Wednesday, November 5, 2008

Pulmonary Hypertension

Thanks to my friend Pam at Rhett's Journey I found out that November is Pulmonary Hypertension Awareness Month. There are not many words, outside of cancer perhaps, that strike terror into the heart of this mother like pulmonary hypertension.

I describe PH to people as high blood pressure in the lungs. It's really a big deal because the high pressures in the lungs cause the heart to work harder to push the blood through, resulting in right heart failure. This can be deadly as the heart simply wears out.

Symptoms of PH are chest pain, coughing, shortness of breath, edema, fatigue and lightheadedness. OK, now imagine asking a baby if they have chest pain or feel fatigued! Ha! Ralph has a regular EKG, and also an echocardiogram which can approximate the pressures in his lungs. He has had a heart cath done twice. With a heart cath the doctors can directly measure the pressures in the right heart.

Ralph is on PH medications as well as oxygen around the clock. He doesn't mind wearing his nasal cannula most of the time. If I leave him in his jumper seat too long, though, he rips it off and starts chewing on it! Also, if his 50ft. of oxygen tubing gets hung up on something he will take it off and keep right on crawling. I suspect that he has learned that I cannot find him if he crawls away without the cannula on!

At night I monitor Ralph's blood oxygen levels with a pulse oximeter. This is a handy dandy piece of equipment, even though it doesn't work when he is awake. When Ralph was sick with rotavirus last Spring it was the oximeter that let me know he needed immediate medical attention. I could not get a signal on either of his feet that one morning. The meter worked on my finger, so I knew that it was working properly. I figured out quickly that his pulse was too weak for the meter to pick up. We raced to the doctor's office and then on to the hospital immediately. Just in time, too.

Ralph has been nearly completely healthy since that scare. And since his heart repair in June and an adjustment in his medication in July, his heart size is improving and his pressures are normalizing. We are on a long and winding road. Will he ever outgrow his PH? Children sometimes do, although it is considered a chronic and incurable condition for adults. Will he EVER get off oxygen? I sure do hope so. Probably not for a while.

When you are dealing with PH, Down Syndrome doesn't seem so scary. Sometimes it seems that the docs, at the hospital especially, are so hung up on Down Syndrome that they don't see him as a baby with PH. I get so worried about the PH that I sometimes forget about the DS!

I'm convinced that there are certain aspects of gene over-expression that come along with Down Syndrome that may aggravate his pulmonary hypertension and it drives me nuts. I don't have the knowledge to read and understand the research in these areas and I don't really have anyone who is all that interested in helping me.

Perhaps Ralph will live to see amazing advances in treatment! We briefly discussed a heart-lung transplant with the doctors in Kansas City last year, but the survival rate is poor and can be a case where the cure is worse than the disease. New drugs are being developed, but many take a terrible toll on the liver. I'm quite thankful for Sildenafil, AKA Viagra! It has done wonders for him. This drug was initially developed for high blood pressure but the researchers found that it had an interesting side effect - and the rest is history.