Identification Number:
DI 23022 TN 41
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. 41, 10/20/2020

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 is a Quick Action Transmittal. These revisions do not change or introduce new policy or procedure.

Summary of Changes

DI 23022.390 Edwards Syndrome (Trisomy 18)

  • Updated table style;

  • Added "ICD-10-CM" to section heading;

  • Added ICD-10 information

  • Switched location of "Progression" and "Treatment" sections

DI 23022.830 Myoclonic Epilepsy with Ragged Red Fibers Syndrome

  • Adjusted spacing in "Diagnostic testing" and "Physical findings" sections

DI 23022.937 Child Lymphoblastic Lymphoma

  • Updated table style;

  • Added "ICD-10-CM" to section heading;

  • Added ICD-10 information;

  • Updated spacing of bulleted lists in "Diagnostic testing" and "Physical findings"

DI 23022.947 Erdheim Chester Disease

  • Added "ICD-10-CM" to section heading;

  • Added ICD-10 information;

  • Updated spacing of bulleted lists in "Diagnostic testing" and "Physical findings"

DI 23022.390 Edwards Syndrome (Trisomy 18)

COMPASSIONATE ALLOWANCE INFORMATION

EDWARDS SYNDROME

ALTERNATE NAMES

Trisomy 18; Chromosome 18; Trisomy 18 Complete; Complete Trisomy 18 Syndrome; Trisomy E Syndrome

DESCRIPTION

Edwards syndrome is a genetic disorder in which a person has an extra third copy (trisomy) of genetic material from chromosome 18, instead of the usual two copies. This chromosomal condition causes severe intellectual disability and congenital abnormalities. It severely affects all organ systems of the body. Children born with Edwards syndrome often have intellectual disability and delayed development, congenital heart disease, seizures, and physical malformations. Associated conditions may include heart defects such as ventricular septal defect and malformations of the digestive tract, urinary tract, and genitals. Due to the presence of several life-threatening medical problems, many infants die within their first month. This disorder is a relatively common syndrome affecting approximately 1 out of 3,000 live births. It is three times more common in girls than boys.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing : The diagnosis of Edwards syndrome is made by genetic testing of the amniotic fluid; Ultrasounds of the heart and abdomen; and x-rays of the skeletal system.

Physical findings: Individuals with Edwards syndrome are characterized by:

  • Small head size;

  • Small wide-set eyes;

  • Small lower jaw;

  • Clenched hands with second and fifth fingers on top of the others;

  • Other defects of the hands and feet;

  • Low birth-weight;

  • Crossed legs;

  • Webbed neck;

  • Underdeveloped finger nails; and

  • A shortened breastbone.

ICD-9 : 758.2

ICD-10: Q91.3

PROGRESSION

Fifty percent of infants with this condition do not survive beyond the first week of life. Some children have survived to teenage years, but with serious medical and developmental problems.

TREATMENT

Treatment of Edwards syndrome consists of symptomatic and supportive care. Treatment is dependent on the medical condition of the individual and the affected organ defects.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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

  • Laboratory tests showing results of chorionic villus sampling (CVS).

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 23022.830 Myoclonic Epilepsy with Ragged Red Fibers Syndrome

COMPASSIONATE ALLOWANCE INFORMATION

MYOCLONIC EPILEPSY WITH RAGGED RED FIBERS SYNDROME

ALTERNATE NAMES

MERRF; Myoclonus with Epilepsy with Ragged Red Fibers; MERRF Syndrome; Myoencephalopathy Ragged Red Fiber Disease; Fukuhara syndrome; Myoclonic epilepsy associated with ragged red fibers; Myoencephalopathy ragged-red fiber disease

DESCRIPTION

Myoclonic Epilepsy with Ragged Red Fibers (MERRF) is a rare inherited neurometabolic disorder that affects the central nervous system, skeletal muscles, and other body systems. Characteristic abnormal muscle cells appear as ragged red fibers when stained and viewed under a microscope.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing :

  • Muscle biopsy with pathology report documenting structurally abnormal mitochondria;

  • CT/MRI for cerebral atrophy;

  • EEG for seizure activity;

  • Blood serum testing for elevated lactate levels; and

  • Hearing and vision testing.

Physical findings:

  • Neurological dysfunction including cerebellar ataxia;

  • Myoclonic seizures (brief, sudden twitching muscle spasms);

  • Progressive spasticity;

  • Dementia;

  • Muscle atrophy with muscle weakness;

  • Neuropathy;

  • Optic atrophy; and

  • Short stature.

ICD-9: 277.87

ICD-10: E88.40

PROGRESSION

Signs and symptoms of this disorder mostly appear during childhood or adolescence, although sometimes after age 20. Clinical course is variable, from slowly progressive to rapidly downhill.

