SSR 14-3p: Titles II and XVI: Evaluating Endocrine Disorders Other Than Diabetes Mellitus

I. Endocrine Disorders Other than DM

1. General

   Endocrine glands produce hormones responsible for controlling various physiological functions such as metabolism, blood glucose levels, digestion, electrolyte balance, water balance, and sexual function. The major glands in the endocrine system are pituitary, thyroid, parathyroid, adrenal, pancreas, and gonads (testes and ovaries). The glands release hormones into the bloodstream where they travel to targeted organs. When an endocrine gland produces either too much of a hormone (hyperfunction) or too little of a hormone (hypofunction), the hormonal imbalance can cause an endocrine disorder, resulting in complications affecting various parts of the body. Although many endocrine disorders usually require lifelong treatment, medical advances in the diagnosis and treatment of endocrine disorders have resulted in better management of these disorders in adults and children.

2. Types of Endocrine Disorders other than DM and Their Treatments

   1. Pituitary gland disorders. The pituitary gland, sometimes called the “master gland” of the endocrine system, controls the functions of all other endocrine glands (except for the pancreas).

      1. Hyperpituitarism primarily refers to excess production of growth hormone (GH). Excess GH, which is less common in children, causes an overgrowth of the tissues in the body that are still capable of growing. During childhood, increased production of GH can result in skeletal gigantism. Symptoms and signs of gigantism include bones of excessive length, abnormal bone and body proportions, and delayed epiphyseal fusion. Adults may develop acromegaly, due to increased production of GH. Symptoms and signs of acromegaly include: enlarged bones of the face, jaw, hands and feet; joint pain or swelling; and vision abnormalities. Treatment includes medications that suppress GH production, radiation therapy, and surgery.

      2. Hypopituitarism. Decreased blood levels of GH cause delays in bone and physical growth in children. However, low GH levels are not clinically significant in adults. Deficient production of antidiuretic hormone (ADH) may result in diabetes insipidus with excessive urination producing dehydration and electrolyte imbalance. Generally, GH deficiency is treated with growth hormone replacement (hGH) in children and adolescents. Treatment with ADH replacement medications and hydration generally will successfully control the symptoms and signs of diabetes insipidus within 12 months.

   2. Thyroid gland disorders. The thyroid gland regulates growth and development, body temperature, metabolic processes, heart rate, blood pressure, and mental function.

      1. Hyperthyroidism. Excess production of thyroid hormone may abnormally increase the body's metabolic rate. Symptoms and signs of hyperthyroidism include altered mood, tremors, heart palpitations, hypertension, weight loss, exophthalmos (bulging of the eyes), and goiter (abnormal enlargement of thyroid gland). The most common type of hyperthyroidism is Graves disease, which involves increased secretion of thyroid hormone. Treatment includes lifelong thyroid- suppression medication, radioactive iodine therapy, or surgical removal of the thyroid. Generally, treatment controls the symptoms and signs of hyperthyroidism within 12 months.

      2. Hypothyroidism. Low production of thyroid hormone may result in an abnormally slow metabolic rate. Some symptoms and signs of hypothyroidism include weakness or fatigue, dry or coarse skin, slow or depressed speech, and adverse mental changes. Adequate amounts of thyroid hormone are critical for the developing brain and nervous system in newborns and infants. The most common cause of hypothyroidism is Hashimoto thyroiditis, in which the body's immune system mistakenly attacks the thyroid gland. Treatment with thyroid replacement therapy will generally control the symptoms and signs of hypothyroidism within a few months.

   3. Parathyroid gland disorders. The four parathyroid glands produce parathyroid hormone or parathormone (PTH), which regulates calcium and phosphorus levels in bone, blood, nerves, muscle, and other body tissues.

      1. Hyperparathyroidism. Excess PTH production may cause mildly elevated blood calcium levels (hypercalcemia), which do not always require treatment. Symptoms and signs of hyperparathyroidism include constipation, nausea, vomiting, fatigue, and kidney stones. Hyperparathyroidism may also cause significant depletion of bone calcium, resulting in osteoporosis. Treatment includes surgical removal of the gland(s), which usually resolves the problem.

