Sickle cell anaemia is caused by a mutation (an abnormal change) in the gene that instructs
the body to produce haemoglobin. The sickle cell gene is inherited (passed on between
family members). To get sickle cell anaemia you have to inherit the defective gene from both parents.

If two people with the sickle cell trait have a child, there's a one in four chance
that the child will be born with sickle cell anaemia.

What is Sickle cell?

Introduction

Sickle cell anaemia is a serious inherited blood disorder where the red blood cells, which carries oxygen around the body, develop abnormally. It affects the red blood cells, causing episodes of sickling, which produces episodes of pain and other serious symptoms. 


Normal red blood cells are flexible and round-shaped, but in sickle cell anaemia they can become rigid and shaped like a crescent (or sickle).The sickle-shaped cells contain defective haemoglobin, which is the iron-rich protein that enables red blood cells to carry oxygen from your lungs to the rest of the body. 

 
The abnormal cells are also unable to move around as easily as normal shaped cells and can block blood vessels, resulting in tissue and organ damage and episodes of severe pain. Such episodes are known as a sickle cell crisis or a vaso-occlusive crisis. They can last from a few minutes to several months, although on average most last five to seven days. The abnormal blood cells also have a shorter lifespan and aren't replaced as quickly as normal blood cells. This leads to a shortage of red blood cells, known as anaemia. Symptoms of anaemia include lethargy (a lack of energy), tiredness and breathlessness, particularly after exercise. 


 
What causes sickle cell anaemia? 

 

Sickle cell anaemia is caused by a mutation (an abnormal change) in the gene that instructs the body to produce haemoglobin. The sickle cell gene is inherited (passed on between family members). To get sickle cell anaemia you have to inherit the defective gene from both parents. 
 
If you only inherit the gene from one parent, you have what's known as the sickle cell trait. It's likely that your blood will contain some sickle cells, but you'll be able to produce normal haemoglobin and won't usually experience symptoms. However, you'll be a carrier of sickle cell anaemia and may pass the abnormal gene on to your children. Those who inherit the gene from both parents do not have this protection. In addition, they suffer from severe effects of SCD and many die before they reach reproductive age.
                                        

If two people with the sickle cell trait have a child, there's a one in four chance that the child will be born with sickle cell anaemia. 

 
Nigeria:

 

Nigeria has an estimated population of 178 million with an annual growth rate of 3.2%. About 25% of adults have the sickle cell trait, AS. This suggests that there is a potential growth with the number of babies who will be born with sickle cell disease. 

The Sickle Cell Foundation Nigeria records that around 150,000 babies are born with sickle cell disease each year.
 

According to the WHO, the geographic distribution of the sickle-cell trait is very similar to that of malaria. The sickle cell trait has a partial protective effect against malaria, and this may explain why it has been maintained at such high prevalence levels in tropical Africa.  
 

Resources

What is Sickle Cell?
 

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A Parent's Guide To Managing Sickle Cell

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Did you know..
(Ages 11-16)

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Let's talk about
sickle Cell

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A Guide to School

Policy (June 2016)

.

Guide To Sickle Cell
& Employment

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SCD in Childhood
 

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Did you know..
(Ages 5-10)

.

Sickle Cell &
Employment (2020)

.

Common Complications

Why are my eyes yellow?

Jaundice is caused by the build-up in your body of a yellow substance called bilirubin.

 

It’s normal in people with sickle cell disease as the red blood cells break up more regularly than people who do not have sickle cell.

JAUNDICE

People with sickle cell disease are more vulnerable to infections, particularly when they're young.

Infections can range from mild, such as colds, to much more serious and potentially life threatening, such as meningitis.

 

Vaccinations and daily doses of antibiotics can help reduce the risk of many infections.

INFECTIONS

Nearly all people with sickle cell disease have anaemia, where the haemoglobin in the blood is low. Haemoglobin is the substance found in red blood cells that's used to transport oxygen around the body.

