If you have been diagnosed with Guillain-Barré syndrome or one of the acute inflammatory neuropathies, this guide will help you find out more about these conditions and plan for the challenges ahead. You are welcome to download this guide. This guide has been written by The Medical Advisory Board of Guillain-Barré & Associated Inflammatory Neuropathies (GAIN) and gives an honest account of the conditions but not all the content will apply to you. Your illness will be unique to you so ask your consultant or doctor if there is anything you don’t understand.
GBS & variants
What is GBS?
Guillain-Barré syndrome (GBS) is a rapid-onset autoimmune condition that damages the peripheral nervous system. This causes weakness and loss of sensation that usually recovers completely, or almost completely, after a few weeks or months.
Is there more than one type of GBS?
Yes – GBS is a clinical syndrome rather than a specific individual illness.
In the majority of GBS cases, when the nerves become inflamed and lose their insulation (demyelinated), the syndrome is due to ‘acute inflammatory demyelinating poly[radiculo]neuropathy’ or AIDP. This myelin sheath can be replaced by the myelin forming cells, named Schwann cells.
In the AMAN (acute motor axonal neuropathy) and AMSAN (acute motor and sensory axonal neuropathy) forms of GBS, the axons are damaged too. Although they can regrow, recovery takes longer and may be incomplete. Patients with AMAN or AMSAN may therefore make poor recoveries.
A variety of the acute condition is Miller Fisher syndrome (MFS) which can affect the eyes. There are several other very rare conditions that are categorised as clinical variants of GBS; often they do not exhibit the full range of symptoms of the ‘classic’ description. Although the efficacy has not been clinically proven, treatment of Miller Fisher syndrome is much the same as ‘classic’ GBS though the different symptoms require modified management with emphasis on the eyes. Intravenous immunoglobulin or plasma exchange treatment is likely in all but the mildest cases. The chances of recovery are good.
What causes GBS?
The disease is due to inflammation of the peripheral nerves, often termed ‘neuritis’, hence GBS is called a ‘polyneuritis’. The peripheral nerves connect the central nervous system to the muscles and to the sensory organs in the joints and skin. When these are damaged the muscles and sensory organs stop working, although they are in themselves undamaged.
The most likely explanation for the inflammation is that immune cells called lymphocytes start attacking the nerves in error, instead of concentrating their energies on fighting off infections. It is believed that the immune system has been tricked into making this mistake by an infection that often precedes GBS. Eventually the immune system realises its mistake and stops the attack on the nerves. A disease in which the immune system attacks its host’s own body is called an autoimmune disease and GBS is one of many diseases affecting the nervous system in this category.
Who can get GBS?
Studies of GBS indicate that:
It affects 1-2 per 100,000 each year in the UK (between 650 and 1300 people)
It affects any person at any age
It is slightly more common in older age
It is more common in men than in women;
It is not hereditary or infectious
It often develops a week or two after a throat or gut infection.
What are the symptoms?
In most people, the first symptoms are:
Tingling or numbness in the toes and fingers
Legs feel heavy and wooden
Arms feel limp
Hands cannot grip or turn things properly.
A few people will recover within a week or two, however, most people will need to go to hospital. When you first feel unwell you might find your doctor doesn’t know what is wrong and sends you home. Within a few days it will be obvious as you struggle to stand or pick things up and your doctor can’t find your tendon reflexes.
How is GBS diagnosed?
The diagnosis of GBS is made from
• The clinical history (the story you tell your doctor)
• Medical examination
• Tests such as blood tests, a lumbar puncture or electromyogram (EMG).
A bit more about the tests
The lumbar puncture tests for protein levels in the spinal fluid and involves lying on one side and having a needle inserted into the base of the spine under local anesthetic.
The electromyogram is an electrical recording of muscle activity and is a very important part of making the diagnosis of GBS Often in GBS, nerve conduction is slowed or even blocked altogether. The test usually lasts about half an hour. Some patients find the electrical stimulation rather uncomfortable but it is entirely harmless.
Occasionally the diagnosis can be delayed for a few days while they are checking the results, and your doctor may start the treatment just to be on the safe side.
Is there a cure or any treatment for GBS?
Trials have shown that, on average, for severely affected patients in the first week or two of the illness the following alternatives can halve the duration of the illness although the do not necessarily lead to an instant cure and some patients will continue to get worse.
