I just sat down and finished my lunch, Mackenzie is napping, I have already showered today, and I find myself with the house reasonably picked up and laundry in the washer. I am dealing with the nuisance of a cold so I am taking a rest on the sofa and thought I would try to catch up on the blog for a bit. I haven’t posted in a while and when I get this far behind, it feels kind of daunting to even know where to start. But I thought I would go back to December and try to jot down what I remember from their most recent clinic visit which was almost exactly 2 months ago (12/13/16)! Historically, this has helped me go back and remember how they were doing from one visit to the next.
Mackenzie was sick the day of their appointment with a nasty respiratory virus and was also diagnosed with an ear infection – her first. Lungs sounded good though, thankfully. Even though Mackenzie had been running a fever for a few days before the visit, Kelsie surprised me and had spiked a fever by the time we checked in which was the beginning of her course with the same illness. She started feeling bad the longer we were there and eventually ended up on antibiotics about a week later after the cold turned into a sinus infection. As is typical with CF, she tends to suffer from more sinus infections than the average kid. And the mucous is so impressively disgusting and distinct and definitely illustrates the description of thicker and stickier mucous that is associated with CF. Even working as a pediatric provider in primary care, I have never seen a kid present with anything like it. It actually turns my stomach. I will spare you the impressive photos I just couldn’t help but take of her nasal discharge! (LOL!) At any rate, it is always pretty clear to me when her illnesses step up from just an average cold to a socked in sinus infection. This past infection was one of her worst and the amount and consistency of the drainage was horrendous. In addition to being congested, she had a cough at night and in the mornings from the post-nasal drip, lost her appetite and her sense of smell. She took antibiotics for 2 weeks and has been well since then.
Mackenzie had an even harder time with with her illness and I am 99% positive she had RSV. I have seen it play out enough clinically and lived it personally when Kelsie had it a few years ago. It has a pretty distinct course, causes tons of nasal drainage, a severe cough and usually gets worse around day 5. Mackenzie went about 5 days without eating anything- literally – just breast milk and fluids and maybe a bite of food in an entire day. She had 4-5 days of fever, was so tired and lethargic, felt terrible, and ended up on steroids by the end because she had wheezing that just wasn’t improving with Albuterol. She was on antibiotics for the ear infection but that obviously didn’t do anything for the nose or cough since those symptoms were caused by the virus. It took a couple of weeks before she was back to herself.
Thankfully both girls have been doing great since that last infection in December and I am holding my breath that we will get through these last couple months of cold and flu season unscathed! I know flu has been going around and I am praying the girls’ flu shots will continue to offer them maximum protection from that nasty germ. Mackenzie did just have about 2 weeks of a snotty nose that just never seemed to want to go away but she never really acted sick, never had a fever or a cough. It is good to see them get over a cold without needing antibiotics.
Aside from their acute infections, which they don’t even usually have at the time of their routine CF check ups, everything else about the visit was pretty routine. Kelsie still wasn’t able to provide accurate blows on her pulmonary function tests but they tell me this is fine and that she will learn the technique soon enough. Both girls were growing well. Kelsie’s weight was 47lbs 30z (70th percentile!) and her height was 3′ 9.5″ (66th percentile!). For a CF kid who hasn’t taken any nutritional supplements since she was 2 years old, I am so proud and impressed that she has maintained her weight so well! I was nervous she may drop her weight some after starting school but that clearly hasn’t been an issue at all. That doesn’t mean she is a wonderful eater, however. She actually never eats everything on her plate and her lunch box comes home half full many days. But she still seems to be getting all the calories she needs to grow, so I try not to pester her about it too much. I do try to make sure I have at least two foods higher in protein in her lunchbox for her to choose from. These are some the things I commonly pack: peanut butter/nutella/banana or turkey/cheese sandwiches, yogurt, cheese, carrots and dip (ranch or hummus), fresh fruit (kiwi, rasberries, oranges, apples), corn dog (although now she tells me she doesn’t like these anymore), chips, crackers, pirate booty, popcorn, yogurt raisins, etc. I change it up every day and do a variety of things. I don’t do a lot of junk food or processed foods and still try to offer nutritious foods including fruits and vegetables. Thankfully, I haven’t had to stress too much about her caloric intake in a while.
