Team helps provide much-needed cystic fibrosis treatment
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.”
Bridge of Hope
Sadeghi, who came to the United States from Iran in 1977, said the life expectancy of a cystic fibrosis patient in Iran is around 18 years. Reza’s brother died from the disease at the age of 23.
After his brother died, Reza’s mother asked their local physician to “recommend whatever they could do for Reza, and that doctor recommended us,” said Sadeghi, who went to see Reza in Iran last September.
With a focus on “reducing the airway secretions, reducing the infection and reducing lung damage,” said Sadeghi, Reza’s treatment will include a swab test, genetic mutation, blood work and two weeks of IV antibiotics.
On his first day at the center, Reza did a lung function test and Sadeghi taught him and his father how to do an airway clearance on Reza, which involves blowing into a device that shakes the airway and loosens up the mucus so he can cough it up.
Sadeghi said treatments are “expensive and time-consuming.”
“During the day, the patients spend about three hours on treatments,” he said, “and each medication costs $2,000 to $8,000 per medication, per month.”
At the end of the two and a half weeks, Sadeghi will give Reza and his father the whole treatment plan to take back to Iran and continue the regimen.
“ I’m hoping to give the treatment, with medications, for one year,” said Sadeghi, “and then hopefully they’ll come back each year [to the United States] until we set up a CF center in their country.”
Reza, who loves soccer and science and aspires to be a doctor one day, said the treatment “really means a lot” to him.
“That’s why he took this hard journey to this country to get better — to get the right treatments and the right information he’s been lacking all his life,” Dr. Golnar Raissi translated. “He has a very good feeling about the treatment. He’s not worried.”
Sadeghi has set up an Iranian Cystic Fibrosis Foundation in Iran, which will “hopefully assist the whole process,” he said, and he plans to set up more in other countries.
“The CF Foundation is a team approach,” he said. “We have a social worker, dietitian, pulmonologist; we have a respiratory therapist and we have the privilege of having an integrative medicine doctor.”
Coming to the United States from Iran wasn’t easy for Reza and his father, but Sen. Richard Blumenthal was “really instrumental” in helping with the process, said Sadeghi.
Raissi, integrative doctor at the Tully Center and part of Reza’s treatment team, said getting a visa was “very difficult.”
Because there is no U.S. embassy in Iran, Reza and his father had to go and stay in Turkey for their visas, which, Raissi said, “is not easy for a father and his sick child to do because of the expense.
“Then they had to go back to Iran and have the anxiety of waiting to see whether or not they would get the visa, then they had to go back [to Turkey] and get the visa,” she said. “It’s a very difficult process from the beginning and a very hard journey.”
The fact that Reza and his father were able to obtain their visas in three months, Raissi said, is “the result of an effort of a lot of people.”
“There is a law firm, Cravath, Swaine & Moore, who has been helping with the whole process of getting the paperwork in order and communicating with the consultant. It’s teamwork,” said Sadeghi.
“Then we have the Wilton Interfaith group, which has helped with hotel arrangements and is donating a meal each day and helping with transportation.”
Sadeghi said “everyone is trying to help,” including the Marriott hotel where Reza and his father are staying, which gave them a discount.
“I’m thankful to God and to Dr. Sadeghi, who gave me this opportunity,” Raissi translated for Reza.
Reza’s father said he and his family are “really appreciative of all the doctors and staff and everyone else” that have helped bring him and his son to the United States, including the U.S. government, Raissi translated.
“He is hoping the government will help other families and patients that have these problems to come here and get the right treatments.”
To learn more about the CF Foundation, visit cff.org. To learn more about Bridge of Hope, visit cfprojectinc.org.