Maya Nair* (name changed on request), 52 year old from Kochi, with a diagnosis of VSD – Ventricular Septal Defect and PH – Pulmonary Hypertension, shares her experience of the complexities of these conditions as a young child, understanding of the diseases, seeking the right treatment at the right time and the journey in managing them.
Symptoms, Diagnosis & Treatment
I was born with Ventricular Septal Defect (VSD) and still have the hole in my heart. When I was about 3-4 months old, my parents rushed me to a hospital in Ernakulam district due to breathlessness and continuous vomiting, where some diagnostic tests were done, possibly catheterisation and was told the condition had to be fixed with a surgery. When I was 4 years old, I was taken for the surgery to the best available hospital at that time. Doctors came from outside India, but said I was very underweight and the surgery would be highly risky and the decision was totally up to the parents. My parents decided not do it till I gained 11kgs of weight based on the doctor’s advice. However by the time I had gained 11 kg, and went to check for surgery at Medical college Kottayam, Pulmonary Hypertension (PH) had started.
Symptoms of PH started with breathless when cycling and unable to swim like my cousins.
In the interim I was on Penicillin injection. After 4 years I had some pain in the legs and was treated with Penicillin when I consulted the doctor around 5-6 years which was continued till I was 11-12 years.
I was also prescribed Digoxin and all medicines were stopped after 15 years. Never had any major hospitalization till I was 22 years old. Until then, I never got to know or asked my parents about my condition as I used to see my mother crying. Only when I was 22 years, the doctor told me about my condition. What happened was that I was at a dance function and the next day saw 1-2 drops of blood came from my mouth. This was Hemoptysis which started the first time. I was admitted in Medical college Kottayam. There, the doctors gave me Amoxycillin and let me go home. They informed me that this is a usual symptom of PH and VSD and to worry if blood came through nose, as that could mean it could affect my brain. My oxygen saturation was also fine.
I now consult a heart Specialist who has a very nice support team.
When I was abroad, I had Hemoptysis again and went to emergency and they put me on oxygen as saturation was around 82-83 and I was also put on home oxygen. Oxygen saturation is 90 now.
I had consulted 2 doctors, Pulmonary and Heart specialist who suggested a heart and lung transplant. They suggested to go for Transplant consultation as my immunity was really low, mainly to gain emotional and physical support. This transplant was very risky and could cause many other serious diseases like cancer, diabetes, later on, due to suppression of my immunity to accept the foreign transplanted organ. However they did not guarantee life post-transplant even for 1 year.
Medications I am currently on
For treatment of PAH, I take Celexipag (Selexipag), Macitentan, and Tadalafil (very heavily priced in India, spend 28000 per month).
I take 3 medicines for Hypertension – Metoprolol, Indapamide, and Candesartan.
I tried homeopathy but it was not helpful.
Social, Medical and Family support
My parents especially my mother, who is 81 years old is very active and supports me when I am in hospital. Financially, my husband takes care of everything. They are my pillars!
All friends and relatives had sympathy for me. When I was a child, I never asked my mom as she was worried and crying and wanted to protect me. Gradually I understood I was different when I overheard my mother crying and telling relatives about my condition.
I never asked for any help from my parents as I didn’t want to hurt them.
I have no regret about not knowing anything. It is a gift given by God to me and not knowing then was best – similar to how I will handle if it happened to my daughter. I know how to manage that very well.
There is no family history of my condition. And during my pregnancy, testing was also done and also for my daughter during pregnancy and after. Thankfully she is fine.
Lifestyle adjustments and accommodations
I did not have to make any major lifestyle changes. I grew up like a normal child – at 5-6 years I started schooling. I had to climb a hill daily to get to school. I wanted to be like other kids because getting different treatment affected me. My grandmother was strict and disciplined and wanted all of us to live together. I wanted to be like my sisters even though I knew I had issues like breathing. Even then if you looked at me, no one could call me a sick kid. People would always compare as they knew about my illness. Since I was very thin and my sister chubby, everyone felt I was very unhealthy. She was treated like the queen in my family and everyone gave her importance. I felt kind of jealous or the society forced me to think like that.