• Plasmapheresis, where plasma is exchanged over 5 days
• Intravenous immunoglobulin (IvIg)
The above two treatments are probably not worthwhile in mildly affected patients, ie those who can still walk across a room unaided.
The longer the delay in starting treatment, the less likely it is to be effective and some experts feel it is not worth giving any treatment after the first couple of weeks, unless the GBS patient is still deteriorating. Occasionally patients require two courses of treatment.
Although they do seem to shorten the duration of the illness, particularly the time on a ventilator and the time to walk unaided, they are a help rather than a cure.
Since GBS usually gets better on its own, a very important part of treatment is general nursing and medical care with physiotherapy and, if necessary, intensive care. No drugs have been proven to make any difference to the speed of recovery at this point in time.
How long does it take to recover?
Most people are usually admitted to hospital and recovery times varies: –
Get weaker reaching the worst degree of weakness within 4 weeks and always within 6 weeks
Then plateau for a few days or weeks, where nothing changes.
During this period it is good to keep the joints moving – a physiotherapist will be able to advise friends and relatives who want to help
The time it takes to recover can vary from a few weeks to a few months, or sometimes several years. The vast majority of people recover within a year.
A small number of people deteriorate very quickly and can be severely paralysed within a couple of days and may require intensive care
Is GBS painful?
Some patients get a lot of pain during GBS, particularly in the spine and in the limbs but other patients report GBS as an entirely painless experience, even when severely paralysed.
What can cause the pain:
inflammation of the nerves themselves,
muscles that have temporarily lost their nerve supply,
lying in an uncomfortable posture.
The doctors will prescribe painkillers and the nurses and physiotherapists will help with repositioning and physical therapy. It helps to know that some pain is common in GBS. This pain should disappear as the condition improves and the occurrence of pain does not mean that anything else is going wrong.
Do patients need intensive care?
Around a quarter of GBS patients are admitted to intensive care units (ICUs, sometimes called intensive therapy units or ITUs), particularly those with weakness of their breathing, swallowing or coughing muscles.
Patients may need to be attached to a ventilator which will simulate their own breathing requirement while he/she is temporarily unable to breath unaided. This may be done via the nose or mouth in the shorter term, or for those patients who are likely to require ventilation for more than a short period, then via a tube placed through a small incision in the neck (tracheostomy). This small opening is quite comfortable and will be closed up as soon as the patient is able to breathe again without assistance from a ventilator.
The patient’s heart will be monitored on a screen to watch for any irregularities. A thin tube (catheter) may be used to drain urine from the bladder. To feed the patient whose swallowing ability is impaired by the GBS or made impossible by the plastic breathing tube, a special tube called a nasogastric tube will be passed through the nose and down the throat and oesophagus into the stomach so that liquid food may be taken in. Each support mechanism will be discarded as the GBS sufferer improves and they are no longer needed.
http://www.criticalcarerecovery.com/ has been put together by researchers, patients, families, healthcare staff and website developers using interviews with over 120 patients and their families in Scotland to answer questions about intensive care.
How does the patient feel?
Patients may take time to adjust to their surroundings and the problems caused by GBS. Hearing is rarely impeded so the patient can generally understand and acknowledge all that is going on and a caring and sympathetic attitude towards them will help. Other things that can affect them can include:-
unable to speak
reduced or absent sense of taste and smell.
visual disturbance. Hearing is rarely impeded, so the patient can generally understand and acknowledge all that is going on.
reduced awareness due to sedative or painkilling
increased skin sensitivity so although touch is important, care must be taken as , although rare, a light touch may cause very severe pain
paralysis, although temporary can be quite extensive
pain may be experienced to a greater or lesser degree
feeling hot or cold.
hallucinations, unusually vivid daydreams or nightmares, are not uncommon for ventilated GBS patients.
patients can feel vulnerable, isolated and locked-up inside their illness.
tearful, bad tempered or panicky.
What can family and friends do to help
This is a very personal illness and each patient has his or her particular set of problems and worries to cope with. Your role is to offer love, comfort and reassurance during this difficult period. To do this effectively, you must remain calm and resolute and give constant encouragement on progress. Patients easily lose sight of how they are doing so keep yourself well informed by the medical staff. Writing a diary of daily events will help you keep a perspective on progress. You can relate this to the patient who may not realise how he/she is getting on.
Some days are better than others for GBS patients, but you must try to keep up a constant flow of encouragement.
For the close family, this period of the illness is quite stressful, so don’t forget
to look after yourself and stay well.
