If your child is diagnosed with cystic fibrosis (CF), you can count on Children’s Health to manage all aspects of their condition with a caring multidisciplinary team of highly-trained experts. We will help your child find ways to fit CF into everyday life, rather than having his or her life revolve around the disease.
What is Pediatric Cystic Fibrosis (CF)?
Pediatric cystic fibrosis is an inherited genetic condition that affects mucus production, leading to lung infections and breathing difficulties. Normally, mucus is a slippery, watery substance that keeps the lining of organs moist so they can function properly. With CF, mucus becomes thick and traps particles in the lungs, causing infections and labored breathing. In addition, CF can cause problems with the pancreas, liver, intestines and sex organs.
What are the signs and symptoms of Pediatric Cystic Fibrosis (CF)?
The severity of the disease can differ from child to child, even in the same family.
Symptoms in infants (birth to 1-year*)
- Delayed growth and failure to gain weight normally
- Meconium (thick, dark putty-like stools) can become too thick and sticky to pass
- No bowel movement in first 24-48 hours following birth
- “Salty” skin
*Age of infants as defined by the World Health Organization (WHO).
Symptoms in children 1-year and older
- Low bone density
- Male infertility
- Clubbed toes and fingers (widening and rounding of the tips of fingers and toes)
- Allergies that last all year
- Lung infections
- Recurring colds
- Shortness of breath
- Sinus infections
- Inflamed pancreas
- Intestinal gas
- Liver disease
- Nausea and loss of appetite
- Pale or clay-colored stools that are foul-smelling, have mucus and float
- Swollen belly from severe constipation
- Rectal prolapse (when the rectum sticks out through the anus) from frequent coughing or problems passing stools
How is Pediatric Cystic Fibrosis (CF) diagnosed?
Most babies are screened for cystic fibrosis as newborns in the hospital, with a blood sample taken from their heel. In Texas, newborns are screened for a protein that can indicate CF.
If your child receives a positive screening, we will admit them to the Children’s Health Claude Prestidge Cystic Fibrosis Center within 24-48 hours. The next step is a sweat test, a painless test in which sweat is collected from the skin and analyzed for salt content. A high chloride content points to a diagnosis of cystic fibrosis. If the sweat test is positive, we will see your child within three days to develop a care plan as soon as possible.
Some babies are diagnosed with the disease in utero based on genetic testing. Parents can speak to counselors at the clinic even before their baby is born. If children aren’t diagnosed at birth, most are diagnosed by the age of 2.
What are the causes of Pediatric Cystic Fibrosis (CF)?
Cystic fibrosis is a congenital disease that is present at birth. It occurs when a child inherits a faulty gene from each parent. The parents likely do not have the disease.
How is Pediatric Cystic Fibrosis (CF) treated?
Treatment begins within days of diagnosis. It will include:
- Airway clearance therapies to loosen the mucus in the lungs
- Inhaled medications to relax airways
- Enzyme therapy (medication that helps with digestion)
- Antibiotics and anti-inflammatory drugs (in some, but not all cases)
If your child is diagnosed with CF as a newborn, you can expect monthly monitoring in the clinic until they are 6 months old. You will then continue with visits every two months until they are 1 year old. After that, your child will have three quarterly visits and one annual visit. If your child experiences a flare-up that requires hospitalization, our team will be there to provide the care they need and answer all of your questions.
Our multidisciplinary approach to treating cystic fibrosis gives your child access to the best care possible. All of the specialties your child needs are located under one roof for convenience and peace of mind. At Children’s Health, we not only treat the disease, but also closely monitor weight and nutrition so they can live their healthiest life possible.
Both a pulmonologist and gastroenterologist direct the Children’s Health Claude Prestidge Cystic Fibrosis Center, ensuring coordinated care for both lung and digestive health. In addition, your child will have a dedicated care team that includes:
- Genetic counselors
- Medical assistants
- Nurse practitioners
- Physical therapists
- Social workers
- Research coordinators
- Respiratory therapists
As part of developing effective treatments that work specifically for your child, we will use our state-of-the-art pulmonary function lab. By coming directly to your child’s exam room, we ensure the highest level of infection control.
At Children’s Health, your child will have access to cutting-edge treatments and clinical trials. We participate in the Cystic Fibrosis Therapeutics Development Network, the largest cystic fibrosis clinical trials network in the world. Sharing information collaboratively with other network treatment centers has helped us continuously improve patient outcomes – our rates of cystic fibrosis flare-ups have fallen while our patients’ pulmonary function has risen.