Steven Jones

Our great nephew, Bryce (BAM), was born in the summer of 2012 and diagnosed with cystic fibrosis at only 5 weeks old. He is now a happy, intelligent, and energetic 11 ½ year old who is crushing it in middle school! 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 30th and 50th percentile which is actually a little low for those with CF but he is working hard to maintain and gain some weight! Bryce loves to run, watch and play sports (especially soccer), play with his younger brother (Paxton), and play video games!

Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30K children and adults in the US (70K 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 and high fat meals, CF vitamins and multi-vitamins, 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 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, manual chest percussion therapy (CPT), and/or use an oscillating positive expiratory pressure device called Aerobika. Prior to the vest, his parents 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 food 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. He’s been taking enzymes and doing airway clearance since he was 5 weeks old! In an effort to keep the weight on our energizer bunny, he is encouraged to eat more and try new things! Bryce used to take an appetite stimulant twice a day but since starting a new modulator (Trikafta), his appetite has improved and his weight seems to be stable, so he was able to eliminate that medication. Bryce has mastered the art of taking pills and can take several at a time – up to 9 or 10 at once!

CF can also complicate things in the GI tract, causing constipation and blockages from the thick mucus. This is where the daily laxative comes into play.

At the moment, Bryce uses a nebulizer at least once per day for a mucus-thinning medication called Pulmozyme. When ill, he will use an inhaler or nebulizer every 4 to 6 hours for delivery of albuterol. This will help 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.

As mentioned above, a CFTR modulator/corrected called Trikafta was added to his daily regimen in July of 2021. This medication has been proven to not only minimize the symptoms of CF but also to slow down the progress of the disease! This drug works by helping the salt to move correctly throughout his body, thereby decreasing the thick, sticky mucus and minimizing some of the problems! This is the best treatment yet as it is able to treat as many as 90% of the CF population! It has been shown to improve lung function, lower sweat chloride levels (the amount of salt lost from the body that is detected with a test used to diagnose CF), reduce exacerbations and hospitalizations, improve appetite and weight gain, and improve overall quality of life!

So far, we are pleased with the GI results of Trikafta – better appetite, weight maintenance/gain, no appetite stimulant and a little less of the laxative! His lung function has remained steady but that was expected since he typically has great results on his PFTs (Pulmonary Function Tests). He still needs to continue his daily therapies for the time being but his overall health should be maintained, if not improve! 
 
Although his family does their best to not to let CF control their lives, they still need to take precautions above and beyond the "typical family"…especially with COVID so prevalent over the past couple of years. But they also feel like CF has prepared them for the pandemic as they have always had some limitations... missing out or delaying events and gatherings, utilizing more soap and sanitizer than you can ever imagine, and wearing masks to doctor’s visits and frequently during cold and flu season.

We are very excited and so thankful to all involved!  And 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 as the modulators cannot treat all patients with CF and they are NOT a cure!

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! The CF Foundation also helps patients and their families find the best insurance coverage and co-pay assistance programs, helps cover the cost of medications and treatments for those that qualify, as well as many other programs so that everyone with/affect by CF can live a full life! Without the CF Foundation and the co-pay assistance programs, we would not be able to afford Bryce’s medications as his enzymes, inhaled treatments, and Trikafta alone would be more than $30,000 per month... and that’s with decent insurance!

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. And don’t forget... your gift is 100% tax deductible!

Will you join us? Support Team BAM and the CF Foundation by making a donation to our Great Strides fundraising campaign! For more information about CF or Great Strides, please visit www.cff.org or feel free to contact us!

Thank you!