Huff & Puff

So before I tell "the rest of the story" I wanted to explain the picture. Even in the midst of so much bad news, we all try to continue to find moments of joy and happiness. At one point, Nick was just sitting quietly in his bed (even as his poor lungs were working overtime to breathe) and Kate asked him how he was and Nick's response was, "I'm happy."
That is our Nick, he just keeps taking it all in and still is able to find moments of peace. And that moment was happy...Nick, Kate, and I were all there in his room just hanging out. It was such a relief to know that Nick was not going to have the lung biopsy and we were all going to enjoy the day. Nick & Sara & I have all been wanting to get another cat (since we lost Mickey) and so I told Nick I thought we should ask the doctor when we could bring a kitten home. Kate asked Nick what he would name his cat and he said Huffenpuff. So I said in that case we should get two kittens and name one Huff and name one Puff!! It was great fun to talk about something other than medical stuff. Kate and Nick watched Slumdog Millionaire and then Nick finally fell off to sleep.

Now for the tough stuff....based on the results of the pulmonary function tests, the doctors believe that Nick has lung graft vs. host disease. This can also be called bronchiole obliterans.
The small bronchioles in the lungs start to become fibrotic and narrow, which makes it difficult for the air to get out of Nick's lungs. It also decreases the surface area where oxygen exchange can take place. This causes hypoxia (lack of oxygen in the body) which basically translates into all the symptoms that Nick has been having dry cough, increased respiratory rate, increased work of breathing with exercise, and an oxygen requirement. I feel that this has been going on since November when Nick started with a dry cough that lasted for eight weeks! It makes me so angry and sad to think that I couldn't make people pay attention enough to actually do that one thing that might have led to an earlier diagnosis. Nick's current lung function studies show that his lung capacity has dropped by about 75% since his pre-BMT testing and his capacity to exchanged that gasses has dropped by about 80%! These are very bad numbers and there is no cure for this process. There are things the doctors want to try that might help and supposedly they do help in some people. But, as the doctor pointed out, this is a very difficult process to treat.

Nick will have to go back on the steroids at high doses for quite a long time. This will increase his infection risk and steroids have many other potential long term effects. In addition, he is possible going to have this thing called extra-corporeal photopharesis. This is where they will run Nick's blood through a machine (like a dialysis machine) to pull out the donor T-lymphocytes. Thoses WBC's will then be exposed to UV light which will render them useless.
The hope is that this puts a halt to the GVHD process in Nick's lungs. This ECP is relatively benign, but, not without risk, including infection. In addition, taking out these cells decreases Nick's leukemia fighting power, so while we hope to fix one problem, it may create another. :(
If he gets this treatment, he has to have it three times per week. Each time last 4-5 hours.

As the doctor was explaining all this, Nick told the doctor he had a magic wand!! His godson, Andrew gave him the wand. It lights up and strobes in multi-color luminescence! So Nick took it out and waved it over his lungs and tapped it on his head. I hope that thing works! That's my boy, so gracious, so brave, so strong to carry this burden for so long with such grace.

Nick is still on oxygen and working hard to breathe at times. His counts remain low and I am not sure how long he can keep breathing at these high rates. The fear is that he will tire and still might end up on a breathing machine. This would not be good because it would be very difficult to mechanically ventilate Nick's lungs in their current state. It is better that he stay off the breathing tube, because his body can much more efficiently manage his breathing. Lately, I have begun to worry when I leave for the night. I wonder if this will be the night I get a call saying Nick is having trouble. I don't want it to be like this...I just want my boy to feel good for just one hour of just one day. I can't remember the last time he actually felt good. We all just want to get Nick home. Please keep the prayers and good wishes coming Nick's way. They are much needed and appreciated.