son, Bryce (BAM), was born in July 2012 and diagnosed with cystic
fibrosis approximately five weeks later. He is now a happy, intelligent,
and energetic six-and-a-half-year-old that weighs around 52 lbs. and is
just shy of four feet tall! When he first visited the Cystic Fibrosis
Clinic in September of 2012, his BMI (body mass index) wasn’t even on
the growth chart! Now he typically falls between the targeted fiftieth
percentile and the seventy-fifth percentile! Bryce loves to run, play
soccer, swim, watch sports, play with his little brother (Paxton), read,
and watch Monster Jam!
fibrosis (CF) is an inherited chronic disease that affects the lungs
and digestive system of about 30,000 children and adults in the United
States (70,000 worldwide). A defective gene and its protein product
cause the body to produce unusually thick, sticky mucus that clogs the
lungs, potentially leading to life-threatening lung infections, and
obstructs the pancreas, stopping natural enzymes from helping the body
break down and absorb food.
are many treatments available for those with CF as each case is
slightly different. In addition, real progress has been made in the
search for a cure, but the lives of people with CF are still cut far too
short. There still is no cure for this devastating disease.
Bryce uses airway clearance, breathing treatments, pancreatic enzymes,
high calorie drinks (Pediasure 1.5 calorie), CF vitamins and
multi-vitamins, an appetite stimulant, and a laxative to maintain his
health and function “normally” each day. Airway clearance is typically
performed using a vibrating vest that pumps air through hoses and into a
vest that vibrates/shakes to help break up the sticky mucus and remove
it from his lungs in an attempt to keep bacteria from setting up camp
and causing infections. When healthy, Bryce sits for 30 minutes twice a
day while using this vest. When he is ill, he will do additional vest
treatments and/or manual chest percussion therapy (CPT). Prior to the
vest, we did manual CPT at least twice a day as he was too small for the
addition to airway clearance, Bryce also takes pancreatic enzymes each
time he eats something with fat and/or protein, which is most of the
time as CFers require those nutrients (and many extra calories!) to
maintain a healthy weight. In an effort to keep the weight on our
energizer bunny, we try to get him to drink at least one 350 calorie
Pediasure daily (if not more) and are constantly encouraging him to eat
more. We have also implemented an appetite stimulant once or twice a day
to help his appetite – it seems to work most of the time but it can
still be a struggle. Bryce has mastered the art of taking pills and can
take several at a time – I think the most I’ve seen is 6 or 7 at once!
He’s been taking enzymes and doing airway clearance since he was 5 weeks
old! In addition to pancreatic insufficiency, the GI tract can be
compromised due to the thick mucus, causing constipation and blockages.
This is where the laxative comes into play. Including this as a part of
his daily routine helps to keep constipation under control and blockages
the moment, Bryce uses a nebulizer at least once per day for a
mucus-thinning medication called Pulmozyme. He also uses an inhaler or
nebulizer for delivery of albuterol twice daily as part of his regular
maintenance and to keep his airways open. When he is ill/has a cough, he
receives additional albuterol treatments to keep his airways open,
alleviate wheezing, and help minimize coughing.
his respiratory culture (a test done at each visit to the CF clinic
that resembles a strep culture/swab) comes back positive for risky
bacteria, he may also use the nebulizer for that medication or he will
be placed on oral antibiotics in an attempt to avoid a lung infection.
Bryce not only sits still for his treatments (over an hour each day) but
he also tolerates using the vibrating vest while doing many of these
we do our best to not to let CF control our lives, we still need to
take precautions above and beyond the "typical family". Unfortunately,
this can mean missing out on or delaying some events. For example, Bryce
needs to avoid being around those who are already ill as well as
minimize his exposure to large groups during cold and flu season. This
is a major challenge now that he is in school! We are constantly washing
hands and using sanitizer… and we request that anyone who comes in
contact with Bryce do the same! Luckily, we have had very supportive
family, friends, caregivers, and school staff! However,
there are some good things associated with CF… Bryce can eat almost
anything he wants as he needs MANY more calories than the average
person. Although the requirement for a high calorie diet can pose some
challenges not only for a first grader but for all ages, we try to focus
on the positive and let him eat to his heart’s content … and many days
he needs a little "encouragement" to continue eating beyond that point!
