Notes From The Field: Tracking An Invisible Killer Of Children

April 25, 2011

Body

Sickle cell disease (SCD) is a serious blood disorder that is passed down through families. When children inherit SCD from their parents who have sickle cell trait, the red blood cells form an abnormal crescent (sickled) shape. As a result, the blood doesn't flow well, causing anemia, pain and damage to the internal organs. In the United States, all babies are tested for SCD at birth, which allows them to receive early care and treatment. As a result, most children with SCD in the U.S. will live into adulthood with an opportunity to lead productive lives.

Worldwide, over 300,000 children are born with SCD each year, mostly in Africa and India. For some countries in Africa, like the Republic of Angola, the impact of SCD is staggering. A high birth rate, coupled with a high prevalence of the sickle cell trait, suggests up to 10,000 new babies are born with SCD each year in Angola. Although most of these babies are never diagnosed with SCD, they suffer serious illness or death. The best estimate from Angola's largest children's hospital is that only 10-20% of the children born with SCD ever reach their 5th birthday. The rest of these children die of complications of SCD — a disease their family never knew they had.

Through an exciting partnership with the Ministry of Health in Angola and Chevron Oil Company, the Texas Children's Center for Global Health will soon initiate a bold new program to test and treat babies for SCD in Angola.

So, how exactly will the program work?

  1. First, blood will be collected from newborns via heel stick.
  2. Next, the blood is placed on special cards to allow samples to be collected at different locations.
  3. The cards are then transported to a central laboratory which has the right equipment to test the blood and diagnose SCD.
  4. When the initial screening results have identified a baby with probable SCD, that baby's family must be located and the baby brought to special SCD clinic for initial medical evaluation. It is anticipated that this "retrieval" step, which requires locating and tracking down a family, will be the most challenging and will require dedicated personnel to find each baby and bring them to medical care.

A critical piece of this newborn screening program for SCD includes early and ongoing education to families about the disease. Educational materials similar to those used in the United States are currently being produced and translated into Portuguese. Once a baby is established in care and their family has been educated about this serious disease, treatment can begin. Babies affected with SCD will be given a course of treatment that includes twice daily antibiotics to prevent infections, additional special vaccines, and possibly a medicine called hydroxyurea to reduce the symptoms of SCD.

We expect to begin this program in the summer of 2011. There are always challenges to working in a new country, including one like Angola with its language and cultural differences. However, if a program like this is successful, thousands of babies with SCD will live instead of die. Through this new sickle cell initiative, Texas Children's Center for Global Health hopes to extend care, treatment and research to more children with SCD around the world.

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