**Cystic Fibrosis Foundation**

**Proposed Changes**

**June 13, 2005**

**ADULT LISTINGS**

**3.00 Respiratory System**

A. Introduction. The listings in this section describe impairments
resulting from respiratory disorders based on symptoms, physical signs,
laboratory test abnormalities, and response to a regimen of treatment
prescribed by a treating source. Respiratory disorders along with any
associated impairment(s) must be established by medical evidence.
Evidence must be provided in sufficient detail to permit an independent
reviewer to evaluate the severity of the impairment.

Many individuals, especially those who have listing-level impairments,
will have received the benefit of medically prescribed treatment.
Whenever there is evidence of such treatment, the longitudinal clinical
record must include a description of the treatment prescribed by the
treating source and response in addition to information about the nature
and severity of the impairment.

It is important to document any prescribed treatment and response,
because this medical management may have improved the individual\'s
functional status. The longitudinal record should provide information
regarding functional recovery, if any.

Some individuals will not have received ongoing treatment or have an
ongoing relationship with the medical community, despite the existence
of a severe impairment(s). An individual who does not receive treatment
may or may not be able to show the existence of an impairment that meets
the criteria of these listings.

Even if an individual does not show that his or her impairment meets the
criteria of these listings, the individual may have an impairment(s)
equivalent in severity to one of the listed impairments or be disabled
because of a limited residual functional capacity.

Unless the claim can be decided favorably on the basis of the current
evidence, a longitudinal record is still important because it will
provide information about such things as the ongoing medical severity of
the impairment, the level of the individual\'s functioning, and the
frequency, severity, and duration of symptoms. Also, the asthma listing
specifically includes a requirement for continuing signs and symptoms
despite a regimen of prescribed treatment.

Impairments caused by chronic disorders of the respiratory system
generally produce irreversible loss of pulmonary function due to
ventilatory impairments, gas exchange abnormalities, or a combination of
both. The most common symptoms attributable to these disorders are
dyspnea on exertion, cough, wheezing, sputum production, hemoptysis, and
chest pain.

Because these symptoms are common to many other diseases, a thorough
medical history, physical examination, and chest x-ray or other
appropriate imaging technique are required to establish chronic
pulmonary disease. Pulmonary function testing is required to assess the
severity of the respiratory impairment once a disease process is
established by appropriate clinical and laboratory findings.

Alterations of pulmonary function can be due to obstructive airway
disease (e.g., emphysema, chronic bronchitis, asthma), restrictive
pulmonary disorders with primary loss of lung volume (e.g., pulmonary
resection, thoracoplasty, chest cage deformity as in kyphoscoliosis or
obesity), or infiltrative interstitial disorders (e.g., diffuse
pulmonary fibrosis). Gas exchange abnormalities without significant
airway obstruction can be produced by interstitial disorders.

Disorders involving the pulmonary circulation (e.g., primary pulmonary
hypertension, recurrent thromboembolic disease, primary or secondary
pulmonary vasculitis) can produce pulmonary vascular hypertension and,
eventually, pulmonary heart disease (cor pulmonale) and right heart
failure. Persistent hypoxemia produced by any chronic pulmonary disorder
also can result in chronic pulmonary hypertension and right heart
failure.

Chronic infection, caused most frequently by mycobacterial or mycotic
organisms, can produce extensive and progressive lung destruction
resulting in marked loss of pulmonary function. Some disorders, such as
bronchiectasis, cystic fibrosis, and asthma, can be associated with
intermittent exacerbations of such frequency and intensity that they
produce a disabling impairment, even when pulmonary function during
periods of relative clinical stability is relatively well-maintained.

Respiratory impairments usually can be evaluated under these listings on
the basis of a complete medical history, physical examination, a chest
x-ray or other appropriate imaging techniques, and spirometric pulmonary
function tests. In some situations, most typically with a diagnosis of
diffuse interstitial fibrosis or clinical findings suggesting cor
pulmonale, such as cyanosis or secondary polycythemia, an impairment may
be underestimated on the basis of spirometry alone.

More sophisticated pulmonary function testing may then be necessary to
determine if gas exchange abnormalities contribute to the severity of a
respiratory impairment. Additional testing might include measurement of
diffusing capacity of the lungs for carbon monoxide or resting arterial
blood gases.

Measurement of arterial blood gases during exercise is required
infrequently. In disorders of the pulmonary circulation, right heart
catheterization with angiography and/or direct measurement of pulmonary
artery pressure may have been done to establish a diagnosis and evaluate
severity. When performed, the results of the procedure should be
obtained. Cardiac catheterization will not be purchased.

