Sterling Way
London N18 1QX
Keeping Well with Sickle Cell Disease
Introduction
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

Allowing yourself to get too cold or too hot

Excessive physical exertion

Emotional stress and anxiety

Infection
Attempts should be made to avoid these trigger factors. There is a common saying …
“PREVENTION IS BETTER THAN CURE”
However . . sometimes sickling pain crisis and other complications can occur
despite doing all the right things to avoid them. As well as offering some ‘healthy
living advice’ particularly for people with sickle cell, this guide outlines some of
the common problems, what to do about them and when to seek emergency help.
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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.
Don’t drink more than small amounts of
alcohol! It can dry the body out and directly cause
sickling pains and crises.
And don’t SMOKE! Smoking – as you know – damages the lungs and increases our risks
of heart attack, stroke, and cancers of various sorts. For people with sickle cell, already at
risk from chest infections, it increases this risk as well as causing long term lung damage. It
also drops the oxygen level in the blood – and this can lead directly to sickling complications.
‘STOP SMOKING’ sessions are held here at the hospital :
[details to be confirmed…]
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DO COME! You’ll be doing yourself a great favour if you can quit.
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 infection which can be very dangerous in people with sickle cell, therefore it is
important to cook these and other foods thoroughly. Beware of takeaway food which
contains eggs or chicken!
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.
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And now – about possible 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 and not due to lack of iron. 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. It is important for ALL women planning pregnancy to take some
folic acid supplements, however, as this reduces the risk of spinal malformations in the baby.
Penicillin / antibiotics:
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
with a bacterium called
pneumococcus. Taking penicillin twice a day is recommended from
age three months to help reduce the rate of pneumococcal infections and this should be
continued at least until the mid to late teens. For the medication to be most effective it
should be taken at around the same time every day and avoid missing a dose. People who
are allergic to penicillin are usually given an alternative, called erythromycin, to use in the
same way.
Some people continue to take antibiotics morning and evening life-long, at a dose of 250
milligrams in the morning and evening, others stop taking it every day. If you don’t take
penicillin regularly, you should ALWAYS be sure to have a supply so that – at the first HINT of
infection, sore throat, shaky / shivery feeling, cough or temperature [do be sure you have a
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thermometer so you can check for this] - you should go straight on to a treatment dose : that
is, 500 mg or two tablets, FOUR times a day for ~ 5 days. If you aren’t quickly getting better,
you must see your doctor or come to A&E [the Emergency Department, also known as ED]
so you can be assessed to see if you need stronger antibiotics.
Pain Relief [also known as ‘analgesia’]:
Pain is the commonest problem that people with sickle cell disease experience. The type of
pain relief you require will depend on how bad the pain is, and which painkillers you find
effective.
In
addition
to
medication,
massage,
warmth
and
relaxation - and drinking LOTS of extra fluid - can be
useful to ease pain. As you will be aware, pain can be mild, moderate or severe and similarly
painkillers are available in different strengths. For mild pain, Paracetamol 1g or two tablets
up to four times a day can be taken plus a NSAID (non-steroidal anti-inflammatory drug such
as ibuprofen 400mg or diclofenac 50mg) up to three times a day. Some people however are
advised not to use NSAIDs, particularly if they have kidney problems. For pain not controlled
by paracetamol and / or ibuprofen, a stronger painkiller such as co-codamol or codydramol
may be needed. Two tablets can be taken up to four times a day, but please note that these
medications contain paracetamol so do not take additional paracetamol.
If those painkillers are insufficient to control your pain you may need to use stronger
painkillers instead, such as codeine, dihydrocodeine and tramadol - for these medications
also, two tablets can also be taken up to four times a day. They do not contain paracetamol
so can be taken together with paracetamol and / or an NSAID. If your pain is very intense
you will require a strong opioid such as morphine or diamorphine. Some patients have a
small supply of these medications at home, and should take them as advised. Otherwise, you
will need to come to A&E where you will be assessed and then given pain relief.
Some people have pain more or less every day – this isn’t acute sickle ‘crisis’ pain but may be
due to damage to one or more joints by previous sickling episodes. Where possible chronic,
daily pain should be managed by non-drug approaches such as Cognitive Behavioural
Therapy or relaxation approaches. When painkilling drugs are needed, they are given as
regular, long acting forms either by skin patch [there are various form, to be replaced every
3 days or once a week] or by slow release tablets, usually taken every 12 hours, which
should manage pain throughout the day and the night. Short acting painkillers, and in
particular injections, are NOT recommended in chronic, long – lasting pain. They can lead to
a ‘vicious circle’ … the drug wears off,
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and the pain kicks in again with a vengeance,
leading to the need for the next dose, and so on.
Vaccinations [also known as ‘immunisations’]
By adulthood most people would have completed their childhood immunisations. You
should also continue to have the booster doses of vaccinations recommended for
adulthood. Your GP will be able to advice about these.
There are a number of additional 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
when the individual is around two years of age and then a booster dose is given every five
years thereafter and right through adulthood. We usually give this in the out-patient clinic.
Meningovax and Haemophilus influenza B:
This combined vaccine gives protection against other bacterial infections which can be more
serious in people with sickle cell than others, and you should be sure you have received this,
at least once. Repeat doses are not thought to be necessary.
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:
Hepatitis B is a virus that causes liver inflammation and in
some people stays around in the liver and can cause long
term liver damage and cirrhosis. It can be caught in a number
of ways, which includes through a contaminated blood
transfusion. The transfusion services in the UK are extremely
careful to screen all blood donors for hepatitis B and while
this greatly reduces the risk of infection from a blood
transfusion, the risk is never ‘zero’. It is therefore recommended that everyone with sickle
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cell disease should be offered this immunisation, as they are more likely to need blood
transfusions, at some time in the future, than other people. In practice, we now screen
everyone who has sickle cell, as some people have already had the infection, and cleared it
