As reported by the New Britain Herald, June 13, 2010.

Area Group Looks to Raise Sickle Cell Anemia Awareness

By Scott Whipple

NEW BRITAIN ó Most anniversaries, especially centennials, are reason to rejoice. However, for patients facing sickle cell disease the fact that it was discovered in America in 1910 seems little cause to break out the champagne.

According to the University of Connecticut Health Center, sickle cell disease is a disorder of hemoglobin, a molecule abundant within the circulating red blood cell. Hemoglobin is responsible for the red blood cellís ability to deliver oxygen to the organs. People with sickle cell disease have a hemoglobin abnormality that distorts the shape of their red blood cells, causing them to be stiff and rigid. This leads to impaired circulation and oxygen delivery to the tissues, eventually leading over time to widespread organ dysfunction and severe pain.

"Complications include severe recurrent pain, stroke, lung, kidney, liver or spleen dysfunction, chronic skin ulcers, and blindness," Dr. Biree Andemariam, who started a sickle cell disease program at the UConn Health Center, told UConn Health Centerís Chris DeFrancesco. "Even pregnancy can be risky. Median survival is around age 40."

Earlier this year, Andemariam was named one of the first recipients of a Connecticut Institute for Clinical and Translational Science Award for Mentored Research for her study of sickle cell disease. She says approximately 80,000 people in the United States and millions worldwide have sickle cell disease. It has a higher prevalence in those of African, Hispanic, Caribbean, South American, Mediterranean, and Indian ancestry. The New Britain-based group Citizens for Quality Sickle Cell Care is a nonprofit partner in the health centerís new adult sickle cell disease program. The program also includes clinical trials and lab research.

Previously, most adults with sickle cell disease used emergency departments for routine care. Now they have a place to call home.

"Bone marrow transplantation is curative, but very risky, and new therapies are virtually non-existent," Andemariam explains. "Most treatment is palliative, not preventative; we have a long way to go. This is why we conduct research in addition to providing comprehensive care. We want to develop novel therapies and perhaps even cures."

Until then, a good defense against sickle cell disease is education. Itís possible for a mother and father who donít have sickle cell disease to have a baby who does.

"Everyone has two copies of hemoglobin DNA," Andemariam says. "If you inherit one abnormal, or sickle cell copy, you have the trait, not the disease. If you inherit two abnormal copies, you have the disease. Sickle cell disease trait can be carried by an individual and not cause disease."

Still, it can cause disease in offspring. The child of two carriers of the sickle cell trait has a one-in-four chance of being born with sickle cell disease.

Andemariam is partnering with New Britain-based Citizens for Quality Sickle Cell Care and the Department of Public Health to encourage screening and raise public awareness about potential risks associated with being carriers of the sickle cell trait.

In a cramped, second floor office on Arch Street Delita Rose-Daniels of Citizens for Quality Sickle Cell Care, patiently explained to a visitor why her agency feels a need to celebrate 100 years of the disease.

"Because there is a bright side," Rose-Daniels exclaimed. "Itís a dark illness, but in the last hundred years there has been progress in life expectancy and in quality of life."

Citizens was founded in 2001 in New Britain by Victoria and Olusegun Odesina. Their daughters Lola, 19, and Sola, 17, have struggled with the disease since childhood. Sola suffered a stroke when she was less than two years old. Though the two young women are still living with the disease they have been able to carry on normal lives.

Hospital for Special Care donated the office space on Arch Street. At the time there was nothing for sickle cell patients in central Connecticut. The agency also got help from State Sen. Don DeFronzo, D-New Britain.

"More people are living longer with the chronic disease," De Fronzo said. "So, we introduced a bill to establish a center. The funds never got on the appropriation, but we worked with the Hospital for Special Care. There is definitely a need for it in the state."

"Sickle cell doesnít have the large number [of victims] other chronic diseases have," said Delories Williams-Edwards, executive director, Southern Regional Sickle Cell Association. "However, it is prominent in populations of African, Greek and Italian descent."

Patient Advocate Rose-Daniels said everyone in the New Britain office, including executive director, Virginia Pertillar, are trying "to rework the community and resurrect the topic of sickle cell."

The disease rarely gets the media attention other diseases get. Some physicians lack an understanding of its severity.

Citizens offers free screenings for potential patients and wants to do more precautionary work.

"We want to develop a documentary highlighting sickle cell over the past one hundred years," Rose-Daniels said. "Weíd also like to do a reality-based story of three people with sickle cell, following their lives so you can see the different faces of the disease."

The agency would like to develop an educational program for the school systems. These objectives require money. Recently, Citizens held its annual fund-raising dinner and silent auction at Central Connecticut State Universityís Memorial Hall. Keynote speaker was Janis Spurlock-McClendon, an adult living with sickle cell anemia.

Rose-Daniels believes Citizens is starting to get the word out. Still, work remains to be done.

"We need to teach young adults to start asking the questions they need to ask, not only about sickle cell, but for HIV," she said.