Nursing Assessment:
1. Obtain history for possible dehydration, hypoxia, infection, or other precipitating event.
2. Obtain history and characterization of pain.
3. Observe for pallor and jaundice, changes in vital signs (elevated temperature, tachycardia, hypotension, tachypnea), change in mental status, swelling of extremities, ulcers or skin lesions, or signs of dehydration (decreased elasticity of skin, dry mucous membranes, decreased urine output, increased urine concentration and specific gravity).
4. Examine for enlarged liver and spleen, tenderness of hands or feet.
5. Evaluate growth and development.
Nursing Diagnoses:
1. Acute Pain related to tissue anoxia from disease process
2. Ineffective Tissue Perfusion related to increased blood viscosity
3. Risk for Infection related to fibrotic changes in the spleen
4. Impaired Gas Exchange related to effects of opioids, anesthesia, and blood loss of surgery
5. Activity Intolerance related to anemia
6. Interrupted Family Process related to frequent medical care, hospitalization, and chronic illness
Nursing Interventions:
Relieving Pain
1. Identify and use effective measures to alleviate pain, such as:
A) Carefully position and support painful areas.
B) Hold or rock the infant; handle gently.
C) Distract the child by singing, reading stories, providing appropriate play activities, or television or videos.
D) Provide familiar objects; encourage visits by familiar persons.
E) Bathe the child in warm water, applying local heat or massage.
F) Give prescribed medications. Do not give aspirin because it enhances acidosis.
G) Maintain bed rest during crisis.
2. Share effective methods of reducing pain with other staff members and family.
3. Patients with chronic pain may not display typical pain behavior; remember, pain is what the patient says it is.
4. Assess effectiveness of intervention.
Increasing Tissue Perfusion
1. Administer blood for severe anemia and vaso-occlusion.
2. Administer oxygen via tent, face mask, or nasal cannula, depending on age.
Reducing Infection
1. Administer antibiotics as prescribed.
2. Give meticulous care to leg ulcers and other open wounds.
3. Use good hand washing and meticulous technique in all procedures.
Preventing Hypoxia
1. Monitor for and prevent respiratory depression caused by opioids.
A) Check respiratory rate and depth frequently.
B) Encourage coughing and deep breathing.
C) Use incentive spirometry.
D) Obtain pulse oximetry reading as indicated.
E) Raise side rails and supervise ambulation if drowsiness occurs.
2. Help reduce the risks of anesthesia and blood loss during surgery.
A) Administer preoperative blood transfusions as prescribed to suppress the formation of new sickle cells and to reduce the threat of anoxia.
B) Maintain adequate hydration before and after surgery.
C) Observe the child closely for signs of infection, especially of the respiratory tract.
D) Inform anesthesia department of child's disease status.
E) Monitor vital signs frequently and obtain pulse oximetry readings.
Improving Activity Tolerance
1. Maintain bed rest during crisis, then increase activity gradually to increase endurance.
2. Encourage rest periods, alternating with activity.
3. Encourage good eating habits, sleep, and relaxation.
Normalizing Family Processes
1. Encourage parents to talk about their child, the illness, and how they feel about it.
2. Expect such feelings as guilt, shock, frustration, depression, and resentment.
3. Accept negative feelings, but try to build on positive coping mechanisms.
4. Provide factual information to child and parents about their concerns.
5. Encourage role-playing and play activities to identify fears.
6. Assure adolescents that although sexual development is delayed, they will eventually catch up with their peers.
7. Stress the normalcy of the child despite sickle cell.
A) Sickle cell disease does not affect intelligence; the child should go to school and keep up with class work while stable.
B) Between periods of crisis the child can usually participate in peer group activities, with the exception of some strenuous sports.
C) The child needs the same discipline as other children in family.
Community and Home Care Considerations
Home care nurses and school nurses can be instrumental in the care of patients with sickle cell anemia, by assuring that their primary health care needs are met.
