CF Clinic Update and First PFTs

Today was Cystic Fibrosis Clinic Day at Dallas Children's Medical Center.  Today's clinic was focused on Bennett's continued struggle to gain weight.

We met with our new Cystic Fibrosis Doctor, Dr. Sharma (our other doctor recently transferred to Houston) who gave us the best news of the day: Bennett's lungs continue to look great!  But, Bennett's weight continues to be an issue.

Studies show people with Cystic Fibrosis at or above the 50th percentile with their BMI do better with their lung health.  Bennett's is at the 11th percentile.

Brian and I have known Bennett's inability to gain weight has been a problem for a long time.  But we have felt helpless.  We offer him whatever high calorie, high fat foods he wants.  But he tends to prefer turkey, oranges and gummies.  Bennett *can* eat and he will eat, at times.  But, overall, he's just not eating enough to keep himself at a healthy weight.

The issue with his low weight isn't so critical now but it will be should his lungs get infected (which is just a matter of time). Bennett needs those extra calories to give energy to his body/lungs to repair itself.  In addition, Bennett's low weight makes him vulnerable to getting sick and, once sick, vulnerable to getting sicker.  Malnourishment is a concern.

Outwardly, I try to be calm and level-headed but, inside, I feel helpless and anxious.  Despite oour best efforts to help Bennett gain weight, what we are doing isn't enough.

Bennett wants to please us and he often indicates he wants to eat...but he usually doesn't feel hungry.  He regularly complains his stomach hurts.  And he, like many with CF, struggles with GI issues when he eats high fat foods...enough to find them not very appetizing.  So, who would blame him for not eating?  The issue is not only that he must eat but that he actually needs more calories than healthy children his age.

Dr. Sharma offered me a few possible solutions: we could put him on a higher caloric formula or we could use calorie additives such as Duocal or Liquigen to his existing food.  I agreed that a calorie additive made sense and suggested that we also begin implementing a daily afternoon g-tube feed as well...something we haven't done in years.

Dr. Sharma thinks we may need to implement a day-time feed for only a short time "just to help him get him over this hump" as she believes social pressure will likely help him begin to eat more normally.  But, I'm not so sure.  At least we have the g-tube to use and we will use it to help him.  As much as we want his life to be "normal," Brian and I would rather sacrifice "normalcy" for good health.  I hate that he will not be doing a tube feed every day after school.  But, I am happy that this should help bring him back to a more optimal place of health.

I witnessed a special moment with Bennett and his doctor today.  Bennett saw a picture of lungs on the back of our clinic room door so he inquired about it with Dr. Sharma.  The doctor spent a few minutes talking to Bennett about how lungs work, specifically his.  Bennett listened intently.  And Dr. Sharma really honored his questions.  It was a neat experience to see Bennett begin to really want to understand his body on a deeper level and I was thankful for a doctor who took the time to care.

In between visits with the doctors, nurses, dietician and social worker, Avonlea, Bennett and I had a picnic on the clinic room floor.

Today, Bennett got to do something super special in the CF world.  Today was his first opportunity to learn how to do a Pulmonary Function Test (aka PFTs).  Megan, the respiratory therapist, first had Bennett to put on a pair of gloves.  Bennett loved this!  CFers must wear gloves when they do their PFTs due to infection-control issues as they handle equipment that must be shared between CFers.

Megan explained to Bennett that he was going to get to play a game on her computer.  She showed him an image of 7 candles.  Once set up, she had him blow as hard as he could to see if he could blow out all of the candles.

Doing PFTs at 5 years old is less about actually getting important information about the lungs and more about teaching the child how to accurately do a PFT.  It takes a few years for children to really get the hang of it.  But, once they do, PFTs become a very good indicator on how healthy or sick the lungs are at any given time.  From this point on, Bennett will practice/do his PFTs at every clinic visit for the rest of his life (typically 4 times per year).

This first time, Megan set up the computer to make it easy for Bennett to blow out all of the candles.  However, over time, Megan will begin making it harder for him so he can eventually get us a good reading of how his lungs are doing.

See Bennett do his first PFTs here:

One of the things Megan did not use this time but will eventually use with Bennett is a noseclip.  This will help prevent any air from getting out of his nose during the test.  On one level, the test looks fun - to see how hard you can blow.  But I've watched an adult do it before and it actually looks rather painful to force out every bit of air from your lungs, particularly when they are not feeling well.  I think Bennett is quite a champ to have to do this every three months for the rest of his life.

I had to include this sweet picture of Bennett playing in between visits from his CF team.  He enjoyed using the plastic Pulmicort model as a base for his superheros.

After our appointment, we went downstairs to take a picture with the Children's Medical Center Christmas tree.  We have a picture with Bennett almost every year with the hospital Christmas tree.  At this point, it's just tradition.

Bennett wanted to include some silly shots of him this year.

Our little monkey boy earned himself two Superman stickers for his good work today.  He was very proud of them!

We left today's appointment feeling a bit disappointed but also happy.  It is annoying when CF makes life harder.  But, we also realized how blessed we are to have Bennett as healthy as he is right now.