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
