About 16 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 bit 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 babys 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 6 months, was called by the doctors 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 3 to 5 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 came 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 loosing 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 right away on this new therapy. I looked up at this machine hanging above me in the hospital and strarted 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 a 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. During all the time I was put on the UNOS list for a Double Lung and Heart Transplant. This scared me to no end. The trip down to UCSD were brutal for me. But I did it, and learned to deal. After being on the 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 2 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 walker 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 boyfriends 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 altogether. 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 4 blood transfusions. I also weighed in @ only 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 2 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 removed from the UNOS list and put on standby. What a relief that was. I was also told about Tracleer, and my doctor, Dr. Oudiz, said lets 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 8 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 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 9 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 if it 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 2 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 by 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.
UPDATE AS OF APRIL 2009: I have now been on Inhaled Remodulin for 1 year now along with Revatio. It has done wonders for my ability to do things in my life. I have been extremely active, traveling, amusement parks (without the use of a wheelchair), and shopping, shopping and more shopping. It's an amazing treatment. It has affected my stomach a bit, but a small price to pay for feeling this great!!! To all you newly diagnosed patients...hang in their and keep a smiling attitude!!!
UPDATE FEBRUARY 2010: Last month my doctor had Oxygen delivered to the house on an "as need" basis...I can handle that! He also is adding Tracleer back into the medication regimen...I can handle that too...my pressures are at 45, which isn't so bad in my head...but we all know, I'm a bit crazy! Will keep everyone posted if anything else changes!!! Love to all of you who read this and can take anything from my ramblings and put it to use in their lives...LOL...
UPDATE MARCH 2010: Still waiting on the approval for Tracleer...forgot how long these things can take! Been a bit short of breath lately, but I think that's due to stress! Oh well...I'll keep on going anyway and pushing myself!!! Love to all!!!
UPDATE MARCH 3, 2010 @ 9:55am: Just got the word that my Tracleer was approved!!! YIPPEEEE!!!
UPDATE MARCH 24, 2010: OK...well I'm really starting to feel one of the side effects of Tracleer now, something I kinda forgot about...one HELL of a headache!!! Aaaahhhhhh...
UPDATE OCTOBER 22, 2010: Things have been going pretty good with my illness...meds are working well. I had to have my Tyvaso lowered to 3 puffs per session so I'm feeling much better in my stomach. October 9th, 2010 was such a forward move for me...I started the First Support Group Meeting for the San Luis Obispo, CA and surrounding areas. The meeting was a HIT!!! Glad to meet fellow PH'rs in my area and help them to navigate this illness. I'm also a Peer Network Mentor now. Helping fellow patients from around the USA get thru the rough areas of meds and knowing what to expect from this illness. I feel very fulfilled.