DI 34001.014 Respiratory Disorders

3.00 Respiratory Disorders (Effective Date: 10/07/16)

A. Which disorders do we evaluate in this body system?

1. We evaluate respiratory disorders that result in obstruction (difficulty moving air out of the lungs) or restriction (difficulty moving air into the lungs), or that interfere with diffusion (gas exchange) across cell membranes in the lungs. Examples of such disorders and the listings we use to evaluate them include chronic obstructive pulmonary disease (chronic bronchitis and emphysema, 3.02), pulmonary fibrosis and pneumoconiosis (3.02), asthma (3.02 or 3.03), cystic fibrosis (3.04), and bronchiectasis (3.02 or 3.07). We also use listings in this body system to evaluate respiratory failure (3.04D or 3.14), chronic pulmonary hypertension (3.09), and lung transplantation (3.11).

2. We evaluate cancers affecting the respiratory system under the listings in 13.00. We evaluate the pulmonary effects of neuromuscular and autoimmune disorders under these listings or under the listings in 11.00 or 14.00, respectively.

B. What are the symptoms and signs of respiratory disorders? Symptoms and signs of respiratory disorders include dyspnea (shortness of breath), chest pain, coughing, wheezing, sputum production, hemoptysis (coughing up blood from the respiratory tract), use of accessory muscles of respiration, and tachypnea (rapid rate of breathing).

C. What abbreviations do we use in this body system?

1. ABG means arterial blood gas.

2. BiPAP means bi-level positive airway pressure ventilation.

3. BTPS means body temperature and ambient pressure, saturated with water vapor.

4. CF means cystic fibrosis.

5. CFRD means CF-related diabetes.

6. CFTR means CF transmembrane conductance regulator.

7. CO means carbon monoxide.

8. COPD means chronic obstructive pulmonary disease.

9. DLCO means diffusing capacity of the lungs for carbon monoxide.

10. FEV ₁ means forced expiratory volume in the first second of a forced expiratory maneuver.

11. FVC means forced vital capacity.

12. L means liter.

13. mL CO (STPD)/min/mmHg means milliliters of carbon monoxide at standard temperature and pressure, dry, per minute, per millimeter of mercury.

14. P _a O ₂ means arterial blood partial pressure of oxygen.

15. P _a CO ₂ means arterial blood partial pressure of carbon dioxide.

16. S _p O ₂ means percentage of oxygen saturation of blood hemoglobin measured by pulse oximetry.

17. 6MWT means 6-minute walk test.

18. VI means volume of inhaled gas during a DLCO test.

D. What documentation do we need to evaluate your respiratory disorder?

1. We need medical evidence to document and assess the severity of your respiratory disorder. Medical evidence should include your medical history, physical examination findings, the results of imaging (see 3.00D3), pulmonary function tests (see 3.00D4), other relevant laboratory tests, and descriptions of any prescribed treatment and your response to it. We may not need all of this evidence depending on your particular respiratory disorder and its effects on you.

2. If you use supplemental oxygen, we still need medical evidence to establish the severity of your respiratory disorder.

3. Imaging refers to medical imaging techniques, such as x-ray and computerized tomography. The imaging must be consistent with the prevailing state of medical knowledge and clinical practice as the proper technique to support the evaluation of the disorder.

4. Pulmonary function tests include spirometry (which measures ventilation of the lungs), DLCO tests (which measure gas diffusion in the lungs), ABG tests (which measure the partial pressure of oxygen, P _a O ₂ , and carbon dioxide, P _a CO ₂ , in the arterial blood), and pulse oximetry (which measures oxygen saturation, S _p O ₂ , of peripheral blood hemoglobin).

E. What is spirometry and what are our requirements for an acceptable test and report?

1. Spirometry, which measures how well you move air into and out of your lungs, involves at least three forced expiratory maneuvers during the same test session. A forced expiratory maneuver is a maximum inhalation followed by a forced maximum exhalation, and measures exhaled volumes of air over time. The volume of air you exhale in the first second of the forced expiratory maneuver is the FEV₁. The total volume of air that you exhale during the entire forced expiratory maneuver is the FVC. We use your highest FEV₁ value to evaluate your respiratory disorder under 3.02A, 3.03A, and 3.04A, and your highest FVC value to evaluate your respiratory disorder under 3.02B, regardless of whether the values are from the same forced expiratory maneuver or different forced expiratory maneuvers.

2. We have the following requirements for spirometry under these listings:

a. You must be medically stable at the time of the test. Examples of when we would not consider you to be medically stable include when you are:

(i) Within 2 weeks of a change in your prescribed respiratory medication.

(ii) Experiencing, or within 30 days of completion of treatment for, a lower respiratory tract infection.

(iii) Experiencing, or within 30 days of completion of treatment for, an acute exacerbation (temporary worsening) of a chronic respiratory disorder. Wheezing by itself does not indicate that you are not medically stable.

(iv) Hospitalized, or within 30 days of a hospital discharge, for an acute myocardial infarction (heart attack).

b. During testing, if your FEV₁ is less than 70 percent of your predicted normal value, we require repeat spirometry after inhalation of a bronchodilator to evaluate your respiratory disorder under these listings, unless it is medically contraindicated. If you used a bronchodilator before the test and your FEV₁ is less than 70 percent of your predicted normal value, we still require repeat spirometry after inhalation of a bronchodilator unless the supervising physician determines that it is not safe for you to take a bronchodilator again (in which case we may need to reschedule the test). If you do not have post-bronchodilator spirometry, the test report must explain why. We can use the results of spirometry administered without bronchodilators when the use of bronchodilators is medically contraindicated.

c. Your forced expiratory maneuvers must be satisfactory. We consider a forced expiratory maneuver to be satisfactory when you exhale with maximum effort following a full inspiration, and when the test tracing has a sharp takeoff and rapid rise to peak flow, has a smooth contour, and either lasts for at least 6 seconds or maintains a plateau for at least 1 second.

3. The spirometry report must include the following information:

a. The date of the test and your name, age or date of birth, sex, and height without shoes. (We will assume that your recorded height on the date of the test is without shoes, unless we have evidence to the contrary.) If your spine is abnormally curved (for example, you have kyphoscoliosis), we will substitute the longest distance between your outstretched fingertips with your arms abducted 90 degrees in place of your height when this measurement is greater than your standing height without shoes.

b. Any factors, if applicable, that can affect the interpretation of the test results (for example, your cooperation or effort in doing the test).

c. Legible tracings of your forced expiratory maneuvers in a volume-time format showing your name and the date of the test for each maneuver.

4. If we purchase spirometry, the medical source we designate to administer the test is solely responsible for deciding whether it is safe for you to do the test and for how to administer it.

F. What is a DLCO test, and what are our requirements for an acceptable test and report?

1. A DLCO test measures the gas exchange across cell membranes in your lungs. It measures how well CO diffuses from the alveoli (air sacs) of your lungs into your blood. DLCO may be severely reduced in some disorders, such as interstitial lung disease (for example, idiopathic pulmonary fibrosis, asbestosis, and sarcoidosis) and COPD (particularly emphysema), even when the results of spirometry are not significantly reduced. We use the average of two of your unadjusted (that is, uncorrected for hemoglobin concentration) DLCO measurements reported in mL CO (STPD)/min/mmHg to evaluate your respiratory disorder under 3.02C1.

2. We have the following requirements for DLCO tests under these listings:

a. You must be medically stable at the time of the test. See 3.00E2a.

b. The test must use the single-breath technique.

(i) The VI during the DLCO maneuver must be at least 85 percent of your current FVC, and your time of inhalation must be less than 4 seconds. (See 3.00E for our rules for programmatically acceptable spirometry.) If you do not have an FVC measurement on the same day as the DLCO test, we may use your FVC from programmatically acceptable spirometry administered within 90 days of the DLCO test.

(ii) Your breath-hold time must be between 8 and 12 seconds.

(iii) Your total exhalation time must be less than or equal to 4 seconds, with a sample collection time of less than 3 seconds. If your FVC is at least 2.0 L, the washout volume must be between 0.75 L and 1.0 L. If your FVC is less than 2.0 L, the washout volume must be at least 0.5 L.

