter are able to not stutter with singing. Music is used to calm breathing, reduce pain, anxiety, and a variety of disorders. Ginny also mentioned how drawing can also help with our mood changes; art is also relaxing. Guided imagery, songwriting, keeping a journal may help.
  A few examples of songs were played on the guitar to demonstrate the use of music in reducing breathing and stress and a ballad created by two boys for their Mom.
  After the presentation; the meeting was opened up by Merle for any questions or concerns. We then had our door prize drawing and the meeting was adjourned.
Thank you Janice for taking these notes, also a thank you to Roberta 

+ + + + + + + + + + + + + + 
March 13, 2010 Merle opened the meeting with a welcome and greetings to all after such a long winter. She hoped the holidays were bright and cherry and wished all a Happy New Year, a Happy Ground Hog Day, A Happy Valentine’s Day and a soon to be Happy St. Patrick’s Day – most importantly, a happy new decade and all it will bring; like the CENSUS.
A moment of silence to remember those who have departed this life, those who are breathing easier and are no longer suffering. Marlene Kersman died this past January
An introduction of all clinicians and those pharmaceutical representatives; with a special thank you to United Therapeutics for the feast of the day. 
Announcements: Birthdays, Anniversaries (wedding or PH) any other special event.
• Updates on the web site.  
• Unity Walk – the donations from the afghan were used toward this event ~ 
• On January 29th Pittsburgh had a Unity Walk and a tour of AGH’s PH Research Dept. Merle explained that Dr. Benza (AGH) and Franz (Mayo) and PA Lazar (AGH) climbed Mt. Kilimanjaro to raise awareness and funds for PAH.
• HEART ~ Function vs Emotion. A ballet on February 19th and 20th Bayham Theater sponsored by UPMC. 4 heart tx patients and 2 ph patients Merle and Holly
• Envelope of Hope for new comers – on the table up front – an info packet will be mailed out
• The PHA 9th Annual Biennial International Conference ~ in Garden Grove, CA this coming June 25th thru the 27th 2010 “Riding the Wave to a Cure” forms/cards available
• Tickets passed out for the Afghan of Hope the Kersman family has donated
• Our 4th Annual Walk & Roll this September 25th - to raise funds for R/A
• As of right now, November 19th will be the one day event for the CCF Summit
• Don’t forget the time change tonight… that must be a sign of spring

HR 1030 - The Tom Lantos Pulmonary Hypertension Research and Education Act of 2009, we can have hope. Letters available to be mailed and also copies of the bill. Please be sure to contact your Federal Representative your Congressperson and ask him or her to co-sponsor this bill. Congressman Tim Ryan (17th) , Congressman Charlie Wilson (6th) and Congressman Stephen LaTourette (14th); have co-sponsored that leaves 15 more from Ohio.
• On November 19, 2009 Senator Robert Casey (PA) introduced into the Senate 
 S 2803. Now we need to write and call our Senators and ask them to co-sponsor this bill.  
• Contact friends and relatives in other states and ask for their support in doing this
• You can call, stop in, mail or e-mail your Congressperson or Senator; asking them for co-sponsorship. If you mail anything, mail to their local office 
• Remember, they don’t always know about these bills, especially if they are not on that respective committee.
• Merle has a list if you’re not sure who your Congressperson is. Politicians are also listed in the Government pages of your phone book or look on the site – zip code needed
• Sen. George Voinovich (R-OH) 1240 East Ninth Street, Room 3061
Cleveland, Ohio 44199 Phone: 216-522-7095216-522-7095 
• Sen. Sherrod Brown (D-OH) 1301 East Ninth Street, Suite 1710
Cleveland, Ohio 44114 Phone:  216-522-7272216-522-7272 
• Please email or call Merle if you need help in doing this. 

Our presentation for the day: Erin Dweik, a registered and licensed dietitian who has a BS in Dietetics and a MEd in Nutrition, both from the University of Cincinnati. Erin also pursued a PhD in Nutrition from Virginia Tech and an MPH in Nutrition from New York Medical College. She has worked in hospitals as a dietitian since 1986. Her primary interest is diabetes, weight control/obesity and public health nutrition as well as pulmonary hypertension. She has contributed to a book chapter on dietary aspects of pulmonary hypertension. Erin will guide us to learning how to eat a healthy and nutritious snack or meal.

  Getting the Most Out of Your Food: Eating for Pulmonary Hypertension
A quick review of the five food groups were stated to be: Protein, Carbohydrate, Fat, Water, and Minerals.
A hand out with the following questions was given and completed by each attendee as well as discussion for each response.
 Your relationship with food.
 Your worst food habit.
 One thing you need to do.
 Your relationship with exercise.
 What is stopping you?
 Your motivation.
Part 2 ~ Eating for Pulmonary Hypertension.
General Recommendations
 Weight Control Mirror the economy
 Sodium Restriction: Choose with sodium in mind – read those labels
 Supplements: See: Vitamin E, Vitamin C, and beta-cryptoxanthin
Avoid these supplements: garlic (if you are on coumadin) ginkgo biboa, ginger (interferes with anticoagulation), feverfew (increase potency of anti-coagulants), aloe vera (increases K) loss/diuretics, African/Caribbean tea-Papilionacea crotalaria – linked to development of Pulmonary Hypertension.
Frequently asked about Supplements ~ always check with your doctor
 Omega – 3 Fatty Acids
 L-Carnitine
 Resveratrol
 Collagen Supplements
 Genseng (green tea extracts)
 Chromium, Zinc
 Coenxyme Q10
 L-arginine
Support Systems with Healthy Eating.
After the presentation, the meeting was opened up by Merle for any questions and discussion. We then had our door prize drawing. Thank you Janice for helping with these notes.  
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Meetings 2009
The meeting for December 5, 2009 opened at approximately 1:00 pm. The confidentiality statement was read and Merle welcomed all. A moment of silence was held for those who were no longer suffering. A thank you to Anthony DuPonty of CuraScript for providing the lunch for the day as well as an introduction of clinicians and pharmaceutical representatives who were present at the meeting. Special Announcements – birthdays ~ 2; 1 wedding anniversary ~ one PH anniversary 15 yearsA reminder to contact our Congresspeople about HR 1030 the Tom Lantos Research and Education Act of 2009 and now S 2803 introduced into the Senate in November so now it's time to contact our Senators.Our presentation on September 12th (see notes below) was given by Landra Slaughter, RN, CCRC from University Hospitals with the topic of “Planes, Trains, Automobiles, and Boats – How to travel having PH” A review of the helpful hints were mentioned.September 19th was our third annual Walk & Roll held at Sand Run Park in Akron, Ohio. Monies are still coming in as some individuals had their own websites and are still receiving donations. The total thus far is ~ $ 12,500 plus or minus.  This does not include the individual websites.November 20th and 21st was the 6th annual CCF Summit titled: Translating Discoveries Into Patient Care. Great summit and good turn out for attendance with Saturday’s focus on patients and Caregivers. Other Items Discussed: next years meeting dates; links to Medicaid, Medicare and websites; PHA's 9th International Conference in CA; this past year in review. Drs. Benza and Franz along with Jessica Lazar, PA will climb up Mt Kilimanjaro, Africa’s highest mountain starting on 2/19/10 to raise money and awareness for PAH. A reminder of getting the flu shot and H1N1 vaccination as well as symptoms of flu and reminder of good hand washing technique was reviewed. 
