Proposed Revised Medical Criteria for
Evaluating Chronic Liver Disease in Adults

Intoduction:

Chronic liver disease is a general term used to cover a heterogeneous group of etiologies. By common usage, chronic liver disease is believed to exist when the diagnostic features of the condition persist for at least 6 months. Most commonly, and in part dependent on the etiology, chronic hepatitis is recognized because of the discovery of persistently elevated serum enzymes (often inappropriately referred to as “liver function tests”) such as the alanine or aspartate aminotransferases (ALT or SGPT; AST or SGOT) or the alkaline phosphatase. However, chronic liver disease can be present even in the absence of abnormal serum enzymes, in which case it may come to attention because of the existence of certain non-specific symptoms such as excessive fatigue, malaise, poor appetite, difficulty in sleeping, depression, etc., or physical manifestations such as upper gastrointestinal bleeding, ascites, peripheral edema, pruritis, psychological or neurological dysfunction, etc.  The common thread in chronic hepatitis, largely independent of the etiology, is the development of scarring of the liver (fibrosis) that has the potential to progress slowly but inexorably to cirrhosis and, in certain instances, culminate in liver failure or the development of hepatocellular carcinoma (liver cancer). The likelihood of developing liver cancer varies according to the etiology, the frequency being high in some instances and negligible in others. 

It must be noted that cirrhosis is itself a dynamic process that begins and exists for many years in the absence of symptoms (compensated cirrhosis) but has the potential to advance to “liver failure” (decompensated cirrhosis), recognized by dysfunction of certain biochemical indices (falling serum albumin, prolongation of the prothrombin time or INR, increasing serum bilirubin) or the physical features noted above. Whereas the development of cirrhosis has long been viewed as an irreversible process, recent evidence suggests that treatment of some forms of chronic liver disease associated with cirrhosis, probably early in its course, may slow or even reverse the process. It is important to note that even though manifest symptoms may not be present at a given or at all times, the disease process may still be advancing and shorten life expectancy. Not all persons with cirrhosis will die as a consequence of liver disease, but a significant proportion will, either as a direct consequence of the failing liver or because of associated complications, such as infections or renal failure. Moreover, advancing cirrhosis clearly adversely affects quality of life. Cirrhosis is undoubtedly a serious condition.

There are many causes for chronic liver disease in the United States. These include, in descending order of frequency, chronic hepatitis C virus (HCV) infection; HCV together with chronic alcoholism; non-alcoholic fatty liver disease, in particular, non-alcoholic steatohepatitis (NASH); a combination of etiologies that include autoimmune hepatitis, primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC); hemochromatosis, drug-induced liver disease, Wilson disease, alpha-1 antitrypsin deficiency, and cryptogenic cirrhosis; chronic alcoholism alone, and hepatitis B virus (HBV) infection. Some of these, HCV infection in particular (and to a lesser extent HBV), can also induce extrahepatic manifestations, such as cryoglobulinemia, glomerulonephritis, porphyria cutanea tarda, lichen planus, salivary gland lesions (sicca syndrome), and Mooren corneal ulcer. 

With this background, we will respond to the invitation from the Social Security Administration to comment on the proposed revised medical criteria for evaluating chronic liver disease, beginning with the Introductory Text focusing on the Adult Criteria and concluding with comments on the Proposed Adult Digestive Listings for Chronic Liver Disease. In so doing, we struggled with the issue of distinguishing between a disease process that has the high likelihood of advancing to serious clinical manifestations or even death within a given time period (as determined by assessment of the Child-Pugh-Turcotte [CPT] or Model for End Stage Liver Disease [MELD] scoring systems but, in rare instances, without presently existing listing-value disability), and simply listing the disability criteria that, in our view, warrant consideration for support. We chose, with some reluctance, not to use these severity scales. We are aware that the disability should be expected to persist for at least one year, and while this may not be the case for some of our suggested listings, these latter that we have selected clearly are indicative of an impending serious outcome.


Introductory Text: 

Proposed Listing 5.05 – Chronic Liver Disease: Federal Register, Vol. 66 No. 220, p 57013

We propose to remove “portal, postnecrotic, or biliary cirrhosis” in the current listing 5.05 and replace it with “cirrhosis of any kind.” …… We also propose to remove “Wilson’s disease” and “chronic active hepatitis” from the examples of chronic liver disease because hepatic impairment due to Wilson disease and chronic active hepatitis is included in the revised term “cirrhosis of any kind.”

