Vol. 3 Issue 23
The goal of this CE offering is to provide nurses with information about Guillain-Barré Syndrome – the disease process, treatment, nursing care and recovery – that can be applied to practice. After you have completed reading this article, you will be able to:
1. Describe major signs and symptoms of Guillain-Barré Syndrome.
2. Discuss treatment modalities related to Guillain-Barré Syndrome.
3. Discuss the nursing implications.
During the late 1970s, several hundred cases of severe muscle weakness, especially noted as tingling in the upper extremities, began to appear in clinics, hospitals and emergency departments. Oddly enough, this influx of patients occurred during the flu season. These patients were later diagnosed with Guillain-Barré Syndrome (GBS).
Guillain-BarrŽ is not a disease, but instead a syndrome. This means that there are no definite or identifiable causes for the sensory and motor problems it produces. Rather, they are generalized symptoms that usually follow a known viral illness. The syndrome is potentially life-threatening and requires immediate hospitalization. A main concern is to monitor the patient's vital capacity and swallowing capability.
This syndrome was given its name by two French neurologists, Georges Guillain and J.A. BarrŽ, who originally described it in the medical literature in 1895. Other names are Landry-Guillain-Barré Syndrome, acute idiopathic ("patchy") polyneuritis, infectious poly.neuritis, acute inflammatory polyneuropathy, or acquired immunomediated neuropathy.1
Idiopathic polyneuropathies can be divided into either acute or chronic conditions. It should be noted that there are many types of polyneuropathies. Unlike other polyneuropathies such as chronic idiopathic demyelinating polyneuropathy, GBS is acute and does not need long-term therapy to maintain improvement. This syndrome is thought to be preceded by a history of a non-specific febrile illness. The individual responds with a hypersensitivity to the particular virus causing the illness. These infections seem to trigger an incorrect response of the immune system.
GBS is a rare and unpredictable syndrome with no known etiology. It affects the spinal nerves and cranial nerves. GBS is a disorder that presents with rapidly developing ascending weakness. The distal parasthesias and loss of deep tendon reflexes can progress to the point of complete paralysis within hours. Absence of tendon reflexes almost always occurs and is the most important clue to diagnosis of GBS. A common reflex lost is the knee jerk.
The attack is directed against myelin, the insulation component of nerves. Generally, the axons of nerves are not attacked, but will worsen the situation if they are concomitantly harmed. The onset has been reported to occur after weeks of a nonspecific febrile illness such as a respiratory infection, intestinal infection, viral infection such as measles or mumps, or after receiving an immunization.
Approximately 1-2 out of every 100,000 people become diagnosed with GBS, with about 3,500 cases reported a year between the United States and Canada. This disorder does not discriminate between the old and young, or male and female, and is neither hereditary nor contagious. About 5-19 percent of patients contracted the syndrome following surgical procedures.1
The destruction of myelin begins with the nerves at the distal part of the spinal cord. All cranial nerves are generally involved except for cranial nerves I and II (olfactory and optic). The most common cranial nerve affected is the facial nerve (VII).
Physiologic effects of the disease include inflammation, demyelination of peripheral nerves, loss of granular bodies and degeneration of the basement membrane of the Schwann cell. The myelin is made up of Schwann cells and these cells will rejuvenate, but may take from several months to a year to completely recover. Damage results when the body's attempt to defend itself produces anti.bodies that strip away the myelin that covers and protects the nerves. The antibodies produced by the body to fight the virus have taken on a new attack of not only the virus, but also of the myelin sheath. Due to molecular mimicry of the myelin and virus components, the body tends to react against itself as a cross reaction to the infecting organism.
In this autoimmune response syndrome the axon of the nerve is not damaged but, with the sheath damaged, the nerve fibers cannot conduct electrical impulses as quickly. The amount of myelin damage determines the loss of function and/or sensation/paralysis. During this process it is unpredictable whether the condition will become mild or severe.2
Progression of paralysis may stop at any point. Once the weakness reaches its maximum, the paralysis remains unchanged for a period of time (ranging from days to weeks). Intercostal paralysis leads to a loss of functional breathing and causes diaphragmatic breathing. Paralysis can occur in fibers of the vagus nerve and the sympathetic fibers of the nervous system. As a result of this loss, the patient loses the ability for bronchodilation and bronchoconstriction, and the ability to protectively respond to bronchial irritation. Mechanical ventilation may need to be considered.
