Pregnancy with my daughter Maggie went so smoothly I feel guilty talking about it. Besides some mild morning sickness and a flash of heartburn here and there, we were both so incredibly comfortable with her in the womb that she was still in there two weeks beyond her due date. But that’s where the comfort and ease ended. Once labor started, life got rough. Maggie and I both developed fevers and when she was finally born 16 hours later, she was taken to the Neonatal Intensive Care Unit for evaluation. Doctors tested her for anything that may have caused her fever, but they never found the source. After four days, she was sent home with a clean bill of health. But the relief and promise of good health was short-lived. Just two days later our lives were forever changed by four painful words from the pediatrician, “She has cystic fibrosis.”
Unlike my husband, I had never heard of cystic fibrosis (CF). I didn’t understand the words. I had no idea what it meant for my daughter or how drastically our lives had just been altered. I prayed repeatedly for the doctors to be wrong, for the tests to be wrong. But they weren’t. At just 6-days-old, my daughter’s life was given an expiration date and I was left wondering why.
Maggie is one of only 70,000 people in the world living with CF, a life-threatening, progressive, unpredictable, and incurable genetic disease. Despite the fact that only 30,000 Americans have CF, 1 in 33 Americans is a carrier of the gene and in 1 in 400 couples, both partners are carriers. Of those couples, there is a 25% chance any child they create will have CF. Maggie has only a 50/50 chance of living to see her 37th birthday.
Within a few days of Maggie’s diagnosis, we were visiting specialists and learning how to take care of our daughter. Her daily routine would include hands-on chest percussion therapy and pills to help her digest food. But that was just the beginning. Over time, we would add nebulized medications, special vitamins, inhalers, and a $16,000 machine to help clear her lungs of mucus.
Now, Maggie is almost four years old and already has mild lung disease. To keep her as healthy as possible and alive, each day she does her treatments without fail. As soon as she wakes up, she uses an inhaler of Albuterol to open her airways. Then she does a 10-minute nebulized medication called Pulmozyme to break up the mucus in her lungs. This is followed by 20-30 minutes of The Vest, an airway clearance device that vibrates, shaking her lungs so she can cough any mucus out. Throughout her day she takes over 16 enzyme pills to help her absorb food (her pancreas doesn’t work) so that she doesn’t die of starvation, as well as numerous medications and vitamins to help her stay healthy. Before bed, she uses the Albuterol inhaler and does another 20-30 minutes of The Vest. When she’s sick, she does nebulized Albuterol and The Vest four times a day for 20-30 minutes. None of this guarantees that she will stay out of the hospital, won’t need a lung transplant, or will even live to see age 37. But it does help. In the 1950s, children with CF could only expect to live to age eight. So while the treatments are inconvenient and Maggie doesn’t always want to do them, they are a blessing because they are literally keeping her alive. As she ages, more will be added to her routine, because CF is sneaky and progressive, and can take a person from healthy to dying with one respiratory illness.
Until a cure comes, every cough is frightening and ever sneeze makes us jump. While we do have fear for Maggie’s future, we also have hope, because the CF Foundation and Vertex pharmaceuticals are working on the next best thing to a cure – medications which target the underlying cause of CF, rather than therapies to treat the symptoms. These drugs, which are still in trials, hold the possibility of a normal, healthier life span for children like Maggie. And isn’t a long, healthy life all a parent really wants for their child?