The story of Jenn
February 28th is Rare Disease Day, with the main goal being to raise awareness.
I have an incurable, life threatening lung/heart disease called Pulmonary Arterial Hypertension. Not to be confused with high blood pressure.
Pulmonary Hypertension is high blood pressure in the arteries of the lungs. The arteries become small and narrow, causing the right side of the heart to need to work extra hard to pump blood to the lungs. The reality here is, eventually, the patient goes into heart failure, because the heart just becomes too weak to pump and maintain its job.
The only “cure” is a double lung transplant.
I was diagnosed almost 11 years ago. At the time of diagnosis, I was told that without treatment I would be dead before the 5 year mark. Luckily enough, I live in a country where we have amazing doctors and healthcare and I have done very well throughout this journey so far.
The main symptoms of PAH are shortness of breath, fatigue, syncope- (which means fainting), dizziness, chest pain, edema.
For the first 6 years, I responded well with oral meds. In 2009, I started fainting again, and could barely walk without needing to stop and rest because I was so out of breath. Not to mention having absolutely zero energy, even day to day activities like taking a shower was completely exhausting. I was admitted again and put on Flolan, a continuous IV therapy administered through a pump I haul around with me. Flolan is scary, but I am grateful to have it.
Having a rare disease is hard, to say the least. No one knows about it, not even some of the doctors and nurses at the hospital are aware. I have not been admitted in the entire 5 year timeline I’ve been hooked up to Flolan, but I am terrified of the day I will be, because I have to be completely on top of EVERYTHING because of my pump. The nurses sometimes will try and stop the pump (It can NEVER be stopped, I can go into what is called Rebound Pulmonary Hypertension without my Flolan, and can actually die in just 6 short minutes without my medicine.) So even if I’m deathly sick, I have no choice but to be alert enough to be on top of everything, until I can get myself 6 hours south, where the PH clinic and my specialist is located. I can’t even explain how unsettling that is.
It’s also hard because a lot of the time, people make assumptions that because I might be having a good day, I am fine every day. I am unable to work, because I cannot commit to a full time job because I’m just too exhausted to do so anymore. I do not look sick. On the outside. It gets old having people tell you this constantly. I realize most people without a chronic illness do not understand and are confused at appropriate things to say to someone who is sick. If I could open up my chest and flash my lungs, (morbid thought), that comment would likely never be spoken again.
11 years ago, I was told I was 1 in a million because this disease is so rare. It’s becoming more recognized today but it’s still unheard of to many. I always get surprised if someone actually knows what I’m talking about when I mention Pulmonary Hypertension. Admittedly, I don’t always believe them either, until we start having a conversation and I realize they in fact DO know what they’re talking about. It’s not always the case though, as like I had mentioned in the beginning, a lot of people mistake it for high blood pressure. More often than not, we are our own best advocate. I have to see a nurse once every 2 months in order for my medicine to be delivered and it can get confusing because a lot of time I am assigned a new nurse. While these nurses have some knowledge of PH, there have been times I have told them stuff they didn’t know, which is always fun. I am also basically my own pharmacist/chemist, as I mix my own medicine on a daily basis. Sometimes people are surprised at how knowledgeable I am with my illness, but knowledge is power and I wouldn’t have it any other way.
As with most illnesses, early diagnosis is so important. I was misdiagnosed for 2 years with asthma before I got my accurate diagnosis. Even though I have this rare, invisible, incurable, potentially fatal illness, I was grateful to finally find out what was wrong with me and get started with treatment.
Having a rare illness is incredibly isolating. Support is key and being able to talk about things, always helps me. Writing has always been my way of getting things out and expressing myself. Every single friend I’ve met online with PH means the world to me and I thank them for their continued support. I don’t know where I would be today without these people in my life.
For more information on PH, check out this amazing website- phassociation.org