According to doctors, parents who carry the sickle cell trait have a 1 in 4 chance of their offspring inheriting sickled hemoglobin. While the odds are only 25% the risk is still great because there are no guarantees that the child will not receive abnormal hemoglobin genes from his or her parents.
Pamela and Chikezie, were surprised when their daughter was born with sickle cell disease. Here’s their triumphant story:
When Elechi was born, routine testing revealed she suffered from sickle cell disease; individuals with sickle cell disease have red blood cells that may be shaped like crescents instead of discs. Instead of moving smoothly through the circulatory system, the sickle-shaped cells clog blood vessels, triggering episodes of extreme pain and causing other dangerous complications that range from organ damage to stroke to pneumonia.
Her parents, Pamela and Chikezie, were shocked. “We cried for days,” said Pamela. After all, their older son, Chinua, was healthy, and they had been assured by a genetic counselor that, even though they both carried the sickle cell trait, chances were low they’d have a child with the full-blown disease.
Elechi’s parents didn’t know where to turn, but their pediatrician guided them to St. Jude Children’s Research Hospital®. “St. Jude was the calming part in all the chaos,” Pamela said. “We met with some incredible doctors, who informed us so much about the disease.” St. Jude is a global leader in finding cures and saving children from cancer and other deadly diseases.
For the next few years, Elechi received penicillin every day to keep infection from developing in her little body. When Elechi was 3 years old, she began receiving a drug, which she continues today, called hydroxyurea to help her body produce healthier cells.
When Elechi turned 5 years old, she was able to stop the daily doses of penicillin. St. Jude continues to monitor Elechi closely, and Elechi looks forward to her checkups. She plays violin, practices Taekwondo and can often be found reading aloud to her stuffed animals.
Many patients with Sickle Cell have fewer pain crisis when they take hydroxyurea. Hydroxyurea increases the fetal hemoglobin in patients and reduces white blood cell production, which may lessen tissue damage and inflammation (Source). According to Dr. Jeremie Estepp pediatric physician at St. Jude Children’s Research Hospital, Hydroxyurea also decreases hospitalizations, acute chest syndrome, dactylitis, the need for blood transfusions, etc (source).
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Stjude.org. Living Well with Sickle Cell. Retrieved September 15, 2014 from http://www.stjude.org/stjude/v/index.jsp?vgnextoid=16a17656bb3a1410VgnVCM100000290115acRCRD&vgnextchannel=d6bd3aabdae91410VgnVCM100000290115acRCRD&SearchUrl=search_results.jsp&QueryText=elechi
Stjude.org. Hematology. Retrieved September 15, 2014 from http://www.stjude.org/stjude/v/index.jsp?vgnextoid=9657f3f7d210c310VgnVCM100000290115acRCRD&vgnextchannel=7616f2919c9ab310VgnVCM100000290115acRCRD&SearchUrl=search_results.jsp&QueryText=hydroxyurea
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