Disability Doc

6.06 Nephrotic syndrome, with anasarca, persisting for at least 3 months despite prescribed therapy (see 6.00E5). With:

A. Serum albumin of 3.0 g per dL (100 ml) or less and proteinuria of 3.5 g or greater per 24 hours.

OR

B. Proteinuria of 10.0 g or greater per 24 hours.

Nephrotic syndrome - a general name for a group of diseases involving defective kidney glomeruli (filtration system) characterized by:

• Heavy proteinuria - large amount of protein in the urine
• Hypoalbuminemia – low albumin levels in the blood
• Varying degrees of edema –swelling

Anasarca - generalized massive edema (swelling) of soft tissues
Proteinuria - excess protein in the urine (most accurately measured with a 24 hour urine test)
Serum albumin - a major protein found in the blood that transports drugs and other substances, and is important for keeping fluid from leaking out of blood vessels into the tissues

In nephrotic syndrome, damaged kidneys leak albumin into the urine. Albumin normally helps keep fluid from leaking out of blood vessels into the soft tissues of the body. When albumin is lost (hypoalbuminemia), fluid leaks out of the blood vessels and cause swelling (edema).

“Serum albumin” is found in a blood test and “proteinuria,” as it pertains to this listing, is found in a 24 hour urine test.

Anasarca must be “persisting for at least 3 months despite prescribed therapy” to meet either Listing 6.06 A. or B.

SSA will evaluate complications of nephrotic syndrome including:

• Orthostatic hypotension (blood pressure drops with changes in body position such as moving from lying to standing)
• Recurrent infections (increased the risk of infections)
• Venous thrombosis (increased risk of blood clots forming in veins)

The longitudinal medical record should include a description of prescribed therapy, response to therapy, side effects of therapy, and expected duration of treatment.

Treatment may involve diuretics to control swelling and blood pressure medications to control high blood pressure. Corticosteroids, such as prednisone, can cause thinning of bone (osteoporosis) and skin; cataracts; destruction of hip and/or shoulder joints due to avascular necrosis (AVN); infections, diabetes, muscle wasting, rounding of faces; and psychiatric disturbances.

Anasarca may cause shortness of breath and be associated with congestive heart failure if not controlled.

Causes of nephrotic syndrome include:

• Glomerulonephritis (inflammation of glomeruli)
• Toxins, drugs, chemicals
• Infections
• Diabetes Mellitus
• Hypertension
• Systemic Lupus Erythematosis
• Multiple Myeloma
• Hodgkin’s Lymphoma

C. Persistent elevation of serum creatinine to 4 mg per deciliter (dL) (100 ml) or greater or reduction of creatinine clearance to 20 ml per minute or less, over at least 3 months, with one of the following:

4. Persistent anorexia with recent weight loss and current weight meeting the values in 5.08, table III or IV.

Blood and urine tests required for this listing must be documented in the medical records on more than one occasion over a period of at least three months.

Creatinine - normal product of muscle metabolism
Serum creatinine - amount of creatinine in the blood, which measures renal function; determined by a blood sample
Creatinine clearance test - test for renal function based on the rate at which creatinine is excreted by the kidney; determined by a blood sample with a 24 hour urine sample

A specific assessment of an individual's overall residual function cannot be directly correlated with a set abnormality of serum creatinine or creatinine clearance alone. An individual with a serum creatinine less than 4 mg/dl may not have significant loss of function; however, as the creatinine begins to exceed 4 mg/dl, complications of chronic kidney disease and loss of the individual’s overall level of function increases.

Anorexia - loss of appetite

Medical records must show a series of weights with a current weight, and the weight loss must be recent (over the past 3-6 months).

Use weight tables III or IV in listing 5.08 to assess current weight. Height should be measured without shoes.

Significant weight loss can be associated with debilitating fatigue, and sometimes, malnutrition.

C. Persistent elevation of serum creatinine to 4 mg per deciliter (dL) (100 ml) or greater or reduction of creatinine clearance to 20 ml per minute or less, over at least 3 months, with one of the following:

3. Persistent fluid overload syndrome with:
a. Diastolic hypertension greater than or equal to diastolic blood pressure of 110 mm Hg;
OR
b. Persistent signs of vascular congestion despite prescribed therapy (see 6.00B5);

Blood and urine tests required for this listing must be documented in the medical records on more than one occasion over a period of at least three months.

Creatinine - normal product of muscle metabolism.
Serum creatinine - amount of creatinine in the blood, which measures renal function; determined by a blood sample
Creatinine clearance test - test for renal function based on the rate at which creatinine is excreted by the kidney; determined by a blood sample with a 24 hour urine sample

A specific assessment of an individual's overall residual function cannot be directly correlated with a set abnormality of serum creatinine or creatinine clearance alone. An individual with a serum creatinine less than 4 mg/dl may not have significant loss of function; however, as the creatinine begins to exceed 4 mg/dl, complications of chronic kidney disease and loss of the individual’s overall level of function increases.