1. Because of the child's predisposition to infections, be sure that the following are carried out routinely:
A) Ophthalmology examinations every 1 to 2 years after age 10.
B) Hearing tests yearly after age 3.
C) A tuberculin skin test every 2 to 3 years (every year in at-risk populations). Any positive findings should be followed up promptly.
D) All routine childhood immunizations, as well as the hepatitis B series, pneumococcal, meningococcal, H. influenzae type B, and yearly trivalent influenza.
E) Dental checkups and teeth cleaning every 6 months.
2. Educate parents, teachers, and daycare providers involved in the care of children with sickle cell disease about their common problems and special needs:
A) Frequent absences because of pain and illness.
B) Fatigue and inattention, caused by anemia or ischemia of central nervous system tissue.
C) Inability to concentrate urine, requiring frequent restroom breaks.
D) Permanent learning disabilities or delays, requiring individualized teaching plans.
E) Need for a controlled environment to prevent sickling, avoiding temperature extremes, dehydration, and excessive stress.
F) Need for moderate physical exercise, with avoidance of rough contact sports and activities.
G) Desire to feel “normal†and have opportunities and environmental stimulation similar to other children their age.
3. Assess the child's home environment for protection from infection and injury, adequate nutritional resources, transportation to medical appointments, and emotional support. Initiate social service and other referrals as needed.
Family Education and Health Maintenance
1. Discuss the genetic implications of sickle cell disease and offer genetic counseling to the family.
2. Instruct the parents in ways that they can help their child to avoid sickling episodes.
A) Do not allow the child to become chilled or to wear tight clothing that might impede circulation.
B) Provide adequate fluids and notify health care provider if excessive fluids are lost through vomiting, diarrhea, fever, excessive sweating.
3. Instruct parents how to recognize signs of dehydration (dry skin and mucous membranes, decreased urine output; irritability or listlessness in the infant).
4. Encourage parents to seek prompt treatment of cuts, sores, and mosquito bites, and to notify the health care provider if the child is exposed to a communicable disease.
5. Encourage good dental hygiene and frequent dental checkups to avoid dental infections.
6. Instruct on preventive care, including all the recommended childhood immunizations and screening tests.
7. Encourage a calm, emotionally stable environment.
8. Warn against trips to the mountains or against trips in unpressurized airplanes that will decrease oxygen concentration.
9. Provide sexually active adolescents with information on contraception and sexually transmitted disease.
10 Teach signs of a mild crisis:
A) Fever.
B) Decreased appetite.
C) Irritability.
D) Pain or swelling in abdomen, extremities, back.
E) Teach parents to palpate spleen.
11. Instruct on home management of mild crisis.
A) Encourage fluids.
B) Administer antipyretic medications, as directed by health care provider familiar with condition.
C) Encourage rest.
D) Keep the child warm.
E) Apply warm compresses to the painful area.
F) Hospitalization may be required for the child if pain becomes severe or if I.V. hydration is required.
12. Teach the signs of severe crises and whom to notify:
A) Pallor.
B) Lethargy and listlessness.
C) Difficulty in awakening.
D) Irritability.
E) Severe pain.
F) Fever of 102آ° F (38.9آ° C) report immediately.
13. Instruct the parents to have emergency information available to those involved in the child's care (school nurse, teacher, baby-sitter, family members).
A) Name and phone number of health care provider or clinic.
B) Closest emergency facility and ambulance phone number.
C) Child's blood type, allergies, medications, and medical records number.
D) Name of informed neighbor or relative to be notified in an emergency.
14. Stress the benefit of wearing a MedicAlert bracelet.
15. For additional information and support, refer to Sickle Cell Foundation,
http://www.scdfc.org.
Evaluation: Expected Outcomes
1. Appears more comfortable and does not cry or complain of pain
2. Less pallor noted with oxygen use
3. Afebrile with no signs of infection
4. No change in respirations; uses incentive spirometer hourly
5. Ambulates 20 minutes four or five times per day
6. Parents verbalize concerns about chronic illness