Keeping Well with Sickle Cell Disease

For information on care of children with sickle cell disease see Care and Management of your child with sickle cell disease – A Parent’s Guide.

A person with sickle cell disease can do a lot to help themselves keep well. The first step is to learn as much as possible about the illness, get to know the things that commonly trigger a sickle cell crisis and sickle related illness, how to avoid those things; if sickling crisis or an illness does occur what to do to reduce their effect and where necessary when and how seek medical help.

It is known that certain things can increase the likelihood of having a sickle cell crisis, these include:

  • Not having enough water in the body (dehydration)
  • Not eating enough food (voluntary or involuntary fasting)
  • Not taking recommended medications and vaccinations
  • Being too cold or too hot
  • Excessive physical exertion
  • Emotional stress and anxiety
  • Infection

Attempts should be made to avoid these trigger factors, if they occur take actions to minimize their effect and if necessary seek medical attention. There is a common saying which states:

“PREVENTION IS BETTER THAN CURE”

It is important to know that sometimes sickling crisis and illness does occur despite doing all the right things to avoid them, but in these situations it is important to know what to do in order to avoid serious complications.

To stay well it is important to pay particular attention to the following:

Fluid

An insufficient amount of water in the body (dehydration) is one of the most common causes of a sickle cell crisis. In this situation the blood becomes thicker and sickled shaped red blood cells are more likely to stick together and cause a blockage in the blood circulation. Therefore it is important for an adult to drink at last 3 – 4 Litres of water daily; this is apart from the water contained in the food they eat.

The water can be in the form of plain water, diluted fruit squash, tea and other fluids. Alcoholic drinks should be avoided and if taken it should be consumed in very small quantities because alcohol increases dehydration and can lead to other complications.

By adulthood most people with sickle cell disease are aware of which drinks or food affect their health as an individual. For example, some people experience stomach upsets if they drink fizzy drinks whilst it has no effect on others who have a similar disease.

If for any reason a person with sickle cell disease is not able to drink normally, for example if they are vomiting, losing fluid because they are sweating excessively due to a fever or they have diarrhoea it is important that they seek medical attention urgently in order to find out the cause of the vomiting, fever or diarrhoea and treat any infection promptly. It is important to make sure that the person does not get dehydrated and it may be necessary to call the GP or go to hospital for treatment.

Balanced Diet

All body cells need food to keep healthy but the type of food eaten will determine how well the body tissues are able to work properly in order to prevent illness or a worsening of a chronic illness. Like anyone else people who have sickle cell disease need a well balanced diet which must include meat, fish, vegetables and fruits and these must be in the correct quantities for healthy living.

In the UK it is recommended that in order to stay healthy people should eat five portions of fruit and vegetables every day, reduce the amount of fatty foods and salt and do regular exercise.

Because people with sickle cell disease are at greater risk of getting infection it is important to handle food with particular care, for example, maintaining a strict hand hygiene, storing foods properly and checking the ‘expiry’ or ‘use by’ dates on food packaging including tinned foods.

It is important to thoroughly defrost frozen food before cooking and to make sure chilled foods from the supermarket are cooked according to the maker’s instructions. Extra care needs to be taken if re-heating previously cooked food, making sure that the food is heated right through, especially if using a microwave oven. Chicken and eggs are a common source of salmonella therefore it is important to cook these and other foods thoroughly. Salmonella can lead to bone infection which can very difficult to treat in people with sickle cell disease.

Not eating for long periods can trigger a sickle cell crisis due to not having enough nutrients in the body for maintaining normal bodily functions. If fasting is part of an individuals religious practice they should discuss this with their doctor or specialist nurse and seek advice on how to practice their religion without putting their health at risk.

It is advisable to avoid self medication even herbal remedies and the use of these and other over the counter medications, tonics and remedies should be discussed with the doctor before taking them in case they disagree with any prescribed medications.

Medications

Although sickle cell disease is a form of anaemia it is not the same as ‘iron deficiency anaemia’. The anaemia of sickle cell disease is caused by a rapid breakdown of red blood cells, see Sickle Cell Disease and not due to lack of iron necessarily. Therefore it is important that individuals should not self medicate with iron and should only use iron medications if it has been prescribed by their doctor following a blood test that confirmed that they genuinely have ‘iron deficiency’.

People with sickle cell disease do not need extra vitamins if they eat a well balanced diet however if they are vegetarian or vegan it may be necessary to have extra vitamins. Taking a daily dose of multivitamins that does not contain iron will do no harm.

Folic acid:

This mineral is essential and helps the bones in their job of making red blood cells. Some clinics recommend use of folic acid, 5mg once a day, but in the UK a normal balanced diet usually contains sufficient folic acid and a daily supplement is generally not required but if it is taken it does no harm.

