A new organ recital

Last month, I had a right heart catheterization and the results were astoundingly good. At my diagnosis, my right heart pressure was 120/52. This time, it was 41/15, with a mean of 26, which is completely normal. You may not have pressures this good.

Today I went to the PH clinic for my quarterly visit. In addition, I went to the pulmonary lab for pulmonary function tests (PFTs), which I had not had since August of 2006, shortly after I began treatment.

The results were awesome.

First, I did the routine six minute walk and I walked 450 meters (1476 feet), Doctors are generally pleased at anything over three hundred meters. And I did it completely without supplemental oxygen, on room air, and I never de-saturated below 94%.

Compare this to one in November 2006, where I walked about 1400 feet, but desaturated to 82% on room air, and dropped below 88% (the baseline for supplemental oxygen) at only 1:27 minutes into the walk.

Now, this is very good. However, without the oxygen, my chest hurt like a sumbich. The respiratory tech who did my tests, my pal Patty (she works in the gym where I go two days a week), kindly turned on the oxygen at the end of the walk so that I could douse the chest pain. And when I bent over to pick something up, that familiar pain of my strained pulmonary artery reminded me of why I was there. I tend to think that when my test numbers are good that I'm completely out of the woods. My body kindly explains otherwise.

Other tests were performed as well. The Forced Vital Capacity (FVC) is the volume of air which can be forcibly and maximally exhaled out of the lungs until no more can be expired. FVC is usually expressed in a percentage of an expected outcome based on your age, size, etc. This tells whether your disease is restrictive (PH) or obstructive (COPD). My percentage in 2006 was 75. Today it was 96, which is COMPLETELY NORMAL!

The FEV1 (Forced Expiratory Volume in One Second) is what it sounds like. Two years ago it was 70%. Today it was 91%, again completely normal.

The Lung Volume actually are just what they sound like, how much air you can take in. The results of the test (TLC) are expressed as a percentage of a predicted value, based on age, size, etc. Two years ago, mine was 66%, today they were 83%.

The only thing that didn't make a dramatic improvement, unfortunately, is the DLCO, the measure of how well you pass gas (no cracks) through the alveoli to the capillaries. This measure is the thing that makes them believe that my PH may be related to scleroderma. Two years ago the DLCO is 42 (which was actually up from 32 at my diagnosis in January of that year), and my DLCO now is 46. Better, but not like the others.

So, what does this all mean?

In three months, the doctor wants me to come back and do another set of PFTs and a CAT scan, to make sure that the PFTs hold true, and the CAT scan to determine if I have any lung scarring from the scleroderma. (Also the CAT will check that pesky pericardial effusion that I've been watching since its dramatic debut).

If the numbers hold good in August, we will then discuss reducing some of my medications.

This is the first time in over two years that I feel like I have any breathing room.

I actually saw two docs today, one who is part of an exchange program with the National Institutes of Health (NIH), the other is Dr. Ahmad, a partner of Dr. Nathan, my doc of record. The doc from NIH said that looking at my heart cath results from last month that my improvement was "unprecedented." He actually said that word! He said that people don't recover from PH this way.

The reason they want to wait for the CAT scan is that scleroderma can greatly complicate PH symptoms and treatment, and they want to make sure that I don't have any signs of connective tissue disease in my lungs before they take their foot off the medication accellerator. And I'm fine with that.

I do feel particularly lucky today. Perhaps I will buy a lotto ticket.