Learning Outcome

At the conclusion of this article the reader be able to:

1. Identify and discuss nursing diagnoses relevant to the care of the Gullain-Barre Syndrome (GBS) patient
2. Identify the etiology of GBS 
3. Identify potential causes and risk factors of GBS
4. Discuss the assessment and evaluation of GBS 
5. Describe key components of medical management of GBS
6. Identify key components of nursing management and monitoring of GBS
7. Identify members of the interprofessional team available for assistance 
8. Discuss selected outcomes for the GBS patient
9. Discuss selected elements of interdisciplinary care of the GBS patient
10. Discuss important elements of health education and health promotion for the GBS patient and family members
11. Identify selected risk management concerns for nurses caring for GBS patients
12. Identify selected important elements of discharge planning for the GBS patient in the acute and recovery phases of the illness
13. Describe evidence-based issues related to GBS
14. Identify nursing pearls related to interprofessional practice in the care of the GBS patient

Introduction

Guillain-Barre syndrome (GBS) is the most common cause of acute, flaccid, neuromuscular paralysis in the United States. Guillain-Barre syndrome was first discovered more than a century ago. Advances in the past century include investigating the immune-mediated pathophysiology of the disease, recognizing the spectrum of presentations, advancing diagnostic modalities, prognostic models, and performing randomized trials of treatments to improve outcome. Given the morbidity that can occur without treatment, all clinicians and nurses should have a knowledge of this rare disease.[1][2][3][4]

Nursing Diagnosis

• Impaired respiratory function related to rapid and progressive weakness and impending respiratory failure
• Immobility related to paralysis
• Nutritional imbalance related to swallowing difficulties 
• Impairments to verbal communication related to cranial nerve dysfunction
• Pain 
• Psychological problems 

Causes

The Guillain-Barre syndrome (GBS) and its variants are considered post-infectious, immune-mediated neuropathies. Evidence from animal models suggests a key role of molecular mimicry. In Campylobacter jejuni gastrointestinal infections, a lipooligosaccharide present in the outer membrane of the bacteria is similar to gangliosides that are components of the peripheral nerves.[5] Therefore, an immune response triggered to fight infection can lead to a cross-reaction on host nerves.

Many infections have been linked with GBS. The most common are gastrointestinal or respiratory illnesses. Up to 70% of patients have reported an antecedent illness in the 1 to 6 weeks before the presentation of GBS.[6] During the Zika virus outbreak, many GBS cases were described.[7] Case reports detail many other possible etiologies linked to GBS including medications and surgeries. (Evidence level III)

In 1976, flu vaccination against the influenza A/H1N1 antigen led to a well-documented, increased incidence of cases of GBS; however, further surveillance data of flu vaccinations in subsequent years have described only one additional case of GBS for every 1 million vaccines. Subsequent studies estimate that developing GBS after a flu infection is up to 7 times more likely than developing GBS after a vaccination.[8][9][10][11][12] (Evidence level IV)

Risk Factors

Although rare, with an incidence of 0.4 to 2 per 100,000, Guillain-Barre syndrome (GBS) has major effects on the health care system. The cost of medical care for a patient with GBS has been estimated at up to $318,966. Overall, the cost of treating patients with GBS has been estimated at $1.7 billion dollars per year. Males are affected at a slightly higher incidence than females. Each year, it is estimated 100,000 patients worldwide would contract GBS.[13][14] (Evidence level III)

Assessment

Guillain-Barre syndrome (GBS) patients describe a fulminant course of symptoms that usually include ascending weakness and non-length dependent sensory symptoms. By definition, the nadir is usually reached within 4 weeks. Symmetric involvement is a key feature of GBS.[6] GBS is usually considered monophasic; therefore, a relapsing or remitting course at presentation would be considered atypical.[15] Additionally, a prior GBS event (recurrent GBS) is also unusual, occurring in < 10% of all patients.[16] If the patient reports progression beyond 8 weeks, other diagnoses should be considered.