 Ph.D., Associate Professor of Medicine, Case Western Reserve University. Medical Director of Lung Transplantation and Pulmonary Vascular Disease at University Hospitals of Cleveland, and Associate Editor for the PHA Journal Editorial Committee.Robert Schilz, D.O.Presentation for the day by 
PE – Thrombus:  PH & Blood Clots ~ 
Role of Blood thinners with Blood Clots & Infusion Rates1) Chronic thrombosis in PH patients2) Do Blood Clots play a role in CTEP3) Clotting of Central Line Catheters4) Other conditions requiring blood thinners1-10% patients can still get clots while taking Coumadin. 4-6 weeks after a clot forms it is gone.Some clots turn into plugs in the lungs, some individuals have a tendency not to clot normally. 1,000 to 11,000 patients a year survive having an embolus.General risk of developing a clot is 1/100 a lifetime. Tests performed to determine if a clot is present are the V-Q Scan and possibly an angiogram.  CT Scan is not as good as the VQ scan.Surgery is performed to remove the clots on ~ 30% patients.Thrombectomy is surgical procedure to remove new clots. Endarectomy is a surgical procedure to remove the hard “plug” clot. These patients are placed on a heart lung machine at the time of the surgery. There is 15% mortality with this procedure. 15% are not cured even with the plug removed. Some patients may continue to have PAH and then require medication as the disease has involved the small vessels and others may be cured of the PH.  Blood clots form first in the lung and then one can develop Pulmonary Hypertension due to the blockage of the blood flow to the lungs. Use of Coumadin with clots and PAH. Coumadin used even if not clot present because as vessels get smaller clots can form. A study comparing those who took coumadin to those who didn’t was performed.  Results are that it may help those with PAH.  Blood level of the INR is now used rather than PT at 1.5 time normal level.  Biggest potential side effect is bleeding. Frequent blood tests are required for safety. A list of drugs that interfere with bleeding times were reviewed. Infusion catheters pump at different rates and are very slow which can contribute to the formation of blood clots. Other conditions requiring anticoagulants are arterial fibrillation an abnormal heart rhythm can lead to a clot in the legs (DVT) or to the lungs (PE).Clotting abnormalities are antiphospholipid syndrome, protein C or S deficiency, and Factor V Leiden deficiency.Home going anticoagulants are of 3 classes.     Coumadin    Heparin    Low Molecular Weight HeparinAntiplatelet medications are Aspirin, Plavix, Lovenox – Sub-cutaneous (given by short needle just under the skin). After the presentation by Dr. Schilz, Merle opened the meeting up for any questions and discussion. Merle explained that she was diagnosed 8 years ago and she is now better and contributes this to the combination of medication and oxygen that she is prescribed.  Several of the newer members shared their concerns about this disease.  Our chat and snack time -- refills. Our next meeting will be March 13, 2010 ~ enjoy your holidays everyone and see you all next year.  Thank you Janice for taking these notes. *    *    *    *    *     *     *     *     *     *     *    *     *     *     *
The meeting for September 12, 2009 opened at approximately 1 p.m.  The confidentiality statement was read and Merle welcomed all.   A moment of silence was held for those who are no longer suffering.  Since we last met, Caroline Hoffert has received her wings.Special Announcements – Don't forget our Let's Walk & Roll next weekend.  I also have some tickets for a lap afghan that Mary Saum has made.  More tickets will be available at the Walk & Roll.  The drawing will be at the Cleveland Clinic Summit November 20 and 21st – Translating Discoveries Into Patient Care -- don't forget that special event.  It's free for patients and caregivers.Don't forget to get your flu shot:  Not sure about H1N1 shot yet – ask your PH doc on that one On September 10th, UT sponsored a Patient PHorum Dinner in Cleveland-Beachwood area; the Patient Phorum dinner started at 6:30.  This was a satellite presentation – live video and similar to the previous presentations.  Dr. Minai and Shiltz from the Cleveland Hospitals were there for Q & A.  I again asked if I could mention HR 1030.  Several of us were there. At our July meeting Dr. Joseph Parambil an Associate Staff Member in the Department of Pulmonary, Allergy and Critical Care Medicine at CCF did a presentation on “How to deal with PH and these summer months.  His hand out is posted on the new page – More Helpful Hints 
, RN, CCRC from University Hospitals gave a presentation on Landra SlaughterOur presentation for the day: 
“Planes, Trains, Automobiles and Boats – How to travel having PH”.   
Landra has been a registered nurse for twenty seven years with twenty three years in pediatric nursing and pediatric clinical trials. She has worked in the Pulmonary Hypertension Division for over four years at University Hospitals Case Medical Center. Landra has held numerous management positions during her nursing career and is a Certified Clinical Research Coordinator. Objectives: Plan early ~ Make a checklist and follow it ~ Choose mode of transportation ~Duration of trip ~ Notify your PH Physician, DMEsupplier, and specialty pharmacy ~ Obtain all required documentation ~ know the laws.Follow that check list ~ Copies of medical history and prescriptions ~ Adequate supply of medications ~ Medical clearance travel letters~ Advance notice to oxygen supplier ~ Flight simulation testing ~ List of local PH physician (to where you are visiting ~ Hotel accommodations ~ Mobility devices ~ If you travel by Plane, Boat, Train or Bus, certain extra paperwork will or may be required especially for oxygen.   Helpful Hints ~ Altitude - difficult breathing ~ Air quality - avoid high pollution areas ~ Climate - low humidity: dehydration; cold air: bronchospasms; hot air: air conditioning ~ No smoking sections - all states do not have this law ~ Medications - never pack in checked luggage ~ Board first ~ Never get off plane until you see your device (wheelchair) ~ Attach instructions for assembling mobility device ~ List of physicians, letters from physicians, Rx and other pertinent info with you.Landra also gave us a copy of a sample letter for physician request and a list of chain restaurants and hotel that are smoke free.%    %    %    %    %    %    %    %    %     %    %     %     %     %     %     %     %     %     %     %                                         *    *    *    *    *     *     *     *     *     *     *    *     *     *     *
The meeting for July 11, 2009 opened at approximately 1 p.m.  The confidentiality statement was read and a moment of silence was held for those who are no longer suffering. A thank you to Todd Thomas and LungRx for providing the feast of the day.  Introduction of clinicians and pharmaceutical representatives. 
