Our CF Regimen

Many people have asked how we care for Madeline regarding her cystic fibrosis. Since we've finally got her routine under control (for now), I thought I'd share what we do each day to keep her as healthy as possible. First, though, it's important to know how CF affects the body. I wasn't clear on the details myself until Madeline was diagnosed, so I'll explain quickly.

CF causes the body to make extremely thick, sticky mucus that leads to problems in the lungs and the digestive system. Because the mucus is so thick, it builds up in the lungs and can't be coughed out very easily. Bacteria gets trapped, causing lung infections and ultimately lung damage. This eventually leads to reduced lung function.

The mucus prevents the digestive system from working properly by clogging the pathway from the pancreas to the small intestine. Since this path is blocked, enzymes necessary for digestion never get to the small intestine, thus making it impossible to absorb fat or break down most food. That's why Madeline only gained six ounces in the first nine weeks of her life. Although she was eating and eating and eating, the nutrition she got from her efforts was basically just enough to sustain life. Once she started taking replacement enzymes, she gained six pounds in nine weeks. Impressive change, I would say! People with CF generally need to consume more calories than the average person, so Madeline likely has a future of adding cream, butter, and lots of cheese to every meal. Possibly the only benefit of having CF.

Now for our CF regimen. It has required a lot of trial and error to find the best way to do all of this, but for now we've got it down. First of all, Madeline takes enzymes before every meal, something she'll do the rest of her life. The enzymes come in capsules that are filled with small beads, like this:

Although Madeline is, of course, an extremely advanced baby, she can't swallow pills yet. Instead, we open the capsules and mix the beads with a couple spoonfuls of applesauce. (The acidity of the applesauce prevents the beads from breaking down before they get to her small intestine.) This part of the routine has gotten much easier now that she knows what to do with the applesauce; we used to just shake the beads into her mouth instead.

Madeline also takes iron drops every day because she is/was anemic. Giving them to her undiluted is what you might call less effective. Instead, we either mix the drops with sugar water or make her a little "iron shake" of iron drops and breast milk. In these forms, she's not a fan but she'll still look you in the eye afterward. I put this mixture in a medicine dispenser that my friend Natalie told me about:

Next up in Madeline's regimen is Prevacid for acid reflux. Thank heavens for Prevacid! She was seriously a new woman once we started giving her this medicine every day. Pre-Prevacid, she would sob from 10 p.m. to 2 a.m. every single night because she was in so much pain. This was also before she started taking enzymes, and the discomfort of reflux was compounded by stomach cramps that were caused by not being able to digest her food properly. (She'll probably be on an acid-reducer the rest of her life because it helps the enzymes work better.) Madeline loves, loves, loves taking Prevacid because it comes as a yummy strawberry-flavored solutab. It makes me feel like I'm giving her candy. She deserves it.

Because Madeline can't absorb the fat-soluble vitamins on her own (A, D, E, and K), she also takes vitamins that are specially formulated for CF patients. Our first attempts at getting the vitamins into her system looked something like this:

I have a big orange stain on my carpet to match. Luckily, however, we soon switched to a different brand of vitamins that's not as nasty and not as orange. I mix the vitamins with some breast milk (also in the medicine dispenser) and call it a VitaSplash to make it sound more appealing and smoothie-esque. Madeline always thinks she's getting a big treat when she gets her daily VitaSplash.

We also give Madeline an extra 1/8 teaspoon salt every day (people with CF lose too much salt through their sweat). Have you ever tried to give a two-month-old baby salt? Or a five-month-old baby, for that matter? Yeah, not easy. I still haven't figured out a great way to get the salt into her system. I do throw a dash of salt into both her iron shake and her VitaSplash, and I also salt her daily bottle (one bottle of breast milk that is fortified with formula to add calories). When she starts eating solids, I'll salt her baby food and it should be a lot easier to reach 1/8 teaspoon.

At the CF clinic this week they told us to start Madeline on the highest calorie vegetable (sweet potatoes) and to introduce meats fairly soon because of the protein. They also said we should add some type of fat to all of her vegetables, meaning any oil or butter. Madeline's baby food is going to be far tastier than what most kids get, that's for sure.

We also recently started chest physical therapy, or CPT. Right now we do it once a day for about 30 minutes. Pretty soon we'll start doing it morning and night, though, and CPT will just be a regular part of Madeline's routine for the rest of her life. The goal behind CPT is to loosen the mucus in her lungs so she can cough it out before it causes lung infections (and thus lung damage).

When we do CPT, we start out by giving Madeline albuterol through an inhaler. The albuterol opens up her airways and makes it easier for the mucus to drain out. Then we take a manual percussor and percuss eight different areas on her chest, side, and back. The percussor looks like this (Madeline's is pink):

Madeline isn't the world's biggest fan of CPT, but she's a good baby and she lets us do it, albeit with a lot of wiggling. Sometimes I sing or Dave plays the piano so she has something else to think about besides the CPT.

Well, that's our day in a nutshell. Madeline's got a lot of equipment for a little baby, but she also gets to do cool things like eat applesauce before other kids and drink a daily VitaSplash. For the most part, she's extremely easygoing--a real star at putting up with everything we do, which is a big blessing.

See what I mean?