For those of you wondering about Toe's weight, we were very pleased at the last clinic visit. As of early March, Toe reached the 50th percentile and if we can maintain this height/weight ratio, there will be no more discussion of a G-tube. But we must maintain a high fat diet and lots of food...
Some people ask me if Toe is "ok" now. Yes and no. He is mostly healthy. Very healthy for a kid with cystic fibrosis. But he will have CF his entire life. Keeping Toe ok means a lot of maintenance.
We recently started a board to manage Toe's daily medicines. Once I wrote it all down, it didn't seem like that much. A friend of mine saw it and was a little appalled - she said it looked like a lot. I guess it's all in the perspective.
This is what we do to keep this four year old healthy:
Every day he gets:
Oral Meds:
Vitamins
CF specific vitamins (high doses of the ones that are difficult to absorb)
Two doses of antacid medicine (Prevacid) per day (must be taken 30 minutes before meals)
Three capsules of Enzymes EVERY Meal
Inhaled Meds:
Albuterol (before inhaled meds or clapping)
Pulmozyme 1ce a day
Hypertonic Saline 2ce a day
Percussive Therapy (also called "clapping" - we call it "Patapon" or "Whack-a-Toe") 2ce a day
An inhaled med plus percussive therapy can take anywhere from 40 minutes to an hour and a half. Right now he is doing 2 treatments (1 saline then pulmozyme, the other just saline).
That is the minimum he will probably ever have have.
If he gets a flare up of the existing bacteria in his lungs, he goes on oral antibiotics (as opposed to inhaled antibiotics or IV antibiotics, which will come later in his life) and we increase the number of breathing treatments during the time his cough is worse (Example, he has just been on a course of 2 weeks of antibiotics administered 3 times a day and 3 breathing treatments per day).
Also, obsessive hand washing and complete avoidance of people who are sick. And clinic visits every three months for a sputum culture and general asssessment.
CF is progressive, so as Toe gets older, we just keep adding treatments and meds to the existing framework. Up next: a PEP breathing apparatus. He's playing with it now and will use it in earnest when he gets a little older.
The good news is that there are more medicines on the market now than ever before to help manage CF and they are working on developing some that address CF at the gene level. The gene therapy is probably at least 10 years away for Toe's mutation and when it comes, it will not correct any damage already done to his body, so he will still have to take many of the meds. But, it is still SUCH an improvement even from 10 years ago. EVERY DAY we are grateful for these medications and the medical support we receive from the CF clinic at OHSU.
I think the hardest thing is that the treatments just take a lot of time.
Ok, and it's hard watching Toe when he is sick. We had the flu the last two weeks and he was really sick. We couldn't give him cough supressants or nasal decongestants because that would dry out his already thick mucus, making his mucus even more sticky, gummy, and sure to hold bad bacteria. So we just had to do more breathing treatments (which thin the mucus in his lungs) and use a ton of tissues and encourage him to cough. And cough. And cough.
This guy is a fighter. He works so hard to be healthy and is so positive. He hates having this disease. He hates doing the treatments. But so far, he has been incredibly compliant and does what needs to be done because he knows he has to.
And we are so lucky to have him EVERY SINGLE DAY. And in so many ways, he is such a normal four year old. He has lots of energy. He has good days and bad. He loves to laugh and play and has a wicked sense of humor. He is beginning to understand complex issues at an astounding rate.We are so used to Toe's routine that to us, he IS perfectly normal.
