Sickle cell anaemia – Handling it

Sickle cell anaemia or sickle cell disease (SCD) is an inherited blood disorder in which the red blood cells form a sickle shape instead of the normal disc shape, they are not able to provide sufficient oxygen to various parts of the body.

When a person with normal genes has a child with a partner who has abnormal genes, they get a child with one normal gene and one abnormal gene. The child is a carrier, meaning that he/she does not have the full blown disease but carries the sickle cell trait. When two carriers have a child, chances are high that the child will inherit two abnormal genes, hence have the sickle disease.

When a red blood cell is defective, it forms the shape of a sickle. This shape does not allow it to flow through the capillaries smoothly and might block them. The disc shape of the red blood cell enables it to flow smoothly through the capillary, the smallest blood vessel in the body. When the capillaries are blocked, blood fails to reach all body parts properly. This may lead to pain in the limbs, chest and abdomen. The joints might also swell, intensifying the pain.

Anaemia is generally a deficiency disease in which the body fails to produce enough red blood cells. A person with SCD produces normal amount of red blood cells, but due to the abnormality of the cells, their health is usually highly challenged. They are prone to anaemia, and thus the fatigue, shortness of breath and dizziness due to lack of enough oxygen in the body cells. In severe cases, SCD may lead to organ failure, stroke or death.

The signs
The most common symptoms of sickle cell anaemia are:

• Anaemia
• Pain and swelling in the joints
• Damage to organs like the heart and kidneys
• Fatigue

In children, SCD can be difficult to cope with. Because there are no outward signs of pain, sometimes people don’t understand the suffering a child experiences. In addition, there are other ways these children’s lives are affected. For example, they may not go swimming with their friends if the pool is too cold. Also, frequent trips to hospital interrupt their lives. Such restrictions can really be frustrating to a child, but with proper care and support, the child can lead a normal life.

Your child should receive regular care from a specialist such as a haematologist (blood specialist) or at a specialty clinic. As a parent, it’s important to share your concerns and discuss any new symptoms or complications with your child’s health care team. In addition, work with your child’s school to ensure he or she is getting the help and support needed.

Emergency medical attention should be sought if a child develops the following symptoms:

• Very high fever
• Pain that isn’t relieved by medication
• Shortness of breath or trouble breathing
• Swollen hands or feet
• Extreme fatigue
• Severe headaches or dizziness
• Severe stomach pain or swelling
• Chest and muscle pain
• Jaundice or extreme paleness
• Painful erection in males
• Sudden change in vision
• Seizures
• Weakness or inability to move any part of the body
• Slurring of speech
• Loss of consciousness
• Numbness or tingling
• Avoiding triggers

Most pain episodes do not have an obvious reason for their occurrence. But there are some of the possible triggers that your child should avoid such as:

• Very cold water and exposure to cold. weather
• Getting too hot (extreme temperatures)
• Getting dehydrated
• Colds and infections
• Lack of enough sleep and rest
• Emotional stress
• Environments with low oxygen such as high altitudes and non- pressurized airplane flights
• Being around tobacco smoke

Treatment

Dr Awiki, a physician at Meru District Hospital says there are measures that can prolong life of SCD sufferers, whose life expectancy is usually 30 years. ‘A child with SCD is advised to take lots of fluids, because dehydration increases chances of blocked vessels that may lead to painful episodes,’ he says.

Diet: Give foods rich in iron like: red meat, beet root, spinach, tomatoes and carrots to assist the body produce blood. Iron tablets can also be prescribed as a supplement. All these help reduce the chances of anaemia. Ensure your child gets plenty of rest, and avoids extreme temperatures.

Blood transfusion: Normal haemoglobin level in blood is more than 12. When it gets to 5 or less, blood transfusion is necessary.

Medication: A child with severe joint and abdominal pain will be prescribed painkillers. Parents are advised that regular visits to a comprehensive sickle cell program or a paediatric haematologist and strict compliance to antibiotic administration are crucial to the health and future well-being of the child.

Bone marrow transplant: Dr. Awiki states that in Kenya, sickle cell anaemia has not been researched
thoroughly; perhaps because it is not wide spread. ‘Treatment is not advanced. Due to lack of knowledge, symptoms may be confused for other diseases, resulting in wrong diagnosis,’ he says. In developed countries, extensive research has lead to a discovery that a special bone marrow transplant can completely cure SCD. Bone marrow transplant has been tried on a number of patients and has proven successful.

Testing

While studies show that a child born with two abnormal genes has complications from very early in life, nothing has been established on how the beginning of SCD can be stalled. In developed countries, children can be tested for SCD at birth to determine if they are carriers or have SCD. Some tests can be done while a woman is pregnant, especially if there is a family history of the disease.

With sickle cell anaemia’s prevalence being higher in some parts of the country, intermarriage is said to lower the possibility of two carriers meeting and having children together, thus reducing the number of carriers in one region.

Dr. Awiki enlightens that SCD carriers lead normal lives, unless they have other health conditions that may aggravate SCD symptoms. They may also have mild and spaced episodes that may disappear or be reduced by medication.

Since there is no definite cure, the best solution is getting tested for the SCD traits before committing to child bearing responsibilities.

END: BL 34 /18-19