Toe Health Update - 2020 year end

We had an in-person appointment at our CF clinic last week and I thought it would be a good time to share a health update. Since March, our trips to the children's hospital have been reduced - we have had Telehealth appointments for quarterly check ins. Toe has recently had an ear/nose/throat appointment, a CF clinic check in, and a meeting with a new gastroenterologist (cystic fibrosis affects so much more than the lungs!). Overall, his health has been fantastic this year but with CF, there are always a few bumps.

Toe started Trikafta, the new triple combination therapy that affects CF at the genetic level last February, right after he turned 12 (the drug has been approved for ages 12 and over). Even though Toe has been on a double combination therapy for a few years now, we have really noticed a difference with this new medication.  

First, and most importantly, although Toe has gotten sick this year, he has not had lung complications. Normally, if Toe catches a cold or gets sick in any way, he starts getting a junky cough that just gets worse after his other symptoms dissipate. We often have to increase his twice-daily breathing treatments to three or four times a day to keep the mucus moving. Most of the winter is spent doing three breathing treatments a day as the cough comes and goes. To eradicate the cough often requires antibiotics. This year, Toe got sick a few times (including a sinus infection and tonsillitis), but the cough never came. We have gone almost a whole year without having to add extra treatments. I can not tell you what a time and energy saver that has been!!

On this new medication, Toe is not having to worry about eating all high fat, high salt, high protein foods any more, which means his body is doing a better job of absorbing fat. This is tempered a little by his huge growth spurt this year. He just polished off a gallon of egg nog in a week and has not gained any weight - ah, puberty! In general, Toe is thrilled that we are giving him larger portions of fruits and vegetables. It will always be a balancing act between getting Toe enough calories and fat and keeping him from having coronary disease later in life (a very new problem for people with CF who never used to live long enough to have coronary disease), but this has also been easier this year.

 So, here's the run-down from the doctor visits:

Ear/Nose/Throat: The nasal polyps he had grown a year ago that were concerning are GONE. His ears and nose look great and the ENT said that she doesn't need to see him again unless he needs something.

CF check in: Toe is now 5'3" and 105 pounds! He has grown FIVE inches this year. His PFTs (lung function test) were the highest ever. His lungs have generally been in pretty good shape, but this was amazing. PFTs are a variable number compared to other kids his height, weight, and age. They are given in percentage form, so mostly you look at the individual's PFTs over time to see whether they increase or decrease. For some people, PFTs of 80 percent are baseline. Toe's baseline is around 110-112 percent. He has been as low as 80. Usually if your PFTs drop more than 10 points suddenly, it requires a hospital admission for IV antibiotics to clear infection from the lungs. Last week, Toe's PFTs were around 122! We were absolutely shocked.

Overall, Toe's health and growth are fantastic and because we have stayed home he has caught fewer bugs this year. 

PFTs (pulmonary function test) measure the volume of air he can inhale and how fast he can expel that air

Gastrointerology: This is the area that caught me off guard this time. His bilirubin count is slightly elevated and they are trying to figure out what is happening. High bilirubin levels often cause jaundice. Toe had jaundice as a preemie but it went away once we got him on pancreatic enzymes as part of his CF diagnosis. Tricafta has a possible side effect of liver damage, so that is something they are very careful about. The doctor told me that his other liver levels were normal, so they don't think he is suffering from liver damage. This visit they did extra blood work to check bilirubin counts again and to test Toe for Gilbert's Syndrome - a rare genetic disorder that mostly only affects the bilirubin count. They also did a liver ultrasound to see if anything is unusual there. Now we wait for results and go from there. I have learned not to worry until we get results. It is a waste of my energy when I have so many other things I could worry about ;).

There were a few other minor things - Toe has been having acid reflux since we took him off Omeprazole (as recommended by the doctor), so he will be going back on that again. We discussed Toe's possible return to school in person (our district has been remote all fall but is looking to return in February). As always, we met with the pulmonologist, the dietician, the respiratory therapist, and the social worker. It was a three hour appointment that involved a blood draw and an ultrasound, but if Toe's health continues we can have our next three month appointment virtually.