O5 Treatment of infantile botulism with botulism immune globulin (BabyBIG)

Background A 7 month old male infant was admitted to their local hospital with poor feeding, reduced urine output, cough and respiratory distress. Worsening respiratory distress and apnoea required ventilation and transfer to a tertiary paediatric intensive care unit, with a presumed diagnosis of respiratory sepsis. Following 1 day of intensive care, intravenous sedation was discontinued with a view to extubation. After 48 hours sedation hold, the patient still had no spontaneous movements and unreactive pupils. Following further review, stool samples were sent to the Health Protection Agency (HPA) for botulism testing. Although initial tests (culture) were negative, a mouse bioassay test was subsequently reported as positive for botulism toxin. HPA and the California Public Health Department (CDPH) confirmed that treatment with botulism immune globulin (BabyBIG) was indicated. How does BabyBIG work: BabyBIG consists of human-derived anti-botulism-toxin antibodies and is approved in the U.S. for treatment of infant botulism types A and B. BabyBIG immediately neutralises circulating neurotoxin and allows motor nerve regeneration to begin. Complete recovery can take several months. 1 2 Supply of BabyBIG: Diagnosis of botulism was made out of hours at a weekend, requiring BabyBIG to be obtained directly from CDPH. Pharmacy contacted the Medicines and Healthcare Regulatory Authority (MHRA) on-call service to authorise UK importation of Baby BIG. Pharmacy worked closely with the clinical team and MHRA to ensure that relevant paperwork required by CDPH was completed. Cost of Baby BIG is $43,000 USD and required the Trust Medical Director to authorise funding. CDPH authorised release of Baby BIG, which was received in the Trust 48 hours later. Administration of BabyBIG: Pharmacy prepared an IV monograph document to assist preparation and administration of BabyBIG, which is presented as 100 mg vials for reconstitution with 2 ml water for injection. BabyBIG is administered as a single dose of 50 mg/kg, infused at an initial rate of 25 mg/kg/hour for 15 minutes which, if tolerated, is increased to 50 mg/kg/hour for the remainder of the infusion. The infusion is administered via an 18 µm filter. Patient outcome Within 24 hours of administration, there was some movement in the patient’s upper limbs and some triggering of the ventilator. By day 12 post-administration pupils were more reactive and there were some antigravity movements. By Day 15 there were signs of facial movement and improved grip strength. A tracheostomy was performed to facilitate weaning form the ventilator. By Day 67 the infant was off the ventilator and on Day 93 was discharged to their local hospital. Summary Pharmacy played a significant role, ensuring correct processes were followed for BabyBIG to be ordered out of hours, liaising with international partners, organising international transit, ensuring import documentation was available, providing important drug information to ensure the drug was prescribed and administered correctly, and answering parent’s questions about the medication. In this case, BabyBIG provided a highly effective treatment for infant botulism. References Payne JR, Khouri JM, Jewell NP, et al. Efficacy of Human Botulism Immune Globulin for the Treatment of Infant Botulism: The First 12 Years Post License. J Pediatr 2018;193:172–177. Ramroop S, Williams B. Infant botulism and botulism immune globulin in the UK: a case series of four infants. Arch Dis Child 2012;97:459–460