A lot of people have been asking specific questions about PVOD, or Pulmonary Veno Occlusive Disease. So, I'm going to do my best at explaining my version of what happens with PVOD.
PVOD is a rare condition that causes Pulmonary Hypertension and symptoms can include dyspnea, dizziness (and fainting), fatigue, edema, and a dry cough. In PVOD the small pulmonary veins in the lungs are affected. Pulmonary Hypertension should not to be confused with Hypertension, or High Blood Pressure. They are two different monsters and are very different.
In PVOD, I imagine the lungs as a tree with big branches and small branches coming off the big branch... and even smaller ones coming out from the small branches. Then I imagine someone snapping off those smaller branches until there is nothing left but the bigger branches. Basically, that has happened to my lungs. PVOD has destroyed all those small branches. And unfortunately, those branches are not expected to regenerate.
No one knows why a person gets PVOD. There are so few of us with PVOD they cannot make any connections. Researchers have a general idea of what we've been exposed to and there are suggestions as to what it could be linked to, but the statistics are just not there. In fact, if I've done my math correctly, a total of 32-63 people are diagnosed with PVOD in the United States each year. Compare that to over 232,000 cases of breast cancer diagnosed each year in the United States. Chances of being diagnosed with PVOD any given year is approximately 1 in six-million. I have a greater risk of being struck by lightning: 1 in 700,000, in any given year, in the United States.
I wonder if I should I buy a lottery ticket.
Anyway, there is no cure for PVOD. It is somewhat similar to stage 4 cancer. I know it's going to take me down, but I just don't know exactly when. And, at this point, treatment options are extremely limited.
A double-lung transplant is an option for some. But, it's not a cure. Double-lung transplants are risky and the recipient has about a 50% chance of surviving into the 5th year on the new lungs. As doctors explain, when a person has a double-lung transplant, they give up one problem for another.
The current treatment for PVOD is to use a variety of medications that dilate the blood vessels and target the symptoms of the PVOD. Other medications are prescribed to help counter pain and side effects of the drugs. It's a vicious cycle. This treatment can include the use of oxygen and additional heart medications when the heart is exhibiting signs of failure. None of these medications cure PVOD. Medication may keep a patient stable or make them a little stronger. But, medications can also do nothing, and in some cases, make things worse.
There is mention of stem cell research eventually providing treatment for Pulmonary Hypertension. Researchers are hoping to find something that will regress the disease. And, perhaps they will find something eventually but it may take many years to research and test, and then gain approval for such treatment. More than likely it won't happen in my lifetime.
I'm thinking a lottery ticket is sounding pretty promising after all. Perhaps it would increase the odds of being able to pay off my medical bills...
My son, Austin, died at age 20 one year after being diagnosed with PVOD at Duke Medical Center in Durham, NC. 10/1/2012 is the day PVOD took my child from me on Earth. Austin is in heaven at rest from the most baffling time in my life. I pray Austin gave the doctors at Duke Hospital some vital information to help others.
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