How long does it take to recover?
The time taken for recovery to occur is very variable. Sometimes it is only a week or two but most people remain affected for between three and six months, some much longer and some will not recover fully.
Those who do not recover completely may be left with minor degrees of weakness, numbness and sometimes discomfort that do not seriously interfere with their lives. A few however are left so disabled that they cannot resume their former occupations. This is usually because of residual weakness of their arms and legs so that manual work and walking are impaired. It is uncommon to be left dependent on a wheelchair for life but this unfortunately does occur in some cases. Improvement is fastest during the first few months but some patients report continued gradual improvement even after a year or two has elapsed.
One lasting problem is fatigue. It can be difficult to measure or treat fatigue but pausing the return to normal life and not overdoing activity too fast usually helps fatigue in the longer term.
What happens next?
Leaving hospital or a rehabilitation centre and heading home can be daunting and take a while to arrange. You may need equipment to help with everyday tasks, your home may need adaptations or you may need a care package in place to help. There are many people and organisations that can help with this starting with the occupational therapists and your care team. Your family can also be a great help in getting the information together and speaking to organisations that have in depth knowledge of what help is available.
Assessment & care plan
If you are likely to have ongoing health and social care needs you may have an assessment carried out by a multidisciplinary team of health or social care professionals such as social worker, physiotherapists, occupational therapist, psychologists or dieticians. You, and/or a family member should be involved in this process. A care plan should include details of:
the treatment and support you will get
who will provide support
when and how often you will get support
how the support with be monitored and reviewd
a named person who will coordinate the care plan
who to contact
The type of support that might be in a care plan
Community care services to allow you to live in your home
NHS continuing healthcare
NHS funded nursing care
Rehabilitation or palliative care
Support from voluntary agencies
You could be entitled to benefits to help you support yourself.
The benefits system is complex and changes often. Citizen’s Advice website has up-to-date information and can help you make an application:
To find out what benefits you (and your family) may be entitled to, you can complete an anonymous benefit check on the Citizen’s Advice website or visit your local office and talk to one of their benefits experts.
PERSONAL INDEPENDENCE PAYMENT (PIP)
For people aged 16 – 64 who have had a disability or long-term health condition for at least three months, which is likely to continue for at least nine months after your claim.
Points system assesses how your condition affects your ability to cope with daily life and mobility.
If awarded, there is a daily living component and a mobility component. Each has two rates; standard and enhanced.
Not affected by income or savings, not taxable and you can get it whether in work or not.
ATTENDANCE ALLOWANCE (AA)
For people aged 65 and over who have a health condition which has lasted at least six months.
Entitlement is based on care needs resulting from how your health affects your everyday life.
Not affected by any income or savings you have; payable alongside any other benefits (except Disability Living Allowance or Personal Independence Payments). You do not have to have paid national insurance contributions.
DISABILITY LIVING ALLOWANCE (DLA)
For adults aged 16 to 64
Starting from April 2013, this benefit is now being replaced by Personal Independence Payments (PIP). All new claimants must now apply for PIP.
People who are already in receipt of DLA will be invited to apply for PIP. To find out more use the PIP checker: www.gov.uk/pip-checker
Some people who currently qualify for DLA will not quality for PIP, and some who do not qualify for DLA will be able to qualify for PIP.
DISABILITY LIVING ALLOWANCE FOR CHILDREN
For children aged under 16 who have a health condition or a disability and need help with personal care/supervision or help with getting around outdoors because of this.
Designed to meet additional expenses of having a child with a long term condition (eg, higher heating bills, special diets, taxi fares, etc).
Some adults may also still be getting DLA if they claimed before 10 June 2013 but will be invited to claim Personal Independence Payment (PIP). To find out when this will affect you, use the PIP checker: www.gov.uk/pip-checker
EMPLOYMENT AND SUPPORT ALLOWANCE (ESA)
Payable to people unable to work because of ill health or disability.
Requires a medical certificate (‘fit note’) from your GP to make a claim.
You will be required to fill out a medical questionnaire, attend a medical assessment and a work-focused interview.
This is intended to determine your capability to work. It is possible to challenge the decision.
Treatment of CIDP is usually very effective with about 80% of new cases having a dramatic response to therapy. Although some patients go into a long term remission after a short course of treatment, many require long term treatment of one form or another. Drug treatments are generally thought to work by suppressing the autoimmune response, which in turn reduces the disabling symptoms of the disease.