naturally, and they do not need additional immunisation. If you are found to be nonimmune, that is: you could still catch the infection if exposed to the virus, we will offer you a
series of extra ‘jabs’ to protect you.
And now some other general advice.
Try not to get too hot or too cold
Extremes of temperature and especially a sudden change from a warm to a cold
environment can trigger a sickle cell crisis. When a person is cold, their blood vessels
contract down and narrow, and sickle shaped red cells can cause a blockage in the narrowed
vessel resulting in pain.
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 kidneys of people with sickle cell disease are not able to
concentrate urine efficiently therefore even when the body is getting dry, the kidney
continues to produce large amounts of diluted urine and this worsens dehydration and can
cause increasing sickling and a sickle cell crisis.
Therefore it is important to avoid extremes of temperatures: wrap up and wear sufficiently
warm clothing when it is cold, and avoiding chilling! During hot weather, try to keep the cool
and drink PLENTY!
Avoid excessive physical stress
Although it is important to maintain physical activity, this should be done in moderation.
Every person is different and while some people with sickle cell disease can withstand quite
high levels of physical exertion, others cannot and they may tire easily and after strenuous
exercise may feel sickle cell pain in the muscles and joints. Each individual will need to
identify their own physical tolerance level and function within their own physical limits.
Avoid emotional stress and anxiety wherever possible!
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Unpleasant levels of stress or worry can trigger a sickle cell crisis. In late childhood and early
adulthood the most common cause of anxiety and stress relates to 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 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
from a local job centre plus office, social security office or job centre.
Some leaflets can also be found in post offices. Leaflets on housing
benefit and council tax benefit are available from local
councils.
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 you are entitled to one or another sort of benefit. We can direct you to the
relevant department of Haringey, or Enfield, or other local council [depending on where you
live] for advice. The Sickle Cell Society [sicklecell society.org; address 54 Station Rd, London
NW10 4UA; phone number 020 8961 7795] and other voluntary organisations also offer
support and have a welfare grants schemes which people can apply to for assistance.
Be careful about infection
Infection is an important and most common trigger for a sickle cell crisis. Although it is not
possible to avoid some of the common infections such as coughs and colds a person with
sickle cell disease should make efforts to avoid infections – for example by not visiting, or
being visited, by family of friends who have obvious infection, and by meticulous attention
to food hygiene: maintaining strict hand hygiene when preparing or cooking food, storing
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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 infection which can be very dangerous in people with sickle cell, therefore it is
important to cook these and other foods thoroughly. Beware of takeaway food which
contains eggs or chicken!
Travel advice and medications
In tropical countries it is recommended that you should cover up 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 painful and slow to heal.
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.
When you are planning to travel, it is important to consider
whether you need any special medications, for example,
malaria-preventing medications,
you should discuss this
with your doctor or a pharmacisit. You should also seek advice
about other
medications and vaccinations
relevant to
the country they are travelling to.
Family planning, fertility and childbearing
Most people with sickle cell can expect to have a healthy family.
Before planning a family, however,
you will need to get your
partner to have a blood test, in order to determine whether they
carry sickle cell or another unusual sort of haemoglobin, which
would mean that you might be at risk of having a child with sickle
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cell disease; this is something you would want to know about so you can discuss what
options would be available to you.
It is also advisable to discuss with your doctor your intention to start a family, or have
additional children, so that your 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. Women planning pregnancy will need to start daily folic acid if they don’t already
take it regularly.
As soon as a 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 careful
specialist joined care between the obstetric and haematology teams.
Active family planning is recommended because an unplanned pregnancy poses health and
social challenges. Any form of contraception can be used including intrauterine
contraceptive devices however this method may cause an increase risk of bleeding and may
increase the risk of pelvic infections. Please discuss your choice of contraception with your
GP and your Haematologist. Many people manage successfully with barrier methods, or
progesterone-only or combined oral contraceptive pill, or sometimes long acting ‘depot’
injected contraceptives can be suitable.
And finally: when should you seek emergency healthcare assessment?
By adulthood, most people whose sickle cell causes them pain episodes will have got expert
at managing these at home, using a variety of techniques to calm the pain down – rest, local
warmth or lying in a hot bath, plentiful fluids, and taking their painkillers as prescribed. Most
pain episodes are managed at home in this way.
However, there are some
RED FLAG symptoms
for which you should always seek
urgent assessment. These are:
 Fever of over 38 C
 Violent shivering [known as rigors] and a feeling of not being able
to get ward enough, accompanied by a fever
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 Pains in the chest, especially if accompanied by any degree of
shortness of breath or difficulty breathing.
 Sudden severe headache
 Any degree of physical weakness in the face or limbs, drooping of
one side of the face, or speech difficulties.
 Fit or seizure.
 Vomiting and / or diarrhoea so that you can’t keep enough fluids
down.
 Bad abdominal pain, especially if accompanied by vomiting or
fever.
 Priapism – that is an erection of the penis in boys or men which is
PAINFUL, and lasts longer than usual. This is a symptom for which
you should seek hospital care urgently as if prolonged – more
than an hour – long term erectile dysfunction can result.
 And, of course, pain that cannot be adequately controlled with the
painkilling medication you have on hand at home.
Urgent care is likely to be through one of the hospital services: the home care nursing team
if you are registered to use them, or - for most of the above - our Emergency Department.
Please see the service information leaflet for contact details. If you have a GP who is able to
do urgent home visits, this might also be a way to get quickly checked over.
Thanks to Brent Sickle Cell and Thalassaemia Centre who authored this leaflet. Modifications
have been made for local use at North Middlesex University Hospital.
Chair: John Carrier
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Chief Executive: Julie Lowe
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