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Hemoglobinopathy

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Sickle Cell Disorder is a hereditary _______________

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replacement of normal hemoglobin wtih abnormal hemoglobin S in RBCs

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Sickle cell is characterized by

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Chronic Hemolytics anemia

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Is sickle cell classified as chronic or acute

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when the percentage of hemoglobin (Hgb) replaced by HgbS increases

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What is sickling?

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Rbcs become abnormally crescent shaped, causing occlusions of small blood vessels, ischemia, and damage to affected organs.

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What happens during episodes of Sickling?

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Weakens the cell membranes of RBCs

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What can occur with repeated episodes of sickling and unsickling

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they are hemolyzed and removed

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What occurs with weakened rbcs?

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Abnormally shaped red blood cells are unable to circulate freely through the blood vessels and become stuck in small vessels, particularly in capillaries forming occlusions with the end result of ischemia because the tissues cannot receive oxygen and nutrients they need.

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Because of RBCs are misshaped, what happens to the circulation?

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damage to the tissues and organs throughout the body, eventually resulting in scarring and impaired function

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repeated or prolonged ischemia resulting form sickle cell induced occlusions cause__________

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High r/t impaired immunity

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What is the infection rate for sickle cell anemia?

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developmental delay, mental retardation, and other neurological outcomes.

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What can occur with children with sickle cell anemia?

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hypoxia dehydration infection venous stasis pregnancy alcohol high altitudes fever emotional or physical stress acidosis strenuous exercise anesthesia

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What triggers sickling?

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the term used to describe periods when the percentage of hgb S increases resulting in the appearance of symptoms, often marked by acute pain resulting from ischemia.

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Define Sickle Cell Crisis?

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increased blood viscosity low fluid intake/dehydration fever hypoxia low oxygen tension infection trauma

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Precipitating factors for sickle cell crisis?

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re-hydration and re-oxygenation; however, membranes are more fragile and cell life is shortened to 10-20 days

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How can sickled cells resume a normal shape?

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genetic autosomal recessive with two allele mutations

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Etiology of sickle cell anemia?

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only one allele mutation Client is a carrier (can only pass the trait) Manifestations are mild even under severe triggers

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Sickle Cell Trait

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1 in 100 Latinos Mediterranean African American's have sickle cell trait; they remain asymptomatic unless stressed by severe hypoxia

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Risk Factors?

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Hypoxia Low environmental temperature low body temperature excessive exercise anesthesia dehydration infection acidosis

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Triggers?

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RBC sickling

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Manifestations of sickle cell? arise from?

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Pallor Fatigue Jaundice Irritability

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general manifestations?

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a crisis as result of occluded circulation, impaired erythropoiesis, or sequestration or large amounts of blood in the liver or spleen.

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Extensive sickling can precipitate what?

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when sickling develops in the microcirculation

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Vasoocculusive or thrombotic crisis occurs when?

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vasospasm that halts all blood flow

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obstruction of blood flow by sickled cells that cannot pass through the vessels triggers _________

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tissue ischemia infarction Pain Large joints are affected abdominal pain may signal infarction or abdominal organs and structures. infarction also may affect bone marrow or lead to aseptic necrosis of affected bones resulting in pain from avascular necrosis of the bone marrow stroke may result from cerebral vessel occlusion skin ulcers may develop as the result of occluded vessels supplying the dermis

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Lack of blood flow leads to:

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SOB fatigue weakness Compare: pulses, temp, cap refill all extremities. HR may be rapid BP maybe low, Decreased pulse pressure.

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Cardiovascular changes

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pallor cyanosis (decr oxygenation) Assessment: check lips, tongue, nail beds, conjunctivae, palms, soles of feet q 8 hr (gray blue tinge) Jaundice r/t bilirubin from damaged RBC OPEN ULCERS on lower legs, ankles, necrotic, infected

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Skin Changes

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hepatomegaly splenomegaly (blood trapping) Gallstones

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Abdominal Changes

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low grade fever seizures manifestations of stroke. assess gait, hand grasps, coordination.

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CNS Changes

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Neonatal screening early intervention prophylactic antibiotics

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Collaboration

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Newborns: testing cord blood using hemoglobin electrophoresis Sickledex may be used for quick screening in children older than 6 months Serum analysis of blood reveals teh degree of anemia, with hemoglobin of 6-10g/dl

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Diagnostic Tests

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TRAIT: 40% Hbs DISEASE: 80-100% HbS HCT: low (20%-30%) RETICULOCYTE: high = anemia long duration BILIRUBIN: high WBC: high d/t chronic inflammation, hypoxia, ischemia

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Lab Assessment

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parenteral analgesics are generally administered around the clock pain medications should not be ordered prn Complementary: (not alone, must use pain meds) warm temp, distraction, relaxation, positioning, aroma, therapeutic touch, warmsoaks and compresses

