Identification Number:
DI 23022 TN 23
Intended Audience:See Transmittal Sheet
Originating Office:ORDP ODP
Title:Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS)
Type:POMS Transmittals
Program:All Programs
Link To Reference:
 

PROGRAM OPERATIONS MANUAL SYSTEM
Part DI – Disability Insurance
Chapter 230 – Special Issues
Subchapter 22 – Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS)
Transmittal No. 23, 02/07/2019

Audience

PSC: CS, DEC, DTE, IES, RECONR;
OCO-OEIO: CR, ERE, FCR, FDE, RECONE;
OCO-ODO: DE, DEC, DS, RECONE;
ODD-DDS: ADJ, DHU;

Originating Component

ODP

Effective Date

Upon Receipt

Background

This transmittal contains updates POMS sections DI 23022.320 through DI 23022.775. Revisions include updates to the content to comply with the Social Security Administration’s plain language initiative and updates to policy guidance.

 

Summary of Changes

DI 23022.320 Small Cell Lung Cancer

• Revised the structure of the summary to improve usability and flow of information,

• Revised “Diagnostic Testing and Coding” section heading to “Diagnostic Testing, Physical Findings, and ICD-9-CM Coding,”

• Revised “Onset and Progression” section heading to “Progression,”

• Added information about physical findings that occur with each condition,

• Added ICD-9-CM coding information,

• Updated the information in the “Suggested MER for Evaluation” section,

• Revised “Meets Listing” heading to “Meets,”

• Revised “Medical Equals” heading to “Equals,”

• Revised “Listing of Impairments” to “the listings,”

• Removed last updated information, and

• Removed component identifying information.

 

DI 23022.325 Small Intestine Cancer

• Revised the structure of the summary to improve usability and flow of information,

• Revised “Diagnostic Testing and Coding” section heading to “Diagnostic Testing, Physical Findings, and ICD-9-CM Coding,”

• Revised “Onset and Progression” section heading to “Progression,”

• Added information about physical findings that occur with each condition,

• Added ICD-9-CM coding information,

• Updated the information in the “Suggested MER for Evaluation” section,

• Revised “Meets Listing” heading to “Meets,”

• Revised “Medical Equals” heading to “Equals,”

• Revised “Listing of Impairments” to “the listings,” • Removed last updated information,

• Removed component identifying information, and

  • Added "Physical findings."

 

DI 23022.760 Hydranencephaly

• Revised the structure of the summary to improve usability and flow of information,

• Revised “Diagnostic Testing and Coding” section heading to “Diagnostic Testing, Physical Findings, and ICD-9-CM Coding,”

• Revised “Onset and Progression” section heading to “Progression,”

• Added information about physical findings that occur with each condition,

• Added ICD-9-CM coding information,

• Updated the information in the “Suggested MER for Evaluation” section,

• Revised “Meets Listing” heading to “Meets,”

• Revised “Medical Equals” heading to “Equals,”

• Revised “Listing of Impairments” to “the listings,”

• Removed last updated information, and

• Removed component identifying information.

 

DI 23022.765 Hypocomplementemic Urticarial Vasculitis Syndrome

• Revised the structure of the summary to improve usability and flow of information,

• Revised “Diagnostic Testing and Coding” section heading to “Diagnostic Testing, Physical Findings, and ICD-9-CM Coding,”

• Revised “Onset and Progression” section heading to “Progression,”

• Added information about physical findings that occur with each condition,

• Added ICD-9-CM coding information,

• Updated the information in the “Suggested MER for Evaluation” section,

• Revised “Meets Listing” heading to “Meets,”

• Revised “Medical Equals” heading to “Equals,”

• Revised “Listing of Impairments” to “the listings,”

• Removed last updated information, and

• Removed component identifying information.

 

DI 23022.770 Hypophosphatasia--Perinatal (Lethal) and Infantile Onset Types

• Revised the structure of the summary to improve usability and flow of information,

• Revised “Diagnostic Testing and Coding” section heading to “Diagnostic Testing, Physical Findings, and ICD-9-CM Coding,”

• Revised “Onset and Progression” section heading to “Progression,”

• Added information about physical findings that occur with each condition,

• Added ICD-9-CM coding information,

• Updated the information in the “Suggested MER for Evaluation” section,

• Revised “Meets Listing” heading to “Meets,”

• Revised “Medical Equals” heading to “Equals,”

• Revised “Listing of Impairments” to “the listings,”

• Removed last updated information, and

• Removed component identifying information.

