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.
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