As a parent, you want to give your child the very best start in life. But when they are diagnosed with a life-threatening disorder like cystic fibrosis (CF), you may worry about their future.
What is cystic fibrosis?
CF is a lifelong illness that causes mucus inside your sinuses and airways to become thick and sticky. With this condition, the mucus builds up and clogs those passages and can lead to chronic infection with serious breathing problems and progressive lung disease. CF impacts many different systems, but often there are also issues with digestion, malnutrition and growth and development.
While there is no cure for CF, advances in treatment have extended both life expectancy and quality of life for those living with the disease.
“Often families are aware of the ‘old CF’ from the early 90s when median survival was meaningfully lower than it is now,” said Wayne Morgan, MD, a pediatric pulmonologist at Banner Health’s Diamond Children’s Multispecialty Services Clinic and the University of Arizona Pediatric Pulmonary Center.
“Thanks to new treatment therapies and improved care, most of our patients are doing better and living well into their 40s—and perhaps even longer—which is still far less than our goal but is much better than 30 years ago.”
Five Things Parents Should Know
Even knowing all the advancements in treatment, learning your child has CF is still scary, and you undoubtedly have lots of questions and concerns. It is normal to feel overwhelmed by how hard it is to care for a child with CF, but you can learn to do all that and more for your child. Dr. Morgan shared five important things parents should know when it comes to the treatment and care of their child.
1. You aren’t alone. Comprehensive care plays an important role in your child’s treatment and safety.
Your child’s CF is highly complex and individual. The key to their progress in health and well-being is when their care is done in partnership with a highly specialized, multidisciplinary team at a Cystic Fibrosis Foundation-accredited center.
At these centers, you, your child and your family are guided every step of the way by experts and specialists in the field, including doctors, nurses, dietitians, pharmacologists, psychologists, social workers and respiratory therapists.
When you first meet them, your team will go over the basics of the disease, address initial therapy, stress nutrition and care. “We try not to overwhelm families with too much information in the beginning, but we do stress that we are here to answer questions, that there are no silly questions, and importantly, that we expect them to ask the same questions again,” Dr. Morgan said. “The CF clinical team works hard to develop family partnerships that are trusting and open to create realistic and optimal treatment plans.”
CF is a lot of work and it can be disruptive to your life and your child’s life, but with proper care and treatment, you can manage it. To find a comprehensive CF care center, visit cff.org to search by your zip code.
2. Nutrition and exercise are important.
Regular physical activity and good nutrition are important—especially when you have CF.
Exercise has many benefits. It can help loosen mucus and improve oxygen flow, which can make your child feel better. Exercises like swimming can also help increase their strength and endurance that are used for breathing. Your doctor can let you know what kinds of exercises will be good for your child.
Good nutrition is different for a child with CF. Your child will have a diet that is high in calories and fat, which will help your child gain and keep weight and energy. They will also take vitamins, mineral and enzyme supplements and may receive tube feeding. Their dietitian can help you plan meals.
3. Treatment therapies are no longer one-size-fits-all.
Today, people with CF are living longer and more fulfilling lives thanks, in part, to specialized CF care and a range of treatment options. In addition to nutrition and exercise, there are a number of medications that help clear the thick and sticky mucus from airways and reduce inflammation. There’s also antibiotics to treat CF lung infections and emerging therapies that target and correct the defective protein made by the CFTR (cystic fibrosis transmembrane conductance regulator) gene.
“In this day and age, the mutation a child has can impact prognosis both due to the variation with mutation type, but even more so because roughly 90 percent of people with CF who have the F508del mutation will be able to receive Trikafta, a new combination medicine that is remarkably effective and should materially expand survival, and as importantly, improve quality of life,” Dr. Morgan said. “For non-F508del or other mutations that cannot respond to highly effective modulator therapy, we are still optimistic, and the CF Foundation is making a substantial investment in research to find a cure for them.”
If you don’t know your child’s mutations, ask their doctor about specialized genetic tests.
4. Your child can live a “normal” life.
When your child is battling a critical illness, you may have a tendency to want to shield them but being an active and “normal kid” can help them stay healthy. Many kids with CF grow up, play sports, go off to college, get married and have families. They may struggle at times but being there to support and guide them with a positive attitude can help your child learn and grow.
5. Take care of yourself as well.
Practice self-care. Being a CF parent is hard. Parents of children with CF are more likely to experience anxiety, depression or both. It can be exhausting, frustrating and scary, and it’s okay to take a time-out for yourself and ask for support.
Talk to your child’s care team, your doctor or a behavioral health specialist who can work with you on ways to reduce stress, anxiety and/or depression. You can also join a support group. Sharing your experiences with other parents who face similar challenges may help you feel less overwhelmed.
Learning how to care for your child with CF can be scary at first but remember you’re not alone. The outlook for those with CF has improved dramatically over the years, thanks to the advancements in treatment and comprehensive care. If your child has recently been diagnosed with CF and you have questions about their treatment and care, find a CF care center near you, or contact a pulmonary specialist at Banner Health.