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Sickle cell Disease

Sickle cell is a red blood cell disorder that is inherited (1). People with the sickle cell disease have an abnormal hemoglobin ( Oxygen-carrying protein) called hemoglobin S or sickle hemoglobin in their red blood cell. Medically, a person’s sickling status doesn’t change. This makes the sickling status of a person a basic requirement in any health facility. Therefore, it is advisable to know your sickling status.

To know your sickling status, a small blood sample will be required by the medical laboratory scientist to run the test ( usually a prick on the finger will do). The sickling test on its own should last for just about an hour ( depending on the health facility). The sickling test results may come out NEGATIVE or POSITIVE.

Interpretation of your sickling status result.

Generally, the result for a sickling test is very simple. It comes out as POSITIVE or NEGATIVE. The positive comes as a result of the presence of Hemoglobin S. On the other hand, a negative is an absence. Usually, a negative test result shouldn’t raise a cause for alarm although it is advised that a further test is done to know the genotype.

There are three genotypes of Hemoglobin present in red blood cells. These are A, C, and S. This gives us as many as 6 genotype combination in any individual.

Hb – Genotype Chart

sickle cell disease, know your sickling status

The hemoglobin A is said to be the normal hemoglobin whereas C and S are abnormal.

Types of sickle cell Diseases

There are several types of sickle cell diseases, the most common ones are (2) the;

SICKLE CELL ANEMIA (SS)

SICKLE HEMOGLOBIN C DISEASE (SC)

WHAT HAPPENS

The average lifespan of a normal Red blood cell (RBC) is 90 – 120 days (3). The red blood cells of people with Sickle Cell Disease (SCD) on the average live for only 16 days (4). These RBCs also become stiff, distorted and have difficulty passing through the small blood vessels in the body ( 5). When this happens, it blocks the path in which the blood passes, thereby, making less blood to reach the body tissues. This results in many complications such as;

  1. Anemia
  2. Severe pain
  3. Chest pain and difficulty in breathing
  4. Strokes

RECOMMENDATION: Before you marry, seek medical help. Get to know your sickling status and Hemoglobin genotype.

I want to know your feedback, please drop your comment and I will respond. If you have any questions regarding this article or general/special health issue, please don’t hesitate to drop a comment or contact us.

 References
1. https://www.genome.gov/10001219/learning-about-sickle-cell-disease/
2. http://bryanking.net/sickle-cell-disease/
3. http://www.nytimes.com/health/guides/disease/sickle-cell-anemia/print.html
4.https://en.wikipedia.org/wiki/Sickle-cell_disease
5. http://www.sicklecelldisease.org/index.cfm?page=about-scd

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4 Response Comments

  • Angelina AppiahMarch 8, 2016 at 2:29 pm

    Please am not a sickle cell person but can I check for my genotype or it is for only those who has the sickle cell?

    Reply
    • Noble Andoh BrakoMarch 8, 2016 at 2:53 pm

      Hello Angelina,
      The sickling test may show negative but then the genotype would reveal the type of hemoglobin that is really present. Therefore I would advice that you proceed with the genotype. For a sickling negative test, the available genotypes are AA, AC, and CC. Any of these genotypes will show negative to the sickling test. I hope the explanation given is clear.
      Thank you for having interest in my website and I hope to have you around more often. Thank you!

      Reply
  • Eugene AbankwaOctober 6, 2016 at 11:27 am

    Was very very helpful. But want to know what the meaning of ‘C’ in the genotype.
    Thanks

    Reply
    • nobleOctober 6, 2016 at 4:51 pm

      Hello Eugene,

      I am really glad you liked this article. I have not come across any scientific article or the like that vividly says the ‘meaning of C’ in the genotype. But if you require a more scientific approach, I would help. There is a change in amino acid in position 6 of the beta-globin of Haemoglobin.

      “Hemoglobin C (abbreviated as Hb C or HbC) is an abnormal hemoglobin in which substitution of a glutamic acid residue with a lysine residue at the 6th position of the β-globin chain has occurred” ..You may refer to this article for more information.

      Thanks for coming around and I hope to see you here more often.
      cheers,
      Noble Andoh Brako

      Reply

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