TREATMENT

There is no current cure for MERRF and treatment is supportive. Medications may be prescribed for seizures and to control muscle movement. Physical therapy and occupational therapy can be used to extend the range of muscle movement and improve dexterity. Vitamin therapies such as riboflavin, coenzyme Q, and carnitine may provide subjective improvement in fatigue and energy levels in some individuals.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Clinical history and examination that describes the diagnostic features of the impairment, and physical and cognitive findings;

  • Muscle biopsy showing the presence of ragged red fibers;

  • Imaging studies such as CT or MRI; and

  • EEG results, vision or hearing testing may be helpful.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

11.02

11.17

111.02

111.17

Equals

11.17

12.02

* 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 23022.937 Child Lymphoblastic Lymphoma

COMPASSIONATE ALLOWANCE INFORMATION

CHILD LYMPHOBLASTIC LYMPHOMA

ALTERNATE NAMES

DESCRIPTION

Child Lymphoblastic Lymphoma is a type of non-Hodgkin lymphoma of the lymphatic system. Child lymphoblastic lymphoma is similar in etiology to acute lymphoblastic leukemia because lymphoblasts infiltrate nodal structures of the bone marrow, spleen, and central nervous system. A difference in this disease is that the tumor may present as a lump in some part of the body, with the most common location being in the abdomen. Occasionally, it may present as a lump in a lymph gland, bone, skin, thyroid gland, or the tonsils.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnostic testing for child lymphoblastic lymphoma may include:

  • Blood and urine tests;

  • Surgical biopsy;

  • Fine needle aspiration biopsy;

  • Bone marrow aspiration and biopsy; and

  • Imaging such as ultrasound, MRI, CT scan and PET scan.

Physical findings: Signs and symptoms of childhood lymphoblastic lymphoma may be different depending on the location of the tumors. Common symptoms may include:

  • High fever for no known cause;

  • Night sweats;

  • Weight loss of more than 10% of body weight in a short period of time (within 6 months);

  • Enlarged liver and kidney;

  • Generalized weakness;

  • Painless swelling of the lymph nodes in the neck, underarm, stomach or groin;

  • Difficulty breathing;

  • Wheezing; and

  • Coughing.

ICD-9: 200.8

ICD-10: C83.5

PROGRESSION

The childhood form of lymphoblastic lymphoma is a disease of rapid onset and progression. In most cases, the disease has progressed to an advanced stage (stage III or IV) by the time of diagnosis. The prognosis depends on histology (structure of the affected tissue); extent of the disease; presence or absence of metastasis; the child’s age; and response to therapy.

TREATMENT

Children with lymphoblastic lymphoma are treated with chemotherapy and radiation, usually following acute leukemia treatment protocols. Chemotherapy often includes intrathecal therapy (injection of chemotherapy into the spinal canal). Radioimmunotherapy is being researched as a possible treatment option.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

  • Clinical history and examination that describes diagnostic features of the disorder and laboratory findings are needed to confirm the diagnosis;

  • Pediatric oncology consultation reports;

  • Imaging studies;

  • Biopsy reports; and

  • Pathology reports.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

113.06 A

Must meet listing level severity.

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 23022.947 Erdheim Chester Disease

COMPASSIONATE ALLOWANCE INFORMATION

ERDHEIM CHESTER DISEASE

ALTERNATE NAMES

Erdheim Chester Syndrome; Lipoid Granulomatosis; Non-Langerhans Cell Histiocytosis; Polyostotic Sclerosing Histiocytosis

DESCRIPTION

Erdheim Chester Disease (ECD) is a rare type of histiocytosis disease that involves an excessive production and accumulation of a certain type of white blood cells (histiocytes) in many different organs of the body. These cells normally are responsible for responding to infection and injury. In ECD, these cells infiltrate the bones (long bones), eyes, pituitary gland and various organ systems (pulmonary, cardiovascular, renal, skin, and central nervous system) causing these tissues and organs to become thickened, dense, and fibrotic. Without treatment, organ failure can occur. Many ECD patients carry gene mutations linked to blood and other cancers. National Institutes of Health researchers have concluded ECD is a type of cancer and should be treated by oncologists.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing:

  • X-rays, CT scans, MRIs, and bone scans indicating abnormal thickening, masses, or lesions;

  • Bronchoscopy with biopsy;

  • Biopsy of tissue; and

  • Pulmonary function tests to provide information on clinical severity

Physical findings : Symptoms and signs depend on specific organ involvement. Symptoms of ECD may include:

  • Bone pain in the long bones of the arms and legs;

  • Weight loss;

  • Night sweats;

  • Muscle and joint aches, weakness and fatigue;

  • Difficulty speaking;

  • Kidney problems;

  • Soft tissue masses or lesions on the body;

  • Bulging eyes due to a mass behind the eye;

  • Vision problems;

  • Shortness of breath;

  • Interstitial lung disease;

  • Increased susceptibility to infections; and

  • Fibrous growth in or around the heart.

ICD-9: 277.89

ICD-10: E88.89

PROGRESSION

ECD occurs in adults, most often in middle age. Age of onset is usually between 40 and 60 years of age. The prognosis for ECD is poor. The mean survival time is less than three years with visceral (organ) involvement. Death typically results from respiratory, heart, and kidney damage.

TREATMENT

Treatment for ECD is symptom-specific and temporary. Treatment with surgical debulking, corticosteroid therapy, interferon, chemotherapy used for hairy cell leukemia, and radiation therapy has been utilized with varying degrees of success.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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

  • Biopsy of affected organ system; and

  • Functional assessment of the organ system involved (for example, pulmonary function tests).

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.06 A

Listing level severity must be documented.

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 41 - Processing Quick Disability Determination (QDD) and Compassionate Allowance (CAL) in the Disability Determination Services (DDS) - 10/20/2020