With the help of many, including the Wilton Interfaith Action Committee (Wi-ACT), Iranian 17-year-old Reza AliAkbari is getting treatment for cystic fibrosis, a life-threatening, chronic genetic disorder that damages the lungs and digestive system.
Reza arrived in the United States with his father, Aziz AliAkbari, on Monday, Jan. 18, and started two and a half weeks of outpatient treatment at Stamford Hospital’s Tully Health Center, under the direction of pediatric pulmonologist Dr. Hossein Sadeghi of Wilton.
“Cystic fibrosis is a genetic disorder that’s autosomal recessive, which means children are born with it,” said Sadeghi, who is also chief of the Columbia Hospital Cystic Fibrosis Program.
“Recessive means each parent has to have the gene. When they do, there’s a 25% chance that their child will have cystic fibrosis.”
Because the gene is abnormal, he said, it produces an abnormal protein, which functions as a chloride channel across cell membranes that produce mucus and digestive enzymes.
“Because the chloride channels are defective [in someone with cystic fibrosis], their lungs have really thick secretions,” Sadeghi said. “It’s like putting tar in the lungs.”
Cystic fibrosis is a multi-organ disease that also affects fertility, the liver, the sinuses, and the pancreas.
When a person eats fat and protein, the pancreas normally produces enzymes, but for someone with cystic fibrosis,” said Sadeghi, “that duct is basically blocked because of the thick secretions.”
“Without treatments,” he said, “they can die from malabsorption and respiratory failure.”