      2. Hypoparathyroidism. Low production of PTH may cause abnormally low levels of blood calcium and increased levels of blood phosphorus. Symptoms and signs include muscle cramps, tetany (muscle spasms), excessive nervousness, and headaches. Treatment includes lifelong calcium, vitamin D oral supplements, and a diet high in calcium and low in phosphorus. Treatment generally controls this condition.

   4. Adrenal gland disorders. The adrenal glands produce several types of hormones that regulate carbohydrate, protein, and fat metabolism; proper functioning of the immune system; and the body's ability to respond to stress.

      1. Hyperadrenalism. Excess cortisol (for example, Cushing syndrome) or other adrenal hormone adversely affects metabolism and cellular functions, together with cardiovascular and musculoskeletal functions. Excess cortisol is characterized by dysmorphic body changes (for example, centripetal obesity), increased facial and body hair in women, and the reduced ability to fight infections. Treatment of adrenal hormone overproduction may include oral medication, surgery, radiation, or a combination of treatments. Treatment generally controls this condition.

      2. Hypoadrenalism (Addison disease or adrenal insufficiency) is characterized by generalized weakness and fatigue, darkening of skin (hyperpigmentation), muscle wasting, loss of appetite, low blood pressure, electrolyte imbalance, and depression. Treatment requires lifelong supplementation of cortisol and other replacement hormones and medications. Treatment generally controls this condition within 12 months.

   5. Pancreatic disorders. The pancreas produces digestive enzymes and insulin. Digestive enzymes help break down food for the absorption of nutrients by the body.^[4] Insulin is essential to the absorption of glucose from the bloodstream into body cells for conversion into cellular energy. Diabetes mellitus (DM) results from the metabolic changes that occur when blood glucose cannot be transferred into the cells. We describe DM and explain how we evaluate it in SSR 14-2p.

   6. Gonadal disorders. The term “gonads” refers to the testes in males and the ovaries in females. Congenital gonadal dysfunction (also referred to as primary hypogonadism) is frequently associated with chromosomal disorders or other genetic syndromes. Treatment involves lifelong hormone replacement therapy, which usually controls this condition.

II. The Sequential Evaluation Process for Adults

We follow a five-step sequential evaluation process when we make a determination or decision whether an adult is disabled due to an endocrine disorder.^[5]

1. Work Activity

   We determine at step 1 whether an adult with an endocrine disorder is working and, if so, whether the work activity is substantial gainful activity (SGA).^[6] If an adult is engaging in SGA, we will find that he or she is not disabled. If an adult is not engaging in SGA, we go on to step 2 of the sequential evaluation process.

2. Severe Medically Determinable Impairment(s)

   We determine at step 2 whether an adult has a medically determinable impairment (MDI) that is severe. An MDI must be established by medical evidence consisting of signs, symptoms, and laboratory findings, not only by a statement of symptoms. When we evaluate the severity of an endocrine disorder, we consider any symptoms, such as fatigue or pain, that could limit functioning.^[7] If the effects of an endocrine disorder, alone or in combination with another impairment(s), significantly limit an adult's physical or mental ability to do basic work activities, we find that the impairment(s) is severe. We find, however, that the impairment(s) is “not severe” if it has no more than a minimal effect on the adult's ability to do basic work activities. If an adult does not have an MDI that is severe, we will find that he or she is not disabled. If an adult does have a severe impairment(s), we go on to step 3 of the sequential evaluation process.

