Question

1. Describe the nursing goals and interventions that would be used when caring for a child with hemophilia.

2. Describe the protocols of isolation for patient with the human immunodeficiency virus (HIV) or the acquired immunodeficiency syndrome (AIDS), and discuss isolation protocols for the patient with severe combined immunodeficiency disease.

3. Describe the underlying pathophysiologic processes and the appropriate nursing goals for sickle cell anemia and β-thalassemia major.

4. Discuss the two primary forms of acute leukemia that appear in children: acute lymphoblastic leukemia (ALL) and acute myelogenous leukemia (AML).

5. Describe osteosarcoma and Ewing sarcoma, including clinical manifestations, diagnosis, and therapeutic management, and nursing care.

6. Review other solid tumors, including Wilms tumor, rhabdomyosarcoma, retinoblastoma, germ cell tumors, and liver tumors, along with their clinical manifestations, diagnosis, staging, and management.

Answer 1

Nursing Diagnoses of child of hemophilia:

Based on the assessment data, the major nursing diagnoses are:

• Acute pain related to traumatic injury to the muscles.
• Impaired physical injury related pain and discomfort with the onset of bleeding episodes.
• Compromised family coping related to incorrect and inadequate information or understanding.
• Risk for bleeding related to decreased concentration of clotting factors circulating in the blood (factor VIII and factor IX).
• Risk for injury related to decreased clotting factor (VIII or IX).

• The major goals are:

• Child will experience decreased pain.
• Child will maintain optimal physical mobility as evidenced by normal range of motion (ROM) and activities of daily living within ability.
• Family will cope effectively with child’s illness.
• Child’s risk for injury from possible bleeding is decreased through the use of appropriate prophylactic measures.

• Nursing Interventions

  The nursing interventions for a child with hemophilia are:

• Relieve pain. Immobilize joints and apply elastic bandages to the affected joint if indicated; elevate affected and apply a cold compress to active bleeding sites, but must be used cautiously in young children to prevent skin breakdown.
• Maintain optimal physical mobility. Provide gentle, passive ROM exercise when the child’s condition is stable; educate on preventive measures, such as the application of protective gear and the administration of factor products; and refer for physical therapy, occupational therapy, and orthopedic consultations, as required.
• Assist in the coping of the family. Encourage family members to verbalize problem areas and develop solutions on their own; encourage family members to express
  feelings, such as how they deal with the chronic needs of a family member and coping patterns that help or hinder adjustment to the problems.
• Prevent bleeding. Monitor hemoglobin and hematocrit levels; assess for inhibitor antibody to factor VIII; anticipate or instruct in the need for prophylactic treatment before high-risk situations, such as invasive diagnostic or surgical procedures, or dental work; and provide replacement therapy of deficient clotting factors.
• Prevent injury. Utilize appropriate toys (soft, not pointed
  or small sharp objects); for infants, may need to use padded bed rail sides on crib; avoid rectal temperatures; provide appropriate oral hygiene (use of
  a water irrigating device; use of a soft toothbrush or softening the toothbrush with warm water before brushing; use of sponge-tipped toothbrush); and avoid contact sports such as football, soccer, ice hockey, karate.

• Evaluation

  Goals are met as evidenced by:

• Child experienced decreased pain.
• Child maintained optimal physical mobility as evidenced by normal range of motion (ROM) and activities of daily living within ability.
• Family coped effectively with child’s illness.
• Child’s risk for injury from possible bleeding was decreased through the use of appropriate prophylactic measures.

2::

Human immunodeficiency virus (HIV), the virus that causes acquired immunodeficiency syndrome (AIDS), is transmitted through sexual contact and exposure to infected blood or blood components and perinatally from mother to neonate. HIV has been isolated from blood, semen, vaginal secretions, saliva, tears, breast milk, cerebrospinal fluid, amniotic fluid, and urine and is likely to be isolated from other body fluids, secretions, and excretions. However, epidemiologic evidence has implicated only blood, semen, vaginal secretions, and possibly breast milk in transmission.

A: Patients with HIV in general do not need to be placed on isolation precautions, however, if the HIV+patient has respiratory symptoms (i.e., cough, shortness of breath, coughing up blood, chest pain, etc.) they need to be placed on Airborne Precautions until an alternative diagnosis can be confirmed.

To prevent the spread of HIV, hospitals follow strict infection prevention and control guidelines. All blood and body fluids from patients are treated as potentially infectious:

• Syringes and needles are ‘single use’ and disposed of in approved sharps containers.
• Reusable medical devices are decontaminated and sterilised after each patient use.
• Many medical devices are disposed of after single use.
• Healthcare workers wear protective equipment including gowns, gloves and eyewear when carrying out any procedures involving a patient’s blood or body fluids.
• All spilt blood and body fluids are cleaned up according to strict cleaning guidelines.
• Laundry is cleaned according to strict Australian Standards (AS/NZS 4146:2000).

• All health-care workers should routinely use appropriate barrier precautions to prevent skin and mucous-membrane exposure when contact with blood or other body fluids of any patient is antici- pated. Gloves should be worn for touching blood and body fluids, mucous membranes, or non-intact skin of all patients, for handling items or surfaces soiled with blood or body fluids, and for performing venipuncture and other vascular access procedures. Gloves should be changed after contact with each patient. Masks and protective eyewear or face shields should be worn during procedures that are likely to generate droplets of blood or other body fluids to prevent exposure of mucous membranes of the mouth, nose, and eyes. Gowns or aprons should be worn during procedures that are likely to generate splashes of blood or other body fluids.

• Hands and other skin surfaces should be washed immediately and thoroughly if contaminated with blood or other body fluids. Hands should be washed immediately after gloves are removed.

• All health-care workers should take precautions to prevent injuries caused by needles, scalpels, and other sharp instruments or devices during procedures; when cleaning used instruments; during disposal of used needles; and when handling sharp instruments after procedures. To prevent needlestick injuries, needles should not be recapped, purposely bent or broken by hand, removed from disposable syringes, or otherwise manipulated by hand. After they are used, disposable syringes and needles, scalpel blades, and other sharp items should be placed in puncture-resistant containers for disposal; the puncture-resistant containers should be located as close as practical to the use area. Large-bore reusable needles should be placed in a puncture-resistant container for transport to the reprocessing area.

• Although saliva has not been implicated in HIV transmission, to minimize the need for emergency mouth-to-mouth resuscitation, mouth- pieces, resuscitation bags, or other ventilation devices should be available for use in areas in which the need for resuscitation is predictable.

• Health-care workers who have exudative lesions or weeping dermatitis should refrain from all direct patient care and from handling patient-care equipment until the condition resolves.

• Pregnant health-care workers are not known to be at greater risk of contracting HIV infection than health-care workers who are not pregnant; however, if a health-care worker develops HIV infection during pregnancy, the infant is at risk of infection resulting from perinatal transmission. Because of this risk, pregnant health-care workers should be especially familiar with and strictly adhere to precautions to minimize the risk of HIV transmission. Implementation of universal blood and body-fluid precautions for ALL