These listings are examples of common respiratory disorders that are
severe enough to prevent a person from engaging in a gainful activity.
When an individual has a medically-determinable impairment that is not
listed, an impairment which does not meet a listing, or a combination of
impairments no one of which meets a listing, we will consider whether
the individual\'s impairment or combination of impairments is medically
equivalent in severity to a listed impairment.

Individuals who have an impairment(s) with a level of severity which
does not meet or equal the criteria of the listings may or may not have
the residual functional capacity (RFC) which would enable them to engage
in substantial gainful activity. Evaluation of the impairment(s) of
these individuals will proceed through the final steps of the sequential
evaluation process.

[Add information about advances in medicine that allow people with
respiratory impairments to obtain lung transplants, and the need
to]{.mark} [continue to]{.mark} [review their functional status]{.mark}
[indefinitely. This should be done several years after the procedure,
and take into account the multi-system impact of the initial diagnosis,
and complications related to the transplant itself and its
treatments.]{.mark}

B. Mycobacterial, mycotic, and other chronic persistent infections of
the lung. These disorders are evaluated on the basis of the resulting
limitations in pulmonary function. Evidence of chronic infections, such
as active mycobacterial diseases or mycoses with positive cultures, drug
resistance, enlarging parenchymal lesions, or cavitation, is not, by
itself, a basis for determining that an individual has a disabling
impairment expected to last 12 months.

In those unusual cases of pulmonary infection that persist for a period
approaching 12 consecutive months, the clinical findings, complications,
therapeutic considerations, and prognosis must be carefully assessed to
determine whether, despite relatively well-maintained pulmonary
function, the individual nevertheless has an impairment that is expected
to last for at least 12 consecutive months and prevent gainful activity.

C. Episodic respiratory disease. When a respiratory impairment is
episodic in nature, as can occur with exacerbations of asthma, cystic
fibrosis, bronchiectasis, or chronic asthmatic bronchitis, the frequency
and intensity of episodes that occur despite prescribed treatment are
often the major criteria for determining the level of impairment.

Documentation for these exacerbations should include available hospital,
emergency facility and/or physician records indicating the dates of
treatment; clinical and laboratory findings on presentation, such as the
results of spirometry and arterial blood gas studies (ABGS); the
treatment administered; the time period required for [daily]{.mark}
[self]{.mark}-treatment; and the clinical response.

Attacks of asthma, episodes of bronchitis or pneumonia or hemoptysis
(more than blood-streaked sputum), or respiratory failure as referred to
in paragraph B of 3.03, 3.04, and 3.07, are defined as prolonged
symptomatic episodes lasting one or more days and requiring intensive
treatment, such as intravenous bronchodilator or antibiotic
administration or prolonged inhalational bronchodilator therapy in a
hospital, emergency room or equivalent setting.

Hospital admissions are defined as inpatient hospitalizations for longer
than 24 hours. The medical evidence must also include information
documenting adherence to a prescribed regimen of treatment as well as a
description of physical signs. For asthma, the medical evidence should
include spirometric results obtained between attacks that document the
presence of baseline airflow obstruction.

D. **Cystic fibrosis** is a disorder that [can]{.mark} [affect]{.mark}
the respiratory, digestive, [endocrine,]{.mark} [reproductive, sinus and
hepatobiliary]{.mark} systems and is responsible for a wide and variable
spectrum of clinical manifestations and complications. **[.
~~In~~]{.mark}** **[~~addition, the diagnosis requires laboratory
evidence of abnormalities in the cystic fibrosis gene or in the function
of its protein product~~.]{.mark}**

[New]{.mark} [wording]{.mark} [for bolded section:\
Diagnosis of CF should be based on the presence of one or more
characteristic phenotypic features, a history of CF in a sibling or a
positive newborn screening test, plus laboratory evidence of a CFTR
abnormality as document by elevated sweat chloride concentrations; or
identification of two CF mutations; or]{.mark} *[in vivo]{.mark}*
[demonstration of characteristic abnormalities in ion transport across
the nasal epithelium.]{.mark} [Phenotypic features include:]{.mark}

[]{.mark}

-   [Chronic sino-pulmonary disease manifested by:]{.mark}

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-   [Persistent colonization/infection with typical CF
    pathogens.]{.mark}

-   [Chronic cough and sputum production.]{.mark}

-   [Persistent chest radiograph abnormalities (e.g., bronchiectasis,
    atelectasis, infiltrates, hyperinflation).]{.mark}

-   [Airway obstruction manifested by wheezing and air trapping.]{.mark}

-   [Nasal polyps; radiograph or CT abnormalities of the paranasal
    sinuses.]{.mark}

