HEALTH TALK: Sickle cell anemia

Dr. Victor Emanuel MD
Dr. Victor Emanuel MD

Many have been touched by this disease. I’ve seen parents whose only child was a sickler, and having to cope with that child’s death.

I’ve seen families that have had to care for two or more sicklers.

You will know that a sickler may have many painful crises in his or her life. You will also know that generally the life span of a sickler is lower that that of the general population. There are other complications though, including:
• Stroke: Everyone knows something about what a stroke is. What you probably don’t know is that it can affect – and kill – babies and children too.
• Organ damage: Nerves, kidney, liver, spleen and other organs can be damaged and lead to death.
• Blindness: When the retina’s blood vessels are blocked, this is the result.
• Acute chest syndrome: This is a life-threatening complication affecting your lungs, requiring emergency treatment with antibiotics and other drugs and a blood transfusion.
• Gallstones: These and/or your gallbladder will need to be removed.
• Necrosis (death) of the head of the femur, which fits into your hip socket, requires a hip replacement.
• Impotence does occur, as does priapism, a prolonged painful erection. Not fun.

Now, a person can have normal red blood cells, they can have the sickle cell trait, or they can have full blown sickle cell anemia. Any child of two normal people can only be normal. A normal person paired with a carrier (trait) will have 50 percent chance of producing a normal child, and a 50 percent chance of a carrier EVERY TIME A CHILD IS PRODUCED. This is important. Each time two carriers have a child, there is a 25 percent chance it will be normal, 50% that it will be a carrier, and a 25 percent chance it will be a sickler. Two sicklers can only produce sicklers.

It is clear that many people have no clue about the disease; education is lacking. Genetic counseling is scarce. But on the other hand, it may surprise you that the desire to bear a child overrides all the applicable education that many have. Having a child in our culture is a measure of our masculinity or femininity. Some religious faiths encourage child, child, child, sickler or not.

As doctors, we’re not allowed to make value judgments, and we cannot dictate lifestyles, or our own preferences to patients, except where there is a clear risk to their health or life. But we try to preserve life or improve its quality for our patients.

And we love children, poor, innocent, helpless children. Children who, of course, never asked anyone to bring them into the world.

So let me ask a question? However you answer is between you and your conscience. If you know there’s a substantial risk, even 25 percent, that you’ll bring a child in the world to suffer serious health problems and a shortened life, should you take that risk? Think about it.

See you next week.

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7 Comments

  1. Onlooker
    February 17, 2014

    Great article Doc. Very informative. Makes us think twice. Great food for thought.

  2. on deployment.
    February 16, 2014

    we both have the trait and got lucky, my daughter only has the trait. we were stressed for months.

  3. Fachine
    February 15, 2014

    Very good information. Food for thought. Make your decisions with much prayer and education of this serious condition.

  4. Anonymous
    February 15, 2014

    I have the trait and thank the good Lord my husband doesn’t. we have 2 kids and they are both normal, but it’s not an easy thing to do because a woman can be in distress over it and that can affect the parent and child .

  5. Odille Thomas
    February 14, 2014

    I agree with the doctor’s advise regarding sickle cell disease. I have worked with sickle cell patients for many years and have seen all the complications the doctor has described. People who are aware of their status as trait/disease carriers should seriously consider whether they want to subject any child to a life with this disease. They should certainly seek professional advise and counseling prior to having children.

  6. Odille Thomas
    February 14, 2014

    9

  7. marco
    February 14, 2014

    I have a sickle cell trait and my partner as well. We have discussed it and it is a scary thing to bring a child into this world knowing that there is a 25% chance that child can get full blown sickle cell.

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