2. Identifying a Claim for Beryllium Illness. The first step a CE must undertake is to determine the type of claim being filed. Generally, this is accomplished by identifying the type of medical condition marked on the claim form by the person making a claim for benefits. If the EE-1 or EE-2 has been checked for beryllium sensitivity or chronic beryllium disease (CBD), a claim for a beryllium illness has been identified.

In some instances, claimants may identify another respiratory illness or check the “other lung condition” box on the EE-1 or EE-2. Respiratory illnesses may consist of, but are not limited to, chronic obstructive pulmonary disease (COPD), pulmonary fibrosis, etc. Examiners may develop these conditions using the CBD criteria that exist under Part B of the EEOICPA; however, the CE must notify the claimant of the statutory requirements for CBD claims and any deficiencies in the medical evidence. If the condition is clearly not CBD (e.g., asbestosis) the claim does not need to be developed as such under Part B. It may still be necessary to evaluate the claim in light of evidentiary requirements for Part E.

3. Proof of a Covered Beryllium Illness for Beryllium Sensitivity. The CE must verify that sufficient medical evidence is presented by the person making a claim under the Act in order to substantiate a covered beryllium illness. Under Part B of the statute, there are specific diagnostic requirements depending on the beryllium illness being claimed.

a. A claimant establishes beryllium sensitivity by submitting the results of either one beryllium lymphocyte proliferation test (BeLPT) or one beryllium lymphocyte transformation test (BeLTT) performed on blood or lung lavage cells showing abnormal findings. The abnormal BeLPT/BeLTT must be evaluated by a physician with the findings/interpretation definitely outlined (i.e., abnormal response to beryllium). A BeLPT/BeLTT exhibiting a “borderline” result is not sufficient to establish beryllium sensitivity.

b. The CE should not attempt to interpret the BeLPT/BeLTT and formulate conclusions as to the findings in the absence of an evaluation of the findings by a physician. If the interpretation is not present, the results must be referred to the treating physician or DMC for an appropriate interpretation.

4. Proof of CBD before January 1, 1993. 42 U.S.C. §7384l(13) describes the evidence that must be present to establish a claim for CBD under Part B of the Act. To determine whether to use the pre or post-1993 CBD criteria, the medical evidence must document that the employee was either treated for, tested for or diagnosed with a chronic respiratory disorder. If the earliest dated documentation is prior to 1993, the pre-1993 CBD criteria may be used. If the employee sought care for a respiratory illness prior to 1993, but the earliest dated document is after January 1, 1993, the post-1993 CBD criteria must be used. This date would then be the principle basis for determining the appropriate set of criteria to be used in evaluating the CBD claim.

A pre-1993 CBD claim must include at least three of the following medical documentation: characteristic chest radiographic (or computed tomography (CT)) abnormalities, restrictive or obstructive lung physiology testing or diffusing lung capacity defect, lung pathology consistent with chronic beryllium disease, a clinical course consistent with a chronic respiratory disorder or immunologic tests showing beryllium sensitivity (skin patch test or beryllium blood test preferred).

a. Characteristic chest radiograph (x-ray). In a chest x-ray, rays are emitted through the chest of an individual and the image is projected onto film creating a picture of the image. Characteristic chest x-ray findings are identified by the following:

(1) Opacities distributed throughout all the lung fields. Mixtures of round and irregular areas of opacity are also often seen.

(2) Other characteristic x-ray findings include: interstitial lung fibrosis, interstitial or pleural fibrosis (pleural fibrosis alone is not enough, there must be other findings too), granulomas (non-calcified and non-caseating).

(3) Caseating granulomas can at times be considered characteristic; however, these findings should be reviewed by a medical consultant for a determination. Caseating is a gross term that identifies necrosis (decay) in the center of a granuloma. Caseating was originally applied to granuloma associated with tuberculosis or fungal infection. A non-caseating granuloma is one without necrosis and is characteristic of CBD.

(4) Calcification in a granuloma is usually associated with healing of the granuloma. A calcified granuloma is not characteristic of CBD.

(5) In advanced CBD, coarse linear fibrosis may be found resulting in progressive loss of lung volume.

b. A Computed Tomography (CT) scan uses X-rays to produce detailed pictures of structures inside the body. Each X-ray pulse lasts only a fraction of a second and represents a “slice” of the organ or area being studied. A CT scan is sometimes referred to as a CAT (computed axial tomography) scan.

CT scan abnormalities indicative of CBD may include the following:

(1) Consolidation, ground glass, septal thickening, diffuse nodules (different distributions), interstitial fibrosis, bronchiectasis and honeycombing.