3. The DLCO test report must include the following information:

a. The date of the test and your name, age or date of birth, sex, and height without shoes. (We will assume that your recorded height on the date of the test is without shoes, unless we have evidence to the contrary.) If your spine is abnormally curved (for example, you have kyphoscoliosis), we will substitute the longest distance between your outstretched fingertips with your arms abducted 90 degrees in place of your height when this measurement is greater than your standing height without shoes.

b. Any factors, if applicable, that can affect the interpretation of the test results (for example, your cooperation or effort in doing the test).

c. Legible tracings of your VI, breath-hold maneuver, and volume of exhaled gas showing your name and the date of the test for each DLCO maneuver.

d. At least two acceptable (see 3.00F2) DLCO measurements within 3 mL CO (STPD)/min/mmHg of each other or within 10 percent of the highest value.

4. We may need to purchase a DLCO test to determine whether your disorder meets 3.02C1 when we have evidence showing that you have a chronic respiratory disorder that could result in impaired gas exchange, unless we can make a fully favorable determination or decision on another basis. Since the DLCO calculation requires a current FVC measurement, we may also purchase spirometry at the same time as the DLCO test, even if we already have programmatically acceptable spirometry.

5. Before we purchase a DLCO test, a medical consultant (see §§ 404.1616 and 416.1016 of this chapter), preferably one with experience in the care of people with respiratory disorders, must review your case record to determine if we need the test. The medical source we designate to administer the test is solely responsible for deciding whether it is safe for you to do the test and for how to administer it.

G. What is an ABG test, and what are our requirements for an acceptable test and report?

1. General. An ABG test measures P_aO₂, P_aCO₂, and the concentration of hydrogen ions in your arterial blood. We use a resting or an exercise ABG measurement to evaluate your respiratory disorder under 3.02C2.

2. Resting ABG tests.

a. We have the following requirements for resting ABG tests under these listings:

(i) You must be medically stable at the time of the test. See 3.00E2a.

(ii) The test must be administered while you are breathing room air; that is, without oxygen supplementation.

b. The resting ABG test report must include the following information:

(i) Your name, the date of the test, and either the altitude or both the city and State of the test site.

(ii) The P_aO₂ and P_aCO₂ values.

c. We may need to purchase a resting ABG test to determine whether your disorder meets 3.02C2 when we have evidence showing that you have a chronic respiratory disorder that could result in impaired gas exchange, unless we can make a fully favorable determination or decision on another basis.

d. Before we purchase a resting ABG test, a medical consultant (see §§ 404.1616 and 416.1016 of this chapter), preferably one with experience in the care of people with respiratory disorders, must review your case record to determine if we need the test. The medical source we designate to administer the test is solely responsible for deciding whether it is safe for you to do the test and for how to administer it.

3. Exercise ABG tests.

a. We will not purchase an exercise ABG test.

b. We have the following requirements for exercise ABG tests under these listings:

(i) You must have done the exercise under steady state conditions while breathing room air. If you were tested on a treadmill, you generally must have exercised for at least 4 minutes at a grade and speed providing oxygen (O₂) consumption of approximately 17.5 milliliters per kilogram per minute (mL/kg/min) or 5.0 metabolic equivalents (METs). If you were tested on a cycle ergometer, you generally must have exercised for at least 4 minutes at an exercise equivalent of 5.0 METs.

(ii) We may use a test in which you have not exercised for at least 4 minutes. If you were unable to complete at least 4 minutes of steady state exercise, we need a statement by the person administering the test about whether the results are a valid indication of your respiratory status. For example, this statement may include information about your cooperation or effort in doing the test and whether you were limited in completing the test because of your respiratory disorder or another impairment.

c. The exercise ABG test report must include the following information:

(i) Your name, the date of the test, and either the altitude or both the city and state of the test site.

(ii) The P_aO₂ and P_aCO₂ values.

H. What is pulse oximetry, and what are our requirements for an acceptable test and report?

1. Pulse oximetry measures S_pO₂, the percentage of oxygen saturation of blood hemoglobin. We use a pulse oximetry measurement (either at rest, during a 6MWT, or after a 6MWT) to evaluate your respiratory disorder under 3.02C3 or, if you have CF, to evaluate it under 3.04F.

2. We have the following requirements for pulse oximetry under 3.02C3:

a. You must be medically stable at the time of the test. See 3.00E2a.

b. Your pulse oximetry measurement must be recorded while you are breathing room air; that is, without oxygen supplementation.

c. Your pulse oximetry measurement must be stable. By "stable," we mean that the range of S_pO₂ values (that is, lowest to highest) during any 15-second interval cannot exceed 2 percentage points. For example: (1) the measurement is stable if the lowest S_pO₂ value during a 15-second interval is 87 percent and the highest value is 89 percent − a range of 2 percentage points. (2) The measurement is not stable if the lowest value is 86 percent and the highest value is 89 percent − a range of 3 percentage points.

d. If you have had more than one measurement (for example, at rest and after a 6MWT), we will use the measurement with the lowest S_pO₂ value.

e. The pulse oximetry report must include the following information:

(i) Your name, the date of the test, and either the altitude or both the city and State of the test site.

(ii) A graphical printout showing your S_pO₂ value and a concurrent, acceptable pulse wave. An acceptable pulse wave is one that shows the characteristic pulse wave; that is, sawtooth-shaped with a rapid systolic upstroke (nearly vertical) followed by a slower diastolic downstroke (angled downward).

f. We may need to purchase pulse oximetry at rest to determine whether your disorder meets 3.02C3 when we have evidence showing that you have a chronic respiratory disorder that could result in impaired gas exchange, unless we can make a fully favorable determination or decision on another basis. We may purchase pulse oximetry during and after a 6MWT if your S_pO₂ value at rest is greater than the value in Table V.

g. Before we purchase pulse oximetry, a medical consultant (see §§ 404.1616 and 416.1016 of this chapter), preferably one with experience in the care of people with respiratory disorders, must review your case record to determine if we need the test. The medical source we designate to administer the test is solely responsible for deciding whether it is safe for you to do the test and for how to administer it.

3. We have the following requirements for pulse oximetry under 3.04F:

a. You must be medically stable at the time of the test. See 3.00E2a.

b. Your pulse oximetry measurement must be recorded while you are breathing room air; that is, without oxygen supplementation.

c. If you have had more than one measurement (for example, at rest and after a 6MWT), we will use the measurement with the lowest S_pO₂ value.

d. The pulse oximetry report must include your name, the date of the test, and either the altitude or both the city and State of the test site. If you have CF, we do not require a graphical printout showing your S_pO₂ value and a concurrent, acceptable pulse wave.

I. What is asthma and how do we evaluate it?

1. Asthma is a chronic inflammatory disorder of the lung airways that we evaluate under 3.02 or 3.03. If you have respiratory failure resulting from chronic asthma (see 3.00N), we will evaluate it under 3.14.

2. For the purposes of 3.03:

a. We need evidence showing that you have listing-level (see Table VI in 3.03A) airflow obstruction at baseline while you are medically stable.

b. The phrase "consider under a disability for 1 year" in 3.03B does not refer to the date on which your disability began, only to the date on which we must reevaluate whether your asthma continues to meet a listing or is otherwise disabling.

c. We determine the onset of your disability based on the facts of your case, but it will be no later than the admission date of your first of three hospitalizations that satisfy the criteria of 3.03B.

J. What is CF and how do we evaluate it?

1. General. We evaluate CF, a genetic disorder that results in abnormal salt and water transport across cell membranes in the lungs, pancreas, and other body organs, under 3.04. We need the evidence described in 3.00J2 to establish that you have CF.

