PAT I E N T T E A C H I N G Be sure to cover:
■ the disorder, diagnosis, and treatment
■ dangers of lead poisoning, especially if the patient reports pica
■ importance of continuing therapy, even after the patient begins to feel better
■ absorption interference of iron supplementation with milk or antacid
■ increased absorption with vitamin C
■ avoidance of staining teeth by drinking liquid supplemental iron through a straw
■ when to report adverse effects of iron therapy
■ basics of a nutritionally balanced diet
■ importance of avoiding infection and when to report signs of infection
■ need for regular checkups
■ compliance with prescribed treatment.
PAT H O P H YS I O L O G Y
■ The abnormal HbS found in the patient’s RBCs becomes insoluble when-ever hypoxia occurs.
■ RBCs become rigid, rough, and elongated, forming a crescent or sickle shape.
■ Sickling can produce hemolysis (cell destruction).
■ The altered cells accumulate in capillaries and smaller blood vessels, making the blood more viscous.
■ Normal circulation is impaired, causing pain, tissue infarctions, and swelling.
CA U S E S
■ Homozygous inheritance of the HbS-producing gene (defective Hb gene from each parent)
A S S E S S M E N T F I N D I N G S
■ Signs and symptoms usually absent before age 6 months
■ Chronic fatigue
■ Unexplained dyspnea, or dyspnea on exertion
■ Joint swelling; aching bones
■ Chest pain
■ Ischemic leg ulcers
■ Increased susceptibility to infection
■ Pulmonary infarctions and cardiomegaly
■ Jaundice or pallor
■ May appear small in stature for age
■ Delayed growth and puberty
■ Spiderlike body build (narrow shoulders and hips, long extremities, curved spine, and barrel chest) in adult
■ Tachycardia
■ Hepatomegaly and, in children, splenomegaly
■ Systolic and diastolic murmurs
■ Sleepiness with difficulty awakening
■ Hematuria
■ Pale lips, tongue, palms, and nail beds
■ Body temperature over 104º F (40º C) or a temperature of 100º F (37.8º C) that persists for 2 or more days
In painful crisis
■ Severe abdominal, thoracic, muscle, or bone pain
■ Increased jaundice
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■ Dark urine
■ Low-grade fever In aplastic crisis
■ Pallor
■ Lethargy
■ Sleepiness
■ Dyspnea
■ Possible coma
■ Markedly decreased bone marrow activity
■ RBC hemolysis
In acute sequestration crisis
■ Lethargy
■ Pallor
■ Progression to hypovolemic shock and death, if untreated In hemolytic crisis
■ Liver congestion
■ Hepatomegaly
T E S T R E S U LT S
■ Stained blood smear shows sickle cells, and hemoglobin electrophoresis shows HbS. (Electrophoresis should be done on umbilical cord blood samples at birth to provide sickle cell disease screening for all neonates at risk.)
■ RBC count and erythrocyte sedimentation rate decrease, white blood cell and platelet counts and serum iron level increase.
■ RBC survival and reticulocytosis decrease; hemoglobin level is normal or low.
■ Ophthalmoscopic examination reveals corkscrew- or comma-shaped ves-sels in the conjunctivae.
■ A lateral chest X-ray reveals the characteristic “Lincoln log” deformity.
(This spinal abnormality develops in many adults and some adolescents with sickle cell anemia, leaving the vertebrae resembling logs that form the corner of a cabin.)
T R E AT M E N T
■ Avoidance of extreme temperatures
■ Avoidance of stress
■ Well-balanced diet
■ Adequate amounts of folic acid–rich foods
■ Adequate fluid intake
■ Bed rest with crises
■ Activity, as tolerated
■ Vaccines, such as polyvalent pneumococcal vaccine and Haemophilus in-fluenzae B vaccine
■ Anti-infectives
■ Analgesics
■ Iron supplements
■ Transfusion of packed RBCs, if Hb level decreases suddenly or if condi-tion deteriorates rapidly
■ Sedation and administration of analgesics, blood transfusion, oxygen therapy, and large amounts of oral or I.V. fluids, in an acute sequestration crisis
K E Y PAT I E N T O U T C O M E S The patient will:
■ demonstrate age-appropriate skills and behaviors to the extent possible
■ exhibit adequate ventilation
■ maintain collateral circulation
■ maintain balanced fluid volume where input will equal output
■ express feelings of increased comfort and decreased pain
■ maintain normal peripheral pulses
■ maintain normal skin color and temperature.
N U R S I N G I N T E R V E N T I O N S
COLLABORATION Promote optimum care by coordinating with dietitian, respiratory therapist, and psychologist.
■ Encourage the patient to talk about his fears and concerns.
■ If a male patient develops sudden, painful priapism, reassure him that such episodes are common and have no permanent harmful effects.
■ Ensure that the patient receives adequate amounts of folic acid–rich foods such as green, leafy vegetables.
■ Encourage adequate fluid intake.
■ Apply warm compresses, warmed thermal blankets, and warming pads or mattresses to painful areas of the patient’s body, unless he has neuropa-thy.
■ Administer analgesics and antipyretics, as needed.
■ When cultures demonstrate the presence of infection, give prescribed an-tibiotics.
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■ Give prescribed prophylactic antibiotics.
■ Use strict sterile technique when performing treatments.
■ Encourage bed rest with the head of the bed elevated to decrease tissue oxygen demand.
■ Administer oxygen as needed.
■ Administer blood transfusions.
■ If the patient requires general anesthesia for surgery, help ensure that he receives adequate ventilation to prevent hypoxic crisis.
■ Check vital signs and intake and output.
■ Monitor complete blood count and other laboratory test results.
■ Refer parents of children with sickle cell anemia for genetic counseling to answer their questions about the risk to future offspring.
■ Refer other family members for genetic counseling to determine whether they’re heterozygote carriers.
■ If necessary, refer the patient for psychological counseling to help him cope.
■ Refer women with sickle cell anemia for birth control counseling.
PAT I E N T T E A C H I N G Be sure to cover:
■ avoidance of tight clothing that restricts circulation
■ conditions that provoke hypoxia, such as strenuous exercise, vasocon-stricting medications, cold temperatures, unpressurized aircraft, and high altitude
■ importance of normal childhood immunizations, meticulous wound care, good oral hygiene, regular dental checkups, and a balanced diet as safe-guards against infection
■ need for prompt treatment of infection
■ need to increase fluid intake to prevent dehydration, which can cause in-creased blood viscosity
■ symptoms of vaso-occlusive crisis
■ need for hospitalization in a vaso-occlusive crisis in which I.V. fluids, par-enteral analgesics, oxygen therapy, and blood transfusions may be neces-sary
■ need to inform all health care providers that the patient has this disease before undergoing any treatment, especially major surgery
■ pregnancy and the disease; birth control options and resources
■ balanced diet, including folic acid supplements during pregnancy.