Nigeria has the largest endemic population of adults and children with Sickle Cell Disease/Disorder (SCD) in the world. A quarter of all Nigerians have the Sickle Cell trait and approximately 150,000 children are born each year with the disorder (World Health Organization, 2006).
Sadly, over 100,000 children die each year from SCD. 8% of child mortality in Nigeria occurs because children under 5 years old die from Sickle Cell Disorder. One of the reasons that these children succumb is the lack of educational material or information for parents on how to manage their child’s Sickle Cell Disease (Disorder).
What is Sickle Cell Disease?
Sickle cell disease is a disease that affects a special protein inside our red blood cells called haemoglobin. Red blood cells have an important job. They pick up oxygen from the lungs and take it to every part of the body. It is the haemoglobin in these red blood cells that carry the oxygen to different parts of the body.
A person with sickle cell disease makes a different kind of haemoglobin called haemoglobin S. This haemoglobin S
causes the red blood cells to change their shape. Instead of being smooth and round, the cells become hard and
sticky. Their shape looks like a banana or like a sickle, a hand tool used to cut wheat or tall grass. It is this sickle shape of the red blood cells that gives “sickle cell” disease its name.
The hard, sticky sickle red blood cells have trouble moving through small blood vessels. Sometimes they clog up
these blood vessels and cause other changes so that blood can’t bring oxygen to the tissues. This can cause pain or
damage to these areas.
Types of Sickle Cell Disease
There are many different types of sickle cell disease. The most common types are: sickle cell anaemia (SS disease) and sickle C disease (SC disease). sickle beta (β) thalassaemia disease (S β thal disease) and sickle cell alpha (α) thalassaemia disease (S α thal disease) are other types but are less common.
There are many rare combinations of sickle haemoglobin with other types of haemoglobin. Some are as serious as
sickle cell anaemia. Some types of sickle cell disease cause fewer problems than others
What problems are caused by Sickle Cell Disease?
Sickle cell disease can cause many kinds of problems. Some of the most common problems are:
- Anaemia (low blood)
- Damage to the body organs
Not everyone who has sickle cell disease will have all of these problems. In fact, many people with sickle cell disease feel well most of the time. However, most people with sickle cell disease will have to deal with these problems during their lives.
One of the most serious problems that people with sickle cell disease have is infections. Infections, like pneumonia, pose a special problem for infants and small children who can get very sick or even die if they don’t get prompt treatment. These infections are caused by problems with the spleen, a large organ in the body that removes damaged red cells and helps fight infections. The sticky sickle cells will clog the spleen so it can’t do its job. This leaves the body open to infections.
Thankfully, we can prevent many of the sickle cell infections by giving young children penicillin every day until
they are at least five years old, or as recommended by the National Institute of Health (NIH) Consensus
Development Conference. There are also many ways to treat infections, especially if they are found quickly. Later
sections of this book describe ways to prevent and treat these deadly infections.
The hard, sticky sickle red blood cells can sometimes cause pain. The shape of these cells makes it hard for them to get through tiny blood vessels. The sickle cells can cause changes in the blood that block the blood vessel. This cuts off the blood supply to nearby tissues so that no cells can get through to bring oxygen. Without oxygen, the area starts to hurt. This is the source of the pain that comes from sickle cell disease. Some sickle cell pain can be very strong and needs to be treated in the hospital. Most pain is milder and can be handled at home. There are many ways to treat the pain to make your child feel better. This pain is sometimes called a “crisis”. In fact, most sickle cell pain is not a crisis.
What Causes Sickle Cell Disease?
Sickle cell disease is inherited
Sickle cell disease is an inherited disease. An inherited disease is one which is passed from parent to their children through their genes.
Genes are our body’s map for development. We have pairs of genes, for the color of our eyes, for our height, for our blood type and for each of our other features, including our haemoglobin type. The most common haemoglobin type
is AA (A is the usual adult haemoglobin). Haemoglobin type and blood type are not the same. Everyone has both a
haemoglobin type and a blood type.
A baby gets one haemoglobin gene from each parent
To make up our pair of haemoglobin genes, we get one gene from our father and one gene from our mother. Each of
our parents has two genes for haemoglobin, but they pass only one of these genes on to each child. Which gene is
passed on is a matter of chance, like having a boy or a girl or tossing a coin and getting heads or tails.
To inherit sickle cell disease, a child must get the sickle (S) gene from one parent and a sickle (S), C, β thalassaemia or another gene that is not A from the other parent. If a baby inherits at least one haemoglobin A gene, he won’t get sickle cell disease.
Both parents have a haemoglobin trait
Someone who has one gene for haemoglobin A and one gene for a different type of haemoglobin has a haemoglobin
trait. This trait could be AS (sickle cell trait), AC (C trait) or Aß (beta thalassaemia trait). In addition there are other less common traits. The trait is not harmful.
A trait is not a disease
People with a haemoglobin trait are healthy. They do not have a mild case of the disease. They do not have a “trace” of the disease. A trait does not cause health problems. If a man and a woman both have haemoglobin trait, some of their children may be born with sickle cell disease.
How to find out about your haemoglobin genes
The only way to know for certain what type of haemoglobin you have is to have a special blood test called
haemoglobin electrophoresis with a full blood count (FBC). Many families carry genes to make other types of
haemoglobin besides haemoglobin A without knowing it. Your doctor or sickle cell centre can order this test for
How many people have sickle cell trait?
In Nigeria, 1 out of 4 persons has sickle cell trait. Sickle cell trait is also found in other ethnic groups, such as African Americans, Ghanaians, Greeks, Yugoslavians, Western Asians, Turks, Southern Iranians, Asiatic Indians,
Mexicans, Puerto Ricans, Cubans, Spaniards, and American Indians. Although sickle cell trait is more common in
some groups of people, anyone could be a carrier of sickle cell trait.
How many people have the sickle cell disease?
Nigeria has the largest population of adults and children with sickle cell disease in the world. Sickle cell disease affects about two to three out of every 100 Nigeria babes. Approximately 150,000 children are born each year with sickle cell disease.
How can I Help my Child with Sickle Cell Disease?
There are many things you can do to help your child manage his disease. While you can’t get rid of the disease, you
can help your child get sick less often and feel better. You can also help your child learn how to live with the
disease so that he can make the most of his life. Here are some of the important things you can do.
Get the best medical care
- Find a doctor for your child who is experienced in sickle cell disease. Take your child for well child exams
as often as the doctor orders.
- Make sure your child gets all his baby shots (immunizations) on time.
- Help the doctor give your child the best care. Answer his or her questions fully and ask any questions you
have about what to do for your child.
- Learn as much as you can about the disease. The more you know, the better care you will be able to give
- Buy a thermometer for taking temperature at home.
- Buy Oral Rehydration Salts (ORS) to prepare the solution at home
Take good care of your child at home
- Give your child penicillin twice a day, until at least age five. Don’t stop until your doctor tells you to stop.
- Give your child anti-malarial drug daily (Proguanil).
- Give your child lots of fluids to drink when he is sick, in pain, very active or taking a trip and when it’s hotoutside. The rest of the time, just make sure your child gets fluids to drink whenever he is thirsty.
- Call the doctor/nurse right away if your child has a fever of 37.5°C or higher. Check your child’s
temperature when you think he might be sick.
- Help your child handle any pain he has from the disease. Try more fluids, quiet play, warm baths, heating
pads or warm towels, massage and or acetaminophen (Paracetamol, Tylenol). If these home remedies don’t
ease the pain, then call the doctor.
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