Penicillin:

From early childhood the spleen of people with sickle cell disease does not work properly, therefore they do not develop good immunity and are prone to getting infections, especially pneumococcal infections. Taking penicillin twice a day is recommended from age three months to help reduce the rate of pneumococcal infections and this should be continued into adulthood.

For dosage of Penicillin is as follows:
62.5mg twice a day until one year of age
125mg twice a day from one until five years of age
250mg twice a day from age five years into adulthood

For the medication to be most effective it should be taken at around the same time every day and avoid missing a dose. It is available in syrup form for very young babies and as tablets for older children and adults.

Vaccinations

By adulthood most people would have completed their childhood immunizations, see Childhood immunizations - Care and Management of your child with sickle cell disease – A Parent’s Guide. They should also continue to have the booster doses of vaccinations recommended for adulthood. The persons GP will be able to advice about these.

There are a number of other vaccinations recommended for people with sickle cell disease.

Pneumovax:

This medication is given because it gives greater protection against a greater number of bacterial types that cause pneumococcal infection. The first dose of the vaccine is given by the GP when the individual is around two years of age and then a booster dose is given every five years thereafter and right through adulthood.

Meningivac:

This gives protection against meningococcus types A and C which cause meningitis. Even if a person has been given Men C, which protects against meningococcus type C, it is recommended that they have Meningivac before travelling to some parts of the world, e.g. Africa and Asia because it will protect against meningitis A which occurs more commonly in those parts of the world.

Influenza (Flu) vaccine:

It is recommended that people who do not have good immunity and prone to getting infections easily should be offered a flu vaccination every year. Getting the flu virus can be hazardous in those with sickle cell disease and this vaccination is highly recommended; in the UK it is offered by the GP usually in autumn or early winter, if a person has not been offered they should ask their GP.

Hepatitis B vaccination:

People can contract Hepatitis B through using contaminated needles, for example, whilst injecting illegal drugs, through sexual contact with an infected person, contamination from a blood transfusion and less commonly from an infected mother to her unborn baby. The blood transfusion services in the UK are extremely careful to screen all blood donors for hepatitis B this reduces the risk of contamination via that route. If an individual with sickle cell disease requires regular blood transfusion it is recommended that they should have this vaccination to give them protection.

Too hot or too cold

Extremes of temperature and a sudden change from a warm to a cold environment can trigger a sickle cell crisis. When a person is cold in order to keep the blood warm and flowing the walls of the blood vessels thickens making the space in the middle of the blood vessel smaller. This is not a problem if the red blood cells are a normal shape and the blood is not too thick however if the cells are sickle shaped, hard and rigid and the blood is thick the cells are more likely to cause an obstruction and sickling crisis.

Conversely if a person is too hot they will sweat more and lose more water from the body, when this happens the blood will become thicker. In order to prevent the body from becoming dehydrated the kidneys will reduce the amount of urine it is producing and will make more concentrated urine. The kidney of people with sickle cell disease is not able to concentrate urine efficiently therefore even when they are too hot the kidney continues to produce large amounts of diluted urine and this leads to dehydration and cause increasing sickling which can lead to a sickle cell crisis.

Therefore it is important to avoid extremes of temperatures, wrapping up and wearing sufficiently warm clothing when it is cold and avoiding chilling is essential. During hot whether it is necessary to keep the body cool and well hydrated as discussed earlier.

Excessive physical stress

During physical exercise the body needs extra oxygen in order to provide for active muscles. People with sickle cell disease have a chronic anaemia which reduces their ability to carry oxygen very well therefore during physical exercise they become breathless easily and get tired quickly.

Although it is important to maintain physical activity this should be done in moderation. Every person is different whilst some people with sickle cell disease can withstand a moderate amount of physical exertion others cannot. Each individual will need to identify their own physical tolerance level and maintain their own physical boundary.

Physical stress includes those things that place a greater physical burden on the body for example pregnancy, it is not uncommon for women to experience more frequent sickling crisis during pregnancy and in the early weeks after delivery of the baby.

Emotional stress and anxiety

A certain amount of anxiety can be helpful because it motivates us to perform, but too much can trigger a sickle cell crisis and this should be avoided. In late childhood and early adulthood the most common cause of anxiety and stress relates to stresses in the educational environment for example when preparing for exams. Emotional stresses such as bullying in school can also contribute to this.

In adulthood work related stresses, adult relationships, financial pressures and other challenges of adulthood can compromise the well being of someone with sickle cell disease. If these cannot be managed by the individual and their family it is be worth discussing the problem with the specialist nurse, GP, hospital doctor, social worker or psychologist.

Employment and finance

To help reduce employment and financial stresses information and leaflets regarding benefit entitlements and their rates can be obtained, free of charge from a local job centre plus office, social security office or job centre, see Sickle Cell Disease and Employment. Some leaflets can also be found in post offices. Leaflets on housing benefit and council tax benefit are available from local councils.

