Sickle cell disease (SCD) is the most common hemoglobin variant disorder in the world affecting millions of people worldwide. SCD is most common in people of African descent but also affects people in Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy.1 The overall prevalence of sickle hemoglobin in Africa ranges from 7-45% depending on the region. By contrast the overall prevalence of the sickle hemoglobin in the US is 1.5% among all births and 7.5% among black births.2 The prevalence of sickle hemoglobin in Haiti is between 8% and 15.2%.3,4
The sickle cell gene is inherited in two severities; sickle cell disease ([SCD] - 2 sickle genes) and sickle cell trait ([SCT] - 1 sickle gene). People with SCD have a life-threatening condition and people with SCT are carriers who live a near normal life. Mortality of African children with SCD before the age of 5 years ranges from 50-90% depending on the economics of the area.5 In comparison, the mortality rate of children born with SCD by age 5 is between 1-2% and falling, reflecting ongoing medical care available to sickle cell patients in the US.1 The prevalence of SCD in Haiti is about 0.6%6 which translates to about 1,500 children born each year with SCD. Based on data from Africa we estimate that approximately 75% of children born with SCD in Haiti die by age 5 years, before they are diagnosed and treated. The Sickle Cell Center of Haiti will change this tragedy.
The alarming death rate in Africa, Haiti, and parts of India is due to a lack of treatment resulting from patients not being diagnosed because of inadequate diagnostic testing methods. Modern, accurate diagnostic testing methods are expensive, require stable electricity, refrigeration, climate control, clean water, and are complicated to perform. Poverty limits patient’s ability to pay for testing which limits the testing methods clinics in underdeveloped countries can make available. Lastly, insufficient education of the lab staff in underdeveloped countries would make the introduction of modern methods difficult even in the absence of financial limitations.
As a result, modern testing is only available in a few, elite clinics in underdeveloped countries while most of the remaining clinics have no testing or offer antiquated, inaccurate testing methods for sickle cell. Therefore, accurate testing for sickle cell is needed that is cheap, fast, and easy, and does not require electricity, clean water, refrigeration, or climate control.