Guillain-Barre – Treatment
There is no known cure for Guillain-Barre syndrome (GB). However, there are treatments and therapies that lessen the severity and long term effects of this illness. A multidisciplinary approach that includes treatment for physical and psychological distress must be formulated to achieve the best outcomes. The goal of this treatment is to prevent complications and accelerate the patient’s recovery.
Emergency Treatment
Patients presenting with symptoms of GB require careful attentions to the basic ABCs of care. One third of GB patients will require intubation, so attention to airway and breathing is paramount. Inability to handle secretions and poor oxygenation will require early intubation. Proper positioning of the patient to optimize lung expansion and secretions are required to minimize respiratory complications.
Cardiac and hemodynamic monitoring should be initiated and monitored closely for changes in blood pressure, heart rate, and arrhythmias. Patients exhibiting bradyarrythmia usually respond to Atropine but temporary pacing may be required. Frequent liability in blood pressure is seen in these patients. Due to the impairment in autonomic function, patients with hypertension should be treated with short acting beta blockers or nitroprusside. Hypotension usually responds to fluid bolus.
Pharmacological Treatments
Current treatments include high-dose immunoglobulin therapy (IGg), and plasmapheresis (PE). Both of these treatments are equally effective. Mixing them or administering one after the other is thought to be no more effective than using either method alone.
Immunoglobulin therapy is easier to administer and may be the only therapy needed in milder cases. Immune globulin products from human plasma were first used in 1952 to treat immune deficiency. Intravenous immunoglobulin (IVIG) contains the pooled IGg from the plasma of approximately a thousand or more blood donors. It is thought to lessen the immune attack on the nervous system by blocking the damaging antibodies thought to cause GB. IVIG started within two weeks from onset hastens recovery as much as PE. Adverse events were not significantly frequent with either treatment but IVIG is significantly more likely to be completed and easier to administer than PE.
Dosage of IVIG is 400mg/kg daily for five days. Some studies have looked at the benefit of repeating this dose if significant improvement is not seen or if the patient deteriorates, but evidence does not support repeat treatment at this date. Steroid hormones have also been used to reduce the severity of Guillain-Barre, but controlled clinical trials have demonstrated that this treatment is ineffective and may even have a deleterious effect on the patient and cause further complications.
Plasma exchange (plasmapheresis) is a method by which whole blood is removed from the body via central line and processed so that the red and white blood cells are separated from the plasma. The blood cells and platelets are then returned to the patient without the plasma, which the body quickly replaces. Researchers still are unsure exactly why plasma exchange works, but the technique seems to reduce the severity and duration of the Guillain-Barre episode. One hypothesis is that plasmapheresis can remove antibodies and other immune cell-derived factors that could contribute to nerve damage.
Treatment with PE is found to be most effective within two to four weeks after the onset of weakness. It is recommended to have five plasma filtrations over the course of 5-7 days depending on the patient’s tolerance. Frequently albumin or donor plasma is reinfused with the blood cells and platelets. Though tolerated well by most patients, they should be observed for signs of hypotension, arrhythmia, hypocalcemia, hypomagnesium, and transfusion reaction. Premedication with acetaminophen, diphenhydramine, and hydrocortisone are often given if the patient is to receive any blood product. Having all the equipment and medications required for the procedure readily available before treatment begins can help minimize complications. Sterile technique is advised in order to reduce the likelihood of infection. PE is contraindicated in patient who are hemodynamically unstable, septic or hypocalcemia.
Pain treatments
Pain is a common and sometimes severe symptom in patients with GB. Pain has been described in up to 89% of patients with GB. Recognition and intervention to ease pain is important, especially in patients unable to communicate due to intubation. Recognition of the presence and type of pain is important because specific alternative treatments or therapies can be offered. For mild to moderate discomfort the patient is usually started with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen. Narcotic agents and opioids are needed in 75% of patients due to irritated nerve roots. Narcotics should be used sparingly due to the high potential for respiratory compromise and ileus. Most patients do not require narcotic analgesics after the first couple of months of illness.
Up to 10% of patients have persistent extremity pain requiring more than analgesics even after the return of motor functions. Two medications, gabapentin and carbamazepine, have been used for reducing nerve pain associated with GB. Both drugs are categorized as anticonvulsive and work by decreasing nerve impulses. Carbamazepine (Tegratol) is given 300mg daily while nerve pain persists, it should not be abruptly stopped unless the patient has a severe allergic reaction or develops Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). These allergic reactions may cause severe damage to the skin and internal organs. Gabapentin (Horizant) is given in increasing dosage up too 1800mg daily. Titration is also required when withdrawing from this drug.
Nonpharmacological pain relief therapies include frequent passive limb movements, gentle massage, and frequent position changes. Desensitization techniques can be used to improve the patient’s tolerance for activities. Addressing upright tolerance and endurance also may be a significant issue during the early part of rehabilitation. Active muscle strengthening can then be introduced and may include isometric, isotonic, isokinetic, or progressive resistive exercises. Mobility skills, such as bed mobility, transfers, and ambulation, are targeted functions. Patients should be monitored for hemodynamic instability and cardiac arrhythmias, especially upon initiation of the rehabilitation program. The intensity of the exercise program also should be monitored, because overworking the muscles may, paradoxically, lead to increased weakness. Modalities such as transcutaneous electrical nerve stimulation (TENS) and heat may prove beneficial in the management of myalgia.
Supportive treatments
Serious consideration should be taken to prevent secondary complications. Non-ambulatory patients should be assessed frequently for skin integrity. Nursing care to prevent decubiti, with frequent position changes, use of pneumonic bed systems, and supportive stockings is necessary. Subcutaneous unfractionated or low molecular weight heparin is recommended for non-ambulatory adult patients until they become able to walk independently. Subcutaneous heparin (5000 U, 12-hourly) to prevent deep vein thrombosis and pulmonary emboli are usually recommended.
Impaired elimination should be evaluated with bowel auscultation and monitoring of opioid administration. In addition to suspension of nasogastric nutritional feedings, erythromycin or neostigmine may be effective in treating ileus. Promotility agents are contraindicated in patients with GB. Bladder catheterization is often needed as part of the monitoring of severely affected patients. Although bowel and bladder dysfunction is generally transitory, management of these functions is needed to prevent other complications.
Increased nutritional demands should be met by enteral or parenteral feedings to ensure that adequate caloric needs are met when the metabolic demand is high. Even patients with mild GBS may require nutritional support. The degree of dysphagia should be evaluated and precautions against aspiration and subsequent pneumonias.
Impaired communication and ineffective coping of hospitalized patients with GB are frequent. Patient who are intubated or with sleep abnormalities may experience mental status changes, hallucinations, delusions, and vivid dreams. These occurrences are thought to be associated with autonomic dysfunction and are more frequent in patients with severe symptoms. Such problems resolve as the patient recovers. Psychiatric and psychological issues such as depression and anxiety are likely to occur. Education, counseling, and medications are necessary to recover and improve from their disability.
Despite immunotherapy and supportive care, 4% to 15% of patients with GB die from this syndrome and nearly 20% have a persistent disability. Death from GB occurs mostly in mechanically ventilated patients with the most severe form of the syndrome. A multidisciplinary care team remains the mainstay of treatment.
References:
Stieglitz, Elliot. (2018). Plasmapherisis Technique. Retrieved from http://emedicine.medscape.com/article/1895577-technique#c5.
Hughes, Richard AC et al. (2014). Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database of Systematic Reviews. Retrieved from http://cochranelibrary-wiley.com/doi/10.1002/14651858.CD002063.pub6/full.
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