Welcome to Cold and Flu Season

Sometime in December Anna got a cold-- just like the rest of the kids. She began to get a cough which didn't go away. As time progressed, so did her breathing rate and "rattles" in her chest. I think the progression was so gradual it was hard for us to notice how bad it was getting. On one of our visits to the CF center, Dr Akhter noticed she was having "retractions" in her ribs and above her stomach. She seemed to be having them in her neck as well. He explained that this was an indication that she was struggling to breathe. At the time her oxygen saturation was good, but the cough persisted and her lungs were congested. Anna was vomiting a large amount of her food and it seemed to contain quite a lot of mucus.

We were becoming more and more alarmed as we realized Anna was not getting better. We were already doing therapy (nebulizer & chest percussion for 20 min) three times a day at home.  (I say "we" but it is really Wende who is the hero in this story. I get to leave the house and hang out in the world of grown-ups all day at work. She is the one taking care of EVERYTHING-- using her supernatural gift to organize, educate and provide a sense of order and foundation amid the chaos of our infant and the other 4 children whom I affectionately refer to as Madame Curie and the Three Stooges. While Padre Pio may have had the supernatural gift of bilocation, Wende has the gift of being organized, and as St. Josemaria Escriva said "being organized multiplies your time." But I digress...) Anna had been on three different rounds of oral antibiotics for the last 6 weeks. Was this "just the CF" symptoms starting to show up or was this an infection? If it was viral, why was it being aggressively treated with antibiotics? Why wasn't she getting better? Dr Akhter informed us that he would like to have her hospitalized, put on IV antibiotics and receive more intensive treatment. Wende and I initially said no and wanted to give it another week. We were caught off guard, not to mention that there are logistics involved concerning the other 4 kids.

 We were told if Anna was not significantly better by the following Thursday she would be admitted into the hospital, so we should "have our bags packed" when we came follow-up visit. My feelings of alarm had turned into fear. 



That next week was spooky. The blizzard hit, I stayed home from work for a few days. Anna's labored breathing continued and her chest and neck retractions were as plain as day. By the time Thursday rolled around they had finally plowed our street (it took 2 days after the snow stopped) and we could get out of our driveway. Wende and I were both mentally prepared to have her hospitalized. We had also arranged the logistics for the other kids.

Getting Anna into the hospital ended up being an all-day event. When we arrived at Hope Children's Hospital to meet the CF team before she was to be admitted, we were told that they couldn't admit her. There were no beds available. Probably not that day. Maybe not even Friday. I couldn't believe we were being sent home. We went back to pick up the kids and waited at my mom's house for a few hours. I called the "bed coordinator" and pleaded with her if there was anything she could do to get us in. She informed me that there were 8 other children ahead of us waiting for a bed. It was late, so we drove home to Joliet, and as we were pulling in to the driveway, I received a call that they could put Anna in the ICU if we turned around and came in immediately. We turned around and finally got her in.

Anna was at Hope Children's Hospital for a week. Wende stayed with her and did not set foot out of the hospital that whole time. Our doctor showed us her chest xrays compared to her normal, baseline films taken just 1 month prior. I was shocked at how dramatically different the two films were. The radiologist who read her films called it pneumonia, and I suppose that is a way to describe what her symptoms. And evidently that is an academic debate and a matter of semantics as our pulmonologist said  it is not "pneumonia," it is a "pulmonary exacerbation."  "P.E. is the code-word now."    ...got it.

So, to put the pieces of the puzzle together: the cold or virus that Anna had evidently caused her cystic fibrosis mucus-thickening-can't-get-it-out-of-you mechanism to kick-in high-gear. Dr Akhter told Wende that he had not seen such severe CF symptoms in someone so little. That was tough to hear. You never like to hear a specialist tell you news like that. CF treatment and management is supposed to be a long-term game plan, not a "4th quarter and the team is down 14 points" game plan.

Anna's therapy was upped to 4 times a day. She was put on Pulmozyme, which breaks down mucus and helps people with CF cough it out-- but most CF patients aren't on this medicine until later in life. She was on IV antibiotics even though her cultures did not show anything bacterial.



Worst case scenario would be that she came up positive for a type of Pseudomonas -- a bacterium that people with CF get-- the proverbial "CF Bug" that never leaves once they get it. God Be Praised they didn't find that.

Over the week her oxygen saturation improved and her respiration rate became more normal. The chest retractions improved too. Except for the nurses who changed her IVs, Anna greeted everyone with a smile. This is not the Proud Pappa making overly sentimental platitudes-- she really smiled. On more than one occasion Wende was told by the staff that it was "nice to have a happy baby." I wish Anna had words. In her own little way she evidently brightened the day for a few others.

Anna was discharged from the hospital last Thursday and we see our CF team tomorrow. She'll have a follow-up chest xray in a couple weeks to see how her lungs are looking.  Wende told me today that she could barely notice the chest retractions. Thank you everyone for your prayers. Please keep it up.
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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!