May is Cystic Fibrosis awareness month.
My daughter, Emily, has Cystic Fibrosis.
Diagnosed just a precious few days after birth, she has never, not been aware of CF. She does not know life without taking multiple medications every day and spending up to two hours every day wearing a vibrating vest to help clear mucus from her lungs. She is as aware of CF as she is of her own breathing, her own thoughts, her own being.
Today, as we participated in Great Strides, the Cystic Fibrosis Foundation’s biggest fundraiser at Milwaukee’s lakefront, I caught a glimmer of how deep her connection of self to CF is. The Emcee of the event said something like “Let’s find a cure for CF” and Emily, in a quiet moment between just the two of us looked up and said, “No.” I wasn’t surprised. She has a tendency to be Little Miss Contrary. But I asked, “Wouldn’t you like a world without enzymes and your vest every day?” “Yes,” she replied, “but without CF, I’m not me!”
At age 8, Emily’s awareness of her disease is changing. She has what some people might find a surprising amount of knowledge about CF. She can give a pretty good, age appropriate explanation of why she has CF and her sister doesn’t. She understands that in order for a person to get CF, they have to have received the “Bad” CF gene from their mom and their dad. (“Bad” is her word in this case. I have always been very careful to use “Changed” when talking about the gene mutation to my girls, to try to avoid a negative association. But, go figure, her brain translated it to “Bad” anyway.) She knows that she got two CF genes and her sister may have gotten just one, or none at all.
Emily can tell you that CF causes her body to make too much mucus. She doesn’t understand, yet, that it is a defect in the movement of sodium and chloride (salt) through the cells that causes the mucus production. She does know, however, that mucus is where infections like to grow, she must not only wear her vest to break up the mucus, she also needs to inhale two medications through a nebulizer, and use an inhaler, just like Dad does when his asthma and allergies make it hard for him to breathe. She also knows that even though she never misses a treatment, infections can still grow. Catching a common cold can bring on a “pulmonary exacerbation” (words she does not know) and land her in the hospital for a “tune up” (words she does know). Tune ups are 2-3 week hospital stays during which Emily gets IV antibiotics through a PICC line placed in her arm, and respiratory treatments four times daily. During her first grade year at school, she did this twice. Her doctors gave her a special 4-hour pass so that she could go to school to sing in her Christmas program, but, she missed a lot of fun End Of The Year activities during her next stay in May/June.
Emily knows that she MUST take her digestive enzymes whenever she eats. At school this year, her teacher simply provides them each day in a small pill case for Emily to keep in her desk. Emily is in charge of knowing how many she needs (it varies with fat content of the food, but we stick to a general “2 with snack, 4 with meal” rule, with a few notable exceptions.) The enzymes are a necessary part of meal time because the same salt imbalance that cause too much mucus in the lungs causes too little enzyme production in the pancreas. Emily can explain that the red and white pills she pops down four at a time do the job that the rest of our bodies do, while we aren’t even aware. In addition to her enzymes, there is a small buffet of pills that she takes at dinner time. There’s prevacid to combat reflux (a common malady in people with CF, due to incomplete digestion); a special fat soluable vitamin ABDEK tablet because her body cannot absorb these nutrients from food; an extra vitamin D tablet because her annual blood work always shows a deficiency; a probiotic to keep her intestinal tract healthy because God-forbid we have to live the C.diff nightmare again, and azithromycin on Mondays, Wednesdays and Fridays which she takes as an anti- inflammatory agent. Emily no longer needs to be asked to take her pills. She just gets the container out of the pantry and does what she needs to do. She just told me that she’d like a “pill schedule box” for her birthday so that she can load it up on Sunday with everything she needs for the week instead of opening each individual bottle every day. Some birthday present!
About 4-6 times a year, this daily regimen is joined by oral antibiotics, which she takes in the morning and evening. They cannot be taken with some of her other medications. We start a three week course of antibiotics as soon as we start to hear an increased cough or see other signs of a looming exacerbation. It is a necessary tool in the fight to stay out of the hospital. Unfortunately, spending so much time on antibiotics can wreak havoc with the gut. C. diff, mentioned in the last paragraph is an overgrowth of bad bacteria in the gut, made possible by the killing off (from antibiotic use) of the good bacteria that normally keeps it in check. C. diff is, well, for us it was hell. It made her a very, very sick baby for a pretty long time. We had a hospital stay when she was 15 months to try and get rid of it, but it wasn’t an easy fix.
There are other things Emily knows about her CF. Like how her feeding tube, which she got at 18 months and still has in her tummy, helps her get extra calories while she sleeps. Even with the help of digestive enzymes, a CF patient’s body has a hard time getting the necessary calories from a normal diet, and need to supplement. Emily is encouraged by her doctors to eat a high fat diet. She does, but the supplement through the tube has been a God-send. She is absolutely a normal height and weight, and her body has the energy to run, play, do karate and fight most infections. She can also explain why, even though she hates them, nightly sinus rinses are a necessity. She has had several sinus surgeries to clean out mucus and remove nasal polyps, commonly found in CF patients.
It’s a given that Emily is aware of and knowledgeable about her disease. She hasn’t been given another choice. None of us has. CF is a family thing. We all take turns sitting with Emily while she shakes in her vest; her big sister sometimes carries enzymes in her purse when we go out; we all take part in fundraising events like Great Strides and Climb for a Cure at the US Bank Building. I have found that connecting with other CF parents (just the parents, though as patients are forbidden by infection control policies from having close contact with each other) helps me to cope with the stress inherent in any chronic disease, so I am active in a Parent Advisory Board for the CF Clinic at Children’s Hospital, and I volunteer at several events each year.
But, we try and make sure that our lives are not ONLY about CF. Emily does all the stuff that any other 8 year old girl does. We’ve even begun to navigate the world of sleepovers…not an easy task when you have to take along a couple large pieces of medical equipment and a host of pills. She loves to play outside with the neighbors and she absolutely LOVES her karate class. Karate has captured her heart to the point that she re-named our Great Strides team this year. We became Emi’s Ninjas, and she designed our team t-shirt! Most of the time, I feel like we do a good job helping her be a balanced, well rounded kid, not just a kid with CF. When she expresses worry about someday not having CF, though, I have to wonder. Have we let her become too aware? Have we pushed her into seeing herself only as a patient? Does CF comprise too much of her life?
I hope that in time she will be able to understand that SHE will not cease to exist if we should be so lucky as to someday live in a world where CF does not. I want her to be able to look back on these years and see how CF has made her who she is becoming. She is responsible, passionate, determined and empathetic because of CF. But these qualities are only part of what make her the person that we love so dearly.
There is no cure for Cystic Fibrosis. Eventually, all CF patients succumb to the mucus that drowns their lungs and other organs, weakened by the stress of not receiving enough oxygen or nutrients begin to fail. Currently the median life expectancy is 42 years. It is our dearest hope that a cure will be found long before Emily reaches that point. Wouldn’t be wonderful if her children got to live in a world where people are only aware of CF as a disease that used to be?
To donate to the Cystic Fibrosis Foundation visit: www.fightcf.cff.com
To read the other posts in this series, click here.