 We also had Scott Marlow a RRT from the Cleveland Clinic who gave us tips and exercises for proper breathing and how to relax.  Ever use pursed lip breathing and do you use diaphragmatic breathing?  Scott will help us learn or relearn how to do these.  Scott and his wife Beth live right here in Twinsburg with their children.At our May meeting, United Therapeutics sponsored a PEER Mentor - PEER stands for Patients Exchanging Experiences with Remodulin®. The PEER Network is a nationwide group of people with PAH who are currently receiving Remodulin therapy and have volunteered to share their personal disease and treatment experiences with others. The Network also offers a Web-based program that enables people with PAH to exchange private, one-to-one e-mails or phone calls with the PEER Mentor of their choice.  Our very own Maria did the patient presentation, telling us of how she found out she had PH and how she has learned to live with it and cope.  Each of our stories are unique but still are so similar. PHA “On the Road”.  PH Patients & Families Education Forum – Saturday, June 13, 2009 at The Dearborn Inn, A Marriott Hotel, Dearborn, MI.  I did a presentation on advocacy... I wonder why???  Several of us from this group did attend... Great meeting Another Patient Phorum Dinner is coming August 10th – if you are interested, let me know and I will be sure you get the information.  I don’t have all the specifics yet.On May 14th, UT sponsored a Patient PHorum Dinner in Cincinnati.  The dinner started at 6:30 with Dr. Sean Studer, Medical Director, Pulmonary Hypertension and Lung Transplantation Program, Beth Israel Medical Center, Newark, NJ and Joan Zhang, PH nurse specialist and NP from UPMC – Pittsburgh as guest speakers.  Also Laura Mausling a PEER mentor spoke of her journey with PH.  Sheila and Rick and Tom and I attended... I asked if I could mention HR 1030 and they graciously gave me 26 seconds  :o)  I took about a minute :o). This immediate area covers districts 9 Kaptur, 10 Kucinich, 11 Fudge (Tubbs Jones), 13 Sutton, 14 LaTourette, 16 Boccieri (Regula) and 17 Ryan.  So far only Congressman Tim Ryan and Charlie Wilson have signed to co-sponsor this bill.*increasing the effectiveness of clinical researchers in the PH field  *continue research and expand understanding of pulmonary hypertension*raising public awareness about PH symptoms both primary and secondary*preparing more physicians for prompt and effective management of PHWith August fast approaching it's time for us to contact our Federal Representatives for co-sponsorship of HR 1030.  They are usually in their home territory during the month of August.  Call or make an appointment to visit or talk with them about co-sponsorship for this much needed bill. Medicare Plan D ~ Prescription drug coverage purchased through private insurers and approved by Medicare.  Plans vary along with cost and drugs covered.Medicare Plan B ~ Medical Insurance - Coverage for major medical costs, including doctor visits, lab costs, also including durable equipment, some “therapies” and oxygen.  They pay 80% - you pay 20%Medicare Plan A ~ Hospital Insurance – inpatient careA Quick Medicare Primer If you are sick – stay at home, do not spread those germs.Avoid touching your eyes, nose or mouth after you have touched anything else.  Sneeze or cough into a tissue then throw that away.  If you don’t have a tissue, sneeze or cough into your elbow, do not sneeze or cough into your hand.  Wash hands afterWipe off phones, door knobs, refrigerator door, light switches, etc. – do this daily.  Remember to wash your hands often (say the alphabet while washing), carry hand sanitizer with you and use when you can’t wash your hands.  About the Flu and summer colds: when sick if you don’t need to, do not go to the ER, call your family physician first then check with your PH doc.  Symptoms are as typical, fever, cough, sore throat, body aches, headaches, chills and fatigue – sniffles would be for a cold.  You could also have diarrhea and vomiting.  
And Now: How to deal with Pulmonary Hypertension and these summer months. Our guest speaker will be Dr. Joseph Parambil who is an Associate Staff Member in the Department of Pulmonary, Allergy and Critical Care Medicine at CCF. He is also Assistant Professor of Medicine at Cleveland Clinic’s Lerner College of Medicine. He Specializes in Diseases and Conditions such as Langerhans Cell Histiocytosis (LCH), Pulmonary Fibrosis, Pulmonary Hypertension and Sarcoidosis.  He and his wife and two sons live in the Cleveland Area – and are expecting their 3rd child.  This hand out is posted on the new More Helpful Hints page. Our next meeting will be September 12th when Landra Slaughter, RN, CCRC from University Hospitals will do a presentation on “Planes, Trains, Automobiles and Boats – How to travel having PH” and let's not forget our Walk & Roll on September 19th  you can now register and also make donations on line.  
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The meeting for May 2, 2009 started at approximately 1 p.m.  Merle read the confidentiality statement and welcomed all.  Those pharmaceutical representatives present introduced themselves.A moment was taken to remember those who have departed this life in our PH Community and are no longer suffering.  At our last meeting I asked you to either pray or have positive for little Nathan Mayer who was not doing well.  I regret to say he did pass away the following week.  Let’s take a moment of remembrance. A thank you to Lian Latham from United Therapeutics for sponsoring our meeting for the day. Announcements ~ Les and Marion Pollack will be celebrating their 61st wedding anniversary this coming weekLet's not forget to contact our Federal Representatives about HR 1030  -- only two have signed.On May 14th, UT is sponsoring a Patient PHorum Dinner in Cincinnati.  I have invitations that will be passed out if you are interested.   Registration is required and space is limited.  The dinner starts at 6:30.  Dr. Sean Studer, Medical Director, Pulmonary Hypertension and Lung Transplantation Program, Beth Israel Medical Center, Newark, NJ and Joan Zhang, PH nurse specialist from UPMC – Pittsburgh will be the guest speakersIf you have a computer you can go to the PHA web site and check out their “On the Road”.  Upcoming: PH Patients & Families Education Forum – the closest to us would be in south east Michigan Saturday, June 13, 2009 at The Dearborn Inn, A Marriott Hotel, Dearborn, MI.  I found out after our last meeting that I have been asked to participate on one of their panel discussions.  It is a free forum but again, registration is required. Don't forget to get a flu shot and remember how to help avoid spreading those germs. Our presentations for the day:  First, United Therapeutics will have a PEER Mentor - PEER stands for Patients Exchanging Experiences with Remodulin®. The PEER Network is a nationwide group of people with PAH who are currently receiving Remodulin therapy and have volunteered to share their personal disease and treatment experiences with others. The Network also offers a Web-based program that enables people with PAH to exchange private, one-to-one e-mails or phone calls with the PEER Mentor of their choice.  Our very own Maria will do the presentation.  Lian will do the introductions. We also have Scott Marlow a RRT from the Cleveland Clinic who will give us tips and exercises for proper breathing.  Scott and his wife Beth live right here in Twinsburg with their children.  Ever use pursed lip breathing and do you use diaphragmatic breathing?  Scott will help us learn or relearn how to do these.    Upcoming meetings ~ July 11th We will have Dr. Joseph Parambil "How to deal with PH and These Summer Months." Our September meeting will be on the 12th and our Walk and Roll on the 19th.   I think we all enjoyed the Akron State Park last September – Our November meeting will be the CCF Summit and will be a 2 day event this year, November 20th and 21st; free to patients and their caregiver.  We will also meet on December 5th, and then our next meeting will be March of 2010.  I do set up the next year’s meetings in June.

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The meeting for March 14, 2009 started at 1 p.m.  Merle read the confidentiality statement and welcomed all.This has been a difficult winter and I am sooooo looking forward to spring.  I want to wish you all a Happy New Year, a Happy Ground Hog Day, A Happy Valentine’s Day and a Happy St. Patrick’s Day. A thank you to Dave Crawford from TEVA Pharmaceuticals for sponsoring our meeting today. A moment of silence was given for those who are no longer suffering.  I was not notified of any one passing. Announcements -- Amy Stamp received her Masters Degree ~ Les Pollack had a birthday this past week ~ Kim Hoim Horton from Gilead has resigned, I wish her well.  Special thoughts to go to Little Nathan Mayer who is not doing well and needs our prayers and positive thoughts.The president of PHA, Rino Aldreghetti, recently had a State of the PHA Address.  He updated the Support Group Leaders on what happened during 2008 and a peek into the future – you can listen to it on their web site at www.phassociation.org. Think on this:  in 1985 there were 187 people diagnosed as PH patients in the United States ~ in 2001 there were 3000 ~ now, with this so called today, there are between 20,000 and 30,000 patients diagnosed with PH.  Another FYI there are 24,000,000 diagnosed with diabetes and 26,000,000 diagnosed with arthritis. PH has a smaller number, our voices are essential to help continue this fight.  We ARE a community of hope fighting for a purpose. Our next meeting will be on May 2nd and United Therapeutics will sponsor a PEER Mentor - PEER stands for Patients Exchanging Experiences with Remodulin®. The PEER Network is a nationwide group of people with PAH who are currently receiving Remodulin therapy and have volunteered to share their personal disease and treatment experiences with others. The Network also offers a Web-based program that enables people with PAH to exchange private, one-to-one e-mails or phone calls with the PEER Mentor of their choice.We will also have Scott Marlow, RRT, who will help us with breathing exercises and tips on how to's... 
