Children born with Sickle Cell Anaemia, SCA (that is, with Sickle Cell Disease of the SS type) cannot form normal, spherical red blood cells due the inheritance of an abnormal gene. They form sickled-shaped, rigid and sticky red blood cells which under certain conditions stick together and form stiffened strands within the blood vessels. The consequence is that some affected children have recurrent bone pain, persistent low blood (haemoglobin) levels, suffer damage to their vital organs and are prone to certain infections.

These children are usually given folate daily to help them make new blood cells; and Penicillin V to protect them against certain deadly bacteria.

Since 1998, a drug called HYDROXYUREA was found to be helpful in reducing the pain episodes and hospitalizations among persons with SCA while increasing haemoglobin levels. In the case of children, this means more effective time at school and to play and less time on admission in the hospital.

HYDROXYUREA or HU was actually developed in the 19th century to treat certain cancers but has been found to be useful in the treatment of persons with SCA at lower doses. It works mainly by ensuring that the red cells do not divide too fast and by ensuring that the sickled red cells are also able to retain more water resulting in a more spherical cell.

SOME FACTS ABOUT SICKLE CELL DISEASE

Question:

My partner’s “blood type” is AS and mine is also AS. Can we marry?

Answer:

Let me clear something in this question before answering the substantive question. Your sickle cell disease status, expressed as “AS” in this question is not the same as blood type which is expressed as O, A, B or AB. The two are completely different. The “AS” is in reference to the genes that give rise to the specific types of haemoglobins (vehicle for transporting oxygen found in red blood cells of your blood). It is technically called your genotype (not blood type).

Now it is this genotype which you inherited from your parents; one each from each parent and as a potential parent yourself, you will also pass it on to your future kids together with your partners’. So in this case, there are four possibilities in passing on the genes to the future should there be procreation between these partners in question:

First, an “A” from one partner and another “A” from the other with a resulting child with “AA” genotype;

Second, an “A” from one partner and an “S” from the other resulting in a child with “AS” genotype; this is possible in two instances and then thirdly,  an “S” from one partner and another “S” from the other with a resulting child with “SS” genotype causing SICKLE CELL ANAEMIA.

A few things to note:

1. The possibilities above are purely based on chance and do not happen in sequence. That is a couple with genotype AS each could have 4 kids all of whom are SS or AS or AA or a mixture two or more of these genotypes

2. There is no way of telling what genotype the next child will have

Now, to answer the substantive question “can we get married?”

This is purely a decision to be made by the potential couple. I can only counsel on the possibilities as described above and the effects a genotype of SS can have on the affected baby including interventions available; based on these the two individuals concerned can make an informed decision. Marriage is more than procreation and individuals may marry for other reasons. It is not the doctor’s or marriage counselor’s role to decide whether two individuals should go ahead and marry or not based on their genotypes.

ARTICLE BY 
DR LAWRENCE OSEI-TUTU
OFFICIAL DOCTOR OF BISA AND SPECIALIST PAEDIATRICIAN AND HEALTH ADVOCATE; KOMFO ANOKYE TEACH HOSP-KATH-GHANA

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  1. March 5, 2016

    why are sickle cell patients given folic acid rather than iron preparations?

    • March 6, 2016

      Thanks for your question Roland. The main problem in Sickle Cell Anaemia is that the abnormal sickled red cells DO NOT leave long compared to normal red cells which have a normal life span of 3 months.

      Sickled cells die off by a month to 6 weeks. The process of dieing results in the release of iron stored in their haemoglobin. This is called haemolysis.

      Now the body naturally tends to replace the red cells being lost earlier than usual by making more red cells and this requires the use of raw materials like iron and folic acid. Since iron is released from the hemolytic process that is not a problem. However, the body will need more folic acid since it is working more to replace the dieing red cells than usual. It is therefore important to supplement patients with folic acid since there is a higher demand.

      But children with sickle cell anaemia are also exposed to the same risks of iron deficiency and iron deficiency anaemia as other kids without the disease. So it is important to also assess them for iron deficiency and if present it should be corrected.

  2. March 21, 2016

    Is hydroxyurea only for SSA children

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