Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Immunologic Disorders.
Treatment: Infusion therapy.
The health plan denied the treatment with IVIG (Intravenous immunoglobulin) as not medically necessary. 
The reviewer has overturned in whole the health plan's determination.

This is a patient with complex cardiac history, history of Kabuki syndrome, hypoplastic left heart syndrome (HLHS) mitral atresia/aortic atresia (MA/AA), double aortic arch, who now has fenestrated Fontan physiology with recent fenestration stent dilation  and history of protein losing enteropathy who is presenting for weight increase of 3 pounds (lbs.) since the week prior. Mother also notes his abdominal girth is up 3 centimeter (cm) as compared to last Monday. She feels that he is retaining fluid and noted periorbital edema as well. Though he has a history of protein losing enteropathy she denied any diarrhea at this time. He was brought to the emergency room (ER) and there his liver edge was noted to be down 5 centimeters (cm), he had some ascites. No respiratory distress, saturating 90% (per cent) at baseline. A chest x-ray (CXR) done at that time revealed mild pulmonary edema with small bilateral pleural effusions. The brain (or B-type) natriuretic peptide (BNP) was up to 303, was 141 12 days prior.  He was admitted to the floor and started on albumin followed by Lasix. He had two episodes of emesis.  Respiratory panel positive for rhinovirus/enterovirus (R/E). Received ceftriaxone for 48 hours rule out. 
The guidelines provided with the case for the administration of IVIG are very specific and narrow. In this complex patient, the use of current standard of care and peer reviewed literature are needed to extrapolate what would be the best course of action to safely treat this patient as his situation is not straightforward. When considering this patient, there are multiple facets to his issue of hypogammaglobulinemia. He has a history of Kabuki syndrome as well as a complex cardiac history s/p Fontan procedure. He has been diagnosed with protein losing enteropathy which is known to be associated with the Fontan procedure. The question to be answered is the medical necessity of IVIG in this case. Based on peer reviewed literature and standard of care, this patient would be a suitable candidate for IVIG infusion.
 "Hypogammaglobulinemia is a frequent finding in children with Kabuki Syndrome. The pattern of antibody abnormalities seen in children with Kabuki syndrome resembles common variable immune deficiency (CVID)." (Hoffman, Ciprero) This patient was found to have IgG level of 100 which is significantly lower than normal range (582-1441). The treatment for hypogammaglobulinemia as a result of CVID is periodic IVIG infusion. The complicating factor here is the patient has also been diagnosed with protein losing enteropathy (PLE). This patient is known to have flares marked by diarrheal stools. At the time the patient presented with his low IgG levels, there was no mention of diarrhea. Theoretically, those with PLE may lose vital proteins through gut losses during a flare which results in hypogammaglobulinemia. As there are two mechanisms at work in this patient that may contribute to his hypogammaglobulinemia and given his critically low IgG levels, IVIG infusion to address the CVID component and medical treatment (steroids or otherwise) to control his PLE component, if it is felt currently to be a contributing factor, would be a logical course of action to ensure the safety of this patient and to improve his immune function. Literature and current standard of care support IVIG for those with CVID to reduce hospitalizations, need for antibiotics and for prevention of serious infection. Recognizing that his CVID like syndrome is a contributing factor to his hypogammaglobulinemia and treating it, accordingly, based on current standard of care, would be in the best interest of this patient.