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DAY TWO: SICKLE CELL AWARENESS CAMPAIGN

What is the treatment for sickle cell disease?

As a rule, SCD cannot be cured, so lifelong treatment and monitoring are needed. There are a number of different treatments which help to prevent sickling episodes, or prevent related problems such as infection.

Principles of treatment

You should be treated by a specialist doctor or team, experienced in treating patients with SCD. If the specialist is a long way from your home then some of your treatment may be with a more local hospital or doctor – but the local doctors should get advice from your specialist.
Because symptoms of SCD can start suddenly, you should be able to see a doctor and get hospital treatment urgently, as and when needed.
You can be shown how to recognise symptoms (in yourself or your child), so that treatment can be started quickly.
Treatment should be tailored to your individual needs.
It is important to take preventative treatments against infection and to attend your check-ups.

Stem cell transplant is the only available treatment that can cure SCD. It is only used for severe SCD. Its use is limited by side-effects of the procedure and the availability of suitable donors.

Staying healthy

A daily antibiotic is usually recommended (penicillin, or erythromycin if you are allergic to penicillin). This is especially important to protect against serious infections in children aged under 5 years.
Immunisations: all the usual childhood vaccinations are advised, PLUS you should have vaccinations against meningitis and hepatit B, PLUS a flu (influenza) vaccination once a year. These vaccines are recommended both for adults with SCD and for children with SCD.
Vitamin supplements: extra folic acid is usually recommended. This helps the body to make new red blood cells.
Travel: if you go to a place where there is malaria, be extra careful to take malaria prevention medication and to prevent mosquito bites (people with SCD can get very ill from malaria).
Avoid smoking (which is bad for blood vessels) and excess alcohol.

Avoid factors which can trigger sickling

Factors which can trigger sickling include:

Cold.
Lack of oxygen.
Lack of fluid in the body (dehydration).
Hard exercise.
High temperature (fever).
Infection.

So it can help to:

Drink plenty of fluid.
Take regular exercise(but avoid over-exertion) and eat a healthy, balanced diet.
Avoid getting cold; wrap up well. Avoid over-exertion.
Treat infections and fevers quickly. You will usually be given detailed advice about how to check for signs of fever or infection in yourself or your child, and how to get treatment quickly.
See a doctor quickly if you feel unwell. Tell doctors and nurses that you have SCD.

Treatment of sickling episodes

The vast majority of people who have a sickle cell crisis do not need to be admitted to hospital for treatment. If the pain is mild and there is no fever then it can be possible to be treated at home. Treatment usually involves:

Painkillers: Depending on the amount of pain, you can take various types of pain medication. Mild painkillers are paracetamol or ibuprofen. Moderate ones are codeine or dihydrocodeine. A strong painkiller such as morphine may be needed for severe pain – this is usually given in hospital.
Good hydration: This usually means drinking extra fluid, or sometimes a drip into one of your veins, which is needed if you are more unwell or cannot drink.
Oxygen: This is usually given to you through a face mask in hospital. If you are not getting enough oxygen then more of your red cells may become sickle-shaped.
Antibiotics: These are used if you have an infection, or when infection is suspected. (You will normally be taking a regular preventative antibiotic already, as explained above. However, if an active infection is suspected, you will need a different antibiotic in a higher dose.)

People with SCD should try to avoid any potential triggers for a sickle cell crisis as much a possible. For example, try to keep warm in cold weather, try to avoid becoming dehydrated and take precautions if you undergo extreme exercise.

What are the possible complications of sickle cell disease, and how are they prevented or treated?

Possible complications in children

Growth, development and nutrition
As with any long-term illness, a child with SCD may grow more slowly than usual, or be undernourished if the illness affects their appetite. Your child’s growth, development and nutrition should be checked regularly, and nutritional supplements may be given if needed.

Some children with SCD take longer than usual to gain control of their bladder at night, so may wet the bed (nocturnal enuresis). Various treatments can help.

