June 2011 Clinic Visits

Brett has 5 visits in the last 5 weeks to the UNC Children's Speciality Clinic! The good news is that all of these visits were scheduled. We seem to be turning the corner, with 6 months now without a hospitalization, to a somewhat long run of wellness.

Brett showing the stickers he got at Infusion

Our clinic visits still average once  a week, but it's easier to deal with when they are planned "maintenance" kinds of visits and not emergency "illness" types of visits. We think this is mainly due to the fact that Brett does not have a central line in his body at this time. And it looks as if the next time he requires one we ought to be able to get away with just having a PICC line now that he's bigger and he's had several successes with PICC lines under his belt now. The main advtantage to the PICC line is that you only have to be sedated to place it. It can stay in place up to around 8 weeks, then it can be pulled out by a nurse; it does not require surgery to remove.

Brett's June 3, 2011 visit was for his IVIG (intravenous immunologlobulin) infusion. It was a little rougher than usual since the first IV did not work so he had to be messed with a lot more and poked twice. His June 7 visit was to see his immunologist, Dr. Michelle Hernandez. On June 9 he had his second set of infant PFTs, June 22 he saw his GI doc, Dr. Katherine Freeman, and July 1 he had another IVIG infusion. I'll break this information into two posts.

June 7 with Dr. Hernandez - she is the doctor that monitors the values we see in his immune function blood tests. I actually get to visit her in the Raleigh location at Rex! I'm always excited for this possibility since it's a good deal closer with much easier parking and walking in. We are still monitoring his Natural Killer cell (NK cell) function, which has been the main focus on whether or not he has an acquired autoimmune problem or a true autoimmune deficit. There are 2 values she monitors, the natural killer cell count and the NK cell function.

The strawberry band-aid that is on his hand was where we had to put the IV this time

I guess it will just take time to see, but she did say she if he had a true autoimmune deficiency, she would expect to see his function values stay down. It appears that they go up and down. (I think I'm saying this right, but don't hold me to it.) It could be that his immune system just used up all the cells that he was born with since he was so, so sick for so, so long. It may take awhile for his body to build them back up, which is why we keep supplementing his immune system with the IVIG every month. It seems to be working, as even though he doesn't have a central line anymore and shouldn't have sepsis, you might expect that he would've had another lung infection by now. Whatever the case, we'll take it! And we'll just be glad for his relative wellness and enjoy somewhat of a more "normal" life where our family is under the same roof every night and we are able to go places, spend time at the pool, and enjoy a vacation or two. This week we're traveling to Atlanta to see our extended families, who all live in the area. It will be the first time our twins have visited Georgia and the first time many of my extended family will meet them!

Hooray for Internet Access

We just got our Internet access repaired today. It has been unreliable at best lately! Which is partly why it's been more difficult for me to post this month. So when I get a minute I'll put up some interesting stuff we've been up to recently. Until then here's a cute photo to hold you over!

Summertime 2011

June 2011 PFTs

Pulmonary Function Tests (PFTs) are routinely done on children and adults with lung disease, such as CF, or respiratory problems, such as asthma.  Infant PFTs are a special type of pulmonary function test that is only done in specialized centers.
In infants, lung function testing requires sedation so the child sleeps and is passive during the test. This allows the infant PFT equipment to perform testing since the child is not old enough to cooperate with PFTs. Infant PFTs can be performed very early in life until about 2 to 4 year of age depending on the equipment and size of the child.
Once children undergoing an infant PFT are asleep, they are placed in the equipment with a mask over their mouth and nose. They breath air with oxygen if needed through the mask. Lung volumes and flows are measured while the child breathes through the mask. An inflatable jacket is also placed around the child's chest to help them blow out air.
Since a baby or toddler cannot comprehend nor perform these tests the way older children and adults can, so the baby must be sedated and the exercises must be done for them. Click here to see Mark performing a set of PFTs last year.
These were Brett's second set of PFTs. The first were done in March of 2010 - (you can read about them here), when he was very, very ill and required oxygen. Not surprisingly, he had very low scores. The chief of the division actually told us they were among the worst she had ever seen in an infant. It was unsettling, to say the least. Back then he was put on an extremely long course of steroids, and his oxygen requirement diminished,
Fast forward until June 9, 2011, and here we are again to repeat the tests. It was a good time to test him again because it is always better to go under anesthesia or sedation when you are not sick. First Brett got some Versed to relax him while they inserted an IV line and began his sedation.
Then they put him in this little box :
then put a mask on his face with some putty so no air can escape. For part of the test they inflate his lungs and then put a bladder-type thing on his stomach to force the air out. They can measure the forced expiratory volume this way. It's usually called FEV1, meaning the volume you can blow out in one second. For babies this is measured in .5 seconds as opposed to 1 second. Again, there are various values measured, but for the CF folks, FEV1 is the one most talked about. After it was over, the doctor coached in learning to read this report:

I'm sure those of you who are farther along in your CF journey can decode this easily, and probably explain all this way better than I can, but I am still fairly illiterate when it comes to reading and understanding these values. The GREAT news is that Brett's FEV.5 went up drastically - 40%. This is an incredible improvement. One we are so very happy about and grateful for. But this blessing is tempered with the knowledge that these scores do not mean that his lung disease is not severe. It is. Worse than most CF kids his age. In fact, after last years, CT scan on his lungs, our doctor told me his lungs look like what you would expect a CF teenager's to look like. Today when we were discussing the findings, I got that sick, sinking feeling you get when you talk about your child having an inspid disease like cystic fibrosis, and the description on his report reads, "severe obstructive airway
Most of the time it's easy enough to just not think about it much - just go along with life, do what we can, be extremely disciplined about medicines and treatments. And persevere. And pray. Please join us in praying for our boy's continued wellness. And most of all for a cure, which still looks promising, and quite possibly could happen in his lifetime.