Sickle Cell Disease
When explaining neonatal testing for sickle cell disease (SCD), which content is most appropriate for the nurse to include in the care plan? When testing for sickle cell disease (SCD) in newborns, a heel stick may be used to obtain blood. Newborn screening for sickle cell disease (SCD) is offered as an optional test in the United States. Initial diagnosis of sickle cell disease (SCD) in newborns most commonly occurs by way of amniocentesis testing. Newborns who are diagnosed with sickle cell disease (SCD) should immediately begin diagnostic testing for prevention of stroke.
A Newborn screening for sickle cell disease (SCD) is mandatory in the United States. Although amniocentesis may be used to diagnose SCD in the prenatal period, the initial diagnosis is most often made by testing a few drops of the newborn's blood. In most cases, for newborns, the blood sample is obtained by way of a heel stick.
Which factor influences the development and manifestations of sickle cell disease (SCD) in children? A high level of fetal hemoglobin in children inhibits sickling of RBCs. Children with sickle cell trait have two Hb S genes. Children with sickle cell trait are commonly symptomatic at birth. Sickle cell crisis is very common among children with sickle cell trait.
A Individuals with sickle cell trait have one sickle cell (Hb S) gene and one normal hemoglobin (Hb A) gene. For children with SCD, because sickling is inhibited by high levels of fetal hemoglobin, these clients usually are asymptomatic until 4 to 6 months of age. Children with sickle cell trait rarely experience sickle cell crisis.
The nurse educator is giving an in-service about the assessment and care of children who are diagnosed with sickle cell disease (SCD). Which information should the educator include in the in-service? Newborn blood testing for sickle cell disease usually involves obtaining a blood sample by way of a heel stick. Newborn screening for sickle cell disease is recommended and optional for children who are born in the United States. Initial diagnosis of sickle cell disease is most often made by amniocentesis testing in the prenatal period. Beginning at 5 years of age, children with sickle cell disease should undergo routine ultrasound head scanning.
A Newborn screening for sickle cell disease (SCD) is mandatory in the United States. Although amniocentesis may be used to diagnose SCD in the prenatal period, the initial diagnosis is most often made by testing a few drops of the newborn's blood. In most cases, for newborns, the blood sample is obtained by way of a heel stick. Starting at 2 years of age, children with SCD should undergo routine ultrasound scanning of the head to evaluate cerebral blood flow.
Which statement best describes the effects of sickle cell disease (SCD) on RBCs? Sickling dramatically decreases the life span of affected RBCs. The cellular membrane of a sickled RBC becomes tough and durable. Sickled RBCs may cause ischemia along with intense vascular dilation. Once sickled, an RBC cannot resume a normal shape.
A Once rehydrated and reoxygenated, sickled cells can resume a normal shape. Even so, the cellular membrane of the affected cells is more fragile. Likewise, sickling dramatically decreases the life of the affected RBC. Compared to the normal life span of an RBC, which is approximately 120 days, the sickled RBC's life span is 10-20 days. Individuals with sickle cell disease (SCD) are at risk for sickle cell crisis. Also called vaso-occlusive crisis, sickle cell crisis is the term used to describe painful periods resulting from ischemia due to vascular occlusion.
Which treatment options are appropriate for a client with sickle cell disease (SCD)? (Select all that apply.) Surgical removal of the client's spleen Bone marrow transplantation Hematopoietic stem cell transplantation Blood transfusions Insulin administration
A, B, C, D For surgical treatment of SCD, bone marrow or hematopoietic stem cell transplantation may be considered. For clients who experience splenic sequestration of RBCs, during which large numbers of sickled RBCs are trapped in the spleen, the most common treatment is administration of blood transfusions. Because splenic sequestration of RBCs may be life-threatening, for some clients who develop this condition, surgical removal of the spleen (splenectomy) may be indicated. Insulin is used to treat diabetes mellitus, not sickle cell disease.
The nursing student is creating a presentation about pharmacologic treatments used in the care of clients with sickle cell disease (SCD). Which information is most appropriate for the student to include in the presentation? (Select all that apply.) Patient-controlled analgesia (PCA) machines may be ordered for clients with sickle cell disease. Analgesics are administered to clients with sickle cell disease to help reduce blood viscosity. Analgesics should be administered to clients with sickle cell disease only on a prn (as needed) basis. Prophylactic antibiotic therapy may be ordered for clients with sickle cell disease. Vaccinations are contraindicated for clients with sickle cell disease.
A, D Analgesics are administered for pain control. For the client with sickle cell disease (SCD), analgesics should be administered at scheduled intervals as opposed to prn (as needed). A patient-controlled analgesia (PCA) machine may be ordered for analgesic administration. Because infection poses severe risks for clients with SCD, prophylactic antibiotic therapy also may be ordered. Vaccinations are recommended for clients of all ages who have SCD. Oral and IV fluid replacement helps to reduce blood viscosity.
