Prader-willi Syndrome: when insurers say no, reviewers often say yes

Where the denial was overturned

Nature of Statutory Criteria/Case Summary: The parent of an enrollee has requested authorization and coverage for physical therapy and occupational therapy in the home setting. Findings: The physician reviewer found that Prader-Willi syndrome is a genetic disorder characterized by early feeding difficulties and failure to thrive, as well as significant hypotonia. Hypothalamic dysfunction may be the basis for short stature and hypogonadism. It may also contribute to problems with temperature regulation. Later on, hyperphagia leading to obesity and learning disabilities are characteristic. Respiratory problems include obstructive apnea and central hypoventilation and/or apnea. Chronic gastroesophageal reflux and aspiration also contribute to respiratory difficulties. Upper respiratory infections are often more serious than initially expected.

The patient is a 10-year-old male who was diagnosed with Prader-Willi syndrome (PWS) at the age of eight months. His current growth velocity at age 10 is 16cm/year. In addition, the patient is Tanner Stage 2 and his IGF-1 level is 142. The patient’s physician indicates the patient has evidence of growth failure dropping from the 95th percentile at age 9 to the 80th percentile by age 10. The physician also states the patient has premature adrenarche and bone age advancement, which compromises his ability to attain his genetic potential of 172.5cm. The physician has recommended Genotropin growth hormone (GH) therapy to preserve the patient’s height projection and improve muscle mass and function.

Where the denial was upheld

Nature of Statutory Criteria/Case Summary: The patient has requested authorization and coverage for 56 hours of licensed vocational nurse (LVN) level of care and two visits registered nurse (RN) supervision weekly for three months.Findings: The physician reviewer found that records provided for review document that this patient has multiple medical problems as a result of prematurity and Prader-Willi syndrome. The provider noted that the patient has required treatment in the emergency department, and he has been hospitalized multiple times with acute respiratory illnesses, including infection with COVID-19. Despite the patient’s acute and chronic issues, his medical condition is currently stable. The patient’s daily care includes services that can be safely and effectively met by a trained caregiver (Parab, et al.; Nageswaran and Golden).

Nature of Statutory Criteria/Case Summary: The parent of an enrollee has requested authorization and coverage for Genotropin for treatment of the enrollee’s Prader-Willi syndrome. Findings: The physician reviewer found that the request for Genotropin is not medically necessary for treatment of the patient’s medical condition. GH treatment for children with Prader-Willi syndrome has been shown to improve linear growth and promote growth of lean body mass, decreases fat mass by increasing fat oxidation and total body energy expenditure, and improves cardiovascular risk factors. Complications of GH therapy in children with Prader-Willi syndrome are infrequent, and with close monitoring, GH therapy is considered to be generally safe in this population.

These outcomes come from California’s external review program — an independent physician panel whose decision binds the insurer. Every state has an equivalent, and internal appeals succeed even more often. If your care for prader-willi syndrome was denied, the published record says the denial is worth fighting.