For those who don't know already my son Charlie was diagnosed with Cystic Fibrosis at 2 weeks old via a PK screen. CF is a lifelong, chronic, and progressive genetic disease that causes Charlie to produce copious amounts of thick sticky mucus (think peanut butter kinda sticky). This mucus wreaks havoc throughout his whole body but for right now we mainly focus on the lungs and digestive tract. The mucus gums up his pancreas so he can't release digestive enzymes and the mucus also makes a very hopsitable environment in his lungs for all sorts of nasty bacteria. The following routine is our attempt to keep the organ damage and lung infections at bay as best we can.
Charlie's Current Cystic Fibrosis Medications and Treatments
Gene Types: Delta F508 (∆F508) homozygous
Diagnosis via PK Screen: September 9, 2010 (2 weeks old), confirmed via sweat test September 13, 2010.
Age: 2 years and 11 months
Weight: 37lbs (>95th percentile)
Height: 40 inches (>95th percentile)
Weight to Length Ratio: 67th percentile!
Bacteria Cultured: staph aureus, pseudomonas aeruginoas
I want to take a quick second to point out that Charlie gained 3 POUNDS in only 2.5 months! This has made his weigh/height percentile SUPER awesome, and we are so thrilled. Also at his appointment his lungs sounded clear as glass :) So we are double thrilled and feel very fortunate that we have such a "healthy" little boy. Our doctors noted that a good chunk of this is because we use a nanny and the other reasons are because we're vigilant with treatment and meds and the CF center is also vigiliant with antibiotics and aggressive therapy. The main reason for the weight gain is because we FINALLY got his Pediasure 1.5 (50% more calories than regular Pediasure) approved by our insurance and it's amazing how quickly we can see a shift in his BMI.
Robb usually starts out the morning with a high calorie breakfast including Pediasure 1.5, cheese sticks, pudding, etc. Charlie is given 3x Creon 1200 pills with applesauce before he's allowed to eat or drink anything with protein or fat (so pretty much anything except water and gatorade). Normally the mucus in his body gums up his pancreas, which then can't release digestive enzymes in order to digest his food. We give him these enzymes in pill form but they're still only partially effective, and this is why he requires such a high calorie diet filled with lots of fatty and salty foods. He is also given 2mL of Pepcid to help him with any reflux issues. After breakfast he does his first breathing treatment of the day. Each treatment consists of the following routine
1. Chest Physiotherapy via Hill-Rom The Vest: this is an awesome invention. It is a vest that shakes Charlie around kind of like a paint can in an effort to loosen some of the mucus in his lungs. This takes 20 minutes so steps #2-4 are done while the Vest is shaking.
Here's a cute video of Charlie doing his Vest treatment and pointing out a flashlight :P
2. Albuterol adminstered via an inhaler + spacer: to help open up and relax the airways. This takes about 1 minute, very quick! (PS yes I understand I'm a horrible mother letting him hold a meat thermometer during a treatment. In my defense he was pretending it was a wrench and fixing his toy cars with it while he was doing his Vest and it kept him distracted. Also, he still has both his eye balls. So sue me)
3. Pulmozyme: a DNAse that 'chops' up the bacterial DNA in his lungs, adminstered via nebulizer, with nebulizer parts that are dedicated to only this medication. This takes about 7-10 minutes to nebulize.
4. Pulmicort: a steroid that reduces any inflammation, adminstered via nebulizer. This takes about 7-10 minutes to nebulize.
5. TOBI: This must be done at the very end of the treatment, after the Vest is done. It is a very strong antibiotic to attempt to keep the Pseudomonas infection in his lungs at bay. The TOBI is one month on and one month off, so as to not create a resistance. This medication takes FOREVER to nebulize, usually around 30 minutes, which is a lot to ask considering he's already spent 30 minutes doing all the other stuff! TOBI months can get pretty rough around here.
During treatments Charlie usually sits calmly and watches movies with his blankie and doggie by his side (and of course Mommy, Daddy, or his nanny are there too). Sometimes he'll decide "no treatment no treatment!" and won't put on his vest, but usually once it's on he's a good sport. The vest makes his voice sound silly so it also happens to be pretty cute. Occassionally he'll decide he doesn't want to hold his nebulizer anymore so we have to keep on top of him to hold it up to his face and not tip it over.
