1. L isting 7.02: Chronic Anemia (Adults)

The many possible causes of anemia include infections, drugs, toxins, autoimmune diseases, vitamin deficiencies, genetic blood disorders such as sickle cell anemia, and cancer. This listing is applicable to any kind of anemia that is severe and lasts, or is expected to last, 12 months.

a. L isting Level Severity For your condition to be severe enough to meet the listing, you must have chronic anemia due to any cause with a hematocrit persisting at 30% or less.

A You have had one or more blood transfusions on an average of at least once every two months. Whole blood or packed red cell transfusions qualify. To determine the frequency of the transfusions, divide the duration of your anemia in months by the number of incidents in which transfusion was required. Count one incident as one transfusion, even if more than one unit of blood was given.

A need for transfusions to keep your hematocrit up to 30% implies that without the transfusions your anemia would be much worse. Also, your hematocrit will go down, reflecting a need for transfusion, and then rise up to a correct level after the transfusion. Because of the rise and fall, you will be considered more impaired than people with a steady hematocrit of 30%.

B. Other impairments associated with the anemia should be evaluated under the criteria of whatever listings are appropriate. For example, sickle cell anemia can cause strokes and should be evaluated under Listing 11.04

b. R esidual Functional Capacity The major limiting factors in anemia are weakness and easy fatigability. This is because fewer red cells than needed are carrying oxygen to the body’s tissues. Also, the heart rate increases in an attempt to compensate for the anemia, meaning the heart has less reserve to handle exertion. For example, if your resting heart rate is increased to 100 beats per minute because of anemia, you’ve already used up a significant amount of your heart rate reserved for exercise.

Still, anemia affects each person differently. A young person is less affected than an elderly one, as are people in better overall physical condition. If your hematocrit is only a couple of percentage points below normal, you would not have any restrictions—­assuming that is your only problem. On the other hand, if your hematocrit is only a couple of percentage points over that required by the listing— say 33%—but otherwise you qualify under Part A, your RFC should be for no more than sedentary work. Of course, if you suffer from other impairments, the SSA should consider the effect of your anemia upon them. For example, the presence of significant anemia can markedly worsen the limitations imposed by heart or lung disease.

2. L isting 107.03: Hemolytic Anemia (Children)

The many possible causes of hemolytic anemia include drugs, autoimmune diseases, infection, and genetic disorders. Genetic disorders resulting in easily destructible red blood cells are a significant cause of hemolytic anemia in children. Such disorders may make the red cells more susceptible to rupture by affecting the outer membranes of red cells or by producing abnormal forms of hemoglobin within the red cells (hemoglobinopathies) or by causing deficiencies of important enzymes that red cells need to function. A common type of genetic hemolytic anemia resulting from hemoglobinopathy is sickle cell anemia; for children, that disorder is separately considered under Listing 107.05.

Two important enzyme deficiencies in children causing hemolytic anemia are pyruvate kinase deficiency and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency.

a. L isting Level Severity

For the child’s condition to be severe enough to meet the listing, the child must have hemolytic anemia due to any cause, manifested by the persistence of a hematocrit of 26% or less despite prescribed therapy. Additionally, the child’s reticulocyte count must be 4% or greater.

There are other sickle cell diseases besides sickle cell anemia and sickle cell trait. A person could inherit Hb S from one parent and a different kind of abnormal hemoglobin from the other parent—for example, hemoglobin S and hemoglobin C (Hb SC). Hemoglobin SC disease results in abnormalities like sickle cell anemia, but generally less severe. There is also a disorder called thalassemia. In thalassemia, the normal hemoglobin is made at a decreased rate.

A reliable way of diagnosing any type of sickle cell disease is by means of a test called hemoglobin electrophoresis. Hemoglobin electrophoresis separates and measures the types of hemoglobin present, using a blood sample. The SSA must have the results of hemoglobin electrophoresis to make an accurate diagnosis of sickle cell disease. A screening test called a sickle cell preparation is not acceptable as a means of diagnosing sickle cell disease.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have sickle cell disease or one of its variants. Additionally, you must satisfy A, B, C, or D,

A. Documented painful thrombotic crises (also known as vaso-occlusive crises) occurring at least three times during the five months before disability determination. The sickle shape of the abnormal red blood cells makes it difficult for them to move through blood vessels and blood flow may be further worsened by clotting in the affected vessel. Complete blockage of blood flow results in death of the tissue served by that vessel. The associated symptoms depend on the organ involved and the size of the infarction. Bone infarctions can be quite painful; infarctions of the brain produce strokes; infarctions of the heart may result in heart failure; infarctions of the lung can cause chest pain and pneumonia; and infarctions of the kidneys can result in kidney failure. Bone infarction can be triggered by a number of things—most frequently exposure to cold—but also infection, dehydration, and pregnancy. Painful bone infarctions can involve the spine, breastbone, long bones in the arms and legs, the ribs, and pelvic bones. It is important that your treating doctor document your pain in your medical records.

