Treatment should not be delayed for test results because the process can take several days to complete.6 If there is no spontaneous bowel movement, then gentle digital collection from the rectal vault can be performed. If an enema is necessary, use only nonbacteriostatic water. Glycerin suppositories should not be used because they will affect the quality of the specimen.

The specimen should be placed in a sterile urine container without preservatives or fixatives and refrigerated. The local Department of Public Health should be contacted for directions on where the specimen should be sent for testing.6

Treatment Options

Early treatment has been shown to dramatically decrease length of hospital stay by up to 3 weeks, length of mechanical ventilation by 2.6 weeks, and mean duration of IV feeding by 6.4 weeks.7

The current US Food and Drug Administration-approved treatment for infant botulism is an IV human-derived botulism immune globulin (BabyBIG®). The orphan drug consists of human-derived antibotulism toxin antibodies for treatment of both types A and B infant botulism in patients younger than 1 year of age. BabyBIG is available only from the California Department of Public Health Infant Botulism Treatment and Prevention Program (IBTPP). It can be obtained by contacting the IBTPP on-call physician at 510-231-7600; the current fee for a treatment is $57,300. This is a flat fee, regardless of the number of vials shipped to treat the patient.8


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Supplied in 100-mg vials, BabyBIG should be reconstituted with 2 mL of sterile water and administered intravenously (50 mg/kg). According to IBTPP, administration of the infusion should be initiated within 2 hours and completed within 4 hours of constitution. The patient should be monitored continually throughout administration for adverse reactions. The most common reaction is a generalized skin rash, which occurs in approximately 5% of patients. Less commonly noted reactions are fever, chills, muscle cramps, and nausea and vomiting. These reactions occur in less than 5% of patients, according to IBTPP.8

Result of Case

EF was evaluated by neurology shortly after admission to the pediatric floor and was noted to have marked hypotonia, poor suck, and a weak cry; her gag reflex was found to be intact. A nasogastric tube was placed and tube feeding initiated. The admitting pediatrician, neurologist, and infectious disease clinicians all concurred with the likely diagnosis of infant botulism. EF’s stool was collected with the assistance of an enema and forwarded to the Centers for Disease Control and Prevention for testing. Arrangements for obtaining BabyBIG were initiated.

The patient received her infusion of BabyBIG on hospital day 2. Over the course of the ensuing hospital days, gradual improvement in head lag and hypotonia were noted. EF was evaluated by speech therapy, physical therapy, and occupational therapy on hospital day 5, and the decision was made to restart oral feedings. EF did well and was tolerating oral intake with only occasional episodes of mild cough or choking noted.

By hospital day 9, EF was noted to have only a rare episode of “cough” with feeding, and both speech therapy and EF’s parents felt she was almost back to her normal feeding status. Neurologic examination on day 9 was unremarkable, with no noted head lag or hypotonia and a good suck and gag reflex. The decision was made to discharge EF, with a plan to follow up with her PCP in a week and with the infectious disease specialist in a month. Continued care by speech therapy, occupational therapy, and physical therapy also was recommended.

EF was seen by her PCP 6 days after discharge. Her neurologic examination at this visit was normal. Episodes of mild coughing or choking during feeding were noted to have resolved almost completely. The result of EF’s stool culture, which was returned shortly after her discharge, was negative for C botulinum. Of note, Khouri et al found that up to 25% of cases tested negative but were considered to be botulism because the presentation, timing, and recovery process were consistent with that seen in infants with positive stool tests for C botulinum who received BabyBIG.3 The route of exposure to C botulinum was believed to be related to excavation and landscaping around EF’s home.

Conclusion

While rare, infant botulism should always remain on the provider’s radar screen when dealing with an infant with a history of constipation and feeding issues. A complete neurologic exam should be completed, and if any question of deficits is noted, the patient should be sent emergently for further workup and evaluation.

Mark Kraljevich, RN, MPA, MSN, FNPC, is a family nurse practitioner currently working in a primary care practice. He has been a nurse for more than 30 years working in critical care and nursing management.

References

1. Waseem M. Pediatric botulism. Medscape. Accessed December 12, 2020. https://emedicine.medscape.com/article/961833-overview

2. Schwartz KL, Austin JW, Science M. Constipation and poor feeding in an infant with botulism. CMAJ. 2012;184(17):1919-1922.

3. Khouri JM, Payne JR, Arnon SS. More clinical mimics of infant botulism. J Pediatr. 2018;193:178-182.

4. Cox N, Hinkle R. Infant botulism. Am Fam Physician. 2002;65(7):1388-1392.

5. Arnon SS. Infant botulism. In: Cherry JD, Harrison GJ, Kaplan SL, Hotez PJ, Steinback WJ, eds. Feigin and Cherry’s Textbook of Pediatric Infectious Disease, 6th Ed. Elsevier-Saunders: 2009.

6. California Department of Public Health. Infant Botulism Treatment and Prevention Program. What is BabyBIG? Accessed December 12, 2020. http://www.infantbotulism.org/general/babybig.php

7. Arnon SS, Schechter R, Maslanka SE, Jewell NP, Hatheway CL. Human botulism immune globulin for the treatment of infant botulism. N Engl J Med. 2006;354(5):462-471.

8. California Department of Public Health. Infant Botulism Treatment and Prevention Program. How to obtain BabyBIG®. Accessed December 12, 2020. https://www.cdph.ca.gov/Programs/CID/DCDC/Pages/ObtainBabyBig.aspx