Information for Patients, Family and Carers
Why Do I Have Pain?
Pain can be a major problem for people living with sickle cell disorder
(SCD), and it is very important that you get the right treatment and
support for your pain.
There are different types and causes of pain which need different types
of treatment.
Acute Pain
You might have short-term pain (this is also called ‘acute pain’) caused
by a vaso-occlusive crisis (VOC) – this is the same as a ‘sickle cell crisis’
or ‘painful crisis’. This pain usually lasts for a few days and then gets
better.
If you feel that your pain feels different and might not be caused by a
VOC, then please let your specialist team know as you might need
different help and treatment.
Remember that you can still experience different types of pain in
addition to pain caused by VOC, but the following information will help
most forms of pain regardless of its cause. The important thing to do is
to seek help if you cannot manage at home, or if your pain is different
from your expected pains.
How Should Acute Pain Be Treated At Home?
You probably already have ways of managing your pain at home, which
work best for you. Here are a few suggestions. Even if you need to go
to hospital, these things are still important to carry on with as much as
possible:
• It is always a good idea to make sure you stay warm and that you
are drinking plenty of fluids. Remember that drinks with caffeine
or alcohol in can actually be dehydrating, so avoid alcohol or a lot
of tea or coffee
• Staying calm with relaxation and breathing exercises. Being
stressed and anxious (the “fight or flight” response) will act to
compress your blood vessels further, so taking control of your
breathing can help induce the relaxation action which opens
blood vessels and helps your blood flow to the areas that need it.
• Distracting yourself from the pain such as watching a movie, TV
show or listening to music can also be helpful and can help reduce
pain.
• If you have access to acupuncture or someone can give you a
massage, these can help relieve your pain.
• Try to stay mobile and keep moving around as much as you can
manage, this helps you to recover more quickly and keeps the
blood moving. Even gentle movement such as Tai Chi, Yoga or
Pilates, can improve pain and maintain blood flow.
Medications you can use at home
You might take over the counter paracetamol, or a non-steroidal
anti-inflammatory medication (NSAID) such as ibuprofen can be helpful.
However, always read the label to make sure that it is safe to take a
medication for you, for example, if you have had a problem with
stomach ulcer, or if you have asthma, kidney problems or are pregnant,
speak to your doctor before taking ibuprofen or other NSAID
medications.
If this is not working, then some people find weaker opioid medications
such as codeine or tramadol for a short period of time can be helpful. If
you are needing to take any opioid medication for more than 5 days,
please speak to your specialist team to find out if alternative treatment
might be more helpful for you.
We know that stress, whether this is physical or psychological, could
increase the chance of sickle cell crisis. Our psychology team may be
able to support you better understand lifestyle triggers and manage
stress that contribute to painful crisis.
When Should I Go To Hospital?
If your pain is severe, or if your home medication and the approaches
above are not controlling your pain, please call your specialist team and
come into hospital for review. It is also important to come to hospital if
you have any of the following problems:
• High temperature (fever) such as over 38°C
• Pain in your chest, difficulty breathing or coughing
• Unable to drink or take medications because you are vomiting
• Weakness or not being able to feel sensation in any area of the
body
• Headache which is more than just mild or normal for you
It is a good idea to bring a copy of your personal care plan when you
come to hospital, if you have one. You might choose to carry a
photograph or electronic copy of this on your phone or other device, for
example, or carry a paper copy with you.
Medications Used In Hospital
Your Individualised Care Plan should be available to be seen by the
hospital team, and they should give you the treatment outlined in it
which you have agreed with your specialist team when you first arrive
to hospital. This might include an injection, or oral dose of a strong
opioid medication such as oxycodone or morphine. You might also be
given pregabalin, which is another medication which helps with severe
pain, as well as paracetamol and ibuprofen (or similar) if you usually
have this. Sometimes other treatments are also used and these will be
discussed with you.
Strong Opioid Medications
Opioid medicines are not usually able to take your pain away
completely. The aim is to improve your pain and make it manageable,
so that you can recover and get back to normal as quickly as possible.
You might receive injectable opioid treatment as a PCA
(patient-controlled analgesia), or you might just have one-off injections
when you need them. If you are given a PCA, we will give you separate
information about this. It is usually best to move to taking this
medication orally after 1-2 days, because each dose will last longer, and
because using injectable opioids for longer than this puts you at risk of
harm. Once your pain is controlled, try to reduce the oral opioid
medicines as well. Most people find they do not need opioid medicines
longer than 5-7 days at the most for a VOC, as pain is usually improving
by then. Speak to your specialist if you feel that you need these
medications longer than 5 days.
Opioid medications in the long-term can cause significant harm to a
person. Even short-term use can lead to addiction and dependence. If
you are struggling to come off these medications, speak to your
specialist or GP about a gradually tapering dose to minimise any risk to
you and should make it easier to come off and control your pain.
Chronic pain
It is very common for people with SCD to develop longer-term pain,
which is called ‘chronic pain’. Pain is called chronic if it lasts for 3 months
or longer.
Chronic pain can be just as bad as acute pain, but the causes are
different, and the treatments that work for chronic pain are often
different as well.
What Causes Chronic Pain?
Chronic pain can be caused by quite a few different things. It can be the
result of damage to the body that hasn’t healed, such as when it is
caused by avascular necrosis in a joint (this is where the bone is
damaged because of poor blood supply). This is ‘nociceptive pain’ due
to ongoing damage and processes that have not yet healed.
