BY GARY WISBY
A UIC physician is helping establish a bone marrow transplant center in Nigeria to treat sickle cell disease.
The need there is far greater than in any other country, says Damiano Rondelli, director of the UI Health blood and bone marrow transplant program.
Nigeria has about 91,000 sickle cell births a year — more than in any other of the 10 countries in sub-Saharan Africa that are most affected.
The Democratic Republic of Congo, at about 39,750 births, is No. 2. The total number of babies born with sickle cell in the 10 sub-Saharan nations is more than 242,000 — more than two-thirds of the 305,800 births worldwide. About 5 million Nigerians have sickle cell, compared with about 100,000 in the U.S.
“The relevance of treating sickle cell disease in Nigeria is huge,” said Rondelli, chief of hematology/oncology at UI Health. He visited Nigeria in late October with Victor Gordeuk, director of the UIC Sickle Cell Program. They met with medical personnel, sickle cell patients and their families. The chief medical director of the University College Hospital of Ibadan, Temitope Alonge, “embraced with enthusiasm the vision that we brought, and is willing to support the project as well as finding funds,” Rondelli said. It could be one to two years before the center opens, he said.
Rondelli has already helped set up bone marrow transplant units in Bangalore, India, and Kathmandu, Nepal, which treat several blood diseases, not just sickle cell. At UI Health, physicians have cured 12 adult patients of sickle cell disease using stem cell transplantation from healthy, tissue-matched siblings.
The technique eliminates the need for chemotherapy to prepare the patient to receive the transplanted cells. The transplants were the first performed outside the National Institutes of Health campus in Maryland, where the procedure was developed. Doctors have known for some time that bone marrow transplantation can cure sickle cell. But few adults received transplants because high-dose chemo was needed to kill off the patients’ own blood-forming cells — and their entire immune system — to prevent rejection of the transplanted cells, leaving them open to infection.
In the new procedure, patients receive immunosuppressive drugs just before the transplant, along with a very low dose of total body irradiation, a treatment much less harsh than chemo. Sickle cell disease is inherited. It primarily affects Africans and people of African descent, including about one in every 500 African Americans born in the U.S. The defect causes the oxygen-carrying red blood cells to be shaped like a crescent, or sickle.
The misshapen cells deliver less oxygen to the body’s tissues, causing severe pain and, eventually, stroke or organ damage.