My son, Eli, 2, was born with cystic fibrosis, a rare and deadly genetic condition that robs a person of the ability to breathe over time.
Doesn’t that just sound like the pits, the absolute pits? Don’t go throwing yourself off a cliff. Back it on up Thelma & Louise.
Below I’m going to let the Cystic Fibrosis Foundation explain what the disease is. I’m gonna weigh in, too.
Cystic fibrosis (CF) is a life-threatening genetic disease that primarily affects the lungs and digestive system. An estimated 30,000 children and adults in the United States (70,000 worldwide) have CF.
In people with CF, a defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
Clogs the lungs and leads to life-threatening lung infections.
Obstructs the pancreas and stops natural enzymes from helping the body break down food and absorb vital nutrients.
In real life, that means my family is wellness-oriented. We wash our hands a lot. He wears a shaking vest an hour each day to get the problematic mucus moving on out of his lungs every day, but he doesn’t sound mucus-y. When Eli gets a cold, because kids do, it sometimes goes away on its own. He wears a vest for an hour and a half each day when he’s getting sick. Even though a cold is a virus, the bacteria that like to stick in his lungs’ mucus have a bacteria party and Eli’s cough may get worse. If it does get worse or doesn’t seem to get better, after five or six days of coughing, he goes on antibiotics. When he’s older, he will probably need more powerful, inhaled antibiotics, because his condition will resemble something like a chronic bronchitis and the infections will get more complicated.
A few more day-to-day things: I’ll never let Eli drink out of a drinking fountain. There are certain strains of bacteria we try to avoid because these can cause lung damage in those with CF. Pseudomonas strains are found everywhere, like in the tap of your bathtub, like in drinking fountains. We don’t have bath toys that squirt because bacteria like pseudomonas live there, along with mold and other yucky stuff. We keep Eli away from laundry or anything damp that’s just sitting around in order to avoid the bacteria. That being said, it’s such a common thing it is actually impossible to avoid, and eventually, he will likely get an infection. We do what we can to prevent or delay this type of thing.
Eli also takes digestive enzymes that help him absorb fat. These are teeny white beads that come in capsules. He takes them in applesauce before meals. The applesauce assures they will travel more safely to the part of the digestive process where they are needed. Without fat, you won’t grow. If you don’t grow, you are subject to all sorts of assaults on your health. Without enzymes, Eli would become weak. He would starve or get sick. That’s why it’s important we give him the enzymes before meals.
Like some babies with cystic fibrosis, Eli was born with a blocked bowel, a condition caused by his thick sticky mucus. He received emergency surgery to unplug his guts within his first day of life. There is a scar on his tummy now that’s like a smile. He’s doing just fine.
Another thing — we live in the present — well, that’s the goal. I don’t think hard about what “could be,” meaning bad things that may or may not happen to me, you, Eli or anyone. I have lots of emotions and feelings regarding CF. I picture these emotions running around in the landscape of my mind. For some reason, negativity is a cartoon cat burglar. He sneaks around, cracks a safe I keep on a shelf over there in the corner. Out comes guilt. Then worry comes out to play, that little menace. Useless troublemakers. I K.O. them old school Streetfighter-style back into the vault, lock it and carry on. It helps me to think like this. If you can’t control your worry, your guilt, your negativity, if they are running all over your mind, wild, all the time — or even if you get the feeling they are holding secret underground meetings to launch an assault — seek professional help now and don’t be ashamed. Chronic disease takes a huge mental toll not just on those who have it, but their caregivers. Our mental health is just as important as our physical well being. It’s all tied together. Don’t ignore the way you feel and don’t be ashamed to ask for help from friends, from therapists, from medication. Life’s tough and we can only be so strong on our own.
I’m getting off track. More about CF. You may want to know more about what it is. You may want to know: Is my son going to die?
Yes, he is. That’s because, everybody dies. You’re born, you live, and at some point, your number’s up.
More about CF:
In the 1950s, few children with CF lived to attend elementary school. Since then, tremendous progress in understanding and treating CF has led to dramatic improvements in the length and quality of life for those with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
That is true. It’s important we stick to our treatments, because to some extent, we can fight this.
Here is more on the symptoms of CF:
People with CF can have a variety of symptoms, including:
Very salty-tasting skin
Persistent coughing, at times with phlegm
Frequent lung infections
Wheezing or shortness of breath
Poor growth and slow weight gain, in spite of a good appetite
Frequent greasy, bulky stools or difficulty in bowel movements
When I kiss my son on the head, especially after he’s been outside running around, he tastes like a Lays potato chip. At 2, he does not have a persistent cough, wheezing or shortness of breath, or poor growth and slow weight gain. His poo is all good so long as he gets his enzymes and the dose is right. If it’s not right his poo is mushy and smells kind of like, well, road kill. He eats 1,900 calories a day at 2 – so he poos more than your average tot.
I hope that helps you understand CF.
I just can’t seem to shut up, so if not:
Here is a multimedia “Day in the life” presentation.
Here’s a day I was feeling really sad about a cough.
Here is a day I was like, my son’s not sick. He’s siiiick. Like, rad.
Here’s the time we decided to drive to the northwest tip of the Oklahoma panhandle, because, why not? My family likes to live life and we have vowed not to let CF get in the way of our lives or Eli’s childhood.
Cystic fibrosis is a mystery that unfurls over time. It develops in childhood. We have no idea how sick my son will get. Some people with CF get much sicker than others, earlier. There are cases where there is little rhyme or reason to it. A child could have great parents doing the best they can and that child could still get hit hard with infections and hospitalizations. In some people, problems are with digestion much more so than the lungs. Other parents do little, or almost nothing and a child with cystic fibrosis is always sick. I don’t understand that, because doing the care 100 percent of the time will help reduce the odds that a person with CF will die young. Love your child? Do the care. Any way, none of us know what’s around the corner and all we can be is the best we can be. Maybe medicine will advance to the point my son won’t ever get sick at all.
I want to encourage anyone stopping in to seek out a variety of informed opinions on CF if you’ve heard something negative or alarming. Not saying it’s all sunshine, boxes of puppies and children skipping around the Maypole, but there is a lot of outdated information floating around. Google is not your friend. Your anti-vaccine, anti-medicine coo coo bird friend is not your friend. Look at the science. Talk to doctors. Read studies. Disease care is changing so fast and getting better all the time. The best place to get info is from the CF Foundation or a lung doctor, specifically, a pulmonologist or pediatric pulmonologist. Talk to multiple lung doctors. I’m a journalist. I’m not into one-source stories.
Speaking of doctors — if you have CF, if your child has CF, or if anyone you know has a chronic disease, heed this advice: Ask questions. Ask why. Demand to understand. Trust your instincts. Good doctors will welcome the chance to explain. Poor ones will weed themselves out in the way they respond — or don’t respond. Not responsive? Red flag. Does that nurse seem like, well, kind of a dip? Tell the charge nurse you want a new one — now. Does this nurse seem on point — I mean great, detail-oriented and caring? Tell the charge nurse you want him/her again. Pay attention. Would you let a bank teller run all over you? Hell no. That’s your money, and they are in a customer service position. Don’t think of doctors, nurses and health care differently than that. They work for us. They are providing healthcare, one of the most expensive products you could ever ask for. And it’s not just about money – it’s life! Demand better for your life, for your child or loved one’s life. Demand the best.
We’re all going to be OK.