ABSTRACT
Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488 Pulmonary Differences in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488 Congenital Disorders vs. Acquired Disorders . . . . . . . . . . . . . . . . . . . . . . . . 489 Asthma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
Introduction and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489 Classification of Disease Severity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 490 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 491 Life Care Planning Implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 492
Cystic Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493 Introduction and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493 Initial Presentation and Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 494 Nonpulmonary Manifestations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 494 Complications and Life Care Planning Implications . . . . . . . . . . . . . . . 495
Life Care Planning for the Child Requiring Long-Term Mechanical Ventilatory Assistance. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 499
Planning for Initial Discharge. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 499 Initial Referrals for Skilled Nursing and Durable Medical Equipment . . 500 Ongoing Care and Life Care Planning Implications . . . . . . . . . . . . . . . 503
Bronchopulmonary Dysplasia or Chronic Lung Disease of Prematurity . . . . . 504 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 504 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505
Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 506 Life Care Planning Implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507
Idiopathic Congenital Central Hypoventilation Syndrome . . . . . . . . . . . . . . . 508 Pathophysiology and Initial Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . 509 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509 Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 510 Ongoing Care and Life Care Planning Implications . . . . . . . . . . . . . . . 510
Tracheoesophageal Fistula with and without Esophageal Atresia . . . . . . . . . 512 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513 Outcomes and Complications. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513 Life Care Planning Implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 514
Congenital Diaphragmatic Hernia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 516 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 516 Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517 Life Care Planning Implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 519
Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 521 Resources for Additional Information . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 References. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 523
Respiratory illnesses are prevalent in children due to maturational, developmental, and immunologic causes and account for many physician visits, hospitalizations, and missed school days. While most children develop minor respiratory illnesses, serious respiratory illness is one of the most frequent causes of admission to pediatric intensive care units. These children can have ongoing health care needs for the span of their lifetime. Within the framework of complex needs, chronic respiratory disorder diagnoses may not initially be considered when developing a life care plan (LCP); however, many of these illnesses span the child’s lifetime into adulthood, resulting in costly long-term care. Development of a life care plan for the patient with a chronic respiratory condition may assist in organizing the child’s care and outlining the appropriate measures to prevent long-term and costly complications — costly in terms of dollars and quality of life.
