as told as part of the Long-Term Survivors Panel at PHA's Sixth International PH Conference held June 24-27, 2004, in Miami, Florida
About 10 years ago in August of 1994, I started having some scary things happen to me. I noticed that my feet started swelling, and I’m not talking just a little here, I’m talking about swelling so bad that they jiggled like jello with every step I took.
I also started shortly thereafter having problems going to my room upstairs over the garage. I could not even get halfway up without having to sit on a step to catch my breath. Now I thought this to be quite odd since at the time I was actively using crystal meth and thought I should be having all the energy in the world. I was still so scared though.
I also noticed that my periods had stopped. Was I pregnant?
At the same time I was also laid off from my job, which I later would be so grateful for.
My friends kept telling me that I needed to be seen by a doctor right away, but I had this slight little problem: I was terrified of doctors, and also was afraid of what they would tell me. I was also deadly afraid of needles.
So my friends got together and decided to trick me. They said they were taking me to dinner, but a few moments later I found myself being pulled into an emergency room by my arms. I was definitely not a willing participant in this little scheme of things.
Needless to say, the doctors admitted me right away, and after a few days of tests, yes, including needles, they found nothing, but said I was possibly pregnant. They did a pregnancy test that came out negative, but still did a Doppler and heard what they told me to be a baby’s heartbeat.
They later realized that this was my own heartbeat echoing off all the fluid in my abdomen. So they made an appointment for me to see a medical specialties doctor at their clinic the following day after my release. I went through hell and back with test after test, for about six months, was called by the doctor’s office with a follow-up appointment, and was given the bad news. I had primary pulmonary hypertension. Prognosis – poor.
All I could do was cry, sitting there in front of the doctor alone. He starts talking to me, but I don’t think I heard everything he was telling me. My head was spinning hearing only words here and there like “no cure,” “only 5 to 10 year survival,” and so on and so on.
My first thought: my kids. How was I going to tell them this type of news? They were so young, only 11 and 14 at the time.
I cried some more. Then the doctor asked me if I had ever done Fen-Fen, or taken any other diet pill. I said no, but I cam clean about the crystal meth use with him. He said I needed to have the gene for PPH, but that the use of meth probably did not help the situation. I was so angry at myself then.
My next step was going home to tell my boys. Their reaction, tears, and the same thing with the rest of my family and friends. Now, what to do next.
The first medication they started me on was Lasix and potassium chloride for all the fluid retention. That helped a lot, since by now I had ballooned up to 238 pounds from 120 pounds. I started losing weight right away.
In a few months I had been in and out of the hospital so many times that I had lost count. I was also diagnosed during this time with congestive heart failure, and was put on Digoxin for it.
By August of 1995, I was told I had an appointment at Harbor UCLA in Torrance, CA, to be seen by Dr. Brundage about being put on Flolan therapy.
I was seen by the doctor, admitted right away to the hospital and immediately started on this new therapy. I looked up at this machine hanging above me in the hospital and started crying again. How would I ever live with this ball and chain???
Well live I did, and found myself starting to get used to my little friend that went with me everywhere I went.
The doctors also had an oxygen machine delivered to the house for me along with some portable tanks for going on the road. Oh God, I thought, something else to be hooked up to. Just what I needed for my self esteem, right? Well, I got over it.
After being on Flolan for a few years, I started having severe skeletal pain in my legs. Walking became extremely difficult. I found myself bedridden. I had stopped walking.
Because of this, I also had severe contractures at my knees, and also developed drop foot. I finally told the doctor what was going on and he had me admitted to the hospital to be seen by a pain management team.
After two weeks, I was sent home with new pain meds, and was to be visited by a nurse to start physical therapy. It took about a year, but I was back on my feet but was told I would have to use a walked or at the very least a cane to get around.
They also told me I would need surgery to release the drop foot so my heel would touch the ground. But the stubborn girl that I am showed them! As you can see I walk by myself just fine. And with the help of my boyfriend’s encouragement, I can also walk barefoot, and I never did get that surgery.
During the time I was bedridden, I noticed I started eating less and less. What was going on now???
Before I knew it, I had completely stopped eating. The coumadin I was taking had also ripped apart the lining of my stomach, causing a severe upper GI bleed, sending me back into the hospital for four blood transfusions. I weighed in at 90 pounds!
My doctors were not happy at all that I had been taking my coumadin on an empty stomach. This is what caused the bleeding, they told me. They also talked to me about a feeding tube being put in surgically into my gegurnam below the stomach.
They asked me why I wasn’t eating, but I told them that the smell of food made me sick to my stomach. I agreed to the feeding tube only because I wasn’t ready to die yet and leave my boys.
So, back into surgery I went. Now I was attached 24/7 to three things: the Flolan; the oxygen; and now the feeding tube. Talk about feeling unattractive! Got over it though. You gotta do what you gotta do to get well, no matter what it takes.
While on the feeding tube, I started passing out here and there because my sugar level was so low. After waking up in the ER the second time, I was diagnosed with Addisons disease. That’s another one added to the list of illnesses. I was started on new medication for this, and within two days was starving and finally ready to eat. After I gained enough weight, up to 110 pounds, the feeding tube was pulled and I was sent home.
Something amazing happened after a few years: I started feeling really great! I was also told about Tracleer, and my doctor, Dr. Oudiz, said let’s try to lower the Flolan and start this new medication.
After awhile, I was still feeling great, able to shop again, and what a thrill that was! After titrating the Flolan down from around 60 ng to 9 ng, I was admitted into the hospital for a heart catheter, where my Flolan was reduced to zero and watched for three days.
I felt so great, so they removed me from the Flolan! I had also been off the oxygen for some time too. Lord almighty, I was free at last!!!
I walked out of the CCU without being attached to one thing. I felt like a walking miracle, considering what I had been through for the last nine years.
Today I feel almost as good as I did before being diagnosed with PPH. My pressures are in the mid thirties. I started in the low 100’s. I feel it if wasn’t for all the research done for PPH, I would not be standing here in front of all of you feeling as good as I do today.
That said, I want to thank everybody involved in ANY way for their help in taking care of us patients. Research is a truly wonderful and amazing thing. I especially want to thank my doctor, Dr. Ronald Oudiz and his two right-hand women, Joy and Daisy. Without the three of you behind me every step of the way, who knows where I would be today.
And to all the patients at my support group, I thank you, too, for being my rock of support. To my family, especially my boys and boyfriend, I love each and every one of you for backing me in my adventure of life. To all of you still going through hell with PPH, all I can say is please don’t give up. Try to stay as positive as you can, and use all the support you can get your hands on. Don’t be afraid to ask for help whenever you feel you need it. You will get through this. Stay Strong everyone. Thank you!