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.
Dr. Victor Emanuel has been an educator of medical professionals in training, and the public, for over 20 years in Dominica. The contents of his articles are based on facts and research conducted, and not of his opinion.
Since my baby became 1yr he has been having vomiting n stoooling towards. D last week of the month.he vomittd in nov 28 2013 dec 28 n jan 28 2014.my hubby is aa while am As is he a sickler
My bro and wife are AS. They want 2 get married, and have kids. Is it posible for us not to have sickler children, and what do yóu advise
If father of my son have sickle cell trait will of out side kids have it to will my child to
docter can u please what will be my problem for i have a gall stone and a small spleen
whats does it mean when a sickler has a gall stone and a reduced spleen
If both parents have the sickle cell trait (Haemoglobin AS), for every pregnancy, there is a 25% chance that this fetus/baby will have sickle cell disease (Haemoglobin SS). The normal haemoglobin type is (AA). A test called Haemoglobin electrophoresis can be used to confirm haemoglobin type.
ISickle Cell Disease is a genetic condition and therefore, genetic counselling during courtship is essential, so both partners can make an informed decision. If both partners are sickle cell trait (Haemoglobin AS) and agree to go ahead and procreate, antenatal counselling is extremely important.
When an individual is diagnosed with sickle cell disease health education is vitally important to help prevent sickle cell crisis. Sickle Cell Disease is a debilitated condition with both physical, emotional,psychological and social consequences.
Health Professional
Correction – debilitating condition
Being SS for 25 year is alot,many say i should dead by ten,but no one know how long we will live,it dont matter if we are taking medication to prevent it,it will still happen…am a young woman i dont drink or do drugs,i live the best way i could…and i will one day have a child,who i know will be AS but its ok..because its very woman dream..and being SS its ok we are humans too…
Its really really painful, I know that for a fact, both my brother and father have it, but they don’t suffer, my father gets problems once in a while but my brother leaves a normal life with no issues.
Medical and nursing professionals need to raise awareness on sickle cell disease. Sickle cell diease affects all the body systems. It is a vicious cycle!
Individuals with sickle celll disease are highly susceptible to infection because their immune system is weakened. Infection is one of the causes sof sickle cell crisis.
Dehydration is a major cause of siclke cell crisis in tropical countries like Dominica, other caribbean islands and the African continent.
Excessive sweating, excessive vomiting, diarrhoea, alcohol, fever, excessive urine output say, as a result of increased alcohol intake or diuretics (water tablets) can trigger a crisis. Therefore it is of paramount importance that sickle cell disease patients drink at least six to eight glasses of water a day to replenish water loss which can lead to dehydration, and ultimately trigger a crisis.
Hypoxia (reduced oxygen to the body tissue) and hypothermia (cold exposure) can also trigger a crisis.
Health Education and Genetic Counselling are key issues in raising awareness on sickle cell disease.
Thanks
Health Professional (qualified in care of people with sickle cell disease and other haemoglobinopathies)
Both my grandparents were carriers. Fortunately, of thre 9 surviving children only one had full blown sickle cell anemia. He died at about 56. I remember very well the troubles he went through all his life dealing with that dreadful disease.
There is a simple test for the trait. Get tested and if both of you have the traits, don’t risk bringing children into a world where their lives will be tormented.
Very informative Doc and good advice…
Doctor can you please tell me what are the risks of having the trait. I’ve been pregnant three times but only have one child lost the other two and everytime i do the routine test for pregnant mothers the Dr. only says u’re have the trait… I say to him what does that mean and he only brushes off my question.
It is the role of the doctor/nurse/midwife to raise awareness on sickle cell disease.
The trait means that your haemoglobin type is (AS) – that is you have part of the normal haemoglobin and part of lthe sickle (shape) haemoglobin. You do not have the disease and will never develop the disease. However, it is a genetic condition that can be passed on from one generation to another through their genetic make up.
If you marry a man with the trait (AS) and go on to have children, for every pregnancy there is a 25% chance that the child you are carrying will have sickle cell disease (SS) or 25% chance that the child you are carrying will have the normal haemoglobin (AA) and 50% chance that the child you are carrying will have the trait (AS).
If one parent has the sickle cell disease (SS) and the other has the trait (AS). All children have a 50:50 chance of having sickle cell disease or sickle cell trait.
If one parent has the normal haemoglobin (AA) and the other has sickle cell disease (SS). All children will have sickle cell trait, because one parent always passes on haemoglobin S and the other parent always passes on haemoglobin A to the children
If one parent has sickle cell train (AS) and the other parent has the normal haemoglobin type (AA). Each child has a 50:50 chance of having normal haemoglobin (A) only or of having sickle cell traint (AS), because all children gets haemoglobin (A) from one parent and have a 50:50 chance of getting haemoglobin S from another parent.
I hope this explanation is helpful.
Health Professional (qualified in caring for patient with sickle cell disease and other haemoglobinopathies)