-   [Digital clubbing.]{.mark}

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-   [Gastro-intestinal and nutritional abnormalities, including:]{.mark}

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-   [Intestinal:]{.mark} [meconium ileus; distal intestinal obstruction
    syndrome (DIOS); rectal prolapse.]{.mark}

-   [Pancreatic: pancreatic insufficiency; recurrent
    pancreatitis.]{.mark}

-   [Hepatic: chronic hepatic disease manifested by clinical or
    histologic evidence of focal biliary cirrhosis or multi-lobular
    cirrhosis.]{.mark}

-   [Nutritional:]{.mark} [failure to thrive (protein-calorie
    malnutrition); hypoproteinemia and edema; complications secondary to
    fat-soluble vitamin deficiency.]{.mark}

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-   [Salt loss syndromes: acute salt]{.mark} [depletion; chronic
    metabolic alkalosis.]{.mark}

-   [Male urogenital abnormalities resulting in obstructive
    azoospermia.]{.mark}

[]{.mark}

[Source:]{.mark} [Rosenstein BJ, Cutting GR. The diagnosis of cystic
fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus
Panel.]{.mark} [[J Pediatr.]{.underline}]{.mark} [1998
Apr;132(4):589-95.]{.mark}

The quantitative pilocarpine iontophoresis procedure for collection of
sweat [and subsequent analysis]{.mark} must be utilized. Two methods are
acceptable: [the test must be]{.mark} [carried out in accordance with
the guidelines of the]{.mark} [National Committee for Clinical
Laboratory Standards (NCCLS), now the Clinical and Laboratory Standards
Institute (CLSI). Source: Sweat testing: sample collection and
quantitative analysis- approved guideline \[Document C34-A2\] Wayne (PA)
2000. (Address; 940 W. Valley Road, Suite 1400, Wayne Pa 19087]{.mark}
[[www.clsi.org](http://www.clsi.org/)).]{.mark} [or the "Wescor
Macroduct System" must be used.]{.mark} []{.mark}

[]{.mark}

The pulmonary manifestations of this disorder should be evaluated under
3.04. The nonpulmonary aspects of cystic fibrosis should be evaluated
under the digestive body system (5.00), [and endocrine]{.mark} [system
(9.00)]{.mark} [.]{.mark} Because cystic fibrosis may involve the
respiratory, digestive[,]{.mark} [endocrine, and hepatobiliary]{.mark}
body systems, the combined effects of the involvement of these body
systems must be considered in case adjudication.\
\
E. Documentation of pulmonary function testing. The results of
spirometry that are used for adjudication under paragraphs A and B of
3.02 and paragraph A of 3.04 should be expressed in liters (L), body
temperature and pressure saturated with water vapor (BTPS). The reported
one-second forced expiratory volume (FEV~1~) and forced vital capacity
(FVC) should represent the largest of at least three satisfactory forced
expiratory maneuvers. Two of the satisfactory spirograms should be
reproducible for both pre-bronchodilator tests and, if indicated,
post-bronchodilator tests.

A value is considered reproducible if it does not differ from the
largest value by more than 5 percent or 0.1 L, whichever is greater. The
highest values of the FEV~1~ and FVC, whether from the same or different
tracings, should be used to assess the severity of the respiratory
impairment. Peak flow should be achieved early in expiration, and the
spirogram should have a smooth contour with gradually decreasing flow
throughout expiration. The zero time for measurement of the FEV~1~ and
FVC, if not distinct, should be derived by linear back-extrapolation of
peak flow to zero volume. A spirogram is satisfactory for measurement of
the FEV~1~ if the expiratory volume at the back-extrapolated zero time
is less than 5 percent of the FVC or 0.1 L, whichever is greater.

The spirogram is satisfactory for measurement of the FVC if maximal
expiratory effort continues for at least 6 seconds, or if there is a
plateau in the volume-time curve with no detectable change in expired
volume (VE) during the last 2 seconds of maximal expiratory effort.

Spirometry should be repeated after administration of an aerosolized
bronchodilator under supervision of the testing personnel if the
pre-bronchodilator FEV~1~ value is less than 70 percent of the predicted
normal value. Pulmonary function studies should not be performed unless
the clinical status is stable (e.g., the individual is not having an
asthmatic attack or suffering from an acute respiratory infection or
other acute illness). Wheezing is common in asthma, chronic bronchitis,
or chronic obstructive pulmonary disease and does not preclude testing.