It is normal to complain of persistent symptoms for weeks and sometimes months after you have been discharged from hospital with GBS. These symptoms vary enormously from patient to patient and include weakness, tingling, and painful tingling, aching in the limbs, cramps and tiredness. It is normal for these symptoms to fluctuate a bit, being worse when you are tired, stressed or affected by an intercurrent illness, such as a cold, sore throat or flu. They gradually wear off, but you may feel some of them coming back in a milder form at times of stresses like that for a year or two. This does not usually mean that the GBS is coming back as recurrence is very rare indeed.
There is nothing which can be done to alter the very, very small risk of recurrence of GBS. Although there has been concern that some immunisations might have precipitated GBS, there is no hard evidence to support this notion with immunisations which are in common usage in the UK today. However, it would seem unwise for someone whose GBS had come on within six weeks of an immunisation to receive the same immunisation again. Furthermore, many neurologists advise patients not to have immunisation for a year after the onset of their GBS, just in case. Since GBS occurs after infections, you might think it desirable to avoid contact with infections. To try to do so would be a practical impossibility, and anyway, not worthwhile since you will now be immune to whatever infection triggered your first attack of GBS.
There is a wide range of different practice regarding hospital follow-up visits. Unless you are taking medicines on account of intercurrent medical illnesses, or unless you are being prescribed medicines for pain or complications, you do not need to attend for prescriptions. In fact the person you are likely to need to see more than anyone else is your physiotherapist, rather than a neurologist. However, if you have been very ill and are recovering from a frightening experience such as GBS, it is appropriate to continue to consult a neurologist at appropriate intervals (gradually decreasing) until your health is back to normal, or you have learned to cope with whatever disability the illness has left you with.
Hygiene and cleanliness
Personal cleanliness for those who are unable to attend themselves fully can be a problem. Many returning home from hospital may have reduced use of their hands, usually temporary, but occasionally permanently. Many will be unable to wash themselves, brush their hair, use the lavatory, wipe their bottoms, brush their teeth, cut their nails etc. It is important for both hygiene and self-esteem that these matters are attended to.
Through no fault of their own, many people’s teeth are neglected during periods of serious illness. Once you have returned home from hospital, arrange an appointment with your dentist as soon as possible. There may be physical barriers making this difficult, as many surgeries have inadequate access for wheelchairs etc. If this is the case there may be a community dental service available that can help. Using an electric tooth brush can be helpful if you have residual weakness in your hands.
Other areas of support
During illness, nutritional needs are at their peak, but it is not unusual for patients to lose their appetites or taste for food. Worry and fear often accompany illness and can also contribute to loss of appetite. Good nutrition can be a powerful ally in the process of recovery, ask to speak to a nutritionist for more advice on diet. If taste has been affected, this will usually improve with time. Plastic utensils can be used if bitter or metallic tastes are experienced whilst eating. Sometimes taste changes can be related to medications, but drugs should not be discontinued without first consulting your GP.
During the recovery stages, physiotherapy, occupational therapy (OT) and speech and language therapy play a vital role in the rehabilitation process as well as maximizing functional ability. At some point during rehabilitation the rate of recovery will plateau and it is often at this point that patients will be discharged from all the support services on which they may have relied. It is also possible that patients may be placed ‘on review’. This means that you may be followed up at regular intervals and can telephone for advice in-between but don’t attend the clinic as often as you did before.
The role of exercise in the ongoing rehabilitation for patients with GBS is still to some extent unclear and clinical trials are being carried out to improve our understanding. However, there is already some evidence that where weakness and fatigue are problems, participation in regular graded exercise can be beneficial.
Exercise can help to improve your muscle strength and reduce your overall sense of fatigue. There are also general benefits of exercise in boosting the immune system, helping your heart and lungs remain healthy and making you feel better about yourself. However, it may take weeks or even months before you feel the benefit of exercise so it is important to pace yourself. Therefore, you should be encouraged to seek advice on whether and how to start regular exercise.
Pain may never be a problem but can occur in the early recovery phase, The problem does tend to resolve as recovery proceeds.
As pain can make one irritable and difficult to live with at times, it is important that family and friends are kept informed, so that they can understand the reason for such behaviour.
Remember that because the nerves to the hands and feet are the longest in the body, pain will linger in the extremities after it has left other parts of the body.