SIGNS & SYMPTOMS
Early symptoms may be mistaken for the flu. Flaccid paralysis can sometimes be mistaken for poliomyelitis. The illness can present in several ways, at times making the diagnosis difficult to establish in its early stages. Significant symptoms for GBS in.clude fever, malaise, nausea and muscular weakness. The weakness usually starts in the lower extremities – the classic symptom being tingling in the feet – and tends to ascend upward through the body. The paralysis is not accompanied by loss of sensation, but rather by abnormal sensations such as numb.ness or tingling. As the weakness develops, it typically travels up the body in a symmetrical fashion. Spontaneous improvement con.tinues for weeks or longer. The prognosis for a full recovery is good.
Symptoms may manifest as acute or chronic. The extent (temporary or permanent) and rate of recovery depend upon the amount of neuron destruction. Clinically, the weakness of muscle tissue continues for 1-2 weeks and remains stable for a period of time, then gradually improves. If nerve injury is limited to demyelination, recovery is usually rapid. However, if damage has occurred to the nerve axons, some weakness may continue. Weak.ness does not ascend in all cases.
There is an absence of a reference diagnostic test that would allow a positive confirmation of the diagnosis. A prominent feature presented in GBS is the absence of tendon reflexes; therefore, this can be used as an important diagnostic clue. A lumbar puncture may show an increase in protein, which is thought to be due to inflammation of de.generative changes of the spinal nerve roots. A significant lab finding is an increased cere.brospinal fluid protein without an increased (white) cell count. Blood tests do not specifically assist in the diagnosis of GBS, but may help rule out other disorders. Sensory changes may or may not be present, and are not used as a significant factor in considering GBS. The condition usually begins to slowly im.prove after a peak at about 2-3 weeks.
TREATMENT OF GBS
There is no specific treatment for GBS. It must run its course. Treatment must be based on the presented symptoms. Treatments for GBS include:
• Good nursing care;
Even though there is no known cure, certain treatments may lessen the severity of the illness. Many patients require intensive care. The nurse must be astute in her skills of ongoing assessment. Physical therapy is needed to lessen the risk of contractures, hypotension from flaccid muscles or deep vein thrombosis.
Plasmapheresis may speed the recovery if done within 2 weeks of diagnosis. The pro.cess of plasmapheresis works like this: fresh plasma is given to the patient in an effort to dilute the insulting antibodies and give the nerve fibers a rest from "attack," allowing them to begin to regenerate. The patient is typed and screened for the plasma and the nurse would follow basic guidelines for giving plasma and watching for a possible trans.fusion reaction as with any other blood products.
Intravenous gamma globulins are proteins given to attack circulating antibodies. Even though this treatment is expensive and time-.consuming, IV immunoglobulins given in high doses is the established maintenance treatment for GBS.
Emotional problems also need to be ad.dressed. This is why a specialized nursing facility is utilized rather than a typical nursing home. The beginning stages of this syndrome can be very frightening. Most pa.tients with GBS were typically healthy. To find themselves paralyzed and helpless, being constantly monitored by machines and tubes, can be emotionally overwhelming. Both victims and families often greatly benefit from support groups. It is helpful for both patient and family to know that most GBS patients get better. Most patients eventually walk and many can ultimately resume a normal life.
If the case is mild, the patient may present with general weakness and in some cases not ever know he had it. The disorder may have been considered virus related.
A moderate case will impair ability to walk. Daily functions are difficult or impossible to perform; it is serious enough that medical attention is sought.
A severe case constitutes a medical emergency and requires hospitalization. A pa.tient with this level of GBS may be partially or totally paralyzed, with compromised heart rate, blood pressure and breathing. Most of the cases will require a ventilator for the pa.tient. Muscle weakness of the pharynx and larynx may lead to difficulty swallowing and impaired or absent gag or cough reflex. Over.all, most patients who experience minimal deficits (or none) have a recovery rate of greater than 75-90 percent.