The effect of the administered bronchodilator in relieving bronchospasm
and improving ventilatory function is assessed by spirometry. If a
bronchodilator is not administered, the reason should be clearly stated
in the report. Pulmonary function studies performed to assess airflow
obstruction without testing after bronchodilators cannot be used to
assess levels of impairment in the range that prevents any gainful work
activity, unless the use of bronchodilators is contraindicated.
Post-bronchodilator testing should be performed 10 minutes after
bronchodilator administration.

The dose and name of the bronchodilator administered should be
specified. The values in paragraphs A and B of 3.02 must only be used as
criteria for the level of ventilatory impairment that exists during the
individual\'s most stable state of health (i.e., any period in time
except during or shortly after an exacerbation).

The appropriately labeled spirometric tracing, showing the claimant\'s
name, date of testing, distance per second on the abscissa and the
distance per liter (L) on the ordinate, must be incorporated into the
file. The manufacturer and model number of the device used to measure
and record the spirogram should be stated. The testing device must
accurately measure both time and volume, the latter to within 1 percent
of a 3 L calibrating volume. If the spirogram was generated by any means
other than direct pen linkage to a mechanical displacement-type
spirometer, the testing device must have had a recorded calibration
performed previously on the day of the spirometric measurement.

If the spirometer directly measures flow, and volume is derived by
electronic integration, the linearity of the device must be documented
by recording volume calibrations at three different flow rates of
approximately 30 L/min 3 L/6 sec) 60 L/min 3 L/3 sec), and 180 L/min 3
L/sec). The volume calibrations should agree to within 1 percent of a 3
L calibrating volume. The proximity of the flow sensor to the individual
should be noted, and it should be stated whether or not a BTPS
correction factor was used for the calibration recordings and for the
individual\'s actual spirograms.

The spirogram must be recorded at a speed of at least 20 mm/sec, and the
recording device must provide a volume excursion of at least 10 mm/L. If
reproductions of the original spirometric tracings are submitted, they
must be legible and have a time scale of at least 20 mm/sec and a volume
scale of at least 10 mm/L to permit independent measurements.
Calculation of FEV~1~ from a flow-volume tracing is not acceptable;
i.e., the spirogram and calibrations must be presented in a volume-time
format at a speed of at least 20 mm/sec and a volume excursion of at
least 10 mm/L to permit independent evaluation.

A statement should be made in the pulmonary function test report of the
individual\'s ability to understand directions as well as his or her
effort and cooperation in performing the pulmonary function tests.

The pulmonary function tables in 3.02 and 3.04 are based on measurement
of standing height without shoes. If an individual has marked spinal
deformities (e.g., kyphoscoliosis), the measured span between the
fingertips with the upper extremities abducted 90 degrees should be
substituted for height when this measurement is greater than the
standing height without shoes.

F. Documentation of Chronic Impairment of Gas Exchange.

1\. Diffusing capacity of the lungs for carbon monoxide (DLCO). A
diffusing capacity of the lungs for carbon monoxide study should be
purchased in cases in which there is documentation of chronic pulmonary
disease, but the existing evidence, including properly performed
spirometry, is not adequate to establish the level of functional
impairment.

Before purchasing DLCO measurements, the medical history, physical
examination, reports of chest x-ray or other appropriate imaging
techniques, and spirometric test results must be obtained and reviewed
because favorable decisions can often be made based on available
evidence without the need for DLCO studies. Purchase of a DLCO study may
be appropriate when there is a question of whether an impairment meets
or is equivalent in severity to a listing, and the claim cannot
otherwise be favorably decided.

The DLCO should be measured by the single breath technique with the
individual relaxed and seated. At sea level, the inspired gas mixture
should contain approximately 0.3 percent carbon monoxide (CO), 10
percent helium (He), 21 percent oxygen (O~2~), and the balance,
nitrogen. At altitudes above sea level, the inspired O~2~ concentration
may be raised to provide an inspired O~2~ tension of approximately 150
mm Hg. Alternatively, the sea level mixture may be employed at altitude
and the measured DLCO corrected for ambient barometric pressure. Helium
may be replaced by another inert gas at an appropriate concentration.

The inspired volume (VI) during the DLCO maneuver should be at least 90
percent of the previously determined vital capacity (VC). The
inspiratory time for the VI should be less than 2 seconds, and the
breath-hold time should be between 9 and 11 seconds. The washout volume
should be between 0.75 and 1.00 L, unless the VC is less than 2 L. In
this case, the washout volume may be reduced to 0.50 L; any such change
should be noted in the report. The alveolar sample volume should be
between 0.5 and 1.0 L and be collected in less than 3 seconds. At least
4 minutes should be allowed for gas washout between repeat studies.

