Get screened for sickle cell disease

Sickle cell disease is an inherited disorder in which red blood cells are abnormally shaped. Normal red blood cells are soft and round and can move through small blood tubes in the body to deliver oxygen.

Sickle red blood cells become hard, sticky and shaped like a crescent. They do not last as long as normal, round red blood cells, which leads to a low blood count. The sickle cells also get stuck in blood vessels, blocking blood flow and this can cause pain and damage to most organs, including the spleen, kidneys and liver.

Persons inherit the abnormal haemoglobin from their parents, who may be carriers with sickle cell trait or parents with sickle cell disease. You cannot catch it. You are born with the sickle cell haemoglobin and it is present for life. If you inherit only one sickle gene, you have sickle cell trait. If you inherit two sickle cell genes you have sickle cell disease. Sickle cell anaemia is present at birth, but many infants do not show signs until after four months old..

The signs and symptoms of sickle cell anaemia vary. Some people have mild symptoms, others have very severe symptoms and are often hospitalised for treatment. The most common signs and symptoms are linked to anaemia, low blood count and pain.

Sickle cell anaemia has no widely available cure. However, there are treatments for the symptoms and complications of the disease. Bone marrow transplants may offer a cure in a small number of cases. Due to improved treatment and care, most children born with sickle cell disease now live into middle age and often well beyond.

A critical time is the first few years of life, which is why early diagnosis and treatment are so important. Medicines are available to help manage the pain and immunisations and daily doses of antibiotics can help prevent infection, which was the cause of many deaths in infants and young children.

A simple blood test can be done by a doctor, which will tell if you are a carrier of the sickle cell trait or if you have the disease. Newborn babies are routinely tested for the disease and it is also possible for the test to be done during the first few months of pregnancy.

The Ministry of Health and Wellness offers screening to all pregnant mothers and newborn babies at all public health facilities, at no cost to the family. Early screening affords medical practitioners the opportunity to determine whether or not the child has the sickle cell disease or if the child is a carrier, that is, they have the sickle cell trait.

The health ministry offers four doses of pneumococcal vaccine and a further booster dose of Haemophilius influenza type B to children who are sickle cell-positive. These vaccines protect patients against pneumonia, meningitis (infection around the brain) and septicaemia (infection of the blood).

Sickle cell disease is a common genetic disorder in Jamaica. One in every 150 persons has the sickle cell disease and one in every 10 persons has the trait. This means that persons can have the trait and do not have any symptoms.

While a cure is yet to be discovered, science and technology has come a far way in developing and improving treatment options and care. Today, persons with sickle cell disease or the trait of the disease, if detected early, can live longer, healthier lives.

SYMPTOMS OF SICKLE CELL ANAEMIA

• Hand-Foot Syndrome – Often the first sign of sickle cell disease. It is caused by a lack of blood flow to the hands and feet.

• Episodes of pain – Referred to as a ‘crisis’, these episodes of pain occur when blood flow is blocked to the chest, abdomen, and joints. The frequency and duration of the episodes vary from person to person, but in severe cases, they can result in hospitalisation.

• Frequent Infections and fever – Sickle cell can cause damage to the spleen, an organ that fights infection, making those with sickle cell at greater risk of developing an infection and an accompanying fever.

• Changes in skin – People with sickle cell disease can develop a yellow tint to their skin or the whites of their eyes. Skin and nail beds can often become pale.

• Delayed growth – By not receiving enough oxygen rich red blood cells, those with sickle cell disease may also not get the necessary nutrients essential for growth.

Sources: Ministry of Health and Wellness, National Health Fund, The Sickle Cell Unit | Caribbean Institute for Health Research