Appeal type: Experimental/Investigational
Decision: Overturned

Physician 1:  The patient is a three-year-old male with spinal muscular atrophy and a history of multiple hospitalizations for pneumonia and atelectasis. He is G-tube dependent and has difficulty with oral secretions. He has been treated with multiple aerosols as well as the Cough Assist machine, the Vest system and oral suctioning. He cannot tolerate chest physical therapy (CPT) because he is resistant to postural drainage. At issue in this case is whether the Vest system is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy.This patient will require aggressive pulmonary disease preventative treatment for life. He is resistant to postural drainage treatment and has done well with the Vest system. There have been excellent results with the Vest system, not only in cystic fibrosis but in patients with spinal muscular atrophy as well. In fact, the submitted records document that the Vest system has been effective for this patient. Therefore, I have determined that the requested device is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. The Health Plans denial should be overturned.Physician 2:  The patient is a 3-year 11-month old male who is medically fragile with multiple problems related to muscle weakness from spinal muscular atrophy type II.  He has had recurrent pneumonia resulting in multiple hospitalizations, handles his oral secretions poorly (and therefore likely aspirates frequently) and has known gastroesophageal reflux disease. He is unable to eat by mouth and is therefore tube fed. He has been using the Vest system along with a cough assist device in addition to multiple nebulized medications.  Addition of treatment with the Vest system and cough assist device has been temporally associated with a marked decrease in the frequency of hospitalizations due to respiratory illnesses. Because of his underlying neurologic condition, the patient is unable to perform other forms of chest physiotherapy such as the Flutter Valve or Acapella.  The Health Plan has denied coverage for the Vest system on the basis that it is considered investigational for treatment of the patients condition.Use of high frequency chest wall oscillation as a form of pulmonary toilet is not experimental.  The Vest system was approved by the U.S. Food and Drug Administration in 1988.  It has been used widely for more than a decade primarily in the care of patients with cystic fibrosis, although children with excessive mucous production and difficulty clearing secretions (such as this patient) also benefit from chest physiotherapy.  Children, such as this patient, with recurrent aspiration pneumonia eventually develop bronchiectasis as a result of retained secretions and recurrent infections. Thus, interruption of this sequence of events is anticipated to reduce the frequency of hospitalizations and to prolong pulmonary health.  Data supporting the use of high frequency chest wall oscillation in children without cystic fibrosis (CF) is generally lacking.  However, the disease process is quite similar and there is ample data in the CF population to support its use. There are a number of studies looking at the quantity of sputum produced in response to manual chest percussion compared to other techniques of pulmonary toilet.  However, there is no data clearly correlating the quantity of sputum produced in response to pulmonary toilet techniques to any measure of short- or long-term pulmonary health.  Thus, these studies do little to support or refute the benefit of high frequency chest wall oscillation to provide pulmonary toilet. A short-term (four week) study comparing manual percussion, high frequency chest wall oscillation and oscillating positive expiratory pressure demonstrated no difference in pulmonary function between techniques, but also demonstrated patient preference for high frequency chest wall oscillation thereby implying that regular use would be enhanced.  Data regarding long-term (nearly two years) use of high frequency chest wall oscillation in patients with CF demonstrates improved maintenance of lung function in children using chest wall oscillation compared to manual percussion.  Whether this effect is due to enhanced compliance or superiority of the technique is immaterial as the goal of pulmonary toilet is to maintain pulmonary health and the outcome in this study is preservation of lung function as measured by pulmonary function testing.In the case of this patient, the prescribing physician has documented a decrease in the frequency of respiratory-related hospitalizations contemporaneously with the institution of the Vest system. Adherence to recommended use as an outpatient from documentation of the hour meter should be demonstrated as well.  Presumably, regular outpatient use is occurring as the frequency of pulmonary related hospitalizations has fallen dramatically.  Based on the above information, while the use of the Vest system in this clinical situation is experimental/investigational, it is widely used for this purpose among pediatric pulmonologists.  Furthermore, it is my opinion that treatment with the Vest system is likely to be more beneficial for the patient than any available standard treatment. Therefore, the Health Plans denial should be overturned.Physician 3:  The patient is a 3-year 11-month old male with spinal muscular atrophy and associated respiratory problems. He has had repeated episodes of pneumonia. Because of the associated problems including G-tube feedings and reflux with aspiration, he cannot tolerate chest physical therapy and postural drainage. The patients pulmonary specialists put him on the Vest system and documented improvement in his respiratory functioning. The Health Plan has denied coverage for the Vest system stating there are inadequate longitudinal studies in the peer-reviewed journals supporting the effectiveness of the Vest system for patients with spinal muscular atrophy.Though there is a lack of peer-reviewed data establishing the efficacy of the Vest system in this clinical setting, the relative rarity of the patients condition makes such studies difficult and unlikely to happen. The efficacy of the Vest system has been established for patients with cystic fibrosis.  In this patients case, his provider has documented significant improvement since the Vest system was instituted. In addition, other standard clearance therapies have not been for this patient. For these reasons, I have determined that the requested device is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. Therefore, the Health Plans denial should be overturned.