CHILDREN AND ADOLESCENTS - under each indication, all bulleted items are required
Children and adolescents with growth hormone deficiency:
- Two (2) pharmacologic provocation tests (insulin, clonidine, arginine, levodopa, glucagon) with a result < 10 ng/ml.
- Because stimulation tests are not a gold standard, patients who do not meet stimulation test criteria < 10ng/ml on both tests or whose stimulation tests are discordant can be approved for a 6 month trial of growth hormone if the other criteria for growth hormone deficiency have been met and both IGF-1 and IGFBP-3 levels are below the normal range for gender and age
- Examination by a pediatric endocrinologist who determines the child's chronological age and stage of pubescence and who performs a complete history, physical examination, and laboratory tests to evaluate the child's growth
- The patient's baseline height must be > 2 standard deviations [SD] below the mean for gender and age (which corresponds to < 2.3 percentile)
- Growth rate is such that the patient is unlikely to attain an adult height in the normal range - 59 inches for girls and 63 inches or boys (i.e. growth velocity below the 25th percentile or, alternatively, in children aged <3 years, pretreatment growth velocity < 7 cm per year and for children aged 3 years and older growth velocity < 4 cm per year measured accurately for at least one year.)
- Diagnostic evaluation excludes other causes associated with short stature that should be observed or treated by other means (e.g. intracranial tumor, neuroendocrine disease, malnutrition, psychosocial deprivation, Down's syndrome, familial short stature, constitutional delay, inflammatory bowel disease, celiac disease, bone dysplasias, chronic disease, hypothyroidism, and epiphyseal closure
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products
SHOX (short stature homeobox-containing gene) deficiency in children
- Shoxdna DxR is a genetic test that detects mutations and deletions in the SHOX gene
- Examination by a pediatric endocrinologist who determines the child's chronological age and stage of pubescence and who performs a complete history, physical examination, and laboratory tests to evaluate the child's growth
- The patient's baseline height must be > 2 standard deviations [SD] below the mean for gender and age (which corresponds to < 2.3 percentile)
- Growth rate is such that the patient is unlikely to attain an adult height in the normal range - 59 inches for girls and 63 inches for boys (i.e. growth velocity below the 25th percentile or, alternatively, in children aged <3 years, pretreatment growth velocity < 7 cm per year and for children aged 3 years and older growth velocity < 4 cm per year measured accurately for at least one year.)
- Diagnostic evaluation excludes other causes associated with short stature that should be observed or treated by other means (e.g. intracranial tumor, neuroendocrine disease, malnutrition, psychosocial deprivation, Down's syndrome, familial short stature, constitutional delay, inflammatory bowel disease, celiac disease, bone dysplasias, chronic disease, hypothyroidism, and epiphyseal closure
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products
Chronic renal failure in pre-transplantation children
- Evaluation and diagnosis by a pediatric endocrinologist
- Glomerular filtration rate (GFR) < 60 ml/min/1.73 m2
- Child's nutritional status has been optimized, metabolic abnormalities have been corrected, and steroid usage has been reduced to a minimum
- The patient's baseline height must be < the 5th percentile for gender and age or 2 or more standard deviations [SD] below the mean measured paternal height
- Growth rate is such that the patient is unlikely to attain an adult height in the normal range - 59 inches for girls and 63 inches for boys (i.e. growth velocity below the 25th percentile or, alternatively, in children aged <3 years, pretreatment growth velocity < 7 cm per year and for children aged 3 years and older growth velocity < 4 cm per year measured accurately for at least one year.)
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products
Prader-Willi syndrome, Turner syndrome, Noonan syndrome
- Turner syndrome confirmed by karyotype or Prader-Willi syndrome or Noonan syndrome confirmed by genetic testing
- Evaluation and diagnosis by a pediatric endocrinologist
- The patient's baseline height must be < the 5th percentile for gender and age or 2 or more standard deviations [SD] below the mean measured paternal height
- Growth rate is such that the patient is unlikely to attain an adult height in the normal range - 59 inches for girls and 63 inches for boys (i.e. growth velocity below the 25th percentile or, alternatively, in children aged < 3 years, pretreatment growth velocity < 7 cm per year and for children aged 3 years and older growth velocity < 4 cm per year measured accurately for at least one year.)
