If you are an individual that likes the why and how of something, tune in. The next series of blog posts are to help everyone understand what Maddox has, how it impacts him and what the future looks like right now. Don’t worry, I have a ton of Maddox pictures to help break up the science stuff. So buckle up and let’s have a crash course in CF.
Cystic Fibrosis or CF is a genetic mutation, essentially CF’ers are like the X Men. The mutation in their DNA is essentially telling the body to follow an incorrect path for their bodies. Their mutation basically is telling the body, ‘you need to make a ton of extra mucus and oh yea, by the way, it needs to be super thick and sticky’. The body follows this ‘error message’ and just makes a ton of mucus. The common impacts that CF’ers have is dealing with the lungs and gastrointestinal tracks.
Lungs. As we non CF’ers, our DNA states that we need to have mucus throughout our body as like a lubricant including our lungs. For CF’ers, the mucus is so thick and sticky that it stays in the lungs and covers up their lung cells. When these cells are covered with the mucus they essentially can’t get oxygen and end up dying. By not having oxygen and healthy cells, the capacity of their lung function decreases. The other concern is that the thick stuff can capture bacteria a lot easier and because it’s so sticky, it’s really hard to get bacteria out of their lungs which is the biggest concern when dealing with CF.
Today, Maddox does a vest treatment twice a day for 30 minutes each treatment. This vest essentially shakes the crap out of him, where it moves the mucus off of the cells in his lungs so he is able to cough the crud up to keep his lungs clear. During one of the treatments, usually at night, he also does two nebulizer treatments. One treatment essentially make the mucus thinner and the other is salt water to be able to provide more lubrication to help him cough everything up. If he’s sick, then we need to up his treatments to 3 times a day and might add more nebulizing treatments to help keep his lungs clear. His vest is hooked up to a compressor and essentially he has a 2 foot radius to move around right now. To help us get through his treatments, he is able to play on his iPad to pass the time.
Digestive Concerns. The thick sticky mucus also goes through their digestive system and impacts their pancreas the most. The easiest way to talk through it is that the body essentially doesn’t know what to do with this thick, sticky stuff and essentially flushes EVERYTHING through their system. So CF’ers have a really hard gaining weight as their body doesn’t absorb nutrients at all. Since Maddox was essentially 9 days old we were giving him enzymes on a spoonful of applesauce to help him absorb nutrients to gain weight. This has always been a struggle for us to be able to get the formula just right between his height and weight to ensure it’s not to strong but strong enough. He is and will need to continue the high calorie, fat packed diet until the next steps. The plan is for him to gain as much weight as he can with the goal to be at 50% of his growth progression for his age. We are struggling a little bit with this one. Thankfully – he loves to dip everything in ranch.
Now that we have scratched the surface on CF through the very basics, next blog post we will learn more specifics and next steps.