Young patient shares her story on Rare Disease Day

This is Kinari Mehta. She is 29 years old.

“Everyone has a deep secret. Today I am revealing my biggest yet. 

For years, I have hidden that I am terminally ill. 

I have Pulmonary Arterial Hypertension. It’s a rare progressive disease affecting the lungs and heart. 

From the outside you can’t see that I have this condition. I kept being ill a secret for many years as I wanted to protect the people I love around me and because I have been determined to live a normal life. 

I choose to tell my story today as it is Rare Disease Day and I want to use my experience to raise awareness of Pulmonary Arterial Hypertension and help others with this condition.” 

Pulmonary Arterial Hypertension causes high blood pressure in the pulmonary arteries, which carry blood to the lungs. This results in the walls of the arteries becoming narrow and stiff, restricting oxygen flow around the body. It puts much more strain on the heart, causing it to become damaged and lead to heart failure. Pulmonary Arterial Hypertension is a life-limiting condition which has no cure.  

Over 3,000 people in the UK are living with managed Pulmonary Arterial Hypertension, with many more thought to be living with the disease undiagnosed.

The symptoms of Pulmonary Arterial Hypertension include shortness of breath, back pain, dizziness, extreme fatigue and chest pain, as well as anxiety and depression. “Back at high school I suffered with back pain, which looking back turned out to be the muscles around my lungs working harder due to the condition. Then at university I started to notice I was getting very breathless and fatigued – I’d walk across campus at my usual pace and get out of breath. Simple things like walking up a flight of stairs or being able to enjoy a meal with family after work were no longer things I could do with ease. I also had blackouts, some of which caused me to temporarily lose my sight, which was terrifying.”

Because the symptoms can be masked by more common conditions, it can be very hard to diagnose. It took several years of investigations before Kinari was referred to the pulmonary hypertension service at Hammersmith Hospital, where she was diagnosed in April 2014. Kinari has to take medication every day to slow down the narrowing of the arteries, and has had a number of operations to treat the condition. Since August 2017 she has also needed to wear a legacy pump 24 hours a day, which gives her a constant stream of intravenous medication.

Kinari has been fighting the condition with the support of her immediate family and medical teams at Hammersmith and Harefield hospitals. She credits her positive outlook on life for keeping her going. “With Pulmonary Arterial Hypertension, you don’t know what your prognosis is going to be – I don’t know how quickly or aggressively it will continue to progress. It is easy to slip into emotional lows, but my sheer determination not to let my illness get the better of me has kept me going. There are many things in life to be grateful for and every day I count my blessings.”

Kinari’s best hope to prolong her life is a double lung transplant. Another reason she’s sharing her story today is to encourage more people to join the organ donation register. “I am eagerly waiting for that call saying that they can give me the transplant.”

Currently people in the UK have to opt in to the organ register in order to donate their organs. There is an Organ Donation (Deemed Consent) Bill currently progressing through parliament, which proposes to change the system to an opt-out one. 

“I was really nervous about letting people know that I have Pulmonary Arterial Hypertension today. But I’ve always known that I wanted to do something with my illness to help others. If I can help even one other person out there by raising awareness of this condition, and by encouraging people to join the organ donation register, then I would be content.”

Read Kinari’s story in her own words at her blog.

You can join the organ donation register here.

GPs: if you suspect a patient may be showing signs of Pulmonary Arterial Hypertension, you can refer them to our community cardiology and respiratory service in the first instance.

Learn more about Pulmonary Arterial Hypertension at NHS Choices.