My parents worried about my future. Father was a pharmacist which helped as he also gave me a lot of additional vitamins.
I would have breathlessness while cycling, swimming or running too much. Was the worst dancer but still wanted to compete with others and sing even if I know I cannot complete the song. But will still try and do it. Didn’t want to be different from others.
During college, I was determined to complete my degree. At the age of 22, I started working for 9 months at my brother’s friend’s place. Found a person who was interested in me there. Once I developed fever and was admitted to hospital, so I resigned from that job. I didn’t want to hurt him or want anyone to be troubled because of me. After 4-6 years, he came to my family and asked me to marry him. When I asked him why he was selecting me with my condition, as he was an engineer and would get many other fitter girls. He said there could be issues with other girls too. All in the family liked him and so I got married.
My brothers wanted me to sit at the family business and were very protective of me. They bought a scooter to ferry me around.
Coping Emotionally
I gathered all my strength and boldness and I felt I could do everything. I was an average kid at studies but a smart girl. I could find an answer for anything other than studies. My mother is a retired teacher. She looked after me very well. We always visited the Etumanoor temple in Kerala and she encouraged me to pray to God who gave me my life.
Growing up, things were not easy. When my younger sister younger started her periods, it was celebrated while there was sympathy for me as I still hadn’t got them. I got mine at 17 years and it was all normal.
When my younger sisters started getting married, my parents were worried about me. I got rejection from the extended family and relatives; sometimes knowingly or unknowingly, they avoided talking about me.
No one mentioned Counselling to me. The whole concept didn’t exist then.
I couldn’t find good friends to talk to me and or give me any answers. I tried sometimes to write it down and throw it, then I found solace in God. This made me feel free and completely out of stress.
Present condition and Challenges
I continue to have hemoptysis in a big volume. I was in hospital beginning of August 2023, when they closed 6 holes in veins – they did Catheterisaton, Embolisation to close the veins. This can happen anytime but takes 1-2 year for major vein to cause issue. Very small veins sometimes heal on their own. Once I spit out the blood, I get back to normal. But I cannot breathe when there is heavy bleeding.
For the first pregnancy no one was ready to support me due to risk for child and me. I had an abortion in Kerala. I felt I could take the risk at the age of 32 and decided that I will conceive and if anything happens to me my husband can find another life partner. I did not discuss this with my family or even with my husband. In 2003 I gave birth to my daughter. The real challenge came in 2008 when I was abroad and medication was started for PH. Back then only 3 medications were available in the world and I am taking all 3. Abroad they are risk averse and were not supportive of my third pregnancy and hence had to undergo an abortion again.
Advice to patients who face similar challenges
You have to find a way out and a dependable person whom you can rely on for relaxation. Plan your future and be prepared for what can happen. Listen to your body. Talk to someone about your worries. Be confident in yourself. Treat this as a gift and accept that you are different from other people and decide which way you have to go and follow that. Have to get through all emotions, especially anger, always be happy and confident. Believe in yourself! Society was telling me I cannot live up to 50years – who makes this decision? Even medical doctors were telling me that I will not live up to 50 years. And this was in 2007 when I was 36 years.
As I was committed to fight back against society, I became confident. This also meant that I took my medication properly.
Worry for the Future
I cannot plan too much ahead and take things one at a time. I have no regrets – no happiness or sadness. I do not keep any emotions and even maintain an emotional distance with my husband or family and remain detached.
My wish for other patients:
I have been very lucky financially and socially – but it is not the case for others, there is no help available. Access to Government hospitals needs to be made easy.
As adults they don’t get a job as society doesn’t accept them as a normal person. But Life needs to be lived.
Best experience was the normal delivery for my daughter. This helped boost my confidence,
Worst experience – As an adolescent, social treatment I received was a bit tough, but I take that as positive coaching.
