Let’s face it, you or someone you know is probably thinking ‘how do I say it?!’
Well, you can thank the Brain Foundation for giving the pronounciation of GBS as ‘ghee-yan bah-ray’ syndrome, named after three French neurologists, ‘who described the condition in two soldiers in 1916’ (2017).
Guillain Barré Syndrome (GBS) is an autoimmune disease in which the peripheral nerves and nerve roots are damaged by the immune system’s antibodies and lymphocytes (Brain Foundation 2017).
Often it will damage the myelin (the nerve insulation) and the axon (the inner covered part of the nerve) which then delays and changes signals to and from these nerves and the rest of the body (Better Health 2014; National Institute of Neurological Disorders and Stroke 2011; Vellozzi et al. 2014).
“As a result of the attack, the nerve insulation (myelin) and sometimes even the inner covered part of the nerve (axon) is damaged and signals are delayed or otherwise changed. The resultant antibodies attack the myelin sheath, and sometimes the axon, causing paralysis and muscular weakness as well as strange sensations, as the sensory nerves of the skin may be affected.”
(Better Health 2014)
Because of the damage caused by GBS, the patient will often present with progressive bilateral and symmetrical weakness and paralysis of the lower limbs as well as disturbed sensory sensations.
Described as ascending paraesthesia (NINDS 2011), the symptoms usually begin in the feet or legs, gradually progressing up the body during the following days and weeks. GBS has also been found to begin in the arms and progress downward but this is less common (Better Health 2014; NINDS 2011; Vellozzi et al. 2014).
The affected person may experience motor nerve related issues such as weakness or paralysis, and/or sensory nerve related issues such as tingling (Brain Foundation 2017).
GBS is not considered to be contagious (Better Health 2014). It can be diagnosed by: muscle strength testing; reflex tests; nerve conduction velocity testing; spinal tap; and physical examination of the signs and symptoms of GBS (Better Health 2014).
(NINDS 2011; Better Health 2014)
(Better Health 2014).
Individuals who are experiencing this range of symptoms will often be at an increased risk of other complications such as DVTs, PEs, pressure injuries and pneumonia (to name a few), further impacting on their recover (Better Health 2014).
Generally the symptoms will initially begin over a period of roughly 12 hours before reaching a plateau. The time in which an individual will reach this plateau varies but can be up to weeks following the initial onset of symptoms. From this plateau, the person will then begin to improve and start their recovery (Better Health 2014; NINDS 2011; Vellozzi et al. 2014).
Note that GBS is relatively rare with roughly 2-8 people being affected by this condition in every 100,000 people, is seen to be more common in the 30-59 year old age group and affects both sexes equally. Most patients do recover, however approximately 5% of patients will die (Better Health 2014; Vellozzi et al. 2014)
The cause of GBS remains unknown. However, it is also important to note that in two-thirds of patients with GBS, the onset of symptoms is preceded by either an upper respiratory tract infection or a gastrointestinal infection occurring 1-3 weeks earlier (Better Health 2014; NINDS 2011; Vellozzi et al. 2014).
The specific infectious agent remains unknown in over half of GBS cases. However there have been several infectious agents that have been associated with the onset of GBS (Vellozzi et al. 2014).
Other events which have been found to potentially be related to the occurrence of GBS include pregnancy, dengue fever, surgical procedures, insect bites and Bell’s palsy (Better Health 2014).
This is why Guillain-Barré is called a syndrome, not a disease. In a disease, there is a clear specific disease causing agent that can be identified. A syndrome however, is classed as a collection of symptoms and signs or what the patient feels and what the doctor can observe – not defined by a clear causing agent. This is why diagnosis can be so difficult for syndromes in comparison to diseases (NINDS 2011).
As with most conditions, it can range from being classed as mild to severe. In the most severe cases, the individual will often need life support as the nerves controlling their respiratory function and the autonomic nervous system are involved. Once the autonomic nervous system is affected, the individual can experience changes to their blood pressure, heart rate, vision, kidney function and regulation of body temperature (Better Health 2014; NINDS 2011).
This difficulty in diagnosing GBS is mainly due to the range of symptoms the individual presents with, that initially appear vague and unrelated to each other. Diagnosis will often rely on a physical examination including muscle strength and activity tests, reflex tests, nerve conduction tests and spinal taps to check for increased protein in the cerebral spinal fluid (Better Health 2014; NINDS 2011).
Note: GBS is an emergency condition because it could lead to dangerous heart rates, blood pressures, and/or respirations (NINDS 2011). Kidney function and body temperature may also be affected (Better Health 2014).
Treatment for GBS always occurs in hospital. The person, no matter how insignificant their symptoms may initially appear, are always admitted into hospital for monitoring. This is so that if any complications occur that may affect vital functions, treatment can be initiated immediately (Better Health 2014; NINDS 2011).
There is no cure for GBS, however some treatment options include Gammaglobulin (IVIG) infusions over a period of 5 days. IVIG infusions help lessen the immune attack on the nervous system, however it is not know completely how or why this works. Plasmapheresis can also be undertaken in which the blood is taken from the person, the immune cells are then removed and the remaining red blood cells are returned to the body. Both are effective in reducing the severity of GBS and assisting in recovery, however IVIG infusions have been proven effective through trials and are often easier to administer so may be done more often than plasmapheresis (Better Health 2014; NINDS 2011).
Recovery from GBS varies. Most people spontaneously recover, however approximately 10-15% of people are also left with lengthy rehabilitation and permanent disabilities. Recovery times can vary between 6 months and two or more years. Most often the recovery time is better for the individual whose symptoms begin to ease earlier (Better Health 2014).
As with any neurological condition, and no exception being made to Guillain Barré Syndrome, assessment of the patient on entry to rehabilitation is essential. This assessment will include areas of the clinical history of the patient, initial presentation and any other medical conditions as well as other presenting symptoms such as paralysis, sensation disturbances, fatigue, spasticity and muscle tone (Mauk 2012).
Assessment is important in order to plan and implement a successful rehabilitation program for the patient. A program will often include extensive therapy from all members of the multidisciplinary team including a physiotherapist, occupational therapist, medical officers, speech pathologist, dietician and psychologist, to name a few, and lets not forget the important role that nurses play in the rehabilitation of someone with Guillain Barré Syndrome.
The role of a nurse during the rehabilitation of someone who has experienced Guillain Barré Syndrome is complimentary to the rest of the multidisciplinary team and involves providing critical education to the patient and family, as well as support and reassurance during recovery.
This education can encompass a variety of areas and will differ depending on the specific individual and their lifestyle. Often the education will be provided to help ensure adherence to the treatment recommendations made by the multidisciplinary team (Mauk 2012). Education may involve strategies to assist in the individual transferring safely or eating their food safely and decreasing their aspiration risk, or medication management for when they discharge home.
As mentioned, recovery from GBS varies, however 30% of patients will still have some residual weakness 3 years following the event, with 3% suffering further relapses of muscle weakness and tingling for many years after the initial illness.
Recovery can also be hampered by the emotional and psychological difficulties that often hinder the patient with GBS (NINDS 2011) – especially those with residual and permanent disabilities following the illness. Therefore, nursing care of the patient with GBS is varied but must look at the holistic view of the patient and take into account where they are at in their recovery from GBS.