FAQs: Condition


I am breathless and easily fatigued. The doctor is unable to cure the condition. Is it PH?

It may or may not be.

Breathlessness and fatigue can happen with many other medical conditions – asthma, COPD, pneumonia, emphysema and the like.

As PH is rare, it could easily be a condition other than that.

 

Is PH diagnosis difficult?

One needs to rigourously test to establish PH and the specific variant one suffers from.

One might need to follow an elaborate ‘testing sequence’ leading upto an expensive CT Scan/ MRI.

More on common tests for PH, here: http://www.phassociation.org/page.aspx?pid=1257

Misdiagnoses are common with PH as it is rare and there are many medical conditions that bear the same symptoms. If symptoms persist, one should check with a specialist - an experienced Pulmonologist or a Cardiologist. For a young one, one can seek a Paediatric Cardiologist. General Practitioners (GPs) might not be able to help with PH.

Bear 4 things in mind, if testing for PH

  • As far as possible, try and be with an experienced and alert doctor (regardless of age), preferably practicing in a well visited hospital or clinic. This matters because medical fraternity’s familiarity with a rare condition like PH is important in monitoring/managing the disease.
  • Take a second opinion with an equally good doctor/ hospital/ clinic.
  • Do not shy away from taking an expensive test. Testing negative for PH could be the best thing you might hear. Testing positive might help you lead your life much better - an early diagnosis helps. Check: http://www.phassociation.org/SometimesItsPH
  • Develop, if possible, an understanding of the medication (at least the sequence the drugs are usually prescribed in). This can also help you judge as to whether you are with a doctor that is right for you.

Note: Some PH variants may require the services of a rheumatologist or an immunologist.


To understand the laboratory and non laboratory tests, visit: http://labtestsonline.org

 

I have PAH. I am told I will not live too long, perhaps only 2 years. Is that true?

The prognosis for the disease is very poor. In other words, no one can tell for sure, including the doctor.

Moreover that kind of timeline was true of the 80s and early 90s, when not many drugs/therapies were available. With newer drugs/ drug deliveries/ therapies, an average patient will, most likely, live healthier and much longer.

Then again, alternative medicine is an option in India and countries of South Asia.

Be happy, enjoy humour and live every day with the trust that you will live long.

 

My PH has been given a “Class”. What is it?

Once diagnosed with pulmonary hypertension, the doctor may classify the disease using World Health Organisation guidelines:

  • Class I: When symptoms (fatigue, shortness of breath or chest pain) do not exist at all / No limitation of physical activity
  • Class II: When symptoms do not exist at rest, but come about with normal activity / Slight limitation of physical activity
  • Class III: When comfortable at rest, but have symptoms with less than normal activity / Marked limitation of physical activity
  • Class IV: When symptoms exist even at rest / Inability to carry out any physical activity without symptoms / Impairment of the heart


Does one need to take precautions as a PH/PAH patient?

Precautions that are desirable:

  • having supplemental oxygen available during air travel
  • careful and medically advised use of antibiotics for respiratory tract infections
  • getting vaccinated for pneumococcal pneumonia and flu (pneumonia can be a  serious complication with PH/PAH patients)
  • prevention of infections (like endocarditis) before undertaking dental procedures


Is there a cure for PH/PAH?

In a lot of cases, PH that is secondary can go away once the underlying condition has been treated.

Pulmonary Thromboendarterectomy, a surgical technique, is helpful in curing patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) due to recurrent or multiple blood clots in the lungs (also known as pulmonary embolism).

See: http://www.phassociation.org/page.aspx?pid=3834

In rare cases, PH may also respond dramatically to calcium channel blockers/ prostacyclin.

PAH, virtually incurable, has become a 'marquee' disease in medical research circles. One reason could be the belief in some quarters of the medical fraternity that a cure for PAH would be a step forward in finding a cure for cancer – as in some PH patients, the condition is a case of undying cells in the pulmonary artery – in theory, similar to cancer.

Another reason could be that a cure for PAH could be an important landmark for gene based/cell therapies.

The interest in finding a cure for the disease is exceptionally high in mainstream academic and research circles. So, there is hope.

Similarly, many underlying causes for secondary PH are being attacked in laboratories worldwide, genetics and biotech being key enablers frequently. 

