TN 4 (06-15)
DI 34223.009 Respiratory Listings from 01/02/01 to 05/23/02
103.00 RESPIRATORY SYSTEM
A. Introduction. The listings in this section describe impairments resulting from respiratory disorder
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. Reasonable efforts should be made to ensure evaluation
by a program physician specializing in childhood respiratory impairments or a qualified
pediatrician.
Many children, especially those who have listing-level impairments, will have received
the benefit of medically prescribed treatment. Whenever there is such evidence, 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 child's functional
status. The longitudinal record should provide information regarding functional recovery,
if any.
Some children will not have received ongoing treatment or have an ongoing relationship
with the medical community, despite the existence of a severe impairment(s). A child
who does not receive treatment may or may not be able to show an impairment that meets
the criteria of these listings. Even if a child does not show that his or her impairment
meets the criteria of these listings, the child may have an impairment(s) that medically
or functionally equals the listings. 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 child'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.
Evaluation should include consideration of adverse effects of respiratory impairment
in all relevant body systems, and especially on the child's growth and development
or mental functioning, as described under the growth impairment (100.00), neurological
(111.00), and mental disorders (112.00) listings.
It must be remembered that these listings are only examples of common respiratory
disorders that are severe enough to find a child disabled. When a child has a medically
determinable impairment that is not listed, an impairment that does not meet the requirements
of a listing, or a combination of impairments no one of which meets the requirements
of a listing, we will make a determination whether the child's impairment(s) medically
or functionally equals the listings. (See ยงยง404.1526, 416.926, and 416.926a.)
B. Documentation of Pulmonary Function Testing. The results of spirometry that are used for adjudication, under the 103.02A and B,
103.03, and 103.04 of these listings should be expressed in liters (L), body temperature
and pressure saturated with water vapor (BTPS). The reported one-second forced expiratory
volume (FEV1) 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 FEV1
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 FEV1 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 FEV1 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 FEV1 value is
less than the appropriate reference value in table I or III, as appropriate. If a
bronchodilator is not administered, the reason should be clearly stated in the report.
Pulmonary function studies should not be performed unless the clinical status is stable
(e.g., the child is not having an asthmatic attack or suffering from an acute respiratory
infection or other chronic illness). Wheezing is common in asthma, chronic bronchitis,
or chronic obstructive pulmonary disease and does not preclude testing. 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 a child from
performing age-appropriate activities, 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 103.02 and 103.04 must only be used as criteria for the level of ventilatory impairment
that exists during the child's most stable state of health (i.e., any period in time
except during or shortly after an exacerbation). 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.
The appropriately labeled spirometric tracing, showing the child's name, date of testing,
distance per second on the abscissa and 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 child should
be noted, and it should be stated whether or not a BTPS correction factor was used
for the calibration recordings and for the child'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 FEV1 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 child's ability
to understand directions, as well as his or her efforts and cooperation in performing
the pulmonary function tests.
Purchase of a pulmonary function test is appropriate only when the child is capable
of performing reproducible forced expiratory maneuvers. This capability usually occurs
around age 6. Purchase of a pulmonary function test 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 pulmonary function tables in 103.02 and 103.04 are based on measurement of standing
height without shoes. If a child 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.
C. Documentation of chronic impairment of gas exchange.
1. Arterial blood gas studies (ABGS). An ABGS performed at rest (while breathing room air, awake and sitting or standing)
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. Before purchasing resting ABGS, a program physician, preferably
one experienced in the care of children with pulmonary disease, must review the clinical
and laboratory data short of this procedure, including spirometry, to determine whether
obtaining the test would present a significant risk to the child.
2. Oximetry. Pulse oximetry may be substituted for arterial blood gases in children
under 12 years of age. The oximetry unit should employ the basic technology of spectrophotometric
plethysmography as described in Taylor, M.B., and Whitwain, J.G., "Current Status
of Pulse Oximetry," "Anesthesia", Vol. 41, No. 9, pp. 943-949, 1986. The unit should
provide a visual display of the pulse signal and the corresponding oxygen saturation.