2. Documentation of CF. We need a report signed by a physician (see §§ 404.1513(a) and 416.913(a) of this chapter) showing both a and b:

a. One of the following:

(i) A positive newborn screen for CF; or

(ii) A history of CF in a sibling; or

(iii) Documentation of at least one specific CF phenotype or clinical criterion (for example, chronic sino-pulmonary disease with persistent colonization or infections with typical CF pathogens, pancreatic insufficiency, or salt-loss syndromes); and

b. One of the following definitive laboratory tests:

(i) An elevated sweat chloride concentration equal to or greater than 60 millimoles per L; or

(ii) The identification of two CF gene mutations affecting the CFTR; or

(iii) Characteristic abnormalities in ion transport across the nasal epithelium.

c. When we have the report showing a and b, but it is not signed by a physician, we also need a report from a physician stating that you have CF.

d. When we do not have the report showing a and b, we need a report from a physician that is persuasive that a positive diagnosis of CF was confirmed by an appropriate definitive laboratory test. To be persuasive, this report must include a statement by the physician that you had the appropriate definitive laboratory test for diagnosing CF. The report must provide the test results or explain how your diagnosis was established that is consistent with the prevailing state of medical knowledge and clinical practice.

3. CF pulmonary exacerbations. Examples of CF pulmonary exacerbations include increased cough and sputum production, hemoptysis, increased shortness of breath, increased fatigue, and reduction in pulmonary function. Treatment usually includes intravenous antibiotics and intensified airway clearance therapy (for example, increased frequencies of chest percussion or increased use of inhaled nebulized therapies, such as bronchodilators or mucolytics).

4. For 3.04G, we require any two exacerbations or complications from the list in 3.04G1 through 3.04G4 within a 12-month period. You may have two of the same exacerbation or complication or two different ones.

a. If you have two of the acute exacerbations or complications we describe in 3.04G1 and 3.04G2, there must be at least 30 days between the two.

b. If you have one of the acute exacerbations or complications we describe in 3.04G1 and 3.04G2 and one of the chronic complications we describe in 3.04G3 and 3.04G4, the two can occur during the same time. For example, your CF meets 3.04G if you have the pulmonary hemorrhage we describe in 3.04G2 and the weight loss we describe in 3.04G3 even if the pulmonary hemorrhage occurs during the 90-day period in 3.04G3.

c. Your CF also meets 3.04G if you have both of the chronic complications in 3.04G3 and 3.04G4.

5. CF may also affect other body systems such as digestive or endocrine. If your CF, including pulmonary exacerbations and nonpulmonary complications, does not meet or medically equal a respiratory disorders listing, we may evaluate your CF-related impairments under the listings in the affected body system.

K. What is bronchiectasis and how do we evaluate it? Bronchiectasis is a chronic respiratory disorder that is characterized by abnormal and irreversible dilatation (enlargement) of the airways below the trachea, which may be associated with the accumulation of mucus, bacterial infections, and eventual airway scarring. We require imaging (see 3.00D3) to document this disorder. We evaluate your bronchiectasis under 3.02, or under 3.07 if you are having exacerbations or complications (for example, acute bacterial infections, increased shortness of breath, or coughing up blood) that require hospitalization.

L. What is chronic pulmonary hypertension and how do we evaluate it?

1. Chronic pulmonary hypertension is an increase in the blood pressure of the blood vessels of the lungs. If pulmonary hypertension is not adequately treated, it can eventually result in right heart failure. We evaluate chronic pulmonary hypertension due to any cause under 3.09.

2. Chronic pulmonary hypertension is usually diagnosed by catheterization of the pulmonary artery. We will not purchase cardiac catheterization.

M. How do we evaluate lung transplantation? If you receive a lung transplant (or a lung transplant simultaneously with other organs, such as the heart), we will consider you to be disabled under 3.11 for 3 years from the date of the transplant. After that, we evaluate your residual impairment(s) by considering the adequacy of your post-transplant function, the frequency and severity of any rejection episodes you have, complications in other body systems, and adverse treatment effects. People who receive organ transplants generally have impairments that meet our definition of disability before they undergo transplantation. The phrase "consider under a disability for 3 years" in 3.11 does not refer to the date on which your disability began, only to the date on which we must reevaluate whether your impairment(s) continues to meet a listing or is otherwise disabling. We determine the onset of your disability based on the facts of your case.

N. What is respiratory failure and how do we evaluate it? Respiratory failure is the inability of the lungs to perform their basic function of gas exchange. We evaluate respiratory failure under 3.04D if you have CF-related respiratory failure, or under 3.14 if you have respiratory failure due to any other chronic respiratory disorder. Continuous positive airway pressure does not satisfy the criterion in 3.04D or 3.14, and cannot be substituted as an equivalent finding, for invasive mechanical ventilation or noninvasive ventilation with BiPAP.

O. How do we consider the effects of obesity when we evaluate your respiratory disorder? Obesity is a medically determinable impairment that is often associated with respiratory disorders. Obesity makes it harder for the chest and lungs to expand, which can compromise the ability of the respiratory system to supply adequate oxygen to the body. The combined effects of obesity with a respiratory disorder can be greater than the effects of each of the impairments considered separately. We consider any additional and cumulative effects of your obesity when we determine whether you have a severe respiratory disorder, a listing-level respiratory disorder, a combination of impairments that medically equals the severity of a listed impairment, and when we assess your residual functional capacity.

P. What are sleep-related breathing disorders and how do we evaluate them?

1. Sleep-related breathing disorders (for example, sleep apnea) are characterized by transient episodes of interrupted breathing during sleep, which disrupt normal sleep patterns. Prolonged episodes can result in disorders such as hypoxemia (low blood oxygen) and pulmonary vasoconstriction (restricted blood flow in pulmonary blood vessels). Over time, these disorders may lead to chronic pulmonary hypertension or other complications.

2. We evaluate the complications of sleep-related breathing disorders under the listings in the affected body system(s). For example, we evaluate chronic pulmonary hypertension due to any cause under 3.09; chronic heart failure under 4.02; and disturbances in mood, cognition, and behavior under 12.02 or another appropriate mental disorders listing. We will not purchase polysomnography (sleep study).

Q. How do we evaluate mycobacterial, mycotic, and other chronic infections of the lungs? We evaluate chronic infections of the lungs that result in limitations in your respiratory function under 3.02.

R. How do we evaluate respiratory disorders that do not meet one of these listings?

1. These listings are only examples of common respiratory disorders that we consider severe enough to prevent you from doing any gainful activity. If your impairment(s) does not meet the criteria of any of these listings, we must also consider whether you have an impairment(s) that meets the criteria of a listing in another body system. For example, if your CF has resulted in chronic pancreatic or hepatobiliary disease, we evaluate your impairment under the listings in 5.00.

2. If you have a severe medically determinable impairment(s) that does not meet a listing, we will determine whether your impairment(s) medically equals a listing. See §§ 404.1526 and 416.926 of this chapter. Respiratory disorders may be associated with disorders in other body systems, and we consider the combined effects of multiple impairments when we determine whether they medically equal a listing. If your impairment(s) does not meet or medically equal a listing, you may or may not have the residual functional capacity to engage in substantial gainful activity. We proceed to the fourth step and, if necessary, the fifth step of the sequential evaluation process in §§ 404.1520 and 416.920 of this chapter. We use the rules in §§ 404.1594 and 416.994 of this chapter, as appropriate, when we decide whether you continue to be disabled.

3.01 Category of Impairments, Respiratory Disorders

3.02 Chronic respiratory disorders due to any cause except CF (for CF, see 3.04) with A, B, C, or D:

A. FEV₁ (see 3.00E) less than or equal to the value in Table I-A or I-B for your age, sex, and height without shoes (see 3.00E3a).

OR

B. FVC (see 3.00E) less than or equal to the value in Table II-A or II-B for your age, sex, and height without shoes (see 3.00E3a).

OR

C. Chronic impairment of gas exchange demonstrated by 1, 2, or 3:

1. Average of two unadjusted, single-breath DLCO measurements (see 3.00F) less than or equal to the value in Table III for your sex and height without shoes (see 3.00F3a); or

2. Arterial P_aO₂ and P_aCO₂ measured concurrently by an ABG test, while at rest or during steady state exercise, breathing room air (see 3.00G3b), less than or equal to the applicable values in Table IV-A, IV-B, or IV-C; or

Tables IV-A, IV-B, and IV-C: ABG Criteria for 3.02C2

3. S_pO₂ measured by pulse oximetry (see 3.00H2) either at rest, during a 6MWT, or after a 6MWT, less than or equal to the value in Table V.

