Sickle Cell Disease in Children

WHAT IS SICKLE CELL DISEASE?

Sickle cell disease (or sickle cell anaemia) 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. This haemoglobin 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.

                                    

 

HOW DOES SOMEONE GET SICKLE CELL DISEASE?

Sickle cell disease is an inherited disease. An inherited disease is one that is passed from parents to their children through their genes. Genes are our body’s map for development. We have pairs of genes for the colour 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).

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, beta thalassaemia or another gene that is not (A) from the other parent. In this case both parents have a haemoglobin trait. If a baby inherits at least one haemoglobin A gene, he won’t get sickle cell disease.

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).

HAVING A TRAIT IS NOT A DISEASE

People with a haemoglobin trait are healthy. If a man and woman both have a haemoglobin trait, some of their children may be born with sickle cell disease.

CHANCES OF HAVING A BABY WITH SICKLE CELL DISEASE

If one parent has the sickle cell trait (AS) and the other has normal haemoglobin (AA) there is a 50 per cent chance with each pregnancy that their child will have the sickle cell trait (AS). None of their children will have sickle cell disease.

 

If both parents have the sickle cell trait, with each pregnancy there is a 25 per cent chance (1 in 4) that the child will have sickle cell disease (SS), a 50 per cent chance (2 in 4) that the child will have the sickle cell trait (AS) and a 25 per cent chance (1 in 4) that the child will have normal haemoglobin (AA). The risk is the same with each pregnancy. So it is possible for these parents to have 4 children who all have sickle cell disease, 4 children who all have normal haemoglobin or any combination in between.

 

If one parent has sickle cell disease (SS) and the other has a trait (AS), there is a 50 per cent chance with each pregnancy that the child will have sickle cell disease and a 50 per cent chance that the child will have the trait. They will not have a child with normal haemoglobin (AA).

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. This test is called haemoglobin electrophoresis with a complete blood count (CBC). Many families carry genes that produce other types of haemoglobin besides haemoglobin A without knowing it. Your doctor or sickle cell centre can order this test for you.

 

PROBLEMS THAT PATIENTS WITH SICKLE CELL CAN HAVE

The sickle cells are harder, stickier and less flexible than normal red blood cells. They can get stuck in small blood vessels and block them. 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.”

The sickle cells are also destroyed more easily than normal red blood cells. This means that people with sickle cell disease have lower blood levels (anaemia).

WHAT PROBLEMS ARE CAUSED BY SICKLE CELL DISEASE?

Sickle cell disease can cause many kinds of problems. Some of the most common problems are:

• Infections
• Pain
• Anaemia (low blood levels)
• Damage to the body organs such as the kidneys

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 lifetime.

COMMON PROBLEMS AND ITS HOME MANAGEMENT

• INFECTIONS/FEVER

Infections are one of the most serious problems. It is important to know when your child has a fever and what to do about it. When your child has a fever, it is often a sign that her body is fighting an infection. Infections can be very serious in children with sickle cell disease. Noticing symptoms of an infection early can let you take actions to prevent it from getting worse. If you think your child might have a fever, take her temperature. Your child has a fever if her temperature remains above 37.5^oC.

What to do if your child has a fever

Take your child’s temperature. If it is heigher than 37.5 ^oC give your child the doctor’s prescribed dose of paracetamol and sponge the child with lukewarm water.

Give the child extra fluids to drink (water, coconut water, juice or any other liquid).

Ensure that child rests and limit activities.

Recheck temperature in fifteen minutes. If temperature stays high (above 37.5^oC) prepare to send child to hospital. It is important for the child to be examined by a doctor.

DO NOT KEEP A CHILD WITH SICKLE CELL DISEASE WHO HAS A FEVER AT HOME. THIS CAN LEAD TO SERIOUS PROBLEMS

You don’t need to take your child’s temperature every day if your child is well. It is not necessary and can be upsetting to your child.

 

• PAIN

Your child may have pain at times from sickle cell disease. 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. Usually the pain is mild enough to treat at home. Infants and toddlers may show pain by crying, refusing to walk, or pointing to the areas that hurt. Pay attention to these signs. Try different ways to ease pain to see which ones help the most.

