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December 2002 28:6 JOURNAL OF EMERGENCY NURSING 581 PEDIATRIC UPDATE I nfant botulism involves symptoms that can be mistak- en for relatively benign illnesses. Children are often brought to the emergency department for complaints of constipation, “sleeping a lot,” or “not acting right,” but these are some of the early signs of infant botulism. Case study Two parents brought their 10-week-old male infant to the emergency department on a Saturday morning with the chief complaint of constipation and poor nursing. The in- fant had been to his primary pediatrician the week before for a well-baby checkup. The mother was concerned that the infant had not had a bowel movement for almost 2 days. When the mother was breast-feeding early the day be- fore, she felt that the infant was not sucking “quite right” but thought this could be because of the early hour. When she nursed the infant on the morning of admission, she re- ported that the baby could not get a good seal around her nipple, and only made a few weak attempts to nurse. She also noticed that the baby had not had a wet diaper that day. The infant’s vital signs on admission were: blood pres- sure, 92/54 mm Hg; heart rate, 136 beats/min; respiratory rate, 38 breaths/min; oxygen saturation, 98%; and temper- ature, 36.6°C. The triage nurse had noted that the baby had a weak cry with vital signs. Physical examination showed a well-nourished white male who was awake and quiet. His lungs were clear, his mucous membranes were tacky, and his anterior fontanel was open and flat. His skin turgor was normal, without mottling. His abdomen was soft and nontender, with markedly decreased bowel sounds. No nuchal ridgity was noted, and his pupils were equal, round, and reactive to Infant Botulism in a 10-week-old Male: A Wolf in Sheep’s Clothing Author: Steven Taylor, RN, BSN, CEN, Philadelphia, Pa Section Editors: Deborah Parkman Henderson, RN, PhD, and Anne Phelan Bowen, MS, RN Steven Taylor, Southern Jersey Chapter, is Clinical Level III Nurse, Emergency Department, The Children’s Hospital of Philadelphia, Philadelphia, Pa. For reprints, write: Steven Taylor, RN, CEN, E-mail: taylors@email. chop.edu. J Emerg Nurs 2002;28:581-3. Copyright © 2002 by the Emergency Nurses Association. 0099-1767/2002 $35.00 + 0 18/9/129936 doi:10.1067/men.2002.129936

Infant botulism in a 10-week-old male: A wolf in sheep's clothing

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December 2002 28:6 JOURNAL OF EMERGENCY NURSING 581

P E D I A T R I C U P D A T E

Infant botulism involves symptoms that can be mistak-en for relatively benign illnesses. Children are oftenbrought to the emergency department for complaints

of constipation, “sleeping a lot,” or “not acting right,” butthese are some of the early signs of infant botulism.

Case study

Two parents brought their 10-week-old male infant to theemergency department on a Saturday morning with thechief complaint of constipation and poor nursing. The in-fant had been to his primary pediatrician the week beforefor a well-baby checkup. The mother was concerned thatthe infant had not had a bowel movement for almost 2days. When the mother was breast-feeding early the day be-fore, she felt that the infant was not sucking “quite right”but thought this could be because of the early hour. Whenshe nursed the infant on the morning of admission, she re-ported that the baby could not get a good seal around hernipple, and only made a few weak attempts to nurse. Shealso noticed that the baby had not had a wet diaper that day.

The infant’s vital signs on admission were: blood pres-sure, 92/54 mm Hg; heart rate, 136 beats/min; respiratoryrate, 38 breaths/min; oxygen saturation, 98%; and temper-ature, 36.6°C. The triage nurse had noted that the baby hada weak cry with vital signs.

Physical examination showed a well-nourished whitemale who was awake and quiet. His lungs were clear, hismucous membranes were tacky, and his anterior fontanelwas open and flat. His skin turgor was normal, withoutmottling. His abdomen was soft and nontender, withmarkedly decreased bowel sounds. No nuchal ridgity wasnoted, and his pupils were equal, round, and reactive to

Infant Botulism

in a 10-week-old Male: A Wolf

in Sheep’s Clothing

Author: Steven Taylor, RN, BSN, CEN, Philadelphia, Pa

Section Editors: Deborah Parkman Henderson, RN, PhD, andAnne Phelan Bowen, MS, RN

Steven Taylor, Southern Jersey Chapter, is Clinical Level III Nurse,Emergency Department, The Children’s Hospital of Philadelphia,Philadelphia, Pa.