GBS, CIDP and associated inflammatory neuropathies can bring on problems in any relationship, sexual relationships are not excluded. Dealing with a long term illness or disability can put a great strain on a relationship, particularly when one partner is partially or totally dependent on the other. Even without the actual physical disability, the emotional upheaval can interfere with a couple’s sex life and this can be difficult to talk about. This can mean that the once close, intimate relationship can become distant and stressful for both partners. Help is available so speak to your GP or a relationship counsellor.
With all the changes in your health it is not uncommon to feel anxious or angry which can be helpful in giving the mental and physical energy that is needed to anticipate and tackle problems. An acute stress reaction is recognised as being an entirely normal part of the process of adjusting to a life change. You can help by seeking information and discussing issues that are worrying you. Most people will make a good psychological recovery but some will continue to experience anxiety and low mood making everyday life difficult.
There is help available and you can speak to your GP or neurologist about this. They will be able to arrange suitable help for you.
What should patients who have had GBS be advised about future immunisation?
The belief that GBS is an autoimmune condition and the knowledge that immunisations are designed to activate the immune system give rise to continued unease about immunisation following the disease. However, many patients have received immunisations after the acute phase of their disease, sometimes repeatedly, without suffering a recurrence.
Statement from a Medicines and Healthcare products Regulatory Agency (MHRA) spokesperson:
Every year, more than eight million doses of flu vaccine are administered across the UK to those aged 65 years and over, with millions more given to healthy younger people and those at higher risk of flu.
As Guillain-Barré syndrome (GBS) can occur naturally across all age groups, and with such high exposure to flu vaccines, it is inevitable that there will be a few cases of GBS after a flu vaccination is given. This association does not necessarily mean the vaccine is the cause. Indeed, for every million people aged 65 years and over who have the vaccine each year, we expect three cases of GBS to occur within six weeks of vaccination by coincidence alone.
Suggestions that flu vaccines may cause GBS can be pinpointed to a very specific type of vaccine given to patients in the USA in 1976. The vaccine was not used in any other countries and a theory was that this risk was due to a contaminant in that particular type of vaccine.
Since then, numerous population-based studies in many countries have looked at whether currently-used flu vaccines may also be a cause of GBS. A couple of these studies have suggested that if there was an association, it would be an extremely small risk – in the order of one case for every million people vaccinated. However, most studies have found no association and, taken together, the evidence is far more suggestive of there being no causal association between flu vaccination and development of GBS.
If you would like to report a suspected side effect you have experienced after taking a medicine or vaccine, including the flu vaccine, then we would encourage you to do so either via the Yellow Card website https://yellowcard.mhra.gov.uk/ or download the free Yellow Card mobile app from iTunes Yellow Card for iOS or Playstore Yellow Card for Android devices.
Advice for the carers
Here are a few practical steps that can help to counteract the stresses and strains of caring for someone suffering from GBS and associated inflammatory neuropathies:
Gather support from family and friends. Invite help from the local social services/social work department both practical and financial
Contact a local caring organisation providing support services in your area. They will often help to bath and dress patient, providing a respite so that the carer can go shopping or have a bit of time of their own.
Take a rest from your duties and allow yourself some personal space. Go for a walk, listen to relaxing music, visit friends etc. Generally take care of yourself, eat healthily, and get plenty of sleep. When friends or relatives visit the patient, take this as an opportunity to have a break and use this time to do something for you.
Take the pressure off by putting some activities on hold.
Be mindful of the patient’s limitations.
Talking is therapy and you may also find it useful to speak to an external source: friends, relatives, caring organisations, GAIN
Get organized. Investigate benefit entitlements with the hospital social worker and/or Social Services/Social Work Department. Liaise with the hospital occupational therapist (OT) and physiotherapist about equipment arrangements.
Be temperature conscious if the patient is suffering from lack of sensation, ie run and test the bath water. This also applies to the cooker, iron etc. as there may be no sensation and a patient can get burnt or scalded very easily.
Taste buds may be affected for a while, so prepare meals to suit the patient. Vitamin supplements can be included if a balanced diet cannot be achieved.
Beware of falls brought about by weakness or unsteadiness.
Be mindful of potential accidents resulting from weakness and/or numbness (ie dropping things). Care should be taken when the patient is using hot appliances, such as when cooking or ironing. A microwave oven is a very convenient, safe way of preparing food.
Help the patient with daily exercises. Ensure that everything is done in moderation and that the patient does not start rushing around too soon. Encourage the patient to talk openly about his/her experiences and fears.