Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Congenital central hypoventilation syndrome, suspected ROHHAD syndrome (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation), dysphagia, chronic respiratory failure status post tracheostomy with dependence on mechanical ventilation at night and speaking valve during the day, constipation, obesity with fatty liver, behavioral issues, and global developmental delay
Treatment: Initiation of private duty nursing with a registered nurse or licensed practical nurse: 10 hours per day, 7 days per week 
The insurer denied: Initiation of private duty nursing with a registered nurse or licensed practical nurse: 10 hours per day, 7 days per week 
The denial is overturned



The patient is a young adolescent female with complex medical history significant for congenital central hypoventilation syndrome, suspected ROHHAD syndrome (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation), dysphagia, chronic respiratory failure status post tracheostomy with dependence on mechanical ventilation at night and speaking valve during the day, constipation, obesity with fatty liver, behavioral issues, and global developmental delay. She is able to walk and talk, and she is fully toilet trained. She receives therapies in the home, including physical and speech therapies. She is able to eat a varied diet, no longer requiring thickened feeds. She tends to overeat, requiring monitoring for food volume. The patient lives with her parents, both of whom are actively involved in her care. She has been approved for 41 hours weekly of Consumer Directed Personal Assistance Services. The patient's skilled nursing needs include continuous monitoring for behavioral issues including self-decannulation and overeating, tracheostomy care, management of mechanical ventilation with continuous monitoring while on the vent to prevent interruption (can be fatal in patients with her respiratory challenges), medication administration, providing airway clearance routinely and as needed with bronchodilators and chest physiotherapy and suctioning, monitoring pulse oximetry with use of supplemental oxygen while sleeping, and assistance with all activities of daily living.

The requested skilled nursing services are medically necessary. 
Children with ROHHAD (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) syndrome generally present in early childhood with severe overeating and rapid weight gain. They may show indications of hypothalamic dysfunction with electrolyte abnormalities, hypothyroidism, growth hormone deficiency. There are symptoms of autonomic dysfunction, including gut dysmotility, inability to maintain body temperature with both hypothermia and hyperthermia, sweating, poor distal perfusion, decreased sensitivity to pain, and bradycardia. These children often have behavioral issues and mental health challenges. They develop obstructive sleep apnea early and then progress to central hypoventilation and eventual respiratory failure. They are also prone to developing tumors of neural crest origin. The most prominent issue for these children is chronic respiratory failure and eventual need for mechanical ventilation, particularly when sleeping.

Children with ROHHAD (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) syndrome generally present in early childhood with severe overeating and rapid weight gain. They may show indications of hypothalamic dysfunction with electrolyte abnormalities, hypothyroidism, growth hormone deficiency. There are symptoms of autonomic dysfunction, including gut dysmotility, inability to maintain body temperature with both hypothermia and hyperthermia, sweating, poor distal perfusion, decreased sensitivity to pain, and bradycardia. 

The requested skilled nursing services 10 hours daily for 7 days/week are medically necessary.