On 22nd December, 2008, the United Nations General Assembly adopted Resolution A/63/L63 that recognises sickle cell disease as a public health problem. Among the objectives of the resolution is the celebration on 19th June of each year as a World-Day of Sickle Cell Disease. On June 19 2009, the first sickle cell disease awareness day was held at the UN headquarters organised by the International Organization for the Fight against Sickle Cell Disease (OILD/SCDIO) in partnership with the World Health Organisation (WHO), the United Nations Children's Emergency Fund (UNICEF) and United Nations (UN). Activities included a video report on sickle cell anaemia, testimonies stand, exhibition by patients' associations and international NGOs, an art exhibition on the disease, panels of discussion and round table, a First Ladies meeting and a gala dinner.
The World Sickle Cell day is celebrated across the globe with special emphasis in African nations and Asia. The celebrations include a press, media campaigns, music shows, cultural activities, and talk shows. Since no known cure exists for sickle cell disease, the main goal is to prevent further complications due to sickle cell disease. The main emphasis is hence on educating medical professionals, care givers, and associated personnel about prevention, research, and resources to minimize the complications due to sickle cell disease. Hence June 19th is devoted mainly to spread awareness, through talks, seminars, pamphlets, literature and consultations. Sunday June 19, 2011 marks the Third World Sickle Cell Day.
What is Sickle Cell Disease?Sickle cell disease is an inherited blood disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent.
It affects haemoglobin, the protein found in red blood cells (RBCs) that help carry oxygen throughout the body. It occurs when a person inherits two abnormal genes (one from each parent) that cause their RBCs to change shape. Instead of being flexible and disc-shaped, these cells are more stiff and curved in the shape of the old farm tool known as a sickle — which is where the disease gets its name. The shape is similar to a crescent moon.
Common variants of the disease include SC, SD, SE, CE, Sβ thalassemia and SS. SS and SC are the most common in Nigeria.
Red blood cells with normal haemoglobin (haemoglobin A, or HbA) move easily through the bloodstream, delivering oxygen to all of the cells of the body. These are shaped like discs or doughnuts with the centres partially "scooped out" and are soft and flexible. They can easily "squeeze" through even very small blood vessels.
HbS molecules on the other hand tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape. Red blood cells containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently. Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body's tissues and organs of the oxygen they need to stay healthy. Unlike normal RBCs that last about 4 months in the bloodstream, fragile sickle cells break down after only about 10 to 20 days, which usually causes anaemia. Anaemia is what happens when the body's number of red blood cells (or amount of haemoglobin) falls below normal. People who are anaemic often feel weak, tire more easily, and may appear "run down."
Signs and SymptomsThe symptoms of SCD follow a general pattern, which includes predominantly jaundice and anaemia characterized by erythroid hyperplasia. Other very common symptoms of sickle cell anaemia based on organ damage are characterized by: painful crises, stroke, paralysis, avascular necrosis of the femoral head, loss of splenic function and pulmonary hypertension. Painful crisis can occur in the chest, stomach, arms, legs, or other parts of the body. This is caused by sickle cells blocking blood flow through the blood vessels in those areas.
People with sickle cell anaemia can also experience complications from impaired blood circulation and infection-fighting problems. These include a higher risk of certain infections as well as a condition called acute chest syndrome, which is caused by inflammation or trapped RBCs in the lungs. Sickle cell anaemia is not contagious, so you can't catch it from someone else or pass it to another person like a cold or an infection.
A child who has inherited the sickle cell gene from only one parent will not develop the disease, but will have sickle cell trait (these are people with the AS or AC genotypes.) People who have sickle cell trait don't have sickle cell anaemia or symptoms of the disease, but they can pass the sickle cell gene on to their own children. Because people with sickle cell trait don't have the disease, they may never discover that they carry the gene. That's why it's recommended that people who are unsure of their genotype status ask their doctors about testing.
The periods of pain commonly referred to as pain crises, vary in their severity, how often they happen, and how long they last. Whereas one person may have only one sickle cell pain crisis a year, another may experience them more often. Crises may be brief, or may last hours, days, or even weeks.
What Can Doctors Do?To diagnose sickle cell anaemia, doctors use a special blood test called haemoglobin electrophoresis to look for HbS in a person's blood. There is no cure for sickle cell anaemia, and it is possible for some people to be ill enough to die from the disease. Doctors can provide treatments that help prevent complications from the disease, though. Folic acid, a vitamin that helps the body produce new red blood cells, is often prescribed for people with sickle cell anaemia. Pain medications help relieve the symptoms of crises.