A snapshot of promising research: http://www.phassociation.org/Research/PHA-NHLBIAwards

Also visit the Vera Moulton Wall Centre (Stanford School of Medicine), which was set up thanks to an anonymous gift of several million dollars: http://wallcenter.stanford.edu/

Promising Research there: http://med.stanford.edu/labs/rabinovitchbland/research1.html

More research: http://www.cam.ac.uk/research/features/breathtaking-new-treatments-for-a-fatal-lung-disease

 

I have PH/PAH. Do I need a heart/ lung transplant?

Heart/ Lung transplant is reserved for patients who have stopped responding to treatment. It is usually a measure of last resort.

In India, there have been sporadic attempts to perform this operation.  A hospital or two might be in the running to establish a practice of sorts, by bringing in trained surgeons.

Issues that concern:

  • Availability of a donor body and organ(s)
  • Establishment of a transparent, nation-wide registry for transplant candidates
  • High, operative costs
  • Post-operative infection management

 

Are certain groups more at risk with respect to PAH?

Yes. Especially young women.

There are anecdotal reports of onset of PAH symptoms during pregnancy and among postpartum women (i.e. after childbirth).

Estrogen, essentially a female harmone, has been linked to PH - both, negatively and positively. This has come to be known as the 'estrogen paradox' *

People older than 50 years are also at a higher risk.

*

http://www.ncbi.nlm.nih.gov/pubmed/22561960

http://ajplung.physiology.org/content/299/4/L435.full.pdf+html


Can children have PH/PAH?

Yes, they can.

FAQs on PH in Children: http://www.phassociation.org/page.aspx?pid=2510


How should one deal with one’s condition?

Golden rules:

  • Ask the doctor to write down the right medical term for the condition on the consultation papers/ prescription. Next ask him/ her DOs and the DONTs, with respect to drug interactions, lifestyle and nutrition.
  • Visit a library or surf the internet for information on the condition. There is likely to be an overflow of medical literature on the internet, especially of the clinical variety (aimed towards medical professionals). Look for 2 things:
    • reliable sources of information based on reputation, association, neutrality and/or expertise/depth
    • level or language you can understand
  • Find and master the basics – symptoms, warnings signs, test results/ readings, drug chronology and action, possible side-effects/toxicity, mechanism and chemistry of the body/organs, dysfunction related to condition
  • Be in touch with the body. Understand it. (Caregivers to listen carefully to the patient in order to do so)
  • Look at credible supplementation and alternative therapies/remedies/cures. Carry the information in print form to your doctor and actively discuss the possibility of its use (including safety).
  • Exchange information and experiences with fellow PH brave hearts.

 

Do drugs cause PH?

Users of, now discontinued, anti obesity (appetite suppressant) drugs Fenfluramine and Phentermine (popularly known as fen-phen) have reported a 23 time increase in the chances of contracting PH in their later years. Aminorex, Dexfenfluramine and Amfepramone (Diethylpropion) are other anti obesity drugs that been shown to bear the risk of contracting PH.

Illicit drugs such as cocaine/ amphetamines and rare toxicity in foods (monocrotaline) can cause PH as well.

Suspicion also points to estrogen compounds used as oral contraceptives or in hormone replacement therapy and aspartame (artificial sweetener) consumption.

 

Should other members of the family be tested for PH?

The chance of PH occurring in the other members of the family is negligible for most variants.

However, for patients with certain kinds of Pulmonary Arterial Hypertension (PAH), members of the immediate family (parents/ siblings/ children) may be at an increased risk of PH. The overall chances of the occurrence of PAH in a family member can be 1 person among 10.

A screening test like an Echocardiogram can only help point to the existence of the disease or otherwise (not carriage of a mutated PH gene). Genetic testing is therefore useful in cases where genetic mutation has been identified as the cause for PAH and/or 2 or more people in a family have or had PAH. Carriage of the relevant BMPR2 mutation bears a 20% chance of developing PAH.

No member in India has yet reported the availability or use of relevant ‘gene testing’. There are 6 labs available in India for gene testing. It is not known at this point in time as to whether they can test for a PAH gene. Lab details are available on http://www.ncbi.nlm.nih.gov

On genetic testing: http://www.phassociation.org/page.aspx?pid=924