If you're new to this blog here's a breakdown of Cystic Fibrosis. CF is an inherited genetic disease (Robb and I are both carriers but we didn't know until Charlie was diagnosed at 2 weeks old) that causes misfolding of a protein that functions as a sodium transporter. This results is super stick thicky mucus that clogs up CF patients' digestive and respiratory systems. In the lungs, this means bacteria has a nice gooey place to thrive so it can lead to constant lung infections not to mention that all the mucus makes it difficult to breath. In the digestive system it means that digestive enzymes cannot be released and food can't be digested (which is why many CF patients are very thin). It affects other parts of the body (reproductive system, etc) as well but it's not really applicable to this posting.
So Charlie's CF care can be broken up into two main parts. First part is Respiratory Care: his nebulizer, puffer, and physical therapy treatments. The second part is Digestive Health: high fat/high salt diet, weight gain, reflux issues, appetite stimulants, etc. And then I suppose you could count his "Sick Plan" as the third part of his care too.
Respiratory Care:
Here's a days supply of cleanly washed nebulizer and puffer accessories. These must be handwashed prior to each use and air dried on a papertowel (to prevent contamination since it's a direct route to Charlie's lungs). They must also be sanitized every other day by using a dilute vinegar solution or boiling for 10 minutes.
Here is Charlie's puffer and spacer (the long tube). He takes 3 puffs of albuterol before doing the nebulizer.
We do his puffs for him with Charlie in our lap but he loves to play with the spacer anyways and put it over his face (he knows what's up!)
Here's the nebulizer part all set up. We give him one vial of a medecine called Pulmicort in the nebulizer cup, which is an inhaled steroid.
Here's a very old photo of me and Charlie doing a neb treatment. If you've never seen a nebulizer before it's virtually identical to a fish pump, at least I think so haha! It's a pump that attaches to a tube which then attaches to the cup portion, which is filled with medecine. The pump aerosolizes the medecine in the cup which then goes through the face mask- which we hold over Charlie's face.
Charlie has learned a few defensive maneuvers for when he doesn't feel like doing PT (although he is a good sport 80% of the time, providing there's a Curious George episode on the TV). He'll shake his head back and forth so fast I can't keep the mask over his face, try and slide down my lap, twist out of my grasp....but I've found some creative ways to hold him in my lap (which involve one arm holding the mask on his face, another arm holding him in my lap, both legs curled around his legs to keep him from pushing away, and my chin holding his head in place...haha..)
So those 3 parts (the puffer, the nebulizer, and the PT) equal up to one "treatment" as we call it, which takes about 30 minutes. We do a treatment each and every morning and another treatment each and every night. When Charlie is sick we do 4 treatments per day, typically for a week or so depending on how sick he is, and it's pretty miserable for all parties involved!
Digestive Health:
Charlie's weight gain has pretty consistently been below where he's needed to be except for these last 2 months! I'm happy to report that Charlie is in the 50th percentile for length to weight ratio, which means statistically he will have better lung function as he gets older and will be able to recover from being sick more easily. YAY!
Here is one of his recent growth charts. If you follow the curve along the bottom you'll see his weight/length ratio was pretty dismal up until very recently. If you look at the top curve (length percentile) it'll give you a partial reason as to why-- this kid is so tall that it shifts the weight/length ratio down.
So what did we do to get him up to the 50th percentile? First of all, you might remember that Charlie can't digest his food on his own (darn mucus gunks up his digestive system) so we give him digestive enzymes before he can eat anything (and i mean ANYTHING! Not even a cracker without the enzymes).
He takes 3 capsules before snacks and 4 capsules before a big meal. We break open the capsules and mix them in some applesauce right before the meal. Once he takes the enzymes he has a 30 minute window to eat before they wear off.
Charlie also has reflux issues so he's one a very high dose of Prilosec. He gets 1.2mL (8mg/mL) each morning and another dose at night.
One of the biggest factors in upping Charlie's weight gain has been a drug called Periactin. Periactin was made as an antihistamine but it was discovered that it also increased appetites in patients, so we use it as an appetite stimulant and it works very well! We give him 5mL every evening, 3 weeks on and 1 week off (we use a calendar to remember because it can get confusing with all these different meds!)