The other thing that impacts her appetite is her stooling patterns and this is always a topic of discussion at her clinic visits. We still have to give her Miralax to help prevent her from becoming constipated. I have tried to just use it as needed instead of giving it daily but every time this comes up, they reiterate the importance of giving it every day. Since she becomes constipated so easily as a result of CF, they prefer for her to take the Miralax every day to keep her stools soft. This prevents her from getting backed up and losing her appetite in the process. She also has to take acid reflux medicine every day and they do not want me to try to wean her off this either. It helps the enzymes work better and keeps any symptoms of acid reflux at bay so she can have an optimal appetite without pain or discomfort getting in the way.
Mackenzie’s weight is not a concern at this time either, however her’s is not as robust as Kelsie’s. She has always hovered right at the 50th percentile mark. In the CF world, that percentile range is very important and they start talking supplements if their weight starts trending below the 50th. Mackenzie’s weight was 21lbs which was 45th percentile at the time of her visit, however, we blamed that little bit of a drop on being sick and not eating anything for the two days prior. She typically has an excellent appetite and eats a variety of meats and table foods. When Kelsie was a toddler, she started developing a lot of issues eating, both from a medical standpoint (likely due to constipation and reflux) as well as behavioral. She actually lost weight at one point and we had to start feeding therapy. So far Mackenzie seems to be doing just fine eating but I hope her weight will continue to keep up with her appetite. We did increase her enzymes from 4.5 to 5 capsules with meals because her stools were starting to show signs of malabsorption. And now it’s been exactly 2 months later and she’s starting to show those same signs (horrendously malodorous, greasy and increased frequency of stools). She has always been at the max on dosing of enzymes so I’m guessing these new signs mean she is already outgrowing this dose as well.
The biggest thing we talked about at this last visit was the new drug Orkambi. It is a new drug, and the first of its kind, that treats the underlying cause of CF in people with 2 copies of the Delta F508 mutation, which both of our girls have. This is very exciting for the CF community and undeniably the result of much research and hard work. It has been on the market for a while but was just recently approved for children 6 and up. However, there still is not a lot of objective data to show it’s effectiveness. To some, it may seem like a no-brainer to start it. But we still have questions about it’s risks. We also question if/why we should start it now since she seems to be doing so well with minimal effects of the disease. There is evidence to show that it decreases the number of pulmonary exacerbations which will in turn decrease the damage to the lungs over time. But to date, Kelsie has yet to have her first pulmonary exacerbation (though we know it is coming one day). Since this drug claims to decrease those exacerbations, could it just prolong her lungs from enduring that irreparable damage that is bound to happen at some point? But what if the side effects and risks of liver damage are greater? We had a lot of questions and our provider admits to having many of the same questions with no solid evidence to counsel us one way or the other. She did say that while she has not seen any hard evidence like an increase in weight gain or improvements in pulmonary function tests in her patients, everyone who is on it says that they just feel better! I don’t know how we would measure that with Kelsie because she never really reports feeling bad. She has a tummy ache from time to time and struggles with constipation and sinus infections, but she doesn’t seem to have any other complaints.
So we have a decision to make at our next appointment as Kelsie will be 6 years old and eligible to start the drug. Will we start it or will we wait? Either way, I think we have reasons to support our decision. Right now, we are just thankful that she is doing so well health wise despite having CF and that we have this choice to make at all. Her doctor put her in the “above average” category in terms of her course with CF and how her body is being affected by the disease. So we are giving praise for that, and for the research and development of this drug (and many others coming along the pipeline) that attempt to correct the CF defect at the cellular level.
More on this next month….
Here are a few more photos from that day:
And Kelsie perked up and still wanted to go to the Carolina Kids Club Meet the Heels event at the Dean Dome. Only the kids were allowed on the court but she went independently and stood in line for autographs from the Men’s Basketball Team. She enjoyed it but was disappointed that she didn’t get to actually play basketball on the court! 😉 Hehe! Here she is with Kennedy Meeks…
And Joel Berry…
And we met up with our dear friends, the Kings! Sweet memories!