Tune Up

Yesterday was my son's first "Tune Up" for his diagnosed Cystic Fibrosis. For those of you unfamiliar with Cystic Fibrosis, the following is a quick summary of what I have found out.

1. It is a genetic disorder.

2. It is a double recessive.

3. It can shorten the life of the person.

4. 85% of all cases are enzyme dependent

5. In 1959 the average life expectancy was 6 months.

6. In 2008 it was 37.4.

7. It impacts not only the person, but all those around them.

So, the last 6 months have been a roller coaster for me. The diagnosis, and learning to deal with the problems that are entailed. My mothers death and the subsequent family squabbles that have come my way since that time.

We are very blessed in the fact that my son is not enzyme dependent at this time. My understanding is that he will most likely remain enzyme sufficient, don't you love the way doctors never commit, the rest of his life.

So today's lesson is all about the "Tune Up". A "Tune Up" is when the CF team preforms a bronchoscopy. What is a bronchoscopy you ask, well you can see here http://en.wikipedia.org/wiki/Bronchoscopy . While Wikipedia is not a quotable source, it is pretty accurate.

After the surgery, we were informed that they failed to install a PICC line into my son. A PICC line helps in the delivery and sampling of the patient. In this case he is on IV antibiotics for the next 14 days, so it was kind of important. Since they did not schedule this correctly, he has to have a second procedure preformed on him today to have the PICC put into him. Yeah modern medicine.

In addition to his daily routine, which is quite a lot for a 11 year old who until March was considered totally healthy, we have added a nebulizer treatment twice a day, a 3rd and 4th vest treatment, vancomycin IV format, bactrim, and rifampin. Each a drug in pill format, two with very interesting side effects.

With vancomycin, my son reacts with "Red Man Syndrome". Really very funny, except how scary it can be to watch your child turn Indian Red, no slur intended, in about 2 minutes. It is accompanied by severe itching and fever of the skin. Solution was to push IV benadryl. Solves the reaction, but this "Syndrome" causes the vancomycin to be introduced over 2 hours instead of 20 minutes. Since he has to have it 4 times a day, he is now hooked up to the IV for 8 hours instead of 80 minutes. Not really practical to do outside of a hospital environment.

The other drug with a side effect is "rifampin". This one is just funny. My boy now pee's orange.

Well that is about it for yesterday. More about the past 6 months and the current going on's in my life soon.

Jeff