2026 年 22 巻 3 号 p. 229-232
Background: Advances in pediatric, neonatal, and home medical care have markedly improved the long-term survival of children with chronic childhood-onset conditions requiring complex medical support. Consequently, the population of adolescents and young adults with special health care needs (A/YASHCN) is increasing. Despite this demographic shift, systems supporting transition from pediatric to adult healthcare and welfare services remain insufficient in Japan, particularly for patients dependent on long-term medical technologies. We report two cases of medically complex adults with severe physical and intellectual disabilities who developed acute deterioration during home care and required hospitalization in our Department of General Medicine.
Case presentation:
Case 1: A 34-year-old woman with congenital laryngeal stenosis, hypotonia, tracheostomy, and chromosomal abnormality (46XX,17q+) had longstanding medical dependence and post-resuscitation encephalopathy at age 29. Although outpatient transition to adult neurology had been planned, she was eventually lost to follow-up and was cared for entirely at home until she was hospitalized with severe hypoxemia (SpO2 68% on 10 L/min oxygen) due to aspiration pneumonia and tracheostomy tube dislodgement. During hospitalization she developed mucus retention and adhesive bowel obstruction, prompting multidisciplinary discussions regarding full-code preferences and surgical intervention. Conservative management was chosen, and hypercapnia necessitated initiation of home mechanical ventilation at discharge.
Case 2: A 19-year-old man with bilateral schizencephaly, heterotopic gray matter, intractable epilepsy, and profound developmental delay transitioned from pediatric to adult epilepsy care at age 18; however, outpatient access was difficult. He developed aspiration pneumonia and was hospitalized. Oral intake and adherence were poor, and refusal of nasogastric tube placement resulted in inadequate antiepileptic drug delivery, leading to status epilepticus. He required transfer to a tertiary epilepsy center for ventilatory management and medication adjustment. After stabilization, gastrostomy was recommended but declined; temporary nasogastric feeding was continued with caregiver education.
Discussion: These cases illustrate three critical challenges in transitional care for A/YASHCN: insufficient system-level implementation of planned transition, fragmentation of adult subspecialty care for medically complex patients, and delayed initiation of advance care planning (ACP). Acute-care general medicine departments can function as hubs, coordinating emergency care, multidisciplinary decision-making, and discharge planning while bridging pediatric, adult specialty, and community-based services. ACP should be developed longitudinally in home-care settings by clinicians familiar with the patient's long-term trajectory, rather than being initiated during emergency admissions.
Conclusion: For A/YASHCN with severe medical complexity, comprehensive transitional care frameworks and proactive ACP are essential. During acute deterioration, hospital-based general medicine plays a pivotal bridging role within the continuum of care.
