I cringe when I see a swan.

Almost two weeks ago I had another right heart catheterization. Other than some minor pain and a nasty rash breaking out all over my neck (probably from the chemical prep), I got through it fine without sedatives. And, if I have to find something good to say... I will admit, it was better than January's right heart catheterization.

In January, the Swan-Ganz catheter was left in my neck for several days. Now I cringe any time I see a swan, or even hear the word swan, despite it being named after the doctors that invented it. I've included a photo of that nasty device here. It is through a large yellow trumpet-like part that goes in the neck's jugular vein (and is not pictured) that several lumens, each with a different function, are threaded. The main yellow line you see, partly covered by a plastic sheath, is the line that ran from my neck, down the vein, through my heart, and up through my pulmonary artery. That is the line remained inside my heart for several days when I was in the MICU. All the rest of those tubes and such hung to the side of my neck.

The test showed that my pulmonary pressures were down a bit. This proved the medicines have helped to relax my heart. However, the following week I had additional pulmonary testing that revealed my breathing is getting worse. It's harder for me to breathe and my lungs are not doing their job getting enough oxygen into my blood. But, this is the expected course of the PVOD and was not a surprise.

What was a surprise, however, is that the doctors have run across a reason for additional testing. My heart sank. I had hoped we were done and I would be officially listed. They ran some more lab-work while I was there and I'm expecting to go back in a couple weeks for additional testing. I won't go into details now, but let's hope it's just a little hurdle.

What's this PVOD thing again?

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...