Wednesday, May 30, 2012

What it's like to have two kids with cystic fibrosis


How does the other half live? Before I had a kid with special needs, I had no idea. Even now, I’m still constantly learning about conditions and diseases that affect children (and adults). Most recently, I got a better understanding of cystic fibrosis (CF) from a parent who has two children with the disease. Brenda Fox is mom to Lucas, 9, and Hannah, 6. She works as a store manager for CVS/pharmacy in Indiana, and I met her through work I’ve been doing with CVS Caremark All Kids Can.

Brenda is co-chair of a new CVS/pharmacy in-store and online fundraising campaign, Advancing Medical Research, to support research to improve the lives of people living with cystic fibrosis and amyotrophic lateral sclerosis. It’s a national effort that’ll run through June 30. Also: May is Cystic Fibrosis Awareness Month. Once you read what Brenda has to say, you’ll understand why funding new research is so important to her and the 30,000 children and adults in the U.S. affected with CF. I asked her to explain the things that people don’t get about the disease.

Cystic fibrosis is a disease, not a condition
“A lot of people don’t understand what CF is. It’s a genetic disease of the mucus and sweat glands. I didn’t know much about it myself. Then when Hannah was two and a half, she had a really bad chronic cough. She’d go on and off antibiotics—our pediatrician was just treating the symptoms. We ended up at the allergist, and he knew right away that she had it from the way she looked—very skinny, belly kind of protruding—to the fact that she had loose bowels. The way they check for CF is to see if there are elevated levels of salt chloride in the body. Within 24 hours, we got a call that she’d tested positive. My husband and I were completely devastated; we went right to the negative, and I read all the bad things. You and your partner both have to be carriers for a child to get CF. We realized we needed to get Lucas tested too. Within a six-week time span, we’d gotten the next phone call. The life expectancy for people with CF is 37 years old, which was my age at the time the kids were diagnosed. I couldn’t believe this was happening—it was like a bad dream.


Living with CF means daily therapy
People who get CF produce a lot of mucus, which gets into their lungs and makes it hard to breathe. It can hurt my kids to take a deep breath. It can also block their pancreas, so food can bypass that and come straight out and their body doesn’t absorb the nutrients it needs. My kids take 15 to 25 pills a day to break up the mucus—four at every meal, and two with snacks. They also do Vest therapy every day, a machine that pounds their chests for a half-hour to break up the mucus. They each have their own vest and they do it every evening. It doesn’t hurt and they’re used to it—Hannah falls asleep on hers!

CF can impact fertility
Lucas will never have children because he’s sterile from the CF. When our pulmonologist told me that, I was heartbroken, though she said that someday there might be help. Hannah could have kids but has a higher risk of having a child with birth defects. We pray for treatments.

It’s not that I’m not feeding my kids!
The majority of kids with CF are thin. Mine down 3000 to 5000 calories a day. We eat McDonald’s three to four times a week! It’s not bad for my kids. They also do nutrients and protein bars. 

We don’t put our kids in bubbles
My kids are typical kids in many ways—they both love to hang out with friends and play soccer, and Hannah’s in gymnastics. When I tell people they have CF, sometimes they say  “Are you sure? They look so healthy!”

States are now screening babies for CF
We don’t know if anyone on either side of our family has CF; my dad is adopted, so it could be on his side. Hannah was born after it became law to do newborn screening. If they catch it early on, the therapies can start early on.

Sometimes, it gets hard for parents
We have a great neighborhood and everyone understands about the kids, but when their friends come over they’ll ask, “What is that thing?” and point to the Vest machine, or “What are those pills?” We’re always explaining and educating friends and neighbors. I try not to think about the long-term. I’m not supposed to live longer than my kids; they should live longer than me. I try to focus on their daily lives. 

There’s major hope for a cure
There are more than 2000 types of the CF gene. Most recently, the FDA approved a miracle drug for the least common one, the G551D mutation. It’s a pill known as blue lightning. Some CF-ers on the pill have an increase in lung function and weight gain. Now they’re in the process of trialing that medication for the 508 gene, the most common one that both my kids have. We are so close to a cure! The hope is that it will happen by 2016. Every year that goes by, a CF patient’s lung function decreases. Lucas and Hannah’s lung function is great—we are so adamant about doing their therapies. We don’t want them to have to get lung transplants. 

We just participated in the Cystic Fibrosis Foundation Great Strides Walk. Our team raised $10,000, which will go toward research. I’m on the committee for the walk. I wish more people would get involved with CFF chapters. It’s so good for familes to realize they’re not the only one out there. And it’s heartening to take action—I’m doing something for my kids. They know that mom and dad are fighting for them. 


This is one of a series of posts sponsored by CVS Caremark All Kids Can, a commitment to helping children of all abilities be the best they can be. Like them on Facebook!

6 comments:

  1. There is some inaccurate info in the article.
    The 'miracle' drug that has been approved for CF is KALYDECO™ and it is for people with the G551D mutation.

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  2. http://jezebel.com/5914179/your-morning-cry-boy-with-cerebral-palsy-runs-race-with-a-little-help-from-his-friends


    You need to see this.

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  3. I don't have kids yet, but found out last fall that I'm a carrier of CF. Having a kid with CF is the outcome that totally frightens me, as well as the decisions I'm going to have to make if the man I want to have children with also turns out to be a carrier. It's nice to see articles that show people really can cope with it. Thanks for posting.

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  4. Thanks so much, Marla, I fixed that typo. Rachael, I am glad Brenda, Lucas and Hannah inspired you. And Julia, YES, and WOW. I'm posting about it tomorrow!

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  5. Thanks so much, Marla, I fixed that typo. Rachael, I am glad Brenda, Lucas and Hannah inspired you. And Julia, YES, and WOW. I'm posting about it tomorrow!

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  6. I am glad that CVS is doing a fundraising campaign that helps people with ALS and CF.My grandmother died of ALS 12 years ago and my best friend has CF so this hits home for me. Thanks for posting

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Thanks for sharing!