;-) That’s where the Pediasure comes into play most of the time –
especially at school. Each day, he gets a 3 oz “shot” of Pediasure with
his enzymes before he eats lunch at school. That little cup packs in at
least 131 calories, 6 grams of fat, and more than 5 grams of protein!
should also get as much exercise as possible - which isn't really a
problem for this 6-year-old boy! He wakes up moving and rarely stops
until his head hits the pillow. Anything that changes his method of
breathing and gets him moving or breathing hard is another form of
airway clearance. Although it does not replace his daily treatments, it
2014, Team BAM became a National Family Team, being represented in 3
walks nationwide! We hope to continue this tradition in 2019. If you
would like to join any or all of the teams, please let me know and I
will get you the dates and locations! And we are always recruiting for
exciting change over the past year is that Bryce was able to start a
new drug called Orkambi
(https://www.cff.org/Trials/pipeline/details/86/Orkambi) in the fall of
2018! This drug helps the salt to move correctly throughout his body,
thereby decreasing the thick, sticky mucus and minimizing the problems
as a result! He still needs to continue his daily therapies (CTP/vest,
nebulizers, enzymes, etc.) for the time being but his overall health
should see an improvement! So far, we are pleased with the results.
Although we haven’t noticed any major changes, we have noticed some GI
improvements as well as a huge increase in his appetite! We don’t know
if this the Orkambi or changing the appetite stimulant around as they
sort of happened at once. But whatever is working, we hope it continues!
however, is not a cure and is not as effective as the current drug on
the market, Kalydeco, which improves the lives of approx. 4% of the CF
population, but it is still an improvement. There are several other
medications in the pipeline that have even greater potential including
something that is being called “The Triple”
Bryce should be able to take this one once it is on the market and
approved for his age range. According to Johns Hopkins, the results for
“The Triple” are better than ANY of the other CFTR correctors currently
out there! For more information, visit the CFF's Drug Development
Pipeline site (https://www.cff.org/trials/pipeline)!
We are very excited and so thankful to all involved! So many great things on the horizon!
though the CF Foundation and the pharmaceutical companies have done
GREAT things, we can't stop now!! There is still a long way to go before
those with ALL CF mutations are given a chance to breathe easy! By
donating to Great Strides, you will help fund research to continue
monitoring of those on the newer medications, development of additional
medications and treatments, research toward finding a drug that will
eliminate not only the root cause but also the need to do hours of daily
treatments and to finding a CURE for not only those with a few
mutations, but ALL CFers!
participating in and/or donating to the Great Strides walk, we are
helping to add tomorrows for not only Bryce but for everyone living with
CF by funding life-saving research and medical progress. CF research is
not funded by the federal government; therefore, we need every single
penny. The CF Foundation has been recognized by publications such as
SmartMoney as one of the most efficient organizations of its kind.
Nearly 90% of every dollar of revenue raised is available for investment
in vital CF programs to support research, care, and education. Your
gift is 100% tax deductible.
you join us? Support us by making a donation to our Great Strides
fundraising campaign! Since we started walking in 2013, Team BAM has
raised over $63,000 for the CF Foundation across our three teams!
Incredible! For more information about CF or Great Strides, please visit
www.cff.org or feel free to contact us!
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Important note on Attendance at Foundation Events:
Infections can exacerbate CF symptoms and worsen lung function, so we ask attendees at Foundation-sponsored events to observe the following best practices to reduce the risk of germ transmission:
Regularly wash hands with soap and water or with an alcohol-based hand gel.
Cover coughs and sneezes with a tissue or your inner elbow.
Maintain a safe 6-foot distance from anyone with a cold or infection.
To further reduce the risk of cross-infection, the Foundation’s attendance policy recommends that all people with CF maintain a safe 6-foot distance from each other at all times while attending an outdoor Foundation-sponsored event. All Great Strides walks are non-smoking events.