Fluid overload syndrome - excessive sodium (salt) and water retention in the body that cannot be adequately removed by the diseased kidneys
Vascular congestion - fluid overload of blood vessels

Signs of vascular congestion:

• Ascites - excess fluid in the abdomen
• Pleural effusions – fluid around the lungs
• Pulmonary edema – fluid inside the lungs
• Pericardial effusion – fluid surrounding the heart
• Diastolic hypertension - elevated diastolic blood pressure (the bottom number of a blood pressure reading)
  

Symptoms of vascular congestion include:

• Dyspnea - shortness of breath
• Fatigue
• Weakness

Medical records should show external and/or internal body swelling (edema) with diagnostic tests consistent with persistent fluid overload and repeated elevated blood pressure readings.

Imaging studies, such as a chest X-ray (CXR), Chest CT, Abdominal CT, and ultrasounds can detect internal body fluid overload showing such conditions as pulmonary edema, pleural effusions, and ascites.

This listing is only concerned with the diastolic (bottom) blood pressure reading of 110 mm. or above. (e.g. 180/110).

This listing does not require a specific degree of vascular congestion, but the intent of this listing is that it should be significant enough to prevent substantial gainful activity.

This condition is commonly associated with hypertension (high blood pressure) and congestive heart failure.

C. Persistent elevation of serum creatinine to 4 mg per deciliter (dL) (100 ml) or greater or reduction of creatinine clearance to 20 ml per minute or less, over at least 3 months, with one of the following:

2. Persistent motor or sensory neuropathy (see 6.00E4);

Blood and urine tests required for this listing must be documented in the medical records on more than one occasion over a period of at least three months.

Creatinine - normal product of muscle metabolism.
Serum creatinine - amount of creatinine in the blood, which measures renal function; determined by a blood sample
Creatinine clearance test - test for renal function based on the rate at which creatinine is excreted by the kidney; determined by a blood sample with a 24 hour urine sample

A specific assessment of an individual's overall residual function cannot be directly correlated with a set abnormality of serum creatinine or creatinine clearance alone. An individual with a serum creatinine less than 4 mg/dl may not have significant loss of function; however, as the creatinine begins to exceed 4 mg/dl, complications of chronic kidney disease and loss of the individual’s overall level of function increases.

Neuropathy - a problem in peripheral nerve function (any part of the nervous system except the brain and spinal cord) that causes pain, numbness, tingling, and muscle weakness.
Sensory neuropathy - a neuropathy or polyneuropathy (more than one nerve) involving only the sensory nerves (nerves of feeling)
Motor neuropathy - a neuropathy or polyneuropathy (more than one nerve) involving only the motor nerves (nerves of muscle function)

Medical evidence should document a sensory and/or peripheral neuropathy on physical exam (decreased sensation and or muscle weakness), which might be supported by studies such as EMG/NCS (electromyography/nerve conduction study).

The preamble specifies the longitudinal clinical record must show the neuropathy is a “severe” impairment that has lasted or can be expected to last for a continuous period of at least 12 months. This means it must be “more than a slight abnormality, and has more than a minimal effect on the ability to do basic physical or mental work activities.”
This gives a lot of leeway here because by this definition, the neuropathy does not have to produce motor loss or a gait impairment. There could just be significant enough pain that interferes with the ability to function and work.

C. Persistent elevation of serum creatinine to 4 mg per deciliter (dL) (100 ml) or greater or reduction of creatinine clearance to 20 ml per minute or less, over at least 3 months, with one of the following:

1. Renal osteodystrophy (see 6.00E3) manifested by severe bone pain and appropriate medically acceptable imaging demonstrating abnormalities such as osteitis fibrosa, significant osteoporosis, osteomalacia, or pathologic fractures;

Blood and urine tests required for this listing must be documented in the medical records on more than one occasion over a period of at least three months.

Creatinine - normal product of muscle metabolism.
Serum creatinine - amount of creatinine in the blood, which measures renal function; determined by a blood sample
Creatinine clearance test - test for renal function based on the rate at which creatinine is excreted by the kidney; determined by a blood sample with a 24 hour urine sample

A specific assessment of an individual's overall residual function cannot be directly correlated with a set abnormality of serum creatinine or creatinine clearance alone. An individual with a serum creatinine less than 4 mg/dl may not have significant loss of function; however, as the creatinine begins to exceed 4 mg/dl, complications of chronic kidney disease and loss of the individual’s overall level of function increases.

Renal osteodystrophy - bone disorders usually caused by chronic kidney failure, and may result in bone pain and pathologic fractures (bone breaks due to weakening of bone structure).

Note that bone pain must be "severe," but the listing does not say that it must be intractable or consistent; therefore, intermittent "severe" bone pain would suffice.