- Diagnostic evaluation excludes other causes associated with short stature that should be observed or treated by other means (e.g. intracranial tumor, neuroendocrine disease, malnutrition, psychosocial deprivation, Down's syndrome, familial short stature, constitutional delay, inflammatory bowel disease, celiac disease, bone dysplasias, chronic disease, hypothyroidism, and epiphyseal closure.)
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products
Therapy of infantile hypoglycemia:
- Spontaneous hypoglycemia and GH level < 10 ng/ml when glucose < 40 mg/dl
- Patient euthyroid** at the time of hypoglycemia or patient has panhypopituitarism
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products
(**If thyroxine is insufficient, then tests of GH secretion should be postponed until the thyroid deficiency is adequately replaced because GH secretion may be subnormal due to hypothyroidism.)
Central nervous system tumor treated with radiation:
- Low IGF-1 or IGFBP-3. Laboratory results indicating the test result and normal ranges required
- The patient's baseline height must be < the 5th percentile for gender and age or 2 or more standard deviations [SD] below the mean measured paternal height
- Growth rate is such that the patient is unlikely to attain an adult height in the normal range - 59 inches for girls and 63 inches for boys (i.e. growth velocity below the 25th percentile or, alternatively, in children aged < 3 years, pretreatment growth velocity < 7 cm per year and for children aged 3 years and older growth velocity < 4 cm per year measured accurately for at least one year.)
- Diagnostic evaluation excludes other causes associated with short stature that should be observed or treated by other means (e.g. neuroendocrine disease, malnutrition, psychosocial deprivation, Down's syndrome, familial short stature, constitutional delay, inflammatory bowel disease, celiac disease, bone dysplasias, chronic disease, hypothyroidism, and epiphyseal closure.)
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products
For continuation of use for the above indications, ANY of the following:
- Increased growth rate by two cm over baseline in first year or one cm over baseline in 6 months for those patients undergoing a 6-month trial
- Continued growth rate exceeds 2.5 cm/year
- For treatment of growth hormone deficiency child's height remains below median adult height (5'10"for males, 5'5" for females) and mid-parental height
- For non-growth hormone deficiency treatment, the child' height continues to be >2.25 standard deviations below the normal adult height for gender (5' 3" for a male, 4' 11" for a female)
ADULTS
Adult growth hormone deficiency, ALL of the following is required:
- Pharmacologic provocation tests (insulin, clonidine, arginine, levodopa, glucagon) with a result <10 ng/ml with RIA (Radioimmunoassay) or <2.5ng/ml with IRMA (Immunoradiometric assay)
- Hypothalmic or pituitary disease
- Significant clinical symptoms related to growth hormone deficiency such as fatigue, poor endurance, poor sense of well being persist despite maximizing treatment of other hormonal disorders, mood disorders, and medical illness
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non preferred products
- If a patient has multiple pituitary hormone deficiencies resulting from structural hypothalamic/pituitary disease, radiation, defined CNS pathology, cranial radiation, trauma, pituitary surgery, or genetic defect affecting the GH axis with low IGF-1 and low IGFBP-3, growth hormone can be approved on a case by case basis
Short-Bowel Syndrome:
- Diagnosis of intestinal failure defined as the continued need for parenteral nutrition 2 years post bowel resection
AND
- Failure of a minimum of four weeks of a high carbohydrate (55%-60%), low fat (20%), low protein (20%) diet, with or without glutamine supplementation, in patients with part of the colon remaining
AND
- Failure or clinically significant adverse effects to Humatrope and Norditropin if requesting non-preferred products