 

DI 23022.775 I Cell Disease

• Revised the structure of the summary to improve usability and flow of information,

• Revised “Diagnostic Testing and Coding” section heading to “Diagnostic Testing, Physical Findings, and ICD-9-CM Coding,”

• Revised “Onset and Progression” section heading to “Progression,”

• Added information about physical findings that occur with each condition,

• Added ICD-9-CM coding information,

• Updated the information in the “Suggested MER for Evaluation” section,

• Revised “Meets Listing” heading to “Meets,”

• Revised “Medical Equals” heading to “Equals,”

• Revised “Listing of Impairments” to “the listings,”

• Removed last updated information, and

• Removed component identifying information.

 

DI 23022.320 Small Cell Lung Cancer

COMPASSIONATE ALLOWANCE INFORMATION

SMALL CELL LUNG CANCER

ALTERNATE NAMES

Small Cell Lung Carcinoma; Oat Cell Lung Cancer; Mixed Small Cell/Large Cell Lung Carcinoma; Combined Small Cell Lung Carcinoma

DESCRIPTION

There are two types of Lung Cancers: Small Cell and Non Small Cell. Small Cell Cancer of the Lung is an aggressive (fast-growing) cancer that forms in tissues of the lung and can spread to other parts of the body. Small cell lung cancer looks small and oval-shaped under a microscope.

Risk factors for small cell lung cancer include: smoking cigarettes, cigars or pipes, now or in the past, exposure to second-hand smoke and exposure to asbestos, or radon.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CODING

Diagnostic testing: The diagnosis of small cell lung cancer is confirmed by a pathologist using laboratory studies obtained by bronchoscopy or a CT-guided procedure (fine-needle biopsy). Specimens (stains) for chromogranin, neuron-specific enolase and synaptophysin are usually positive. Staging of the carcinoma is performed by imaging studies (CT scans, MRI, PET scans). These studies provide the information needed for staging at diagnosis, response to treatment, resectionability and metastases.

Physical findings:

• Persistent cough;

• Wheezing;

• Hoarseness;

• Hemoptysis (expectoration of blood or of blood-stained sputum);

• Dyspnea (shortness of breath);

• Chest pain;

• Fatigue;

• Decreased appetite; and

• Weight loss.

ICD-9: 162.9

PROGRESSION Although this disease is very responsive to chemotherapy, the overall survival rate is poor. Of those diagnosed with extensive small cell lung carcinoma (most patients) the 2 year survival rate is < 2%.

TREATMENT

Management of limited stage small cell lung carcinoma involves a combination of chemotherapy and thoracic radiation therapies. Treatment can involve either a single modality or a series of multiple modalities. If a complete remission is obtained, prophylactic cranial radiation is offered. At this level of treatment, the disease is potentially curable. However, most individuals are diagnosed with extensive disease and are generally considered incurable but may achieve remission with the use of a combination chemotherapy regimen.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: A pathology report of a lung biopsy.
Suggested Listings for Evaluation:
DETERMINATION LISTINGS REMARKS
Meets

13.14 B

Small cell lung cancer currently meets the criteria in listing 13.14.
Equals
* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

DI 23022.325 Small Intestine Cancer

COMPASSIONATE ALLOWANCE INFORMATION

SMALL INTESTINE CANCER

ALTERNATE NAMES Small Intestine Adenocarcinoma; Small Intestine Sarcoma; Small Intestine Gastrointestinal Stromal Tumor; Small Intestine Carcinoid; Small Intestine Carcinoma

DESCRIPTION

Small Intestine Cancer forms in tissues of the small intestine. The most common type is adenocarcinoma. Most of these tumors occur in the part of the small intestine near the stomach. They may grow and block the intestine.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CODING

Diagnostic testing: The following may be used to diagnose the disease:

• Physical exam and history;

• Laboratory tests;

• X-rays;

• Barium enema;

• Fecal occult blood test (FOBT);

• Endoscopy;

• Biopsy;

• CT scan; and/or

• Surgery.

Physical findings: Physical findings for small intestine cancer may include:

• Blood in the stool (feces);

• Dark/black feces;

• Diarrhea;

• A lump in the abdomen;

• Pain or cramps in the abdomen;

• Unexplained weight loss; and

• Episodes of abdominal pain that may be accompanied by severe nausea or vomiting.

ICD-9: 152

PROGRESSION The overall 5-year survival rate for resectable adenocarcinoma is only 20%.

TREATMENT

Treatment may include surgery, radiation, biologic therapy, and/or chemotherapy.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:
  • A pathology report;

  • An operative report; or

  • A physician's opinion that indicates the cancer is inoperable or unresectable based on described objective findings.