A DLCO should be reported in units of ml CO, standard temperature,
pressure, dry (STPD)/min/mm Hg uncorrected for hemoglobin concentration
and be based on a single-breath alveolar volume determination. Abnormal
hemoglobin or hematocrit values, and/or carboxyhemoglobin levels should
be reported along with diffusing capacity.

The DLCO value used for adjudication should represent the mean of at
least two acceptable measurements, as defined above. In addition, two
acceptable tests should be within 10 percent of each other or 3 ml
CO(STPD)min/mm Hg, whichever is larger. The percent difference should be
calculated as: 100 x (test 1 - test 2)/average DLCO.

The ability of the individual to follow directions and perform the test
properly should be described in the written report. The report should
include tracings of the VI, breath-hold maneuver, and VE appropriately
labeled with the name of the individual and the date of the test. The
time axis should be at least 20 mm/sec and the volume axis at least 10
mm/L. The percentage concentrations of inspired O~2~ and inspired and
expired CO and He for each of the maneuvers should be provided.
Sufficient data must be provided, including documentation of the source
of the predicted equation, to permit verification that the test was
performed adequately, and that, if necessary, corrections for anemia or
carboxyhemoglobin were made appropriately.

2\. Arterial blood gas studies (ABGS). An ABGS performed at rest (while
breathing room air, awake and sitting or standing) or during exercise
should be analyzed in a laboratory certified by a State or Federal
agency. If the laboratory is not certified, it must submit evidence of
participation in a national proficiency testing program as well as
acceptable quality control at the time of testing. The report should
include the altitude of the facility and the barometric pressure on the
date of analysis.

Purchase of resting ABGS may be appropriate when there is a question of
whether an impairment meets or is equivalent in severity to a listing,
and the claim cannot otherwise be favorably decided. If the results of a
DLCO study are greater than 40 percent of predicted normal but less than
60 percent of predicted normal, purchase of resting ABGS should be
considered. Before purchasing resting ABGS, a program physician,
preferably one experienced in the care of patients with pulmonary
disease, must review all clinical and laboratory data short of this
procedure, including spirometry, to determine whether obtaining the test
would present a significant risk to the individual.

3\. Exercise testing. Exercise testing with measurement of arterial
blood gases during exercise may be appropriate in cases in which there
is documentation of chronic pulmonary disease, but full development,
short of exercise testing, is not adequate to establish if the
impairment meets or is equivalent in severity to a listing, and the
claim cannot otherwise be favorably decided.

In this context, \"full development\" means that results from spirometry
and measurement of DLCO and resting ABGS have been obtained from
treating sources or through purchase. Exercise arterial blood gas
measurements will be required infrequently and should be purchased only
after careful review of the medical history, physical examination, chest
x-ray or other appropriate imaging techniques, spirometry, DLCO,
electrocardiogram (ECG), [hematocrit]{.mark} or hemoglobin, and resting
blood gas results by a program physician, preferably one experienced in
the care of patients with pulmonary disease, to determine whether
obtaining the test would present a significant risk to the individual.

Oximetry and capillary blood gas analysis are not acceptable substitutes
for the measurement of arterial blood gases. Arterial blood gas samples
obtained after the completion of exercise are not acceptable for
establishing an individual\'s functional capacity.

Generally, individuals with a DLCO greater than 60 percent of predicted
normal would not be considered for exercise testing with measurement of
blood gas studies. The exercise test facility must be provided with the
claimant\'s clinical records, reports of chest x-ray or other
appropriate imaging techniques, and any spirometry, DLCO, and resting
blood gas results obtained as evidence of record. The testing facility
must determine whether exercise testing presents a significant risk to
the individual; if it does, the reason for not performing the test must
be reported in writing.

4\. Methodology. Individuals considered for exercise testing first
should have resting arterial blood partial pressure of oxygen (P0~2~),
resting arterial blood partial pressure of carbon dioxide (PC0~2~) and
negative log of hydrogen ion concentration (pH) determinations by the
testing facility. The sample should be obtained in either the sitting or
standing position. The individual should then perform exercise under
steady state conditions, preferably on treadmill, breathing room air,
for a period of 4 to 6 minutes at a speed and grade providing an Oxygen
consumption of approximately 17.5 ml/kg/ min (5 METs).

If a bicycle ergometer is used, an exercise equivalent of 5 METs (e.g.,
450 kpm/min, or 75 watts for a 176 pound (80 kilogram) person) should be
used. If the individual is able to complete this level of exercise
without achieving listing-level hypoxemia, then he or she should be
exercised at higher workloads to determine exercise capacity. A warm-up
period of treadmill walking or cycling may be performed to acquaint the
individual with the exercise procedure. If during the warm-up period the
individual cannot achieve an exercise level of 5 METs, a lower workload
may be selected in keeping with the estimate of exercise capacity.