 Who to Treat and With What
Our guest speaker:  Dr. Omar A. Minai, who is a Staff Physician in the Department of Pulmonary, Allergy, and Critical Care Medicine and the Lung Transplant Center at the Cleveland Clinic.Today I will talk about the diagnosis and then the options for treating pulmonary hypertension and to go over a few of the basics of pulmonary hypertension.Pulmonary hypertension is a disease of the lungs but those with pulmonary hypertension will also have a strain on the right side of their heart.Doctors have to make an accurate diagnosis:  an Echo is not enough; a RHC is needed before initiating therapy.  If you don’t have PH you won’t respond to therapy.  When evaluating therapy, RV (right ventricular) function is the key, don’t focus on the pressure as much.  It is an issue of the lungs and just because your pressure is going down is not always a good thing – just because the pressures are going up is not always a bad thing.  Focus on how well you are doing; the pressure is just a number. Focus on how well you are doing; are you feeling better, if you can do more, if you can walk more and be less short of breath; then you are probably doing better even though your pressures look like they are going up.Physicians need to treat the patient, not just a number.How to diagnose? The heart has 4 chambers, when you have a right heart catheterization the catheter goes into the right ventricle and up into the pulmonary artery and measures the pressures in your lungs (He had a slide on what a RHC was and showed what was measured)PAH mPAP > normal 12-15mmHg, with very small increase with exercise but>25 mmHg at rest is considered PH    >30mmHg with exercise
The pressures in the lungs go up very little so when looking at 12 to 25 it really is a significant difference.  2003 Classification by WHO – World Health Organization
There were 5 classifications given. 1.PAH – IPAH, occurs without any apparent cause – Familial – APAH (Associated with PAH) similar to IPAH but expose to toxins or effects of other diseases (scleroderma, lupus, chronic liver disease, HIV, diet drugs etc2.PVH – caused by diseases of the left side of the heart 3.PH associated w/ disorders of the respiratory system – COPD, Sleep Apnea, developmental abnormalities etc4.PH due to chronic thrombotic and/or embolic disease – obstructions (clots) etc5.Miscellaneous – Sarcoidosis, tumor, fibrosing, etc
Diagram of pulmonary vascular tree (Dr. Minai had a slide of the vascular tree and showed where each of the different types of PH was located)  Pulmonary Arterial Hypertension may include: IPAH, familial, pulmonary hypertension associated with cardio vascular diseases, portal hypertension.  The disease can also result from chronic liver disease, some rheumatologic disorders, congenital heart malformations or illicit drug use.
Patients may also develop pulmonary hypertension due to secondary issues, some underlying condition (scleroderma, lupus etc), or infections, it could also be a wrong medication; these can lead to pulmonary hypertension.  Those with PAH may respond to treatment, those with PVH do not respond well to PH specialty treatments and could cause harm.  Progression of the disease: asymptomatic, symptomatic (poor diagnosis percentage), more symptomatic (good diagnosis percentage).The challenge is to improve the ability to diagnose early in the poor diagnosis stage Vasoreactivity to CCB should always be done.  IF you respond (10% acutely, 5% chronically), you respond better to this than to other meds.  Also CCB responders have a VERY good (95%) 5 year survival.  Only about 5% of those with IPAH respond well to CCB’s but of those, 95% have a better survival rate.  We need to find out who those patients are and how well they will respond.  This would be for patients who are not therapy right now.If you are NOT a responder, do not use CCBs.  They can cause detrimental slowing of the heart, and reduction of cardiac output. All PH patients should use anticoagulation unless there is clear contraindication. It is proven that those who are on anticoagulation therapy survive longer than those who are not on anticoagulation therapy. This helps to eliminate blood clots which in turn help them, the patient, survive longer.   USE OXYGEN IF YOU NEED IT.  If you are better with oxygen, you should use it.  You are not “better” because you aren’t using it.  Not using it doesn’t make you “less sick”. Oxygen is made to produce energy, think of it like food.  You need food to survive just as you need oxygen to survive. Use oxygen to be able to exercise more, be more active and rehab more.  Think of oxygen as your friend – it is a good thing, not a bad thing.  Some only need oxygen during the night while sleeping; some need it both during the night and during the day.  Oxygen IS a medication and will lower the pressure in your lungs. PH rehab – for those on therapy it is more of benefit to you in terms of your walk, function capacity and shortness of breath.  Dr. Minai mentioned our presentation on exercise in December.Old idea:   no exercise which we found leads to deconditioning, possible depression.New idea:  rehab is used in STABLE patients this again will benefit them in terms of 6MW, function capacity and help eliminate shortness of breath.
Stable is defined as being evaluated, staged, and placed on therapy long enough to assume you are “stable”If you get SOB, have dizziness, or chest pain occurs during exercise, stop, take a break and check to see if you should discontinue or scale back the rehab. Therapeutic Options and decision Initial therapy decisionDisease stage (symptoms and cath results)Class 4 – aggressive, IV, multiple or combinationClass 1, 2 – single, oral, possible combinationPatient input – a personal decision process -- what stage do you come to the doctor – passing out, can’t walk across the floor, shortness of breath, mild, moderate or severe.A RHC will decide which is the best therapy for you – oral, IV, combination.  Some patients do not want to go on an IV therapy but this may be needed with certain patients.Survival in PH -- Congenital patients do well – HIV patients do poorlyPAH determinants of riskClinical evidence of RV failureProgression rateWHO class6 minute walk timeEcho can be evidence of RV failure Prostacyclins (Pros and Cons) Epoprostenol, Treprostinil, Iloprost - IV’s or Sub Q’s… site infection could be an issue.  Life of the medication if disconnected but immediate results when first put on these therapies.Inhaler patient compliance is a big issue (Iloprost, 6 xs per day) Oral Agents (Pros and Cons) Bosentan, Ambrisentan, Sildenafil Convenience of taking a pill, if you experience added fluid retention, call your physician right away – an added diuretic could solve the problem.  Monthly blood work is required.  Lower risk patients are those who do well on 6MW have little or no symptoms, they will do well on oral therapies. Patients may also benefit from combination therapy if required.
Higher risk patients are those who are very symptomatic, do not do well with a 6MW, have more difficulty with every day living.  Those need to be on the IV medications at the beginning or when first diagnosed.Combination therapy has very little study evidence backing it up.  Nevertheless, Dr. Minai believes combination therapy is beneficial.  Mostly anecdotal and borrowed experience from other diseases (CHF, HIV, etc.).  When a second therapy is added you often do better.   Studies/trials are now going on with medications that not only open the vessels but could possibly reverse the problem. Randomized controlled trials of 6MW for ALL the drugs listed showed similar benefits (i.e. about 300m before treatment; about 400m after treatment).  6MW results are an important part of your examination.Dr. Minai mentioned the upcoming CCF Symposium on November 19th and 20th which is free to all patients and guest.  Part of the second day will be an open forum when patients will be able to “ask the doctors” Questions and Answers followed the presentation. 