GBS often presents (up to 70% of patients) within 1 to 6 weeks of antecedent illness.[17] Other antecedent events that have been linked with GBS include vaccinations (specifically a 1976 strain of swine flu vaccine), surgery, trauma, or other infections. [17][11] 

Classically, patients with GBS will have a pattern of proximal and distal weakness, which is flaccid and often profound if hospitalized. Significant neck flexion weakness may be present and can portend the need for intubation. Areflexia or hyporeflexia is usually present. (Rare cases without hypo/areflexia have been described, mostly in the AMAN variant of GBS).[18] Besides the flaccid weakness and areflexia, patients experience non-length-dependent sensory symptoms; therefore, unlike more common chronic neuropathies such as diabetic neuropathy, patients may report dysesthesias in the hands followed by the feet. Patients can develop facial diplegia due to the involvement of both facial cranial nerves. They can also develop dysphagia due to the involvement of the glossopharyngeal, vagus, and hypoglossal cranial nerves.[6] Autonomic nerves can lead to significant morbidity; therefore, most physicians recommend monitoring in an intermediate or intensive care unit for cardiac arrhythmias or blood pressure lability. Dysautonomia is a primary etiology of the morbidity and mortality attributable to GBS. Additionally, the involvement of the lower cranial nerves (glossopharyngeal, vagus, and hypoglossal nerves) or involvement of the nerves to the muscles of respiration may lead to the need for artificial ventilation. Respiratory failure can occur in up to 30% of patients, usually leading to prolonged hospitalization and recovery.[19]

Besides the classic GBS presentation described above, many variants of GBS have been described. There is a variant with pure motor involvement called "AMAN (acute motor axonal neuropathy)" that is more common in Asian countries.[20] Rarely,  these patients can have normal reflexes.[18] There is also a regional variant involving primarily the pharyngeal, neck, and upper extremity muscles called the "pharyngeal-cervical-brachial" variant).[21] Some variants can involve the central nervous system, termed "Bickerstaff Encephalitis."[22] There is also a variant that presents with paraparesis.[23] Arguably, the most famous variant is the Miller Fisher syndrome.[24][25] This is classically described as a triad of ophthalmoplegia, areflexia, and ataxia; however, other cranial nerves besides the oculomotor nerves have been reported in this variant.[25]

Evaluation

Guillain-Barre syndrome (GBS) is considered a clinical diagnosis; therefore, a diagnosis can be made with confidence at the bedside in most cases. For atypical cases or unusual subtypes, ancillary testing can be useful.[15]

Electromyography and nerve conduction studies may be helpful in distinguishing GBS from its mimics. Nerve conduction studies (NCS) utilize technology to help distinguish between demyelinating and axonal forms of neuropathy. Needle electromyography may help to determine the acuity of a patient’s symptoms. In some cases, these studies may be helpful in evaluating for other considerations in the differential diagnosis such as neuromuscular junction disorders or diabetic neuropathy. Classically, electrodiagnostic studies should be undertaken at 10 to 14 days after symptom onset due to the time for Wallerian degeneration of sensory and motor nerve fibers; however, there have been many studies that reveal that early, nonspecific findings may be helpful in diagnosing GBS as early as 3 to 7 days after symptom onset.[26][27] 

The more common early electrodiagnostic findings in GBS include absent or prolonged H-reflexes and/or F-wave latencies.[28][27] The sural sparing pattern is considered specific for GBS as compared to other polyneuropathies.[29] This pattern would show an intact sural sensory response with abnormal upper extremity sensory responses. Other findings would depend on the variant of GBS. Acute inflammatory demyelinating polyneuropathy would be more likely to have partial motor conduction block, temporal dispersion, slow conduction velocities, prolonged/absent F-wave latencies, and prolonged distal latencies.[30][20] AMAN would usually show a pattern of low, compound muscle action potential amplitudes or even inexcitable motor nerves; however, partial motor conduction block or complete conduction block can be seen in AMAN nerve conduction study (NCS). This phenomenon is explained by “reversible conduction failure.”[31] Complement is deposited in nodes of Ranvier and paranodal regions on peripheral nerves. Subsequently, the nerves can undergo Wallerian degeneration leading to significant and prolonged axonal damage or can reverse, deemed conduction failure.[32][33] This phenomenon explains the relatively rapid recovery of some severely weak patients with AMAN. Sensory nerves would be spared both clinically and electrodiagnostically in AMAN. Acute motor and sensory axonal neuropathy (AMSAN) would show low amplitude motor and sensory potentials. Miller Fisher syndrome is more often described with reduced or absent sensory nerve action potentials.[34] 

Cerebrospinal fluid (CSF) shows a classic pattern of albuminocytologic dissociation. This term means that spinal fluid shows a normal amount of white blood cells and an elevated CSF protein level.[4][15] However, this pattern is only present in 80% of patients at 2 weeks following symptom onset. Therefore, the absence of this classic finding does not exclude the diagnosis. If the white blood cell count is elevated, this should prompt consideration of other infectious GBS mimics, such as HIV seroconversion.[6] 

