Our 5 year old son Devland has Cystic Fibrosis. Cystic Fibrosis is a life-shortening genetic disease that affects the movement of water and salt in the body and causes the progressive scarring and loss of function of the respiratory system and the digestive system. The projected life expectancy for Devy was 37 years old when he was born in 2015, and today, with the advancements in medications and treatments, the Cystic Fibrosis Foundation announced that it's gone up to 40 years. But 40 years is not long enough to give Devy the full life that he deserves. There is no cure for CF--yet. That is why we raise awareness, advocate, fundraise, and fight for our son.
Devy was born in September of 2015. At just 2 weeks old, we found out he has CF through the California Newborn Screening. My husband Brendan and I are both genetic carriers. Immediately, we were referred to a specialty facility that can handle his complex diagnosis, treatment, and care. Because it is so rare, with 33,000 cases in the US (70,000 worldwide), there are only about 100 hospitals in the country accredited to treat CF. Our nearest CF Centers are in Madera or Hollywood, both over 120 miles away from us here in Bakersfield.
We make CF Clinic visits to Hollywood anywhere from every 2 weeks to every 3 months, depending on how well he's doing. It's a complex balance of whether he has any bacteria culturing in his lungs, new symptoms, or signs of poor weight gain/growth. Since CF is a progressive disease, we initiated all of his treatments and therapies proactively to help prevent lung deterioration and allow for his lungs to develop as fully as possible before they begin to lose function. Appointments are at least 3 hours long and mean another 4-5 hours on the road.
Every cold matters. Something as minor as a cold could hospitalize Devy. CF makes his body trap bacteria in his respiratory tract and prevents him from coughing up the thick and sticky mucus. This is why we tend to stay isolated away from large groups of people, or why we might stay distant at parties or out in public. It's extremely difficult to explain to a 2.5 year old why he has to wear a surgical mask around large groups of people when he doesn't see anyone else wearing one. Sometimes it's easier to just stay away. Since 7 months old, Devy has spent over 950 hours in his "Vest" doing Chest Physiotherapy (CPT), an airway clearance system that helps knock-loose the mucus so it's easier for him to cough up. He's spent at least another 700 hours doing breathing treatments, inhaling medications that help move the mucus, dilate the airways, and draw water into his secretions. If he has an active infection, we may add an aerosolized antibiotic, extra CPT, and extra treatments. This little guy will spend as much as 1 hour at a time doing a single treatment.
For food, Devy, like most toddlers, doesn't always want to eat. To maintain his caloric needs, he has to take digestive enzymes before he eats or drinks anything with fats or protein, otherwise his body can't process it. Currently he's at 24 pills a day for meals, 2 pills for his antacid, fat-soluble vitamins (because his fats stores don't exist), and supplemented salt. (He's been as high as 40 pills a day!) As a result of strep-throat in November 2017, he lost a significant amount of weight and dropped in his percentiles enough that in January of 2018, Devy had another tool added to his CF arsenal: a gastrostomy tube (Gtube). Devy currently gets at least 50% of his daily caloric needs from nightly tube feedings while he sleeps. That's another 10 hours that Devy is attached to some sort of machine that helps extend his life. November of 2018 we added Orkambi which helped Devy move salt and water more effectively in his cells--the hope was that this medicine could help slow the progression of lung damage, boost his appetite, and help him gain weight. Unfortunately, he was unable to tolerate the side effects of Orkambi and had to stop.
We've been extremely fortunate that Devy has only been hospitalized for his Gtube placement, and adenoid removal, as we were told he'd be hospitalized on average once a year for at least 2 weeks a year. We are incredibly blessed that we've been able to manage most of his symptoms between Clinic and home. We are even more grateful that I have insurance, as his medications and equipment retail over $50,000/month. This CF journey is long and can be an isolating trip. We miss having the spontaneity that we once did to just drive somewhere for the weekend with an overnight bag. [I can't even imagine flying with ALL Devy's paraphernalia!]
What's the best part of all of this is Devy, and being his Mamma. I have never met a more optimistic little human. He is full of joy, full of wonder, and full of life--his personality is exploding, and he is at the center of all his care. I have never been more proud than watching Devy show off his "button" (Gtube--it glows in the dark!), holding his own breathing treatment mask, or extending out his arm for "one more poke" that he knows will hurt. Devy is a brave and beautiful fighter.
So thank you for staying with us on this CF Journey, whether you've been with us since the beginning of our family, or you're new to our community. We need your prayers, we need your encouragement, and we need your support. Nearly every dollar donated to the CF Foundation goes to fund drug development (there's no Federal funding), advocate for pts and families with CF, and create a community for our families. Please consider a tax deductible donation to the CFF and share our story.
All my Love,
Devy's Mamma, Alison
Here's a link to videos about our CF journey: