Cellular (sickle cell)

A nurse working with clients with sickle cell disease (SCD) teaches about self-management to prevent exacerbations and sickle cell crises. What factors should clients be taught to avoid? (Select all that apply.) a. Dehydration b. Exercise c. Extreme stress d. High altitudes e. Pregnancy

A, C, D, E Several factors cause red blood cells to sickle in SCD, including dehydration, extreme stress, high altitudes, and pregnancy. Strenuous exercise can also cause sickling, but not unless it is very vigorous

Meperidine (Demerol) is not recommended for children in sickle cell crisis because it: a. may induce seizures. b. is easily addictive. c. is not adequate for pain relief. d. is given by intramuscular injection.

A. A metabolite of meperidine, normeperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with sickle cell disease are particularly at risk for normeperidine-induced seizures. Meperidine is no more addictive than other narcotic agents. Meperidine is adequate for pain relief. It is available for IV infusion.

A client presents to the emergency department in sickle cell crisis. What intervention by the nurse takes priority a. Administer oxygen. b. Apply an oximetry probe. c. Give pain medication. d. Start an IV line.

A. All actions are appropriate, but remembering the ABCs, oxygen would come first. The main problem in a sickle cell crisis is tissue and organ hypoxia, so providing oxygen helps halt the process.

A client in sickle cell crisis is dehydrated and in the emergency department. The nurse plans to start an IV. Which fluid choice is best? a. 0.45% normal saline b. 0.9% normal saline c. Dextrose 50% (D50) d. Lactated Ringer's solution

A. Because clients in sickle cell crisis are often dehydrated, the fluid of choice is a hypotonic solution such as 0.45% normal saline. 0.9% normal saline and lactated Ringer's solution are isotonic. D50 is hypertonic and not used for hydration.

A client hospitalized with sickle cell crisis frequently asks for opioid pain medications, often shortly after receiving a dose. The nurses on the unit believe the client is drug seeking. When the client requests pain medication, what action by the nurse is best? a. Give the client pain medication if it is time for another dose. b. Instruct the client not to request pain medication too early. c. Request the provider leave a prescription for a placebo. d. Tell the client it is too early to have more pain medication

A. Clients with sickle cell crisis often have severe pain that is managed with up to 48 hours of IV opioid analgesics. Even if the client is addicted and drug seeking, he or she is still in extreme pain. If the client can receive another dose of medication, the nurse should provide it. The other options are judgmental and do not address the client's pain. Giving placebos is unethical.

When both parents have sickle cell trait, which is the chance their children will have sickle cell anemia? a. 25% b. 50% c. 75% d. 100%

A. Sickle cell anemia is inherited in an autosomal recessive pattern. If both parents have sickle cell trait (one copy of the sickle cell gene), then for each pregnancy, a 25% chance exists that their child will be affected with sickle cell disease. With each pregnancy, a 50% chance exists that the child will have sickle cell trait. Percentages of 75% and 100% are too high for the children of parents who have sickle cell trait.

Which clinical manifestation should the nurse expect when a child with sickle cell anemia experiences an acute vasoocclusive crisis? a. Circulatory collapse b. Cardiomegaly, systolic murmurs c. Hepatomegaly, intrahepatic cholestasis d. Painful swelling of hands and feet; painful joints

D. A vasoocclusive crisis is characterized by severe pain in the area of involvement. If in the extremities, painful swelling of the hands and feet is seen; if in the abdomen, severe pain resembles that of acute surgical abdomen; and if in the head, stroke and visual disturbances occur. Circulatory collapse results from sequestration crises. Cardiomegaly, systolic murmurs, hepatomegaly, and intrahepatic cholestasis result from chronic vasoocclusive phenomena.

A client with sickle cell disease (SCD) takes hydroxyurea (Droxia). The client presents to the clinic reporting an increase in fatigue. What laboratory result should the nurse report immediately? a. Hematocrit: 25% b. Hemoglobin: 9.2 mg/dL c. Potassium: 3.2 mEq/L d. White blood cell count: 38,000/mm3

D. Although individuals with SCD often have elevated white blood cell (WBC) counts, this extreme elevation could indicate leukemia, a complication of taking hydroxyurea. The nurse should report this finding immediately. Alternatively, it could indicate infection, a serious problem for clients with SCD. Hematocrit and hemoglobin levels are normally low in people with SCD. The potassium level, while slightly low, is not as worrisome as the WBCs.

Parents of a child with sickle cell anemia ask the nurse, "What happens to the hemoglobin in sickle cell anemia?" Which statement by the nurse explains the disease process?" a. Normal adult hemoglobin is replaced by abnormal hemoglobin. b. There is a lack of cellular hemoglobin being produced. c. There is a deficiency in the production of globulin chains. d. The size and depth of the hemoglobin are affected.

A. Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, in which normal adult hemoglobin is replaced by abnormal hemoglobin. Aplastic anemia is a lack of cellular elements being produced. Thalassemia major refers to a variety of inherited disorders characterized by deficiencies in production of certain globulin chains. Iron deficiency anemia affects the size and depth of the color.

