Created in conjunction with Anita Simonds MD FRCP Royal Brompton Hospital and Francesco Muntoni MD FRCPCH Hammersmith Hospital.
What is it?
Pain is not something that is normally associated with SMA. However some of the complications of SMA (hip dislocation, curvature of the spine), their surgical treatment, or special investigations (blood tests) can all cause pain.
Pain is the body's natural way of saying that something is wrong. We all feel pain, adults and children alike. What varies is our pain threshold, with people having a higher or lower ability to tolerate pain.
If your child is old enough, they are likely to be able to tell you that they are uncomfortable, but if not there are other signs that may tell you they are in pain. Your child may seem noisier or quieter than usual, they may be sleepier, more tense or anxious than normal or they may look flushed or pale. As parents you are often in the best position to judge, as you will usually notice when your child looks different.
Terminology Explained
Pain-relief Drugs - any pills, tablets, medicines, injections, creams, sprays or suppositories that help reduce pain.
Analgesia - mild to moderate pain relief.
Anaesthetist - Doctor who specialises in pain relief and the administration of anaesthetics.
Anaesthetic - Pain relief given when you have an operation.
Local Anaesthesia - given to provide loss of feeling to stop pain in a part of the body either by - a special cream or gel rubbed into the skin which dulls or blots out the pain from needles - or by epidural /spinal: a tiny tube fitted near to the spine, through which pain relief can be slowly fed.
General Anaesthesia - when the child is unconscious and put to sleep and feels no pain, for example during an operation.
Anti-emetics - drugs that stop you feeling and being sick.
Acute - short-term, sudden onset
Chronic - long term
What Can be Done?
There are two main ways of coping with pain: with drugs and without, depending on the severity and duration of the pain.
Examples of Pain Relief Drugs
For mild pain: Paracetamol; paracetamol suspensions such as Calpol,Tixymol, Disprol. (These are available to buy without prescription in a pharmacy.)
For moderate pain: Codeine, Diclofenac, lbruprofen, lndomethacin, Naproxen
For severe pain: Morphine, Diamorphine, Pethidine
A child who continues being in pain may need to have a higher dose, or to take the drug more often, or to go onto a stronger drug, within safe limits.
Severe pain needs powerful drugs, and they will not make your child addicted. Even if they have to take morphine for weeks, children come off it quite easily when the pain goes.
Drugs will work slightly differently in everyone and so there is no such thing as a standard dose. The amount is right if it suits your child and working out the best safe dose is vital.
Some drugs make children feel sick or itchy, they may become constipated, or have strange dreams. These side effects can be reduced by using extra drugs, or by changing the pain control method.
Most mild analgesic drugs have few side effects. However, strong analgesia particularly with opiate drugs such as codeine, morphine, diamorphine and pethidine can depress respiration in children with significantly reduced breathing capacity. In this situation it is perfectly possible to control pain, but breathing should be monitored closely and in some cases breathing support is required e.g. Non-invasive ventilator. Breathing should also be monitored closely in children with respiratory muscle weakness following surgery under general anaesthesia. Sedative drugs should not be used unless respiratory support is available. Paracetamol, ibruprofen, diclofenac, naproxen and other non-steroidal anti-inflammatory drugs, and local anaesthetic preparations do not depress respiration. If in doubt about medications that can affect breathing it is best to ask for medical advice.
How Can Drugs be Given?
Pills, tablets or medicine by mouth - this is the easiest way but relies on children being able and willing to take them. It also takes a while to have an effect.
Injections - either directly into a vein or into the muscle.
IV Drip - if it is not possible for your child to have pills or medicine, they will probably have a drip fixed into a vein. If the ward has the right kind of pump, drugs may be fed painlessly through the drip.
Cannula/Butterfly/Central Line - a needle left in the skin, taped on so that it can't be felt. Drugs can then be given painlessly through the needle.
Spinal or Epidural - in some hospitals, after some major operations, anaesthetists set up an epidural near the spine. They may also give a nerve block injection - before the child wakes up which can dull the pain for hours.
Other Options - creams, skin patches, suppositories and nose sprays can relieve some pain.
Patient Controlled Analgesia (PCA) - a pain relief drip timed to give a set dose, very slowly. There is a small button or pump for patients to press when they want or need it; either because their pain is coming on again, before physiotherapy, or when a wound is dressed. It is not possible to overdose with these drips.
Other Strategies
Pain is much worse when children are scared, tense or tired, so providing comfort and support is a real help. Simply being there helps, especially during tests and treatment, so do ask if you can stay if your child wants you to and you can cope! Stroking and cuddling can make them feel better and distracting them with stories, videos and games can all help.
Fear increases pain and our children's imagination can be just as lurid as ours. Children need to know what to expect and to be given some control where possible. Don't tell them it's not going to hurt if it is! They will feel less upset if they are told honestly: when something is going to hurt, why they have to have something painful done to them that it is meant to help them and what can be done to reduce the pain. If there is time, i.e. for planned surgery, it is useful to talk through with your child what is going to happen and let them ask any questions they have. Even if you don't immediately know the answers it should be possible to obtain them from the professionals involved.
Working on keeping yourself relaxed can also help your child. Deep breathing or slowly counting breaths in and out can help and you can encourage your child to try them too.
If your child has to have an injection, then rubbing the other arm may help.
Pain Clinics
Where ongoing pain is a problem, then a referral to the local pain clinic may well be helpful. Pain clinics have experts in both medical management of pain and psychology management techniques as well as having access to alternatives including things like TENS machines and acupuncture. | 
