36: Management of Patients With Immune Deficiency Disorders

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More than 50% of individuals with this disease develop pernicious anemia:

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

A child has just been diagnosed with a primary immune deficiency. The parents state, "Oh, no. Our child has AIDS." Which response by the nurse would be most appropriate?

"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.

The nurse receives a phone call at the clinic from the family of a client with AIDS. They state that the client started "acting funny" and reported headache, tiredness, and a stiff neck. Checking the temperature resulted in a fever of 103.2°F. What should the nurse inform the family member?

"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.

Antiretroviral medications should be offered to clients with T-cell counts less than

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.

A nurse educator is preparing to discuss immunodeficiency disorders with a group of fellow nurses. What would the nurse identify as the most common secondary immunodeficiency disorder?

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.

When assisting the patient to interpret a negative HIV test result, the nurse informs the patient that the results mean which of the following?

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.

A client who has AIDS reports having diarrhea after every meal, and wants to know what can be done to stop this symptom. What should the nurse advise?

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.

A client is to have a hip replacement in 3 months and does not want a blood transfusion from random donors. What option can the nurse discuss with the client?

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.

What treatment option does the nurse anticipate for the patient with severe combined immunodeficiency disease (SCID)?

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

When administering intravenous gamma globulin infusion, the nurse recognizes that which report by the client may indicate an adverse effect of the infusion

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.

Which condition is an early manifestation of HIV encephalopathy?

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.

Which of the following indicates that a client with HIV has developed AIDS?

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.

A client with severe combined immunodeficiency is to receive a hematopoietic stem cell transplant. What would the nurse expect to be started?

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.

A patient in the clinic states, "My boyfriend told me he went to the clinic and was treated for gonorrhea." While testing for the sexually transmitted infection (STI), what else should be done for this patient?

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.

HIV is harbored within which type of cell?

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.

A nurse knows that more than 50% of clients with CVID develop the following disorder.

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.

Which adverse effect(s) should the nurse closely monitor in a client who has secondary immunodeficiencies due to immunosuppressive therapy?

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.

When assisting the client to interpret a negative HIV test result, what does the nurse tell the client that this result means?

The body has not produced antibodies to the AIDS virus.

A client that is HIV+ has been diagnosed with Pneumocystis pneumonia caused by P. jiroveci. What medication does the nurse expect that the client will take for the treatment of this infection?

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

A female client comes to the clinic and tells the nurse, "I think I have another vaginal infection and I also have some wartlike lesions on my vagina. This is happening quite often." What should the nurse consult with the physician regarding?

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.


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