A number of ganglioside antibodies have been associated with GBS. Antibodies include anti-GM1, anti-GD1A, anti-GT1A, and anti-GQ1B. These range in sensitivity from up to 60% (anti-GM1 antibodies in acute motor axonal neuropathy) to up to more than 90% (anti-GQ1B antibodies in Miller Fisher syndrome). However, these laboratory studies usually require some time to obtain results and, therefore, may not be as helpful in decision making at the time of a patient admission.[35][36][37]

Imaging studies such as magnetic resonance imaging (MRI) spine may show enhancement of the nerve roots, indicating a breakdown of the blood-nerve barrier due to inflammation in GBS. However, MRI utility in GBS is most useful to rule out other etiologies of quadriparesis or facial diplegia such as transverse myelitis or intracranial disease.[38][39]

A negative inspiratory force (NIF) should be performed on patients with suspected GBS.  Serial NIFs should be followed in patients with a high risk of respiratory compromise.  Patients that are unable to perform a NIF of -20 to -30 cm H2O should be considered at very high risk.  

Medical Management

In randomized controlled trials, there are two treatment options currently considered the standard of care in Guillain-Barre syndrome (GBS). These include either intravenous immunoglobulin (IVIG) or plasma exchange. IVIG is thought to act by its immune-modulating action; however, the exact mechanism remains to be elucidated. IVIG is given 2 grams/kilogram divided over 5 days. (Evidence level I)[40]) Plasma exchange is thought to act by removing pathogenic antibodies, humoral mediators, and complement proteins involved in the pathogenesis of GBS. Similar to IVIG, its exact mechanism of action in the treatment of GBS has not been proven. Plasma exchange is generally given as a volume of an exchange over five sessions. Plasma exchange and IVIG have been shown to be equally efficacious. (Evidence level I)[41] The effect is present if either treatment is given within 4 weeks, but the stronger effect may be present if treatment is administered within two weeks. (Evidence level II)[42][43][44][42] Surprisingly, corticosteroids (both oral prednisone and intravenous methylprednisolone) have not shown benefit over placebo or in combination with IVIG and plasma exchange over either modality alone. Overall, treatment is generally considered to shorten the course of recovery of GBS. Treated patients in one study achieved independent ambulation 32 days faster than untreated patients. (Evidence level I)[45][46][43][41][47][48]

 Overall, most patients with GBS do well, with up to 85% of patients achieving independent ambulation with recovery; however, there is a significant proportion of patients (20%) with morbidity. (Evidence level III) Further studies of plasma exchange followed by IVIG and IVIG concurrent with steroids have not shown significant improvement. (Evidence level I)[49][50] An ongoing trial of 2 courses of IVIG should have results within the next year. (Evidence level III) [51] There are also ongoing trials of complement inhibitors in patients with refractory GBS. (Evidence level II)[52][53][54]

Nursing Management

Nursing management for identified nursing diagnoses includes:

1. Impaired respiratory function

The nurse will need to carefully monitor vital signs for changes in respiratory rate, quality of respirations, and decreasing vital capacity. Respiratory assessment for ascending paralysis and impending respiratory failure due to weakness of intercostal muscles and diaphragm along with shallow and irregular breathing, the use of accessory muscles, and difficulty in clearing secretions. If the patient is intubated, the nurse will need to work with the physician and respiratory therapist to manage all aspects of mechanical ventilation. During the course of GBS, the nurse will need to assess and monitor the patient for respiratory infections including pneumonia.

2. Immobility

The nurse will need to assess for problems associated with immobility related to muscle weakness and paralysis. The nurse should support and maintain paralyzed extremities in function positions, perform passive ROM exercises at least twice daily, ensure that the patient has position changes every two hours or ensure the patient is positioned on a foam, air, water, or gel support surface bed. Skin assessment for skin breakdown, assessment of bowel function through monitoring of bowel sounds and frequency of bowel movements, use of anti-embolism stockings and compression boots to prevent DVT and  PE, and maintenance of adequate hydration to decrease the risks of pressure ulcers are important nursing management considerations for the immobility related to GBS.

3. Nutritional imbalance

The nurse will need to carefully work to assist in the maintenance of optimal nutrition in the patient with GBS. The patient with impaired swallowing due to muscle weakness should be carefully assessed for aspiration. The nurse will need to manage gastrostomy tube feedings, IV fluid administration, or the administration of parenteral nutrition as needed to ensure that the patient receives necessary nutrients. 

