Considering my body is falling apart, this week has been a much needed rest from the dangers I subject myself to; whether it is hurling my body down the course against 3 other guys or simply walking on a slick surface with no-traction boots. Whatever the reason, I always seem to end up back in physical therapy at the Aspen Club when I come home.

A great thing about being home is spending time with my girls, Riley and Annalisa. Last week I went to Denver for a “Care Conference” at Children’s Hospital regarding Annalisa’s tests. As I wrote before, she was just recovering from her 2^nd biopsy surgery. The doctors biopsied some lung tissue from the non-functioning part of her lungs and they were just starting to test it and get another look at what is going on in there. Although it was difficult for Annalisa to recover from the operation, her wounds are now healing well and she is back to her wonderful self again.

This is a synopsis of what I learned at the “Care Conference”:

As I said before, one of the concerns the doctors had in Annalisa’s case was that she had a surfactant gene mutation. She has been tested and re-tested with the leading researcher at Johns Hopkins University and has come up negative for any of the known genetic problems. Bonus!

The reason Annalisa has to be on oxygen 24/7 is because there is something in her lungs that the body is identifying as foreign and the body wants to snuff it out. So the body is sending lymphocytes (white blood cells) to deal with the problem. Annalisa’s airways have been, and are still clogged with these lymphocytes.

Since the airways are clogged we have to send more oxygen in to push through those lymphocytes and get O2 to the cells. Off oxygen Annalisa’s O2 saturation is around 70-80%. On oxygen she is at normal to above normal levels of about 95-99%. At 75% oxygen saturation, not only do you have brain problems, but the heart starts beating harder to get oxygen to the blood and you get right ventricular hypertrophy, which can be irreversible and certainly detrimental to the heart.

The problem the doctors have been facing from the start has been: Why is her immune system acting like this? Is it fighting infection? Does she have a hypersensitivity to something in her environment? For God’s sake, what is her body trying to fight???? If they figured this out, they might be able to help her. At the moment they believe her immune system could be malfunctioning. They think the T-cells (these are the generals in the white blood cell army) could be misguided. The body sees a lung infection, then it sees that the lymphocytes aren’t working, so the body sends more, but it doesn’t get rid of the stimulus so it keeps sending more. Just like any injury, the body tends to over do it when fighting infection. That’s why we ice when we hurt ourselves – to slow down the healing response. Nothing is slowing down the army of white blood cells to her lungs.

One of the results of this onslaught of white blood cells, and this is the scary part for us, is that she now has scarring in some of her airways. Some of her airways have scarred and have been shut down forever. This has been irreversible damage. She will never use those airways again.

This is a serious problem that requires immediate action. The doctors now have Annalisa on immune suppressing drugs, including prednisone steroids and another, more accurate drug – Cell Sept. And since she is on immune suppressing drugs, they also have her on antibiotics in case of any other infections she might acquire along the way. The poor thing is on so much medication it’s frightening.

Another problem she is facing now is that she has flattened out on the growth curve. She is expending so much energy to breathe because of her condition that she requires more than twice the caloric intake of a normal 2 1/2 year old. Considering she is 2 and has strong opinions on what she eats and, more importantly, what she does not eat, this is a problem. So she may also have to get a stomach tube so that we can feed her enough calories to keep growing, because growth is one of the most important things she needs to get out of this problem. If she stops growing her chances of healing are drastically reduced.

I have been blessed in my life for a number of reasons. I have never lost anyone near and dear to me except my grandparents who all led very full lives. The only person I can think of that I loved very much and she died young was Laura Flood (by the way Laura, if you’re up there with your wings listening in, we sure could use your help). The idea of losing Annalisa is a reality. We are doing everything humanly possible to make her better. It’s been the biggest challenge of my life and of Kate’s life to watch our daughter, who is so full of life, so beautiful, so loving, so vibrant, such a 2-year-old, lose the function of her lungs. I’m not one to cry, but you so much as say her name and I am apt to break down into tears. My cheeks are soaked with tears as I write this. This is actually therapeutic for me to write this stuff. I hope it’s not too heavy for you folks.

She is going to be fine. All we can do right now is pray. Pray that the doctors solve this problem. Pray that the medications are effective. Pray that she grows out of this and that her lungs function well enough to live a normal life. Thanks to everyone for your thoughts and prayers.

This entry was posted on Wednesday, February 20th, 2008 at 10:52 am and is filed under caseypuckett. You can follow any responses to this entry through the RSS 2.0 feed. You can leave a response, or trackback from your own site.