Sickle Cell Anaemia

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Sickle Cell Anaemia

What Is Sickle Cell Anaemia?

Sickle Cell Anaemia is an inherited condition that affects the red blood cells. Normally, the red blood cells are flexible and round so that they can easily move through our blood vessels. In Sickle Cell Anaemia, the red blood cells become rigid and sticky, and are shaped like sickles or crescent moons. These irregularly shaped cells get stuck in some blood vessels slowing or blocking the flow of blood and oxygen to parts of the body.

Sickle Cell Anaemia, sometimes also referred to as Sickle Cell Disease, is a serious and lifelong condition, although long-term treatment helps manage many of the problems associated with it.


What Causes Sickle Cell Anaemia?

Sickle Cell Anaemia is caused by a faulty gene that affects how red blood cells develop. Genes come in pairs and all of us inherit one set from our mothers and one set from our fathers. To be born with Sickle Cell Anaemia, a child must inherit a copy of the faulty sickle gene from both of their parents.

If both parents are sickle cell carriers, there’s a:

  • One in four (25%) chance each child they have will not inherit any faulty genes and won’t have sickle cell anaemia to pass it on.
  • One in two (50%) chance each child they have will just inherit a copy of the faulty gene from one parent and be a carrier.
  • One in four (25%) chance each child they have will inherit copies of the faulty gene from both parent and will be born with the disease.
What Are The Symptoms Of Sickle Cell Anaemia?

People born with the disease sometimes experience problems from early childhood, but most children have few symptoms and lead normal lives.

The various signs and symptoms of Sickle Cell Anaemia vary from person to person and change over time. They include:

  • Anaemia where red blood cells can’t carry enough oxygen around the body causing tiredness and shortness of breath
  • Episodes of pain called Sickle Cell Crises
  • Painful swelling of hands and feet
  • Frequent infections
  • Delayed growth in infants and children and delayed puberty in teenagers
  • Vision problems such as floaters, blurred or patchy vision, reduced night vision and occasionally sudden vision loss
  • Some people also experience other problems such as delayed growth, strokes, and lung problems

Overall, the life expectancy for someone with Sickle Cell Anaemia tends to be shorter than normal. Currently, people with the disorder typically live until 40-60 years of age, although milder types of sickle cell anaemia may have no impact on life expectancy.

How Is Sickle Cell Anaemia Diagnosed?

Sickle Cell Anaemia is usually detected during pregnancy or soon after birth.

Screening During Pregnancy: Screening To Check If A Baby Is At Risk Of Being Born With Sickle Cell Disease Should Be A Must For All Pregnant Women. The test should be carried out before the 10th week of pregnancy so that the couple gets enough time to consider the option of further tests to find out if the baby will be born with the disease.

New-born Screening:
This can help to:

  • indicate whether the new-born has sickle cell disease if the pregnancy screening suggested the parents were at high risk and they decided not to have tests to confirm the diagnosis
  • identify any babies with sickle cell disease whose parents weren’t screened while pregnant
  • show if the baby is a carrier and is at risk of having children with the condition

Another way to diagnose the disease is a blood test that will help determine the possibility of person being a carrier of the sickle cell trait.

How Is Sickle Cell Anaemia Treated?

Sickle Cell Anaemia usually requires lifelong treatment and the patient needs specialist care throughout their lives. The treatment is usually aimed at avoiding crises, relieving symptoms and preventing complications.

Some of the main treatments are:

  • Preventing Painful Episodes by Avoiding Possible Triggers – drinking plenty of fluids to avoid dehydration; wearing appropriate clothing to avoid getting a cold; avoiding sudden temperature changes such as swimming in cold water.
  • Preventing or Reducing the Risk of Infections – doctors prescribe antibiotics and will ensure that the patient is fully vaccinated.
  • Blood Transfusions when the condition becomes severely anaemic

While the above options address the symptoms, the only treatment that can potentially cure Sickle Cell Anaemia is a Stem Cell or a Bone Marrow Transplant.

Sickle Cell Anaemia can also cause several other problems that may need to be treated:

  • Short course of hormonal medication to help trigger puberty in children with delayed puberty
  • Gallbladder Remover Surgery to treat gallstones