2.0 POLICY
2.1 Coverage
for treatment of rare diseases may be considered on a case-by-case
basis. In reviewing the case, the contractor is authorized to approve
coverage when it is determined that the proposed treatment for the
rare disease is medically necessary, including that the treatment
is safe and effective.
2.1.1 In reviewing the case, any
or all of the following sources of clinical literature may be used
to determine if the proposed treatment is considered safe and effective.
2.1.1.1 Trials published in refereed
medical literature.
2.1.1.2 Formal technology assessments.
2.1.1.3 National medical policy organization
positions.
2.1.1.4 National professional associations.
2.1.1.5 National expert opinion organizations.
2.2 If case review indicates that
the proposed benefit for a rare disease is safe and effective for
that disease, benefits may be allowed. If benefits are denied, an
appropriate appealing party may request an appeal.
2.3 The contractor shall code TRICARE
Encounter Data (TED) Record Special Processing Code “RD -
Rare Diseases” on all TED records where the contractor has approved
treatment for a rare disease. Assignment of Special Processing Code RD will
allow the DHA to identify procedures approved by contractors under
the Rare Diseases policy and will allow bypass of TED edit 2-160-01R
when the procedure code is on the No Government Pay Procedure Code
List.
2.4 The contractor shall provide
a monthly report. For reporting requirements, see DD Form 1423,
Contract Data Requirements List (CDRL), located in Section J of
the applicable contract. The report should not include the rare
disease treatments previously approved for coverage and listed herein.
2.5 Off-label use of rituximab
may be considered for cost-sharing for the following treatments:
2.5.1 Effective January 1, 2003,
for recurrent nodular CD20 positive lymphocyte predominant Hodgkin’s disease.
2.5.2 Effective March 31, 2005, for
Stiff Person Syndrome.
2.5.3 Effective
May 1, 2007, for Immunoglobulin A (IgA) nephropathy (proliferative
glomerulonephritis) to reduce proteinuria.
2.5.4 Effective March 26, 2010, for
neuromyelitis optica.
2.5.5 Effective
July 20, 2016, for N-methyl-D-aspartate (NMDA) receptor encephalitis.
2.5.6 Effective August 22, 2016,
for constitutional (pure) red blood cell aplasia.
2.5.7 Effective September 16, 2016,
for autoimmune sclerosing pancreatitis.
2.5.8 Effective October 6, 2016,
Immunoglobin G4-related disease (IgG4-RD).
2.5.9 Effective October 27, 2016,
for autoimmune hemolytic anemia.
2.5.10 Effective November 1, 2016,
for Graft-Versus-Host-Disease (GVHD).
2.5.11 Effective November 9, 2016,
for bullous pemphigoid.
2.5.12 Effective November 14, 2016,
as a second-line treatment for autoimmune encephalitis.
2.5.13 Effective November 22, 2016,
for cryoglobulinemia.
2.5.14 Effective January 3, 2017,
for Thrombotic Thrombocytopenic Purpura (TTP).
2.5.15 Effective January 19, 2017,
for polymyositis.
2.6 Effective May 13, 2009, Intraperitoneal
Hyperthermic Chemotherapy (IPHC) (Current Procedural Terminology
(CPT) codes 77600, 77605, and 96445) in conjunction with cytoreductive
surgery or peritonectomy for treatment of pseudomyxoma peritonei
resulting from appendiceal carcinoma may be covered on a case-by-case basis
for adult patients when all of the following criteria are met:
• There is no evidence of distant
metastasis.
• There is evidence of low histological
aggressiveness of the disease.
• The patient has not undergone
preoperative systemic chemotherapy.
• The patient’s condition does
not preclude major surgery.
• The chemotherapeutic agents
used are Mitomycin C, Cisplatin (also known as Cisplatinum), or
Fluorouracil.
2.7 Effective January 21, 2009,
External Infusion Pumps (EIPs) for insulin may be considered for
cost-sharing when the diagnosis is Cystic Fibrosis-Related Diabetes
(CFRD) with fasting hyperglycemia. See
Chapter 8, Section 2.3 for policy regarding
EIPs.
2.8 Post-operative
proton beam radiosurgery/radiotherapy (CPT codes 77520, 77522, 77523,
and 77525) may be considered for cost-sharing when the diagnosis
is sacral chordoma. See
Chapter 5, Section 3.1 for policy regarding
proton beam radiosurgery/radiotherapy.
2.9 Extracorporeal photopheresis
(CPT code 36522) may be considered for cost-sharing when the diagnosis is
Bronchiolitis Obliterans Syndrome (BOS) that is refractory to immunosuppressive
drug treatment. See
Chapter 4, Section 9.2 for policy regarding
photopheresis.
