Karthika Ramachandran narrates her story of pain of living with Pulmonary Arterial Hypertension (PAH), and the strength and resilience that helped her manage the whole process of PAH treatment and lung transplant recovery. What started as a habit of journaling her daily experiences eventually inspired her to turn those reflections into a book.
Please tell us about your conditions and when you were diagnosed.
I am a 33 year old single mother and a Logistics Officer in Dubai, born and raised in UAE.
During my pregnancy in 2016, I did not realize what was healthy to eat and what was not and was told by my family that I need to eat for two and hence had gained a lot of weight. Therefore, after my pregnancy I got into fitness and lost about 32kgs. Since then, I avoided unhealthy food, ate only home cooked food, followed a routine of 8pm bedtime and waking up at 4am. I was leading a normal and healthy life with a stringent work out and fitness schedule. In 2019, I suddenly noticed a drop in my stamina, some giddiness, low blood pressure, so consulted my GP (Internal medicine). I was given a drip and my condition was diagnosed as vertigo. I took medicines for a week and then stopped as I generally hate to take medicines and also primarily as I had no respite from my symptoms.
As my symptoms persisted, I was asked to get all ENT related tests, MRI and many other tests, including pregnancy tests. They were all found to be normal.
My GP then suggested an ECG which showed some variation and it was recommended that I consult a Cardiologist. After this I was asked to get an Echo test done. My cardiologist revealed that the ECHO tests indicated an issue with my right heart and also noticed an issue with my lung, so I was required to consult a Pulmonologist. It was only after this consultation that I discovered that the pressure of flow from my heart to lungs was 120 instead of the normal range of around 20. Both doctors - my cardiologist and pulmonologist consulted on this and confirmed the diagnosis as Pulmonary Arterial Hypertension (PAH).
What was the treatment after the diagnosis of PAH?
A Right Heart Catheterization (RHC) procedure was performed immediately; wherein a thin flexible tube was inserted via my thigh leading to my right ventricle, atrium and the pulmonary artery to measure the exact pressure and oxygen levels within my heart. The pulmonologist explained to me that the PAH could be caused due to many reasons, some of which could be tested but this would then be a very long process. I was asked whether I was on contraceptive pills for long or had any complications during pregnancy since PAH could happen with pregnancy complications. However, my pregnancy was normal and very smooth. Eventually after some more tests, my condition was diagnosed as Idiopathic Pulmonary Arterial Hypertension.
What was your reaction to the diagnosis?
For a person who did not ever visit a hospital, this was all very new to me. Even during the ECHO test, I was alone in the room. At that time, I wasn’t aware of any serious condition other than cancer. For the diagnostic consultation too, I visited the hospital on my own. The pulmonologist shared the diagnosis with me, explained what PAH was and specifically told me not to check on Google. I was keen to know the life expectancy for PAH. The only thought I had was about my daughter - a girl who was just 3 years old needed her mother and her responsibility was my biggest concern.
What was the initial treatment? And how has the treatment changed over time?
I was started initially on one medication - Tadalafil once a day, then the doctor changed this to twice daily for better control on the blood pressure in the heart. Tadalafil helped bring the pressure down from 120 to 50.
A second medication Ambrisentan was started only after a week due to an availability issue, but had to be stopped due to side effects. As the medication was administered on a high dose, I gained 4kgs in a week.
Were there any complications from the primarycondition and how did your transplant happen? How did you handle it? How did you mentally/emotionally cope with it all?
After two years in early 2021, my breathlessness came back again. The oxygen saturation level dropped to 94 which was lower than the normal (typically 95-100). No tests on this were done earlier and hence there was no reference. I was taken to Emergency immediately and then to ICU after ECHO. The pressure was high and had gone up to 107. I was therefore immediately shifted, in an ambulance, to a premier hospital, where the best treatment was available for PAH. I was now prescribed triple therapy Selexipag, Riociguat and Macitentan, which I ideally should have started earlier. I had started with Selexipag at a lower dose earlier. I couldn’t tolerate this even at a lower dose initially as I had severe headaches and vomiting. I had no side effects of Riociguat and Macitentan.
I had swelling all over my body, vomiting and severe headache that caused me to roll on the bed in pain. The appearance of my face and skin tone had changed, had a lot of hair fall and joint pain along with headache, which was also triggered with high humidity. In addition, exposure to sunlight was a torture. There was a lot of jaw line pain while eating. All these side effects settled down in 6 months - by November/December 2021 after my body had tolerated the dosage of Selexipag. I then went back to work. Along with medication, walks and cardio workout without any exertion were important to help bring down pressure. I was advised against any weight training workouts. In January 2022, the dosage of Selexipag was further increased.