Functional return is variable and usually progresses in a reverse pattern, with respiratory improvement occurring first and lower extremities improvement last. A guideline to neural repair is that they regenerate at an inch a month. Rehabilitation is a major form of treatment. As muscle strength returns, efforts are directed at returning the patient to his former lifestyle.
IMPLICATIONS FOR NURSING
Since the severity of Guillain-Barré Syndrome is so variable, it is difficult to de.scribe an average patient; however, the main nursing goals are to provide optimal supportive care and to anticipate and prevent complications.
Skilled nursing care is essential. The po.tential for rapid deterioration is so great in patients with a presumed GBS that hospitalization is usually required for observation. Early care is often provided in an intensive care unit so that potential complications can be treated quickly should they occur.
• Monitor for signs of respiratory distress;
• Assess for extent of involve.ment/paral..ysis;
• Assess for swallowing difficulties;
• Monitor fluids and electrolytes as needed;
• Evaluate precautions to prevent compli.cations; and
• Provide physical/psychological support as needed.
Intensive care observation includes early initiation and insertion of an airway should the patient's ventilatory strength decline. An emergency tracheostomy kit should be placed at the bedside of a patient with potential respiratory complications due to paralysis ascending to the abdominal and thoracic muscles. The level of sensory and motor function and/or deficit needs to be monitored on an ongoing basis to determine possible progression of the syndrome.
If swallowing is difficult, nutrition may not be optimal for the patient and alternative-feeding protocols must be established. The nursing considerations for the GBS pa.tient would be no different from any other patient with swallowing difficulties. A baseline of fluids and electrolytes needs to be established to help with nutritional management. If the patient is eating and swallowing, but intake is low, a calorie count may be started and then supplements given based on the caloric results. If aspiration is taking place, the patient may need to be taught a special swallowing technique or may need to be placed npo. The nutritional status of the npo patient would need to be addressed appropriately with IV fluids or a feeding tube.
The nurse needs to continue to monitor the patient, looking for potential complications such as pneumonia or congestion, ab.normal heartbeat or blood pressure, infections, blood clots or other life-threatening problems. Passive range of motion 3-4 times per day is most advantageous for the immobilized patient. The nurse should continue to give support (with the purpose to reduce anxiety and depression) as well as education through all stages of the recovery depending on the needs of the patient and the pa.tient's family.
A nursing strategy would be to facilitate control of the situation, reduce and alleviate possible complications by utilizing an individual plan of care. Some nursing goals for patients with GBS would be to assist the patient to obtain the highest level of functioning within given limitations and modified lifestyle. Educate the patient/family through understanding of the syndrome and to man.age daily living consistent with abilities spared by the GBS debilitating process.
GBS continues to be a rare and unpredic.table syndrome that causes much chaos and turmoil in a person's life. To date, no specific test can diagnostically identify GBS. It is important that nurses be sharp with patient assessments following a viral invasion. A de.tection of weakness or tingling in the lower extremities in the very early stages could bring forth treatments necessary to lessen the severity of the syndrome and perhaps de.crease the possibility of complications. Ac.curate diagnosis, therapy, emotion.al support and education are needed for both the physio- and psychopathology of GBS. Together these will promote the highest functional capacity and improve the patient's quality of life with regard to their overall life situation. n
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2. Guillain-Barré Syndrome Support Group of the United Kingdom. The Guillain-Barré Syndrome (GBS) É A quick guide. Retrieved Dec. 22, 2000 from the World Wide Web: http://glaxocentre.merseyside.org/gbs.html
3. Green, D., & Ropper, A. (2001). Mild Guillain-Barré Syndrome. Archives of Neurology, 58(7), 1098.
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Ensrud, E., & Krivickas, L. (2001). Acquired inflammatory demyelinating neuropathis. In Carter, G. (Ed.), Physical Medicine and Rehabilitation Clinics of North America, 12(2), 321-334.
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Miyazaki, T., et al. (2001). Guillain-Barré Syndrome associated with IgG monospecific to ganglioside GD1b. Neurology, 56(9), 1227.
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Pam Hentschke is clinical nurse specialist/nurse educator with Charlotte (NC) Institute of Rehabilitation.