About a week later, I returned to the Methodist Hospital after passing out in the small town I lived in. I woke up from my very first Right Heart Catheterization (RHC) and found myself attached by catheter, tubing and other medical device, to a pump infusing medication into my heart 24 hours a day. Flolan, the med I had to mix every single day, and keep on ice while attached to the pump, came with some heavy heavy side effects. Nausea, vomiting and diarrhea made me loose nearly a hundred pounds in a matter of months. Pain in the jaw didn't help, that I would get with every first bite during each meal, or snacking, of the day. If I salivated at all during the day, I'd get jaw pain. If I thought of food, got a craving, became at all thirsty or hungry, this jaw pain would hit. it was so bad thata most of the time, I'd skip meals to avoid it. Exhaustion, a symptom of Pulmonary Hypertension, was even worse for me while on Flolan. So was leg pain and fatigue, which I still to this day get, when walking for any length of time. There were many more side effects, but these were the some of the worst.
Four years later, Tracleer came on the market, an oral pill taken twice a day. Taking a pill a day took place of mixing powdered medications vials, with sterile diluent, a fluid used to make the powdered Flolan into a liquid. Unlike Flolan, Tracleer had very few side effects, dizziness and the possibility of elevated liver enzymes, were the worst, and were easily tolerated by me. Monthly blood draws helps to show if the liver enzymes became elevated and if they did, they would either lower the dose, or discontinue the use of Tracleer. I was on Tracleer for 11 years.
On my eleventh year, I was diagnosed with Pulmonary Venous Hypertension, (PVH) also called Diastolic Dysfunction, HFPEF, Heart Failure with Preserved Ejection Fraction.and a few other names. I was transitioned off of Tracleer and put onto Sildenafil, the generic form of Viagra. Tracleer, like most other PAH medications, negatively affects PVH.
I have learned a lot through my eighteen years since diagnosis and the two and a half years prior , where I was constantly being misdiagnosed with things such as depression, anxiety, and my fav "We dont know why people pass out, they just do".
a few things that I've learned off the top of my head:
1. You must advocate for yourself. and if you can't (Like I cannot, have never been able to), take someone with you to doctor's appointments and hospital stays or ER visits to advocate for you.
2. Always keep a list of doctors - with phone numbers and addresses - in your wallet or purse, or cell phone. Also add all your medications with dosages, who prescribed them for you, and what they are used for. I keep a written document on my computer to print out when I go see the docs, and I keep a list on my cell phone.
3. Use the ICE - In Case of Emergency - on your phone's contacts. I have my sister, her husband, and myself as ICE. I have myself because in the notes section of the contacts, Be sure, if you do use ICE, the people you chose know your condition(s) and medication(s) and any allergies you might have....
4. Know the meds you are on. Know any side effects. Know the dosages and what it's for. If you have multiple conditions, (CoMorbidity), it's even more important to know what they are for. If you are on IV or subq meds, know your dosage and when you increase the rate.
5. Be prepared for any emergency. the Pulmonary Hypertension Association has lots of ideas to help you be prepared. Contact your local fire department, emergency room, electric company, to notify them of your condition. Fire department to let them know what PH is, in case they have never heard of it, to let them know to NEVER turn off your pump, if you are on iv therapy,and what to do if your catheter line breaks. Emergency Rooms also need to be contacted. PH is such a rare disease that a lot of small town hospitals dont know about it. I had to educate the head nurse on what to do, how to mix my Flolan, and phone numbers to call if she needed more help. The Electric Company should be called if your medications need to be kept cold, if you are on oxygen and use a concentration, in case of loss of power due to storm or anything, they need to put you on a list of people who need their power turned on as soon as possible. Sometimes they will advice you to go to the hospital and use their fridges for meds, and use their o2. Mine did this, but that was only because we expected electricity to be out for a few days due to a hurricane that became a tropical depression. Call them too if a storm is expected. Sometimes they are so swamped they don't always check the lists.
6. Make sure to have all your docs communicating with each other. Sometimes, it's as easy as a letter of what happened at your latest appointments, or results from blood work, echo, or RHC. If at all possible, keep all your doctors in one hospital system. It makes things so much easier!
7. Get as much support as you can. Any Chronic Illness will take so much out of you and can cause depression. With a big support network, of people you can rely on, you know you will always have a meal if you don't have enough energy to cook, or a way to get to appointments if you cannot drive yourself. But more importantly, in my opinion, is emotional support. Online, people you can talk to on the phone, clergy, family, can help so much!! Lean heavily on your beliefs. Pray often. Believe and have faith that a cure will come! and when you can't pray or believe, call someone to help you!! or jump on line.
There's a lot more I'm sure, but just can't think of any at this precise moment.
There is one more thing I have to say. I know I pretty much told you all the bad things about Flolan, but if a doctor were to tell me I needed to get back on it, I'd do it in a heart beat! Flolan saved my life all those years ago. Flolan gave me back a quality of life I never thought I would have. And after being given two years to live, Flolan gave me 4 years and if not for Flolan, I doubt I would be here today!!
Thank you to much loved ones my friends and PHriends. My family and PHamily. You guys are so amazing! I doubt I would be here today if not for you guys too!!!! I love you more than you will ever know!!!!
