Approach Considerations
To date, treatment for Guillain-Barré syndrome (GBS) has been aimed primarily at immunomodulation. [38]
In pediatrics, the most effective form of therapy is intravenous immunoglobulin (IVIG). Each batch of IVIG is made of human plasma derived from pools of 3,000–10,000 donors. Plasmapheresis may also be used.
Corticosteroids were previously used to treat GBS, but current data indicate they provide little benefit.
Go to Guillain-Barre Syndrome and Emergent Management of Guillain-Barre Syndrome for complete information on these topics.
Intravenous Immune Globulin
IVIG has been shown to be safe and effective in the treatment of pediatric Guillain-Barré syndrome (GBS). [39, 40] Although only one prospective, randomized treatment trial in childhood GBS has been published, [41] multiple studies have shown that IVIG seems helpful in reducing the severity of the disease as well as the duration of symptoms. However, the long-term outcome may not be affected.
Several regimens have been used. The optimal dose and dosage schedules for IVIG have not been rigorously determined in childhood GBS. One possible regimen includes daily administration of IVIG for 5 days at a dose of 0.4 g/kg/d, which can lead to improvements 2-3 days after the start of therapy. IVIG can be given by way of a peripheral intravenous route.
Some authors use 2 g/kg of IVIG given as a single dose or 1 g/kg/d over 2 days in children who are showing rapid signs of deterioration. Although, in a small, randomized trial, the outcomes between the 2 treatment regimens were equivalent, treatment-related fluctuation (deterioration after receiving IVIG) occurred more often in children who received the 2-day course of IVIG. [41]
Plasmapheresis
Studies in children using both historical and case controls indicate that plasmapheresis may decrease the severity and shorten the duration of Guillain-Barré syndrome (GBS). Between 4 and 5 plasmapheresis treatments may be performed over 7–10 days, as described in standard protocols. Potential complications include autonomic instability, hypercalcemia, and bleeding due to depletion of clotting factors.
Results of plasmapheresis and IVIG are similar, with possibly fewer side effects seen with IVIG. It stands to reason that plasmapheresis should not typically follow IVIG administration.
The availability of plasmapheresis is generally limited to major referral centers that have the requisite equipment and trained personnel. Central line vascular access dictates intensive care hospitalization. In addition, plasmapheresis is limited to larger children; in most institutions, children weighing less than 10–15 kg may not be considered for volume exchange therapy. These features distinguish plasmapheresis from IVIG, which can be given to smaller children and can be administered via peripheral IV in specialized ambulatory clinic settings, advanced home nursing programs, and at ward level hospital settings.
Exchange Transfusion
When high cost limits the use of IVIG or unavailability limits the use of plasmapheresis, exchange transfusion can be used as an alternative therapy for severe Guillain-Barré syndrome disease in children.
Complications of Monitoring and Treatment
Careful attention should be paid to multiple issues that may require intervention and specialist consultation. Temperature, blood pressure, heart rate, respiratory capacity, and urine output of the patient should be monitored. In pediatric patients, monitoring for dysautonomia is paramount.
Among the concerns of Guillain-Barré syndrome (GBS) comorbidities are cardiorespiratory function, nutrition, urinary retention, decubitus ulcers, constipation, gastritis, dysesthesias/pain, mood and anxiety issues, iatrogenic infectious complications, and contractures in patients who are severely ill or who have a particularly prolonged course.
During the acute phase of the illness, orthostatic hypotension and urinary retention also may cause significant problems. In addition to the weakness, autonomic symptoms (eg, orthostatic hypotension) may also restrict activity and should be monitored.
Respiratory status and signs of dysautonomia
During the acute phase of the disease, close attention should be paid to respiratory status and signs of dysautonomia. Intubation and mechanical ventilation should be considered when vital capacity falls below 15 mL/kg body weight or arterial pressure of oxygen falls below 70 mm Hg (or the patient has significant fatigue).
In cooperative children older than 5 years, respiratory function measurements, such as vital capacity or maximal inspiratory force (MIF), can be valuable. MIFs are also known as negative inspiratory force (NIF). MIFs are normally greater than -40 mL water pressure; thus, the more negative, the better MIF. MIFs less than -20 mL water pressure can be an indication of poor inspiratory ability and respiratory distress.
MIFs provide objective data to follow and compare. This measure is unfortunately difficult to monitor in young children (< 5 y) and in uncooperative children. Experienced pediatric respiratory therapists can be very valuable in these measures.
Experienced pulmonary care is vital if neuromuscular weakness is affecting pulmonary function. Possible interventions include continuous positive airway pressure (CPAP), bilateral positive airway pressure (BiPAP), mechanical ventilation, or cough-assist devices.
Blood gases are not helpful in assessing neuromuscular respiratory failure, as they do not become abnormal until there is no longer any respiratory reserve. Other means of assessing respiratory function, such as respiratory rate and dyspnea on lying supine, are far more valuable early indictors of respiratory dysfunction.
Chest radiographs can be obtained to look for signs of infection. Cardiac monitoring is essential to detect any signs of cardiovascular instability and treat any arrhythmia.
Consultations
Consultation with a pediatric neurologist should be considered to confirm the diagnosis of Guillain-Barré syndrome. Intensivists may need to be involved quickly if critical care (cardiorespiratory) issues are suspected.
Once the patient’s condition has stabilized, patients should benefit from consultation with a rehabilitation medicine specialist, especially if it appears that recovery will be prolonged. Physical therapy, occupational therapy, and orthotics are also helpful.
Advances in Management
Currently, management of Guillain-Barré syndrome (GBS) is not dependent on preceding infection. Some preceding infections are related to specific clinical variants or subtypes. These could be important factors in management, prediction of clinical course, and treatment in the future.
There have been trials to evaluate safety and efficacy of eculizumab-complement 5 factor inhibitor in adults, but none that studied effects in children.
In a Japanese study of eligible GBS patients who were unable to walk independently, patients were assigned to receive 4 weeks IVIG plus either eculizumab 900 mg or placebo. At 4 weeks, 61% of patients in the eculizumab group were able to walk compared with 45% in the placebo group. Two patients in the eculizumab group experienced adverse effects (intracranial hemorrhage and abscess).
This was a very small study without any statistical comparison with illicit drug growth. The efficacy and safety of eculizumab should continue to be investigated in larger randomized controlled trials. [42]
