Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Chronic productive cough; tubular bronchiectasis.
Treatment: High Frequency Chest Wall Oscillation (The Vest Airway Clearance System - E0483).
The insurer denied: High Frequency Chest Wall Oscillation (The Vest Airway Clearance System - E0483).
The denial is overturned.

The patient is a school-aged female child with medical history significant for chronic productive cough, chronic rhinitis, tubular bronchiectasis, and situs inversus totalis. Subsequent sputum culture revealed moderate white blood cells (WBCs) and many Haemophilus (H) influenza. The family was compliant with a twice daily airway clearance regimen that included albuterol, hypertonic 7% saline, Vest therapy, Cough Assist, and inhaled corticosteroid (ICS). Since starting this regimen, the patient's cough and nasal symptoms improved. The patient's work-up included chest computed tomography (CT) revealing diffuse bronchial wall thickening with mild tubular bronchiectasis and situs inversus totalis. Sinus computed tomography (CT) was negative. Sweat test was negative. Genetic testing for primary ciliary dyskinesia revealed that the patient was heterozygous for two variants of uncertain clinical significance. Vest therapy was added to the patient's airway clearance regimen. Bronchoscopy revealed significant mucopurulent bronchitis and thick secretions bilaterally. Nasal nitric oxide (NO) was normal at 337 nanoliters per minute (nL/min). Sputum culture at that time contained moderate white blood cells (WBCs) and many Haemophilus (H) influenza. The plan was to complete the work-up for primary ciliary dyskinesia (PCD) with nasal brushing and biopsy for electron microscopy evaluation of ciliary microstructure. The patient was doing well with her airway clearance regimen but was advised to increase to three to four times daily when ill. Flovent was prescribed to replace the nebulized steroid to address the elevated eosinophil count noted from bronchoalveolar lavage. The patent remained on azithromycin three times a week for prophylaxis. 
Vest therapy was ordered. The patient had received manual chest physiotherapy. It was not successful or effective. The patient was in need of better therapy for airway clearance.

The use of Vest therapy is medically necessary for this child.

Primary ciliary dyskinesia is an autosomal recessive condition in which the ultrastructure of ciliated cells is abnormal, impairing ciliary function. There is defective airway clearance, similar to cystic fibrosis, but with incomplete information on disease progression and mortality. Because primary ciliary dyskinesia is an orphan disease, with little data from randomized controlled trials, management strategies are currently being extrapolated from the cystic fibrosis literature. As with cystic fibrosis, the goals of therapy are to prolong stable lung function and delay the inevitable bronchiectasis. Treatment involves airway clearance, infection control and prevention, and elimination of exposure to inflammatory triggers. Chest physiotherapy includes manual percussion and drainage, forced cough and expiration, positive expiratory pressure valves, and chest wall oscillating devices. This child has not yet been formally diagnosed with primary ciliary dyskinesia, with some equivocal results and biopsy pending. However, the patient has a number of features of the disorder, including chronic productive cough and tubular bronchiectasis demonstrated on computed tomography studies. Vest therapy was added to her airway clearance regimen along with bronchodilator, mucolytic, cough assist, and inhaled steroid. This combination was successful, and the patient clinically improved. 

This child has demonstrated ability to tolerate Vest therapy, the family is compliant with her airway clearance regimen, and she has clinically improved. Based on the current standard of care, and clinical situation, high frequency chest wall oscillation (Vest therapy) is medically necessary and appropriate for this child.