Diagnosis: Osteogenesis Imperfecta
Treatment: Zometa
The insurer denied the Zometa.
The determination is overturned.

The patient is diagnosed with osteogenesis imperfecta (OI). As reported, treatments with bisphosphonate have already been delayed for many months secondary to COVID, therefore, she is at increased risk of sustaining life threatening fractures. She has a history of severe OI, failure to thrive, developmental delay, seizures, obstructive sleep apnea (OSA). Her severe OI has caused her multiple lifetime fractures. She has had 12 biphosphonate infusions with pamidronate which have helped to reduce the number and severity of fractures. She has been unable to be hospitalized for the 3 day stay for pamidronate secondary to the inability to obtain a bed secondary to the global COVID pandemic. During a hospitalization for pamidronate infusion, she was noted to have breast budding consistent with a small amount of estrogenized breast tissue under bilateral areola.
Her C-terminal telopeptide (CTX) was 310; N-terminal telopeptide (NTX) (serum) was 37.6; NTX (urine) was 457; alkaline phosphatase (alk phos) was 56.9.
Her Vitamin D was 37.9; parathyroid hormone (PTH) was 56.1.
At issue is the medical necessity of Zometa.
Treatment of osteogenesis imperfecta with intravenous bisphosphonates (pamidronate or zoledronic acid) is standard of care(1-5). This indication is listed in Micromedex for pediatric patients with osteogenesis imperfecta. This patient has osteogenesis imperfecta with fractures. Non-treatment of this patient would be expected to cause ongoing fractures. The request is consistent with the standard of care based on the above cited studies. The request is therefore medically necessary.