4. Communication impairments

The nurse will need to provide strategies for adequate communication with the patient who is unable to verbally communicate due to paralysis associated with GBS. Management of adequate patient communication may include the use of strategies such as eye blinks, use of pictures, or the use of computer graphics. The nurse should also consider discussing the use of communication strategies with family members and friends. Referrals to a speech therapist may also assist to address communication impairments due to GBS.

5. Pain 

The nurse will need to carefully assess the patient for pain related to GBS related muscle changes. If the patient is unable to communicate verbally, the nurse should use assessment strategies including pictures or pain management scales to obtain an adequate understanding of the patient's pain level. Non-verbal signs of pain including restlessness, facial grimaces, or restlessness should also be noted in this assessment. Adequate pain management is essential, especially in the acute stages of GBS. 

6. Psychological problems

The nurse will need to carefully assess and manage the GBS patient for potential psychological problems. The sudden onset of loss of control in the acute phase of a potentially life-threatening illness may result in anxiety, fear, and feelings of helplessness. Patients and family members may also confront uncertainty, helplessness, and loneliness when dealing with GBS. The impact of GBS on family members may be influenced by the role of the patient in the family. Social and economic issues may be exacerbated if the patient is the primary breadwinner or major source of family support. Patients and family members may experience fear and helplessness, especially within the ICU or acute care setting. The nurse will need to educate the patient and family members, providing information about GBS and the equipment, medications, and therapies used to treat GBS. The nurse may also suggest patient education materials, referrals to support groups, social workers, or psychologists as strategies to improve patient and family coping with GBS. 

When To Seek Help

Management of GBS requires that the nurse participates in an interprofessional team dedicated to collaborative patient care delivery. The nurse should plan to assess and monitor the patient with GBS for potential complications. Changes in respiratory function with decreased vital capacity require the nurse to contact the MD. Complications including cardiac dysrhythmias, hypertension, hypotension, DVT, PE, urinary retention, or alterations in swallowing are findings that require the nurse to contact the MD. The nurse should contact the pharmacist for problems associated with IVIG or corticosteroids frequently used in the management of acute GBS. Monitoring of patients and determination of appropriate mechanical ventilation settings should occur with assistance from the MD and respiratory therapist. Expressions of fear, anxiety, hopelessness, and helplessness by the patient or family members should prompt the nurse to contact the social worker. psychologist, or other mental health providers in the interprofessional team. 

Outcome Identification

Expected patient outcomes include the following:

1. Normal respiratory function with normal respiratory rate, normal vital capacity, effective respiratory functioning and airway clearance, and O2 levels within normal limits

2. Increased mobility with no contractures or muscle weakness, ability to ambulate and regain function in all extremities

3. Ability to swallow, maintain diet that provides adequate nutrition and hydration

4. Maintain adequate verbal communication with recovery of speech

5. Pain is resolved or adequately controlled through oral pain medications or other pain management strategies such as exercise, relaxation exercises, and stress managment

6. Reduction or elimination of psychological problems including fear, anxiety, uncertaintly, helplessness, loss of control, and lonliness

7. Reduction or elimination of complications including decubitus ulcers, DVT, bowel or bladder dysfucntion

Monitoring

The nurse will need to carefully monitor the GBS patient for:

1. Respiratory impairment- monitoring includes assessment of vs, measurement of vital capacity, observation for use of accessory muscles and difficulty clearing secretions 

2. Cadiac dysrhythmias associated with autonomic dysfunction- monitoring includes measurement of blood pressure for hyper or hypotension, use of cardiac monitoring devices to measure heart rate and presence of dysrhythmias

3. Weakness and paralysis  in extremities- monitoring includes checking for the absence of lower extremity tendon reflexes and the patient's ability to walk,  and use extremities for lifting or movement

4. Changes in skin due to immobility- monitoring for erythema or early signs of skin breakdown and decubiti 

5. Fluid and electrolyte imbalance- monitoring of IVs, parenteral nutrition, and intake and output

6. Changes in bladder and bowel function due to autonomic dysfunction, immobility, or use of pain medications- monitoring of output through use of indwelling catheter and monitoring of bowel movement frequency