The individual should be monitored by ECG throughout the exercise and in
the immediate post-exercise period. Blood pressure and an ECG should be
recorded during each minute of exercise. During the final 2 minutes of a
specific level of steady state exercise, an arterial blood sample should
be drawn and analyzed for oxygen pressure (or tension) (PO~2~), carbon
dioxide pressure (or tension) (PCO~2~), and pH. At the discretion of the
testing facility, the sample may be obtained either from an indwelling
arterial catheter or by direct arterial puncture.

If possible, in order to evaluate exercise capacity more accurately, a
test site should be selected that has the capability to measure minute
ventilation, O~2~ consumption, and carbon dioxide (CO~2~) production. If
the claimant fails to complete 4 to 6 minutes of steady state exercise,
the testing laboratory should comment on the reason and report the
actual duration and levels of exercise performed. This comment is
necessary to determine if the individual \'s test performance was
limited by lack of effort or other impairment (e.g., cardiac, peripheral
vascular, musculoskeletal, neurological).

The exercise test report should contain representative ECG strips taken
before, during and after exercise; resting and exercise arterial blood
gas values; treadmill speed and grade settings, or, if a bicycle
ergometer was used, exercise levels expressed in watts or kpm/min; and
the duration of exercise. Body weight also should be recorded. If
measured, O~2~ consumption (STPD), minute ventilation (BTPS), and CO~2~
production (STPD) also should be reported. The altitude of the test
site, its normal range of blood gas values, and the barometric pressure
on the test date must be noted.

G. Chronic cor pulmonale and pulmonary vascular disease. The
establishment of an impairment attributable to irreversible cor
pulmonale secondary to chronic pulmonary hypertension requires
documentation by signs and laboratory findings of right ventricular
overload or failure (e.g., an early diastolic right-sided gallop on
auscultation, neck vein distension, hepatomegaly, peripheral edema,
right ventricular outflow tract enlargement on x-ray or other
appropriate imaging techniques, right ventricular hypertrophy on ECG,
and increased pulmonary artery pressure measured by right heart
catheterization available from treating sources).

Cardiac catheterization will not be purchased. Because hypoxemia may
accompany heart failure and is also a cause of pulmonary hypertension,
and may be associated with hypoventilation and respiratory acidosis,
arterial blood gases may demonstrate hypoxemia (decreased PO~2~), CO~2~
retention (increased PCO~2~), and acidosis (decreased pH). Polycythemia
with an elevated red blood cell count and hernatocrit may be found in
the presence of chronic hypoxemia.

P-pulmonale on the ECG does not establish chronic pulmonary hypertension
or chronic cor pulmonale. Evidence of florid right heart failure need
not be present at the time of adjudication for a listing (e.g., 3.09) to
be satisfied, but the medical evidence of record should establish that
cor pulmonale is chronic and irreversible.

H. Sleep-related breathing disorders. Sleep-related breathing disorders
(sleep apneas) are caused by periodic cessation of respiration
associated with hypoxemia and frequent arousals from sleep. Although
many individuals with one of these disorders will respond to prescribed
treatment, in some, the disturbed sleep pattern and associated chronic
nocturnal hypoxemia cause daytime sleepiness with chronic pulmonary
hypertension and/or disturbances in cognitive function. Because daytime
sleepiness can affect memory, orientation and personality, a
longitudinal treatment record may be needed to evaluate mental
functioning.

Not all individuals with sleep apnea develop a functional impairment
that affects work activity. When any gainful work is precluded, the
physiologic basis for the impairment may be chronic cor pulmonale.
Chronic hypoxemia due to episodic apnea may cause pulmonary hypertension
(see 3.00G and 3.09). Daytime somnolence may be associated with
disturbance in cognitive vigilance. Impairment of cognitive function may
be evaluated under organic mental disorders (12.02).

I. Effects of obesity. Obesity is a medically determinable impairment
that is often associated with disturbance of the respiratory system, and
disturbance of this system can be a major cause of disability in
individuals with obesity. The combined effects of obesity with
respiratory impairments can be greater than the effects of each of the
impairments considered separately. Therefore, when determining whether
an individual with obesity has a listing-level impairment or combination
of impairments, and when assessing a claim at other steps of the
sequential evaluation process, including when assessing an individual\'s
residual functional capacity, adjudicators must consider any additional
and cumulative effects of obesity.