Types of renal osteodystrophy include:

• Osteitis fibrosa - fibrous degeneration with weakening and deformity of bone 
• Osteomalacia - softening of bone
• Osteoporosis - thinning of bone with reduction in bone mass resulting from the depletion of calcium and bone protein

In this new listing, SSA has replaced the word “marked” with the word “significant” in the phrase describing osteoporosis. SSA notes they are not changing the degree of osteoporosis required to meet this listing, but in my opinion, changing one vague quantifier (“marked”) for another (“significant”) adds nothing but confusion to this listing.To help satisfy SSA criteria for this listing, I recommend you have the treating doctor quantify any existing osteoporosis as “significant.”

Medically acceptable imaging includes, but is not limited to x-ray imaging, computerized axial tomography (CAT scan), or magnetic resonance imaging (MRI), myelography, and radionuclear bone scans. These tests may be useful in documenting osteodystrophy (bone deterioration), osteoporosis (best test is a DEXA scan), and pathologic fractures due to bone thinning.

Complications of chronic kidney disease and associated loss of an individual’s overall level of function becomes more prevalent the longer chronic renal disease exists. In deciding residual function, SSA must assess the totality of the evidence including the chronicity and degree of renal failure, any complications and associated loss of function, other underlying conditions, and side effects of medications.

6.02 Impairment of renal function, due to any chronic renal disease that has lasted or can be expected to last for a continuous period of at least 12 months. With:

B. Kidney transplantation. Consider under a disability for 12 months following surgery; thereafter, evaluate the residual impairment (see 6.00E2).

End stage renal disease may result in the need for kidney transplantation to provide a healthy kidney that will filter waste from the blood.

The date of surgery for kidney transplantation is the established onset date (EOD) for meeting Listing 6.02 B.

SSA considers an individual disabled for 12 months following the surgery because there is a greater likelihood of rejection of the transplanted kidney and infections due to immunosuppression during the first year.

Signs and symptoms after kidney transplantation are only pertinent if at least 12 months have passed since the surgery, otherwise the individual meets 6.02 B, which recognizes the need for a one year post-operative recovery period.

All individuals who have kidney transplants must take immunosuppressants (medications that suppress the body’s immune system) to prevent "graft versus host disease" (GVHD) and rejection of the transplanted kidney. GVHD is an immune system response that occurs when the transplanted organ is attacked by the individual’s immune system, as if it is foreign material.

After the first year posttransplantation, SSA decides whether an individual is still disabled based on:

• Any residual impairment(s)
• Occurrence of rejection episodes
• Side effects of immunosuppressant drugs, including corticosteroids
• Frequency of any renal infections
• Systemic complications such as other infections, neuropathy, or deterioration of other body systems

In deciding whether an individual is still disabled, SSA considers whether there has been medical improvement of their condition based on symptoms (how you feel), signs (physical findings), and laboratory findings (diagnostic testing including blood work).

Immunosuppressant medications used to prevent GVHD and transplant rejection can cause fluid retention, high blood pressure, diabetes, kidney problems, and infection. Other potential side effects of therapy include itching (pruritus), nausea, vomiting, diarrhea, fatigue, and weight loss.

Corticosteroids, such as prednisone, can cause thinning of bone (osteoporosis) and skin, cataracts, destruction of hip and/or shoulder joints due to avascular necrosis (AVN), infections, diabetes, muscle wasting, rounding of faces, and psychiatric disturbances.

Immunosuppressant drugs, such as corticosteroids increase the risk of infection, which may result in chronic or recurrent bacterial (e.g. Tuberculosis, Listeria, Nocardia), viral (e.g. Hepatitis B & C, Herpes, Cytomegalovirus), and/or fungal (e.g. Candida, Aspergillus, Cryptococcus) infections.

Major causes of death after kidney transplantation include infection, coronary artery disease, liver failure and cancer.

6.02 Impairment of renal function, due to any chronic renal disease that has lasted or can be expected to last for a continuous period of at least 12 months. With:

A. Chronic hemodialysis or peritoneal dialysis (see 6.00E1).

When renal failure worsens to “end stage renal disease” (creatinine clearance of about 10 mg/dl or less), the kidneys no longer effectively filter waste from the blood, and either kidney transplantation or dialysis is needed to keep the patient alive.

Hemodialysis - removal of toxic waste form the blood by filtering it through an artificial kidney machine
Peritoneal dialysis - type of dialysis in which dialyzing solution is introduced and removed from the peritoneal (abdominal) cavity either continuously of intermittently

Listing 6.02 A. cannot be met until an individual actually begins dialysis, though it is not uncommon for individuals to have an arteriovenous (AV) shunt inserted in preparation for dialysis 6-12 months prior to beginning dialysis.

If an individual is undergoing dialysis and meets listing 6.02 A., but the alleged onset date (AOD) is prior to the onset of dialysis in a Title II case, SSA will have to assess evidence for the period prior to meeting 6.02 A to decide if a Residual Functional Capacity (RFC) is appropriate, or to see if the claimant equals or meets another listing for that period.

If a kidney biopsy was performed, evidence should include a copy of the report of the microscopic examination of the kidney tissue (pathology report). If the pathology report of the biopsy is not available, SSA will accept a statement from an acceptable medical source that a biopsy was performed, with a description of the results.