For reprints, write: Steven Taylor, RN, CEN, E-mail: [email protected].

J Emerg Nurs 2002;28:581-3.

Copyright © 2002 by the Emergency Nurses Association.

0099-1767/2002 $35.00 + 0 18/9/129936doi:10.1067/men.2002.129936

582 JOURNAL OF EMERGENCY NURSING 28:6 December 2002

PEDIATRIC UPDATE/Taylor

light and accommodation (PERRLA) at 4 mm. His muscletone was decreased, and his legs fell apart in a frog-leg con-figuration when he was lying on the bed. When he waspicked up, his head lolled floppily.

Most notable was the infant’s lack of reaction to exter-nal stimuli. He lay quietly on the stretcher or in hismother’s arms. He did not attempt to suck when a pacifierwas placed in his mouth, and minimal rooting was notedwhen his cheek was stroked. Attempts to elicit a cry fromhim resulted only in feeble efforts on his part.

Infant botulism is caused byClostridium botulinum, a gram-positive spore-forming anaerobe that isfound in dust and soil, most commonlyin California, Utah, and SoutheasternPennsylvania. Once they contaminatethe gut, the spores germinate, repro-duce, and produce a neurotoxin thatblocks neuromuscular transmission ofacetylcholine.

Because of the infant’s questionable hydration status, aperipheral IV line was established. He did not react to theneedle stick. Because of his inability to nurse and overallweakness, he was kept at nothing-by-mouth status, and theparents advised accordingly. Routine labs were obtained; allresults were within normal limits for his age. A focused neu-rological examination revealed absent deep tendon reflexesin upper extremities. Although the infant was initially PER-RLA, the pupillary reflex extinguished after rapidly re-peated tests. A weak gag reflex was noted. A negative inspi-ratory force test was performed, with a reading of –10 cmH2O (normal < –25 cm H2O).

A clinical diagnosis of infant botulism was made, andarrangements were made to transfer the patient to the pedi-atric ICU. While the ED staff were preparing for the trans-fer, the mother was found attempting to express milk intothe baby’s mouth. The patient was unable to handle themilk, and suctioning was necessary to clear the airway. Theparents were reminded of the strict nothing-by-mouth

status of the infant. The patient was positioned with a small(3-finger width) roll under the neck to allow secretions topool in the anterior pharynx away from the trachea, wherethey could be more easily swallowed.1

The patient was admitted to the pediatric ICU andelectively intubated, primarily for airway protection. Stoolsamples obtained after sterile non-bacteriostatic enemasconfirmed the diagnosis of infant botulism. Aspirationpneumonia was noted on chest radiograph, presumablyfrom the infant’s inability to handle secretions and feedings.Botulism antitoxin therapy was initiated, and the baby wassuccessfully extubated at 12 days and discharged at 28 dayswithout major sequelae.

Discussion

Infant botulism is caused by Clostridium botulinum,2

a gram-positive, spore-forming anaerobe that is found indust and soil, most commonly in California, Utah, andSoutheastern Pennsylvania.3 Once they contaminate thegut, the spores germinate, reproduce, and produce a neuro-toxin that blocks neuromuscular transmission of acetyl-choline. The transmission site is permanently blocked; re-sumption of nerve transmission depends on out sproutingof new terminal ends.4 The spores do not germinate in pa-tients more than one year of age because of the change ofpH in the gut from acidic to basic as the intestinal florachanges from Lactobacillus to normal childhood flora.4 Theincubation time is 3 days to 30 days and has a peak inci-dence of 2 months to 4 months,5 although infant botulismhas been noted in patients from 6 days6 to 351 days.7

The most common initial sign noticedin infant botulism is constipation.

A human-derived botulism immune globulin (BIG) isavailable in the United States, and has been shown to re-duce hospital stays and costs associated with infant botu-lism.1 BIG may be obtained 24 hours a day from the Cali-fornia Department of Health Services by calling(510)540-2646.

Equine-derived botulism antitoxin is also available, al-though its efficacy is debatable in infant botulism,2,5,8 and,

December 2002 28:6 JOURNAL OF EMERGENCY NURSING 583

PEDIATRIC UPDATE/Taylor

because of the rather high incidence (8%-20%)8 of anaphy-lactic reaction, many infectious disease experts do not rec-ommend routine administration of antitoxin for the infantvariant of the disease.5,8 The antitoxin only neutralizes circu-lating toxins; it does not treat the nerve junctions that havealready been affected.2 The effects of the neurotoxin are asymmetrical descending paralysis,4 unlike Guillain-Barresyndrome, which has an ascending symmetrical paralysis.9

The most common initial sign noticed in infant botu-lism is constipation, as it was for this patient. However, inpatients who normally have a bowel movement only everyfew days, the parents may not notice the change as rapidly,so constipation may not be recognized early.

