Rett Syndrome: when insurers say no, reviewers often say yes

Where the denial was overturned

Nature of Statutory Criteria/Case Summary: The patient’s parent has requested authorization and coverage for speech generating device and accessories (CPT E2510 and E2512). Findings: The physician reviewer found that records provided for review document that this patient presents with a history of Rett syndrome, and she is non-verbal. The patient’s provider has recommended treatment with a speech generator device and related accessories. On review of the peer-reviewed medical literature, the requested equipment is medically necessary for treatment of this patient. Rett syndrome is a severe, progressive neurodevelopmental disorder with multisystem comorbidities that evolve across a patient’s lifespan.

Findings: The physician reviewer found that Nature of Statutory Criteria/Case Summary: The patient’s parent has requested authorization and coverage for a Tobii Dynavox I-13 speech device (CPT E2510), eye gaze interaction accessory (CPT E2512), and mount (CPT E2599). Fu and colleagues describe Rett syndrome as a severe, progressive neurodevelopmental disorder with multisystem comorbidities that evolve across a patient’s lifespan, characterized by an initial period of normal development followed by psychomotor regression, deceleration of head growth and development of distinctive repetitive, purposeless hand movements. The period of dramatic psychomotor regression between six and 18 months of age may include loss of language, hand skills, and gross motor function, along with the onset of stereotypical hand movements.

Where the denial was upheld

This patient is a two-year, eleven-month old female with a diagnosis of Rhett’s syndrome. Associated with the Rhett’s syndrome is a seizure disorder and cognitive/developmental delay, which includes significant motor delay. The patient is able to stand independently while holding onto furniture, but cannot stand independently without support. She requires maximum assistance to ambulate and is currently taking two to three steps using a Rifton Pacer Gait Trainer. When ambulating, she does not respond to commands and does not walk with intention toward an object. The patient requires moderate to maximum assistance to walk with the ambulatory assistance device. The Health Plan has denied authorization for the device based upon their determination that the patient cannot use it for its intended function.

The parent of a female enrollee requested 40 hours per week of applied behavioral analysis (ABA) services for medical treatment of the enrollee’s Rett syndrome and autism spectrum disorder. Findings: Two physician reviewers found that there is a lack of medical literature supporting the use of ABA or other behavioral therapies for the treatment of Rett syndrome. However, ABA therapy is the standard of care in the therapeutic intervention of autistic children. According to Myers and Johnson, “Educational interventions, including behavioral strategies and habilitative therapies, are the cornerstones of management of ASDs. These interventions address communication, social skills, daily-living skills, play and leisure skills, academic achievement, and maladaptive behaviors.” In this case, the Health Plan has authorized 25 hours per week of direct ABA services.

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 rett syndrome was denied, the published record says the denial is worth fighting.