Now it's about an hour and a half (on a good day) after waking up and we can finally leave the house! We do all sorts of fun stuff with Charlie: he has memberships to the Mystic Seaport, Mystic Aquarium, Niantic Children's Museum, Stay and Play Indoor Playground, and the Dinosaur Park. He also loves to swim in our pool and go to his grandparent's beach house and play on the beach. Charlie doesn't go to daycare because we are worried about him being sick constantly, so he has a nanny instead. We definitely don't want him to miss out on having fun with other kids, though, so he has lots of memberships to all the fun places nearby.
Lunch time is around 1230 and Charlie needs 3 more Creon 1200 enzymes before he can eat anything. Typically lunch can be McDonalds (yuck!), french fries, hot dogs, chicken nuggets, more cheese sticks, pepperoni, etc with whole milk. We shoot for about 700 calories per meal which is a very tall order (at least 2000 calories per day). He won't eat bread, fruit, vegetables, shakes or ice cream. It's mostly just meat and cheese...if only he was on Atkins we'd be golden ;)
Around 1:00 he "naps", although this is not much of a nap for Robb and I. He usually sits up there quietly playing and talking to himself for an hour or so. It's technically more 'quiet time' than 'nap time'.
Once he's done there's more playing outside and crashing of toy cars. Around 4:00 we usually gear up for another breathing treatment. This entails the same as above, except there is no Pulmozyme because it's only given once per day.
Around 5:00 is bath time, and 6:00 is when Robb and I get home from work during the week and start cooking dinner. Dinner is more of the same: 3 Creon 1200 enzymes plus mac + cheese, fries, cheese, pepperoni, meatballs, milk, M&Ms, etc and whole milk. It's very important Charlie keeps his weight up because when he gets sick his weight drops very quickly and a higher BMI is tied to better lung function.
After dinner is med time, which Charlie hates 50% of the time and doesn't even notice the other 50% of the time. When he DOES care, it's awful. He spits medicine out, lets it dribble down his chin, and Robb and I need to hold down his arms and pretty much force it into his mouth. Not fun! As you can see from the video (from this past winter) above, those stupid orange vitamins stain EVERYTHING in a matter of seconds-- and that was with him taking them WILLINGLY. Also, in case you're wondering why I didn't immediately come to the rescue and clean up my sweet little man-- he had more orange stuff and 4 other meds to suffer through, meaning cleaning at the end is a heck of a lot easier than in between each one! haha!
Meds are as follows:
2mL Pepcid (reflux med)
2mL AquaADEK vitamins (fat soluble special CF kid vitamins)
4mL Azithromycin (antibiotic to complement the TOBI, MWF Only)
2.5mL Vit-D-Sol (extra vitamin D supplement)
6mL Periactin (appetite stimulant)
And then he's off to bed :) Pretty routine for us right now. This makes his total tally of medications at 10 per day plus using his Vest, and he's administered medication 16X per day. What a trooper he is! If you're curious the cost of these medications I'm going to do a post shortly detailing how much it costs- but to give you a rough idea there are $13,240 of medications in our refrigerator right now. Seriously. (PS please dont rob us)
Notable changes in Charlie's CF over the past year
1. Culturing Pseudomonas: this is a very nasty bug that is really difficult to eradicate. We are managing it by using TOBI but haven't been able to get rid of it. If Charlie gets sick we run the risk of this bacteria growing out of control and causing extensive lung damage.
2. His first CT Scan: Charlie had his first CT Scan a few months ago and did GREAT! We went there planning on sedating him because it involves lying still for 1 minute, but we tried it the normal way first. He was scared to go on the conveyor belt but I rode on it to show him how it works and then he wanted a "ride" too and thought it was more fun. He didn't stay PERFECTLY still but he did really well. Our doctors saw a few areas of infection (thanks Pseudomonas) but nothing to be very worried about and were overall very pleased.
3. Our first trip to the ER
4. Charlie received his Vest machine last August and has since logged 271.7 hours on it! That's 815.1 breathing treatments in one year (each treatment is 20 minutes). This boils down further to 2.23 treatments per day, every day, for a year straight - yay for compliance! The reason it's slightly above 2 treatments per day is that whenever Charlie has a cough or slight sickness we do double the treatments (4xday), so that skews the final number. But still amazing when you think that Charlie spent 272 hours chained to his Vest machine in the last year!
5. Charlie still hasn't had to be admitted for a hospital stay! YAY!
So that's Charlie's routine-- I wouldn't wish it on anybody, it sure is a lot of work! But luckily Charlie is one awesome kiddo so he puts up with us shoving food into his mouth and nebulizers in his face pretty valiantly ;)