**3.01 Category of Impairments, Respiratory System**

**3.02 Chronic pulmonary insufficiency**

A. Chronic obstructive pulmonary disease due to any cause, with the
FEV~1~ equal to or less than the values specified in table I
corresponding to the person\'s height without shoes. (In cases of marked
spinal deformity, see 3.00E.);

**Table I**

  -------------------------- --------------------- -----------------------
  **Height\                  **Height without      **FEV~1~ Equal to or
  without Shoes              Shoes (inches)**      less than (L,BTPS)**
  (centimeters)**                                  

  154 or less                60 or less            1.05

  155-160                    61-63                 1.15

  161-165                    64-65                 1.25

  166-170                    66-67                 1.35

  171-175                    68-69                 1.45

  176-180                    70-71                 1.55

  181 or more                72 or more            1.65
  -------------------------- --------------------- -----------------------

*Or*

B. Chronic restrictive ventilatory disease, due to any cause, with the
FVC equal to or less than the values specified in Table II corresponding
to the person\'s height without shoes. (In cases of marked spinal
deformity, see 3.00E.);

**Table II**

  ------------------------- ----------------------- ----------------------
  **Height\                 **Height\               **FVC Equal to or less
  without Shoes             without Shoes           than (L,BTPS)**
  (centimeters)**           (inches)**              

  154 or less               60 or less              1.25

  155-160                   61-63                   1.35

  161-165                   64-65                   1.45

  166-170                   66-67                   1.55

  171-175                   68-69                   1.65

  176-180                   70-71                   1.75

  181 or more               72 or more              1.85
  ------------------------- ----------------------- ----------------------

*Or*

C. Chronic impairment of gas exchange due to clinically documented
pulmonary disease. With:

1\. Single breath DLCO (see 3.00Fl) less than 10.5 ml/min/mm Hg or less
than 40 percent of the predicted normal value. (Predicted values must
either be based on data obtained at the test site or published values
from a laboratory using the same technique as the test site. The source
of the predicted values should be reported. If they are not published,
they should be submitted in the form of a table or nomogram); or

2\. Arterial blood gas values of PO~2~ and simultaneously determined
PCO~2~ measured while at rest (breathing room air, awake and sitting or
standing) in a clinically stable condition on at least two occasions,
three or more weeks apart within a 6-month period, equal to or, less
then the values specified in the applicable table III-A or III-B or
III-C:

**Table III-A**

(Applicable at test sites less than 3,000 feet\
above sea level)

  ------------------------------------- ---------------------------------
  **Arterial PCO~2~ (mm Hg) and**       **Arterial PO~2~ Equal to or Less
                                        than (mm Hg)**

  30 or below                           65

  31 . . . . .                          64

  32 . . . . .                          63

  33 . . . . .                          62

  34 . . . . .                          61

  35 . . . . .                          60

  36 . . . . .                          59

  37 . . . . .                          58

  38 . . . . .                          57

  39 . . . . .                          56

  40 or above                           55
  ------------------------------------- ---------------------------------

**Table III-B**

(Applicable at test sites 3,000 through 6,000 feet above sea level)

  ------------------------------------- ---------------------------------
  **Arterial PCO~2~\                    **Arterial PO~2~ Equal to or Less
  (mm Hg) and**                         than (mm Hg)**

  30 or below                           60

  31 . . . . . . .                      59

  32 . . . . . .                        58

  33 . . . . . .                        57

  34 . . . . . .                        56

  35 . . . . . .                        55

  36 . . . . . .                        54

  37 . . . . . .                        53

  38 . . . . . .                        52

  39 . . . . . .                        51

  40 or above                           50
  ------------------------------------- ---------------------------------

**Table III-C**

(Applicable at test sites over 6,000 feet above sea level)

  ------------------------------------- ---------------------------------
  **Arterial\                           **Arterial PO~2~ equal to or less
  PCO~2\                                than (mm Hg)**
  ~(mm Hg)\                             
  and**                                 

  30 or below .                         55

  31 . . . . . . .                      54

  32 . . . . . . .                      53

  33 . . . . . . .                      52

  34 . . . . . . .                      51

  35 . . . . . . .                      50

  36 . . . . . . .                      49

  37 . . . . . . .                      48

  38 . . . . . . .                      47

  39 . . . . . . .                      46

  40 or above                           45
  ------------------------------------- ---------------------------------

*Or*

3\. Arterial blood gas values of PO~2~ and simultaneously determined
PCO~2~ during steady state exercise breathing room air (level of
exercise equivalent to or less than 17.5 ml O~2~ consumption/kg/min or 5
METs) equal to or less than the values specified in the applicable table
III-A or III-B or III-C in 3.02 C2.