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Pain Control

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Oral and IV fluid replacement promotes pain relief, since dehydration is often a cause of crisis Fluids reduce the viscosity of the blood, so adequate hydration is essential

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Hydration

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administered to provide comfort and decrease the incidence of pulmonary complications

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Oxygenation

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children who are functionally asplenic or have had a splenectomy have a resultant decreased capability to fight infection

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Prevention and treatment of infection

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improved blood and tissue oxygenation reduction in sickling temporary suppression of the production of RBCs containing hgbS

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Transfusion of RBCs

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iron is stored in tissues and organs because the body has no way to excrete it

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Complications of transfusion

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HX of past crisis precipitating events medical treatmetn home management weigh/height (failure to thrive is common) so compare with past use pain scale and identify pain perception in each body part assess pain management protocol multisystem assessment: fever neurologic changes respiratory symptoms are emergency condition that necessitate prompt treatment monitor for shock, hypotension, changes in LOC, dizziness, lightheadedness, increased capillary refill

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Assessment

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risk forimpaired tissue perfusion (cerebral) Acute Pain Caregiver role Strain Interrupted family process delayed growth and development Impaired physical mobility

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Nursing Dx

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client will experience reducing complications caregiver will provide support and assistance client will meet growth and development needs client will optimize physical mobility as tolerated

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Plan

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Pain Control Hydration Oxygenation Preventino and treatment of infections Transfusion Hydroxurea Stem cell Transplant

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Treatment

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Decreased abnormal cell production results in decreased pain

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Hydroxyurea

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Hand-Foot Syndrome(Swollen hands and feet may be the first signs of sickle cell anemia) Splenic Crisis ( spleen traps to much rbcs) Infections (decreased immune function) Acute Chest Syndrome: like pneumonia, sickle cells get trapped in lungs resulting in decreased O2 levels Pulmonary HTN Delayed Growth and puberty Stroke eye problems priapism ( persistent and usually painful long-lasting erection) Gallstones Ulcers on the legs Multiple organ failure Fever Tachycardia LOC

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Complications

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LOW

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A patient with sickle cell anemia has a____ hematocrit?

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C

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A newly admitted client has sickle cell crisis. The nurse is planning care based on assessment of the client. The client is complaining of severe pain in his feet and hands. The pulse oximetry is 89. Which of the following interventions would be implemented first? Assume that there are orders for each intervention. a. Adjust the room temperature b. Give a bolus of IV fluids c. Start O2 d. Administer meperidine (Demerol) 75mg IV push

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D

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A 43-year-old African American male is admitted with sickle cell anemia. The nurse plans to assess circulation in the lower extremities every 2 hours. Which of the following outcome criteria would the nurse use? a. Body temperature of 99°F or less b. Toes moved in active range of motion c. Sensation reported when soles of feet are touched d. Capillary refill of < 3 seconds

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B

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A 25-year-old male is admitted in sickle cell crisis. Which of the following interventions would be of highest priority for this client? a. Taking hourly blood pressures with mechanical cuff b. Encouraging fluid intake of at least 200mL per hour c. Position in high Fowler's with knee gatch raised d. Administering Tylenol as ordered

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D

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A 14 year old girl has been hospitalized with Sickle Cell Anemia in vasoocclusive crisis. Which of these Nursing diagnoses should receive priority in the Nursing plan of care? -- A. Impaired social interaction -- B. Alteration in body image -- C. Pain -- D. Alteration in tissue perfusion

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C

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Which of the following foods would the nurse encourage the client in sickle cell crisis to eat? -- A. Peaches -- B. Cottage cheese -- C. Popsicles -- D. Lima beans

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D

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The male client with sickle cell anemia comes to the emergency room with a temperature of 101.4 F and tells the nurse that he is having a sickle cell crisis. Which diagnostic test should the nurse anticipate the emergency room doctor ordering for the client? -- A. Spinal tap. -- B. Hemoglobin electrophoresis. -- C. Sickle-turbidity test (Sickledex). -- D. Blood cultures.

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1. Management of Pain 2. Administration of oxygen 3. Promoting Hydration to decrease blood viscocity 4. Monitor for

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A Client in crisis should:

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"Sickled cells clump in the smaller blood vessels and obstruct blood flow." Rationale: All the symptoms of sickle cell are a result of the clumping of the sickled cells in the microvasculature, causing obstruction of blood flow. The other statements are inaccurate.

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The nurse is teaching a group of parents whose children have sickle cell anemia. When a parent asks the cause of the symptoms, the nurse responds with which of the following?

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Reticulocyte count Rationale: The reticulocyte count will be increased because the life span of sickled red blood cells is shortened. Hemoglobin, hematocrit, and platelet levels will be decreased.

