"I have a beautiful 8-year-old son who I want to keep up with and I can barely walk across my house without resting. My husband is at his wits end having to handle everything, but he is trying to stay strong and supportive. I am a very independent active person so to have it all taken away is been very hard on me as a person."
My journey began many years ago. I was diagnosed with a rare pulmonary disease before the age of 2 called idiopathic pulmonary hemosiderosis (IPH). I had it most of my young childhood, in and out of hospitals. Then early tween years I went into a remission and for many years following I led a normal, active life. Around age 30 I noticed I had more breathing limits and after having my first child at age 32 I felt even more limited. I then went to see some new doctors who diagnosed me with a form of emphysema with underlying IPH (my childhood illness). I began treatments of inhalers and stayed stable for many more years. Then things began to get way worse. My pulmonary function tests were staying stable but my activity level decreased dramatically. I then decided to start the steps toward getting worked up for a lung transplant, and one of the required tests is a right-heart catheterization.
After those tests I was told I had secondary pulmonary hypertension (PH), as a result of my lungs being under so much stress my heart was overcompensating. I started out on calcium channel blockers and they did nothing, then I went on Revatio and felt way worse. Now I am on Adcirca and Lasix and still no big signs of improvement. I work out of my home as a designer but not much anymore these days, I have a beautiful 8-year-old son who I want to keep up with and I can barely walk across my house without resting. My husband is at his wits end having to handle everything, but he is trying to stay strong and supportive. I am a very independent active person so to have it all taken away is been very hard on me as a person . . . I have changed. The next steps would be to test me on the inhaler drug in the hospital for a week . . . just the idea of having to do a nebulizer four times a day for 45 minutes is not ideal, but if that does not work then it would be the catheter self-mixing continuous drug . . . so I do not want that. And lastly the infamous lung transplant, but with those odds I can only see choosing that if I am already dying.
I have many family and friends who are always sending me stories of people they have heard had successful lung transplants and when I look those people up to see how they are today most are dead. I am a positive person, but those odds are terrible and the life you have post-surgery, the only pro is you can breathe again but you are at the doctor’s or hospital all the time dealing with tests and drug reactions. Sometimes I wonder if it is worth just living out the days my lungs and heart will give me then to deal with all that and most likely die from effects of the drugs rather than the disease itself.
I am at a loss of where to turn. I would love to get on the right drug that treats my PH so that I can resume some activity for a while and deal with a possible transplant years down the road.