The Science Journal of the American Association for Respiratory Care

2001 OPEN FORUM Abstracts

NON-INVASIVEPOSITIVE PRESSURE VENTILATION OF A PEDIATRIC PATIENT WITH SPINAL MUSCLE ATROPHY:A CASE SUMMARY

CherylTuckerman AS, RRT, P/P Specialist, Theresa Ryan Schultz BA, RRT, P/P Specialist,CPFT, RN, Roberta Hales BS, RRT, P/P Specialist, Angela D. Hedgman AS, RRT,P/P Specialist, Joseph Bolton BS, RRT, P/P Specialist, Troy Dominguez MD. TheChildren?s Hospital of Philadelphia. Philadelphia, PA

Introduction: Non-invasivepositive pressure ventilation (NPPV) is one of the tools that we use to treatdyspnea and improve the quality of life of the patient with terminal disease1.Its use, however, has limitations and adverse effects. NPPV?s associated adverseeffects include nasal congestion, nasal/oral dryness, nasal redness, nasal ulceration,mask discomfort/leakage, gastric distension, eye irritation, aspiration, inabilityto ventilate, failure to tolerate, and skin breakdown. The following case summarywill focus on the challenges of maintaining skin integrity of a pediatric patientmanaged with NPPV.

Case Summary: Thisis a 5-month old with reactive airway disease and gastric esophageal refluxwho was diagnosed at 1 month of age with Spinal Muscle Atrophy (SMA) Type 1.SMA Type 1 is an autosomal recessive disease; the most common inherited neuromusculardisease of the hypotonic newborn2. During a scheduled, outpatientdiagnostic barium swallow, the patient suffered a cyanotic event. Subsequentinterventions included oxygen, intubation and positive pressure ventilation.She was transferred to the Pediatric Intensive Care Unit for management of presumedaspiration pneumonia. The transition to NPPV immediately post extubation, enabledus to wean from invasive positive pressure after a period of 8 days. The goalwas to support the child non-invasively while she continued to recover fromthis acute episode. It soon became evident that her chronic illness had progressedsuch that a full recovery to her previous baseline would not occur. Ongoingcomplications of NPPV were addressed regularly in this patient who now requiredcontinuous NPPV. The goals of the therapy were successfully met, but not withoutthe serious adverse effect of skin breakdown. Over the next 6 weeks, trialsoff NPPV and multiple mask type interfaces proved to be unsuccessful, as thechild?s skin breakdown worsened. Plastic surgery was consulted and nasal bridgewound management was more specifically addressed; meanwhile, the search foralternatives continued. A nasal prong interface was attempted. This alternativemethod of delivery was well tolerated by the patient and allowed for the healingof her wound. Eventually this patient was successfully transitioned to homeon continuous NPPV utilizing a combination interface of nasal mask and nasalprongs.

Discussion: Maintainingskin integrity is one of the biggest challenges to the implementation of non-invasivepositive pressure ventilation, particularly in patients with neuromuscular diseasewho are high risk for complications related to limited mobility. Pressures sorescreated by the nasal mask fit many times lead to staged wounds requiring surgicalintervention. Nasal prongs, used as an alternative NPPV system interface, seemedbeneficial in the care of this 5-month-old infant with SMA Type I. More avenuesneed to be explored regarding the limited availability of equipment to interfacesmall children with NPPV.

 

1. Benditt, JO. NoninvasiveVentilation at the End of Life. Respiratory Care 2000: 45:1376-1381.

2. Bach JR, NiranjanV., and Weaver B. Spinal Muscle Atrophy Type 1* A Noninvasive Respiratory ManagementApproach. Chest 2000: 117: 1100-1105.

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