In commemoration of this years world sickle cell day the sickle cell foundation Nigeria made it known that “Nigeria stands out as the most sickle cell disease endemic country in Africa and globally.. with an annual infant death of 100 000 representing 8% of infant mortality, while those that survive suffer end organ damage”. This necessitated the call for Government and stakeholders to invest resources and effort in advocacy, awareness education, genetic counseling, newborn screening, research to profer solutions for care.
Sickle Cell Disorder (SCD) management begins with an awareness and sensitization as the disease condition is an inherited condition. Sickle cell disorder is a genetic blood disorder caused by a mutation in the hemoglobin gene, leading to the production of abnormal hemoglobin S (Hb S). This results to crescent shaped red blood cells that can obstruct blood flow.
“Hope Through Progress: Advancing Sickle Cell Care Globally” is the theme for 2024, highlighting the importance of partnerships and government policies to reduce stigma , ensure adequate care for the affected millions affected by sickle cell disease. The United Nations General Assembly on December 22, 2008 adopted June 19th as United Nations sickle cell day recognizing the disease as a public health problem.
In the University of Port Harcourt Teaching Hospital, the department of Haematology and Blood Transfusion has been in the front line for the management of the disease condition and the campaign for awareness. The Head of Department (HOD), Dr Uchechukwu Prince Okite, giving insight on the condition said sickle cell disorder is an inherited disorder known as sickle cell. It affect the shape of red blood cells which carry oxygen to all part of the body. A person will be born with sickle cell disease only if two ‘Hb genes S” are inherited, i.e one from the mother and one from the father, but a person who inherits just one gene is healthy and said to be a ‘carrier ‘of the disease..
Symptoms such as
- Vision problem; where tiny blood vessels that supply blood to the eye become plugged with sickle cells, this can damage the portion of the eye that processes visual image. 2. Anemia; sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days living a shortage of red blood cells, this is known as anemia. without enough red blood cells the body can’t get enough oxygen. 3. Delayed growth or Puberty; Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers.
4.Episode of pains: Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anaemia. Pain develops when sickle- shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints.
The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay. 5. Swelling of hands and feet; sickle shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell.
Management of sickle cell disorder: is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications as noted by Dr. Okite.
Treatments might include medicines and blood transfusions. For some children and teenagers, a stem cell transplant might cure the disease.
The clinic in UPTH runs weekly, every Thursday 8:00am to 4:00, while admission run 24/7.
Advocacy is aimed at encouraging people to ensure they go for genotype test before marriage. And for people with genotype AS not to have children with same AS partner.
This year commemoration had the cordinating Minister of Health and Social Welfare Prof. Muhammad Ali Pate through the director of public health Dr. Chukwuma Anyaike at a briefing stating that the government is instituting policies and intervention taking into account genetic counseling diagnosis, newborn screening and comprehensive care package for SCD within the primary health care level with in built cost effective referral system.
Advocacy from physicians and stakeholders is to continue to call for support for increased research, improved access to health care and better treatment options especially in Africa and Nigeria where SCD has been endemic and a public health concern