(2) Other CT scan findings may include parenchymal nodules, septal lines, patches of ground-glass attenuation, bronchial wall thickening and thickening of the interlobular septa. Nodules are often seen clustered together around the bronchi or in the subpleural region. Subpleural clusters of nodules may form pseudo plaques. In advanced CBD, large subpleural cysts may be found.

c. The following chart represents radiographic (x-ray/CT) patterns characteristic of CBD.

Chest x-ray CT/HRCT (high-resolution computed tomography)

Alveolar Patterns Alveolar Patterns

- Consolidation - Consolidation

- Ground glass - Ground glass

Interstitial Patterns Interstitial Patterns

- Reticular(irreg. lines) - Septal thickening

- Diffuse Nodules - Diffuse Nodules

- Reticulonodular (different distributions)

- Ground glass

Interstitial Fibrosis Interstitial Fibrosis

- Honeycombing - Traction Bronchiectasis

- Upper lobe retraction - Honeycombing

The above radiographic patterns represent characteristic chest x-ray and CT abnormalities. In CBD claims containing the above-listed chest x-ray and CT scan abnormalities, the DEEOIC staff may accept these diagnostic findings as being either characteristic or denoting abnormalities consistent with CBD.

d. Restrictive or obstructive lung physiology testing or diffusing lung capacity defect. Obstruction (i.e., severe, mild, etc.) is the most common abnormality found by spirometry. Severe obstruction prevents complete exhalation (air trapping). A definitive diagnosis of restriction (reduced lung volumes) through spirometry cannot be made without lung volumes. Generally, the pulmonary function studies include the physician’s interpretation of whether there is restriction or obstruction.

e. Arterial blood gas (ABG) tests may not be used in lieu of a pulmonary function test. There are many factors that are involved in interpreting an ABG test. If the CE is unable to obtain a pulmonary function test and the ABG test is the only test available, the ABG result along with the medical evidence of record must be reviewed by a medical consultant for a determination on whether it is indicative of restrictive or obstructive lung physiology. An ABG test generally does not show a diffusing lung capacity defect.

f. Lung pathology consistent with chronic beryllium disease is generally revealed in the interpretation provided with in the pathology report. If the interpretation is not provided or if the CE is unsure whether the findings are consistent with CBD, the CE should obtain clarification from the treating physician or the DMC.

g. Clinical course consistent with chronic respiratory disorder may include the following disorders and methods of treatment:

(1) Hypoxemia: supplemental oxygen and supplies.

(2) Air Flow Obstruction (COPD, Emphysema) Asthma/wheezing like symptoms: inhalers (e.g. Flovent, Advair, Serevent, Albuterol, etc.), corticosteroid drugs, bronchodilators, oxygen therapy.

(3) Right heart failure, Cor pulmonale: Cardiology consult and subsequent management, diuretics (e.g. Lasix, HCTZ, Spironolactone, etc.), digoxin and supplemental oxygen. Be aware that these medications and methods of treatment may be used in the treatment of disorders other than right heart failure, such as systemic hypertension and coronary artery disease. Keep in mind also that just because a medication or method of treatment is not listed here, does not signify that an individual does not have heart failure.

(4) Pulmonary Hypertension: Cardiology consult and subsequent management, supplemental oxygen, epoprostenol, treprostinil, iloprost, and sildenafil.

(5) Respiratory infections (Pneumonia, Acute bronchitis): Antibiotics, sputum cultures, blood cultures, sometimes bronchoscopy.

(6) Sarcoidosis: corticosteroid drugs, such as prednisone.

h. Immunologic tests showing beryllium sensitivity (skin patch or beryllium blood test preferred). Examples of immunologic tests may include test results involving the interaction of antigens with antibodies.

5. Proof of CBD on or after January 1, 1993.

a. The medical documentation must include an abnormal BeLPT/BeLTT performed on either blood or lung lavage cells, together with lung pathology consistent with CBD and include one or more of the following: a lung biopsy showing granuloma or a lymphocytic process consistent with CBD; a computerized axial tomography (CAT) scan showing changes consistent with CBD; or a pulmonary function or exercise testing showing pulmonary deficits consistent with CBD.

b. A lung biopsy showing granulomas or a lymphocytic process consistent with CBD;

(1) Lung Biopsy. The term lung biopsy is interpreted as any sampling of lung tissue. Lung tissue samples directly indicative of lung pathology may include any one of the following:

(a) Lung tissue obtained from whole lung specimens at the time of autopsy;

(b) Lung tissue obtained by open or video-assisted thoracotomy;

(d) Lung tissue obtained by bronchoalveolar lavage (which includes alveolar and bronchial epithelial cells, macrophages, lymphocytes, neutrophils, eosinophils, and other lung cells).

Tissue samples obtained by any one of these methods can be used to document a lymphocytic process consistent with CBD.

