Coagulation Defects

Can I Get Disability Benefits for Coagulation Defects?

If you have a coagulation defect, Social Security disability benefits may be available. To determine whether you are disabled by your coagulation defect, the Social Security Administration first considers whether it is severe enough to meet or equal a listing at Step 3 of the Sequential Evaluation Process. See How to Get Disability Benefits for Coagulation Defects by Meeting a Listing. If you meet or equal a listing because of your coagulation defect, you are considered disabled. If your coagulation defect is not severe enough to equal or meet a listing, the Social Security Administration must assess your residual functional capacity (RFC) (the work you can still do, despite the coagulation defect), to determine whether you qualify for disability benefits at Step 4 and Step 5 of the Sequential Evaluation Process.

About Coagulation Defects and Disability

Abnormal bleeding, called coagulation defects by the Social Security Administration, occurs in diseases that interfere with the clotting of blood. The formation of blood clots is necessary to the body’s ability to stop bleeding. A variety of plasma proteins, some manufactured by the liver, are critical to blood coagulation. Although a number of disorders can affect coagulation, only the hemophilias in severe form are likely to cause the SSA to grant you Social Security disability benefits. See Residual Functional Capacity Assessment for Coagulation Defects.

Hemophilia

The hemophilias are generally classified as hemophilia A and B. Hemophilia A and B occur only in males, and may be of variable degrees of severity. Hemophilia A is about 4 times as frequent as hemophilia B, so most of the coagulation disorders seen by SSA are hemophilia A.

The clinical manifestations seen with the hemophilias involve easy bruising, hematuria, bleeding with minor trauma, bleeding following dental procedures, and bleeding into joints (hemarthroses). A hemarthrosis would not be fatal, although very destructive to a joint. Joint deformity is a common problem in the hemophilia claims seen by the SSA. Intracranial bleeding from a bump on the head, however, could be lethal.

In hemophilia A and B, there is a normal prothrombin time (the time it takes plasma to clot after addition of tissue factor), normal bleeding time, and normal fibrinogen level (fibrogen converts into fibrin, a protein that forms the “mesh” that clots over a wound site during blood coagulation). There is a prolonged partial thromboplastin time (ineffectual coagulation pathways) in both disorders. Hemophilia A is characterized by a low level of normal Factor VIII coagulation protein; hemophilia B involves a low level of normal Factor IX coagulation protein. Coagulation factor assays can determine the activity of these factors in the patient’s blood.

Treatment for Hemophilia

Hemophilia A can be treated by transfusion of Factor VIII concentrate (antihemophilic globulin, AHG). Desmopressin (DDAVP), a synthetic analog of a natural pituitary hormone known as vasopressin, can be used to treat some cases of mild hemophilia A. The desmopressin causes a release of Factor VIII stores in the walls of blood vessels. There is a Factor IX concentrate that can be used for hemophilia B. There is no question that in the relatively near future all hemophilias will be permanently curable with gene therapy; experimentation in lower animals has already been extremely promising.

Related Genetic Disorders

There are other genetic disorders of blood coagulation which involve deficiencies of normal clotting factors other than VIII or IX. For example, there are genetic deficiencies in Factors I, II, V, X, VII and XII.

Von Willebrand’s disease involves a reduction in Factor VIII as in hemophilia A, but is not involved with as severe bleeding problems. Other than in rare cases, bleeding is only mild to moderate and many people are unaware that they have this disorder. Von Willebrand’s Factor (vFW) is an antigenic protein distinct from, but associated with, Factor VIII. Von Willebrand’s Factor is secreted by the cells lining the walls of blood vessels (endothelial cells). Its function is to bind to the wall of the vessel and to receptors on platelets; thus, its function is a first step in the body’s attempt to control bleeding (hemostasis). Treatment of von Willebrand’s disease is not usually necessary, and involves administration of desmopressin or a Factor VIII-vFW concentrate. There are multiple types (Types 1,2,3) of von Willebrand’s disease, associated with multiple types of genetic abnormalities.

Table 7.08.1 Clotting Factor Deficiencies: General Information

Human Factor Deficiency

Normal Circulating Half-life

Estimated Incidence

Bleeding Severity

II

3 days

Rare

Mild to moderate

V

36 hours

1:1 million

Mild to moderately severe

VII

3–6 hours

1:500,000

Mild to severe

VIII

12 hours

1:10,000

Mild to severe

IX

24 hours

1:30,000

Mild to severe

X

40 hours

1:500,000

Mild to severe

XI

80 hours

Rare**

Mild to moderate

XII

50–70 hours

Unknown

No bleeding

XIII

9 days

<1:1 million

Moderate to severe
* Adapted from Hoffman: Hematology: Basic Principles and Practice, 4th ed** Rare except in those of Ashkenazi Jewish descent

 Causes of Bleeding Disorders

While many bleeding disorders are genetic, there are also acquired disorders which can cause coagulation defects. For example, the liver produces several important clotting factors the blood levels of which can fall in the presence of liver disease—especially alcoholic cirrhosis. A very severe disorder called disseminated intravascular coagulation (DIC) is associated most often with obstetrical problems, infection, and malignancy. DIC will result in death unless the underlying cause is quickly corrected. There are disorders in which antibodies form to a coagulation factor and neutralize it. Vitamin K deficiency can lead to coagulation abnormalities.

Continue to How to Get Disability Benefits for Coagulation Defects by Meeting a Listing.