“Inoperable” refers to a physician's opinion that surgery would not be beneficial based on a review of imaging studies, laboratory results, and physical examination findings. “Unresectable” cancer is established when the operative report indicates that the cancer is not completely removed or the pathology report notes that the surgical specimen has positive margins.
Suggested Listings for Evaluation:
DETERMINATION

LISTING

REMARKS
Meets 13.17 Small Intestine Cancer that is inoperable, unresectable, recurrent, or with distant metastases meets the criteria in listing 13.17.
Equals
* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

DI 23022.760 Hydranencephaly

COMPASSIONATE ALLOWANCE INFORMATION

HYDRANENCEPHALY

ALTERNATE NAMES

Hydroanencephaly

DESCRIPTION

Hydranencephaly is a rare brain malformation in which the brain’s cerebral hemispheres do not develop, and are replaced by sacs filled with cerebrospinal fluid. Hydranencephaly is considered an extreme form of porencephaly (a disorder characterized by a cyst or cavity in the cerebral hemispheres), and may be caused by intrauterine strokes or infections, or maternal trauma. Hydranencephaly should not be confused with hydrocephalus, which is an increased accumulation of cerebrospinal fluid within the ventricles.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: The diagnosis is established by imaging such as MRI, CT, after ruling out conditions that are amenable to treatment. Preliminary diagnosis can be made in utero by ultrasound, but must be confirmed after birth.

Physical findings: Infants with hydrancephaly appear normal at birth, but within a few weeks, they may show:

• Irritability;

• Abnormal breathing patterns; and

• Increased muscle tone.

Other features developing over time may include:

• Seizures;

• Abnormal head growth;

• Deafness;

• Blindness;

• Spastic quadriparesis (paralysis); and

• Cognitive deficits.

ICD-9: 742.3

PROGRESSION

Hydranencephaly develops after the 12th week of pregnancy. The prognosis is generally poor, and many children die before the age of 1 year.

TREATMENT

There is no definitive treatment for hydraencephaly. Treatment is symptom-specific and supportive.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Clinical history and examination that describes the diagnostic features of the impairment;

  • Cranial MRI or CT scan; and

  • Developmental assessment.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

110.08 A

 

Equals

 

 

* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

DI 23022.765 Hypocomplementemic Urticarial Vasculitis Syndrome

COMPASSIONATE ALLOWANCE INFORMATION

HYPOCOMPLEMENTEMIC URTICARIAL VASCULITIS SYNDROME

ALTERNATE NAMES

Hypocomplementemic Vasculitis; Urticarial Vasculitis; McDuffie Syndrome

DESCRIPTION

Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS) is a rare type of chronic autoimmune inflammation of small blood vessels and abnormally low levels of complement. HUVS is often associated with systemic diseases such as COPD, systemic lupus, and Sjögren syndrome.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: Laboratory tests of serum documenting complement deficiency and positive C1q antibody, and skin or organ biopsy documenting leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis. The clinical criteria for diagnosis of HUVS include characteristic chronic urticaria with residual hyperpigmentation, and inflammatory vasculitis with angioedema, glomerulonephritis, chronic obstructive pulmonary disease, arthritis, or uveitis.

Physical findings: HUVS usually involves the skin with painful urticaria (hives), and may cause inflammatory changes in joints, kidneys, gastrointestinal tract, lungs, heart, and eyes.

ICD-9: 279.4

PROGRESSION

The prognosis of HUVS depends on the organ system involved. Lung disease results in significant morbidity and mortality, and is made worse by smoking. Kidney involvement with glomerulonephritis may ultimately result in end stage renal disease with need for kidney transplant. Death may also occur due to acute laryngeal edema.

TREATMENT

The treatment of HUVS is determined by the organ involved and severity of the disease, and may include combinations of antihistamines, glucocorticoids and other immunosuppressive medications. COPD and cardiac valvular defects may require specific treatment.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Claimant’s medical source(s) records of clinical history and physical findings;

  • Skin and/or tissue biopsy report;

  • Laboratory reports showing abnormal complement levels and C1q antibody; or

  • Evidence of organ dysfunction, especially eye, renal, cardiac, and respiratory systems (for example, pulmonary function tests).