(2) Gold Standard. In claims that contain a normal or borderline LPT and the lung tissue biopsy confirms the presence of granulomas consistent with CBD, the CE may accept the claim for CBD. The lung biopsy is considered the "gold standard." However, the following steps must be followed before accepting a claim in this manner.

(a) If the claimant is living, the CE should contact the treating physician and obtain a detailed narrative report detailing the past history of the claimant’s LPT results (if possible). Specifically, the physician should address whether the claimant has a past history of positive LPT’s with recent normal or borderline LPT results. The CE should note that if the claimant has a history of steroid use, this may cause a false negative on the LPT result. A repeat LPT may be warranted in this instance.

(b) If the claimant is deceased, the CE should try to obtain as much information as possible on past LPT results and possible steroid use. If exhaustive efforts produce little or no results and the claim contains the normal/borderline LPT result along with a biopsy of the lung tissue showing the presence of granulomas, the CE may accept the claim.

In these instances, the tissue evidence must be very obvious and the recommended decision must address all the statutory requirements for CBD claims in a well- reasoned manner. (i.e. LPT negative due to steroid medications giving a "false negative").

The CE should review the evidence to ascertain whether other medical causes for pathological abnormalities have been eliminated, in order to determine if the lung pathology is consistent with CBD. Possible medical causes can include: cancer, TB, drug use, mycobacteria, fungal disease, lymphoma, sarcoidosis, or other foreign bodies.

c. Lymphocytic Process Consistent with CBD. A lymphocytic process consistent with CBD can be measured in the lungs by any one of the following methods:

(1) biopsies showing lymphocytes (part of the population of so called mononuclear cells) in bronchial or interstitial (alveolar) lung tissue;

(2) biopsies showing non-caseating granuloma;

(3) bronchoalveolar lavage (BAL) showing an increase in the percentage of lymphocytes in the differential cell count (typically >10% lymphocytes is considered a BAL lymphocytosis);

(4) BAL Beryllium Lymphocyte Proliferation Test showing that the lymphocytes washed from the lungs exhibit a pathologic ability to respond to beryllium salts.

An abnormal BeLPT test performed on either blood or lung lavage cells and lung tissue obtained through a positive BAL BeLPT showing a lymphocytic process and established by a physician as being consistent with CBD, are sufficient to support the diagnosis of CBD. This is especially important when the BAL BeLPT is the only test used in the diagnosis. However, the CE may not use a positive BAL BeLPT solely to support a claim for CBD on or after January 1, 1993.

d. A computerized axial tomography (CAT) scan showing changes consistent with CBD. A CAT scan uses X-rays and computers to produce an image of a cross-section of the body. For post-1993 CBD claims, the results of the CAT scan must be evaluated by a physician for a determination on whether the findings are consistent with CBD. An opinion on whether the findings are consistent with CBD should be obtained through the treating physician or a medical consultant. Note: This statutory requirement is different from the statutory requirement for a CT scan discussed in 4(b) and 4(c) of this chapter.

e. Pulmonary function or exercise testing showing pulmonary deficits consistent with CBD. For this criterion, the results of the pulmonary function study or exercise tests must be evaluated by a physician for a determination on whether the deficits are consistent with CBD. An opinion on whether the findings are consistent with CBD should be obtained through the treating physician or a medical consultant.

6. Making decisions using the totality of evidence. The fact that the pre-1993 criteria is more generalized speaks to the fact that before 1993, it was difficult to confirm beryllium sensitization. As such, the respiratory problems potentially linked to beryllium were often misdiagnosed or thought to be linked to other causal factors. After 1993, diagnostic measures were in place that could reliably identify a patient’s sensitivity to beryllium and link it to the potential onset of CBD. The CE must be aware that the post-1993 criteria are viewed as being significantly more accurate for confirming or negating the existence of beryllium sensitization or CBD.

During the claims process, the claims staff will encounter claims containing medical evidence which support a chronic respiratory disorder and meet 3 of the 5 criterion for pre-1993 CBD claims. However, if the totality of the medical evidence indicates that the employee does not have CBD based upon the post-1993 criteria, the pre-1993 criteria should not be used to approve the claim. The CE should always utilize the most current medical evidence when evaluating a CBD claim, and more recent medical findings are given greater weight when reviewing the case as a whole. If the employee is living, he/she must undergo or submit additional medical testing to substantiate the post-1993 CBD criteria. If the most recent evidence (post-1993) does not support a finding of CBD, the CE does not proceed to establish CBD based upon older evidence (pre-1993) that may be contained in the record.