OR

D. Exacerbations or complications requiring three hospitalizations within a 12-month period and at least 30 days apart (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review). Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization.

3.03 Asthma (see 3.00I), with both A and B:

A. FEV₁ (see 3.00E1) less than or equal to the value in Table VI-A or VI-B for your age, sex, and height without shoes (see 3.00E3a) measured within the same 12-month period as the hospitalizations in 3.03B.

AND

B. Exacerbations or complications requiring three hospitalizations within a 12-month period and at least 30 days apart (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review). Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization. Consider under a disability for 1 year from the discharge date of the last hospitalization; after that, evaluate the residual impairment(s) under 3.03 or another appropriate listing.

3.04 Cystic fibrosis (documented as described in 3.00J2) with A, B, C, D, E, F, or G:

A. FEV₁ (see 3.00E) less than or equal to the value in Table VII-A or VII-B for your age, sex, and height without shoes (see 3.00E3a).

OR

B. Exacerbations or complications (see 3.00J3) requiring three hospitalizations of any length within a 12-month period and at least 30 days apart (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review).

OR

C. Spontaneous pneumothorax, secondary to CF, requiring chest tube placement.

OR

D. Respiratory failure (see 3.00N) requiring invasive mechanical ventilation, noninvasive ventilation with BiPAP, or a combination of both treatments, for a continuous period of at least 48 hours, or for a continuous period of at least 72 hours if postoperatively.

OR

E. Pulmonary hemorrhage requiring vascular embolization to control bleeding.

OR

F. S_pO₂ measured by pulse oximetry (see 3.00H3) either at rest, during a 6MWT, or after a 6MWT, less than or equal to the value in Table VIII, twice within a 12-month period and at least 30 days apart (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review).

 

OR

G. Two of the following exacerbations or complications (either two of the same or two different, see 3.00J3 and 3.00J4) within a 12-month period (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review):

1. Pulmonary exacerbation requiring 10 consecutive days of intravenous antibiotic treatment.

2. Pulmonary hemorrhage (hemoptysis with more than blood-streaked sputum but not requiring vascular embolization) requiring hospitalization of any length.

3. Weight loss requiring daily supplemental enteral nutrition via a gastrostomy for at least 90 consecutive days or parenteral nutrition via a central venous catheter for at least 90 consecutive days.

4. CFRD requiring daily insulin therapy for at least 90 consecutive days.

3.05 [Reserved]

3.06 [Reserved]

3.07 Bronchiectasis (see 3.00K), documented by imaging (see 3.00D3), with exacerbations or complications requiring three hospitalizations within a 12-month period and at least 30 days apart (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review). Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization.

3.08 [Reserved]

3.09 Chronic pulmonary hypertension due to any cause (see 3.00L) documented by mean pulmonary artery pressure equal to or greater than 40 mm Hg as determined by cardiac catheterization while medically stable (see 3.00E2a).

3.10 [Reserved]

3.11 Lung transplantation (see 3.00M). Consider under a disability for 3 years from the date of the transplant; after that, evaluate the residual impairment(s).

3.12 [Reserved]

3.13 [Reserved]

3.14 Respiratory failure (see 3.00N) resulting from any underlying chronic respiratory disorder except CF (for CF, see 3.04D), requiring invasive mechanical ventilation, noninvasive ventilation with BiPAP, or a combination of both treatments, for a continuous period of at least 48 hours, or for a continuous period of at least 72 hours if postoperatively, twice within a 12-month period and at least 30 days apart (the 12-month period must occur within the period we are considering in connection with your application or continuing disability review).

DI 34001.018 Digestive Disorders

5.00 DIGESTIVE DISORDERS (Effective Date: 10/06/23)

A. Which digestive disorders do we evaluate in this body system? We evaluate digestive disorders that result in severe dysfunction of the liver, pancreas, and gastrointestinal tract (the large, muscular tube that extends from the mouth to the anus, where the movement of muscles, along with the release of hormones and enzymes, allows for the digestion of food) in this body system. Examples of these disorders and the listings we use to evaluate them include chronic liver disease (5.05), inflammatory bowel disease (5.06), and intestinal failure (5.07). We also use this body system to evaluate gastrointestinal hemorrhaging from any cause (5.02), weight loss due to any digestive disorder (5.08), liver transplantation (5.09), small intestine transplantation (5.11), and pancreas transplantation (5.12). We evaluate cancers affecting the digestive system under the listings in 13.00.

B. What evidence do we need to evaluate your digestive disorder?

1. General. To establish that you have a digestive disorder, we need medical evidence about the existence of your digestive disorder and its severity. Medical evidence should include your medical history, physical examination findings, operative reports, and relevant laboratory findings.

2. Laboratory findings. We need laboratory reports such as results of imaging (see 5.00B3), endoscopy, and other diagnostic procedures. We may also need clinical laboratory and pathology results.

3. Imaging refers to medical imaging techniques, such as x-ray, ultrasound, magnetic resonance imaging, and computerized tomography. The imaging must be consistent with the prevailing state of medical knowledge and clinical practice as a proper technique to support the evaluation of the disorder.

 

C. What is chronic liver disease (CLD), and how do we evaluate it under 5.05?

1. General. CLD is loss of liver function with cell necrosis (cell death), inflammation, or scarring of the liver that persists for more than 6 months. Common causes of CLD in adults include chronic infection with hepatitis B virus or hepatitis C virus, and prolonged alcohol abuse.

a. We will evaluate your signs of CLD, such as jaundice, changes in size of the liver and spleen, ascites, peripheral edema, and altered mental status. We will also evaluate your symptoms of CLD, such as pruritus (itching), fatigue, nausea, loss of appetite, and sleep disturbances when we assess the severity of your impairment(s) and how it affects your ability to function. In the absence of evidence of a chronic liver impairment, episodes of acute liver disease do not meet the requirements of 5.05.

b. Laboratory findings of your CLD may include decreased serum albumin, increased International Normalized Ratio (INR), arterial deoxygenation (hypoxemia), increased serum creatinine, oliguria (reduced urine output), or sodium retention. Another laboratory finding that may be included in the evidence is a liver biopsy. If you have had a liver biopsy, we will make every reasonable effort to obtain the results; however, we will not purchase a liver biopsy.

2. Manifestations of CLD.

a. Gastrointestinal hemorrhaging (5.05A), as a consequence of cirrhosis and high pressure in the liver's portal venous system, may occur from varices (dilated veins in the esophagus or the stomach) or from portal hypertensive gastropathy (abnormal mucosal changes in the stomach). When gastrointestinal hemorrhaging is due to a cause other than CLD, we evaluate it under 5.02. The phrase "consider under a disability for 1 year" in 5.02 and 5.05A does not refer to the date on which your disability began, only to the date on which we must reevaluate whether your impairment(s) continues to meet a listing or is otherwise disabling. We determine the onset of your disability based on the facts of your case.

b. Ascites or hydrothorax (5.05B) is a pathologic accumulation of fluid in the peritoneal cavity (ascites) or pleural space (hydrothorax). Ascites or hydrothorax may be diagnosed by removing some of the fluid with needle aspiration (paracentesis or thoracentesis), physical examination, or imaging. The most common causes of ascites are portal hypertension and low serum albumin resulting from CLD. We evaluate other causes of ascites and hydrothorax that are unrelated to CLD, such as congestive heart failure and cancer, under the listings in the affected body systems.

c. Spontaneous bacterial peritonitis (SBP) (5.05C) is an acute bacterial infection of peritoneal fluid and is most commonly associated with CLD. SBP is diagnosed by laboratory analysis of peritoneal fluid (obtained by paracentesis) that contains a neutrophil count (also called absolute neutrophil count) of at least 250 cells/mm³. 5.05C is satisfied with one evaluation documenting peritoneal infection. We evaluate other causes of peritonitis that are unrelated to CLD, such as tuberculosis, malignancy, and perforated bowel, under the listings in the affected body systems.

d. Hepatorenal syndrome (5.05D) is renal failure associated with CLD in the absence of underlying kidney pathology. Findings associated with hepatorenal syndrome include elevation of serum creatinine, sodium retention with low urinary sodium excretion, and oliguria. We evaluate renal dysfunction with known underlying kidney pathology, such as glomerulonephritis, tubular necrosis, and renal infections, under the listings in 6.00.

e. Hepatopulmonary syndrome (5.05E) is arterial deoxygenation due to intrapulmonary vascular dilation and arteriovenous shunting associated with CLD. Clinical findings of hepatopulmonary syndrome include platypnea (shortness of breath relieved when lying down) and orthodeoxia (low arterial blood oxygen while in the upright position), when presenting in the context of CLD. We evaluate pulmonary dysfunction with known underlying respiratory pathology, such as asthma, pneumonia, and pulmonary infections, under the listings in 3.00.