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A child with suspected sickle cell disease (SCD) is in the clinic for laboratory studies. The parents ask the nurse what results will tell the physician that their child has SCD. The nurse responds that which of the following is increased in this disease?

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Both the mother and father must carry the gene for the baby to be affected. Rationale: Sickle cell is inherited as an autosomal recessive disorder. Both parents must carry the gene for the baby to be affected. The other statements are inaccurate.

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A pregnant woman tells the nurse that there is a history of sickle cell disease in her family and she is afraid that the baby will have the disease. The nurse provides the client with which of the following information?

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Treatment is aimed at pain control, oxygen therapy, and hydration, but does not provide a cure. Rationale: Treatment for sickle cell crisis is pain control, oxygenation, and fluid resuscitation. There is no cure for sickle cell disease. The nurse teaches families how to prevent sickle cell crisis.

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The nurse is caring for a child who is in the hospital experiencing sickle cell crisis. The parents are asking the nurse which treatment will help cure the child. The nurse responds with which of the following?

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Delayed Growth and Development Rationale: The child with sickle cell disease is often developmentally delayed due to the effects of physical disability, pain, and inpatient hospital stays. The nurse would plan activities that help maintain developmental levels the child has reached. The child in sickle cell crisis does not experience ineffective airway clearance, bleeding, or constipation as a result of sickle cell disease. The child may have an illness that could cause one of these symptoms, but they are not common to children with sickle cell disease.

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The nurse is admitting a 7-year-old client who is experiencing sickle cell crisis and plans care based on which of the following nursing diagnoses?

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C

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The 24-year old African American female client tells the nurse she has a brother with sickle cell disease. She is engage to be married and is concerned about giving this disease to her future children. which information is most important to provide too the client? A. tell the client that she won't pass this on if she has never had symptoms B. Encourage the client to discuss this concern with her fiance C. Recommend that she and her fiance see a genetic counselor D. Discuss the possibility of adopting children after she gets married.

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4. Morphine

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The nurse is caring for a client in a sickle cell crisis. which is the pain regiment of choice to relieve the pain? 1. Frequent aspirin 2. Motrin 3. Demerol 4. Morphine

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4. sickle cell anemia is a disorder of the client rbcs characterized by abnormally shaped red cells that sickle or clump together, leading to oxygen deprivation and resulting in crisis and severe pain.

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the student nurse asks the nurse, "what is sickle cell anemia?" which statement by the nurse would be the best answer to the student's question? 1. There is some written material at the desk 2. it is a congenital disease of the blood 3. the client has decreased synovial fluid 4. the blood becomes thick when the client is deprived of oxygen

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2. explained in layman's terms

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the client's nephew has just been diagnosed with sickle cell anemia. the client asks the nurse, "how did my nephew get this disease? which statement would be the best response by the nurse? 1. sickle cell is an inherited autosomal recessive disorder 2. he was born with it and both his parents were carriers of the disease 3. at this time, the cause is unknown 4. your sister was exposed to a virus

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2

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the client diagnosed with sickle cell anemia comes to the ED c/o joint pain throughout the body. Oral temp 102.4, SpO2 91%. Which action should the nurse implement first: 1. Request ABGs 2. Administer oxygen 3. Start IV 4.Administer analgesic

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3

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Client is experiencing vaso-occlusive sickle cell crisis secondary to infection. which medical tx should the nurse anticipate 1. administer demerol 2. admit the client to a private room 3. Infuse D5W 4. Insert a 22-french foley

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3. Vaso-occlusive crisis, the most frequent crisis, is characterized by organ infarction, which will result in bloody urine secondary to kidney infarction.

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Which s/s will the nurse expect to assess in teh client diagnosed with a vaso-occlusive sickle cell crisis 1. lordosis 2.epistaxis 3. hematuria 4. petechia

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Sickle cell Crisis

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Periods marked by acute pain resulting from ischemia

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Sickle Cell Trait

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An individual with one normal gene and one hemoglobin S gene has

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Hemosiderosis

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Iron stored in tissues and organs

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Sickle Cell Disorder

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Characterized by replacement of normal hemoglobin S in RBCS

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alloimmunization

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When the immune system reacts against antigens on the donated tissues

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Priapism

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Persistent, painful erection of teh penis

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vaso-occlusion

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Can occur when sickling develops in the microcirculation

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Sequestration crisis

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marked by pooling of large amounts of blood in the liver and spleen.

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Tissue ischemia

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Caused by lack of blood flow

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Acute Chest Syndrome

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a symptom complex that includes fever, chest pain, increased WBC count and pulmonary infiltrates

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sickling

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When the percentage of hemoglobin replaced by hemoglobin S increases it is called

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sickle cell anemia

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a chronic hemolytic anemia that is the most common type of sickle cell disorder

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