We had our chat and snack time and drawing for the door prize.
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MEETINGS FOR 2008

Notes for December 6, 2008 Meeting ~ • Confidentiality Statement, Mission Statement and the Vision of PHA was read• Welcome by Merle and request to sign in - introduction of pharma reps• A moment of silence for those who are no longer suffering ~ for those in the PH community in general• A brief statement about eating first, chatting some and then our presentation – reminder of chat and snack after our presentation (refill our plates and door prize)• Thank you to Actelion for our feast of the day
Merle mentioned HR 6568 and how we still need to contact Senators –> Voinovich and Brown. The bill passed when presented to Congress with overwhelming response – on September 25, 2008. She mentioned we will have to go through the whole process again during the 111th Congress ~ sure does seem like a lucky number though.• Report on the Walk & Roll September 20, 2008 at Akron State Park• CCF 5th Annual Symposium on November 8th – Merle did a presentation on Grief & Grieving – then introduced Cheryl Arnett who did a presentation on Caregiving. Check the web site for all the sessions that were available. It was a great day for PHers• PhRMA Marketing Code – the restrictions now placed on that industry – 36 pages
At our September 13, 2008 meeting Dr. Akhil Bindra from Akron General Hospital was our guest speaker. Dr. Bindra started a PAH clinic and treatment center at Akron General Medical Center in 2007; he also has a very busy outpatient practice. He has lived in the Cleveland area since he was 5 and he and his wife now live in the Akron area. His presentation: Pulmonary Hypertension - Improving Life in the Present and Hope for the Future. See previous meeting notes.
Dr. Schilz did a presentation on. 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.Robert Schilz, D.O., Ph.D.,Presentation by:  
                            Winter and PH Exercise -- Staying in the Groove  
 I think we all realize how important it is to keep active and come winter this is more of a challenge. As the saying goes: if you don’t, you won’t. Keeping our bodies active will help our lungs stay in shape. Dr. Schliz gave us a few pointers on how to do this and he recently spoke to the Cincinnati Group and they sent their Hello’s to us. He also mentioned that since he is on the Education Committee for PHA he will do what he can to help us all with either education programs or just updating us with the latest PH news and advancements. To begin with “if you don’t, you won’t” but also remember to “move it or lose it” and that will help us to stay in the groove.Dr. Schilz also mentioned since last year there have been 3 new trials: Freedom - a trial by UT for oral Remodulin also Phrist – similar to Revatio but a once a day tadilaphil a 40 mg instead of 3xs a day… this is by Eli Lily along with UT and last but not least Triumph -an inhaled Remodulin similar to Ventavis (which is 6 to 9 times a day) – the new one will be only 4 times daily. These are some of the new treatments which can be beneficial to PAH patients – remember what is good for one may not be good for another. It is always trial and error for us. As always, we need to let our doctors know what is going on.
Exercise – improves function and endurance ~improves muscle metabolism ~ improves strength and balance ~ improves mobility. Exercise provides a positive physiological effect ~ and can be used for weight control, maintenance or to lose weight ~ improves HDL cholesterol –> the good cholesterol ~ improves diabetes control –> sugar to muscle. If we are deconditioned that would be one of the reasons for our huffing and puffing. Muscles in order to exercise need to be in shape. Having a lung disease is an additional reason for not being able to exercise or a good excuse. Low oxygen levels – if you don’t have enough oxygen and your body asks for more – there will be problems. Have you noticed if you are overweight or carrying items that can be too much weight to handle; this is or can be a challenge for us. Ask your doctor what would be an allowable weight to lift. It is best to have a controlled exercise program. This also applies to our medications and symptoms as well as exercise – if something seems to have changed such as weight/fluid gain; sore feet or whatever; you may not need an increase in your meds, there may be something else going on in your life style. Stress, tension, etc.. We should not over exercise and those signs would be lightheadedness; chest pain; severe shortness of breath – at that point you should talk with your PH specialist. After exercise if you can’t get out of bed for a day or two –> you did too much. Do you know how far you can go, how much exercise you can do –> remember that saying –> don’t hit the wall – don’t push that envelope..  
Recommended types of Exercise
Light resistance –> no heavy lifting; light to moderate –> aerobic – walking or swimming.Avoid using upper and lower limbs at the same time (i.e. Nordic track) unless patient has little or no symptoms – watch tension control. It is possible to be too ill to do too much but you need to do some exercise –> walking seems to be the best overall exercise –> and that should be on a level -- not an incline.Location for Exercise Outdoor exercise should be avoided during hot or humid days; or extremely cold days as that weather can clamp down on our vessels. A clinic or hospital based facility would be the most beneficial for doing an exercise program – being wired up and monitored is best for our first attempt at a formal exercise program and can also be beneficial for continued maintenance. 
Pulmonary Rehab provides a supervised environment – monitors our heart rate, oxygen level and symptoms. Going to a clinic provides scheduled commitment. The possibility of additional education – aerobic and weight training and can also be an individually tailored program with goals we could follow and maintain and support from other patients with similar needs. Questions & Answers followed Any suggestions for programs for next year -- there a couple of slots open ~ Are there any concerns or issues to discuss -- does any one have any helpful hints ~ Our next meeting will be March 14. 2009 -- That's next year (wow) and Dr. Omar Minai will present "Who to treat and With What" We had a break, refilled our plates and had some sharing time and the drawing for the door prize. Many of the members shared their stories and the difficulty of being diagnosed but also mentioned how much their quality of life has improved with treatment. A big thank you to United Therapeutics for all the extra goodies that were passed out.
HAPPY HOLIDAY'S EVERYONE ~~ see you all next year ~ SMILE -- IT'S CONTAGIOUS  

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Notes for September 13, 2008 Meeting Confidentiality Statement read and introduction of Support Group Leader - Merle and those pharmaceutical representatives and clinicians present introduced themselves.A moment of silence was given for those who are no longer suffering: Barb Fugo has passed on since our last meeting.
**About 2 years ago I read this to you and several asked to hear it again: Lynn Redgrave speaking for Bristol-Myers-Squibb said: I want to die from eating too much chocolate or from exhaustion dancing the tango. I want to die of laughter on my 87th birthday. But I refuse; I refuse to die from breast cancer. I want to die from something else. 
And that’s exactly how I feel about PH. So it’s not a question of whether we really deserve the things we get, like PH among others. The important question is this: how well do we handle those things. If we have grace, we can endure, and enjoy the unexpected ups and downs of life. Do I grumble about my health or am I grateful that I am alive. Should I cry because roses have thorns or should I rejoice because thorns have roses?
Special Announcement: After our last meeting Amy Stamp told me that she has completed her Masters Degree -- let's hear it for Amy.
Old Business: Recently HR 6331 Medicare Improvement for Patients and Providers Act of 2008 passed in both the House and Senate – this bill encompasses many issues that pertain to us and many of the disabled or for those on Medicare and Medicaid. Among those, Ending the Medicare Disability Waiting Period – Competitive biding has been delayed – 3 year home ownership of 02 equipment has been delayed. There are many other issues to view. For more information go to the site – News & Views. The summary is 9 pages, the text is 104**And let us not forget ~ HR 6568 it replaces HR 3368. With the passing of HR 6568 we will have HOPE for the future. The Tom Lantos Pulmonary Hypertension Research and Education Act of 2008 ~ is in honor and memory of Congressman Tom Lantos who was an advocate for those of us with Pulmonary Hypertension. He died this past February from cancer. We need to contact our Congresspeople again and tell them HR 3368 has been replaced with HR 6568. This newest bill is still similar to the previous bill in the 109th Congress and has 5 major components. A form letter was passed out to be mailed to our respective members of Congress. 
