Management of respiratory infection in a child with Dravet syndrome and tracheostomy

Anitha H R^1*, Shalini H S², Vijayakumari. D³

¹Assistant Nursing Superintendent, Kauvery Hospital, Electronic City, Bangalore

²Chief Nursing Officer, Kauvery Hospital, Electronic City, Bangalore

³Nurse Educator, Kauvery Hospital, Electronic City, Bangalore

*Correspondence

Abstract

Dravet syndrome is a rare developmental and epileptic encephalopathy characterized by recurrent seizures, developmental delay, and multiple neurological complications. Children with Dravet syndrome who have a tracheostomy are at increased risk of recurrent respiratory infections, particularly with multidrug-resistant organisms. We report the case of a 7-year-old female with known Dravet syndrome and tracheostomy who presented with breathing difficulty. Respiratory culture revealed Pseudomonas aeruginosa. The child was managed with appropriate intravenous antibiotics, supportive care, nebulization, electrolyte correction, and close monitoring in the Pediatric Intensive Care Unit (PICU). She showed significant clinical improvement and was discharged in stable condition. This case highlights the importance of early diagnosis, culture-guided antibiotic therapy, multidisciplinary management, and comprehensive nursing care in achieving favourable outcomes.

Keywords: Dravet syndrome; Tracheostomy; Pseudomonas aeruginosa; Respiratory infection; Pediatric intensive care.

Introduction

Dravet syndrome is a rare genetic epilepsy syndrome that usually presents during the first year of life. It is associated with prolonged seizures, developmental delay, cognitive impairment, speech difficulties, behavioral abnormalities, and motor dysfunction. Due to neurological impairment and poor airway clearance, these children are highly susceptible to recurrent respiratory infections.

Children with long-term tracheostomy have an increased risk of airway colonization by multidrug-resistant organisms, especially Pseudomonas aeruginosa. Respiratory infections in these patients can rapidly worsen and require intensive care management. Early recognition, prompt microbiological diagnosis, and timely administration of appropriate antibiotics are essential to reduce morbidity and improve outcomes.

Case Presentation

A 7-year-old female child, a known case of Dravet syndrome with tracheostomy in situ, presented to the Emergency Department with complaints of fast breathing for one day. She was receiving multiple antiepileptic medications regularly. There was no history of fever, cough, cold, vomiting, or recent seizure episodes. Because of her underlying neurological disorder and respiratory symptoms, she was admitted to the Paediatric Intensive Care Unit (PICU) for further evaluation and management.

On admission, the child was conscious, alert, and active. Her weight was 15.2 kg.

Vital signs

Heart Rate	93 Beats/min
Respiratory Rate	25 Breaths/min
Temperature	98 F
Oxygen Saturation	93% on room air.
Blood Pressure	90/60 mm of Hg
Respiratory examination	Bilateral equal air entry with tracheostomy tube in situ.
Cardiovascular examination	Normal heart sounds (S1 and S2) without murmurs.
Abdomen	soft and non-tender

Laboratory investigations

Hemoglobin	12.2 g/dL
Total leukocyte count	9,290 cells/mm³
Platelet count	1.79 lakh/mm³
Sodium	123 mmol/L
Potassium	5.5 mmol/L
Chloride	94 mmol/L
C-reactive protein (CRP)	1.2 mg/dL

Culture & Antibiotics Details

Respiratory culture

Pseudomonas aeruginosa

Respiratory culture identified Pseudomonas aeruginosa, which showed resistance to ampicillin, amoxicillin-clavulanate, cephalosporins, ertapenem, and cotrimoxazole. The child was managed with culture-guided intravenous antibiotics, intravenous fluids, electrolyte correction, nebulization, routine tracheostomy care, airway suctioning, oxygen therapy as required, and supportive care. Continuous monitoring was performed in the PICU to assess respiratory status and detect complications early. Following treatment, the child’s respiratory distress gradually improved. Oxygen saturation remained stable, and there were no further episodes of respiratory compromise. She was shifted to the pediatric ward and subsequently discharged in stable condition with advice for regular follow-up, tracheostomy care, and continuation of prescribed medications.

Discussion

Dravet syndrome is a lifelong neurological disorder requiring continuous multidisciplinary care. Respiratory infections remain one of the major causes of hospitalization in these children because of impaired airway clearance, recurrent aspiration, reduced cough reflex, and tracheostomy dependence. Pseudomonas aeruginosa is a common pathogen in children with chronic airway disease and tracheostomy. The organism is often resistant to multiple antibiotics, making culture and sensitivity testing essential before initiating definitive antimicrobial therapy. In this case, early admission to the PICU, prompt microbiological investigations, appropriate intravenous antibiotics, meticulous tracheostomy care, airway clearance, electrolyte correction, and supportive management resulted in a favorable outcome. Nursing care played a vital role through continuous monitoring of respiratory status, maintenance of airway patency, infection prevention, medication administration, family education, and early identification of clinical deterioration.

This case emphasizes the importance of coordinated multidisciplinary care involving pediatricians, intensivists, microbiologists, respiratory therapists, nurses, and caregivers to ensure successful management and reduce the risk of recurrent respiratory complications.

Conclusion

Respiratory infections are a significant cause of morbidity in children with Dravet syndrome and tracheostomy. Early recognition of respiratory distress, prompt microbiological evaluation, culture-guided antibiotic therapy, meticulous airway and tracheostomy care, and comprehensive supportive management are essential for successful treatment. A multidisciplinary approach involving physicians, nurses, respiratory therapists, and caregivers’ plays a crucial role in improving patient outcomes. Regular follow-up, caregiver education, and infection prevention strategies are equally important in minimizing recurrent hospitalizations and enhancing the quality of life of these children.

References

Wirrell EC, Laux L, Donner E, et al. Optimizing the diagnosis and management of Dravet syndrome. Epilepsia. 2017;58(9):1431-1442.
World Health Organization. Pocket Book of Hospital Care for Children. 3rd ed. WHO; 2022.
Nelson Textbook of Pediatrics. 22nd Edition. Elsevier; 2023.

Management of respiratory infection in a child with Dravet syndrome and tracheostomy

Management of respiratory infection in a child with Dravet syndrome and tracheostomy

Anitha H R1*, Shalini H S2, Vijayakumari. D3

Nightingale Journals

Anitha H R^1*, Shalini H S², Vijayakumari. D³