As previously mentioned, our new little guy was diagnosed with cystic fibrosis several weeks ago. Today, I want to share with you a little about this chronic disease because…
1) I recognized the name of the disease, but had no idea what it actually was prior to finding out about his diagnosis, and
2) I believe that awareness is vital for any disease.
According to the Cystic Fibrosis Foundation, the basic definition of cystic fibrosis (CF) is:
“Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening lung infections; and
- obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.”
“Beyond” is the key word for our son, as far as I’m concerned. Once I got past the initial shock and devastation of his diagnosis, I moved into a state of determination. Like anyone who is faced with a difficult challenge (and aren’t we all in some way?), I truly believe that hope and optimism are imperative to conquering that challenge. No doubt, we have a long and bumpy, sometimes emotional and frustrating road ahead of us, but we are taking it one day at a time and using the knowledge of our wonderful team of CF doctors to keep our boy healthy and happy.
Some answers to frequent questions that I have been asked (or that I have asked myself!) so far include:
- We found out that he had CF through his newborn screening. I’m not sure if every state now tests for CF this way, but they absolutely should! As hard as it was to learn the bad news right away, it is MUCH better to find out ASAP than to let a child get behind on gaining weight. Good weight gain means stronger lungs, so the earlier the diagnosis the better!
- Since our son has CF, that means that my husband and I are both carriers of the disease. Every conception that we have together, there is a 1 in 4 chance of having a child with CF, a 1 in 4 chance of having a “normal” child, and a 2 in 4 chance of having a child who is a carrier like us. The only way to know if you are a carrier of CF is to be tested, as there are no symptoms. There is no history of CF in either of our families, so neither of us knew that we were a carrier prior to his diagnosis. Our oldest son was recently tested and was negative for CF, so he can be tested to see if he is a carrier when he is older.
- He does not need to be a “kept in a bubble,” but he and everyone he is close to should use good hand-washing and hygiene etiquette, since he is more prone to lung infections. If you have a cold, admire his cute little face from a few feet away please!
- Exercise is vital for improvement of lung quality for our son and others with CF, so we will be starting at a young age with lots of physical activity. Good thing we are a family who loves sports! I have found a wonderful blog entitled Run Sickboy Run by Ronnie and Mandi Sharpe. Ronnie is truly inspiring as a CF husband, father, and an advocate for the disease. He has an infectious positive outlook on CF and life in general.
- There are many medications that our son will need in his lifetime. Currently, he takes enzymes before he eats anything (just breast milk for now!), and since an infant can’t swallow a pill, we pour the enzymes from inside the capsules onto a spoon of applesauce. He takes it surprisingly well, although he does occasionally gag. He will have to take enzymes before he eats, every time he eats, for his whole life, so he I’m glad we are starting early so that he won’t know any different! He already associates that applesauce=Mommy’s milk, and he gets very excited (and stops crying) as soon as he sees us with the spoon. Thanks, “Pavlov’s dog!” Since his body has difficulty digesting fat and protein, and certain vitamins need these nutrients to be absorbed, he also takes a special multi-vitamin that is water-soluble. Also, we give him 1/8 tsp of table salt per day, to make up for the extra salt that he loses. We divide it between two breast milk bottles, and he doesn’t seem to know it’s in there!
- Finally (for now), he also gets a treatment for his lungs called CPT (chest percussion therapy) or “pounding” twice per day. This is to help break up the mucus that gets trapped in the airways of CFers. It takes us about 15 minutes to complete each session, and most of the time it puts him to sleep.
So far, we are doing great with all of this. His big brother is a huge help to us, and he loves his little brother so much! While we are embracing his CF, and we never want him to feel ashamed of it, we also want him to feel normal. First, he is our son, a brother, a normal boy with hopes and dreams and fears like the rest of us. Second, he has CF, and that just means that his body requires extra care and attention to function properly.
I will update occasionally with CF topics and to let you all know how he is doing. If you or someone you know has CF, please feel free to follow our journey and let us follow yours! One thing I have learned quickly is that the community of those with CF and their families is a strong one with a wonderful, supportive, and optimistic bond. I’ve never known anything like it, and for that part of this disease, I am grateful, because not all diseases have such amazing support, research, and hope for a cure that appears extremely obtainable.
Feel free to comment with any additional questions or information! Keep in mind that I am new to this, and cystic fibrosis is an extremely complex disease with a lot to learn about it. As with any disease, you should always consult your own healthcare professionals for the best advice! A stay-at-home mama can only know so much! Thanks for taking interest in our journey!