A PH Story

Part I

By Eye Shaa Malik

Everyone has a story.  Here's mine...

How long you have been with PH? What medication are you on?

It’s been more than a decade, approx. 12 years, I am 21 and I was diagnosed with ''severe'' pulmonary hypertension in June 2003 due to large VSD (ventricular septum defect) with bidirectional shunt after angiography. Prior to that I have had a "small" congenital VSD (a small hole in heart) but when I was 3 it was “filled” naturally, and I was alright without “surgery”. I lived a healthy life until I was 9.

I found myself breathless, exhausted and fatigued even after little exertion. We thought it was asthma and went to many physicians and ENT specialists. Finally a doctor noticed my hands were blue and questioned if I had any heart problems in past. He suggested going back to my cardiologist as he thought there might be something wrong with my heart.

My cardiologist asked for an immediate angiography after which there was a meeting of various cardiologist and my family.  They (doctors) predicted my longevity to be 2.5 years.  The repeated what was written on my reports, '' ITS TOO LATE, NO ELEMENT OF RECOVERY COULD BE FOUND.'' This very line literally reshaped the perception of my entire life.

                            

I went to every possible place where the best doctors were available, but because there were and are no medications approved for children, I have been continually referred.  I have undergone various treatments, trials & experiments. Finally they found a medication regimen that worked, I was put on tracleer (Boonton), sildenafil (penegra), warfarin and lesoride (my current treatment, as well). While they said I would never survive without oxygen, I only use it at night.

My life was forever changed after diagnosis.  Since that day, I have always been treated as "Regina" by my loved ones, nobody ever dared to scold me. My parents and siblings were always gentle with me. I used to tell my friends I had asthma, as  I thought it would make sense to them.  I actually thought PH was another name for ''Heart asthma.'' However, my cardiologist scolded me and said, "If you knew the severity of what you have you would never call it asthma again! I can expect only an illiterate person to compare it to asthma."

What are you able to do that doctors said you couldn't? 

For the first five years, I was completely unaware of the fact that I had PH and that the doctors had thought I wouldn't live longer than 5 years. My doctors asked me to quit my studies after 11th grade.  I was doing pre-medical which they thought was too demanding and stressful. I argued and promised them I would avoid stress and I somehow passed it with A+. I was a good student and never ever thought, "Oh! I'm chronically ill.  I am about to die, why am I studying?"

However, I fell more severely ill and this time as the hole in my heart enlarged, it was decided my pre-med studies were a no-go. It was suggested I choose an easier course of study to have less impact on my health. I used to think about it for the days and nights and cry the whole night because I felt I wanted to do so much, to be so infused with all the energy to put into my work, but I was not able to, and that was really frustrating....

I had nothing else to do and an empty mind is a devils workshop. Pessimism started creeping up on me, and it felt there was no way to cast off melancholy. But, my parents helped me to choose a course of study, honors in English literature & linguistics. There have been many days of exacerbation, but I think to "give up" is accepting your defeat and that being a PHighter or warrior you are not supposed to give up.

They told me to avoid stairs and sports, I was actually supposed to limit my activities that demands more physical exertion... but, I am good at aerobics (an amazing choreographer, or so everyone says). My cardiologist says it’s not good for me, but whenever I listen to my favorite music I cannot help it, I consider it my exciting pulmonary rehab.

I am studying at a place far away far from home and living in hostel.  I have to do everything on my own and that's a big deal for me. I am able to do everything, but I need  "little breaks,” my friends call it "pause time". I think PH doesn't hinder you from living a normal life, we may just need to do it a little more "carefully." 

What is your dating life or marriage like with PH/Oxygen?

I was in 11th grade when I was being told by my doctor that "you are not supposed to get married." It was a great shock for me, I have never  considered myself as a patient, so I argued with my doctor and asked why.  He said "You cannot take care of yourself,  how can you manage a home of your own?”

After 2 years my cardiologist said the same thing but in a condescending way, he remarked "NO ONE ACCEPT AN OPEN HEART WITH OPEN HEART " ... a brutal statement indeed.  It served as an eye-opener for me, I couldn't argue this time because everything was so clear, tears blurred my vision because the initial acceptance of some facts is really a hard pill to swallow but once you make up your mind everything becomes easy.

It still resonates in my mind and hits me hard emotionally, but I am not convinced by this idea. My parents wanted to me to grow-up an independent and brave girl. People will definitely not be calling me a "Spinster." 

I am not saying a PHighter should or shouldn't get married, I am just trying to say this world is so cruel and being patient there is a possibility that you can be rejected or considered as a big "responsibility,” by a potential partner. Nearly all of the PH patients I know do marry, have kids (biological or adopted) and are living their lives happily because they are lucky ones to get their "rainbow" after the hurricane. But not everyone is lucky enough, I am an eternal optimist, I do believe in soul-mate theory.

But my focus and priority is my health so my ''BIG DAY'' would be the day I’ll get perfectly alright, yes that miracle day! I have seen many people posting in PH groups they are so anxious, perplexed & concerned about it (finding a partner), I would say "HAVE A FIRM FAITH" stay "STRONG" , KEEP YOUR SPIRITS HIGH , if luck favors you ! Great! If it doesn't that's not end of life...

One thing that my mother says when pessimism lapses on me "DON'T WORRY WHO KNOWS WHERE THE WIND MAY BLOW FOR MY GIRL."

Hardest part of life with PH?

The hardest part was the "acceptance" that I have a chronically terminal illness and I've a short time to live and being a kid this acceptance-phase prolonged  ... my doctor told me that only "will power '' will keep me alive. I've conceded to PH and every reality related to it with dignity....

Another hard part was when I couldn't study what I wanted to or I grew up dreaming of, because a person with right heart failure was not eligible.

I've had my days when I was really down and losing hope, but this was a greatest "learning-journey”. That pain must have taught me about the life, people & myself. It’s been a massive learning curve, so much about everything .I've experienced good things and bad things myself and that has taught me that the world is not all flowery, everybody doesn't want good for you ,friendship's changing equations, dynamics .I've also started to " grow-up" after fiercely resisting for many years.

Besides PH I have vigorously swollen "tonsils” for approx. 14 years and it hurts, in extreme condition it becomes red  ... My ENT specialist says I have needed surgery for the past 13 years, but due to anesthesia risk, they call me a HIGH RISK PATIENT and no one is ready to take that risk. That's why I am desperately waiting for a “miracle", seemingly all doors are closed for me but I have a blind faith in God that he’ll open all the doors at right time.

So, this was some of my story. In nutshell, I think my disease has helped me focus on what's important and let go of little things. I've made peace with the fact that there is a possibility I’ll die young, but HOPE is everything. Even healthy people aren't promised tomorrow. Respect the fact that you're a PHighter, it demands bravery as each day is fight and adventure,  pain demands to be felt, life isn't about how to survive the storm, but how to dance in rain so be thankful for what you have and you'll end up having more.

I literally count my own blessing and think that if the pain is so damn bigger, reward would be colossal. 

Stay tune for Part II of "A PH Story"