The client with sickle cell disease (SCD) asks the nurse to explain vaso-occlusive crisis. Which information is most appropriate for the nurse to include in the explanation? (Select all that apply.) Vaso-occlusive crisis consists of pain resulting from ischemia caused by vascular occlusion. Prolonged vaso-occlusive crisis may progress to a more severe condition called sickle cell crisis. Overhydration increases the risk for developing vaso-occlusive crisis. Conditions that impair oxygen transport to tissues may lead to vaso-occlusive crisis. Infection increases the risk for developing vaso-occlusive crisis.
A, D, E Individuals with SCD are at risk for vaso-occlusive crisis, which is also described as sickle cell crisis. Vaso-occlusive crisis consists of painful periods resulting from ischemia due to vascular occlusion. Risk factors that predispose an individual with SCD to development of sickle cell crisis include dehydration, which increases blood viscosity, and any condition that increases the body's oxygen needs or impairs oxygen transport to the tissues, such as infection or trauma.
The nurse is caring for Alishia Baldwin, a 24-year-old African American client who is pregnant with her first child. Mrs. Baldwin and her husband are talking about their family history of sickle cell disease (SCD). Mr. Baldwin asks the nurse, "What factors affect our baby's chances of having sickle cell disease?" Which response by the nurse is most appropriate? "Because sickle cell disease is caused by an autosomal recessive genetic defect, you both must have the trait in order for your child to have the disorder." "Sickle cell disease occurs only in individuals who are of African American descent and approximately 50% of African Americans are born with some form of the disorder." "If both you and your wife have the genetic trait for sickle cell disease, then there is a 50% chance that your baby will have the disease." "There are several forms of sickle cell disease, but only one form of the disorder is influenced by factors related to inheritance of genes from the parents."
B SCD is most common among people of African descent. In the United States, an estimated 1 in 12 African Americans carry one abnormal hemoglobin (Hb S) gene and thus have sickle cell trait. SCD is caused by an autosomal recessive defect of the genes that are involved in producing hemoglobin. If both parents have the genetic trait for SCD, then with each pregnancy, there is a 25% risk of having a child with the disease. The severity and manifestations of SCD depend on genetics, too. There are several forms of SCD, each of which is dependent on genetic inheritance. Sickle cell anemia, which is a form of chronic hemolytic anemia, is the most common type of SCD.
The client with sickle cell disease (SCD) is ordered to receive hydroxyurea. To correctly explain the rationale for administering hydroxyurea to the client, which statement is most appropriate for the nurse to include in the teaching? "Hydroxyurea decreases the body's production of fetal hemoglobin." "Hydroxyurea decreases the production of abnormal blood cells." "Hydroxyurea will help decrease your nausea and vomiting." "Hydroxyurea may cause you to have more pain."
B For the client with sickle cell disease (SCD), hydroxyurea is used for cytotoxic effects. This medication decreases the production of abnormal blood cells, which leads to decreased pain. Hydroxyurea also increases fetal hemoglobin production and RBC mean corpuscle volume (MCV). Side effects of hydroxyurea include bone marrow suppression, headaches, dizziness, nausea, and vomiting.
The nurse is assessing the client with sickle cell disease (SCD) for manifestations of shock. Which assessment finding is most reflective of a classic manifestation of shock? Hypertension Pallor Increased urine output Persistent abdominal pain
B Manifestations of shock include hypotension, changes in level of consciousness, pallor, dizziness or lightheadedness, decreased urine output, and increased capillary refill time. Clients with sickle cell disease (SCD) may experience pain in a variety of locations, including the abdomen, but pain is not a classic manifestation of shock.
A client and her husband both carry the genetic trait for sickle cell disease (SCD). The client asks the nurse practitioner, "What is the risk that we will have a child with sickle cell disease?" Which response by the nurse practitioner is most accurate? "All of your children will have the SCD trait, but none will have SCD." "There is a 25 percent chance that any of your children will have SCD." "There is a 50 percent chance that any of your children will have SCD." "All of your children will have some form of SCD, though severity will vary."
B SCD is caused by an autosomal recessive defect of the genes that are involved in producing hemoglobin. If both parents have the genetic trait for SCD, then with each pregnancy, there is a 25% risk of having a child with the disease.
The nurse educator asks the nursing student to explain the primary cause of anemia related to sickle cell disease (SCD). Which response by the nursing student is most accurate? Deoxygenation of affected RBCs Early destruction of RBCs Deformity of RBCs Sickling of RBCs
B The primary cause of anemia related to sickle cell disease (SCD) is early destruction of RBCs. Effects of SCD include deoxygenation of RBCs, which causes a characteristic sickling deformity of the affected RBCs.
