Frequently Asked Questions

Sickle cell anaemia is a life-long inherited disorder of haemoglobin (Hb). Hb is an essential substance within the red blood cells, as it carries oxygen from the lungs to all parts of the body. The usual Hb is known as HbA but there are many variant haemoglobins, of which, the most common is the sickle haemoglobin (HbS). Sickle cell anaemia occurs when a child inherits HbS from each parent and thus would have HbSS.

The most frequent symptom is pain, often occurring without apparent cause, in the bones of the back, the chest and the limbs. The commonest sign is pallor of the palms of the hands and soles of the feet, signifying anaemia. Yellowness of the white area around the pupils of the eyes also occurs occasionally and is known as jaundice.

In early childhood, usually before the age of 4 years, the hands and feet including fingers and toes may become swollen and painful. This is known as the Hand - Foot syndrome and is often the first sign of the disorder in young children. Fever (high body temperature) can occur when there is infection and about one in ten children can suffer a stroke.

An intriguing feature of sickle cell anaemia is the fact that the severity and frequency of known symptoms can vary greatly from person to person and also from time to time in the same person.

Sickle cell crisis is mostly used to describe the pain that comes on suddenly and can range in severity from mild to very severe. It may last from a few hours to a few days.

The goal of management is to ensure a normal quality and duration of life in each patient. As soon as the diagnosis is made it is important for the parents or the person affected to receive expert genetic counselling. This would explain the probable course of the disorder and recommended preventive measures and how to detect complications that would require urgent medical attention. This is why it is best to have newborn screening so that the diagnosis is known even before the parents realise it is too late to prevent deadly infection or complication. As it is known that the affected young children are prone to deadly pneumococcal and malarial infections, these two infections are prevented, particularly in young children.

Pneumococcal infection is prevented with prophylactic oral penicillin taken twice daily and pneumococcal vaccines given from the first few months of life. Children with a risk of developing strokes can be detected by special brain ultrasound screening so that preventive measures can be applied.

Oral hydroxyurea (hydroxycarbamide) can be safely given under medical supervision, even from childhood to reduce the frequency and severity of sickle cell symptoms and prevent complications including strokes.

Folic acid supplementation is also given throughout life to help cope with the effects of the high turnover of red blood cell production. It is important for affected persons to attend dedicated Sickle Cell Clinics for follow up 3 to 4 times a year and to know when and where to report serious symptoms to.

It is important for us to build the capacity of health care workers to apply standard modern care practices to the management and treatment of sickle cell disorder. Apart from appropriate professional continuing education, a cadre of Specialist Sickle Cell Nurses, as they have in the UK and USA, should be trained and deployed to supplement our capacity to manage the disorder.

About one in four Nigerians is a healthy carrier of sickle haemoglobin and has HbAS while about one in fifty (2%) children are born with sickle cell anaemia. About one million persons may be living with sickle cell anaemia in Nigeria today.

It is diagnosed by laboratory tests that are designed to detect the ability of red blood cells to assume a sickle shape under certain conditions. Furthermore, the haemoglobins in a blood sample are distinguished by a process known as electrophoresis.

You should seek and obtain expert counselling on your disorder and attend a Sickle Cell Clinic, even when feeling well, about thrice a year. There are helpful books to read such as one we have written for affected persons, titled "How To Live With Sickle Cell Disorder". Avoid health risks such as smoking and drink no more than the occasional alcoholic beverage in moderation. Avoid identified triggers of sickle cell crisis.

The average life-expectancy depends on the environment and the health care facilities available to them. This has been clearly demonstrated in the United States of America where the average life-expectancy was 7.4 years in 1974 but after the introduction of sickle cell centres and appropriate care programmes, the life-expectancy increased to 53 years in 2003 and may now be close to 60 years.

In Nigeria it was shown about four decades ago that in unprotected malaria areas only a handful of affected children lived up to 10 years and this improved after some malaria control measures were introduced to the surveyed villages. Now we have people with access to good care in Nigeria living to old age and becoming parents and grandparents themselves but these are relatively few as most of the affected persons don't have access to appropriate care. We do not yet have the data to accurately estimate the average life-expectancy in Nigeria.

Unlike acquired communicable disorders such as small pox, polio and HIV that are preventable by the application of specific interventions and life-style changes, inherited non-communicable diseases such as hypertension, diabetes, albinism and sickle cell anaemia, with our present state of knowledge, are not similarly amenable to prevention.

Hence while the deadly small pox has been eradicated world-wide by vaccination, sickle cell anaemia exported to America with African slaves five centuries ago still very much exists there.