  **The Medicare Rights Center will be hosting monthly free web seminars on important Medicare topics. The monthly seminars will be held on the second Thursday of each month. To register for these seminars, please visit http://www.medicarerights.org/webinars.html If you have a Medicare prescription drug plan (Part D), but still cannot afford your medications, there are programs that can help. Depending on where you live, your earnings and your health needs, different assistance programs can help you pay your co-pays and premiums. These programs include State Pharmaceutical Assistance Programs, Medicaid, charities, and pharmaceutical-sponsored Patient Assistance Programs. I have toll free numbers if you need them or again, they are listed on the site.New Business: Ideas for next year’s meetings.Let’s Walk & Roll** – It’s that time again, and it’s next Saturday starting at 11 a.m.. We are set up for having our Let’s Walk & Roll at the same shelter in Sand Run Parkas last year -- see top of page. We will need help with setting up and taking down. Fun picnic time for all.** There are raffle tickets for a PH flame of Hope afghan. Our very own Mary Saum has donated her time and yarn to make this beauty. $3 each 2/$5 and 6/$10.**I want to thank Helen Albright who is a new member and donated the door prize for today. And our guest speaker: Dr. Akhil Bindra - Director - Pulmonary Hypertension at Akron General Medical Center. Dr. Bindra went to Rush Presbyterian in Chicago for his residency, did his pulmonary critical care fellowship at University Hospitals finishing in 2001. Dr. Bindra started a PAH clinic and treatment center at Akron General Medical Center in 2007; he also has a very busy outpatient practice. He has lived in the Cleveland area since he was 5 and he and his wife live in the Akron area. His presentation will be:
               Pulmonary Hypertension - Improving Life in the Present and Hope for the Future.  
He learned along the way that with this patient population, PHers read a lot and are very well educated and this may be a complete review for us – he hopes to add something to that knowledge base. PH is still uncommon and the definition is somewhat standardized, but a lot of patients are benefiting and treated that weren’t being targeted before. Definition: obviously elevated pulmonary artery pressures. What that means is the mean artery pressure in the pulmonary artery is greater than 25 but it’s the affect it has on your cardiac output. The amount of blood that the left side of your heart pumps out is the deal. The pressure that’s hiding in those arteries is the thing that leads us to know that something is wrong and the therapeutic endpoint. So the effect of not getting enough blood out of your left heart or having a low cardiac output – shortness of breath, lightheadedness and of course swelling in either your legs, abdomen or both and also associated with this is weight gain. Dr. Bindra explained the circulation of blood flow through our heart chambers and squeezed vessels. When you haven’t been treated the reason why the pressure is high is because that vessel is tight. Ultimately the most important thing is getting flow through that artery and getting flow into the left side of the heart and out into the rest of your body. 
He spoke of the BMP2R gene and how some studies suggest that up to 25% of patients with idiopathic pulmonary arterial hypertension may have a mutation in the BMPR2 gene.” (Richard Tremblath’s work in Europe) and of those who have that gene could develop PH. He reviewed the left side heart issues and PH; also PH secondary or having Lupus or Scleroderma; HIV; also associated exposure to drugs; sleep apnea and others. The testing process: a 6MW test – and different levels of testing done: like right heart cath, sleep studies, CT scans, pulmonary function tests and what not; but once the diagnosis has been made the simplest thing that gives the most information is how far can you walk. And that one test really gives the doctors a wealth of information 
He mentioned the NY Heart Association Classes (on the web site) and how the last two need more aggressive treatment as opposed to the first two classes. He then explained the different types of medicines being used (also on the web site): prostacycline analogs; endothelin receptor antagonist; diuretics, oxygen, blood thinners. 
Combination therapies are also being used. Lung transplants are not being done as frequently but in some instances if treatment is not improving, lung transplant may still be the way to go. 
Dr. Bindra spoke of trials in progress, up coming trials and of course the controversial stem cell research. He mentioned you also need to trust your doctor and nurses that you are being treated by; when you are asking questions and they are giving you advice; you do need to have faith that they are looking out for your best interest and they are leading you in the right direction. 
Another thing he found useful; is to think of yourself as healthy. You are going to have bad days and people that don’t have PH have bad days. Not everything that happens is because of the PAH so don’t think that you are necessarily doing worse just because you are having a bad day. Think of yourself as – healthy -- approach life with that attitude; it can’t hurt and it might help.Questions and Answers.Chat and snack followed and the drawing for the door prize.  

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AT our July 10th 2008 Meeting – Confidentiality Statement read and greetings and introduction of pharma reps and clinicians and thank you to AccredoTherapeutics for providing our luncheon for the day. A moment was taken to remember those who have departed this life and are no longer suffering. Elaine Cunningham on 4-2-08, Gina Williams on 6-14-08, Phil Hastings 6-12-08 Let’s take a moment to remember these phriends. 
Bills and issues -- HR 3368 New bill in honor of Cong. Tom Lantos…the Pulmonary Hypertension Research and Education Act of 2007 we can have hope.More Pressure to Reduce Medicare Coverage for Home Oxygen UseThe Nat'l Home Oxygen Patients Assn. (NHOPA) and others have sent out urgent and immediate calls For Action. NHOPA says, "The House of Representatives has taken action that seriously threatens the availability of portable oxygen equipment. The Medicare Rights Center will be hosting monthly free web seminars on important Medicare topics. The monthly seminars will be held on the second Thursday of each month. To register for these seminars, please visit http://www.medicarerights.org/webinars.html 
Let’s Walk & Roll – It’s that time again. Akron State Park September 20, 2008 Raffle tickets for a PH flame of Hope afghan. Our very own Mary Saum has donated her time and yarn to make this beauty. $3 each 2/$5 and 6/$10. Let’s see what we can do to raise some extra money for research and awareness. The drawing will in November at the CCF Summit. 
At our May 10th meeting Dr. Taylor did a presentation on Pulmonary Hypertension Associated with Left Heart Disease. He discussed the development of pulmonary arterial hypertension from chronic heart disease and the importance of the testing necessary to tell the difference between this entity and primary pulmonary arterial hypertension. The possible cardiac causes of PH ~ why do patients with PVH develop PAH? Also diagnostic issues and treatment options. Thanks to Fred, a new member, I was able to post the information Dr. Taylor presented. Hope you are looking at the site – past notes are on there.
Dr. Akhil Bindra, Director, Pulmonary Hypertension Department at Akron General Medical Center will be our guest speaker for our September 13th meeting. As some of you know, Akron General recently opened a PH center. I looked forward to hearing all about it. 
Our Guest speaker: Dr. Raed Dweik ~ The Director of the Pulmonary Vascular Program in the Department of Pulmonary, Allergy & Critical Care Medicine or fondly known as A90 and who is board certified in internal medicine, pulmonary disease and critical care medicine which are also his clinical interests. He has a joint appointment in the department of Pathobiology in the Lerner Research Institute (LRI) and is Associate Professor of Medicine at the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University. Dr. Dweik is also an advisor for NASA and about 2 more pages of info.He spoke to us about. 