Further information is available from the Department for Work and Pensions, Jobcentre Plus and the Pension Service.

The Benefit Enquiry Line is a free confidential telephone service set up for people with disabilities, their Carers and representatives. Phone: 0800 88 22 00 Text phone: 0800 24 33 55

Disability Living Allowance (DLA) Helpline gives advice on existing DLA claims and send out application packs to new claimants. Phone: 0845 123 456 Text phone: 0845 722 4433

The Citizens Advice Bureau (CAB) will advise on a range of earnings-related and other benefits which a person may be entitled to, whether they are working or not. This may include housing, welfare and state benefits. They will advise on whether the person is entitled to family income support, invalidity care allowance, disability living allowance, family fund and a range of means and non means-tested benefits.

Many local Sickle Cell & Thalassaemia Centres also offer welfare advice and or are able to direct people to local organizations for further advice or support.

The Sickle Cell Society and other voluntary organizations also offer support and have a welfare grants schemes which people can apply to for assistance.

Infection

Infection is an important and most common trigger for a sickle cell crisis. Although it is difficult to avoid some of the common infections such as coughs and colds a person with sickle cell disease should make efforts to avoid infections.

The most common causes of infection is failing to take daily prescribed Penicillin and recommended vaccinations, not avoiding situations which places one at greater risk of infection, for example improper preparation of food, poor hygiene, lack of attention to treating wounds including insect bites and stings. In tropical countries it is recommended that those with sickle cell disease should wear socks especially in the evening when mosquitoes are most active and an insect bite on the ankle can easily become an infected wound causing a chronic leg ulcer which can be very difficult to treat in people with sickle cell disease.

Stings and bites must be taken seriously, they should be covered immediately to prevent them from getting infected and must be treated by a doctor if they appear to be getting infected.

Travel advice and medications

If a person with sickle cell disease is travelling it is important to consider whether they need any special medications, for example, malaria-preventing medications, they should discuss this with their practice nurse or GP. They should also seek advice about other medications and vaccinations relevant to the country they are travelling to.

When travelling especially if going abroad it is important to get a summary of the person’s sickle cell disease, the type of sickle cell disease the person has, any special treatments they are being given, details of the doctor or hospital medical team that is caring for them and who can be contacted in an emergency in case the person becomes unwell.

Some people use a medical alert bracelet or necklace which contains details of their illness and allows doctors abroad to access their relevant health information. For further information about these items see Medical Alert Bracelets.

Before travelling abroad it is advisable for someone with sickle cell disease to obtain the name and contact details of a reputable doctor in the country they are visiting, the Sickle Cell Society may be able to help with this.

For further information and advice with travelling see in Traveling and going abroad - Care and Management of your child with sickle cell disease – A Parent’s Guide.

Family planning, fertility and childbearing

The teenage years are a time of rapid growth and development, the hormonal changes of pubertal development may contribute to increasing episodes of crisis and pain. The long-term effects of sickle cell disease often begin to emerge during the teenage years and this may be a time of increased episodes of sickle cell crisis, especially in young men. Some women have reported an increase in episodes of painful crisis during their menstrual periods.

Before planning a family it is advisable for the individual to get their partner tested in order to determine whether they are at-risk of having a child with sickle cell disease, see Inheritance of Haemoglobin and in order for them to make an informed choice about childbearing.

It is also advisable for the person to discuss with their doctor their intention to start a family or have additional children so that their medical treatment can be reviewed and any potential medications or treatments can be taken into consideration. It may be necessary to stop certain drugs before becoming pregnant as these may be harmful to a developing pregnancy. It may also be necessary to ensure that the level of anaemia is managed properly so as to reduce the risk of miscarriage and physical defects in the growing pregnancy.

Women and men with sickle cell disease are generally fertile and able to have children however they are potentially vulnerable to complications which may affect their fertility. In men frequent and prolonged episodes of priapism may cause impotence and subsequent infertility and women who are fertile complications are increased during pregnancy and childbirth.

As soon as the woman suspects that she is pregnant she should inform her GP and hospital medical doctor because pregnancy and childbirth can pose a greater health risk for the pregnant woman with sickle cell disease and her unborn baby. She will need specialist obstetrician and haematologist care in order to limit and prevent complications.

Active family planning is recommended because an unplanned pregnancy poses many health and social challenges for people with sickle cell disease especially the women who will carry the pregnancy.

Any form of contraception can be used including intrauterine contraceptive devices however this method may cause an increase risk of bleeding and perhaps infections.

Several studies have demonstrated that oestrogen has a beneficial effect on the red blood cells giving females of reproductive age a marginal advantage over males. Hence the use of inject able contraceptive preparations such as Depo-Provera is popular among women of reproductive age because it is reported to reduce the occurrence of vaso occlusive crisis.