B. Sickle cell disease requiring extended hospitalization (beyond emergency care) at least three times during the 12 months before disability determination. Hospitalization may be required due to aplastic episodes, in which the hematocrit falls rapidly because of the bone marrow’s inability to produce enough red cells. Hospitalization might also be the result of hyperhemolytic crises, in which the unusually rapid destruction of red cells causes a sudden fall in hematocrit. Other possible complications to sickle cell disease that could result in hospitalization include strokes, heart attacks, heart failure, kidney failure, and pneumonia.

C. Chronic, severe anemia with persistence of hematocrit of 26% or less. The SSA should apply this requirement reasonably. For example, if your hematocrit values are consistently low with a single value of 27%, you should not be denied benefits. Remember that if your hematocrit is as high as 30%, you might still be granted benefits under Listing 7.02A when you receive blood transfusions.

D. Evaluate the resulting impairment under the criteria for the affected body system. For example, a stroke would be evaluated under Listing 11.04

4. L isting 107.05: Sickle Cell Disease (Children)

a. L isting Level Severity

For the child’s condition to be severe enough to meet this listing, the child must have sickle cell disease or one of its variants. Additionally, the child’s condition must satisfy A, B, C, D, or E,

A Recent, recurrent, severe vaso-occlusive crises involving the musculoskeletal system (including the spine) or abdominal organs (liver, spleen, etc.). This is similar to part A under Listing 7.05. The SSA does not define recent or recurrent.

The more severe and prolonged the crises, the fewer episodes would be required to be disabling. Using adult Listing 7.05 as a guide, the child would have to have at least three episodes during the five months before disability determination.

B A major complication involving a large organ during the 12 months before application for disability benefits. Complications might include meningitis, lung infection (pneumonia) or infarction, osteomyelitis, heart failure, kidney infarction, liver infarction, intestinal infarction, or cerebrovascular accident. A major complication would ordinarily be one that required hospitalization, although the listing includes no such requirement. The fact that the child may have improved by the time you apply for benefits does not prevent qualification.

C A hyperhemolytic or aplastic crisis within 12 months before the application for disability benefits. The fact that the child may have improved by the time you apply for disability does not prevent qualification.

D Chronic, severe anemia with persistence of hematocrit of 26% or less. This is the same as part C under adult Listing 7.05. or the appropriate child mental disorder listings. This is essentially the same as part D under adult Listing 7.05.

5. L isting 7.06: Chronic Thrombocytopenia (Adults)

Several disorders, including leukemia, myelofibrosis, drugs, toxic substances, immune diseases, and genetic disorders, can cause decreased platelets. This lowered platelet count (thrombocytopenia) can result in repeated episodes of serious, even life-threatening, bleeding. Furthermore, idiopathic thrombocytopenia is of unknown cause. Platelets circulating in the blood help prevent serious bleeding by sticking together at sites where bleeding tries to start; they are also important in starting the coagulation process. Platelet counts are easy to make with a sample of blood and are routinely done with a complete blood count (CBC). Spontaneous bleeding starts without any traumatic cause.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have chronic thrombocytopenia due to any cause, with platelet counts repeatedly below 40,000 per mm3. Additionally, you must satisfy A or B, below.

A At least one episode of spontaneous bleeding within five months of the date of disability determination requiring transfusion.

B Intracranial (within the head) bleeding within 12 months before disability determination.

6. L isting 107.06: Chronic Idiopathic Thrombocytopenic Purpura (Children)

The comments about thrombocytopenia under Listing 7.06 are relevant to child applicants, although they are not exactly the same. Unlike the adult listing, this child listing concerns only one disorder involving a low platelet count—idiopathic thrombocytopenic purpura (ITP).

a. L isting Level Severity

For the child’s condition to be severe enough to meet the listing, the child must have chronic idiopathic thrombocytopenic purpura and platelet counts of 40,000 per mm3 or less despite prescribed therapy or recurrent upon withdrawal of treatment.

7. L isting 7.07: Hereditary Telangiectasia (Adults)

Hereditary telangiectasia is a disorder associated with a tendency to form abnormal blood vessels that can affect any organ or other living tissue. The danger of this disorder is that life-threatening bleeding may occur, such as hemorrhaging in the lungs, intestine, or brain.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have hereditary telangiectasia with bleeding requiring transfusion at least three times during the five months before your disability determination.

