The World Sickle Cell Day is a United Nation’s recognized day to raise awareness about sickle cell disease (SCD) at a national and international level. On 22nd December 2008, the UN General Assembly adopted a resolution that recognizes sickle cell disease as a public health issue and “one of the world’s foremost genetic diseases.” The resolution calls for UN member states to raise awareness about sickle cell on June 19th of each year.
In this article, I would be creating awareness on sickle cell disease, the causes, symptoms, treatment and prevention.
What is sickle cell disease (SCD)
Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by inherited abnormal hemoglobin (the oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells.
SCD is more common in certain ethnic groups, including:
· People of African descent,
· including African-Americans (among whom 1 in 12 carries a sickle cell gene)
· Hispanic-Americans from Central and South America
· People of Middle Eastern, Asian, Indian, and Mediterranean descent
· Approximately 2000 infants are born annually with the disease
· SCD affects approximately 200,000 Americans annually
· 1 in 365 African Americans
· 1 in 13 African Americans have the traits (carrying only 1 of the gene, S)
(CDC August 2017, Mayo Clinic
Economics of SCD
10 years ago; Medical expenditure for children with SCD averaged $12,000 yearly for those with Medicaid and $15,000 yearly for those with commercial insurance.
There were also 113,000 hospitalizations costing over $500,000 paid by Medicare and Medicaid of which 75% of the visits were in adults and each with at least 3 Emergency Room visits per year. Children with SCD miss a minimum of 18 days per school year
Total healthcare costs nowadays for SCD is estimated at $2billion per year.
According to (David A.N et al 2018), ‘In Nigeria, the prevalence of SCD is 20–30/1000 live births. The burden of the disease has reached a level where it contributes 9–16% to under-five mortality in many West African countries. Hemoglobinopathies alone represent a health burden comparable to that of communicable and other major diseases’
Causes of SCD
Healthy red blood cells are round, and they move freely through small blood vessels to carry oxygen to all parts of the body. In SCD, the red blood cells become hard and sticky and look like a C-shaped called a “sickle” and they are not able to carry enough oxygen. When they travel through small blood vessels, they get stuck and clog the blood flow.
The sites most often affected by clogging or stacking of sickle cells are found in the lungs, liver, muscle, bone, spleen, eyes, and kidneys and other parts and tissues of the body: explains why patients complain of a lot of pain in these areas as the symptom of the disease.
Patients also have immunity suppression which leads to infections by bacteria, and viruses.
Symptoms of SCD include
· Excessive fatigue, irritability from anemia
· Jaundice (yellowing of eyes and skin), may also include retina damage
· Swelling and pain in hands, and feet, Pain in chest, back arms and legs, also damage of hip
· Frequent infections,
· Pain and problems in the spleen, (Nausea, vomiting, diarrhea)
· Delayed growth
· Stroke (20–30% of children stroke, 23% in African Americans)
· Genitalia (priapism, a constant erection, in which severe episodes may lead to impotency)
Treatment of Sickle Cell Anemia
Treatment of SCD pain or crisis is done in the following manner:
Rehydration: with IV fluids, helps Red blood cells return to normal shape
· Antibiotics: used to treat underlying infections. In some cases antibiotic prophylaxis, penicillins are recommended.
· Pain medications to treat acute pain
· Hydroxyurea: helps increase production of red blood cells
Immunization: Pneumococcal and Meningococcal vaccines have drastically reduced the rate of infections in SCD
Blood transfusion: improves oxygen and nutrients needed
Supplemental oxygen by mask makes breathing easier and improves oxygen levels in the blood
Bone marrow transplant: for severe complications and matching donors.
· Genetic counselling and testing (better before marriage and at pregnancy) can help prevent the likelihood of passing gene to your child
· Preventing infections can be achieved by practising simple hand washing techniques at every opportunity. Hand sanitiser gels and wipes are also available and affordable
· Immunisation is very important and one must assure shots and records are current to cut down on the rate of common infections.
· Re-hydration with fluids at all times is essential.
· Avoid staying in places with low concentration of oxygen, e.g. unpressurised air planes, or high altitudes
For more information about SCD, please speak to your Pharmacist or Doctor.
Article by Kunle Tometi a Pharmacist,Entrepreneur and Public Health Advocate. Kunle writes from the USA
· Sickle cell Disease: Public health agenda & Social, Economic and Health implications by CDR Althea M Grant, PhD September 2012
· Overview of the management & prognosis of sickle cell disease, Joseph Palermo, D.O.
· Economic impact of sickle cell Hospitalization. R Singh, Ryan Jordan and Charin Hanlon
· Prevalence and impact of sickle cell trait on the clinical and laboratory parameters of HIV infected children in Lagos, Nigeria