Grand Rounds Presentation

SICKLE CELL ANEMIA Grand Rounds Melanie Almonte Spring 2009 Instructor: Ruth Reeder http://www.mun.ca/biology/desmid/brian/BIOL2250/Week_Three/ssaRBC.jpg Thursday, February 26, 2009

OUTLINE Objectives Plan of care Pathophysiology Nursing diagnoses Signs and symptoms Goals Labs Interventions Diagnosis Results Family impact Cultural considerations NCLEX practice questions Patient demographics Summary Past medical history References Physical assessment Thursday, February 26, 2009

OBJECTIVES To increase the knowledge of the sickle cell disease process. To identify risk factors that induce sickle cell crisis. To learn how to manage acute pain related to ischemia. Thursday, February 26, 2009

PATHOPHYSIOLOGY genetic disorder autosomal recessive pattern of inheritance for diagnosis at least 40% of the total Hgb contains an abnormality of the beta chains (HbS) HbS is very sensitive to changes in O2 concentrations in RBCs Thursday, February 26, 2009

PATHOPHYSIOLOGY genetic disorder autosomal recessive pattern of inheritance for diagnosis at least 40% of the total Hgb contains an 50% abnormality of the beta chains (HbS) HbS is very sensitive to changes in O2 concentrations in RBCs Thursday, February 26, 2009

PATHOPHYSIOLOGY genetic disorder autosomal recessive pattern of inheritance for diagnosis at least 40% of the total Hgb contains an 25% 50% abnormality of the beta chains (HbS) HbS is very sensitive to changes in O2 concentrations in RBCs Thursday, February 26, 2009

PATHOPHYSIOLOGY genetic disorder autosomal recessive pattern of inheritance for diagnosis at least 40% of the total Hgb contains an 25% 25% 50% abnormality of the beta chains (HbS) HbS is very sensitive to changes in O2 concentrations in RBCs Thursday, February 26, 2009

STRUCTURE Thursday, February 26, 2009

SIGNS & SYMPTOMS CARDIOVASCULAR NEUROLOGICAL cool extremities pain (most common symptom) slow capillary reﬁll seizures cyanosis stroke heart murmurs behavioral changes tachycardia MUSCULOSKELETAL ANEMIA joint degeneration dyspnea on exertion, SOB, bone density changes (seen in weakness xray) limited ROM Thursday, February 26, 2009

SIGNS & SYMPTOMS, CONT’D GI jaundice organ failure (abd organs 1st to be damaged due to multiple episodes of hypoxia, ischemia) INTEGUMENT stasis and pressure ulcers on lower extremities Thursday, February 26, 2009

DIAGNOSIS Abnormal labs elevated %age of HbS reticulocytes WBC count decreased H/H platelet count Thursday, February 26, 2009

DIAGNOSIS Abnormal labs elevated %age of HbS Reticulocyte counts are reticulocytes increased with sickle cell disease WBC count because the life span of sickled decreased red blood cells is shortened. H/H platelet count Thursday, February 26, 2009

CULTURAL CONSIDERATIONS Also Caribbean, Middle Eastern and central american peoples. http://discovermagazine.com/2005/mar/human-study-thyself/gene-map.jpg http://www.sicklecellsociety.org/img/research/italy.gif Thursday, February 26, 2009

PATIENT INFORMATION 31 yo AAF 6’1” 171 lbs CHIEF COMPLANT: pain in right side of neck, chest PRIMARY DIAGNOSIS: superior vena cava thrombosis ALLERGIES: Ceftriaxone, Dilaudid, Morphine, Rocephin, Fentanyl DIET: Regular No IV access due to inﬁltration PICC consult placed ACTIVITY: as tolerated; pt allowed to go outside (2/19) CONFIDENTIAL status Thursday, February 26, 2009

ADMISSION Admitted 2/15/09 Presented to Summerville Medical Center Chief complaint: chest discomfort that has been consistent, moderate, but waxes and wanes began 2 days ago worsened by movement unrelieved by meds (home med is: Demerol PO) Chest CT upon admission, with and w/o contrast revealed thrombus in SVC & right subclavian Thursday, February 26, 2009

PAST MEDICAL HISTORY Anemia Cholelithiasis - advised by PCP to have cholecystectomy Port-a-cath x 3 Heart murmurs Pulmonary embolism, carotid clots Cardiomegaly (mild) 10 hospitalizations due to sickle cell crisis since 2003 Typically between January and May Recently began taking herbal supplements in place of Coumadin Hospitalized in January due to L breast blood clots Mondor’s Syndrome Thursday, February 26, 2009

ASSESSMENT CARDIOVASCULAR NEUROLOGICAL cool extremities pain (most common symptom) slow capillary reﬁll seizures cyanosis stroke heart murmurs behavioral changes tachycardia MUSCULOSKELETAL ANEMIA joint degeneration dyspnea on exertion, SOB, bone density changes (seen in weakness xray) limited ROM Thursday, February 26, 2009

ASSESSMENT, CONT’D GI Labs jaundice RBC 2.89 organ failure (abd organs 1st to Hgb 8.6 be damaged due to multiple Hct 25.0 episodes of hypoxia, ischemia) RDW 18.2 Na 139 INTEGUMENT K 3.9 stasis and pressure ulcers on WBC count 7.4 lower extremities PT 11.6 (9.0 - 11.5 sec) INR 1.18 (2.0 - 3.0 What does this mean?! Thursday, February 26, 2009