8. L isting 7.08: Hemophilia and Other Coagulation Disorders (Adults)

Coagulation is the ability of the blood to form clots, and is caused by a long complex chain of chemical reactions involving a number of proteins in the blood called coagulation factors. Coagulation factors are made in the liver.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have coagulation defects (hemophilia or a similar disorder) with spontaneous hemorrhage requiring transfusion at least three times during the five months before disability determination.

9. L isting 107.08: Inherited Coagulation Disorders (Children)

a. L isting Level Severity For the child’s condition to be severe enough to meet the listing, the child must have an inherited coagulation disorder. Additionally, the child’s condition must satisfy A or B, below.

A Repeated spontaneous or inappropriate bleeding. Inappropriate bleeding means bleeding starting as a result of trauma (such as a mild bump or fall) that would not cause bleeding in a normal person or that results in excessive bleeding.

B Bleeding into a joint with resulting joint deformity. Marked deformities would obviously qualify—such as fixation of the joint in a particular position (ankylosis), destruction of the joint, determined by x-ray, or instability of the joint requiring a brace to walk; however, this listing does not say that a marked joint deformity is necessary. It is reasonable to expect that significant— more than slight—joint damage is required to produce a deformity, and that the child would have some difficulty walking.

10. L isting 7.09: Polycythemia Vera (Adults)

Polycythemia vera is a serious disease of the bone marrow resulting in abnormally increased numbers of red blood cells, white blood cells, platelets, and blood volume. Also, the patient usually has an enlarged spleen.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have polycythemia vera with erythrocytosis, splenomegaly, and leukocytosis or thrombocytosis. The SSA will evaluate any resulting impairment under the listings dealing with the affected body system.

b. R esidual Functional Capacity Medical judgment must be applied to individual cases, taking into account the response to treatment and the nature and extent of major organ damage. Even without organ damage, the presence of increased blood volume (indicated by a high hematocrit, especially of 60% or more) can produce headaches, weakness, and easy fatigability. It is important that you have your symptoms clearly documented, especially regarding your functional limitations and preferably in your treating doctor’s medical records.

11. L isting 7.10: Myelofibrosis (Adults)

Myelofibrosis (myeloproliferative syndrome) means a change of normal bone marrow into fibrotic tissue.

The three most serious complications of myelofibrosis are: • anemia secondary to the loss of red blood cells • recurrent bacterial infections secondary to decreased white blood cells, and • bone pain created by a bone abnormality called osteosclerosis. Myelofibrosis must be diagnosed by a bone marrow biopsy. Your treating doctor must perform the biopsy; the SSA will not order this test because it is invasive.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have A, B, or C, below,

A Chronic anemia. Evaluate according to Listing 7.02.

B Documented recurrent systemic bacterial infections occurring at least three times during the five months before disability determination.

C Severe and intractable bone pain with x-ray evidence of osteosclerosis.

b. R esidual Functional Capacity No absolute rules can be given. Early myelofibrosis might produce no symptoms or limitations, but at a severe stage you could be restricted to as low as sedentary work. Your symptoms and resulting functional limitations need to be documented in your treating doctor’s medical records. If you have anemia, see the RFC discussion under Listing 7.02.

12. L isting 7.15: Granulocytopenia (Adults)

Granulocytopenia refers to low levels of certain types of white blood cells called granulocytes, especially neutrophils. Neutrophils are important in fighting infection, and a low neutrophil blood count is specifically referred to as neutropenia. Granulocytopenia might be caused by infection, toxic substances, or drugs, or be of unknown cause.

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have chronic granulocytopenia (due to any cause). Additionally, you must satisfy A and B, below.

A Absolute neutrophil counts repeatedly below 1,000 cells/cubic millimeter of blood (1,000 neutrophils per mm3). This number is significant, because when the neutrophil count falls that low, you are in great danger of life-threatening systemic infections.

B Documented recurrent systemic bacterial infections occurring at least three times during the five months before disability determination. Such systemic infections would require treatment in a hospital with intravenous antibiotics.

13. L isting 7.17: Aplastic Anemia With Bone Marrow or Stem Cell Transplant (Adults)

a. L isting Level Severity

For your condition to be severe enough to meet the listing, you must have aplastic anemia with bone marrow or stem cell transplantation. The SSA will consider you to be under a disability for 12 months following transplantation. Thereafter, you will be evaluated according to the type of impairment remaining.