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Sickle cell anemia

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Sickle cell anemia is a heredity problem wherein the red blood cells are sickle shaped. On the contrary, normal RBCs are round, flexible and move easily through the blood vessels.

 

Symptoms of sickle cell anemia

There are certain classical symptoms for sickle cell anemia which appear when the baby is four months old.

They are: 

  • Anemia symptoms- fatigue, dizziness, shortness of breath, etc.
  • Crises- sudden pain in whole body, especially in bones, lungs, joints and abdomen
  • Hand foot syndrome i.e. swelling
  • Retarded growth
  • Susceptibility to infections
  • Problems with vision

 

What causes sickle cell anemia?

It is an inherited disease; therefore the problem lies in the genes that produce haemoglobin. Individuals with sickle cell anemia have two genes (inherited from sickle celled parents) for sickle haemoglobin which make the red blood cells crescent.

Anemia develops because the abnormal haemoglobin makes the RBCs sticky, stiff and rigid which further hinders their ability to transport oxygen to the lungs and other parts of the body.

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When to see a doctor?

New born babies are screened for sickle cell anaemia in the United States. However, if you observe the telltale signs of anaemia along with swelling in hands and feet, crankiness due to pain, fever, and slow growth in your baby then you must see a doctor.

The same applies to adults.

 

Complications of sickle cell anemia

An individual with sickle cell anemia, unfortunately is not completely free from health issues.

The complications are: 

  • Acute chest syndrome
  • Pulmonary hypertension
  • Abnormal enlargement of spleen
  • Stroke- can lead to paralysis
  • Bleeding from eyes, blindness
  • Priapism in males
  • Multiple organ failure

 

Diagnosis of sickle cell anemia

A simple blood test done at any age can confirm presence of sickle haemoglobin, but it should be diagnosed early. The blood cells are further analysed under a microscope to confirm presence of marker sickle cells.

Sickle cell anemia can also be detected before birth by collecting a sample of amniotic fluid from the placenta for presence of sickle cell gene.

 

Treatment of sickle cell anemia

Complete cure of sickle cell anemia is probably limited only to bone marrow and blood stem cell transplantation, which is highly expensive. However, the procedure has risks too.

So basically the treatment is directed towards relieving the symptoms of pain and anemia, controlling infections and evaluating the possibility of complications in order to avoid them. We sum up the treatment options below:  

  • A special medicine known as hydroxyurea helps the body to produce fetal haemoglobin which prevents formation of S-haemoglobin
  • Blood transfusions
  • Infections: antibiotics and vaccinations are offered
  • Pain is controlled through heat therapy and NSAIDs

Apart from those, you doctor will schedule routine health check-ups for assessing probability of stroke, organ failure, eye problems, etc. and to evaluate the improvement in your health.

 

Self care and living with sickle cell anemia
  • Lead a normal lifestyle; improve your immunity through natural sources of foods. Quit smoking
  • Learn how to deal with the pain- physiotherapy and heat compress helps
  • Educate yourself about the disease. This will help you to recognise the symptoms like chest pain, breathing trouble, etc. in you as well as your baby.
  • Avoid temperature extremities and travelling to high altitudes where oxygen is deficient

 

What to think about?

If you are having a sickle cell trait (one S-haemoglobin gene and other normal) then you must consider seeing a genetic counsellor before planning for pregnancy to avoid passing on the defective gene to your baby.

Written by: healthplus24.com team
Date last updated: March 26, 2014