(i) Under 5.05E1, we require a resting arterial blood gas (ABG) measurement obtained while you are breathing room air; that is, without oxygen supplementation. The ABG report must include the P_aO₂ value, your name, the date of the test, and either the altitude or both the city and State of the test site.

(ii) We will not purchase the specialized imaging techniques described in 5.05E2; however, if you have had the test(s) at a time relevant to your claim, we will make every reasonable effort to obtain the report.

f. Hepatic encephalopathy (5.05F), also known as portosystemic encephalopathy, is a recurrent or chronic neuropsychiatric disorder associated with CLD.

(i) Under 5.05F2, we require documentation of a mental impairment associated with hepatic encephalopathy. A mental impairment can include abnormal behavior, changes in mental status, or an altered state of consciousness. Reports of abnormal behavior may show that you are experiencing delusions, paranoia, or hallucinations. Reports of changes in mental status may show change in sleep patterns, personality or mood changes, poor concentration, or poor judgment or cognitive dysfunction (for example, impaired memory, poor problem-solving ability, or attention deficits). Reports of altered state of consciousness may show that you are experiencing confusion, delirium, or stupor.

(ii) Signs and laboratory findings that document the severity of hepatic encephalopathy when not attributable to other causes may include a "flapping tremor" (asterixis), characteristic abnormalities found on an electroencephalogram (EEG), or abnormal serum albumin or coagulation values. We will not purchase an EEG; however, if you have had this test at a time relevant to your claim, we will make every reasonable effort to obtain the report for the purpose of establishing whether your impairment meets the criteria of 5.05F.

(iii) We will not evaluate acute encephalopathy under 5.05F if it results from conditions other than CLD. For example, we will evaluate acute encephalopathy caused by vascular events under the listings in 11.00 and acute encephalopathy caused by cancer under the listings in 13.00.

3. SSA Chronic Liver Disease (SSA CLD) score (5.05G). Listing 5.05G requires two SSA CLD scores, each requiring three or four laboratory values. The "date of the SSA CLD score" is the date of the earliest of the three or four laboratory values used for its calculation. The date of the second SSA CLD score must be at least 60 days after the date of the first SSA CLD score and both scores must be within the required 12-month period. If you have the two SSA CLD scores required by 5.05G, we will find that your impairment meets the criteria of the listing from at least the date of the first SSA CLD score. [We provide the SSA CLD Calculator to calculate SSA CLD scores as described below.]

a. We calculate the SSA CLD score using a formula that includes up to four laboratory values: Serum creatinine (mg/dL), total bilirubin (mg/dL), INR, and under certain conditions, serum sodium (mmol/L). The SSA CLD score calculation contains at least one, and sometimes two, parts, as described in (i) and (ii).

(i) The initial calculation is:

SSA CLD_i =

9.57 × [log_e(serum creatinine mg/dL)]

+ 3.78 × [log_e(serum total bilirubin mg/dL)]

+ 11.2 × [log_e(INR)]

+ 6.43

rounded to the nearest whole integer.

(ii) If the value from the initial calculation is 11 or below, the SSA CLD score will be the SSA CLD_i value. If the value from the initial calculation is greater than 11, the SSA CLD score will be re-calculated as:

SSA CLD =

SSA CLD_i

+ 1.32 × (137 − serum sodium mmol/L)

− [0.033 × SSA CLD_i × (137 − serum sodium mmol/L)]

(iii) We round the results of your SSA CLD score calculation to the nearest whole integer to arrive at your SSA CLD score.

b. For any SSA CLD score calculation, all of the required laboratory values (serum creatinine, serum total bilirubin, INR, and serum sodium) must have been obtained within a continuous 30-day period.

(i) We round values for serum creatinine (mg/dL), serum total bilirubin (mg/dL), or INR less than 1.0 up to 1.0 to calculate your SSA CLD score.

(ii) We round values for serum creatinine (mg/dL) greater than 4.0 down to 4.0 to calculate your SSA CLD score.

(iii) If there are multiple laboratory values within the 30-day interval for serum creatinine (mg/dL), serum total bilirubin (mg/dL), or INR, we use the highest value to calculate your SSA CLD score. We will not use any INR values derived from testing done while you are on anticoagulant treatment in our SSA CLD calculation.

(iv) If there are multiple laboratory values within the 30-day interval for serum sodium (mmol/L), we use the lowest value to calculate your SSA CLD score.

(v) If you are in renal failure or on renal dialysis within a week of any serum creatinine test in the period used for the SSA CLD calculation, we will use a serum creatinine value of 4.0, which is the maximum serum creatinine level allowed in the calculation, to calculate your SSA CLD score.

(vi) If your serum sodium is less than 125 mmol/L, we will set your serum sodium to 125 mmol/L for purposes of calculation of the SSA CLD score. If your serum sodium is higher than 137 mmol/L, we will set your serum sodium to 137 mmol/L for purposes of calculation of the SSA CLD score.

c. When we indicate "log_e " (also abbreviated "ln") in the formula for the SSA CLD score calculation, we mean the "base e logarithm" or "natural logarithm" of the numerical laboratory value, not the "base 10 logarithm" or "common logarithm" (log) of the laboratory value, and not the actual laboratory value. For example, if a person has laboratory values of serum creatinine 1.4 mg/dL, serum total bilirubin 1.3 mg/dL, INR 1.32, and serum sodium 119 mmol/L, we compute the SSA CLD score as follows:

SSA CLD_i =

9.57 × [log_e (serum creatinine 1.4 mg/dL) = 0.336]

+ 3.78 × [log_e (serum total bilirubin 1.3 mg/dL) = 0.262]

+ 11.2 × [log_e (INR 1.32) = .278]

+ 6.43

= 3.22 + 0.99 + 3.11 + 6.43

= 13.75, which we round to an SSA CLD_i score of 14.

Because the SSA CLD_i score is over 11, we then move to the second step of calculating the SSA CLD:

SSA CLD = 14

+ 1.32 × (137 − serum sodium 125 mmol/L)

− [0.033 × SSA CLD_i 14 × (137 − serum sodium 125 mmol/L)

= 14 + 15.84 − 5.54

= 24.3, which we round to an SSA CLD score of 24.

 

D. What is inflammatory bowel disease (IBD), and how do we evaluate it under 5.06?

1. IBD is a group of inflammatory conditions of the small intestine and colon. The most common IBD disorders are Crohn's disease and ulcerative colitis. Remissions and exacerbations of variable duration are a hallmark of IBD.

2. We evaluate your signs and symptoms of IBD, such as diarrhea, fecal incontinence, rectal bleeding, abdominal pain, fatigue, fever, nausea, vomiting, arthralgia, abdominal tenderness, palpable abdominal mass (usually inflamed loops of bowel), and perianal disease (for example, fissure, fistulas, abscesses, or anal canal stenosis), when we assess the severity of your impairment(s). You may require supplemental daily nutrition due to IBD. There are two forms of supplemental daily nutrition we consider under 5.06B5: enteral nutrition (delivered directly to a part of your digestive system) via a gastrostomy, duodenostomy, or jejunostomy, and parenteral nutrition delivered via a central venous catheter. Enteral tube feedings delivered via nasal or oral tubes do not satisfy the requirement in 5.06B5.

3. Surgical diversion of the intestinal tract, including ileostomy and colostomy, does not preclude the ability to perform any gainful activity if you are able to maintain adequate nutrition and function of the stoma. However, if you are not able to maintain adequate nutrition, we will evaluate your impairment under 5.08.