Proper positioning to avoid aspirationand position changes to prevent skinbreakdown are also vital.

In most cases of infant botulism, the cause of contami-nation is not found, although honey has been implicated,5

and parents who work on construction sites may bringhome spores on their shoes or clothing. However, this in-fant was not fed honey, both parents worked in offices, anda careful review of contacts in the preceding weeks did notreveal the source of contamination.

Infant botulism can present with a myriad of symp-toms that, early in the disease, are innocuous and subtleenough that only parents would notice the problem. With-out careful and complete serial examinations, it is easy tomiss the symptoms of the disease. For example, this infantinitially had normal pupil reflexes; however, these normalreflexes became extinguished.

Infant botulism requires proper positioning to avoidaspiration, position changes to prevent skin breakdown,and nutritional care.10 Routine antibiotics are avoided be-cause they may lyse bacteria and release more neurotoxinsinto the bloodstream.11 Preventable complications includeapnea, pneumonia, urinary tract infections, and sepsis; therisks of these complications can be reduced through propernursing care and pulmonary toilet. Our patient developedan aspiration pneumonia. We found that the parents wereworried that their infant was not being fed, and attemptedto feed him even after the infant’s nothing-by-mouth status

was conveyed. In the future, we will emphasize the infant’slack of muscle tone and absent gag reflex, and underscorethe possibility of aspiration for other parents.

Overall, our patient’s course showed that recovery isachievable, but only with early recognition, proper care,early endotracheal intubation, and prompt BIG therapy.

REFERENCES1. Arnon SS. Infant botulism. In: Fegin RD, Cherry JD, editors.

Textbook of pediatric infectious disease. 4th ed. Philadelphia:Saunders; 1998. p. 722-30.

2. Centers for Disease Control and Prevention. Botulism in theUnited States, 1899-1996. Handbook for epidemiologists, clini-cians, and laboratory workers. Atlanta: 1998.

3. Schreiner MS, Field E, Ruddy R. Infant botulism: a review of 12years’ experience at the Children’s Hospital of Philadelphia. Pedi-atrics 1991;87:159-65.

4. Long S. Clostridium botulinum (botulism). In: Long S, Picker-ing LK, Prober C, editors. Principles and practice of pediatric infectious diseases. New York: Churchill Livingstone; 1997. p.1085-93.

5. American Academy of Pediatrics. Botulism and infant botulism.In: Peter G, editor. 1997 red book: Report of the committee oninfectious diseases. 24th ed. Elk Grove Village (IL): AmericanAcademy of Pediatrics; 1997. p. 174-6.

6. Thilo EH, Townsend, SF, Deacon J. Infant botulism at 1 week ofage: report of two cases. Pediatrics 1993;92:151-3.

7. Spika JS, Shafer N, Hargrett-Bean N. Risk factors for infant bot-ulism in the United States. Am J Dis Child 1989;143:828-32.

8. Bleck TP. Clostridium botulinum (botulism). In: Mandell GL,Bennett JE, Dolin R, editors. Principles and practices of infec-tious diseases. 5th ed. New York: Churchill Livingstone; 2000. p.2543-8.

9. Turbiak TW, Reich J. Bacterial infections. In: Rosen P, Barkin R,editors. Emergency medicine: concepts and clinical practice. 4thed. St Louis: Mosby; 1998. p. 2504-31.

10. Few B. Pulmonary critical care problems. In: Curley MA, SmithJB, Moloney-Harmon PA, editors. Critical care nursing of infantsand children. Philadelphia: Saunders; 1996. p. 619-55.

11. Midura TF. Update: infant botulism. Clin Microbiol Rev 1996;9:119-25.

Submissions to this column are welcomed and encouraged. Contri-butions can be sent to one of the following:

Deborah Parkman Henderson, RN, PhD1255 Linda Ridge Rd, Pasadena, CA 91103

310 328-0720 • [email protected]

Anne Phelan Bowen, MS, RN12 Harrington St, East Falmouth, MA 02536

508 540-4108 • [email protected]