PULMONARY ARTERIAL HYPERTENSION
Pulmonary Arterial Hypertension (PAH)/ Pulmonary Hypertension (PH) is having high blood pressure in the arteries of the lungs
- Unrecognized disease of the lungs
- Serious lung disease that can be life threatening
- Can have devastating affects if left untreated
- Known as the “other high blood pressure”
- Simple name, complex health problem
- As of yet incurable
- Treatment options are available
- PH knows no boundaries it affects people of all ages and ethnic backgrounds
- An estimated 2000-10,000 have PAH in Canada
Science of Pulmonary Arterial Hypertension/Pulmonary Hypertension
What happens to pulmonary arteries to cause PH?
- Spasm
- Infection/Inflammation
- Clots
- Genetic Abnormalities
- Scarring
Mediators/Chemical involved
- Nitric Oxide
- Prostacyclin
- Endothelin
- Thromboxane
- Serotonin
- VIP
- VEGF
Quick Facts
- People with PAH often do not look sick; an invisible health problem
- Raising awareness about Pulmonary Arterial Hypertension SAVES LIVES
- An unrecognized and misdiagnosed illness
- Average person waits 3 years to be diagnosed
- PAH has several early symptoms similar to other diseases, such as asthma.
- Most cases are detected by echocardiogram and right heart catheterization
- Takes a series of tests to get final diagnosis – diagnosis through exclusion
- PAH research is rapidly advancing
- Doctors better understand the causes and mechanisms of PAH
- A PAH gene has been identified
- Gene/stem cell research currently going on in Toronto Canada
- Human trials started in Toronto, Canada December 2006
- This trial looks very promising – we believe a cure is possible
- Research advances will increase life span and quality of life
- Research will help reduce the cost of treating PAH
- Devastating impact on families
- Known as the silent killer
- Often affects mostly women in their child bearing years, mean age is 36
- Good days and bad days
- Can make the simplest tasks of daily living difficult
- 30% of patients do not survive beyond 4 years following diagnosis, even with current reatments
- Until recently 50% of patients died 2 years after being diagnosed
- Today 50% of patients survive 5 years or longer
- At 2000 PHA international PH conference “long-term club” was redefined to mean 10 or more years survival
- Many approaching or passed 20 years since definite diagnosis
- Only cure is lung (double/single) transplant
- Lead to death if left untreated – right sided heart failure will eventually occur
- Fundraising is important to those with PAH
- find new treatment options ~to give longer and better life for patients
- so very important to support the expanding public and private research
Signs & Symptoms
A combination of the following:
- Persistent unexplained shortness of breath (especially when climbing stairs)
- Fatigue
- Swelling of the ankles and legs
- Dizziness upon standing up too fast or climbing stairs
- Chest pain
- Fainting
- Dizzy spells
- Intolerance to exercise
| Epidemiology in Canada |
Condition |
Incidence/yr |
Prevalence |
Idiopathic (pph) |
60 |
500 |
Scleroderma Spectrum |
90 |
1200 |
HIV |
~20 |
400 |
Cirrhosis |
~120 |
4000 |
Thromboembolism |
~120 |
500 |
Congenital Heart Disease |
20 |
600 |
Treating Pulmonary Hypertension
Goals:
- Lower Pulmonary Artery pressure (PAP)
- Prolong survival
- Improve quality of life
- Reduce symptoms
- Improve ability to be active/exercise
- Provide opportunity to participate in life
- Minimize side effects
- Avoid bankrupting the patient
Treatments:
- Flolan (epoprostenol)
- Remoduli (treprostinil)**
- Tracleer (bosentan)**
- Revatio (Viagra/Sildenafil Citrate)
- Calcium Channel Blockers (CCB’s)
- Warfarin (coumadin)
- Lasix (diuretics)
- Thelin
- Digoxin
- Oxygen
In trials
- Sitaxsentan (Thelin)
- Tadalafil (Cialis)
- Ambrisentan
- Beraprost sodium
- Nitric Oxide – trials with PPHN infants,
- Iloprost (Ventavis) basically inhaled Flolan
Combination therapy being used now:
- Epoprostenol with bosentan and/or sildenafil**
- Remodulin with bosentan and/or sildenafil**
- Bosentan with sildenalfil**
**very costly anywhere from $5,000 to over $100,000 a year for approved PAH treatments
- very important for patient to see Pulmonary Hypertension specialist
- Only 2 adult PH specialists in BC
- Only 1 children PH specialist in BC
- Vancouver PH Clinic treats adults with PAH from all over BC
- All PAH children go to Children’s Hospital in Vancouver BC
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