                 “The PHUTURE: scientific discoveries that may change how we diagnose, treat, and monitor PH”  
Although PH was discovered in the 1950’s, it wasn’t until the mid 80’s that treatments were directed toward PH. Now there are 6 medicines specific for Pulmonary Hypertension – to help us maintain a better quality of life. The subjects he spoke on were: biomarkers ~ remodeling ~ stem cells If you see smoke, you know there is fire – this is what is known as a biomarker and there are many biomarkers including various tests. . Depending on the specific characteristic, biomarkers can be used to identify the risk of developing an illness (antecedent biomarkers), aid in identifying disease (diagnostic biomarkers), or predict future disease course, including response to therapy (prognostic biomarkers). Testing involves either invasive or non invasive procedures and the non-invasive test would have to be at least as accurate or close to accurate as a biopsy or catherization, which are invasive procedures. This is where biomarkers come in – seeing that smoke and knowing there is a fire. Remodeling is when you try to clean something out, as you would weeds from a garden or the clutter from a closet ~ so it is with the lungs or arteries; to use those meds and technology to open up the arteries. The richest source of stem cells is in bone marrow. A stem cell is a cell that can make more cells like its own self but it can also make other types of cells and that’s where we have to be careful. PH is one of the diseases where stem cells are being tried for treatment and of course there is controversy. In some cases research is using ones own stem cells.
Questions and Answers followed the presentation and a description of the meds we now use; some concerns we have and some ‘what can we or should we do’ were discussed and again are posted; i.e. exercise. 
Questions and Answers followed the presentation then our chat and snack time and the drawing for the door prize.

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Notes from the May 10, 2008 meeting
Welcome and Introduction of me and pharmaceutical representatives *;*
A moment of silence for those who have passed on ~ Juanita Beer April 4, 2008
Old Business: Bills in the House and Senate The GINA (S358) act did pass and is ready to be signed by the President ~ The President has signed ~ the bill has passed.We still need to contact our politicians on... HR 3368 , Pulmonary Hypertension Research and Education Act of 2007 Ending the Medicare Disability Waiting Period Act of 2007 HR 154What do I say when I contact my politician: (and please call between 9am & 5pm) Ask For: Legislative Health Aide if your politician isn't there.1. When you call your Politician. Identify yourself as a constituent and ask to speak with the Legislative Health Aide. 2. Introduce yourself and BRIEFLY explain what pulmonary hypertension is and how it impacts you and your family. (i.e. PH is high blood pressure in the vessels between the heart and the lungs. It is deadly, and incurable, we want your help to change that.) 3. Emphasize the importance of research to find new and better treatments, and eventually a cure, for PH. 4. Give the aide your contact information and ask to be kept informed of the legislator’s actions on your request. 
*Medicare Rights Center are hosting monthly free web seminars on important Medicare topics. The monthly seminars will be held on the second Thursday of each month. To register for these seminars, or to see previous seminars, please visit http://www.medicarerights.org/webinars.html Applications are still available for the PHA International Conference in Houston this June
New Business**A Class I recall of all lots and strengths of Digitek oral digoxin tablets was made because some tablets are double the normal thickness and may contain twice the labeled amount of the heart medication. Please contact your doctor and pharmacy for more info.
Let’s Walk & Roll – It’s that time again. We are set up for having our Let’s Walk & Roll at the same shelter in Sand Run Park as last year – thank you Zahi for doing this. I have changed the page on the site with information on the new date and how to make donations and registration. If you want to have your own team, and I hope you do, please be sure to puts CAPHS before your team name, i.e. CAPHS - Jane Doe or CAPHS - John Doe or CAPHS - the Doe PHamily etc. I think you get the idea.

Dr. Taylor will do a presentation on FACC ~ A Professor of Medicine for the Cleveland Clinic Foundation ~ Director, Heart Failure Special Care Unit ~ Director, Heart Failure/Transplantation Fellowship Program Dr. David O. Taylor,
                        Pulmonary Hypertension Associated with Left Heart Disease.  
Dr. Taylor discussed the development of pulmonary arterial hypertension from chronic heart disease and the importance of the testing necessary to tell the difference between this entity and primary pulmonary arterial hypertension. The possible cardiac causes of PH ~ why do patients with PVH develop PAH? Also diagnostic issues and treatment options. Pulmonary hypertension is a relatively simple term. It means high blood pressure in the lungs. Doctors have used it for decades; however, it does not convey what is causing the problem. To aid in this, and reduce confusion even among doctors, recently the terms “pulmonary arterial hypertension” and “pulmonary venous hypertension” have come into use. Pulmonary arterial hypertension encompasses much of what was previously called primary pulmonary hypertension, although the two terms are not interchangeable. It means simply that the problem causing the elevated blood pressure is seen in the pulmonary arteries and not the pulmonary veins or farther downstream in the heart.
  What do we mean by “downstream”? If you look at the drawing above you will see the heart, not as you usually see it drawn, but as a diagram of how it functions as a pump. Since we are considering the lungs in pulmonary hypertension, any thing that comes after them is downstream; namely, the left atrium and left ventricle. If these downstream parts of the heart are causing the pulmonary hypertension, we term it pulmonary venous hypertension.
  Most of you are familiar with the causes of pulmonary arterial hypertension. The main cause of pulmonary venous hypertension is heart failure. Heart failure isn’t a great term. It doesn’t mean the heart has completely failed, it simply means it cannot pump enough blood for the body. When this happens on the left side of the heart, blood tends to back up into the lungs and the pressures in the lungs rise. This is how pulmonary hypertension occurs in heart failure, there is not something intrinsically wrong with the lungs, they are simply the recipients of the left side of the heart not working well.
  Left sided heart failure can be thought of as divided into two types. In systolic heart failure, the left ventricle is a weak and flabby muscle. It is unable to squeeze forcefully and pump enough blood to the body. Systole is the part of the heart cycle where the ventricle contracts and pushes blood out. In systolic failure you will hear discussed the ejection fraction, which is the percentage of blood the heart is able to pump each beat. Ejection fractions > 55% are normal. Less than that and you have some degree of systolic heart failure.
Diastole is the part of the heart cycle where the heart relaxes and fills with blood. In some cases the heart gets stiff, and requires more pressure to fill with blood. You can think of it as the heart acting more like a basketball than a balloon. A balloon you can fill with the air pressure from your lungs, a basketball you can’t. This is called diastolic dysfunction or diastolic heart failure. In this case the diastolic pressure (the lower or second number in the blood pressures shown above) increases in the left ventricle. As such, higher pressures are needed in the left atrium to push blood into this chamber. This is simple a mathematical fact of pressure mechanics. Finally the “upstream” pressures in the lungs and right ventricle must also rise to push blood into this stiff chamber.
  Therefore, when there is a problem on the left side of the heart causing elevated pressures, the pressures in the lungs increase NOT because there is anything wrong with them, but simply because these pressures are necessary to maintain cardiac output. It is this point that is key to making treatment decisions. If the primary problem is in the lungs themselves, treatments with vasodilators targeting the lungs to reduce the pressure there is the key. If the primary problem is in the left side of the heart, these treatments will not be beneficial and can even be harmful to patients!
  A definitive way of finding where the problems occur is through a right heart catheterization. Here a catheter with a balloon at its tip is advanced into a pulmonary artery branch. The balloon is then inflated so that it completely cuts off blood flow through this branch. The pressure then seen at the balloon is ONLY the pressure seen looking forward (as the blood flow behind it has been stopped). This is called the pulmonary wedge pressure and is nearly equal to the pressure seen in the pulmonary veins and left atrium. In pulmonary arterial hypertension, this pressure is normal or nearly so. In pulmonary venous hypertension, this value is elevated. Except for some fairly rare conditions, such as pulmonary veno-occlusive disease, pulmonary venous hypertension is caused by left sided heart issues. Treatment of these varies based on what the issue is with the left heart but generally fall into the category of heart failure medicines such as beta blockers, ACE inhibitors, and diuretics.
