What is Pulmonary Hypertension ~ now known as Idiopathic Pulmonary Arterial Hypertension (IPAH), previously known as primary pulmonary hypertension (PPH) or PH or PAH. I will refer to it as PH.
Often misunderstood; the severity of PH is not recognized by many insurance companies, employers or others who associate PH with common high blood pressure instead of a distinct and grave illness. Someone with high blood pressure can live 30 or 40 more years. Someone with pulmonary hypertension, which is the same but in the lungs, can live 2.8 years (??) That is without treatment. It is doable with treatment.
Often misunderstood; the severity of PH is not recognized by many insurance companies, employers or others who associate PH with common high blood pressure instead of a distinct and grave illness. Someone with high blood pressure can live 30 or 40 more years. Someone with pulmonary hypertension, which is the same but in the lungs, can live 2.8 years (??) That is without treatment. It is doable with treatment.
Symptoms of PH may include: Shortness of breath with minimal exertion, chest pain, unusual fatigue, a dry cough, edema, heart palpitations, fainting and dizzy spells.
The disease, described as "progressive and fatal", causes blood vessels in the lungs to thicken, restricting blood flow therefore making the right side of the heart overwork and lead to heart failure. It can be a silent killer but can also be treatable and therefore prolong your life. Studies show that most patients experience a one-year delay between the onset of symptoms and a confirmed diagnosis of PH.
The disease, described as "progressive and fatal", causes blood vessels in the lungs to thicken, restricting blood flow therefore making the right side of the heart overwork and lead to heart failure. It can be a silent killer but can also be treatable and therefore prolong your life. Studies show that most patients experience a one-year delay between the onset of symptoms and a confirmed diagnosis of PH.
Because of the rarity of this disease, most doctors do not know what it is. It can be present in all ages and does not discriminate with being a male or female or ethnic background but is most common in women of child bearing years.
People with PH before realizing what they have, often go to a doctor complaining of shortness of breath and unusual fatigue. If they are overweight or getting a bit older, their doctors may tell them to lose some weight and get more exercise.
It is often misdiagnosed or overlooked as a lung disorder or as a complication of a large number of respiratory or cardiac disease disorders. But remember, with proper treatment you can maintain a somewhat normal life and many
It is often misdiagnosed or overlooked as a lung disorder or as a complication of a large number of respiratory or cardiac disease disorders. But remember, with proper treatment you can maintain a somewhat normal life and many
SUPPORT ~ OUR BEACON OF HOPE
For more information about this disease go to:
You will find a wealth of information about PH as well as input from patients and caregivers.
Another good source for PH information is
Another good source for PH information is
To insure good health: Eat lightly, breathe deeply, live moderately, cultivate cheerfulness, and maintain an interest in life.
William Londen
William Londen
Merle Reeseman
Support Group Leader
For every journey no matter how long, it does begin with that first step.
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For a personal view about oxygen go to:
www.portableoxygen.org
It is mostly about COPD but very informative.
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Always ask your doctor if you are concerned about or don't understand the testing or numbers of your reports. This is information only and should be used only as a guide.
Pulmonary Function Testing
A look at Echos and other tests
Bubble Study with Echo
Pursed lip breathing~ breath in through the nose and out through your lips + slowly ;-0
www.portableoxygen.org
It is mostly about COPD but very informative.
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Always ask your doctor if you are concerned about or don't understand the testing or numbers of your reports. This is information only and should be used only as a guide.
Pulmonary Function Testing
A look at Echos and other tests
Bubble Study with Echo
Pursed lip breathing~ breath in through the nose and out through your lips + slowly ;-0
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American Lung Association Air Pollution,
How's your air, enter your zip code and you will find out
Quality of air means quality of life - EPA
USFood&DrugAdministration
American Lung Association Air Pollution,
How's your air, enter your zip code and you will find out
Quality of air means quality of life - EPA
USFood&DrugAdministration
Centers for Disease Control PH charts and info
Secondary Pulmonary Hypertension ~ SPH or some of the 'other' causes of PH
If there is a pre-existing disease it may trigger PH and then PH would be secondary to that primary problem/disease and be called SPH. Secondary pulmonary hypertension is relatively common but is still underdiagnosed. Reliable estimates of this condition are difficult to obtain because of the diversity of identifiable causes.
