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CF Clinic Update

Thursday, October 2, 2014

On Monday, Bennett had his quarterly Cystic Fibrosis check-up at our CF Clinic in Dallas. 

Avonlea came with us so I made sure to bring blankets, toys, snacks and an iPad so we could survive the several hour appointment with a 1 year old and preschooler crammed in a small clinic room. Both kiddos did great.  They had each other, which seemed to help them both stay entertained.


Thankfully, according to the CF doctor, Bennett's lungs sounded great.  

The doctor and I some took time during our appointment to look at Bennett's recent CT Scan results together:

A high resolution CT Scan takes pictures of lungs as though it's cutting a slice of cookie dough.  This is a picture of his lungs from the inside.  We are able to see his lungs much more clearly than what could be detected on an x-ray.  However, the radiation from a CT Scan is the same as 75 x-rays so Bennett will not get another CT for quite some time.

The most promising news of Bennett's CT Scan was there were no signs of bronchiectasis, which is scarring damage to the airways.  Some inflammation to some of the airways (see small arrow in picture) can be seen.  But, thankfully, it hasn't turned in to bronchiectasis yet.

Bronchiectasis is what you don't want to see but is inevitable at some point with the progression of Bennett's disease.  The fact that we've made it to 5 years old without bronchiectasis is really wonderful.  We are thankful for every good day of health!!


Here is a close-up of the inflammation in Bennett's airway.  See the long black tube with white lines along side of it?  That's inflammation.


We discussed Bennett's atelectasis (his partial right lobe collapse) results on the CT Scan.  We aren't sure if this atelectasis is just a captured moment in time - or if it's something that is there permanently. Atelectasis is hard to see on x-rays so we don't know if it's been there a while.  As long as Bennett's asymptomatic (not showing any lung issues), the doctor doesn't want to do anything about it.  It may turn in to an infection in the future.  But, right now, we aren't going to do anything.


The close-up of the atelectasis.  (See the long white image next to the arrow.  This partial lobe collapse is laying next to his heart.)

Having said all that, the big focus of this appointment was not Bennett's lungs but his weight. Bennett is continuing to struggle with weight gain.

He is not alone.  Those with CF often struggle to maintain a healthy weight due to their higher-than normal caloric needs and digestive/malabsorption of fat issues.  But, long-term survival and overall lung function is linked with nutrition status in both children and adults with CF.  

Bennett's struggle with appropriate weight gain, may ultimately affect his lungs in the long-term.  So, we are continuing to evaluate our options here.


This is Bennett's height chart.  He is between the 25th-50th percentile in height.  Pretty normal, we think.


This is Bennett's weight chart.  His plotting shows he is gaining weight but, because he keeps getting taller without gaining more weight, he's essentially losing weight over time.  He is currently in the 18th percentile for weight gain.  


Our biggest concern is the downward pattern in his BMI.   Bennett's BMI is currently in the 13th percentile.  

Nutritional status has a significant effect on pulmonary disease progression and survival in patients with cystic fibrosis.   In fact, young underweight patients have worst pulmonary function outcomes, likely because "accrual of lean body mass is the factor that is involved in the preservation of lung function."  The Cystic Fibrosis Foundation recommends that CF patients stay in the 50th percentile.

So, in order to keep Bennett well, we have to get him to put on some weight.  This is part of our fight against his disease.  CF makes gaining weight hard.  We are fighting back with all the ways we know how.

We have decided to try new enzymes before his tube feds every night.  If this doesn't work, we may have to increase the amount he gets tube fed at night or even do tube feedings during the day.  

It's not that Bennett doesn't eat or that he doesn't like to eat.  It's just that he needs to eat a lot to stay healthy and, like any preschooler, he eats small amounts and can be picky.  

We have a follow up appointment with the CF Clinic in two months to see if our efforts are working.

Overall, despite seeing some progression of the disease in his CT Scan and hearing his BMI continues to drop, we are very thankful for the thumbs up to good health that Bennett received at his CF appointment!  

2 Responses to “CF Clinic Update”

  1. Breck, Every time I read your blog I can't help but be struck by how similar our families are. I have a 7 year old boy, an (almost!) 5 year old with CF, and a our caboose baby girl that is 17 months. While there are some differences in how CF manifests itself in Finn and Bennett, there are some interesting similarities. I'm glad things seem to be going well, except for the weight issue. We have the same issue here. Finn's BMI fell to 7% at our September visit. I have a feeling a gtube will be in our future this winter. Anyway, just wanted share with you and I'm glad the MRI looked pretty good. --Angie

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  2. With MRI scans, there is no radiation involved. That is one of the main benefits of using MRIs. They use magnets (hence Magnetic Resonance Imaging, aka MRI) and radio waves that "talk" or communicate back and forth to create a picture or image of whatever body part is being studied. On the other hand, CT scans (Computerized tomography) use high levels of radiation, which is why they are used as infrequently as possible.

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