Thanks to all of you who so generously donated thus far - we're at $1,420 which is wonderful but still far from our goal of $5,000. I know these are hard times (gas, food, utilities) and if you can't give, I completely understand. But no amount is too small and if you could find your way to somehow donate, you'd help a cause that will directly help thousands of people who are struggling with this often horrible disease. Many researchers believe that by unlocking the mechanism behind PNH we will discover the trigger for leukemia and many other cancers and autoimmune disorders. Beyond that, you'd have the gratitude of this humble blogger.
Because PNH is so rare, few people know about it. Let me tell you: don't let my looks fool you. Most of the time I look perfectly well and there would be no reason for anyone to believe I wasn't. But there isn't a day that goes by that I'm not affected by my illness - chronically, my red cells are constantly hemolyzing (breaking down)...my bone marrow for some reason doesn't make normal cells- they lack a certain protein which protects them from the immune response. As a result, every time I'm exposed to germs, bacteria, viruses (which is pretty much all the time), I hemolyze. Hemolysis makes me shake; my heart palpitates and I sweat then become very cold. Sometimes this will give me a fever. As you may know, red cells are responsible for oxygen - so with a diminished supply of red cells, I'm usually short of breath, especially upon exertion. Fatigue is an issue for me, and if I'm somehow unable to get a full 8 hours of sleep at night I will hemolyze even more. I am highly susceptible to dehydration and must be vigilant in my fluid intake - if not, I most certainly will wind up in the ER. When my red cells die, the remnants bind to the available nitric oxide in my blood. Nitric oxide is responsible for smooth muscle contraction. And your body's biggest smooth muscle is the esophagus. I often can't swallow, and I assure you - the GERD that develops from this is maddening. The best way I can describe the feeling of your esophagus freezing up is one of a cold sword being stuck down your throat into your stomach. PNH also causes clotting, and I must remain on anticoagulant therapy to ensure I don't develop a deadly embolism. Despite this, tiny clots often form and pool in my hepatic veins (near my liver). The pain this causes is tremendous - there is no position which causes any relief and no way to prevent it. The only thing I can do when this happens is wait it out and take some morphine. Periodically I will suffer a bout of PNH "arthritis" in which my joints freeze up and everything hurts. For this I'll take prednisone and lots of ibuprofen. PNH also affects my white counts and platelets making me more susceptible to infection and viruses. My immune system takes a lot longer to fight things off (a cold for you that lasts a week will stay with me for three).
The only current cure for PNH is a bone marrow transplant. For me, without a sibling match, this is a very dangerous undertaking. A BMT is highly dramatic, involving the harshest and most aggressive chemotherapy, isolation for up to 100 days, and an almost certainty of coping with GVHD afterward. Many people die from BMT complications. With three children at home, I can't take that risk (unless of course my bone marrow begins to fail completely in which I'll have no choice). PNH often will progress to AML (leukemia), and this threat takes a great emotional toll on me. Learning to live knowing you're very high risk for an aggressive deadly cancer is hard enough without being already sick.
I currently attend infusion therapy biweekly. This involves spending most of the day at the hospital. The treatment is like chemo except it is mild and I will have to remain on it for life and that is mandatory. It also costs over $26,000 EACH TIME I GO. This creates a lot of stress ensuring that our health insurance is covering it and that I'm not stuck with any copayments. If I miss an infusion, I risk severe illness for which I would need to be hospitalized.
After treatment I spend a good 36 hours feeling nauseous and wiped out. Thankfully, I bounce back after a few days. The treatment is an immunosuppressant which renders me even more susceptible to illness and infection. I have to be very careful.
The fact that I overcame infertility and survived two pregnancies and have three healthy children is nothing short of a miracle. PNH caused me to suffer severe preeclampsia and HELLP. The details of the complications are too much to go into here, but suffice to say it was dramatic and frightening. My boys all came prematurely (full-term babies for PNH moms who are lucky enough to be deemed healthy enough to have children is a rarity) and all spent over a month in the NICU. In short, no woman (or babies) should ever have to be faced with this. All in all, my boys are my reason to live and my reason to have hope and my reason to keep going.
When I was first diagnosed with PNH I was given 5-10 years to live with the caveat that "anything can happen." Once the shock wore off and I learned to live with PNH, I decided to live DESPITE PNH. I wasn't going to give up but instead do all I could to live as normally as possible.
I'm 13 years in now. And though it's made me an emotionally tough person and all the challenges have changed me in many ways for the better, I'm tired. I deserve better than all this. Enough is enough. I want to be healthy. I want to feel well. And I want to live long enough to be there for my children. Hell, I want to be around for THEIR children. I know full well that I will likely die from PNH someday. I just want that someday to be decades from now - not years.
I know this is a shameless plea but like I've said before, if I don't help myself, who will? Please go here and consider donating. It has the power to affect my life in untold ways... even if the research does nothing more than merely create medicines to better control my symptoms or stave off the course of the disease, any incremental improvement is monumental to me.
You can help change my life.
Thank you!
Any fellow bloggers out there who throw up a link (or two!) to this post will get a happy little crafted treat from me. Swear!
PS Thank you GC and Michelle!