To name a few:
COPD
HIV
Lupus
Raynaud's Syndrome
Scleroderma
Sleep Apnea
Underlying congenital heart defects
such as VSD or ASD
Questions on Autoimmune Disease
Psoriasis
Scleroderma and PH
Fen Phen
Pulmonary Arteriovenous Malformation
CREST
If there is a pre-existing disease it may trigger PH and then PH would be secondary to that primary problem/disease and be called SPH. Secondary pulmonary hypertension is relatively common but is still underdiagnosed. Reliable estimates of this condition are difficult to obtain because of the diversity of identifiable causes.
To name a few:
COPD
HIVLupusRaynaud's SyndromeSclerodermaSleep ApneaUnderlying congenital heart defectssuch as VSD or ASDQuestions on Autoimmune Disease
Psoriasis
Scleroderma and PH
Fen Phen
Pulmonary Arteriovenous Malformation
CREST
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Caring Voice Coalition:
a source for: Insurance Reimbursement Program
Medicare Plan D Questions
Financial Assistance Program
Patient support Program
Public Advocacy Program
Caring Voice Coalition:
a source for: Insurance Reimbursement Program
Medicare Plan D Questions
Financial Assistance Program Patient support Program Public Advocacy Program ~~~~~~~~~~~~~
FYI~The average total lung capacity is about 5.8 liters (5800 cm3), however it varies from one person to the next and is dependent upon factors such as weight, sex, age and activity.
Women tend to have 20 - 25% lower capacity than males. Tall people tend to have a greater total lung capacity than shorter people. Heavy smokers generally have a drastically lower total lung capacity than non-smokers. To find out if your lung capacity is "good" or "bad", you should talk to your doctor - preferably a pulmonologist.
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Pharmaceutical Companies and Providers
(what they provide beyond the meds)
(what they provide beyond the meds)
Actelion Ventavis & Tracleer ~ Sure Steps
Encysive Pharmaceutical
Gilead, Inc. Flolan and NEW Letairis
Encysive Pharmaceutical
Gilead, Inc. Flolan and NEW Letairis
United Therapeutics, Inc. Remodulin PeerNetwork -
NEW Adcicra
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What do your pressures mean
PAP SYSTOLIC (Pulmonary Artery Pressure Systolic) represents the rapid blood flow from the right ventricle into the pulmonary artery. Occurs with the opening of the pulmonic valve.
Normal: 15-30mmHg
PAP DIASTOLIC (Pulmonary Artery Pressure Diastolic)represents the diminished blood flow from the right ventricle into the pulmonary artery. Occurs with the closure of the pulmonic valve.
Normal: 5-15mmHg
Normal: 5-15mmHg
MPAP (Mean Pulmonary Artery Pressure) is calculated as follows:
SPAP + (DPAP x 2)
3
3
have been known to go back to their regular routine. It is scary but it is also doable. It is a lung / heart disease so we need to take care of both.
Various testing will be preformed to eliminate other disorders and those are listed on the right (-->) BUT the golden rule of all tests is the right heart cathertization (RHC) this will let the doctor know your pulmonary pressures and what would be the best treatment for you. Some patients are on single therapy treatment some are on multiple medications. We are each unique in our treatment and we need to listen to what our bodies are telling us and in turn, relate that to our doctor.
Some who are currently on medication can look the picture of health but inside they can come apart with worry. They worry about where their next breath will come from. They worry if they can maintain enough strength to live a proper and somewhat normal life. (Remember doable) If there should be some form of an emergency or accident, they worry will this doctor know what I have and how to treat it properly. Many PH patients cannot follow standard procedure for normal/standard ER care.
Various testing will be preformed to eliminate other disorders and those are listed on the right (-->) BUT the golden rule of all tests is the right heart cathertization (RHC) this will let the doctor know your pulmonary pressures and what would be the best treatment for you. Some patients are on single therapy treatment some are on multiple medications. We are each unique in our treatment and we need to listen to what our bodies are telling us and in turn, relate that to our doctor.
Some who are currently on medication can look the picture of health but inside they can come apart with worry. They worry about where their next breath will come from. They worry if they can maintain enough strength to live a proper and somewhat normal life. (Remember doable) If there should be some form of an emergency or accident, they worry will this doctor know what I have and how to treat it properly. Many PH patients cannot follow standard procedure for normal/standard ER care.
