Neuromuscular Compromise and Respiratory Failure Clinical Pathway — Inpatient and PICU

Neuromuscular Conditions Affecting Respiratory Status

This pathway is intended for use in the inpatient pulmonary service and the PICU. See table below for common conditions.

Exclusions: Patients without an underlying neuromuscular disease.

Condition	Features
Spinal Muscular Atrophy	Disease of the central nervous system (CNS) that results in the deterioration of motor function and strength over time Rate and severity of deterioration may vary
Myotubular Myopathy	Muscles used for movement (skeletal muscles) most affected Occurs almost exclusively in males Muscle weakness (myopathy) and decreased muscle tone (hypotonia) usually evident at birth
Transverse Myelitis	Caused by inflammation of the spinal cord resulting in varying degrees of weakness, sensory alterations, and autonomic dysfunction
Duchenne Muscular Dystrophy	Results in progressive muscle wasting that gets worse over time Occurs primarily in males Progressive weakness and loss (atrophy) of both skeletal and heart muscle Early signs may include delayed ability to: Sit Stand Walk Difficulties learning to speak
Guillain-Barre	Characterized by progressive weakness The most common form of the disease, acute inflammatory demyelinating polyradiculoneuropathy, presents as progressive motor weakness, usually beginning in the legs and advancing proximally Respirations and vital functions are often compromised with advanced symptoms
Congenital Muscular Dystrophy	Congenital muscular dystrophy (CMD) refers to a group of muscular dystrophies that become apparent at or near birth Patient with CMD will have overall muscle weakness

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

Accordingly, these clinical pathways are not intended to constitute medical advice or treatment, or to create a doctor-patient relationship between/among The Children’s Hospital of Philadelphia (“CHOP”), its physicians and the individual patients in question. CHOP does not represent or warrant that the clinical pathways are in every respect accurate or complete, or that one or more of them apply to a particular patient or medical condition. CHOP is not responsible for any errors or omissions in the clinical pathways, or for any outcomes a patient might experience where a clinician consulted one or more such pathways in connection with providing care for that patient.