CF stands for Cystic Fibrosis.
By definition from the Mayo Clinic – Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body. Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery.
-This is not a contagious disease or virus. It is a genetic disease inherited from both parents. Both parents have to have the gene mutation for CF to even begin to be present.
-There is a 1-in-4 chance, a small 25% chance, of a child being born with CF if both parents have the gene mutations.
-There is no cure…currently.
-When you kiss a CFers skin, it is salty.
-There are medicines that help aide in day to day life of CFers, but even with a lung transplant, the CFer still has CF. It doesn’t just go away.
-Many CFers complete hours worth of breathing and chest physical therapy treatments a day (or they wear a vest that shakes them vigorously to move the mucus).
-CF patients are not supposed to come in contact with each other making this a very lonely disease as CFers get older. They could potentially pass a bug back and forth from their lungs that would then mutate further and could potentially mean a 14-day, or longer, hospital stay.
-Even on clinic visit days, we are sent back to a room right away to avoid possible exposure and all the people that make up the CF team come into the room gowned, gloved, and masked. This is to reduce the possibility of passing germs from one CF room to the next even by clothing. Hospital stays mean being confined to one room the entire time. Even cleaning staff has to gown, glove, and mask to come in the room.
-Speaking of CF teams…each CFer has a team that is made up of a group of pulmonologists, nurses, dieticians, social workers, respiratory therapists, and child life specialists that are seen monthly. Most CF monthly appointments can take up to 3 hours due to all of the people to be seen and labs to be done. There are sometimes extras on the team such as GI since CF impacts the GI tract as well.
-Cystic Fibrosis is often called “Sixty Five Roses Disease.” Back in 1965, a woman by the name of Mary Weiss had three young sons with cystic fibrosis. After learning about their diagnosis, she began volunteering at the CF Foundation by calling groups for financial support for the foundation. Her 4-year-old son overheard her calls and said, “I know what you’re working for.” She asked what he thought because she knew he did not understand his diagnosis, nor whom she was working for, and that is when he said, “You are working for 65 roses.” It’s a hard name for kids to say, so 65 roses is a term that is used a lot in the CF world. June 5th is 65 Roses Day.
-Scarring of the lungs is not abnormal for CFers, nor is lots of coughing.
-Respiratory illnesses can cause irreparable damage to the lungs of a CFer.
-Illnesses that take the average human days to get over could take a CFer weeks to get over.
-If someone with CF or a parent of someone with CF declines invites to places or large crowds, don’t take it personally. It’s usually for health reasons.
What does a day in the life look like for Brooks as far as medicine goes?
He currently takes a modulator twice a day. This helps to make one of his gene mutations function as normal, or close to normal. As the doctors have described it, “On the CFTR gene there is a little “door” that is supposed to open and close to let liquid in and out. CFers CFTR gene doesn’t do this. The modulator helps the “door” to open.”
A lot of CFers have really bad reflux, as do most babies, theirs just happens to usually last for life. So he takes two different types of medicines for this, plus a GI medicine to keep the belly pains low at night.
Most CFers are pancreatic insufficient, meaning they do not absorb the nutrients from food that they need. To aide in this, they take enzymes before every single thing they eat every day. These enzymes, help the pancreas to absorb the needed nutrients to keep the body alive and going. Otherwise, a CFer might be severely malnourished. That was actually one of the first indicators of CF many years ago before much was known about CF! Speaking of food…a high fat diet is key! Most CFers have a hard time absorbing certain vitamins as well, so liquid vitamins are a must each day.
Brooks does breathing treatments twice a day, more when he is sick. Right now each treatment can take anywhere from 30 minutes to a hour, depending on cooperation at the time and how many medicines we are having to administer. We do “hand CPT” where we cup our hands and pat certain areas of his back and chest to loosen the mucus that settles throughout the day, but that the inhaled medicines help to loosen. We hope to be in line for a vest soon so that he can do his vest treatments and breathing treatments at the same time.
Honestly, we have it pretty easy compared to other CF patients and families. I know I am forgetting a ton…we are learning every single day something new about cystic fibrosis. These patients are warriors in all senses of the word!
Visit the CF Foundation Website for more information: https://www.cff.org/
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