However towards the mid of 2022, my pressure started rising again. Family issues and separation stress added to the complications. Right Heart Catheterization was repeated again in March 2023, through my neck this time. After this, the doctors had to resort to Sub Cutaneous delivery of Treprostinil which was more efficacious than the oral medication course and also helped manage some of the side effects. I was told the next step was transplant in case Treprostinil medication doesn’t bring down my pressure.
This prompted me to take a second opinion in India. There I was advised not to take sub-cutaneous treatment as this could cause a lot of bleeding for women during menstruation as a side effect. I was told that I would need heart and lung transplant only by checking the hospital reports I carried with me, and that the reports showed my heart has fibrosis, but my doctors in UAE had never mentioned something like this. I was also told straight to the face, with no empathy shown, that the life expectancy will be 5 years in case I do the transplant now and if I delay it then that can reduce the life expectancy too.
I then made the decision to go back to Dubai and opt for sub-cutaneous treatment with Treprostinil. The technician came home and explained the whole procedure in detail. Luckily, there was no extra bleeding or issues with my periods.
The pain from the procedure though was really bad. This medicine has to be delivered continuously through a tiny tube (cannula) connected to a small battery-powered pump. Infusion catheters are placed under the skin preferably abdomen area as it would be easier to manage on my own. However this too came with issues. The pump had to be connected 24/7. It could only be disconnected while taking a shower, as the pump is not waterproof, but could be disconnected for a maximum of 30 minutes. The abdomen site area had to be always covered with a waterproof plaster to avoid any kind of infection. The plaster had to be changed with great care using sanitized hands and also the skin around that area got very sensitive and painful. I started medicine with low dosage and as the dosage increased, the pain also increased. I had a swollen abdomen for about 12 days when the catheter was inserted onto a new site. The site had to be changed every month or whenever there was any infection. I was getting an infection every 2-3 weeks. The pain was unbearable. I was in pain 24/7. Even after 12 days of site change, the pain only reduced and did not go away completely.
I continued my Bharatnatyam dance classes but eventually took a break because there were chances of infection while sweating.
I had to sleep in one position on the chair and couldn’t move, with most of my activities being restricted. But eventually it was the sub cutaneous pump that kept my pressure stable at 70. However in 2 months my infection frequency had increased and I had to go back to my doctor for antibiotic treatment to understand how deep the infection was at the site of the patch. Once during a full body check up including a Mammogram, the nurse happened to notice that my body temperature had risen to 39.5F which indicated that I probably had contracted an infection. I was taken to emergency and swab tests were done immediately. I tested positive for Covid and Pneumonia and was admitted to the hospital in November 2023 for 10 days.
All these issues were affecting me both physically and emotionally. Once, I was in so much pain that I almost regretted taking Treprostinil subcutaneous treatment. My breathlessness also had returned and even doing small tasks became difficult. Once, when my parents were on holiday, I had almost given up on the medication, But my daughter entered the room and a big tight hug from her made me realize she needed me and I then decided to continue with the medication.
Preparing for the transplant
I then received a call from the clinic, that my name was listed for lung transplant. Once the donor was confirmed I was told I would be given a 24 hour notice for the transplant surgery. As the lung transplant was discussed as a last resort even earlier, and tests were already done for the preparedness in Nov 2023, I started evaluating this seriously in January 2024. I was advised a psychiatric consultation to mentally prepare myself for the transplant and to ensure any family issues didn’t affect me. All home care and support was planned.
On February 14th 2024, during my routine check up, doctor told me that the Donor is ready and I got admitted on the same day and surgery was planned for the next day. The double lung transplant surgery lasted 8 hours.
After the transplant
I regained consciousness after about 24-30 hours, was on ventilator for a day and oxygen support for 2-3 days. I did not feel any pain through the procedure or after and was in consultation with pain management team for any back pain. I found it difficult to sleep after I was moved from the ICU to the cardiology ward. I had to remain in a sitting position constantly, which helped me sleep better. In 2 weeks time I was able to walk and shower on my own. I got discharged on March 1st 2024.
Everything was much easier post transplant as there were less issues with my breathing and no pain as before. Care had to be taken to ensure there was no infection. My mother was a strong pillar of support and she made sure the house was clean all the time and I got clean cooked food to avoid any kind of sickness. Hygiene is very important post transplant.
Follow ups
Initially, immediately after surgery, regular check up was scheduled for every week and then the frequency of follow up was in 1 month, followed by Bronchoscopy after 6 months. I contracted Covid again in August 2024, which luckily didn’t affect my lungs.
After a month I resumed work from home and then started going out in 3 months for walks for about 4 kms. It has now been over 1.5 years post the transplant and am back to normal with my regular activities, with the course of medication that is needed post transplant.
What medications are you currently on?
I am currently on Immuno-suppressants, antibiotics and vitamins and iron supplements. The body doesn’t produce enough iron and this further drops during menstruation. I also take a number of supplements like Calcium, magnesium.
