Cystic Fibrosis Management for a 4 month old

Hi everyone! I thought maybe some of you had wondered at some point what a typical day is like for Charlie, in terms of managing his CF.  SOOooo I thought I'd show you!


First thing we do around here is use LOTS of hand sanitizer to make extra sure that Charlie doesn't get sick! 

One of the first things we do in the morning and last things we do at night is chest PT, or physiotherapy, or clapping.  We manually try and break up the globs of mucus in his lungs by thumping on his chest.  We clap 3 areas on each lung for about two minutes each (ends up being about 15 minutes or so total per session).  Most spots we cup our hands and clap manually but for the front we use something called a percusser because it's a little smaller for his small chest.  Even though Charlie isn't showing any outward signs of lung issues, such as coughing, it's still important to do PT to break up smaller mucus blockages in his lungs and to try and prevent any damage from happening in the meantime.

In the very beginning Charlie HATED chest PT and screamed the entire time, it was horrible!  But now he's gotten used to it and smiles during PT time, sometimes he even falls asleep :)

Then I usually make up his bottles for the day (typically around 4x 5oz bottles).  I both breastfeed and formula feed Charlie, CF patients need anywhere from 10-100% more calories than a healthy baby. Charlie in particular eats a LOT, I can't produce enough breastmilk so we supplement with formula.

Using formula also gives us the opportunity to add extra calories.  I make up his bottles at 26 calories/oz as opposed to the 20cal/oz most other babies have.  We also add in some rice cereal as a thickener because of Charlie's reflux, the thicker formula helps it stay in his stomach a little better.  It's very important that Charlie gets as many calories as he can (and keeps as much food down as he can) because a weight/length ratio in the 50th percentile has been shown to correlate with better lung function and life spans further on down the line.  Charlie had been pretty consistently in the 3rd percentile for weight to length (he's a very long baby!) and just recently was bumped up into the 10th!  He had such bad reflux that it was affecting his weight gain and since that has been treated he's been doing much better.  So we're making good strides but we still need him to get up into the 50th.  I also add 1/8th tsp of salt into one of his bottles.  Since cystic fibrosis affects the sodium channels we add that salt back into his diet.  Before he started taking formula we had to stick our finger in some salt and then pop it into his mouth- he made some pretty hilarious faces when he got a mouthful of salt! haha

Once its time to actually feed Charlie I start getting his medications together.  One of the most important things Charlie needs is his Creon, or pancreatic enzymes.  His pancreas gets gummed up with mucus and can't release some of his digestive enzymes so we give those to him before he eats anything.  Without these enzymes Charlie isn't able to digest fats or proteins (and because he needs to gain weight he needs to eat a lot of fat); he will need to take these enzymes before any snack or meal for the rest of his life.  He needs 1.5 capsules before every feeding, I open the capsules and divy out the beads inside since he obviously can't swallow them.  Each enzyme dose gives him a 40min window to eat.  I typically aliquot an entire days worth of enzymes (6-8doses) so they're always ready to go.

Next Charlie is on Prilosec and Erythromycin for his reflux issues, he gets 0.8mL of each in the morning and 0.8mL of each again at night, he also gets an extra 0.8mL of Erythromycin mid-afternoon.  After trying many doses and combinations this seems to work the best and his reflux is much better now!

Charlie also takes fat soluble vitamins.  Like I've been mentioning, Charlie can't digest fat so we give him these vitamins and minerals so we can be sure he's getting what he needs.  He gets 0.5mL of this twice a day and Robb and I both HATE it because it's bright orange and stains EVERYTHING it comes in contact with.  It's the worst!  After all those meds Charlie can finally eat :)

Now I bet you're wondering how we keep all these medications straight.  I have a worksheet I print out weekly that has all of Charlie's medications per day on there as well as spaces to make notes about other issues.  We check off each thing as it gets done and we're able to keep things straight and not over/under dose.

You'll notice that 'stool' is on there.  One of the things we need to keep an eye on with Charlie is greasy stools.  If he has a greasy stool it means that the fat in his diet isn't getting absorbed (thus why it's in the stool, making it greasy) and probably needs an adjustment in his enzyme dose.

Charlie also has a 'sick plan' given by his team at the CF Center.  In case he DOES get sick it's important to try and keep his airways clear so we have something called a nebulizer.  The nebulizer aerosolizes medication so he can inhale it. 

We also have a medication called Albuterol which helps to break up the mucus in his lungs.  So if Charlie ever gets sick and congested we're prepared with medecine and the nebulizer to help prevent any lung damage.  Luckily we haven't had to use it yet! Although as he gets older the nebulizer treatments will become part of his normal routine, typically getting them twice per day.

Charlie also gets a once/month shot of Synagis.  This is an antibody against Respiratory Syncytial Virus that he'll get throughout the flu season.  A visiting nurse comes to the house once per month to adminster it for us.

The visiting nurse also comes every week (that he doesn't already have a dr's appt) to do a weekly weight check.  As I mentioned before the CF team is worried about Charlie's weight and we're trying to get him up to the 50th percentile so they like to do weekly weight checks to see how he's doing and to make sure he's gaining (not losing!) every week.

Charlie sees his CF Team at CT Children's Hospital in Hartford once per month now (initially it was once/week, then every other week, etc).  Each appt takes about 2 hours and he meets with a pulmonologist, a dietician, clinician, social worker, and nurses who are all fantastic!  Charlie also meets with a great gastro intestinal specialist once per month as well to keep an eye on the reflux and weight gain.  And of course he still has all his normal pediatrician appointments for vaccines, etc.  I use a binder to keep all these appointments straight, it's organized by month and has all the items discussed at each meeting as well as any handouts/important information.  This is also where I store the weekly medication checklists for quick reference back during any appointment.

Well I hope that gives you an idea about Charlie's CF and what it means for him.  He's a fantastic little guy and takes it all in stride :) He's always got a big grin on his face even when we're giving him his foul tasting medications!  I'm going to try and keep the blog updated more about his doctors appointments and things like that from now on so everyone can keep up with how he's doing.  In the meantime if you have any questions feel free to ask or go to the Cystic Fibrosis Foundation  (http://www.cff.org/) to get more information- and might I add that it's a swell organization to donate money to as well :)