                            

 

Home remedies for pain

1. More fluids

Extra fluids can help keep the sickle cells from clogging up small blood vessels. Since this is a major cause of pain, extra fluids can do a lot to ease the pain. Give your child up to double the amount of fluids she usually drinks.

2. Quiet play

Cutting back on physical activity can be helpful. Complete bed rest may not be needed, just less active play. Find things for your child to do quietly inside for a while. See if quiet play will help her feel better.

3. Apply warm, moist towels

Apply warm towels to painful parts, change them when they cool off. Wet them with warm water, and then wring them out. You can apply these as often as it helps.

4. Massage

Gently massage the painful area with warm baby oil or lotion to relax tense muscles and increase blood flow. You can massage the arms, legs, back and neck areas easily.

   5.  Pain medication

Paracetamol can provide a lot of relief from the pain caused by sickle cell disease. Make sure to give you child the right dose for her weight as prescribed by the doctor. If the pain is more severe the doctor may add brufen or another form of pain medication to the paracetamol. Give medication only as prescribed by your doctor.

If the pain persists, take the child to the hospital.

 

• ANAEMIA (LOW BLOOD LEVELS)

People who have sickle cell disease have fewer red blood cells than normal. Since red blood cells carry oxygen to the muscles, they often become tired more quickly than people with normal blood counts. In general, people with SS disease have the most severe anaemia. These are times when your child’s blood count may fall much lower than usual. This can happen with a fever or an infection. Either the body stops making new cells or the cells are destroyed more quickly than usual. When this happens, the destroyed red blood cells in the body fluids can make the eyes look more yellow (jaundice) and the urine look darker than usual.

WHAT TO DO

Bring your child into the doctor to have her blood count checked if you notice any of these signs of an extra low blood count:

1. More tiredness, drowsiness or child is unresponsive
2. Pale colour in the soles of the feet and palms
3. Fast breathing
4. Loss of appetite
5. Yellow eyes or skin
6. Dark urine

Treatment

If your child’s blood count falls very low, a blood transfusion may be needed. An extremely low blood count can result in heart failure and death if not treated in time.

IF YOU OBSERVE ANY OF THE ABOVE SYMPTOMS PLEASE SEND YOUR CHILD TO THE HOSPITAL

 

• JAUNDICE (YELLOW EYES)

Your child’s eyes can become yellow. This is due to red blood cells breaking down and releasing a chemical into the blood.

  WHAT TO DO

Give your child plenty of fluids to drink, recommended twice his daily fluid requirement. Observe          your child’s eye colour for 24 hours

If the colour appears to be less yellow continue the fluids and the jaundice should subside in a few days. However if your child has any of the other signs of low  blood count (anaemia), or the jaundice persist or appears to be worsening after 24 hours send your child to the hospital.

 

• DARK URINE (COCA COLA-COLOURED URINE)

Sometimes your child’s urine will appear dark looking like coke.

WHAT TO DO

Give your child plenty of fluids to drink, recommended twice his daily fluid requirement. Observe your child’s urine colour for 24 hours. If urine appears to be clearing continue fluids till urine is clear.

However if urine persistently stays darker after 24 hours of giving adequate fluids or if your child has any of the signs of low blood count at any point in time, SEND THE CHILD TO THE HOSPITAL.

 

HOSPITAL MANAGEMENT

On arrival at the hospital you will be taken through the following process

• You will be required to give details about why you brought the child to the hospital which will include:
• When the symptoms started
• Any medications you have given or any other intervention you have done

Some laboratory investigations may be requested depending on the complaint. Your child might receive some painkillers to relieve the pain, antibiotics if there is infection and/or IV fluids (drip). You might also be required to do an x-ray or an ultrasound. If necessary, your child may be admitted to the hospital until he is feeling better and is well enough to go home.

Remember that it is important not to wait too long to bring your child to the hospital if they are not feeling well. It is better to bring them in to be examined early than to wait until their condition worsens. This will usually mean a longer hospital stay and a greater chance of complications. Let us all work together to ensure that our children with sickle cell disease remain happy and healthy.

 

By Diana Dwuma-Badu, Paediatric Haematology Nurse, KBTH