Charlie also needs special vitamins because his digestive system isn't very good at providing his body with the nutrients he needs. He gets 2mL of vitamins every evening. We finally found a brand that doesn't stain as badly as the Source CF vitamins, aren't bright orange, and actually taste better too- yahoo!
We also add in extra feedings aside from Charlie's meals with Pediasure. He was prescribed PediaSure 1.5 (which has 50% more calories than normal PediaSure) but our insurance wouldn't cover it and it was $400/month otherwise. So we've been making do with regular PediaSure. Good news however: I appealed the denial of coverage and recently found out we've been approved for a YEARS supply of PediaSure 1.5! SO exciting! It hasn't come in the mail yet from our pharmacy (we use a CF Services mail order pharmacy) but I'm expecting a big crate any day now :)
Aside from these meds Charlie also needs to eat. Here's an example of a typical dinner we feed Charlie
1. Applesauce with 4 enzymes added
2. Baby food sweet potatoes with ~2tbsp of olive oil added
3. Slice of cheese
4. French fries
5. Chicken Nuggets
6. Ranch for dipping
7. Pediasure for a beverage
8. Goldfish crackers (the last thing we try as they have hardly any calories)
Charlie needs a very high fat diet (because he can't digest his food properly he has a hard time gaining weight) so he gets a lot of fried food, cheese, butter, etc. He also needs a high salt diet so we add a LOT of salt to his food. We only have 30 minutes to feed him so we try and cram in as many calories as possible into those 30 minutes...he usually doesn't eat everything we offer him but we sure do try!
Now I know what you're thinking- it must be awesome being able to feed your kid all the junk food he wants, right? Wrong! I can pretty much guarantee we spend a lot more energy trying to think of ways to get calories into Charlie than most people spend worrying about their kids junkfood intake....and alot more time stressing about it when he refuses to eat his meals (although we try not to!). As CF kids get older some of them find the calories they are required to eat to be a huge burden, they are constantly full (and not the satisfying kind, the uncomfortable kind) and sometimes that still doesn't seem to help them gain weight. A lot of people with CF eventually get Gastrointestinal tubes (G tubes), which are ports directly into their stomach so they can have partially digested food (like Pediasure) delivered into their stomach while they sleep. Charlie is doing great so this isn't even a remote possibility at this point, but these high calorie requirements definitely arent always as fun for the patient as some people think it would be :)
Other:
Some other extra things that we do to manage Charlie's CF.
1. We have a nanny so Charlie isn't in daycare. Aside from the huge risk of Charlie constantly getting sick we also would worry that a daycare wouldn't be diligent remembering his enzymes, etc. Our nanny helps us A LOT with Charlie's treatments and cramming him with food hehe
2. Monthly Synagis vaccinations: Synagis is a monoclonal antibody against Respiratory Synctial Virus. It costs our insurance $5000/month to put Charlie on this medication....and THANKFULLY it is 100% covered! Very lucky with that. Charlie is on it during the flu season and it is adminstered by a visiting nurse in our home. Speaking of which.....
3. We have a visiting nurse come to our house every other week for weight checks (to make sure he's gaining, or atleast not losing, weight)
4. CF Clinic visits: since Charlie is doing pretty well these are only every other month...but if his weight takes a dip it will increase to every month. He meets with a nutritionist, pulmonologist, social worker, clinician, and a bunch of nurses at each visit. He also gets a throat culture (so far he's cultuing Staph Aureus, which apparently is pretty common for CF patients at this stage in the game).
5. Other visits: blood draws for liver function, allergy screening, lung X rays, Gastrointestinal Specialists, Htfd Hospital Feeding Team, etc etc....depending on the issue that month ;)
Well i think that's EVERYTHING (long enough for you?)....now you know what it's like to be Charlie for a day :). I would be happy to answer any questions and if your child has CF (as I know this site has been made available to other parents with CF children) I'd love to know what their routine is, too!
