I was diagnosed in November of 1991. I was recently (six months) divorced with a three year-old son. I worked as a nurse in a doctor's office. I was never sick, but the year before I had been hospitalized with a strange virus that they never identified. After that, I developed pneumonia and was sent to a pulmonologist. He did a chest x-ray and saw something on it, but he didn't know what it was. So then he took the x-ray to the local radiologist at the hospital who said it was nothing, it was normal. I found out a year later at the cardiologist office that is was a severely enlarged pulmonary artery.
One day, a year later, I passed out at work, but everyone laughed it off because I had just drawn blood on my dad. As the day progressed I couldn't make it from one end of the hall to the other end, so one of the doctors scheduled an echocardiogram to look for mitral valve prolapse. Well, of course, that wasn't the problem. The next afternoon I went to see a cardiologist.
He looked at the chest x-ray and put me on a treadmill. I only lasted three minutes and my heart rate was over 200 beats per minute. He told me he wanted to do a right heart catheterization the next morning, so my mom and I went to the hospital. After the catheterization my mom acted strange. My preacher was there, and he acted strange too, but no one would tell me anything. I should have known something was going on because my baby brother brought me a brush, perfume, chocolates, and books for my night in the hospital after the catheterization. My best friend brought me what I still call my "sick gown."
The next morning I went through some more tests and then the doctor sat on the side of my bed and told me, "you have primary pulmonary hypertension, and you will not live two years without a heart/lung transplant." I laughed and pulled the covers over my head, and then I heard my mom crying. Whoooooo. You think the strangest things at a time like this. I sat up and said to him, "you mean I can't have any more children?" You see, even though I was divorced, I still wanted more than one child.
The doctor got me an appointment at the University of Alabama, Birmingham to be evaluated for the transplant. I went to Birmingham with my mom and my son in tow. The hospital was great. They arranged daycare for my son and a room for my mom. We went by the daycare to see where it was so my mom could take Nathan the next morning, and on the door of the daycare they had a sign that said we have chickenpox. Oh well. This was not my year.
The good news was that after a week of literally being poked, prodded and catheterized, I found out that I didn't need the transplant right away after all because I was responding to calcium channel blockers. My doctor stayed on top of my pressures by doing right heart catheterizations every six weeks for years. I did great on calcium channel blockers. Lasix was later added, but that wouldn't work so one doctor tried Demadex. That worked, and then Coumadin was added, and then potassium because of the Demadex. I did great for a long time.
Let me backtrack again. In 1992, I met a wonderful man who knew about my primary pulmonary hypertension and my limited prognosis and he fell in love with me and my son. We fell in love with him. We were married in June 1993. He does more than just take care of me. He's amazing. He has been with me every step of the way since the day I met him. Best caregiver in the world. He knows before I do if I am getting sicker or if I am doing too much, which I tend to do.
I had a collapsed lung in 1994 and started going downhill in about 1995. We changed calcium channel blocker brands, and that worked for a while. Several years later I was still searching for the perfect pulmonary hypertension specialist and I find one in New Orleans. We tried Viagra before it was even being tested, but I couldn't handle it. But the calcium channel blockers started working again. When my doctor moved to Houston, his office referred me to another doctor in New Orleans and I still use him. I finally had a doctor that listens. Nothing can compare to having a doctor you trust and you can tell everything to. He is all about the quality of life not the quantity for me and that is what I need.
I got worse again and he tried Sub-Q Remodulin. I think all of you that are able to use that are the toughest people in the world. My stomach actually had big holes in it from the medication. I developed cellulitis, so thank goodness we stopped that. I went on Tracleer the day it came out on the market and it worked for five years with my other medications. Last spring I got a cold—and I never get colds—but this one went to my chest and I ended up in the hospital; I nearly got pneumonia. That was the beginning of a spiraling downhill time for me. We were ready to try Flolan, but I begged my doctor to try Ventavis again. I had been on it the year before, but didn't do well on it. This time though, Letaris was coming out and I wanted to hold out for it. Well, the Letaris, Ventavis and Norvasc combination seems to be my cocktail of the moment.
That was 3 years ago. Tyvaso came out shortly after that and I begged again. It just seemed to be able to fit with my lifestyle so much easier than the Ventavis. I love the Tyvaso. So, for three years now, I have been stable and even my pulmonary artery pressures have improved. I use Tyvaso, Letairis, and Norvasc. I also use Demadex for the fluid that gets out of control sometimes still and potassium to compliment the loss from the Demadex. I also use Coumadin to thin my blood, hormones because I had a complete hysterectomy in 2000, (abdominal cut, and they used an epidural so I wouldn't be put to sleep), Prilosec for my tummy.
I had my gallbladder removed almost two years ago and my pulmonary hypertension specialist talked to the surgeon and told him the precautions they would need to take. I was able to do a Tyvaso treatment right before the surgery and right after and I had no problems at all.
Now, some lighter stuff. I have been interviewed by the local CBS station a couple of times to talk about primary pulmonary hypertension. I have been in a magazine. The magazine was a faith based publication. I would not be able to handle this at all if it weren't for my Lord and my church. I was interviewed and had a whole page article in a local newspaper and was the getting to know person in another paper. Another patient and I started a support group in Mobile. I keep telling them I'm going to separate off one day and start a Baldwin County group. Well, I did start a Pensacola group and turned the Mobile one over to someone else. My Pensacola support group is amazing. It's small but everyone is close and talks to each other all the time. We are growing.
I have been living with idiopathic PH for 20 years now and I am going to be here a long, long time.
God is good.
If you ever need me, email me at firstname.lastname@example.org. I will be happy to offer hope.