Both girls had their CF clinic visits last week. This was our 3rd “double” visit, meaning both girls are seen by the same doctor at the appointment. These visits are kind of like a typical child’s yearly well child visit, in that they have to go routinely – even when they are well – but instead of just going once a year for a checkup, they have to go every 3 months to check in with their pulmonologist. These visits are also much longer than a typical well child physical. Like 4 hours long! It does not take that long to see the doctor but we just have to see several different disciplines at the same visit. We see the nurses, physician, respiratory therapists, dietician, pharmacist, and social worker. I really appreciate the comprehensive care we receive. But besides the length of it (which we have come to expect), it was a good and routine visit.
When we check in, Kelsie always gets a mask to wear. This is to keep her from breathing in any harmful germs in the air. She doesn’t love it, but she always complies. We explain why it’s important and that makes her actually want to do it. Teaching her the “why” behind things instead of “just because I said so” is typically a lot more effective for us. I have always tried to teach her the importance of staying healthy and remind her how much better we feel when we are healthy. So I tell her – this mask is a good thing, to keep you safe and healthy, so even if you don’t like it, it’s the right thing to do to wear it to protect yourself. And she doesn’t argue.
I usually take along a few new activities for her to help pass the time. This $1 Cinderella coloring page was a BIG hit and was quite beautiful and colorful by the end of the visit!
It seemed like a pretty straightforward visit, yet, I still came home with a number of things to report to my husband.
This was the first time since Mackenzie was born that my husband has not been able to go with us to the appointment. He had a work commitment, but thankfully, my mom was gracious to take off work and go along to help me out with the girls. It was such a blessing having her there. Despite what we were there for, it was nice just getting to spend the day with her.
Thankfully, both girls have been well since their last appointment in February. Mackenzie was on antibiotics in April after a bad cold that wasn’t going away on its own. Kelsie, on the other hand, went the entire 3 months between visits without needing an antibiotic! She has only needed antibiotics for sinus infections so far in her life, but this winter she seemed pretty resilient and the few colds she had passed on their own without the need for antibiotics. She did have a cough for about 3-4 weeks at one point but it seemed mostly related to post-nasal drip. It also occurred around the time when the yellow pollen was abundant in the air, and this seems to affect her every year, so we treated it as allergies and used nasal steroid spray (Flonase) to combat the congestion. It eventually went away on its own and there hasn’t been any cough since.
Weight/growth is always a big topic of conversation at CF visits since many people with CF have difficulty gaining weight. I am always curious to see their growth charts and make sure they are growing appropriately.
Kelsie’s weight looked fantastic! She was 75th percentile for weight and 60th for height!! She looks thin but feels solid! All of her activities seem to be building some serious muscle! 😉 She weighed 43lbs 7oz. Her appetite and picky eating behaviors have greatly improved over the past year. There are still some eating issues (mainly not eating enough at meal time), but according to her weight, she is eating exactly what she needs to maintain a good healthy weight!
Mackenzie’s weight for age has been dropping at each visit (it was 54th%, then 47th%, then 41st, now 37th%), however her weight for length is holding steady just above 51st percentile which is still above goal of 50th percentile. So there were no concerns and no changes made. Her weight was 17lbs 10oz. They will recheck again at her next visit. The dietitian thought she was eating well based on my reports and was impressed/surprised at her preference for finger foods. I explained that she seems to have a mild aversion to being spoon fed and will pull/push away from food on a spoon, however she will suck it right out of one the baby food pouches. She also likes to self feed and will gladly pick up and eat small pieces of food on her tray. I asked how often I should offer her solid foods and she said 2-3 times per day depending on what she is eating. She needs a shocking 1200 calories per day, which is 30% more than the average baby her age. I am certainly not counting calories at this point, just found it to be an interesting tidbit of information.