For years lung transplants were the only treatment for primary arterial hypertension and just a few short years ago there were only three medications available for treatment. Today through research there are several available treatments and a number are still in trial. Those include:
Conventional Medical Treatments
-- Calcium Channel Blockers (CCBs)
-- Digoxin
-- Diuretics
-- Oxygen Therapy
-- Calcium Channel Blockers (CCBs)-- Digoxin-- Diuretics-- Oxygen Therapy -- Warfarin (Coumadin®)
Also
Also
Bosetan (Tracleer®)
Epoprostenol (Flolan®)
Iloprost (Ventavis®)
Sildenafil (Revatio®)
Sitaxsentan (Thelin®)
Epoprostenol (Flolan®)
Iloprost (Ventavis®)Sildenafil (Revatio®)
Sitaxsentan (Thelin®)
Tadalafil (Adcirca a PDE5 inhibitor) Trepostinil (Remodulin®)
Air travels to the lungs though a series of tubes and airways. The two branches of the trachea, called bronchi, subdivide within the lobes into smaller and smaller air vessels. They terminate in alveoli, tiny air sacs surrounded by capillaries. When the alveoli inflate with inhaled air, oxygen diffuses into the blood in the capillaries to be pumped by the heart to the tissues of the body, and carbon dioxide diffuses out of the blood into the lungs, where it is exhaled.
The smallest subdivisions of the bronchial tubes are called BRONCHIOLES, at the end of which are the air sacs or alveoli (plural of alveolus).
The ALVEOLI are the very small air sacs that are the destination of air breathed in. The CAPILLARIES are blood vessels that are imbedded in the walls of the alveoli. Blood passes through the capillaries, brought to them by the PULMONARY ARTERY and taken away by the PULMONARY VEIN. While in the capillaries the blood gives off carbon dioxide through the capillary wall into the alveoli and takes up oxygen from the air in the alveoli
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New York Heart Association Classification
I. PH patients in this category have no symptoms during ordinary physical activity, their hearts function normally.
New York Heart Association Classification
I. PH patients in this category have no symptoms during ordinary physical activity, their hearts function normally.
II. Although these patients are comfortable at rest, ordinary physical activity is somewhat limited by undue breathlessness, chest pain, fatigue, or near fainting.
III. These PH patients usually have no symptoms at rest, but their physical activity is greatly limited by breathlessness, chest pain, fatigue, or near fainting while doing routine things.
III. These PH patients usually have no symptoms at rest, but their physical activity is greatly limited by breathlessness, chest pain, fatigue, or near fainting while doing routine things.
IV. These PH patients are often breathless and tired even while resting and can't do any physical activity without symptoms. They show signs of right-heart failure. Under the WHO system anyone who is prone to fainting goes into this class.
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Though the right lung has three lobes, the left lung, with a cleft to accommodate the heart, has only two. The two branches of the trachea, called bronchi, subdivide within the lobes into smaller and smaller air vessels. They terminate in alveoli, tiny air sacs surrounded by capillaries. When the alveoli inflate with inhaled air, oxygen diffuses into the blood in the capillaries to be pumped by the heart to the tissues of the body, and carbon dioxide diffuses out of the blood into the lungs, where it is exhaled.
Though the right lung has three lobes, the left lung, with a cleft to accommodate the heart, has only two. The two branches of the trachea, called bronchi, subdivide within the lobes into smaller and smaller air vessels. They terminate in alveoli, tiny air sacs surrounded by capillaries. When the alveoli inflate with inhaled air, oxygen diffuses into the blood in the capillaries to be pumped by the heart to the tissues of the body, and carbon dioxide diffuses out of the blood into the lungs, where it is exhaled.
Questions on Irregular Heartbeat or Arrhythmia's
1 866-474-8326 Specialty Pharmacy
2003 Classification by WHO – World Health Organization
There were 5 classifications given for defining different kinds of PH.
1. PAH – IPAH, occurs without any apparent cause – Familial – APAH (Associated with PAH) similar to IPAH but exposure to toxins, or the effects of other diseases (scleroderma, lupus), congenital heart disease, chronic liver disease, HIV, diet drugs etc
2. 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 etc
4. PH due to chronic thrombotic and/or embolic disease – obstructions (clots) etc
5. Miscellaneous – Sarcoidosis, tumor, fibrosing, etc
2. 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 etc
4. PH due to chronic thrombotic and/or embolic disease – obstructions (clots) etc
5. Miscellaneous – Sarcoidosis, tumor, fibrosing, etc
Pulmonary Artery Pressures another view:
Systolic Pressures: (ECHOs show Systolic Pressure.)
Mild PH: 40-55 mm hg
Moderate: 55-75 mm hg
Severe: above 75 mm hg
Moderate: 55-75 mm hg
Severe: above 75 mm hg
Mean Pulmonary Pressures:
Mild PH: 26-35 mm hg
Moderate: 36-45 mm hg
Severe: above 45 mm hg
Moderate: 36-45 mm hg
Severe: above 45 mm hg
15 is considered a normal mean pulmonary artery pressure and anything over 25 is considered PH.
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