2.10 Effective
May 1, 2008, the off-label use of Selective Internal Radiation Therapy
(SIRT) with yttrium-90 microspheres (resin or glass) may be considered
for cost-sharing for the treatment of unresectable liver metastases from
neuroendocrine tumors. See
Chapter 5, Section 3.2 for policy regarding
brachytherapy/radiation therapy.
2.11 Effective April 15, 2016, Collagen
Cross-linking for the treatment of corneal ectasia due to the rare
disease Keratoconus is safe and effective and may be considered
for cost-sharing.
2.12 Effective
June 1, 2010, Radiofrequency Ablation (RFA), when performed using
a United States (US) Food and Drug Administration (FDA) approved
electrosurgical cutting and coagulation device, may be considered
for cost-sharing for the treatment of liver metastases from gastric
cancer.
2.13 Effective
September 1, 2012, the NovoTTF-100A system (Healthcare Common Procedure
Coding System (HCPCS) codes A4555 and E0766) may be cost-shared
for the treatment of adult patients (22 years of age or older) with
recurrent glioblastoma after surgical and radiation options have
been exhausted.
2.14 Effective
February 4, 2011, Radiesse® Voice laryngoplasty injections may be
cost-shared for the treatment of type 1 laryngeal cleft (also described
as supraglottic interarytenoid defects that extend no further than
the true vocal folds).
2.15 Effective November 27, 1995,
Orthotopic Liver Transplantation (OLT) may be cost-shared for the treatment
of Crigler-Najjar Syndrome Type I. OLT may be performed both prior
to the onset of neurological symptoms or after the onset of neurological
symptoms.
2.16 Effective
June 5, 2013, off-label use of intravenous immune globulin for the
treatment of Hashimoto’s Encephalopathy, may be considered in exceptional
circumstances where there is progressive neurologic decline despite
appropriate steroid therapy or where steroid therapy is contraindicated.
2.17 Effective April 30, 2009, Intrapulmonary
Percussive Ventilation (IPV) may be considered for cost-sharing when
the diagnosis is Cystic Fibrosis (CF). See
Chapter 8, Section 16.1 for policy regarding
IPVs.
2.18 Effective
January 4, 2013, allogeneic hematopoietic cell transplant (CPT code
38240) may be considered for cost-sharing for the treatment of primary
plasma cell leukemia.
2.19 Effective February 1, 2008,
the off-label use of Photodynamic Therapy (CPT code 67221) with
Visudyne (HCPCS code J3396) may be considered for cost-sharing for
the treatment of retinal astrocytic hamartoma in Tuberous Sclerosis.
2.20 Effective June 25, 2014, intracranial
angioplasty with stenting (CPT code 61635) of the venous sinuses may
be considered for cost-sharing for the treatment of pseudotumor
cerebri (also known as idiopathic intracranial hypertension and
benign intracranial hypertension).
2.21 Effective February 1, 2012,
OLT (CPT code 47135) may be considered for cost-sharing for the
treatment of Acute Intermittent Porphyria.
2.22 Effective December 1, 2014,
Photodynamic Therapy may be considered for cost-sharing for the
treatment of Central Serous Chorioretinopathy.
2.23 Effective July 22, 2016, chemotherapy
injections (CPT code 96542) may be considered for cost-sharing for the
treatment of Central Giant Cell Granuloma (CGCG) of the mandible.
2.24 Effective July 22, 2016, Peg
interferon alfa-2A/180 (HCPCS code J3490) may be considered for
cost-sharing for the treatment of CGCG of the mandible.
2.25 Effective August 11, 2016,
a Fluorodeoxyglucose (FDG) PET scan (CPT code 78815) may be considered
for cost-sharing for the treatment of Takayasu's Arteritis (also
known as aortic arch syndrome).
2.26 Effective August 22, 2016,
Gammagard liquid injections (HCPCS code J1569) may be considered
for cost-sharing for the treatment of branch retinal artery occlusion
secondary to Susac’s Syndrome.
2.27 Effective October 13, 2016,
an autologous bone marrow transplant, the harvest of autologous
stem cells, and the cryopreservation of stem cells may be considered
for cost-sharing for the treatment of recurrent medulloblastoma.
2.28 Effective December 15, 2016,
a Magnetic Resonance-guided High Intensity Focused Ultrasound (MRgFUS)
may be considered for cost-sharing for the treatment of Desmoid
fibromatosis.
2.29 Effective
January 24, 2017, Stereotactic Body Radiation Therapy (SBRT) (CPT
codes 77435 and 77373) may be considered for cost-sharing for the
treatment of a benign neoplasm of the aortic body and other paraganglia.
2.30 Effective February 16, 2016,
Proton Beam Therapy (PBT) may be considered for cost-sharing for
the treatment of thymoma.