3. Evaluating the Effects of Endocrine Disorders under Other Body Systems

   We next determine at step 3 whether the impairment(s) meets or medically equals a listing, which also considers the medical severity of your impairment(s). Endocrine disorders are not listed impairments for adults. However, the effects of an endocrine disorder, either alone or in combination with another impairment(s), may meet or medically equal the criteria of a listing in an affected body system(s).^[8] [9] Below are some examples of the effects of specific endocrine disorders and the body systems under which we evaluate them:

   • Effects of hyperpituitarism: Compromised use of the upper or lower extremities due to complications of boney structures, under the musculoskeletal system listings (1.00).

   • Effects of hypopituitarism: ADH deficiency that is untreated or unresponsive to treatment resulting in heart failure or arrhythmia, under the cardiovascular system listings (4.00).

   • Effects of hyperthyroidism: Irregular heartbeat (arrhythmia) or other cardiac dysfunction, under the cardiovascular system listings (4.00); weight loss, under the digestive system listings (5.00); strokes, under the neurological listings (11.00); and mood or anxiety disorders, under the mental disorders listings (12.00).

   • Effects of hyperparathyroidism: Fractures related to osteoporosis, under the musculoskeletal system listings (1.00); PTH-induced hypercalcemia that leads to cataracts, under the special senses and speech listings (2.00); kidney stones, kidney dysfunction, and bone demineralization (osteodystrophy), under the genitourinary impairments listings (6.00); and mood disorders (such as depression) and anxiety disorders, under the mental disorders listings (12.00).

   • Effects of hypoparathyroidism: Cardiac anomalies associated with congenital absence of the parathyroid glands, under the cardiovascular system listings (4.00); muscle spasms (tetany) or convulsions, under the neurological listings (11.00); immune deficiency disorders associated with congenital absence of the parathyroid glands, under the immune system disorders listings (14.00).

   • Effects of hyperadrenalism: Fractures, under the musculoskeletal system listings (1.00); and elevated blood pressure and cardiovascular disease, under the cardiovascular system listings (4.00).

   • Effects of hypoadrenalism: Compromised use of the upper or lower extremities, due to generalized muscle weakness and joint pain, under the musculoskeletal system listings (1.00); weight loss, under the digestive system listings (5.00); and mood disorders including depression, under the mental disorders listings (12.00).

   • Effects of gonadal disorders: Congenital heart disease associated with female hypogonadism, under the cardiovascular system listings (4.00); and learning problems and emotional changes associated with male hypogonadism (for example, Klinefelter syndrome) and female hypogonadism (for example, Turner syndrome), under the mental disorders listings (12.00).

4. Assessing Residual Functional Capacity

   1. When the effects of an endocrine disorder(s), alone or in combination with another impairment(s), are severe but do not meet or medically equal the criteria of a listing, we assess an adult's residual functional capacity (RFC).[10] RFC is the most an adult can do despite his or her limitation(s).

   2. The combined effects of an endocrine disorder and another impairment(s) can be greater than the effects of each of the impairments considered separately. We consider all work-related physical and mental limitations, whether due to an adult's endocrine disorder, other impairment(s), or combination of impairments. For example, some endocrine disorders, such as skeletal gigantism, may cause pain and difficulty walking effectively, due to complications with bone growth. Other endocrine disorders, such as hypothyroidism, may cause severe fatigue because of a hormonal imbalance, limiting an adult's ability to perform work activities on a sustained basis. Limitations in an adult's functioning associated with an endocrine disorder or a combination of impairments may also result from the effects of treatment, such as hormone replacement medications, or complications that persist despite treatment.

   3. We then proceed to step 4 and, if necessary, step 5 of the sequential evaluation process. We use the RFC assessment at step 4 to evaluate whether an adult is capable of performing any past relevant work (PRW) as he or she actually performed it or as the job is generally performed in the national economy. If an adult's RFC precludes the performance of PRW (or if there was no PRW), we use the RFC assessment to make a finding at step 5 about his or her ability to perform other work that exists in significant numbers in the national economy. The usual vocational considerations apply.^[11]

III. The Sequential Evaluation Process for Children

We follow a three-step sequential evaluation process when we make a determination or decision whether a child is disabled due to an endocrine disorder.^{[12] [13]}

1. Work Activity

   We determine at step 1 whether a child is working and, if so, whether the work activity is SGA.^[14] If a child is engaging in SGA, we find that he or she is not disabled. If a child is not engaging in SGA, we go on to step 2 of the sequential evaluation process.