  Occasionally, a patient with definite left sided heart failure is seen where the pulmonary pressures are MUCH higher than should be seen as the simple consequence of this. We say the pulmonary pressure has risen “out of proportion” to the left sided pressures. In these patients, both pulmonary venous hypertension and pulmonary arterial hypertension are present and treatment targeting both sides may be employed.
  It was once thought that diastolic heart failure was not as prevalent nor as serious as systolic heart failure, but we are finding out that is not the case.
Example of pressures in pulmonary arterial hypertension:Right atrial pressure > 10mmHgRight ventricular pressure > 30/15mmHgPulmonary wedge/Left atrial pressure < 13mmHgLeft ventricular pressure < 120/10 mmHg Example of pressures in pulmonary venous hypertension due to left ventricular diastolic dysfunction:Right atrial pressure > 10mmHgRight ventricular pressure > 30/15mmHgPulmonary wedge/Left atrial pressure > 13mmHgLeft ventricular pressure > 120/10 (most notably a markedly elevated diastolic number) I want to thank a new member for taking these notes -- sure did save me some time. Thanks so much Fred. 
At our July 12th meeting, Dr. Raed Dweik ~ Associate Professor and Director, Pulmonary Vascular Program ~ Department of Pulmonary, Allergy & Critical Care Medicine or fondly known as A90 will speak to us about. “The PHUTURE: scientific discoveries that may change how we diagnose, treat, and monitor PH”Thank you United Therapeutics for our feast for the day.The door prize was won by Dolly -- a new member to our group
HAPPY MOTHER'S DAY EVERYONE 

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Notes from March 22, 2008 Meeting ~ postponed from March 8th This has been a difficult winter and I am sooooo looking forward to spring. I want to wish all a Happy New Year, a Happy Ground Hog Day, A Happy Valentine’s Day and a Happy St. Patrick’s Day. Introduction of pharma reps and clinicians and thank you to Gilead Sciences for our feast of the day.
A moment was taken to remember those who have departed this life and are no longer suffering. We have lost three of our members since we last met in December. Jean Lelonis, Debbie Dean and John Ryland.
Happy Birthday was sung to Les Pollack who celebrated his birthday on the 22nd.
Old Business ~ These are Bills or items that need our attention ~ be sure to contact your Congressperson or Senator – I have a list if you’re not sure who your Congressperson is.
Genetic Information Nondiscrimination Act of 2007 S358
Ending the Medicare Disability Waiting Period Act of 2007 – HR 154Pulmonary Hypertension Research and Education Act of 2007 HR 3368Please be sure to contract your Federal Representative your Congressperson and ask him or her to co-sponsor this bill. Congressman La Tourette, Congressman Ryan and Congresswoman Marcy Kaptur have already co-sponsored; that leaves 15 more from Ohio. Please write or call me if you need help in doing this. Right now in Congress there are only45 who have signed to co-sponsor. We also need to contact friends and relatives in other states and get their support.
We are victims and survivors of this dastardly disease so let’s do what we can to keep it that way. The definition of victim is someone who is adversely affected by an action or circumstance or someone who experiences misfortune and feels helpless to remedy it. The definition of Survivor states: someone who remains alive despite being exposed to life-threatening danger 2. Someone with great powers of endurance ~ someone who shows a great will to live or a great determination to overcome difficulties and carry on. That's me and you and many others with Pulmonary Hypertension. We need to get our politicians involved with research and a cure.
The Medicare Rights Center will be hosting monthly free web seminars on important Medicare topics. The monthly seminars will be held on the second Thursday of each month. To register for these seminars, please visit http://www.medicarerights.org/webinars.html 
New Business: I have applications for PHA’s 8th International Conference to be held in Houston this year. The theme is: A Journey of Hope and Discovery. It will start Friday, June 20th and end noonish on June 22nd. The discount has been extended until April 1. The registration fee includes Conference Sessions, five meals, breaks, conference bag and t-shirt. Hotel reservations must be made directly with the Hilton Americas and are not included in the registration fee.
To be finished during our Chat and Snack Time -- ideas for next years meetings and other items Our meeting in September would be on the 13th and the Walk and Roll on the 20th. We decided to again hold our Walk and Roll at Akron State Park even though it was cold that day last September, the fireplace added an extra something.
November: we will attend the CCF Summit – it will be a one day event this year - Saturday, November 8th; we will then meet in December on the 6th..
At our March 22nd (only a little snow) meeting, our scheduled speaker was unable to attend do to the upcoming holiday and a family commitment so I spoke on 
                             Stress Management – 
Stress isn’t an external situation or event, but our personal response to change… our physical, mental and emotional reactions to “those” situations that arise in our daily routines. When we face a number of major changes in a short period of time, stress can feel overwhelming and without effective coping techniques, chronic stress can contribute to serious health problems, including heart disease, high blood pressure, panic attacks, a weakened immune system, depression, ulcers, chronic fatigue and migraine headaches. Learn to say NO—you may be taking on more responsibility than you can or should handle. No one is perfect; so don’t expect perfection from yourself or others. Meditate—just 10 to 20 minutes of quiet reflection a day may bring relief from chronic stress while increasing your tolerance to it. Use the time to listen to music, relax and think of pleasant things. Other ways of reducing stress ~ get enough sleep, exercise and control your diet – don’t overeat, have a support network, create peaceful times in your day (use a stress ball which is also good for strengthening your hands and arms, and breathing exercises), limit caffeine and alcohol – and for most of us those last two are no-no’s anyway, and no “drugs”. Remember that success with reducing your stress level will not come from a half-hearted effort, nor will it come overnight. It will take determination, persistence and time.

  At our December '07 meeting, Dr. Robert Schliz from University Hospital spoke to us about the Ins and Outs of Lung Transplantation. We learned the important process – what the blood work shows (how many gallons is required for that blood work), the contraindications of the process – who can and who cannot have or qualify for a transplant. How that infamous envelope cannot be pushed. We need to be in good shape and not be too ill or deformed (such as having kidney problems). The lung has to be the right size for our body, cannot be too big or too small. We also need a positive mental attitude and need support from family or friends. It takes approximately 60 to 150 days to wait for a transplant and during that wait there are many questions asked and answered which will also be useful in research. There are approximately 70 centers in the USofA that will do this, two being right here in Cleveland. We are very fortunate to be so close.

  On May 10th, Dr. David O. Taylor, ~ A Professor of Medicine for the Cleveland Clinic Foundation ~ Director, Heart Failure Special Care Unit ~ Director, Heart Failure/Transplantation Fellowship for the Department of Cardiovascular Medicine ~ Dr. Taylor will do a presentation on Pulmonary Hypertension Associated with Left Heart Disease. He will discuss the development of pulmonary arterial hypertension from chronic heart disease and the importance of the testing necessary to tell the difference between this entity and primary pulmonary arterial hypertension. Do to the holiday weekend, our speaker was unable to attend and as we all know, it is important to spend time with our families. Therefore, I did a presentation on Stress Management and some tips on how to deal with stress.
Zahi and Janice Kakish won the doorprize.
Thank you Gilead Sciences for providing our feast for the day.