4. IBD may also be associated with significant extraintestinal manifestations in a variety of body systems. These include, but are not limited to, involvement of the eye (for example, uveitis, episcleritis, or iritis); hepatobiliary disease (for example, gallstones or primary sclerosing cholangitis); urologic disease (for example, kidney stones or obstructive hydronephrosis); skin involvement (for example, erythema nodosum or pyoderma gangrenosum); or non-destructive inflammatory arthritis. You may also have associated thromboembolic disorders or vascular disease. These manifestations may not correlate with the severity of your IBD. If your impairment does not meet any of the criteria of 5.06, we will consider the effects of your extraintestinal manifestations in determining whether you have an impairment(s) that meets or medically equals another listing, and when we assess your residual functional capacity.

5. Repeated complications of IBD.

a. Examples of complications of IBD include abscesses, intestinal perforation, toxic megacolon, infectious colitis, pyoderma gangrenosum, ureteral obstruction, primary sclerosing cholangitis, and hypercoagulable state (which may lead to thromboses or embolism). When we evaluate repeated complications of IBD, we consider all relevant information in your case record to determine the effects of your IBD on your ability to function independently, appropriately, effectively, and on a sustained basis. Factors we consider include, but are not limited to: your symptoms, the frequency and duration of your complications, periods of exacerbation and remission, and the functional effects of your treatment, including the side effects of your medication. Your impairment will satisfy this criterion regardless of whether you have the same kind of complication repeatedly, all different complications, or any other combination of complications; for example, two of the same kind of complication and a different one.

b. To satisfy the requirements described under 5.06C, your IBD must result in repeated complications and marked limitation in one of three areas of functioning: activities of daily living; maintaining social functioning; or completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace. If the complications do not last as long or occur as frequently as required under 5.06C, we will consider whether your IBD medically equals the listing.

c. Marked limitation means that the signs and symptoms of your IBD interfere seriously with your ability to function. Although we do not require the use of such a scale, "marked" would be the fourth point on a five-point rating scale consisting of no limitation, mild limitation, moderate limitation, marked limitation, and extreme limitation. We do not define marked by a specific number of activities of daily living or different behaviors in which your social functioning is impaired, or a specific number of tasks that you are able to complete, but by the nature and overall degree of interference with your functioning. You may have marked limitation when several activities or functions are impaired, or when only one is impaired. Additionally, you need not be totally precluded from performing an activity to have marked limitation, as long as the degree of limitation interferes seriously with your ability to function independently, appropriately, and effectively. The term "marked" does not imply that you must be confined to bed, hospitalized, or in a nursing home.

d. Activities of daily living include, but are not limited to, such activities as doing household chores, grooming and hygiene, using a post office, taking public transportation, or paying bills. We will find that you have "marked" limitation in activities of daily living if you have a serious limitation in your ability to maintain a household or take public transportation because of symptoms, such as pain, severe fatigue, anxiety, or difficulty concentrating, caused by your IBD (including complications of the disorder) or its treatment, even if you are able to perform some self-care activities.

e. Maintaining social functioning includes the capacity to interact independently, appropriately, effectively, and on a sustained basis with others. It includes the ability to communicate effectively with others. We will find that you have "marked" limitation in maintaining social functioning if you have a serious limitation in social interaction on a sustained basis because of symptoms, such as pain, severe fatigue, anxiety, or difficulty concentrating, or a pattern of exacerbation and remission, caused by your IBD (including complications of the disorder) or its treatment, even if you are able to communicate with close friends or relatives.

f. Completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace involves the ability to sustain concentration, persistence, or pace to permit timely completion of tasks commonly found in work settings. We will find that you have "marked" limitation in completing tasks if you have a serious limitation in your ability to sustain concentration or pace adequate to complete work-related tasks because of symptoms, such as pain, severe fatigue, anxiety, or difficulty concentrating, caused by your IBD (including complications of the disorder) or its treatment, even if you are able to do some routine activities of daily living.

E. What is intestinal failure, and how do we evaluate it under 5.07?

1. Intestinal failure is a condition resulting in gut function below the minimum necessary for the absorption of macronutrients or water and electrolytes, resulting in a requirement for intravenous supplementation ( i.e.,parenteral nutrition) to maintain health. Examples of conditions that may result in intestinal failure include short bowel syndrome, extensive small bowel mucosal disease, and chronic motility disorders.

2. Short bowel syndrome is a malabsorption disorder that occurs when ischemic vascular insults (caused, for example, by volvulus or necrotizing enterocolitis), trauma, or IBD complications require(s) surgical resection of any amount of the small intestine, resulting in chronic malnutrition.

3. Extensive small bowel mucosal disease means that the mucosal surface of the small bowel does not efficiently absorb nutrients or loses nutrients. Common causes of small bowel mucosal disease include microvillous inclusion disease and tufting enteropathy.

4. Chronic motility disorder refers to a chronic disorder of the propulsion of gut content without fixed obstructions, causing intolerance to oral nutrition and inadequate nutritional intake. This type of disorder may also be known as a chronic intestinal pseudo-obstruction (CIPO), because the gut dysfunction mimics that of an obstructed intestine, but without evidence of an actual obstruction. Primary CIPO may have an unknown underlying cause. Chronic motility disorders may also result from congenital, neuromuscular, or autoimmune conditions, such as gastroschisis, omphalocele, long segment Hirschprung's disease, Crohn's disease, and mitochondrial disorders.

5. For short bowel syndrome, we require a copy of the operative report that includes details of the surgical findings, or postoperative imaging indicating a resection of the small intestine. If we cannot get one of these reports, we need other medical reports that include details of the surgical findings. For other chronic motility disorders or extensive small bowel mucosal disease, we need medical reports that include details of your intestinal dysfunction. For any impairment evaluated under 5.07, we also need medical documentation that you are dependent on daily parenteral nutrition to provide most of your nutritional requirements.

F. How do we evaluate weight loss due to any digestive disorder under 5.08?

1. In addition to the impairments specifically mentioned in these listings, other digestive disorders, such as esophageal stricture, pancreatic insufficiency, and malabsorption, may result in significant weight loss. Impairments other than digestive disorders that cause weight loss should be evaluated under the appropriate body system for that impairment. For instance, weight loss as a result of chronic kidney disease should be evaluated under our rules for genitourinary disorders (see 6.00), and weight loss as the result of an eating disorder should be evaluated under our rules for mental disorders (see 12.00). However, if you develop a digestive disorder as the result of your other impairment, we will evaluate the acquired digestive disorder under our rules for digestive disorders. We evaluate weight loss due to any digestive disorder under 5.08 by using the body mass index (BMI).

2. BMI is the ratio of your weight to the square of your height. Calculation and interpretation of the BMI are independent of sex in adults.

a. We calculate BMI using inches and pounds, meters and kilograms, or centimeters and kilograms. We must have measurements of your weight and height without shoes for these calculations.

b. We calculate BMI using one of the following formulas:

English Formula

BMI = [Weight in Pounds/(Height in Inches × Height in Inches)] × 703

Metric Formulas

BMI = Weight in Kilograms/(Height in Meters × Height in Meters)

BMI = [Weight in Kilograms/(Height in Centimeters × Height in Centimeters)] × 10,000

G. How do we evaluate digestive organ transplantation? If you receive a liver (5.09), small intestine (5.11), or pancreas (5.12) transplant, we will consider you disabled under the listing for 1 year from the date of the transplant. After that, we evaluate your residual impairment(s) by considering the adequacy of your post-transplant function, the frequency and severity of any rejection episodes you have, complications in other body systems, and adverse treatment effects. People who receive digestive organ transplants generally have impairments that meet our definition of disability before they undergo transplantation. The phrase "consider under a disability for 1 year" in 5.09, 5.11, and 5.12 does not refer to the date on which your disability began, only to the date on which we must reevaluate whether your impairment(s) continues to meet a listing or is otherwise disabling. We determine the onset of your disability based on the facts of your case.