A nurse caring for a client with sickle cell disease (SCD) reviews the client's laboratory work. Which finding should the nurse report to the provider? a. Creatinine: 2.9 mg/dL b. Hematocrit: 30% c. Sodium: 147 mEq/L d. White blood cell count: 12,000/mm3

A. An elevated creatinine indicates kidney damage, which occurs in SCD. A hematocrit level of 30% is an expected finding, as is a slightly elevated white blood cell count. A sodium level of 147 mEq/L, although slightly high, is not concerning.

Which should the nurse teach about prevention of sickle cell crises to parents of a preschool child with sickle cell disease? (Select all that apply.) a. Limit fluids at bedtime. b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. d. Use ice packs to decrease the discomfort of vasoocclusive pain in the legs. e. Notify the health care provider if your child begins to develop symptoms of a cold.

B, C, E The most important issues to teach the family of a child with sickle cell anemia are to (1) seek early intervention for problems, such as a fever of 38.5° C (101.3° F) or greater; (2) give penicillin as ordered; (3) recognize signs and symptoms of splenic sequestration, as well as respiratory problems that can lead to hypoxia; and (4) treat the child normally. The nurse emphasizes the importance of adequate hydration to prevent sickling and to delay the adhesion-stasis-thrombosis-ischemia cycle. It is not sufficient to advise parents to "force fluids" or "encourage drinking." They need specific instructions on how many daily glasses or bottles of fluid are required. Many foods are also a source of fluid, particularly soups, flavored ice pops, ice cream, sherbet, gelatin, and puddings. Increased fluids combined with impaired kidney function result in the problem of enuresis. Parents who are unaware of this fact frequently use the usual measures to discourage bedwetting, such as limiting fluids at night. Enuresis is treated as a complication of the disease, such as joint pain or some other symptom, to alleviate parental pressure on the child. Ice should not be used during a vasoocclusive pain crisis because it vasoconstricts and impairs circulation even more.

A client admitted for sickle cell crisis is distraught after learning her child also has the disease. What response by the nurse is best? a. "Both you and the father are equally responsible for passing it on." b. "I can see you are upset. I can stay here with you a while if you like." c. "It's not your fault; there is no way to know who will have this disease." d. "There are many good treatments for sickle cell disease these days."

B. The best response is for the nurse to offer self, a therapeutic communication technique that uses presence. Attempting to assign blame to both parents will not help the client feel better. There is genetic testing available, so it is inaccurate to state there is no way to know who will have the disease. Stating that good treatments exist belittles the client's feelings.

A school-age child is admitted in vasoocclusive sickle cell crisis. The child's care should include: a. correction of acidosis. b. adequate hydration and pain management. c. pain management and administration of heparin. d. adequate oxygenation and replacement of factor VIII.

B. The management of crises includes adequate hydration, minimization of energy expenditures, pain management, electrolyte replacement, and blood component therapy if indicated. Hydration and pain control are two of the major goals of therapy. The acidosis will be corrected as the crisis is treated. Heparin and factor VIII is not indicated in the treatment of vasoocclusive sickle cell crisis. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels.

The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. Which is appropriate for the nurse to explain about narcotic analgesics? a. Are often ordered but not usually needed b. Rarely cause addiction because they are medically indicated c. Are given as a last resort because of the threat of addiction d. Are used only if other measures, such as ice packs, are ineffective

B. The pain of sickle cell anemia is best treated by a multidisciplinary approach. Mild to moderate pain can be controlled by ibuprofen and acetaminophen. When narcotics are indicated, they are titrated to effect and are given around the clock. Patient-controlled analgesia reinforces the patient's role and responsibility in managing the pain and provides flexibility in dealing with pain. Few, if any, patients who receive opioids for severe pain become behaviorally addicted to the drug. Narcotics are often used because of the severe nature of the pain of vasoocclusive crisis. Ice is contraindicated because of its vasoconstrictive effects.

A client has a sickle cell crisis with extreme lower extremity pain. What comfort measure does the nurse delegate to the unlicensed assistive personnel (UAP)? a. Apply ice packs to the client's legs. b. Elevate the client's legs on pillows. c. Keep the lower extremities warm. d. Place elastic bandage wraps on the client's legs.

C. During a sickle cell crisis, the tissue distal to the occlusion has decreased blood flow and ischemia, leading to pain. Due to decreased blood flow, the client's legs will be cool or cold. The UAP can attempt to keep the client's legs warm. Ice and elevation will further decrease perfusion. Elastic bandage wraps are not indicated and may constrict perfusion in the legs.

The nurse is conducting a staff in-service on sickle cell anemia. Which describes the pathologic changes of sickle cell anemia? a. Sickle-shaped cells carry excess oxygen. b. Sickle-shaped cells decrease blood viscosity. c. Increased red blood cell destruction occurs. d. Decreased adhesion of sickle-shaped cells occurs.

C. The clinical features of sickle cell anemia are primarily the result of increased red blood cell destruction and obstruction caused by the sickle-shaped red blood cells. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. Increased adhesion and entanglement of cells occurs.