Our Introduction to CF

It was the end of October. Anna was two weeks old and we were still getting used to having a little baby around the house again. Wende and I had just put the other four kids to bed and were enjoying that blessed peace and silence that ensues once the kids finally fall asleep.  The phone rang. It was Dr Reicherts, our pediatrician and I was alarmed by the fact that he was calling us so late in the evening for no apparent reason. He sounded very concerned. He was calling to inform us that the State of Illinois now screens all newborns for Cystic Fibrosis, and Anna had two genetic markers for the disease: F508 and G542X. He had just received the fax from the State and was calling us to let us know the results. He recommended we find a Cystic Fibrosis Center and set up an appointment for follow-up testing.  I hung up the phone and shared with Wende what the doctor had just told me. I was in disbelief. Surely they were wrong. Anna was not sick.

The next day I spoke with Dr Akhter, a pediatric pulmonologist. His office made arrangements for Anna to have a Sweat Test two days later, which would determine if Anna really had Cystic Fibrosis (CF). We had an appointment to meet Dr Akhter and his team after the sweat test to discuss the results. I was looking forward to having some peace of mind knowing that though she may have the gene, she did not have the disease, and we could put this little scary episode behind us.

We went to Hope Children's Hospital in Oak Lawn and Anna had her sweat test. We met with Dr Akhter and his team at the Cystic Fibrosis Center to discuss the results. The tests was strongly positive for CF. My disbelief had turned into shock: that sudden alarming type of shock that takes your breath away, like how it feels having jumped into freezing cold water and you can't get out fast enough. They did another test to check her pancreas which showed she was producing almost no digestive enzymes whatsoever. She was already in a state of malabsorbtion and not getting sufficient nutrition.

The doctor went on to explain that Anna will have "typical" CF, which meant that we could presume she will manifest lung and pancreas symptoms, but there was no way of knowing how severe her symptoms would be. The doctor and the CF team presented Anna's diagnosis in a hopeful, positive manner, but neither Wende or I were able to appreciate what he was saying. He was very optimistic about the future of people with CF in light of the early detection in newborns and new treatments on the horizon--  but it still felt like a death sentence for Anna. It was going to take some time for Wende and I to accept the bomb that was just dropped on us.

We met with the entire CF team every Thursday. The team consisted of Dr Akhter, his nurse Jean, a nutritionist, respiratory therapist and a social worker. They monitored her status and began to give us instructions for her daily treatment.  First, we began giving pancreatic enzymes with every feeding so her body could absorb the nutrients in her food.

This was achieved by opening a capsule of Creon and dumping the contents into Anna's mouth immediately before feeding. The goal was to have her in the 75th percentile for weight of children her age. I wanted to see fat baby thighs. Anna also began to receive  a bottle at night with salt and fat-soluble vitamins. We were taught how to daily chest percussion as a means to keep her airway as clear of mucus as possible. Several weeks later we began giving her a daily dose of albuterol medication using a nebulizer, followed by CPT(just like in this video clip). Over the course of a few weeks we began to get into the routine.


Anna is now 4 months old and is showing her personality already. She likes to talk and she is extremely generous with her smiles!