2004 OPEN FORUM Abstracts
GUILLAIN-BARRE’SYNDROME AND PULMONARY INSUFFICIENCY IN THE YOUNGER PATIENT:
W. Kingrey, RRT; Robert H. Warren, MD -- Arkansas Children's
Hospital, Little Rock, AR
Introduction: Guillain-Barre’ Syndrome (GBS) is an acute, demyelinating polyneuropathy often triggered by a preceding bacterial or viral infection. GBS is characterized by rapidly progressive ascending symmetrical limb weakness and loss of tendon reflexes; often resulting in paralysis of the legs, arms, respiratory muscles, and face. Because of the potential for respiratory muscle involvement, pulmonary function is carefully monitored, especially the forced vital capacity (FVC). Intubation and mechanical ventilation may be required due to declining FVC measurements or cardiovascular instability. We report the case of a toddler in whom initial presentation was atypical and challenges were faced in respiratory status monitoring.
Case Summary: A 2 y/o female was admitted with a 6-day history of irritability with intermittent screaming, refusal to sleep in her usual position of lying on her back and instead preferring to sleep prone with her buttocks in the air, and progressive difficulty in ambulation beginning with arching of the back and walking on tiptoes to refusing to walk altogether. Physical exam also revealed sacral pain with leg stiffness and unwillingness to flex her legs or move her chin to her chest. Chest auscultation was clear. History included up-to-date immunizations and mother’s recent recovery from gastroenteritis. Over the next 5 days, diagnostic testing revealed negative TB skin test and toxicology screens. Bone marrow biopsy, CT, and MRI scans were normal. Following repeat lumbar punctures with continued high protein levels of 246 and abnormal findings from a nerve conduction study, a diagnosis of GBS was made. Intravenous IgG (IVIG) was introduced. A respiratory care consult was ordered to evaluate her respiratory status. Due to the patient’s age and inability to cooperate, attempts to perform bedside pulmonary function studies failed. Over the next 24 hours, she developed tachycardia, tachypnea, O2 desaturations to 92%, left eye pstosis, and drooling. With ABG analysis on room air revealing a pH 7.38, PaCO2 50, PaO2 79, HCO3 29, BE +2.9, the patient was transferred to the ICU for closer monitoring. Although BiPAP was considered, it was not necessary as respiratory deterioration was avoided through bag-valve-mask hyperinflation therapy of 10 breaths given with a peak inspiratory pressure of 25 cmH2O and pressure hold of 5-10 seconds. This therapy was continued until discharge. After completion of IVIG, the patient steadily improved and was discharged home after a 17-day hospital stay.
Discussion: Although GBS is readily diagnosed in adults who present with typical features, diagnosis in young children can be delayed when presenting neurological symptoms are generalized. The assessment of ventilatory reserve through objective pulmonary function measurements is often not possible in a young child who cannot perform the FVC maneuver. The respiratory therapist, therefore, must depend on serial observations, primarily of physical parameters, that indicate pulmonary dysfunction. The determination that progressive pulmonary dysfunction is occurring will allow for early intervention with respiratory therapy modalities, with the great potential to prevent frank respiratory failure and associated acute pulmonary events. This case of GBS in a young child is a clear example of the need for careful physical assessment of pulmonary function over time to determine the potential for respiratory insufficiency and failure.