NURSING PROCESS:THE PATIENT WITH SICKLE CELL CRISIS
Patients in sickle cell crisis should be assessed for factors that could have precipitated the crisis, such as symptoms of infection or dehydration, or situations that promote fatigue or emotional stress.
Patients are asked to recall factors that seemed to precipitate pre-vious crises and measures they use to prevent and manage crises. Pain levels should always be monitored; a pain-rating scale, such as a 0-to-10 scale, best accomplishes this. The quality of the pain (eg, sharp, dull, burning), the frequency of the pain (constant ver-sus intermittent), and factors that aggravate or alleviate the pain are included in this assessment. If a sickle cell crisis is suspected, the nurse needs to determine whether the pain currently experi-enced is the same as or different than the pain typically encoun-tered in crisis.
Because the sickling process can interrupt circulation in any tis-sue or organ, with resultant hypoxia and ischemia, a careful assess-ment of all body systems is necessary. Particular emphasis is placed on assessing for pain, swelling, and fever. All joint areas are care-fully examined for pain and swelling. The abdomen is assessed for pain and tenderness because of the possibility of splenic infarction.
The respiratory system must be assessed carefully, including auscultation of breath sounds, measurement of oxygen saturation levels, and signs of cardiac failure, such as the presence and extent of dependent edema, an increased point of maximal impulse, and cardiomegaly (as seen on chest x-ray). The patient should be as-sessed for signs of dehydration by a history of fluid intake and care-ful examination of mucous membranes, skin turgor, urine output, and serum creatinine and blood urea nitrogen values.
A careful neurologic examination is important to elicit symp-toms of cerebral hypoxia. However, ischemic findings on MRI or Doppler studies may significantly precede the findings on the physical examination. MRI and Doppler studies are used for early diagnosis and may be more beneficial to improve patient outcome, because therapy can be initiated more promptly.
Because patients with sickle cell anemia are so susceptible to in-fections, they are assessed for the presence of any infectious process. Particular attention is given to examination of the chest, long bones, and femoral head, because pneumonia and osteomyelitis are especially common. Leg ulcers, which may be infected and are slow to heal, are common.
The extent of anemia (as measured by the hemoglobin level and the hematocrit) and the ability of the marrow to replenish RBCs (as measured by the reticulocyte count) should be monitored and compared with the patient’s baseline values. The patient’s current and past history of medical management should also be assessed, particularly chronic transfusion therapy, hydroxyurea use, and prior treatment for infection.
Based on the assessment data, major nursing diagnoses for the patient with sickle cell crisis may include:
• Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels
• Risk for infection
• Risk for powerlessness related to illness-induced helplessness
• Deficient knowledge regarding sickle crisis prevention
Based on the assessment data, potential complications may include:
• Hypoxia, ischemia, infection, and poor wound healing lead-ing to skin breakdown and ulcers
• Cerebrovascular accident (CVA, brain attack, stroke)
• Renal dysfunction
• Heart failure, pulmonary hypertension, and acute chest syndrome
• Poor compliance
• Substance abuse related to poorly managed chronic pain
The major goals for the patient are relief of pain, decreased inci-dence of crisis, enhanced sense of self-esteem and power, and ab-sence of complications.
Acute pain during a sickle cell crisis can be severe and unpre-dictable. The patient’s subjective description and rating of pain on a pain scale must guide the use of analgesics, which are valu-able in controlling the acute pain of a sickle crisis. Any joint that is acutely swollen should be supported and elevated until the swelling diminishes. Relaxation techniques, breathing exercises, and distraction are helpful for some patients. After the acute painful episode has diminished, aggressive measures should be implemented to preserve function. Physical therapy, whirlpool baths, and transcutaneous nerve stimulation are examples of such modalities.
Nursing care focuses on monitoring the patient for signs and symp-toms of infection. Prescribed antibiotics should be initiated promptly, and the patient should be assessed for signs of dehydra-tion. If the patient is to take prescribed oral antibiotics at home, he or she must understand the need to complete the entire course of antibiotic therapy and must be able to identify a feasible adminis-tration schedule.
