Typically, when we think of progress, it’s in a positive light, as moving forward or making headway.
“As the year progressed, the quality of her gymnastics skills improved.”
“He made tremendous progress with physical therapy.”
This month, as test results have trickled in regarding our boys’ most recent appointments at the University of Minnesota Children’s Hospital, we are reminded that CF is a progressive disease. Seeing words like “insufficient”, and “decrease”, tell me that this kind of progress is going in the wrong direction.
Here is what we learned:
Carson cheers his brother on during pulmonary function tests.
I'll start with Carson: His PFT's (pulmonary function test) were lower and he struggled some during the assessment. I’ve listened to my boys perform this test dozens of times, over the past four years. I’ve literally memorized the sound they make when they reach their full potential, blowing air into the mouth piece as hard as they can. Call it “mom-sense”, but I can hear when they’ve reached their capacity. This time, Carson sounded different to me and I knew there was a change without even looking at the results. Disappointedly, his numbers were the same as when he was first brought into the clinic in 2013, at diagnosis. It’s discouraging because I see the time he puts into his airway clearance therapy every day.
Carson thinks his spine looks like a gummy worm!
We're trying some new and stronger steroids and will reevaluate in December. Also, his x-rays showed us a few things. Scattered “bronchial cuffing with hilar fullness”, meaning excess fluid or mucus buildup in the small airway passages of the lungs, with shadows. We also discovered a dextrocurvative of the superior thoracic spine. Basically, this means that his spine curves to the right, in the top portion of his middle back. (Thank goodness for Google!) Patients with cystic fibrosis have an increased risk for developing kyphosis and scoliosis. Hopefully, physical therapy can help correct this and we can avoid surgical intervention. We also want to take care of the discomfort that he feels every day.
Anders: His GTT (Glucose tolerance test) came back elevated, showing he is more susceptible to diabetes. CF-related diabetes is common in CF patients. This is the second time he's had results like this. We'll need to watch sugar intake and monitor.
So far, the boys have been pancreatic sufficient and we feel so grateful. (Up to 90% of CF patients are pancreatic insufficient.) Unfortunately, Anders' most recent Pancreas Elastase test showed mild to moderate insufficiency. I’ll admit this came as a shock to me. During one appointment at least a year ago, I ventured to ask our CF care provider what her thoughts were on the boys remaining pancreatic sufficient. She felt confident that they would remain stable in this area. But our boys have rare mutations and it’s unfamiliar just how their disease will progress. There are only 1 or 2 cases reported like theirs, in the world. So we have nothing to compare them to.
Anders will be taking approximately 140 pills a week.
Being pancreatic insufficient means the pancreas loses its ability to secrete digestive enzymes. Patients become unable to digest food properly, which leads to the malabsorption of nutrients, or even malnutrition. Thus, we are entering the wonderful world of enzymes. Anders will need to take 3-4 pills before every single meal, and 1-2 enzymes with any kind of snack. I did the math, factoring in the medication he currently takes and that will equate to approximately 20 pills a day or 140 pills a week. Typically, those with CF are diagnosed during infancy or early childhood. And for most, this enzyme regimen is all they’ve ever known. Anders will be adapting to it at the age of 10. My normally upbeat and positive boy didn’t take this news very well. I brought him to his favorite ice cream and candy shop and explained the upcoming change. He reacted with complete disappointment-and rightly so. This summer, Anders has been maturing in his understanding of what a life-threatening disease with no cure actually means. He’s been asking us really tough questions too.
“Will I have to do this Vest every single day for the rest of my life?”
“How long am I going to live?”
“Am I going to die from CF?”
We’ve been honest with him as we discuss, telling him the truth:
“We don’t know. But really, none of us knows how long we have or how we will die. But we can be sure that God is always with us and has a plan for our lives.”
We also tell him how close doctors and scientists are coming to an actual CURE. Now, this is where I prefer to see the word PROGRESS when it comes to Cystic Fibrosis. It’s not just wishful thinking either. I truly believe we will see a cure for this devastating and multi-faceted disease in our lifetime. (If you’d like to learn more about research and developing new treatments, visit www.cff.org.) I encouraged Anders, telling him about all of the amazing drugs that are becoming available, and fundraisers that support this research. People are working hard- for him!
Thank you to each and every one who support us as we LIVE with CF and progress towards a cure.
When you receive bad news, the world continues moving forward, even when you wish it would just stand still so you can at least catch your breath. But it doesn’t. I realize that no matter what comes our way, we only have two choices in life. We can give up, or we can put one foot in front of the other and keep moving forward. With God, we will press on.
Philippians 3:14