**3.03 *Asthma***. With:

A. Chronic asthmatic bronchitis. Evaluate under the criteria for chronic
obstructive pulmonary disease in 3.02A;

*Or*

B. Attacks (as defined in 3.00C), in spite of prescribed treatment and
requiring physician

intervention, occurring at least once every 2 months or at least six
times a year. Each in-patient hospitalization for longer than 24 hours
for control of asthma counts as two attacks, and an evaluation period of
at least 12 consecutive months must be used to determine the frequency
of attacks.

**3.04 *Cystic fibrosis****.* With:

A. An FEV~1~ equal to or less than the appropriate value specified in
table IV corresponding to the individual\'s height without shoes. (In
cases of marked spinal deformity, see. 3.00E.);

*Or*

B. Episodes of bronchitis or pneumonia or hemoptysis (more than
bloodstreaked sputum) or respiratory failure (documented according to
3.00C[)]{.mark}, requiring physician intervention, occurring at least
once every 2 months or at least six times a year, [or pneumothorax
occurring once in a 2-3 year period]{.mark}. Each inpatient
hospitalization for longer than 24 hours for treatment counts as two
episodes, and an evaluation period of at least 12 consecutive months
must be used to determine the frequency of episodes;

*Or*

C. Persistent pulmonary infection accompanied by superimposed,
recurrent, symptomatic episodes of increased bacterial infection
occurring at least once every 6 months and requiring intravenous or
nebulization antimicrobial therapy.

[(Per Summary]{.mark} [Statement- Consider]{.mark} [adding a]{.mark}
[reference to Digestive Listings for CF, due to importance of good
nutrition to maintain good lung function. This would be similar to the
reference in the Children's Listing under 103.04 E to the Growth
Impairment]{.mark} [Listings.)]{.mark}

**Table IV**

(Applicable only for evaluation under\
3.04A - cystic fibrosis)

  ---------------------------- ------------------------ ------------------
  **Height without Shoes       **Height without Shoes   **FEV~1~ Equal to
  (centimeters)**              (inches)**               or less than
                                                        (L,BTPS)**

  154 or less                  60 or less               1.45

  155-159                      61-62                    1.55

  160-164                      63-64                    1.65

  165-169                      65-66                    1.75

  170-174                      67-68                    1.85

  175-179                      69-70                    1.95

  180 or more                  71 or more               2.05
  ---------------------------- ------------------------ ------------------

**3.05** *\[Reserved.\]*

**3.06 *Pneumoconiosis*** (demonstrated by appropriate imaging
techniques). Evaluate under the appropriate criteria in 3.02.

**3.07 *Bronchiectasis*** (demonstrated by appropriate imaging
techniques). With:

A. Impairment of pulmonary function due to extensive disease. Evaluate
under the appropriate criteria in 3.02;

*Or*

B. Episodes of bronchitis or pneumonia or hemoptysis (more than
bloodstreaked sputum) or respiratory failure (documented according to
3.00C), requiring physician intervention, occurring at least once every
2 months or at least six times a year. Each inpatient hospitalization
for longer than 24 hours for treatment counts as two episodes, and an
evaluation of at least 12 consecutive months must be used to determine
the frequency of episodes.

**3.08 *Mycobacterial, mycotic, and other chronic persistent infections
of the lung*** (see 3.00B). Evaluate under the appropriate criteria in
3.02.

**3.09 *Cor pulmonale secondary to chronic pulmonary vascular
hypertension****.* Clinical evidence of cor pulmonale (documented
according to 3.00G) with:

A. Mean pulmonary artery pressure greater than 40 mm Hg;

*Or*

B. Arterial hypoxemia. Evaluate under the criteria in 3.02C2;

*Or*

C. Evaluate under the applicable criteria in 4.02.

**3.10 *Sleep-related breathing disorders***. Evaluate under 3.09
(chronic cor pulmonale), or 12.02 (organic mental disorders).\
\
**3.11 *Lung transplant.*** Consider under a disability for [36]{.mark}
months [or indefinitely]{.mark} following the date of surgery;
thereafter, evaluate the residual impairment([s]{.mark}).

[For individuals with cystic fibrosis]{.mark} [who received lung
transplant, evaluate under listings for respiratory (including sinus),
growth]{.mark} [impairment, digestive,]{.mark} [endocrine]{.mark} [and
hepatobiliary]{.mark} [systems]{.mark} [to assess multi-system impact of
disease.]{.mark} [Also consider impact]{.mark} [of transplant]{.mark}
[on health, such as episodes of rejection, side effects of transplant
medications, fractures, liver]{.mark} [dysfunction, bronchiolitis
obliterans, as well as the development of other complications such as
diabetes]{.mark} [mellitus]{.mark} [and cancer.]{.mark}