The nurse is teaching the client with sickle cell disease (SCD) about hemosiderosis, which is a complication associated with frequent blood transfusions. Which statement is most appropriate for the nurse to include in the client teaching? "Hemosiderosis occurs when your immune system reacts to antigens." "Hemosiderosis is storage of iron in tissues and organs." "Vitamin C can increase your risk for developing hemosiderosis." "In most cases, hemosiderosis is caused by iron-chelating medications."
B Hemosiderosis, which is a complication associated with frequent blood transfusions, is storage of iron in the tissues and organs. To help prevent hemosiderosis, an iron-chelating drug (such as deferoxamine) may be given with vitamin C to promote iron excretion. Alloimmunization happens when the child's immune system reacts to antigens on the donated tissues (e.g., blood and stem cells).
Which conditions are known triggers for a sickle cell crisis? (Select all that apply.) Hyperventilation Fever Emotional stress High altitudes Physical stress
B, C, D, E Conditions that may trigger sickling of red blood cells (RBCs) include fever, high altitudes, poorly pressurized airplanes, and emotional or physical stress. Hypoventilation also may trigger RBC sickling.
The client with sickle cell disorder (SCD) is preparing to be discharged to home. Which topics are most appropriate for the nurse to include when providing home care teaching for the client with SCD? (Select all that apply.) Restricting oral fluid intake Maintaining adequate nutritional intake Recognizing manifestations of splenic sequestration Keeping current with vaccinations Balancing rest with activity
B, C, D, E Home care planning for the client with sickle cell disease (SCD) includes teaching the client about avoiding triggers for sickling of RBCs, such as dehydration. Rather than teaching the client to restrict oral fluid intake, the client with SCD should be taught to maintain adequate hydration. Appropriate home care teaching topics for the client with SCD include keeping current with vaccinations, balancing rest with activity, maintaining adequate nutritional intake, and recognizing manifestations of splenic sequestration.
The nurse is teaching the client about the potential effects of sickle cell disease (SCD). When explaining the potential effects of sickling, which examples are most appropriate for the nurse to include in the teaching? (Select all that apply.) Malformation of WBCs Tissue damage Prolonged life span of RBCs Organ damage Occlusion of small blood vessels
B, D, E Because of the characteristic crescent or sickle shape of the malformed RBCs associated with sickle cell disease (SCD), this process is called sickling. Chronic sickling of RBCs shortens the life span of these cells. Sickled RBCs can occlude small blood vessels, especially capillaries. Recurrent or prolonged ischemia due to sickle cell-induced occlusions causes tissue and organ damage. WBC malformation is not characteristic of SCD.
The nurse is analyzing and synthesizing assessment data, related to the care of the client with sickle cell disease (SCD), to formulate client-specific nursing diagnoses. Which client problem represents the highest priority when planning nursing care of the client with SCD? Activity intolerance Imbalanced nutrition Altered comfort Impaired oxygenation
D During the diagnosis phase of the nursing process, the nurse analyzes and synthesizes assessment data to formulate client-specific nursing diagnoses. The nurse also identifies emergent and urgent problems that require immediate attention, and provides prompt client care as indicated. Nursing diagnoses that are reflective of safety risks or infectious disease transmission should be addressed immediately. While altered comfort, imbalanced nutrition, and activity intolerance should be addressed, impaired oxygenation represents the highest priority client problem.
The nurse is caring for a 58-year-old Marc McRyan, who is diagnosed with sickle cell disease (SCD). Mr. McRyan's healthcare provider plans to use pharmacologic therapy to stimulate his production of fetal hemoglobin. Which therapy should the nurse anticipate will be ordered by Mr. McRyan's healthcare provider? Hydroxyurea Oxygen Penicillin Vitamin C
A Hydroxyurea is used to promote the production of fetal hemoglobin. Penicillin is prescribed to treat certain bacterial infections and, in some cases, to prevent infection. Oxygen is prescribed to reduce hypoxemia and promote tissue oxygenation. Vitamin C is prescribed to promote iron excretion in association with frequent blood transfusions.
The nurse is assessing the client with sickle cell disease (SCD) for signs and symptoms of acute chest syndrome. Which assessment finding is most reflective of a manifestation of acute chest syndrome? Urine output > 0.5 mL/kg/hr Absence of adventitious breath sounds Increased white blood cell count Oxygen saturation of > 90%
C Manifestations of acute chest syndrome include chest pain, adventitious breath sounds, pulmonary infiltrates, and increased white blood cell (WBC) count. Absence of adventitious lung sounds, oxygen saturation of > 90%, and urine output of > 0.5 mL/kg/hr are normal findings and do not reflect manifestations of acute chest syndrome.
