A Taste of Cystic Fibrosis

Today marks the fourth anniversary of when my daughter Madeline was diagnosed with cystic fibrosis. After her first CF anniversary, I wrote several detailed installments about our journey to finding a diagnosis, which you can find here, here, here, and here. As long as it is, that series is still missing the final installment. I was never able to write that last bit, partly because life got really busy and mostly because after a lot of emotional progress and only a year into our CF journey, I didn't feel like taking myself back to those raw emotions. Well, today still isn't the day I'm going to finish that series. But I do want to commemorate Madeline's fourth CF anniversary by offering you a peek into my own experience as we went through those first difficult but beautiful months of Madeline's life. 

I used to lick my baby. The first time I did it, she was only five and a half pounds—still several ounces shy of her birth weight at a time when she should have been well past it. I had just finished cleaning both of us up after yet another meal had ended with projectile vomiting, and the skin folds on her scrawny legs, just waiting to be filled out with fat, were a glaring reminder that my baby was simply not growing. That even though she ate and ate and ate, she was still smaller than she had been when she hollered her way into the world four weeks earlier.

Although many people dismissed my concerns, implying that I was an overly hyper new mother and telling me that all babies grow at their own rate, a few medical professionals had tentatively mentioned cystic fibrosis (or CF), a disease I was only mildly familiar with. I had known that it is a lung disease caused by thick, sticky mucus in the body, but not that it also causes severe digestive issues and makes it impossible to absorb fat and other nutrients without supplemental enzymes.

It was also news to me that people with cystic fibrosis often taste salty because they secrete too much salt when they sweat. Once I found out, I couldn’t resist testing it out. I could just lick my baby and put all this nonsense to rest.

That first morning, I picked her up, kissed her on the forehead, and let my lips linger for a moment, breathing in her sweet new baby smell before tentatively sticking out my tongue and touching her forehead. Relief washed over me: she wasn’t salty. At least, I didn’t think she was salty. I wasn’t in the habit of licking people, so maybe I just didn’t know what to expect. I licked her again. Still not salty.

For the next several weeks, as we visited the pediatrician weekly and tried everything to help her gain weight, I held this moment in the back of my mind as hope that my baby would, in fact, just suddenly start growing like other babies and not have anything seriously wrong at all. I couldn’t resist licking her now and then, just to see if something had changed. She was never salty.

When she was eight weeks old, we were transferred to a specialty nutrition clinic, where they were impressed that we had already eliminated all of the usual reasons for a newborn’s lack of growth. The doctors there ordered a sweat test to check for cystic fibrosis, which came back inconclusive because she had not sweat enough, and a fecal elastase test, which showed she needed to take enzymes to digest her food. As soon as we started giving the enzymes to her, she put on two pounds in two weeks.

One day I got my baby up from a nap and noticed that her hair was a bit damp around her temples. She had finally gotten fat enough to sweat. I hesitated only briefly before licking her: she was salty. Relief washed over me again. Finally, we could get a real answer as to why she was struggling and help her begin to thrive. It wasn’t a surprise when, at ten weeks old, her test results for cystic fibrosis came back positive.

Since that day, I have felt a huge range of emotions about her diagnosis with cystic fibrosis. I have cried a lot. I have felt overwhelmed a lot. But today, I am simply grateful that she is a funny, brilliant four-year-old girl who is just like any other four-year-old girl. She is active and strong and healthy. And although we spend a couple of hours each day doing treatments that keep her that way, I am grateful that when people find out she has cystic fibrosis, they most often say, “But she looks so normal! You would never know by looking at her that anything is wrong.”

No, you wouldn’t. Because she is normal. She just has this one little problem called CF.