A hard copy of the readings (heart rate and saturation) should be provided. Readings
should be obtained for a minimum of 5 minutes. The written report should describe
patient activity during the recording, i.e., sleep rate, feeding, or exercise. Correlation
between the actual heart rate determined by a trained observer and that displayed
by the oximeter should be provided. A statement should be made in the report of the
child's effort and cooperation during the test.
Purchase of oximetry 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.
D. Cystic fibrosis is a disorder that affects either the respiratory or digestive body systems or both
and may impact on a child's growth and development. It is responsible for a wide and
variable spectrum of clinical manifestations and complications. Confirmation of the
diagnosis is based upon an elevated sweat sodium concentration or chloride concentration
accompanied by one or more of the following: the presence of chronic obstructive pulmonary
disease, insufficiency of exocrine pancreatic function, meconium ileus, or a positive
family history. The quantitative pilocarpine iontophoresis procedure for collection
of sweat content must be utilized. Two methods are acceptable: the "Procedure for
the Quantitative Iontophoretic Sweat Test for Cystic Fibrosis," published by the Cystic
Fibrosis Foundation and contained in, "A Test for Concentration of Electrolytes in
Sweat in Cystic Fibrosis of the Pancreas Utilizing Pilocarpine Iontophoresis," Gibson,
I.E., and Cooke, R.E., "Pediatrics", Vol 23: 545, 1959; or the "Wescor Macroduct System."
To establish the diagnosis of cystic fibrosis, the sweat sodium or chloride content
must be analyzed quantitatively using an acceptable laboratory technique. Another
diagnostic test is the "CF gene mutation analysis" for homozygosity of the cystic
fibrosis gene. The pulmonary manifestations of this disorder should be evaluated under
103.04. The nonpulmonary aspects of cystic fibrosis should be evaluated under the
listings for the digestive system (105.00) or growth impairments (100.00). Because
cystic fibrosis may involve the respiratory and digestive body systems, as well as
impact on a child's growth and development, the combined effects of this involvement
must be considered in case adjudication.
Medically acceptable imaging includes, but is not limited to, x-ray imaging, computerized
axial tomography (CAT scan) or magnetic resonance imaging (MRI), with or without contrast
material, myelography, and radionuclear bone scans. "Appropriate" means that the technique
is the proper one to support the evaluation and diagnosis of the impairment.
E. Bronchopulmonary dysplasia (BPD). Bronchopulmonary dysplasia is a form of chronic obstructive pulmonary disease that
arises as a consequence of acute lung injury in the newborn period and treatment of
hyaline membrane disease, meconium aspiration, neonatal pneumonia and apnea of prematurity.
The diagnosis is established by the requirement for continuous or nocturnal supplemental
oxygen for more than 30 days, in association with characteristic changes on medically
acceptable imaging and clinical signs of respiratory dysfunction, including retractions,
rales, wheezing, and tachypnea.
103.01 Category of Impairments, Respiratory System.
103.02 Chronic pulmonary insufficiency. With:
A. Chronic obstructive pulmonary disease, due to any cause, with the FEV1 equal to
or less than the value specified in Table I corresponding to the child's height without
shoes. (In cases of marked spinal deformity, see 103.00B.);
Height without shoes (Centimeters)
|
Height without shoes (Inches)
|
FEV1 equal to or less than (L, BTPS)
|
119 or less
|
46 or less
|
0.65
|
120 - 129
|
47-50
|
0.75
|
130 - 139
|
51-54
|
0.95
|
140 - 149
|
55-58
|
1.15
|
150 - 159
|
59-62
|
1.35
|
160 - 164
|
63-64
|
1.45
|
165 - 169
|
65-66
|
1.55
|
170 or more
|
67 or more
|
1.65
|
OR
B. Chronic restrictive ventilatory disease, due to any cause, with the FVC equal to
or less than the value specified in Table II corresponding to the child's height without
shoes. (In cases of marked spinal deformity, see 103.00B.);
Height without shoes (Centimeters)
|
Height without shoes (Inches)
|
FVC equal to or less than (L, BTPS)
|
119 or less
|
4 or less
|
0.65
|
120 - 129
|
47-50
|
0.85
|
130 - 139
|
51-54
|
1.05
|
140 - 149
|
55-58
|
1.25
|
150 - 159
|
59-62
|
1.45
|
160 - 164
|
63-64
|
1.65
|
165 - 169
|
65-66
|
1.75
|
170 or more