This can lead to a sudden drop in the number of red blood cells and may cause additional symptoms, such as headaches, a rapid heartbeat, dizziness and fainting.

It's usually treated with a blood transfusion.

FATIGUE & ANAEMIA
 

Delayed puberty is when:
 

  • boys have no signs of testicular development by 14 years of age

     

  • girls have not started to develop breasts by 13 years of age, or they have developed breasts but their periods have not started by 15

DELAYED
PUBERTY

 

(ACS) is a serious lung condition which can cause a fever, coughchest pain and breathing difficulties

ACS is a term used to cover conditions characterized by chest pain, cough, fever, hypoxia (low oxygen level) and lung infiltrates. Acute chest syndrome may be the result of sickling in the small blood vessels in the lungs causing a pulmonary infarction/emboli or viral or bacterial pneumonia.

ACUTE CHEST
SYNDROME

 

Eyesight problems, such as floaters, blurred or patchy vision, reduced night vision and occasionally sudden vision loss

Sickle cell retinopathy is a major ocular complication of the sickle cell disease (SCD) which causes permanent loss of vision. Retinopathy can occur in sickling hemoglobinopathies like sickle cell disease, sickle cell C disease, and sickle cell thalassaemia disease

EYESIGHT
 

STROKE OR T.I.A 
(TRANSIENT ISCHAEMIC ATTACK)

A transient ischaemic attack (TIA) or "mini stroke" is caused by a temporary disruption in the blood supply to part of the brain.

The disruption in blood supply results in a lack of oxygen to the brain.

This can cause sudden symptoms similar to a stroke, such as speech and visual disturbance, and numbness or weakness in the face, arms and legs.

But a TIA does not last as long as a stroke. The effects last a few minutes to a few hours and fully resolve within 24 hours.

STROKE OR TIA TRANSIENT ISCHAEMIC ATTACK
 

A stroke is a serious life-threatening medical condition that happens when the blood supply to part of the brain is cut off.

Strokes are a medical emergency and urgent treatment is essential.
 

The sooner a person receives treatment for a stroke, the less damage is likely to happen.

If you suspect that you or someone else is having a stroke, phone emergency immediately and ask for an ambulance.

A leg ulcer is a long-lasting (chronic) sore that takes more than 2 weeks to heal. They usually develop on the inside of the leg, just above the ankle.

The symptoms of a venous leg ulcer include pain, itching and swelling in the affected leg.

There may also be discoloured or hardened skin around the ulcer, and the sore may produce a foul-smelling discharge.

See your GP if you think you have a leg ulcer, as it'll need specialist treatment to help it heal.

LEG ULCER
 

PRIAPISM
(painful erections)

DO:

  • Try To pee

  • Have a warm bath or shower

  • Drink lots of water

  • Go for a gentle walk

  • Try exercise (squats or running on the spot)

  • Take painkillers like paracetamol if you need to

Priapism is a long-lasting painful erection. It can cause permanent damage to your penis if not treated quickly.

Things you can try yourself. Priapism may get better on its own within 2 hours. 
There are things you can try to reduce your erection

DON’T:

  • Do not apply ice packs or cold water (This can make things worse)

  • Do not have sex or masturbate (This won’t make the erection go away)

  • Do not drink alcohol

  • Do not smoke

HIGH BLOOD PRESSURE

High blood pressure in the blood vessels that carry blood from the heart to the lungs (pulmonary hypertension) 

SWELLING OF THE
SPLEEN

Swelling of the spleen,
which can cause shortness of breath, a rapid heartbeat, tummy pain, a swollen tummy and anaemia

KIDNEY PROBLEMS

Kidney or urinary problems, including blood in the urine and bedwetting

However, following improvements in preventative treatments, many complications associated with sickle cell anaemia can be avoided. The life expectancy of a person with sickle cell disease has increased significantly over recent decades.

A person with the condition can expect to live into their late 40s or early 50s.