Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Speech delay; branchial cleft; oral aversion
 
Treatment: Dysphagia Rehabilitation 

The insurer denied coverage for Dysphagia Rehabilitation (Intensive Swallow Therapy x 40 units). The denial is reversed. 

This patient is a male child with a history notable for very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), branchial cleft, speech delay, iron deficiency anemia, oral aversion, vomiting and gastrostomy tube dependent for feeds/supplements/medications.  To address his deficient oral motor skills and profound oral aversion/hypersensitization he has been receiving speech/feeding therapy a couple days per week outpatient feeding program with little reported progress.  Although not even tolerating an empty spoon in his mouth initially he had at least progressed to taking small volumes of water via syringe. Because of his slow progress the team has suggested enrollment into their Intensive Day Program for feeding disorders.
This program provides 4 sessions of therapy per day totaling 20 sessions per week or the equivalent of 2.5 months on his prior intervention schedule.  The goals of therapy included: 1) improve oral motor skills 2) increase overall oral intake 3) reduce negative feeding behaviors 4) decrease GT dependence, and 5) parental/caregiver training to perpetuate therapy at home. 

This child has both a branchial cleft leading to anatomic alterations in feeding routines and a primary neuromuscular disorder leading to dysfunctional feeding mechanics. Unfortunately, the longer this child's feeding disorder continues the longer it will take to rehabilitate. This child has VLAD, a fatty acid oxidation disorder (FAOD) or inborn error of metabolism resulting in failure of mitochondrial beta-oxidation or the carnitine-based transport of fatty acids into mitochondria. FAODs lead to deficient energy production and produce widely variable clinical presentations ranging from mild hypotonia in adults to sudden death in infants.

In a second review of FAOD from UpToDate the authors conclude the following: 1) Common presenting signs include hypoglycemia, hyperammonemia, liver disease and liver failure, cardiac and skeletal myopathy, rhabdomyolysis, and retinal degeneration. Patients are most at risk during times of fasting or intercurrent illnesses. 2) Most FAODs are detected through newborn screening (NBS) by tandem mass spectrometry in developed countries. The preferred tests to confirm a diagnosis in an infant with a positive NBS are a plasma acylcarnitine profile and total and free carnitine levels. DNA testing is the most common definitive test and can provide information on final diagnosis. Leukocyte, fibroblast, or liver enzyme assays can be used to determine functional significance of unknown variants identified on DNA sequencing.

This child will clearly benefit from the ability to maintain oral intake to prevent exacerbation of his FAOD and precipitation of a crisis. A program that would improve oral tolerance would add stability to management of his inborn error of metabolism. The insurer conditionally approved the first 10 unit sessions on demonstrated improvement in feeding tolerance. There is clear documentation of his progress that merits continuation of the intensive feeding program. The literature would suggest that a multidisciplinary team with experience in feeding disorder management would yield the best results. Therefore, continued intensive therapy is warranted in this particular child and medically necessary to effective manage his underlying multifactorial feeding disorder. 

The health plan did not act reasonably with sound medical judgment in the best interest of the patient.

Based on the above, the medical necessity for the requested Dysphagia Rehabilitation (Intensive Swallow Therapy x 40 units) is substantiated.  The insurer's denial is reversed.