For example, if a claim contains a post-1993 BeLPT yielding normal results and the other medical evidence of record supports the pre-1993 CBD criteria (i.e., 3 of the 5 criteria are met), the claim should not be approved or adjudicated using the pre-1993 CBD criteria. If the employee is alive, he/she must undergo additional testing to satisfy the post-1993 CBD criteria and to determine if the disease is actually present. The exception to this would be if the claim contains a lung biopsy showing granuloma, then the “gold standard” (EEOICPA PM Chapter 2-0700(4)(b)(2)) would be applicable. In all other instances, the claims staff must consider the totality of the evidence when issuing a determination. Under no circumstances should a claimant be advised to submit evidence supporting the pre-1993 CBD criteria or be advised that the claim may be considered or adjudicated for pre-1993 CBD when the current medical evidence indicates that the employee does not have CBD.

In any instance where the evidence or record documents both pre and post-1993 evidentiary criteria for CBD, the CE must defer to the findings substantiated by the post-1993 criteria.

7. Required tests may not be interchanged. EEOICPA case files must contain the specified test results that are required for each criterion or the test results must be documented in a narrative medical report. For example, DEEOIC staff may not accept a characteristic chest x-ray (pre-93 criteria) in lieu of a CAT scan showing changes consistent with CBD (post-93 criteria).

8. Referring case files to the District Medical Consultants (DMC) for review. CE’s should refer claims to the DMC for medical review if they are unable to interpret the medical reports and/or tests and have no success with obtaining clarification from the treating physician or have a specific question(s) on the medical evidence (See PM Chapter 2-300.9-Developing Medical Evidence). If a referral is made, all medical records must be sent to the DMC for review.

The DMC’s determination, at times, may be dependent upon the totality of the evidence. If the findings contained in the test result are clear, the CE should not refer the claim for review by a DMC. If the findings are not clear, a referral is necessary.

Examples of situations when a referral is needed may include:

a. Medical tests are submitted which do not provide a clear interpretation (i.e., pathology report, LPT, x-ray, CT scan).

b. Pre-1993 medical evidence is submitted that includes a lung biopsy report that is inconclusive.

c. Pre-1993 test (lung pathology) and/or Post-1993 tests (CAT scan, lung biopsy showing granulomas, lymphocytic process, pulmonary function or exercise) is submitted and does not contain the finding “consistent with chronic beryllium disease.”

The opinion of the DMC, when properly supported by medical rationale, carries significant probative value and should be considered reliable when issuing the recommended and/or final decision.

9. Issuing a beryllium sensitivity decision when CBD is claimed. In a situation where a claim for a living employee is submitted and CBD is claimed on the EE-1, but the evidence supports the existence of beryllium sensitivity only, the CE must still develop the claim for CBD.

a. Advising the Claimant. The CE must advise the claimant of the medical evidence necessary to establish a claim for CBD, and provide the claimant with a reasonable amount of time to submit the evidence.

b. Claimant Response. If the claimant responds with additional evidence, the CE evaluates the claim and issues a recommended decision accepting the beryllium sensitivity (if established) and either accepting or denying the claim for CBD.

(1) If the claimant either does not respond within the allotted period of time, or provides evidence that he or she has not yet developed CBD, the CE issues a recommended decision accepting the claim for beryllium sensitivity. The CE also sends a letter to the claimant advising that there is currently no evidence of CBD, but that if the beryllium sensitivity later develops into CBD, the claimant should contact the office and provide medical evidence.

(2) If the claimant later advises the office of the development of CBD, the CE develops the case accordingly and issues a decision based upon the medical evidence the claimant submits.

(3) If the claimant advises that he/she would like a decision on the CBD immediately, despite lack of supporting evidence, the CE issues a denial of the CBD.

10. Common consequential illnesses stemming from CBD or the treatment of CBD. At times, individuals diagnosed with CBD may develop an illness as a consequence of this condition or the treatment thereof, especially when the patient uses steroids, such as Prednisone. These conditions may include, but are not limited to: weight gain; elevated blood pressure; hypertension; elevated cholesterol and abnormal lipids; liver function abnormalities; blood sugar change; diabetes; eye/vision problems such as cataracts, glaucoma, and visual acuity changes; gastrointestinal conditions such as gastric reflux or peptic ulcers; psychiatric or psychological conditions such as depression or anxiety; skin problems such as thrush or other fungal infections; metabolic changes such as folic acid depletion; decreased immune response leading to infections and viruses; sleep apnea and other sleep disorders; deconditioning requiring pulmonary rehabilitation, physical therapy, and/or nutritional counseling; and decreased bone density leading to osteoporosis/ osteopenia. Before developing and rendering a decision on a consequential injury, the CE should refer to the EEOICPA Procedure Manual Chapter 2-1000 (Consequential Injuries).