Our first visit to the CF Clinic was an educational visit. My parents came with us, and we learned about Cystic Fibrosis, and what it would mean for Monkey and our family. We found out that Monkey's 2 mutations are fairly uncommon, and the combination of the two was very rare, therefore they did not have a lot to go on as far as what to expect. We were very encouraged to discover that Monkey is pancreas sufficient, which means that his pancreas functions normally, unlike most people with CF. He would not have to take the tens of enzyme pills a day in order to absorb the nutrients in the food he eats that most people with CF have to take. This was a huge relief. They do not know for sure that his pancreas will continue to function throughout his life, but they believe that it will.
So, keeping Monkey healthy was going to be mainly about keeping his lungs healthy. We learned about the different medications he will take and about airway clearance. They taught us how to do Chest Physiotherapy (CPT). This is a way of giving percussions, or pounding, on specific spots around his chest, back, and sides to loosen the mucus in his lungs in order for him to be able to cough it out. This is done with a small rubber percussor. There are 12 different locations on his body that need to be percussed for 2 minutes each. This is done 2-4 times a day. I used to joke that I had to beat my baby everyday per doctor's orders. In all reality, giving CPT was incredibly difficult and emotionally draining. Monkey was 4 weeks old when we started giving him these treatments. He would scream and cry the entire time. He was so tiny and vulnerable, and it broke my heart to have to continue to pound on him as he screamed. Every bit of me wanted to pick him up and comfort him , but I knew that to keep him healthy, I had to finish the treatment.
These are Monkey's percussors. The small pink one was his first, and when he got a little bigger, we got the cream one.
This is Monkey having his CPT treatment when he was about 4 months old.
Eventually, when he was 10 months old, Monkey was big enough to fit into an airway clearance vest, which is attached to a machine and shakes vigorously in order to break up the mucus in his lungs. This was much easier for everyone than doing the CPT by hand. He was able to sit upright, and we could read books or play while he was getting his treatment, he was even able to move around a little bit. This is how he gets his airway clearance treatments still. When he turned 2, his settings on his machine were changed from 24 minutes to 30 minutes. It was also set to shake a bit harder. As he gets older, his settings will continue to change and shake harder to break up as much of that nasty mucus as possible.
This is Monkey having a vest treatment at 10 months old. This is one of the first few times he wore the vest.
In addition to the airway clearance, Monkey also takes 2 medications through a nebulizor. Albuterol is taken before every airway clearance, and is a bronchiodialator, which helps to open things up so he can cough out the mucus easier. This is a medication common to people with asthma and other respiratory illnesses. He also takes Pulmozyme once a day. This is a mucus thinner and is a medication specific to CF. It helps to keep his mucus from getting overly thick and sticky. He also takes Flovent through an inhaler twice a day. This is another medication common for asthma, it is a corticosteroid.
In all, his medications and treatments take a little over an hour a day when he is healthy, and up to 2 and a half hours a day when he has a cold, cough, or any other respiratory illness. People often tell me they don't know how I do it. My response is always, I don't know either, but if it were your kid, you'd do it to.