7. Pain- monitor by freequent patient assessment using numeric or picture pain scales

8. Psychological problems- monitor the patient for symptions of fear, anxiety, or depresion 

Coordination of Care

The care of the patient with Guillain-Barre syndrome (GBS) requires collaboration by all members of the healthcare team. Nurses are integral in coordinating care services in the acute phase of GBS and in recognizing and preventing GBS complications, including decubitus ulcers, and infection prevention. Pharmacists should be well-versed in the adverse effects that may occur with the administration of medications including IVIG as well as pain and cardiac medications. Respiratory therapists should assist in preventing problems such as aspiration pneumonia. Physical and occupational therapists are crucial in assisting with muscle strengthening exercises, gait training, ROM exercises, use of assistive devices such as walkers, canes, and wheelchairs as well as activities to improve functional status and activities of daily living (eating, grooming, bathing, etc). Psychological support should be coordinated with social workers, psychologists, or psychiatrists. GBS patients should have care coordinated in the acute phase of the illness by a hospital-based clinical care manager. Patients should be assessed for rehabilitation and care coordination with an assessment of rehabilitation potential by members of the rehabilitation team including MDs, physiatrists, speech therapists, social workers, and rehabilitation care managers and nurses.  Following GBS recovery, patients often find it helpful to enlist in support groups available through the GBS foundation. Education of the GBS patient and family members should be coordinated by the nurse throughout the course of the illness in collaboration with health educators. 

Health Teaching and Health Promotion

Health education is critical throughout the course of GBS. In the initial acute phase of GBS, the nurse should educate both the patient and family members about GBS including the symptoms of GBS, disease progression, medical management of the disease, current treatments for GBS including IVIG and plasmapheresis, risks associated with GBS, required monitoring of autonomic dysfunction including cardiac and respiratory monitoring, and morbidity and mortality associated with GBS. Patients admitted to the ICU and their family members will need to be educated about the equipment and monitoring routinely performed in this setting.  

Health education in the continuing or recovery phase of GBS should focus on rehabilitation efforts by the members of continuing care interprofessional teams. Team members educate both the patient and family members on rehabilitation expectations, and activities designed to return the patient to functional status. Education is focused on the preparation of the patient and family members to assume activities in acute rehabilitation, long-term care, outpatient or home settings with an emphasis on specific activities such as physical therapy, occupational therapy, speech therapy, and nutritional therapy. Education for family members caring for the recovering GBS patient should also include training in the use of adaptive devices such as canes, walkers, wheelchairs, bedside commodes, bathtub or shower benches, and safety measures such as ramps for easy home access. Health education during the recovery phase of GBS should also include information about health promotion and health maintenance including education about optimal nutrition, exercise, adequate sleep, and the importance of social interactions with friends, co-workers, and family members. Health education about routine health maintenance screenings such as mammograms and screening for colorectal cancer and skin cancer should be provided to both patients and family members. 

Risk Management

Specific nursing liability risks in the acute and recovery phases of GBS include medication errors, inadequate monitoring of respiratory function resulting in respiratory arrest, inadequate assessment of risks such as cardiac dysrhythmias resulting in cardiac arrest, inadequate reporting of fluid  and electrolyte imbalance,  inadequate positioning and  failure to perform ROM exercises resulting in contractures and development of decubiti, inadequate monitoring of IVs, and lines for parenteral nutrition, inadequate positioning and monitoring of feeding tube resulting in aspiration pneumonia, inadequate assessment and referrals for psychological problems including fear and anxiety resulting in psychological problems such as depression and inadequate monitoring of ambulation resulting in falls and possible fractures. 

Discharge Planning

Discharge planning for the GBS patient should begin at the point of hospital admission for the acute phase of GBS with an emphasis on providing continuing care for the patient in acute rehabilitation, long-term care, or home care settings. Discharge planning is coordinated by members of the interprofessional care team and specific discharge plans determined for each individual patient. The focus of discharge planning should be on how best to return the patient to complete recovery. As the GBS patient begins the recovery process from the acute phase of GBS, plans should focus on transitioning the patient from the ICU. This may require plans to wean the patient from mechanical ventilation, the introduction of food following use and removal of IVs, parenteral nutrition or feeding tube, the introduction of sitting and standing activities, and introduction of ambulation. Discharge planning should also include specific recommendations from individual team members. For example, the physical therapist should recommend specific plans to promote muscle strength in extremities and assistance in ambulation. The occupational therapist should recommend specific plans to promote activities of daily living including feeding, bathing, and dressing. The nurse should plan to provide care coordination and health education for patients and family members to ensure that the patient makes as smooth a transition from acute illness to recovery as possible.  

Evidence-Based Issues

Evidence-based issues are related to:

1. limitations of evidence to identify and describe possible causal relationships of GBS to infections and immunizations  [55]

2. lack of definitive evidence in support of either IVIG or plasmapheresis as effective treatments for GBS [56][57][58][59]

Pearls and Other issues

The nurse serves an important and sometimes overlooked role in the management of the patient with GBS. It is also important to note the relevance of an interprofessional team in care delivery to manage the complex issues connected to GBS.