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

14.02

 

114.02

Equals

3.02 A

* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

DI 23022.770 Hypophosphatasia--Perinatal (Lethal) and Infantile Onset Types

COMPASSIONATE ALLOWANCE INFORMATION

HYPOPHOSPHATASIA--PERINATAL (LETHAL) and INFANTILE ONSET TYPES

ALTERNATE NAMES

Alkaline Phosphotase Deficiency; Perinatal Lethal Hypophosphatasia; Hypophosphatasia Perinatal Lethal Form; Perinatal Rathburn Disease; Phosphoethanolaminuria; Hypophosphatasia Lethal Form; Hypophosphatasia Infantile Onset; Rathburn's Disease

DESCRIPTION

Hypophosphatasia is a rare, inherited metabolic disorder that affects the development of bones and teeth. There are several forms of this disorder, with the perinatal (lethal) and infantile onset types being the most severe.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: Diagnostic testing consisting of subnormally low activity of tissue non-specific alkaline phosphatase (TNALP) enzyme in serum; bone imaging of the bones shows severe demineralization, especially in the metaphyses (the wider part at the extremity of the shaft of a long bone).

Physical findings:

• A near absence of skeletal mineralization;

• Fractures;

• Skin-covered spurs of the knees and elbow;

• Large fontanels (space between the bones of the skull);

• Shortened bones;

• Chest deformities;

• Muscle weakness;

• Hypercalcemia (higher than normal level of calcium in your blood); and

• Multiple congenital abnormalities, leading to respiratory failure.

ICD-9: 275.3

PROGRESSION

For perinatal (lethal) onset hypophosphatasia, death occurs either in-utero or within the first months of life. Infantile onset hypophosphatasia presents within the first year of life, and has 50% mortality. The cause of death is usually due to respiratory failure. Perinatal (benign) hypophosphatasia type is characterized by prenatal skeletal manifestations that slowly resolve into the milder childhood and adult onset hypophosphatasia types, which have variable clinical courses.

TREATMENT

Currently there is no cure or definitive treatment for this disorder.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Clinical history and examination that describes the diagnostic features of the impairment;

  • Laboratory testing for identified enzyme changes; or

  • Imaging documenting decreases in bone mineralization.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

110.08 A

Evaluate the perinatal (lethal) onset type of hypophospatasia, under listing 110.08 A.

110.08 B

Evaluate perinatal (benign), child, and adult onset hypophosphatasia types on a case-by-case basis.

Equals

 

 

* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

DI 23022.775 I Cell Disease

COMPASSIONATE ALLOWANCE INFORMATION

I CELL DISEASE

ALTERNATE NAMES

Mucolipidosis Type II; MLType II; Inclusion Cell disease; Mucolipidosis II Alpha/Beta; ML II; Mucolipidosis 2; ML 2; GNPTA; Leroy Disease; N-acetylglucosamine 1 phosphotransferase deficiency; ML disorder type 2; I Cell Syndrome; Inclusion Cell Syndrome; Inclusion Cell Disease

DESCRIPTION

I Cell Disease is a rare genetic disorder in which the body lacks a critical metabolic enzyme to break down long chains of sugar molecules.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing:

• Genetic tests for mutations in the IDUA gene;

• Testing for the GNPTAB gene;

• Testing for the GNPTA gene that leads to a deficiency in the enzyme UDP-N- acetylglucosamine 1 phosphotransferase; and

• Elevated serum levels of lysosomal enzymes.

Physical findings:

• Psychomotor deterioration;

• Short stature;

• Characteristic coarse facial features with bulging eyes, low nasal bridge, and overgrown gums (gingival hyperplasia);

• Corneal clouding;

• Organomegaly (enlarged organs);

• Growth failure;

• Thickened skin with distinct facial features;

• Generalized hypotonia (low muscle tone);

• Contractures in all large joints;

• Thoracic chest wall deformity;

• Kyphosis (excessive outward curve of spine);

• Clubbed feet;

• Deformed long bones;

• Dislocation of the hips;

• Thickening and insufficiency of the mitral and aortic valves; and

• Progressive mucosal thickening narrows the airways and stiffening of the thoracic cage resulting in respiratory insufficiency.

ICD-9: 272.7

PROGRESSION

Developmental delay and growth failure are the first signs of I Cell Disease, and present in the first year of life. As the child develops, difficulties with motor coordination are evident and manifests with rapid and progressive deterioration. Heart disease and complications of pneumonia are the major causes of death.

TREATMENT

There is no current cure for I Cell Disease. Treatment is supportive. Bone marrow transplantation may be used to delay or correct neurological deterioration. Intravenous treatment with pamidronate may prevent break down of bone tissue, decrease bone pain, and increase mobility.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Clinical history and examination that describes diagnostic features of the impairment;

  • Laboratory tests showing results of genetic testing for mutations in the IDUA gene; and

  • Evidence of neurodevelopmental delay.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

110.08 B

 

Equals

 

 

* Adjudicators may, at their discretion, use the Medical Evidence of Record or the listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.

DI 23022 TN 23 - Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS) - 2/07/2019