2. Severe Medically Determinable Impairment(s)

   We determine at step 2 whether a child has an MDI that is severe. An MDI must be established by medical evidence consisting of signs, symptoms, and laboratory findings, not only by a statement of symptoms.^[15] When we evaluate severity, we consider the effects of the endocrine disorder on the child's functioning, including: limitations as a result of treatment; and the kinds and extent of help, support, and supervision the child needs compared to that of children the same age who do not have impairments.^[16] If the child's endocrine disorder, alone or in combination with another impairment(s), causes more than minimal functional limitations, we find that the impairment(s) is severe. We find that the impairment(s) is “not severe” if it causes no more than minimal functional limitations. If a child does not have an MDI that is severe, we find that he or she is not disabled. If a child does have a severe impairment(s), we go on to step 3 of the sequential evaluation process.

3. Meets or Medically Equals a Listing, or Functionally Equals the Listings

   1. Evaluating the effects of endocrine disorders under other body systems. Endocrine disorders (except DM for children who have not attained age 6 and who require daily insulin) are not listed impairments for children of any age. However, endocrine disorders may be of listing-level severity because of their effects in other body systems. We determine whether the effects of an endocrine disorder, alone or in combination with another impairment(s), meet or medically equal the criteria of a listing in an affected body system(s).^{[17] [18]} Below are some examples of the effects of specific endocrine disorders and the body systems under which we evaluate them:

      • Effects of hyperpituitarism: Compromised use of the upper or lower extremities due to complications of boney structures, under the musculoskeletal system listings (101.00); and mental status changes due to a child's physical appearance, under the mental disorders listings (112.00).

      • Effects of hypopituitarism: Delayed long bone growth that does not respond to GH replacement treatment, under the musculoskeletal system listings (101.00); and ADH deficiency that is untreated or unresponsive to treatment resulting in heart failure or arrhythmia, under the cardiovascular system listings (104.00).

      • Effects of hyperthyroidism: Irregular heartbeat (arrhythmia) or other cardiac dysfunction, under the cardiovascular system listings (104.00); weight loss, under the digestive system listings (105.00); strokes, under the neurological listings (111.00); and mood or anxiety disorders, under the mental disorders listings (112.00).

      • Effects of hypothyroidism: Growth failure with delayed physical development (children may gain weight yet still have a slowed growth rate) resulting from undiagnosed or inadequately treated hypothyroidism, under the growth impairment listings (100.00); and intellectual disability or other cognitive disorders resulting from inadequately treated hypothyroidism, under the mental disorders listings (112.00).

      • Effects of hyperparathyroidism: Fractures related to osteoporosis, under the musculoskeletal system listings (101.00); PTH-induced hypercalcemia that leads to cataracts, under the special senses and speech listings (102.00); kidney stones, kidney dysfunction, and bone demineralization (osteodystrophy), under the genitourinary impairments listings (106.00); and mood disorders, such as depression and anxiety disorders, under the mental disorders listings (112.00).

      • Effects of hypoparathyroidism: Cardiac anomalies associated with congenital absence of the parathyroid glands, under the cardiovascular system listings (104.00); muscle spasms (tetany) or convulsions, under the neurological listings (111.00); and immune deficiency disorders associated with congenital absence of the parathyroid glands, under the immune system disorders listings (114.00).

      • Effects of hyperadrenalism: Fractures, under the musculoskeletal system listings (101.00); and elevated blood pressure and cardiovascular disease, under the cardiovascular system listings (104.00).

      • Effects of hypoadrenalism: Compromised use of the upper or lower extremities, due to generalized muscle weakness and joint pain, under the musculoskeletal system listings (101.00); weight loss, under the digestive system listings (105.00); and mood disorders including depression, under the mental disorders listings (112.00).

