People with sickle cell disease need comprehensive healthcare; the disease comes with many complications that can be anticipated, prevented and managed effectively. Many patients are living full and healthy lives.
Children are born with the sickle cell disease; it is not contagious. It is passed down from parent to child when both parents have the sickle cell trait “AS”, or one parent has sickle cell disease “SS”, and the other parent has the trait “AS”.
Sickle cell disease (SCD) is an inherited disorder of the protein haemoglobin in the red blood cell that affects millions of adults and children around the world. It is caused by a genetic mutation in the body system that makes it produce a different kind of haemoglobin called haemoglobin S, and this causes a change in the shape of the red blood cell from its normal disc-shape to being sickled in shape giving it its name “sickle” cell disease. These misshaped cells break down easily causing anaemia, and they live for only 10-20 days instead of the normal 120 days.
To be infected with the sickle cell disease, you need to have two altered haemoglobin genes; one from each parent. If you have only one of these altered genes, you will be referred to as a carrier of the disease. Nigeria has the highest burden of the Sickle Cell Disease in the world where the trait occurs in 25% to 30% (40 million) of the population. About 150,000 babies are being born every year yearly with this disease with infant deaths of about 100,000, which represents 8% of child mortality in the country. Testing for sickle cell disease can be done in the newborn (HPLC, IEF) or through haemoglobin electrophoresis (genotyping).
The hallmark of the disease is anaemia (decrease in red blood cells). Complications of the disease include pain crises, infection, stroke, kidney and liver dysfunction, and can also affect the lung, spleen and heart. Due to the high mortality rate and complications of the disease, many developed countries have adopted comprehensive healthcare for SCD and the mortality reduced from 16-30% to less than 1%. In 2003 the University of Benin teaching hospital also adopted this extended health care for the management of their SCD patients with similar results.
Infections, pain, crises, anaemia and damage to body Organs are the most common complications of sickle cell disease. You should contact your doctor when you or your child/ward has a fever, chest pain, Jaundice (Eye/Skin looking yellow) or headache/dizziness.
Nisa Premier Hospital recently launched the Sickle cell disease clinic during the Sickle Cell Disease Awareness Day on June 18th, 2016 to better care for our patients with SCD. The clinic which has adopted the comprehensive healthcare management officially opened on the 10th of August 2016. The aim of the clinic is early detection and prevention of the complications of the disease, though:
- Parent and patient counselling and education
- Routine medical care for the SCD patient through regular medical follow-up
- Immunizations (routine and additional vaccines for patients with SCD)
- Routine laboratory testing
- Prophylaxis for malaria and bacterial infections
- Early identification of fever/infection and its management
- Vitamins (Folic Acid and Vitamin C)
Some of the services we currently offer at Nisa include:
- Pre-implantation genetic diagnosis (PGD) for single cell genetic defects
- Testing for sickle cell disease through haemoglobin electrophoresis (genotyping)
- Sickle Cell Disease Clinic: The clinic is run by the consultants at a discounted price on consultation and some laboratory tests. The clinic is by appointment every Wednesday from 9 am to 4 pm at the Congo building outpatient department (Adults) and Lieberman OPD (Paediatrics).
At Nisa Premier Hospital, our goal is to provide the best comprehensive care for our sickle cell patients that will afford them the full and healthy life that they deserve. We look forward to seeing and serving you at our clinic!
For all enquiries on sickle cell and Anaemia, please call: 08090457744, 08174210023.