We also talked about the use of PPIs (proton pump inhibitors) at this visit. This is a type of drug used to reduce acid and treat acid reflux (ex. Omeprazole/Prilosec, Lansoprazole/Prevacid). There has been data to suggest that the chronic use of PPIs can lead to complications later in life. Since Kelsie has now been on daily use of these medications since birth and Mackenzie is on track to do the same, I was interested in hearing if there was any data to support trying to wean them off of these medications at some point. The information shared was quite informative and validated the need to keep both of them on these drugs for the long term. A higher pH (less acidic) help the enzymes work more effectively and thereby aid in digestion. Chronic reflux and acid exposure can also damage the esophagus which has been shown to increase cough and inflame airways. It is also important to consider that the studies that have indicated poor outcomes with chronic use looked only at the elderly population. Based on this information, the conclusion was made that the benefits of PPI use in pediatric patients with CF far outweighs the potential risks.
Kelsie attempted PFTs as she routinely does but her results were not as good as they have been. It was deemed a trial run since the technique seemed to skew the outcomes. She had really good PFTs a couple of visits back and it appeared that she had figured out the technique. However, she has not performed as well the past 2 times. Since she has no symptoms of airway disease (no cough, no difficulty breathing), her performance this time was considered “practice” and not a true representation of her lung function. She is still young so this isn’t surprising or concerning.
The respiratory therapist came in and reviewed chest PT procedures for both girls. For Kelsie we just discussed proper technique for using the aerochamber/mask with the Albuterol (which she continues to do twice a day with chest PT prior to hypertonic saline). She uses the inCourage airway clearance machine.
For Mackenzie we also reviewed proper technique for infant chest PT. It is admittedly hard to get a full session in on her in the mornings between feedings, naps, preschool drop offs etc. Instead I tend to pat her chest for short periods throughout the day when I am able, but as she reminded me, it is better to get a full session in than several small segments. She also gave me an small infant mask to use with her pats. This really helps. It is hard to cup your hands in a way to target those small areas over each lung field. We are supposed to devote 3 minutes to each of the six different areas on her back covering both right and left upper, middle, and lower portions of her lungs. Then we do the upper anterior lobes on the front of the chest above the breasts, as well as each side.
In this video, you can see what this looks like, as well as Mackenzie’s emerging love of baby dolls! (Just like her big sister!) Of all the toys surrounding her, she will go for the baby every time!
Kelsie’s biggest fear surrounding these visits is the throat culture. They have routinely done this test at each visit since she was a newborn so she expects it, and anticipates it with the utmost dread. This time she asked if she could have a shot instead. Seriously! Even though we did the “magic lollipop” last time, she still has a lot of anxiety built up around this test. It is getting better though. The lollipop definitely helped last time and a couple of days before the appointment, she asked if she could get one this time too. Since I didn’t have one, I panicked the night before and went out in search of something that looked believably “magical”. The closest store is actually the Dollar Tree so it was my first stop went and thankfully I found this gem! I laughed at the sight and imagined what our dear nurse would think when I handed her that thing! 😂
Of course, the nurse brings it in and it’s an official, from-the-pharmacy, magic, throat-numbing lollipop! (Well, at least it helps her to believe that!) 😜 It may not actually numb the throat but it numbs her anxiety over the whole ordeal and that’s what matters most.
She enjoyed that lollipop for about 15 minutes while we waited and then when the nurse came in to do the test, she held it in front of her face and took 10 frantic licks, trying to get as much magic power from it as possible, before I took it down and held her in a big hug for the actual throat swab. She was still nervous about it and had a hard time willing opening her mouth wide enough with tongue out but she did it. This time the nurse didn’t even have to use the wooden tongue blade to press her tongue down. She eventually stuck her tongue out and opened wide enough and it was over in seconds at that point.
Now we wait for culture results (looking for any problematics organisms in their sputum) but I don’t expect anything out of the ordinary. And with a few months out of school for the summer, I am hoping everyone continues to stay healthy! Next thing on the agenda is to get paperwork completed for Kindergarten, school letters written regarding CF, and meet with administrators for her 504 plan. And then fingers crossed and prayers will be lifted as we enter this new season of Kindergarten and public school attendance. More on that to come.