For teenagers, puberty may start about 2-3 years later than average.

The growth of bones can also be affected. For example, there may be changes in the hip or shoulder joints due to blocked blood vessels in that part of the bone. If a joint is severely affected, surgery may be needed.

Stroke or brain injury
This is a serious complication and affects about 1 in 10 children or teenagers with SCD. If sickle cells block blood vessels in the brain, this may cause a stroke. There may be symptoms of stroke such as weakness of the face or limb, or speech difficulty. For some children, there may be no obvious symptoms. However, many tiny strokes may cause a subtle brain injury and make learning more difficult.

Strokes are treated with blood transfusion, which improves blood flow to the brain. Also, research has found that regular blood transfusions help to prevent strokes. An ultrasound test called a transcranial Doppler can be used to look at the blood flow to the brain. This helps doctors to decide whether your child needs blood transfusions for prevention. Children aged 3 years should be offered these scans.

Spleen problems
The spleen is an organ located in the tummy (abdomen), in the top left-hand side. Its function is to help the immune system. Sickle cells can block blood vessels in the spleen. This can make the spleen swell up suddenly with blood – in effect, it is like losing blood into the spleen. This is one cause of sudden and severe anaemia, when your child becomes suddenly ill. The medical term is splenic sequestration. It needs urgent treatment with a blood transfusion.

If this problem happens more than once then one option is surgery to remove the spleen. However, by adulthood the problem normally resolves because the spleen becomes hard (fibrosed) and cannot swell.

Parvovirus infection
Parvovirus is a common infection in childhood. Normally it causes a mild illness with high temperature (fever), flushed cheeks and a rash. With SCD, the virus can upset the bone marrow, which then stops making blood for a while. This causes a severe anaemia and needs treating with blood transfusions until the bone marrow recovers.

Possible complications in older teenagers and adults

Damage to various organs can develop gradually during teenage and adult years, due to repeated, small blockages of tiny blood vessels. The amount of complications varies from person to person.

Lungs, heart and kidneys
Any of these organs may suffer some damage. Therefore, you will normally be offered regular checks on your heart, lungs and kidney function. Various treatments can help.

Eyes
Regular eye checks are important. SCD may cause changes to blood vessels in the back of the eye (retina); this is called retinopathy. For retinopathy, laser treatment is given to prevent further damage.

Also, sickle cells may cause sudden blockage of a blood vessel in the eye. If this happens, you will have a sudden reduction in your vision. This needs immediate treatment. So, always see a doctor quickly if your vision reduces suddenly in any way.

Unwanted erections
Some teenage boys and men with SCD may get unwanted erections of the penis, which may be painful. The medical name for this is priapism. This can be quite brief but if an erection does not subside within one hour then urgent treatment is needed. There are various treatments to relieve or prevent unwanted erections.

Gallstones
Stones in the gallbladder are more common in people with SCD, and can cause bouts of pain in the upper right side of the abdomen. They may need treatment which is usually an operation to remove the gallbladder.

Leg ulcers
Leg ulcers can occur with SCD but are not common. Treatment is with dressings, and zinc supplements may help.

What is the outlook?

Sickle cell disease (SCD) is a serious condition which may shorten life. Without treatment, people with SCD may die in childhood, from problems such as infection. Good treatment makes a great difference. Improvements in treatment mean that life expectancy has increased.

Even with modern treatment, SCD can still cause serious or life-threatening problems. Dangerous problems are severe infection, acute chest syndrome and sudden severe anaemia. Awareness of symptoms and early treatment are important.

There is a lot of individual variation in the severity and outlook (prognosis) for SCD. Some people get very few problems from their SCD; others have more symptoms or more complications.

The treatment of sickle cell anaemia is a developing area of medicine. New treatments continue to be developed and the information on outlook above is very general. The specialist who knows your case can give more accurate information about the outlook for your particular situation.

To be continued…

Written By: Akpus Adgidzi Immanuel