It can also be caused by damage to the nerves that detect pain, or the
way your brain receives and processes those signals. Nerve damage pain
might be called ‘neuropathic pain’.
Sometimes these nerves can also be over-sensitised or transmit the
wrong signals. They might produce signals telling your brain that pain is
very severe, when actually it is being caused by something quite minor
or even something which would not usually cause pain at all. This can
happen as a result of severe or repeated acute VOC pain, especially if it
is not controlled well when you first have that pain. When you have
been in pain for a long time, your body is able to “learn” more pain, and
changes happen in your nervous systems, so that even if all the damage
is healed and there is no longer any pain being transmitted to nerves,
you still feel significant pain. This is called ‘nociplastic pain’ and can get
worse over time without appropriate treatment, as your body continues
to “learn more and more pain”.
There are also other types of pain related to your previous pain
experiences, your previous traumas, and spiritual and emotional pains,
and these will all feed into your pain experience in the present moment.
Sometimes opioid medication (such as morphine or oxycodone) can
cause changes in your body’s pain detection systems and can make your
pain feel worse. Also, if you have been taking opioid medication for a
longer period of time, when you reduce the dose, that can cause an
increased feeling of pain because the body has become used to this
medication. Opioids, at higher doses, can switch from being
pain-relieving to pain-producing, and so some people feel a lot better
for reducing or stopping their opioid medication.
For some people, more than one of these causes of chronic pain can
happen at the same time. We don’t fully understand all these causes of
chronic pain, but we do know that people with chronic pain benefit
from different treatment approaches, and we want to make sure you
are getting the right help and advice.
How Should Chronic Pain Be Treated?
Sometimes the cause of the pain can be treated directly – such as
surgery for a joint affected by avascular necrosis. However, for most
people this is not the case. Often, for very many people with chronic
pain, it is impossible to completely remove the pain, even with these
recommended treatments. Instead, the aim is to work with you to
improve your pain and how you live with it, to help you live a happier
and fuller life even though you might still be living with some chronic
pain. Pain is just a set of electrical impulses that your brain detects and
gives you the experience of “pain” which is different for everyone and
why not everything works for everyone. However, just like your brain
and body are able to “learn pain”, and upregulate it over time, the
opposite is also true, and you can “learn” over time to reduce how your
brain and body interprets those electrical impulses and how to reduce
your pain experience.
Non-Medical Treatments
The following approaches can be very helpful. Some may work very well
for you, and others may not be quite as effective for you – but it is worth
trying them to see if they are right for you:
• Relaxation, mindfulness and meditation: regular deep relaxation
can really help with your pain, especially when used daily. Our
psychology team can help you to learn and use these techniques.
Alternatively, there are relaxation and mindfulness apps you can
download and use for free on your smartphone or other devices.
• Staying active: Regular gentle or moderate exercise will help you
to stay well and improves chronic pain. If it has become difficult to
move a particular joint, or to stay mobile overall, getting
physiotherapy advice on exercises that can help you strengthen
your muscles and become more active will be important. Take a
look at these videos produced by specialist physiotherapists
specifically for people living with sickle cell which will guide you
through some exercises: Resources
Yoga, pilates or tai chi can be particularly helpful types of exercise
because they help with relaxation as well as keeping your body
strong and mobile. These can be done in bed or a chair, as well as
standing, and can be found for free on websites such as Youtube.
There are also many classes around your local area that may help
you even more, and can be quite sociable.
• Helping your emotional wellbeing: living with a long-term
condition like sickle cell disorder can be hard, and chronic pain can
impact your emotional and psychological wellbeing. If you are
feeling stressed, anxious or depressed this will also make your pain
worse, so it is very important to get support with your mental
health. Our psychology team can provide tailored support and
therapies to help with improving your pain and to help you live
well even with pain.
• It is also important to make sure that you are getting the financial
support that you may be entitled to – our Benefits Advisor can help
with this.
• Massage and acupuncture can be very helpful, although
unfortunately these are not routinely available on the NHS outside
of hospital. If you have someone who can give you a massage at
home this could help, or some people are able to pay for private
treatment and choose to do so.
Medication Options
• Sometimes medication such as paracetamol or ibuprofen can be
helpful long-term. Speak to your doctor if you are finding
ibuprofen (or NSAID medication) helpful long-term so that they
can monitor for any harmful effects such as kidney damage.
• Opioid medication is not usually helpful for chronic pain and
causes harmful effects if used for more than a short time. If you
are on long-term opioids, your medical team can discuss with you
the benefits of trying to gradually reduce this medication, and how
to do this safely, as whilst opioids are useful in acute sickle cell
crises, they are generally not useful in the long-term for chronic pain as they stop working and will cause more harm (see Opioids
Aware below). Minimising opioids also means that you keep them
in reserve and they will work better when you really need them in
future, for example in a painful crisis.
• Other, more specialist medications or treatments might be used to
help with chronic pain, and can work much better for you than
opioid medications
Specialist Pain Team
If your chronic pain is very difficult to manage, or is not improving with
these treatments, you may be referred to a specialist chronic pain team.
They have access to other treatments and can work with you to plan
best how to manage your pain longer-term.
If you have any further questions about managing your pain,
or if you would like referral for any of the services listed here,
please speak to your nurse or doctor.