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The Children's Hospital of Southwest Florida
Specialized Care for Children with Cystic Fibrosis
Just as children inherit physical or personality traits from their parents, they also
can acquire illnesses, diseases or other medical conditions. One in particular is
cystic fibrosis (CF)—a life-threatening disease that causes mucus to build up
and clog the lungs and pancreas. While there is no cure for CF, proper medical
treatment and nutrition can improve length and quality of life.
The first step is a definitive diagnosis. Oscar Alea, M.D., pediatric pulmonologist,
says all newborn babies in Florida are screened for CF. Positive screens in Lee,
Collier, Charlotte and Hendry counties are referred to The Children’s Hospital of
Southwest Florida’s Cystic Fibrosis Center for a more thorough evaluation. If a
newborn screening is negative, but symptoms occur later in life, there are other
testing mechanisms. A sweat test is the most common and involves measuring
the amount of chloride—a component of salt—in the sweat. A high level of
chloride signifies CF. Genetic testing also can be done for 1,900 gene mutations
to confirm diagnosis. Often, genetic testing is used if the sweat test is unclear.
“The prognosis of CF has improved dramatically,” Dr. Alea says. “A lot of the
treatments—including aerosolized bronchodilators, aerosolized antibiotics and
intravenous antibiotics—are used to clear mucus from the airways. Nutrition is
also extremely important to provide ample growth and energy to fight infection
and inflammation.”
Dr. Alea explains that the cystic fibrosis transmembrane regulator (CFTR)
protein is the gate of the airway—controlling the movement of sodium and
chloride. “The CFTR regulates sodium, chloride and water transport,” he says.
“In patients with CF, this gate system is abnormal and causes the mucus in the
lungs to be very thick. Thickened mucus allows bacteria to harbor within the
lungs, which causes inflammation, recurrent bronchitis, pneumonia and gradual
damage to the lungs.”
Dr. Alea shares that, on the horizon, there are drugs that have the potential of
restoring CFTR function and gating in certain known genetic mutations.
“CF is a complex disease and affects each patient differently,” Dr. Alea says. “Early
detection and access to an accredited CF Center helps ensure proper, specific and
the most effective care possible.”
Oscar Alea, M.D.
Pediatric Pulmonology
Children’s Specialists of Florida
7970 Summerlin Lakes Drive, Suite 200
Fort Myers, FL 33907
239-437-5500
