I am a current survivor of a rare and devastating lung and heart disease known as Pulmonary Hypertension – it is a silent killer and a DASTARDLY DISEASE. I don’t want to make lite of other (lung) diseases as they, too, are devastating.
When I do get to go out and about, people that I haven’t seen for a while will come over (remembering that I have some kind of a “condition”) and say – “Gee, you’re looking good” to which I reply: “Why, thank you, the medicine must be working”. Or they might say: “How are you doing?” and depending upon the day, I may say: “How much time do you have” or my favorite one is “If I were any better, I’d be almost “normal”. The point I’m trying to make is: I am sick, I am
very sick but with the medicines I take, I look healthy. We shouldn’t take it for granted that just because someone looks healthy they are healthy.
Unfortunately many doctors do not know what Pulmonary Hypertension is and don’t pick up on the symptoms.
I was not feeling well; I had a lot of trouble breathing
The good news is that more and more Dr’s in this area are becoming aware of PH. For me it took a considerable amount of time to be diagnosed with Pulmonary Hypertension, about two years of pokes and probes and a battery of testing.
Obviously, I was not feeling well; I had a lot of trouble breathing. At the request of my daughter, and believe me it was more than a request, I went to a heart specialist and that is when I was sent to the Cleveland Clinic Foundation – more testing, more pokes, more probing which led to the “gold seal” of all tests, a right heart catherization– it was after this that I was told I had Primary Pulmonary Hypertension and with what they found, I would need the medication called Flolan, an infusion type medication; a catheter is put in your chest which leads into your heart; the tubing is attached to a pump for continuous flow of the medicine.
By this time in my illness I was going downhill and was given a 6 month death sentence, which had changed from the original two years to live if a treatment was not found.
This was the only option I had
The right side of my heart was three times its normal size from being overworked and I was going downhill faster than anyone had expected. With Flolan I could possibly have an additional 10 years added to my life.
My insurance company refused to pay for any infusion type medicine so CCF asked if I would be interested in going on a research program and if it were successful, I would receive the medicine free for the rest of my life.
This was the only option I had at the time.
Unfortunately, this medication did not work for me so I had to have Flolan. It was a major ordeal to get this medication but I got it and by that time I was told that I would have had only a few more days and it had been only a few months since the previous time line or death sentence.
I went through major surgery, a major move and change in my life style and had to recover on my own even though I was told to have someone with me 24/7 for at least two weeks. That was part of my ordeal but I’m not going to get into that right now.
I thought after the operation I would be able to run the marathon, instead it felt like the marathon had run over me!
After the surgery, a nurse came to my house to show me how to mix the Flolan and to be sure I hooked up to the pump correctly. This medicine has to be mixed daily and has to be changed the same time every day as it only has a shelf life of 48 hours. It also has to be kept chilled.
there is hope and there is support
As I’m sure all of you can say, we’ve been through a lot but the important thing to remember is that there is hope and there is support available from and for each other.
For PH patients there is an international support line through www.PHAssociation.org, it has a wealth of information and the people there are just great. They will do what they can to help and inform you and always seem to be available when you need them.
It is very important to be a part of your disease but don’t let it dominate you.
When PH is found and treated soon enough, you can have a normal life. I have met patients who bowl, play tennis, ride motorcycles, go golfing, children who learned to ride two wheelers; some have become ballerinas.
Just because you have a devastating disease doesn’t mean you have to be devastated.
Know as much about your disease as you possibly can, let your doctor know (often) how you feel, try to keep a daily journal of how you feel, have there been any changes that you have noticed; bring that with you for your appointments – highlight the important issues you want your Dr to know or have questions about.
It is very important to be a part of your disease but don’t let it dominate you. You are in charge of your body, learn to have some control. Remember, your doctor is your
employee, you are paying to be taken care of – tell him/her what’s happening to your body.
Right now and for me, my disease is treatable and I consider myself on a maintenance program; my death sentence has temporarily been lifted. I am a fighter and by the grace of God I will survive! Hoo Rah!!!