We agree with this proposition.

We propose to revise listing 5.05A, esophageal varices, by defining our criteria for massive hemorrhage. By providing a specific transfusion requirement, we intend to exclude minor variceal bleeding which would not be an indicator of listing level severity.
Newer techniques in primary prevention and treatment of esophageal varices . . . .Thereafter, we will evaluate your residual impairment(s).

We agree that there should be a listing for upper gastrointestinal bleeding but we have extended it to include other sources of bleeding in persons with portal hypertension and suggest that the number of units of blood be reduced from 5 over a 48 hour period (we are not aware of the source of this requirement) to 2 units given because of hemodynamic instability.

We are proposing to change current listing 5.05D, ascites due to chronic liver disease to 5.05B. We propose to clarify how the persistence of ascites over 6 months must be demonstrated. We are revising. . . .and to ensure that we are evaluating separate episodes.

We agree with the importance of listing ascites, but we have added hydrothorax that not uncommonly accompanies ascites or may be present as a consequence of portal hypertension even in the absence of ascites. We agree that the ascites must be evident on physical examination and not simply identified solely by an imaging procedure. We agree also that the ascites should be resistant to routine diuretic therapy and should require periodic paracenteses for treatment. We believe also that even if the ascites is not intractable but there is superadded spontaneous bacterial peritonitis, that this combination is sufficiently serious as to warrant separate listing because of consideration for liver transplantation

We believe that there are other clinical manifestations of chronic liver disease, not currently shown, that should be listed.  Some may be already listed in other sections of the document but we believe strongly that they should appear in this section. An example is hepatic encephalopathy that currently is listed and defined in the section on neurology. It is our belief that hepatic encephalopathy is better identified and managed by health care personnel caring for individuals with chronic liver disease and, hence, should be clearly displayed in this section on chronic liver disease. 

Our greatest dilemma is the issue of fatigue. We unquestionably believe that some persons with chronic liver disease, especially those with chronic hepatitis C, can have profound, disabling fatigue, even individuals without end-stage liver disease. The problem, of course, is that fatigue is a subjective symptom that, as far as we know, is not definable by a scientifically validated process. Clearly, fatigue can be real and intrinsic, can be fabricated for financial or other gain, can be psychologically stimulated by new knowledge of an infection, or can be precipitated by treatment of the underlying infection. Unless an acceptable definition has been established elsewhere in this document, assessment of the validity of claimed fatigue will need to rest on the judgment of the caring physician or other health care worker.

Proposed Listing 5.09 – Liver Transplant
We propose that you should be considered under a disability for 12 months following the surgery, due to the nature and course of recovery for this procedure. After that time, we will evaluate the residual impairment(s). This is consistent with our criteria for assessing other organ transplant, such as kidney and heart.

We agree with this modification.


Our Proposed Adult Digestive Listings:
5.05 Chronic liver disease and cirrhosis of any kind, WITH:

A. Bleeding as a result of portal hypertension from esophageal, gastric or ectopic 
      gastrointestinal varices, or from portal hypertensive gastropathy demonstrated   
      by X-ray, endoscopy, or other appropriate medically acceptable imaging, with 
      hemorrhage attributed to one or other of these findings, which leads to 
      hemodynamic instability requiring the transfusion of 2 or more units of blood. 
      Consider under a disability for 1 year following the last documented 
      hemorrhage for hemodynamic instability; thereafter, evaluate the residual 
      impairment(s).

	Bi. Intractable ascites, documented on physical examination as moderate to 
                 severe, or hydrothorax, poorly controlled by or unresponsive to diuretic 
                 treatment, requiring paracenteses for control;

	Bii. Ascites, complicated by spontaneous bacterial peritonitis (SBP), defined as 
                   the presence in the ascitic fluid of an absolute neutrophil count of >250 mm3.

C.  Hepatic encephalopathy causing mental confusion or dysfunction sufficiently         
       severe to interfere with normal daily activity;

D. Liver disease-related renal failure (hepatorenal syndrome [HRS], 
cryoglobulinemia, glomerulonephritis)

E. Intractable pruritis, interfering with normal daily activities and/or with sleep, 
      unresponsive to routine treatment.

F. Persistent and severe fatigue, unrelated to treatment, that interferes with 
      normal daily activity.