Cystic Fibrosis (CF), the Cystic Fibrosis Foundation (CFF) tells us, is an inherited (genetic) chronic disease that affects approximately 30,000 individuals in the U.S. and 70,000 individuals worldwide. Almost 1,000 new cases of CF are diagnosed in the U.S. annually. According to Disabled World, “One out of every 3,500 children in the U.S. are born with it.” CFF tells us 70 percent of patients are diagnosed before age 2 in the U.S. But how does this disease present itself and how does it affect children?
The Cystic Fibrosis Foundation tells us that CF is caused by “A defective gene and its protein product [which] cause the body to produce an unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening lung infections; and
- obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.”
Parents of children need to be able to recognize the symptoms of CF which include:
- very salty-tasting skin;
- persistent coughing, at times with phlegm;
- frequent lung infections;
- wheezing or shortness of breath;
- poor growth/weight gain in spite of a good appetite; and
- frequent greasy, bulky stools or difficulty in bowel movements.
The disease affects more people of Caucasian background than those of Native American, Hispanic, or African American origin. The disease is also found to a lesser extent in Canada and in the United Kingdom.
How would you, as a parent or caregiver, be able to tell if your child has Cystic Fibrosis? If a child is experiencing frequent breathing problems and other symptoms noted above, a parent can take their child to the emergency room or their primary care physician in order for the child to be medically evaluated. A doctor is able to prescribe tests that can confirm or rule out CF.
The primary test, which is the “Sweat Test,” is easy and painless. This test determines the salt level in a child’s perspiration, which is one of the major determining factors in CF. Biological parents can also take a blood test which determines whether or not they are carriers of the CF gene. Children would have to inherit one copy of the defective CF gene from each parent in order to have the disease.
In the U.S., a tremendous advancement in identifying CF is that in all states newborns are screened for CF. Newborns screened for CF can benefit from early diagnosis and treatment, which can:
- Improve growth;
- Maintain healthy lungs;
- Reduce the number of hospital stays; and
- Add years to an individual’s life.
The prognosis for those with CF is much better today than it was in the 1950s. Airway clearance, nutrition, and drug therapies are important in controlling CF in children and adults. Today, there is hope, as many individuals with CF are living longer, healthier lives than in the recent past.
This child safety blog piece is dedicated to the memory of Mrs. Jamie Maria Mick Ferber who died in January 2003, at age 27, due to complications of Cystic Fibrosis. As a child, Jamie was a poster child for promoting awareness of Cystic Fibrosis. Jamie grew up in Broadway, Virginia, attended Eastern Mennonite schools in Harrisonburg, graduated from Shenandoah University in Winchester, and became a respiratory therapist and advocate for Cystic Fibrosis patients. We are grateful for her life and dedication.