Our son, Bryce (BAM), was born in the summer of 2012 and diagnosed with cystic fibrosis at only five weeks old. He is now a happy, intelligent, and energetic seven-and-a-half-year-old that weighs around 55 lbs. and is just over four feet tall! When he first visited the Cystic Fibrosis Clinic in 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 & basketball, watch sports, play with his little brother (Paxton), play Wii, read, and watch Monster Jam!
Cystic 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.
There 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. Currently, Bryce uses airway clearance, breathing treatments, pancreatic enzymes, high calorie drinks, CF vitamins and multi-vitamins, an appetite stimulant, a laxative, and a CFTR modulator 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 vest.
In 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 from forming.
At 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. If 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 nebulized medications or eating a meal!
But the most recent addition to his treatment plan is Symdeko – a CFTR modulator/corrector that has been proven to not only minimize the symptoms of CF but also to slow down the progress of the disease! This drug working by helping 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 remain steady, if not improve! So far, we are pleased with the results. Although we haven’t noticed any major changes, we have noticed some GI improvements and he seems to weather cold viruses a little easier at times! We don’t know if this the Symdeko or just his immune system improving but whatever is working, we hope it continues!
Although 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!
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 second 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 4 oz “shot” of Pediasure with his enzymes before he eats lunch at school. That little cup packs in at least 175 calories, 9 grams of fat, and more than 7 grams of protein!
Bryce should also get as much exercise as possible - which isn't really a problem for this 7-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 does help.
In 2014, Team BAM became a National Family Team, being represented in 3 walks nationwide! We plan to continue this tradition in 2020. 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 additional walks!
More exciting news over the past few months is the approval of a new drug called Trikafta! This is, by far, the best treatment yet as it is expected to be able to treat as many as 90% of the CF population! Additionally, it has been proven to improve lung function, lower sweat chloride levels (the amount of salt lost form the body and a test to diagnose CF), reduce exacerbations and hospitalizations, and improve overall quality of life… among other things! Although Bryce cannot take this drug yet (it’s only approved for those 12 and older), we hope that he can take it in the next year or two to help maintain his good health!
We are very excited and so thankful to all involved! So many great things on the horizon! And although there are several CFTR modulators/correctors available (Kalydeco, Orkambi, Symdeko, and Trikafta), they do not treat all those with CF and they are not a cure. For more information, visit the CFF's Drug Development Pipeline site (https://www.cff.org/trials/pipeline)!
Even 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!
By 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.
Will 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 $75,000 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!
- Josh & Amy
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Due to the COVID-19 pandemic, indoor events sponsored by the CF Foundation are strictly prohibited at this time. Scheduled outdoor events must adhere to strict requirements to minimize the risk of COVID-19 infection. Events may be subject to change at any time based on guidance from the Centers for Disease Control and Prevention and local health officials.
IMPORTANT NOTE ON ATTENDANCE AT OUTDOOR FOUNDATION EVENTS:
To minimize the risk of COVID-19 infection, attendees at CF Foundation events must adhere to the following requirements:
Practice physical distancing and maintain at least a safe 6-foot distance from persons outside of their household at all times
Face masks strongly encouraged and expected to be worn in accordance with local guidelines. (Note, children under two years of age should not wear masks due to safety concerns and therefore should not attend CF Foundation events where there is risk of interacting with someone outside of their household.)
Follow basic infection, prevention and control practices by regularly washing hands with soap and water or with an alcohol-based hand gel, covering your cough or sneeze with a tissue or your inner elbow.
Persons with CF should consult their physician before participation in any in-person event as they may be at an increased risk for severe illness from COVID-19. People with CF should maintain a safe 6-foot distance from persons outside of their household at all times.