Quarterly CF Update

The orange construction cones, the 172 bridge that everyone speeds on, the road in Green Bay that needs to be repaired but has beautiful flowers and we arrive at St Vincent Hospital in Green Bay. We say hi to the friendly receptionist at the desk and give our names. Maddox is super shy in the beginning but warms up to the ladies and says hi. We are handed his hospital wrist band and we go to the 5th floor. The elevator doors open and we are escorted to the new CF wing of the hospital.

When we say the new wing, it’s really an old floor of the hospital that’s been renamed the CF wing. The rooms look like they used to be double rooms when the hospital had a ton more patients and space was limited. We walk in and the room feels like a huge suite compared to the rooms we were used to. This room has a great view (aka a window as the last ones didn’t have that)!

The CF team greets us as we walk past the office and we go through the typical questionnaire that is asked of all CF patients with Billie our nurse. ‘How are things going?’ The usual question as we sit down. Personally, this is a loaded question that Billie asks. In my head, we are living in a zoo with a very strong willed two year old that is testing us on completing his treatments every time and we are working two full time jobs. We respond, ‘things are great’. Because they are, compared to others that have bigger struggles in their lives. We will survive the terrible twos 🙂

Maddox’s biggest struggle since he was born has been his weight gain. We dread and are anxious for him to hop on the scale as everything in your mind is weighing on this red number that pops up. Normally Tammy, our dietitian, is preaching to us that he needs to gain weight (here’s why). CF’ers that are higher than 50% of the normal growth curve for their age group have a better chance to fight off infection and to have a stronger/longer life span. Maddox has always been in the 10-30 percentile. It’s always been our anxiety and push that he just needs to eat. This past appointment, Tammy wasn’t there so we didn’t have our preach.  He steps off the scale and he is measured how tall he is.

Sherry, our pharmacist, walks in with our new PT and Suzanne sneaks in, so we have a full room (but that’s ok this time as we are in a suite ;)). Sherry asks and follows up on our medications and questions of insurance; everything is good. Then she drops the news. ‘I just wanted to let you guys know of a new drug on the market to keep an eye on.’ (she mentioned the name but I forgot and you’ll know why). This drug is in the final testing phases that it is ‘reprogramming’  genetic codes. To keep a long blog post short, the DNA in Maddox basically has a weak protein and it’s not strong enough to keep mucus off the cell and the cell dies. The drug is providing strength to the cell’s protein to help shake the mucus off. BIG NEWS! Sherry stated that patients that have been on this drug are seeing an increase in quality of life, their PFTS, are better, and gaining weight. Cool! So I asked, why are you sharing this and what does this mean for Maddox?

The drug is in the final stages of testing and is not available for kids Maddox’s age just yet. There are side effects of headaches and nausea but nothing that is worse that what they are already dealing with. Here’s the catch to keep us grounded. The medication is targeting the most common CF genetic mutation where the patient’s both mutations are the most common. When looking at Maddox’s DNA, he only had one copy of this mutation (one is common – one is rare). Sherry stated that there is testing being done on individuals that have only one copy (like Maddox) but that it’s in the early testing to make sure there are not negative side effects (medication could turn and make the symptoms worse). She told us not to get to excited just yet, but she had a big smile on her face. I’m hoping our future conversations continue to have this great of news.

We wrap up with all our other team members and Dr Pete walks in with a grin. ‘Wow guys, what has changed?’ Maddox had gained awesome weight even having strep for a month. We told him that he couldn’t get big and strong unless he ate a ton of steak! Dr Pete was impressed. He listened to his heart and lungs and from what he could hear, his lungs sounded great! Maddox and Dr Pete have a love/hate relationship as Dr Pete likes to test Maddox to see his responses and Maddox gets annoyed (all in good fun).

The only concern/apprehension we have yet are the culture tests that come back from Maddox’s spit tests. He hates this test, it’s just a cotton swab to the back of the throat but fights it every time. The swab is sent down to Froedert in Milwaukee for the final testing and they are checking all the bacteria that is growing in his lungs. Last time I had a panic attack. Up until the last test, his tests have always come back clear, but in spring he had a bunch of stuff on the report. I quickly called Billie, the nurse, and asked what was up. She stated that the stuff that showed up was the cold/flu that everyone had and no concerns from the CF perspective. If there was a concern, they would call me. At least she has my back, but man that scared the crap out of me. So, we wait until the next results are completed (about 2 weeks).

In the meantime, we rock out our vest treatments and appreciate visitors that stop in to say hi, like Lance from California. And always a treat when Ollie can stop over.

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