20. Pulmonary Hypertension patient support group

May 5th - World Pulmonary Hypertension day 

Pulmonary hypertension is a severe life limiting condition where the blood pressure in the lungs gets elevated due to thickening of the blood vessels within the lungs. This can be due to various causes; in some patients there is no identifiable cause and this is called idiopathic pulmonary hypertension; in others, it may be due to lung disease; the third group of patients is our focus as they have a form of pulmonary hypertension that is potentially preventable. These patients suffer from a condition called Eisenmenger syndrome. In this condition pulmonary hypertension is a preventable complication of a heart defect that is present from birth.  If the heart defect had been detected and treated early during childhood the pulmonary hypertension could have been prevented and the patient would have had a normal lifespan and quality of life.

Birth defects of the heart occur in approximately 1% of all babies born today.They can present at various ages depending on the type and severity of the defect. Some severe defects are obvious right from birth but other less severe conditions may be detected only later in life. Children with heart defects can have breathlessness and feeding difficulties. They are also more prone to getting chest infections and have slow weight gain.

Delayed detection and treatment of birth defects of the heart can lead to permanent damage and high pressure in the lungs (pulmonary hypertension) making the patient unsuitable for definitive treatment like surgery or keyhole therapy. This underlines the importance of early detection and treatment of birth defects of the heart.

Once a diagnosis of Eisenmenger syndrome is made many patients are simply sent away with 'nothing can be done'. Although a complete cure may not be possible for these patients they do require regular follow up, medication and lifestyle advice on issues like pregnancy, contraception, exercise etc.
   

Until recently good medical therapy for this group of patients was not available and they were condemned to a life of gradual deterioration and early demise. Now, there are a wide range of medications that are available that can improve the quality of life of these patients and prolong their survival. The medications do come at a cost (between 750 and 5000 INR per month depending on the drug that is used) but they have been shown to improve the exercise capacity and quality of life of these patients and are indirectly believed to improve their long-term survival as well. Prior to starting medical therapy patients may require a cardiac catheter test to establish clearly that there is no scope for curative treatment.

Apart from medications these patients need counseling regarding various issues in life. Pregnancy in women with severe pulmonary hypertension should be strongly discouraged as it can lead to deterioration and death. Good advice regarding safe and reliable contraception should be given. Mild exercise like walking and swimming can be carried out but patients should be advised not to push themselves or participate in a competitive fashion.

Above all this, patients with pulmonary hypertension are often adolescents and young adults who suddenly find themselves having to deal with the burden of living with a condition without a cure. The doctor's consultation should allow them to express their doubts and apprehensions and help them face life with positivity and courage.

 To this end we have established a Pulmonary Hypertension Patient Support Group, which will function out of the CHIME office at MIOT hospital. We welcome all patients, families and members of the lay public affected by this cause to get involved with our movement and make our vision of eradicating preventable pulmonary hypertension a reality. Our first meeting was held on the 12th of April. Parents and children, came from places like Erode,  Vellore, Arkonam, and Thoothukudi and from Suburbs like Chrompet, Kolathur, Injambakkam. They shared their experiences and gained strength from each other. Parents said they always thought they were alone in this but they realized after coming to this meeting that so many people have been affected with PH. 

  

As a society we should aim to eradicate preventable pulmonary hypertension by early detection and treatment of heart defects in children. This can only happen with increased health education and awareness amongst the lay public and medical fraternity. Patients with pulmonary hypertension need support from their families, peers and the medical community to face the challenges that lie ahead.

I invite you to be part of this effort. Please email me on drsangeethaviswanathan@gmail.com for further correspondence

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