H. How do we evaluate your digestive disorder if there is no record of ongoing treatment? If there is no record of ongoing treatment despite the existence of a severe impairment(s), we will assess the severity and duration of your digestive disorder based on the current medical and other evidence in your case record. If there is no record of ongoing treatment, you may not be able to show an impairment that meets a digestive disorders listing, but your impairment may medically equal a listing, or be disabling based on consideration of your residual functional capacity, age, education, and work experience.

I. How do we evaluate your digestive disorder if there is evidence establishing a substance use disorder? If we find that you are disabled and there is medical evidence in your case record establishing that you have a substance use disorder, we will determine whether your substance use disorder is a contributing factor material to the determination of disability. See §§ 404.1535 and 416.935 of this chapter. Digestive disorders resulting from drug or alcohol use are often chronic in nature and will not necessarily improve with cessation in drug or alcohol use.

J. How do we evaluate digestive disorders that do not meet one of these listings?

1. These listings are only examples of common digestive disorders that we consider severe enough to prevent you from doing any gainful activity. If your impairment(s) does not meet the criteria of any of these listings, we must also consider whether you have an impairment(s) that satisfies the criteria of a listing in another body system.

2. If you have a severe medically determinable impairment(s) that does not meet a listing, we will determine whether your impairment(s) medically equals a listing. See §§404.1526 and 416.926 of this chapter. Digestive disorders may be associated with disorders in other body systems, and we consider the combined effects of multiple impairments when we determine whether they medically equal a listing. If your impairment(s) does not meet or medically equal a listing, you may or may not have the residual functional capacity to engage in substantial gainful activity. We proceed to the fourth step and, if necessary, the fifth step of the sequential evaluation process in §§ 404.1520 and 416.920 of this chapter. We use the rules in §§ 404.1594 and 416.994 of this chapter, as appropriate, when we decide whether you continue to be disabled.

5.01 Category of Impairments, Digestive Disorders

5.02 Gastrointestinal hemorrhaging from any cause, requiring three blood transfusions of at least 2 units of blood per transfusion, within a consecutive 12-month period and at least 30 days apart. Consider under a disability for 1 year following the last documented transfusion; after that, evaluate the residual impairment(s).

5.03-5.04[Reserved]

5.05 Chronic liver disease (CLD) (see 5.00C) with A, B, C, D, E, F, or G:

A. Hemorrhaging from esophageal, gastric, or ectopic varices, or from portal hypertensive gastropathy (see 5.00C2a), documented by imaging (see 5.00B3); resulting in 1 and 2:

1. Hemodynamic instability indicated by signs such as pallor (pale skin), diaphoresis (profuse perspiration), rapid pulse, low blood pressure, postural hypotension (pronounced fall in blood pressure when arising to an upright position from lying down), or syncope (fainting); and

2. Requiring hospitalization for transfusion of at least 2 units of blood. Consider under a disability for 1 year following the documented transfusion; after that, evaluate the residual impairment(s).

OR

B. Ascites or hydrothorax not attributable to other causes (see 5.00C2b), present on two evaluations within a consecutive 12-month period and at least 60 days apart. Each evaluation must document the ascites or hydrothorax by 1, 2, or 3:

1. Paracentesis; or

2. Thoracentesis; or

3. Imaging or physical examination with a or b:

a. Serum albumin of 3.0 g/dL or less; or

b. INR of at least 1.5.

OR

C. Spontaneous bacterial peritonitis (see 5.00C2c) documented by peritoneal fluid containing a neutrophil count of at least 250 cells/mm³.

OR

D. Hepatorenal syndrome (see 5.00C2d) documented by 1, 2, or 3:

1. Serum creatinine elevation of at least 2 mg/dL; or

2. Oliguria with 24-hour urine output less than 500 mL; or

3. Sodium retention with urine sodium less than 10 mEq per liter.

OR

E. Hepatopulmonary syndrome (see 5.00C2e) documented by 1 or 2:

1. Arterial P_aO₂ measured by an ABG test, while at rest, breathing room air, less than or equal to:

a. 60 mm Hg, at test sites less than 3,000 feet above sea level; or

b. 55 mm Hg, at test sites from 3,000 through 6,000 feet above sea level; or

c. 50 mm Hg, at test sites over 6,000 feet above sea level; or

2. Intrapulmonary arteriovenous shunting as shown by contrast-enhanced echocardiography or macroaggregated albumin lung perfusion scan.

OR

F. Hepatic encephalopathy (see 5.00C2f) with documentation of abnormal behavior, cognitive dysfunction, changes in mental status, or altered state of consciousness (for example, confusion, delirium, stupor, or coma), present on two evaluations within a consecutive 12-month period and at least 60 days apart and either 1 or 2:

1. History of transjugular intrahepatic portosystemic shunt (TIPS) or other surgical portosystemic shunt; or

2. One of the following on at least two evaluations at least 60 days apart within the same consecutive 12-month period as in F:

a. Asterixis or other fluctuating physical neurological abnormalities; or

b. EEG demonstrating triphasic slow wave activity; or

c. Serum albumin of 3.0 g/dL or less; or

d. INR of 1.5 or greater.

OR

G. Two SSA CLD scores (see 5.00C3) [ SSA CLD Calculator ] of at least 20 within a consecutive 12-month period and at least 60 days apart. Consider under a disability from at least the date of the first score.

5.06 Inflammatory bowel disease (IBD) (see 5.00D) documented by endoscopy, biopsy, imaging, or operative findings, and demonstrated by A, B, or C:

A. Obstruction of stenotic areas (not adhesions) in the small intestine or colon with proximal dilatation, confirmed by imaging or in surgery, requiring two hospitalizations for intestinal decompression or for surgery, within a consecutive 12-month period and at least 60 days apart.

OR

B. Two of the following occurring within a consecutive 12-month period and at least 60 days apart:

1. Anemia with hemoglobin of less than 10.0 g/dL, present on at least two evaluations at least 60 days apart; or

2. Serum albumin of 3.0 g/dL or less, present on at least two evaluations at least 60 days apart; or

3. Clinically documented tender abdominal mass palpable on physical examination with abdominal pain or cramping; or

4. Perianal disease with a draining abscess or fistula; or

5. Need for supplemental daily enteral nutrition via a gastrostomy, duodenostomy, or jejunostomy, or daily parenteral nutrition via a central venous catheter.

OR

C. Repeated complications of IBD (see 5.00D5a), occurring an average of 3 times a year, or once every 4 months, each lasting 2 weeks or more, within a consecutive 12-month period, and marked limitation (see 5.00D5c) in one of the following:

1. Activities of daily living (see 5.00D5d); or

2. Maintaining social functioning (see 5.00D5e); or

3. Completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace (see 5.00D5f).

5.07 Intestinal failure (see 5.00E) due to short bowel syndrome, chronic motility disorders, or extensive small bowel mucosal disease, resulting in dependence on daily parenteral nutrition via a central venous catheter for at least 12 months.

5.08 Weight loss due to any digestive disorder (see 5.00F), despite adherence to prescribed medical treatment, with BMI of less than 17.50 calculated on at least two evaluations at least 60 days apart within a consecutive 12-month period.

5.09 Liver transplantation (see 5.00G). Consider under a disability for 1 year from the date of the transplant; after that, evaluate the residual impairment(s).

5.10 [Reserved]

5.11 Small intestine transplantation (see 5.00G). Consider under a disability for 1 year from the date of the transplant; after that, evaluate the residual impairment(s).

5.12 Pancreas transplantation (see 5.00G). Consider under a disability for 1 year from the date of the transplant; after that, evaluate the residual impairment(s).

DI 34001.020 Genitourinary Disorders

6.00 GENITOURINARY DISORDERS (Effective Date: 12/09/2014)

A. Which disorders do we evaluate under these listings?

We evaluate genitourinary disorders resulting in chronic kidney disease (CKD). Examples of such disorders include chronic glomerulonephritis, hypertensive nephropathy, diabetic nephropathy, chronic obstructive uropathy, and hereditary nephropathies. We also evaluate nephrotic syndrome due to glomerular dysfunction under these listings.