|
67 or more
|
2.05
|
OR
C. Frequent need for:
1. Mechanical ventilation; or
2. Nocturnal supplemental oxygen as required by persistent or recurrent episodes of
hypoxemia;
OR
D. The presence of a tracheostomy in a child under 3 years of age;
OR
E. Bronchopulmonary dysplasia characterized by two of the following:
1. Prolonged expirations; or
2. Intermittent wheezing or increased respiratory effort as evidenced by retractions,
flaring and tachypnea; or
3. Hyperinflation and scarring on a chest radiograph or other appropriate imaging
techniques; or
4. Bronchodilator or diuretic dependency; or
5. A frequent requirement for nocturnal supplemental oxygen; or
6. Weight disturbance with:
a. An involuntary weight loss (or failure to gain weight at an appropriate rate for
age) resulting in a fall of 15 percentiles from established growth curve (on standard
growth charts) which persists for 2 months or longer; or
b. An involuntary weight loss (or failure to gain weight at an appropriate rate for
age) resulting in a fall to below the third percentile from established growth curve
(on standard growth charts) which persists for 2 months or longer;
OR
F. Two required hospital admissions (each longer than 24 hours) within a 6-month period
for recurrent lower respiratory tract infections or acute respiratory distress associated
with:
1. Chronic wheezing or chronic respiratory distress; or
2. Weight disturbance with:
a. An involuntary weight loss (or failure to gain weight at an appropriate rate for
age) resulting in a fall of 15 percentiles from established growth curve (on standard
growth charts) which persists for 2 months or longer; or
b. An involuntary weight loss (or failure to gain weight at an appropriate rate for
age) resulting in a fall to below the third percentile from established growth curve
(on standard growth charts) which persists for 2 months or longer;
OR
G. Chronic hypoventilation (PaCO2 greater than 45 mm Hg) or chronic cor pulmonale
as described under the appropriate criteria in 104.02;
OR
H. Growth impairment as described under the criteria in 100.00.
103.03 Asthma. With:
A. FEV1 equal to or less than the value specified in Table I of 103.02A;
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 inpatient 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;
OR
C. Persistent low-grade wheezing between acute attacks or absence of extended symptom-free
periods requiring daytime and nocturnal use of sympathomimetic bronchodilators with
one of the following:
1. Persistent prolonged expiration with radiographic or other appropriate imaging
techniques evidence of pulmonary hyperinflation or peribronchial disease; or
2. Short courses of corticosteroids that average more than 5 days per month for at
least 3 months during a 12-month period;
OR
D. Growth impairment as described under the criteria in 100.00.
103.04 Cystic fibrosis. With:
A. An FEV1 equal to or less than the appropriate value specified in Table III corresponding
to the child's height without shoes. (In cases of marked spinal deformity, see 103.00B.);
OR
B. For children in whom pulmonary function testing cannot be performed, the presence
of two of the following:
1. History of dyspnea on exertion or accumulation of secretions as manifested by repetitive
coughing or cyanosis; or
2. Persistent bilateral rales and rhonchi or substantial reduction of breath sounds
related to mucous plugging of the trachea or bronchi; or
3. Appropriate medically acceptable imaging evidence of extensive disease, such as
thickening of the proximal bronchial airways or persistence of bilateral peribronchial
infiltrates;
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 treatment;
OR
D. Episodes of bronchitis or pneumonia or hemoptysis (more than blood-streaked 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 period of at least 12 consecutive months must be used to determine the
frequency of episodes;
OR
E. Growth impairment as described under the criteria in 100.00.
Height without shoes (Centimeters)
|
Height without shoes (Inches)
|
FEV1 equal to or less than (L, BTPS)
|
119 or less
|
46 or less
|
0.75
|
120 - 129
|
47-50
|
0.85
|
130 - 139
|
51-54
|
1.05
|
140 - 149
|
55-58
|
1.35
|
150 - 159
|
59-62
|
1.55
|
160 - 164
|
63-64
|
1.85
|
165 - 169
|
65-66
|
2.05
|
170 or more
|
67 or more
|
2.25
|
103.05 Lung transplant. Consider under a disability for 12 months following the date of surgery; thereafter,
evaluate the residual impairment(s).