List of my current medications include liposomal iron and ascorbic acid , atovaquone, tacrolimus immediate release capsule, calcium carbonate plus Vitamin D, mycophenolate mofetil, prednisolone, azithromycin, metoprolol tartarate, pantoprazole, magnesium capsule, voricinazole, aspirin, rosuvastatin.
Have you tried complementary medicine or therapies? If yes, did it help?
I did evaluate Ayurvedic medicines, but then was advised that if I started Ayurvedic medicines I had to stop the allopathic medicines, so did not take the chance.
Were there any interventions done other than medication and why and when was this done? What kind of specialists (including Physio/ Occupational therapist/ Psychiatrist etc.) do you consult and how often?
Cardiologist and Pulmonologist were the main specialists for my treatment and surgery. I did go to a Psychiatrist/ Psychologist, who prescribed an anti-depressant which I started but stopped soon after since I was afraid of the possible side effects I had heard of and also because I thought I could manage without these medications.
My personal life and separation from my husband was affecting me. To avoid all the arguments related to this, I did opt for online counselling sessions. Tried to adjust and manage this on my own, however the physical pain was more troublesome than the emotional support needed
Do you have a family history of this condition? Was genetic testing done?
There was no family history, yet genetic testing was done, but all of those results were negative.
What changes have you made to your lifestyle because of this condition?
I did not find the energy and time to stand and cook for long time. My diet had also dropped and was restricted to some protein, brown rice pulao with some vegetables to add on. I was not allowed to lift any weight and I always preferred workouts with weights, so did not resume my workouts either. After my treatment with medication and sub-cutaneous therapy, I had to continue my work outs to keep up my energy levels and also weight under control.
How do you navigate work? What kind of accommodations, if any does your employer need to provide?
My work place was very flexible. I was allowed to work from home whenever needed. Even while in office I used the lounge facility to rest if I did not feel well or felt tired. I did not want to burden anyone with handover and continued work to as much as was possible.
How has your family supported you?
My mother supported me a lot throughout my illness. I couldn’t have managed without her. She helped and took complete control of my daughter - getting her ready for her school and taking care of all her needs. She also took care of all the house work in addition to my daughter’s school work. I also got a lot of support from my brother in Abu Dhabi, with whom I stayed during the hospital sessions. Apart from this my sister-in-law, sister and brother-in-law who were also in Abu Dhabi, took care of all my needs.
What is your present condition? What were some of the challenges you continue to face?
As far as PAH is concerned, I have no issues and am carrying on with my normal life with all my medications prescribed. I still experience some pain during menstruation and once had severe bleeding. My doctor did a complete check up and all was normal
What made you write a book?
I had a habit of writing a diary and a connected habit of keeping things to myself. If I experienced any pain, I started journaling in a a book and then onto the phone, especially when I had sleepless nights. I then decided to write a book to create awareness as there aren’t many support groups for this. For instance, no one was available to advise me on the sub-cutaneous treatment. Also all PAH medications are expensive.
I realised that not everyone is ready to open up about their condition. Once I contacted this lady who had done crowd funding for PAH, but she did not agree to openly talk about her condition or about crowd funding too.
Another person had written a book and that helped me build confidence in starting to write a book. I realised that if I told the world about my experience, it may help someone with PAH to build peer connections. Now I know four people who have PAH who did not have peer support, which was a similar situation to me in my initial days. If there was knowledge, awareness and needed support, I would have had some hope then of managing this condition even better through others experiences and knowledge.
There was no one to help me. Nor was anyone aware of PAH. I had to explain PAH to my parents and I only told them once I got to know about it. It is now easier to share about my condition, with the expectation that there is no cure but can be managed.
The hope and wish I have now is for a little longer life with medications and through transplant. I am aware that even after the transplant, life expectancy with leading a normal life can be 10 years and of course this is subjective and may vary with individuals, after which I may need another transplant.
I am now determined to going back to a lifestyle I followed before PAH and have decided to live like there is no tomorrow. I follow a low carbohydrate and protein rich diet, ensure I get proper sleep and have regular check ups.
Link to the book: Smiling Through The Pain
What are some of your learnings that you would like to share for other patients who face similar challenges?
I think it is always better to take care of your health from the start - Wake up early, go to the gym and work out routinely. One needs to prioritize health. Watching my 60 year old mother who is regular with her workouts was very inspiring and encouraging. Also my advice is to get into the habit of drinking enough water. Keep a check your daily screen time.
What are you worried about for the future?
At present I feel I have gone back to my normal life. When the diagnosis happened and I was told, as well as researched, that with PAH I would have a life span of about 5 years, my only concern was my daughter, as she was just 3 and was worried how and who would take care of my daughter if things did not go well. But the right diagnosis and the lung transplant, which my body tolerated, was the best thing that could happen to me and I have recovered well due to my age.