      • Effects of gonadal disorders: Congenital heart disease associated with female hypogonadism, under the cardiovascular system listings (104.00); and learning problems and emotional changes associated with male hypogonadism (for example, Klinefelter syndrome) and female hypogonadism (for example, Turner syndrome), under the mental disorders listings (112.00).

   2. Evaluating the effects of endocrine disorders under functional equivalence. When the effects of a child's endocrine disorder, alone or in combination with another impairment(s), are severe but do not meet or medically equal a listing in any affected body system, we determine whether they result in limitations that functionally equal the listings.^[19] By “functionally equal the listings,” we mean that the child's impairment(s) must be of listing- level severity. In evaluating the effects of a child's endocrine disorder, alone or in combination with another impairment(s), on his or her functioning, we consider what the child cannot do, has difficulty doing, needs help doing, or is restricted from doing because of his or her impairment(s). We must explain any limitation in a child's ability to function age-appropriately on the basis of an MDI(s).^[20]

      To functionally equal the listings, an impairment(s) must be of listing-level severity; that is, it must result in “marked” limitations in two domains of functioning or an “extreme” limitation in one domain of functioning. Domains are broad areas of functioning intended to capture all of what a child can or cannot do.

      When we determine whether a child's impairment(s) functionally equals the listings, we use the following six domains:

      • Acquiring and using information;

      • Attending and completing tasks;

      • Interacting and relating with others;

      • Moving about and manipulating objects;

      • Caring for yourself; and

      • Health and physical well-being.

      When we evaluate a child's functioning in these six domains, we consider how the child functions compared to children the same age who do not have impairments. The first five domains describe the abilities a child uses to develop the skills that he or she uses to function in day- to-day activities. In domain six, we consider the cumulative physical effects of physical and mental impairments and their associated treatments on a child's health and functioning. This domain does not address typical development and functioning. Rather, it addresses how such things as recurrent illness, the side effects of medication, and the need for ongoing treatment affect a child's body; that is, the child's health and physical well-being.

      An endocrine disorder, alone or in combination with another impairment(s), may affect a child's functioning in any domain. We evaluate each child's limitations by considering all relevant information from acceptable medical sources (for example, a pediatrician or psychologist), other medical sources (for example, a physical or occupational therapist), and non-medical sources such as parents, teachers, and other people who know the child.^{[21] [22]} We also consider factors such as the:

      • Kinds and extent of help, support, and supervision a child with an endocrine disorder needs that exceed what a child the same age would typically need; and

      • Effects of medications and other treatments, including adverse and beneficial effects.

      Some children with inadequately treated hypothyroidism (or hyperthyroidism), for example, may have limitations in Attending and completing tasks due to their difficulty listening to the teacher, maintaining focus, staying on task in the classroom, or excessive fatigue. Other children with panhypopituitarism after surgery for craniopharyngioma (a benign tumor that develops near the pituitary gland), for example, may experience partial loss of visual fields in both eyes (hemianopsia) and, therefore, have difficulty Moving about and manipulating objects.

      Some adolescents with inadequately treated growth hormone deficiency may appear many years younger than their chronological age. These children may have difficulty Interacting and relating with others due to their physical appearance, or may experience difficulty shopping or getting an afterschool job because of being mistakenly perceived as a much younger child.

      The effects of an endocrine disorder may differ from child to child. We evaluate the effects of a child's endocrine disorder, alone or in combination with another impairment(s), including the effects of medication or other treatment, in all relevant domains. When considering the functioning of a child with an endocrine disorder, we use the “whole child” approach to evaluate the particular effects of the endocrine disorder on a child's activities in any and all of the domains that the child uses to do those activities, based on the evidence in the case record. We find a child disabled if the effects of his or her endocrine disorder, alone or in combination with another impairment(s), result in “marked” limitations in two domains of functioning or an “extreme” limitation in one domain of functioning.