This illness, because of its acute exacerbations that often result in chronic health problems, frequently leaves the patient feeling powerless and with decreased self-esteem. These feelings can be exacerbated by inadequate pain management. The patient’s ability to use normal coping resources of physical strength, psychological stamina, and positive self-esteem is dramatically diminished. En-hancing pain management can be extremely useful in establishing a therapeutic relationship based on mutual trust. Nursing care that focuses on the patient’s strengths rather than deficits can enhance effective coping skills. Providing the patient with opportunities to make decisions about daily care may increase the patient’s feelings of control.
Patients with sickle cell anemia benefit from understanding what situations can precipitate a sickle cell crisis and the steps they can take to prevent or diminish such crises. Keeping warm and main-taining adequate hydration can be very effective in diminishing the occurrence and severity of attacks. Avoiding stressful situations is more challenging. Group education may be more effective if it is carried out by members of the community who are from the same ethnic group as those with the disease.
Management measures for many of the potential complications were delineated in previous sections. Other measures follow.
Leg ulcers require careful management and protection from trauma and contamination. Referral to a wound care specialist may facili-tate healing and assist with prevention. If leg ulcers fail to heal, skin grafting may be necessary. Scrupulous aseptic technique is war-ranted to prevent nosocomial infections.
Male patients may develop sudden, painful episodes of priapism (persistent penile erection). The patient is taught to empty his bladder at the onset of the attack, exercise, and take a warm bath. If an episode persists longer than 3 hours, medical attention is recommended. Repeated episodes may lead to extensive vascular thrombosis, resulting in impotence.
Many patients have considerable difficulty coping with chronic pain and repeated episodes of sickle crisis. Those who feel they have little control over their health and the physical complica-tions that result from this illness may find it difficult to under-stand the importance of complying with a prescribed treatment plan. Being nonjudgmental and actively seeking involvement from the patient in establishing a treatment plan are useful strategies.
Some patients with sickle cell anemia develop problems with substance abuse. For many, this abuse results from inadequate management of acute pain during episodes of crisis. Some clini-cians suggest that abuse may result from prescribing inadequate amounts of opioid analgesics for an inadequate time. The patient’s pain may never be adequately relieved, promoting mistrust of the health care system and (from the patient’s perspective) the need to seek care from a variety of sources when the pain is not severe. This cycle is best managed by prevention. Receiving care from a single provider over time is much more beneficial than receiving care from rotating physicians and staff in an emergency department. When crises do arise, the staff in the emergency department should be in contact with the patient’s primary health care provider so that optimal management can be achieved. Once the pattern of substance abuse is established, it is very difficult to manage, but continuity of care and estab-lishing written contracts with the patient can be useful man-agement strategies.
Because patients with sickle cell anemia are typically diagnosed as children, parents participate in the initial education. Based on the parents’ education, literacy, socioeconomic level, and interest, teaching focuses on the disease process (including some patho-physiology), treatment, and the assessment and monitoring skills for potential complications (see previous discussion). As the child ages, educational interventions with the child prepare the child to assume more responsibility for self-care.
Vascular access device management and chelation therapy can be taught to most families. Follow-up and care for patients with vascular access devices may also need to be provided by nurses in an outpatient facility or by a home care agency.
The illness trajectory of sickle cell anemia is highly varied, with unpredictable episodes of complications and crises. Care is often provided on an emergency basis, especially for some pa-tients with pain management problems (see previous section). Nurses in all settings used by this patient population need to communicate regularly with each other. Patients need to learn which parameters are important for them to monitor and how to monitor them. Parameters should also be given as to when to seek urgent care.
Expected patient outcomes may include:
1) Control of pain
a) Acute pain is controlled with analgesics
b) Uses relaxation techniques, breathing exercises, distrac-tion to help relieve pain
2) Is free of infection
a) Has normal temperature
b) Shows WBC count within normal range (5000 to 10,000/mm3)
c) Identifies importance of continuing antibiotics at home (if applicable)
3) Expresses improved sense of control
a) Participates in goal setting and in planning and imple-menting daily activities
b) Participates in decisions about care
4) Increases knowledge about disease process
a) Identifies situations and factors that can precipitate sickle cell crisis
b) Describes lifestyle changes needed to prevent crisis
c) Describes the importance of warmth, adequate hydra-tion, and prevention of infection in preventing crisis
5) Absence of complications
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