36: Management of Patients With Immune Deficiency Disorders

350 cells/mm3 In general, antiretroviral medications should be offered to individuals with a T-cell count less than 350 cells/mm3 or plasma HIV RNA levels exceeding 100,000 copies/mL.

Headache Early manifestations of HIV encephalopathy include headache, memory deficits, difficulty concentrating, progressive confusion, psychomotor slowing, apathy, and ataxia. Later stages include hyperreflexia, a vacant stare, and hallucinations.

"Although AIDS is an immune deficiency, your child's condition is different from AIDS." Primary immune deficiencies should be not be confused with AIDS. They are not the same condition. In addition, a primary immune disorder does not increase the child's risk for developing AIDS later in life. Primary immune deficiency diseases are serious, but they are rarely fatal and can be controlled. Testing will reveal the evidence of a primary immune disease, not AIDS. AIDS is classified as a seconary immunodeficiency.

Immunosuppressive agents For a client undergoing a hematopoietic stem cell transplant, immunosuppression is started to ensure engraftment of depleted bone marrow. Antibiotic therapy may or may not be indicated. Chest physiotherapy would be appropriate for clients with ataxia-telangiectasis who have chronic lung disase. Anticoagulation would not be used.

Herpes simplex ulcer persisting for 2 months A diagnosis of AIDS cannot be made until the person with HIV meets case criteria established by the Centers for Disease Control and Prevention. The immune system becomes compromised. The CD4 T-cell count drops below 200 cells and develops one of the opportunistic diseases, such as Pneumocystis carinii pneumonia, candidiasis, cytomegalovirus, or herpes simplex.

Common variable immunodeficiency (CVID) More than 50% of clients with CVID develop pernicious anemia. Pernicious anemia is not associated with the other conditions.

AIDS AIDS, the most common secondary disorder, is perhaps the best-known secondary immunodeficiency disorder. It results from infection with the human immunodeficiency virus (HIV). DAF refers to lysis of erythrocytes due to lack of decay-accelerating factor (DAF) on erythrocytes. CVID is a disorder that encompasses various defects ranging from IgA deficiency (in which only the plasma cells that produce IgA are absent) to severe panhypoglobulinemia (in which there is a general lack of immunoglobulins in the blood). Severe combined immunodeficiency disease (SCID) is a disorder in which both B and T cells are missing.

Pernicious anemia More than 50% of clients with CVID develop pernicious anemia. Although chronic diarrhea may occur in clients with CVID, it does not happen in 50% of them. Hypocalcemia and neutropenia are not concerns for clients with CVID.

Flank pain Flank pain, tightness in the chest, or hypotension indicates adverse effects of gamma globulin infusion. Nasal stuffiness is not recognized as an adverse effect of gamma globulin infusion. Increased thirst is not recognized as an adverse effect of gamma globulin infusion. Burning urination is a sign of urinary tract infection, not an adverse effect of gamma globulin infusion.

Antibodies to HIV are not present in his blood. A negative test result indicates that antibodies to HIV are not present in the blood at the time the blood sample for the test is drawn. A negative test result should be interpreted as demonstrating that if infected, the body has not produced antibodies (which take from 3 weeks to 6 months or longer). Therefore, subsequent testing of an at-risk patient must be encouraged. The test result does not mean that the patient is immune to the virus, nor does it mean that the patient is not infected. It just means that the body may not have produced antibodies yet. When antibodies to HIV are detected in the blood, the test is interpreted as positive.

Lymphocyte Because HIV is harbored within lymphocytes, a type of white blood cell, any exposure to infected blood results in significant risk of infection. HIV infection is not harbored in platelets, erythrocytes, or nerve cells.

Avoid fibrous foods, lactose, fat, and caffeine. Diarrhea may subside when the client avoids fibrous foods, lactose, fat, and caffeine. Although eating may seem to cause diarrhea, the client must understand that limiting the intake of food to control diarrhea only exacerbates wasting. The client will tolerate a low-fat, high-carbohydrate, and soft or liquid diet better than large, high-fat meals. The client should be advised to avoid large doses of iron and zinc because they can impair the functioning of the immune system.

testing the client for the presence of HIV Abnormal results of Papanicolaou tests, genital warts, pelvic inflammatory disease, and persistent vaginitis may correlate with HIV infection. Wearing cotton underwear can help with the prevention of candidiasis but does not address the recurrent vaginal infection that may not be caused by a fungus. Abstaining from sexual intercourse does not address the recurrent vaginal infection. A medicated douche can alter the normal flora of the vaginal wall.

Bank autologous blood. Signing the refusal form does not give the client any information about the options that are available and place the client at risk. Banking autologous blood that is self-donated is the safest option for the client. Directed donor blood may be no safer than blood collected from public donors. Those who support this belief say that directed donors may not reveal their high-risk behaviors that put the potential recipient at risk for blood-borne pathogens such as HIV.

Trimethoprim-sulfamethoxazole To prevent and treat Pneumocystis pneumonia, trimethoprim-sulfamethoxazole (Bactrim, Septra) is prescribed. The other medications are antifungals and used to treat candidiasis.

Respiratory or urinary system infections Secondary immunodeficiencies occur as a result of underlying disease processes or the treatment of these disorders, including administration of immunosuppressive agents. Abnormalities of the immune system affect both natural and acquired immunity. Because immunodeficiencies result in a compromised immune system and pose a high risk for infection, careful assessment of the client's immune status is essential. The nurse assesses and monitors the client for signs and symptoms of infection.

The body has not produced antibodies to the AIDS virus.

Bone marrow transplantation Treatment options for SCID include stem cell and bone marrow transplantation.

Inform the patient that it would be beneficial to test for HIV. HIV screening is recommended for all persons who seek evaluation and treatment for STIs. HIV testing must be voluntary and free of coercion. Patients must not be tested without their knowledge. HIV screening after notifying the patient that an HIV test will be performed (unless the patient declines) is recommended in all health care settings. Specific signed consent for HIV testing should not be required. In most settings, general informed consent for medical care is considered sufficient.

"The client may have cryptococcal meningitis and will need to be evaluated by the health care provider." A fungal infection, Cryptococcus neoformans is another common opportunistic infection among clients with AIDS, and it causes neurologic disease. Cryptococcal meningitis is characterized by symptoms such as fever, headache, malaise, stiff neck, nausea, vomiting, mental status changes, and seizures.