November Clinic & Weight Gain
It's been several months since our last clinic visit, so I was anxious to see how Sienna was doing at yesterday's appointment. I'm happy to report, she had another great visit!
Si weighed in at 17lbs 6ozs, which has her in the 90th percentile for weight. This is almost a two pound weight gain from her four month wellness appointment! I'm pretty sure her CF team loves having her as a patient, as she makes their overall numbers look pretty good :) Her Pulmonologist was elated about how well she is doing and made us feel great about her overall health. He said the combination of breastfeeding (this is a subject for a whole other post!), keeping her out of daycare, and the 30 minutes of nightly CPT we've been doing are all making an impact and keeping her healthy. It was music to my ears.
I realize I have put a lot of emphasis on Sienna's weight throughout my posts, but may have never fully explained the importance of weight gain for a CFer. Many people with Cystic Fibrosis deal with malabsorption, in which the body struggles to break down and absorb fats and nutrients. These individuals tend to be pancreatic insufficient. When the body doesn't have fat stored, it loses the ability to fight illness off. Research actually suggests a direct correlation between BMI and lung function...which makes sense, because if a person maintains a healthy (or higher than average) BMI, the likelihood of getting sick is lower. If a person has a low BMI, the likelihood of getting sick is higher. Each lung infection causes irreversible damage which slowly diminishes lung function over time. That's what makes Cystic Fibrosis a progressive disease.
Now, Sienna is currently borderline pancreatic sufficient...or insufficient, depending on how you look at it. Apparently normal enzyme levels are 500 + (for a healthy individual). I've read that insufficiency is anything below 200 and extreme insufficiency is anything below 100. Sienna's levels were low 100. Even knowing this, her steady weight gain has kept her off the need for supplemental enzymes which we are thankful for. I have thought about having her re-tested, but I didn't ask about it at our clinic visit yesterday.
This post has gotten long...fast, so I won't dive into too much else from her clinic appointment. Her Pulmonologist did say he thought of us when he was at the CF Conference this past October during the discussions around genotyping and Kalydeco™. I asked him his thoughts on a drug like Kalydeco™ being a good fit for her and he agreed he thought it would help her. I don't want to wish away these precious baby & toddler years, but man I can't wait for the day where she becomes old enough to take a drug like this! He has also been trying to connect with the doctor who wrote a case study about one of the only other documented CF cases with Ex22Dup - but hasn't had luck getting a response. It's nice that our team is really trying to understand what her genes may mean for her and it makes me feel confident that she's in good hands.
I'll save some other details for another post. I'll also have to start saving the articles I read so I can link them here. I'll get better at that!
Please wish us a healthy winter, as this time of year can be tough for a CFer!
Si weighed in at 17lbs 6ozs, which has her in the 90th percentile for weight. This is almost a two pound weight gain from her four month wellness appointment! I'm pretty sure her CF team loves having her as a patient, as she makes their overall numbers look pretty good :) Her Pulmonologist was elated about how well she is doing and made us feel great about her overall health. He said the combination of breastfeeding (this is a subject for a whole other post!), keeping her out of daycare, and the 30 minutes of nightly CPT we've been doing are all making an impact and keeping her healthy. It was music to my ears.
I realize I have put a lot of emphasis on Sienna's weight throughout my posts, but may have never fully explained the importance of weight gain for a CFer. Many people with Cystic Fibrosis deal with malabsorption, in which the body struggles to break down and absorb fats and nutrients. These individuals tend to be pancreatic insufficient. When the body doesn't have fat stored, it loses the ability to fight illness off. Research actually suggests a direct correlation between BMI and lung function...which makes sense, because if a person maintains a healthy (or higher than average) BMI, the likelihood of getting sick is lower. If a person has a low BMI, the likelihood of getting sick is higher. Each lung infection causes irreversible damage which slowly diminishes lung function over time. That's what makes Cystic Fibrosis a progressive disease.
Now, Sienna is currently borderline pancreatic sufficient...or insufficient, depending on how you look at it. Apparently normal enzyme levels are 500 + (for a healthy individual). I've read that insufficiency is anything below 200 and extreme insufficiency is anything below 100. Sienna's levels were low 100. Even knowing this, her steady weight gain has kept her off the need for supplemental enzymes which we are thankful for. I have thought about having her re-tested, but I didn't ask about it at our clinic visit yesterday.
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| Loving these baby rolls |
This post has gotten long...fast, so I won't dive into too much else from her clinic appointment. Her Pulmonologist did say he thought of us when he was at the CF Conference this past October during the discussions around genotyping and Kalydeco™. I asked him his thoughts on a drug like Kalydeco™ being a good fit for her and he agreed he thought it would help her. I don't want to wish away these precious baby & toddler years, but man I can't wait for the day where she becomes old enough to take a drug like this! He has also been trying to connect with the doctor who wrote a case study about one of the only other documented CF cases with Ex22Dup - but hasn't had luck getting a response. It's nice that our team is really trying to understand what her genes may mean for her and it makes me feel confident that she's in good hands.
I'll save some other details for another post. I'll also have to start saving the articles I read so I can link them here. I'll get better at that!
Please wish us a healthy winter, as this time of year can be tough for a CFer!
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