Some crises can be managed at home with pain medicines, rest, and extra fluids. But if a crisis is especially intense, one may need to go to the hospital for intravenous (IV) fluids and stronger pain medications. People with sickle cell anaemia may also use oxygen to help ease symptoms during a crisis or an episode of acute chest syndrome. People with sickle cell anaemia may need to get transfusions of healthy red blood cells to help carry oxygen to the tissues of their bodies more effectively, and some may need many transfusions on a regular basis. Several new treatments have helped reduce painful crises and episodes of acute chest syndrome in adults with the condition. These drugs have also decreased the need for hospitalisation.
What Can Patients Do?With the right precautions, people with sickle cell disease can do most of the stuff other people do. To stay as healthy as possible, they should take these steps:
• Eat a balanced, healthy diet.
• Take vitamins, including folic acid supplements, as prescribed. They should however
avoid iron supplements.
• Drink plenty of fluids to prevent dehydration.
• Avoid extreme cold or heat.
• Exercise regularly, but in moderation. Exercise is important for staying healthy, but
overdoing it can trigger a crisis in some people, particularly if they become dehydrated,
overheated, or exhausted.
• Get plenty of rest.
• Avoid alcohol, drugs, and smoking, which can aggravate sickle cell disease and its
symptoms. Some people with sickle cell disease are prone to lung problems, so smoking
is particularly risky and must be avoided.
• Avoid places low in oxygen. (For example, it's not a good idea to go hiking at high
altitudes or spend lots of time swimming under water or in the air in planes.)
• Prevent serious infections by contacting your doctor as soon as illness symptoms start. Be
sure to get any immunisations (such as pneumonia and flu vaccines) that the doctor recommends, and always call your doctor if you have a high fever (over 101° Fahrenheit).
• Learn as much as you can about the disease and see your doctor regularly to help prevent
complications.
People with sickle cell disease may need to put some limits on their lives, but with the help of doctors, friends, and family, they can manage the condition and live their lives to the fullest.
Over 5 million people are known to be afflicted by the Sickle Cell Disease in Nigeria. Sickle-cell anaemia contributes the equivalent of 5% of under-five deaths on the African continent, and up to 16% of under-five deaths in individual West African countries (WHO, 2006). The statistic in Nigeria though revealing may be even more due to unavailability of adequate statistics in a nation like ours. While an average of 150,000 babies are born annually with the disorder, only 25% of them might make it to their 5th birthday. It’s important to note that Nigeria being the most populous country in Africa accounts for three quarters of the populations living with SCD. It is therefore imperative, that we consider the alarming rate at which the population of those living with SCD is increasing.
People living with SCD, apart from the painful crises may experience depression, low self esteem and stigmatisation. Parents abandon children, marriages are destroyed, relationships are broken, those employable are stigmatised all as a result of SCD. Statistics predicts that by the year 2015, if care is not taken, the population of people living with SCD would have hit 40 million. It is not uncommon to have people tag the disease ignorantly as a death sentence.
Nigeria being a vast country and as a result of inadequate health information, basic elements of knowledge and information have been reduced to a minute percentage of the populace, thereby rendering the majority of the population ignorant. Counselling, research and management of the disease is also severely limited. The deficiency of genotype awareness and testing, inadequate understanding of basic management needs; poor health care and treatment in our hospitals has further created an abyss in solving the problems associated with SCD.
In Nigeria, the medical management of the sickle cell anaemia is achieved by the administration of some routine drugs which have been shown to be able to improve the quality of life of the patients. Apart from Folic Acid as mentioned above, other commonly prescribed drugs for sickle cell anaemia in Nigeria include anti-sickling agents like: Niprisan, Ciklavit and pain medications, which could include but not limited to; Paracetamol, Aspirin, Feldene, to stronger medications like Morphine, Fortrin, Buscopan depending on the physicians prescription. Several Nigerian families and caretakers of SCD patients have resorted to untested herbal and unreliable drug options to get by which are much cheaper than medically clinically tested drug solutions.
More specifically, people in the lower strata of the society do not have easy access or funding towards proper treatment. In Nigeria the poverty rate stands at about 50-60% (World Bank report 2006). However, the average cost of medication on a monthly basis for the effective management in a person living with SCD is about $60. This places most Nigerian families at a disadvantage in being able to purchase the basic drugs. There is therefore an urgent need to facilitate proper medical care through drug provisions at subsidised rates to provide a healthy and enabling environment for their survival. Continuous awareness must also be maintained through the mass media, the various Sickle Cell Clubs across the country, NGOs and religious organisations. Funding for research into viable treatment procedures and drugs must be made available for our research institutes, teaching hospitals and universities. Regular seminars should also be organised for General Medical practitioners to update their knowledge on the disease.