B. What evidence do we need?

1. We need evidence that documents the signs, symptoms, and laboratory findings of your CKD. This evidence should include reports of clinical examinations, treatment records, and documentation of your response to treatment. Laboratory findings, such as serum creatinine or serum albumin levels, may document your kidney function. We generally need evidence covering a period of at least 90 days unless we can make a fully favorable determination or decision without it.

2. Estimated glomerular filtration rate (eGFR). The eGFR is an estimate of the filtering capacity of the kidneys that takes into account serum creatinine concentration and other variables, such as your age, sex, and body size. If your medical evidence includes eGFR findings, we will consider them when we evaluate your CKD under 6.05.

3. Kidney or bone biopsy. If you have had a kidney or bone biopsy, we need a copy of the pathology report. When we cannot get a copy of the pathology report, we will accept a statement from an acceptable medical source verifying that a biopsy was performed and describing the results.

C. What other factors do we consider when we evaluate your genitourinary disorder?

1. Chronic hemodialysis or peritoneal dialysis.

a. Dialysis is a treatment for CKD that uses artificial means to remove toxic metabolic byproducts from the blood. Hemodialysis uses an artificial kidney machine to clean waste products from the blood; peritoneal dialysis uses a dialyzing solution that is introduced into and removed from the abdomen (peritoneal cavity) either continuously or intermittently. Under 6.03, your ongoing dialysis must have lasted or be expected to last for a continuous period of at least 12 months. To satisfy the requirements in 6.03, we will accept a report from an acceptable medical source that describes your CKD and your current dialysis, and indicates that your dialysis will be ongoing.

b. If you are undergoing chronic hemodialysis or peritoneal dialysis, your CKD may meet our definition of disability before you started dialysis. We will determine the onset of your disability based on the facts in your case record.

2. Kidney transplant.

a. If you receive a kidney transplant, we will consider you to be disabled under 6.04 for 1 year from the date of transplant. After that, we will evaluate your residual impairment(s) by considering your post-transplant function, any rejection episodes you have had, complications in other body systems, and any adverse effects related to ongoing treatment.

b. If you received a kidney transplant, your CKD may meet our definition of disability before you received the transplant. We will determine the onset of your disability based on the facts in your case record.

3. Renal osteodystrophy. This condition is the bone degeneration resulting from chronic kidney disease-mineral and bone disorder (CKD-MBD). CKD-MBD occurs when the kidneys are unable to maintain the necessary levels of minerals, hormones, and vitamins required for bone structure and function. Under 6.05B1, “severe bone pain” means frequent or intractable (resistant to treatment) bone pain that interferes with physical activity or mental functioning.

4. Peripheral neuropathy. This disorder results when the kidneys do not adequately filter toxic substances from the blood. These toxins can adversely affect nerve tissue. The resulting neuropathy may affect peripheral motor or sensory nerves, or both, causing pain, numbness, tingling, and muscle weakness in various parts of the body. Under 6.05B2, the peripheral neuropathy must be a severe impairment. (See §§ 404.1520(c), 404.1521, 416.920(c), and 416.921 of this chapter.) It must also have lasted or be expected to last for a continuous period of at least 12 months.

5. Fluid overload syndrome. This condition occurs when excess sodium and water retention in the body due to CKD results in vascular congestion. Under 6.05B3, we need a description of a physical examination that documents signs and symptoms of vascular congestion, such as congestive heart failure, pleural effusion (excess fluid in the chest), ascites (excess fluid in the abdomen), hypertension, fatigue, shortness of breath, or peripheral edema.

6. Anasarca (generalized massive edema or swelling). Under 6.05B3 and 6.06B, we need a description of the extent of edema, including pretibial (in front of the tibia), periorbital (around the eyes), or presacral (in front of the sacrum) edema. We also need a description of any ascites, pleural effusion, or pericardial effusion.

7. Anorexia (diminished appetite) with weight loss. Anorexia is a frequent sign of CKD and can result in weight loss. We will use body mass index (BMI) to determine the severity of your weight loss under 6.05B4. (BMI is the ratio of your measured weight to the square of your measured height.) We calculate your BMI using the formulas in the digestive disorders body system (5.00).

8. Complications of CKD. The hospitalizations in 6.09 may be for different complications of CKD. Examples of complications from CKD that may result in hospitalization include stroke, congestive heart failure, hypertensive crisis, or acute kidney failure requiring a short course of hemodialysis. If the CKD complication occurs during a hospitalization that was initially for a co-occurring condition, we will evaluate it under our rules for determining medical equivalence. (See §§ 404.1526 and 416.926 of this chapter.) We will evaluate co-occurring conditions, including those that result in hospitalizations, under the listings for the affected body system or under our rules for medical equivalence.

D. How do we evaluate disorders that do not meet one of the genitourinary listings?

1. The listed disorders are only examples of common genitourinary disorders that we consider severe enough to prevent you from doing any gainful activity. If your impairment(s) does not meet the criteria of any of these listings, we must also consider whether you have an impairment(s) that satisfies the criteria of a listing in another body system.

2. If you have a severe medically determinable impairment(s) that does not meet a listing, we will determine whether your impairment(s) medically equals a listing. (See §§ 404.1526 and 416.926 of this chapter.) Genitourinary disorders may be associated with disorders in other body systems, and we consider the combined effects of multiple impairments when we determine whether they medically equal a listing. If your impairment(s) does not meet or medically equal the criteria of a listing, you may or may not have the residual functional capacity to engage in substantial gainful activity. We proceed to the fourth and, if necessary, the fifth steps of the sequential evaluation process in §§ 404.1520 and 416.920 of this chapter. We use the rules in §§ 404.1594 and 416.994 of this chapter, as appropriate, when we decide whether you continue to be disabled.

6.01 Category of Impairments, Genitourinary Disorders

6.03 Chronic kidney disease , with chronic hemodialysis or peritoneal dialysis (see 6.00C1).

6.04 Chronic kidney disease , with kidney transplant. Consider under a disability for 1 year following the transplant; thereafter, evaluate the residual impairment (see 6.00C2).

6.05 Chronic kidney disease , with impairment of kidney function, with A and B:

A. Reduced glomerular filtration evidenced by one of the following laboratory findings documented on at least two occasions at least 90 days apart during a consecutive 12-month period:

1. Serum creatinine of 4 mg/dL or greater; or

2. Creatinine clearance of 20 ml/min. or less; or

3. Estimated glomerular filtration rate (eGFR) of 20 ml/min/1.73m² or less.

AND

B. One of the following:

1. Renal osteodystrophy (see 6.00C3) with severe bone pain and imaging studies documenting bone abnormalities, such as osteitis fibrosa, osteomalacia, or pathologic fractures; or

2. Peripheral neuropathy (see 6.00C4); or

3. Fluid overload syndrome (see 6.00C5) documented by one of the following:

a. Diastolic hypertension greater than or equal to diastolic blood pressure of 110 mm Hg despite at least 90 consecutive days of prescribed therapy, documented by at least two measurements of diastolic blood pressure at least 90 days apart during a consecutive 12-month period; or

b. Signs of vascular congestion or anasarca (see 6.00C6) despite at least 90 consecutive days of prescribed therapy, documented on at least two occasions at least 90 days apart during a consecutive 12-month period; or

4. Anorexia with weight loss (see 6.00C7) determined by body mass index (BMI) of 18.0 or less, calculated on at least two occasions at least 90 days apart during a consecutive 12-month period.

6.06 Nephrotic syndrome , with A and B:

A. Laboratory findings as described in 1 or 2, documented on at least two occasions at least 90 days apart during a consecutive 12-month period:

1. Proteinuria of 10.0 g or greater per 24 hours; or

2. Serum albumin of 3.0 g/dL or less, and

a. Proteinuria of 3.5 g or greater per 24 hours; or

b. Urine total-protein-to-creatinine ratio of 3.5 or greater.

AND

B. Anasarca (see 6.00C6) persisting for at least 90 days despite prescribed treatment.

6.09 Complications of chronic kidney disease (see 6.00C8) requiring at least three hospitalizations within a consecutive 12-month period and occurring at least 30 days apart. Each hospitalization must last at least 48 hours, including hours in a hospital emergency department immediately before the hospitalization.