NURSING DIAGNOSES Acute pain related to ischemia of blood vessels in the anterior chest region as evidenced by patient reporting discomfort, guarding and grimacing. Ineffective tissue perfusion: cardiopulmonary related to decreased hemoglobin concentration in blood as evidenced by cold extremities, weak distal pulses, and chest pain. Risk for infection related to decreased WBC count. Activity intolerance related to imbalance between oxygen supply and demand as evidenced by exertional discomfort and verbal report of fatigue. Thursday, February 26, 2009

NURSING DIAGNOSES, CONT’D Deﬁcient knowledge related to information misinterpretation as evidenced by inaccurate follow through of medication administration instruction. Impaired physical mobility related to activity intolerance as evidenced by limited ability to perform gross motor skills and slowed movement. Decreased cardiac output related to altered preload as evidenced by tachycardia and fatigue. Anxiety related to change in health status and decreased social interaction as evidenced by displaying an apprehensive and distressed affect and voice quivering. Thursday, February 26, 2009

GOALS Consistently report pain levels of 0 - 3/10 Maintain O2 saturation > 92% Maintain adequate hydration via PO intake and continuous IVF Improve musculoskeletal function Patient will accurately verbalize understanding of importance of taking medication in order to prevent sickle cell crisises Patient will remain free from infection, injury or falls Thursday, February 26, 2009

INTERVENTIONS Frequently assess O2 saturation and treat low saturations with oxygen per humidiﬁed NC. Treat pain with Demerol on a regular schedule (q2h), not PRN. Encourage PO ﬂuid intake. Teach patient to take breaks and rest as needed throughout the day in order to conserve energy. Teach patient to keep temperature in their room warm and to avoid cold, stressful environments. Administer drinks with little to no ice. If possible, maintain IV access in order to prevent need for IM medication. Teach patient importance of not wearing tight, constricting clothing. Thursday, February 26, 2009

PHARMACOLOGICAL INTERVENTIONS Ativan PRN’s Soma Benadryl Coumadin Meperidine HCL Cyanocobalamin (Vit B12) Phenergan Cytotec Folic Acid K-Dur Lovenox (d/c’ed 2/21) Pneumonia Vaccine (PNU-IMUNE 23) Prilosec D5W 1/2NS @ 125cc/hr Thursday, February 26, 2009

PROGRESSION OF INTERVENTIONS Level of Pain Coumadin (mg) PT (sec) Thursday, February 26, 2009

PROGRESSION OF INTERVENTIONS Level of Pain Coumadin (mg) PT (sec) 13.0 11.7 10.4 9.1 7.8 6.5 5.2 3.9 Sun 2/15 2.6 Mon 2/16 1.3 Tues 2/17 Wed 2/18 Thurs 2/19 Fri 2/20 Sat 2/21 Thursday, February 26, 2009

PROGRESSION OF INTERVENTIONS Level of Pain Coumadin (mg) PT (sec) 13.0 11.7 10.4 9.1 7.8 6.5 5.2 3.0 4.6 3.0 3.9 Sun 2/15 4.0 2.6 Mon 2/16 2.3 1.3 Tues 2/17 4.0 2.6 Wed 2/18 Thurs 2/19 Fri 2/20 Sat 2/21 Thursday, February 26, 2009

PROGRESSION OF INTERVENTIONS Level of Pain Coumadin (mg) PT (sec) 13.0 11.7 10.4 10.0 10.0 9.1 10.0 7.8 5.0 5.0 6.5 5.0 5.2 3.0 4.6 3.0 3.9 Sun 2/15 4.0 2.6 Mon 2/16 2.3 1.3 Tues 2/17 4.0 2.6 Wed 2/18 2.5 Thurs 2/19 Fri 2/20 Sat 2/21 Thursday, February 26, 2009

PROGRESSION OF INTERVENTIONS Level of Pain Coumadin (mg) PT (sec) 12.5 11.6 13.0 12.3 12.6 13.0 11.7 12.3 12.1 10.4 10.0 10.0 9.1 10.0 7.8 5.0 5.0 6.5 5.0 5.2 3.0 4.6 3.0 3.9 Sun 2/15 4.0 2.6 Mon 2/16 2.3 1.3 Tues 2/17 4.0 2.6 Wed 2/18 2.5 Thurs 2/19 Fri 2/20 Sat 2/21 Thursday, February 26, 2009

TREATMENT MODALITIES - RESEARCH Yoon, S.L. & Black, S. (2006). Comprehensive, integrative management of pain for patients with sickle-cell disease. The Journal of Alternative and Complementary Medicine, 12(10), 995 - 1001. Design: cross-sectional Subjects: 63 caregivers of children w/ sickle cell disease questionnaire examined use of pharmocological and complementary therapies wanted to see if type of therapy administered related to age of patient Thursday, February 26, 2009

TREATMENT MODALITIES - RESEARCH Results: Children < age 9 were typically treated with NSAIDs or combination of acetaminophen and an opioid Majority (> 70%) of caregivers utilized some form of complementary therapy for the child Most common: prayer, spiritual healing, massage, relaxation Use of complementary therapy was higher in children taking 2+ analgesics (compared to children taking 0 - 1) Thursday, February 26, 2009

OUTCOMES Discharged 2/25 reported pain as being controlled reported pain level as being consistently < 5/10 O2 saturations on RA > 98% accurately verbalized understanding of coumadin therapy and food contraindications reported feeling relieved and less anxious as compared to last week *and is currently not taking or going home on benzodiazepines remained free from infection, falls and injury during hospitalization activity level increased; patient ambulated frequently maintained IV access for 3 days (placed 2/22) with continuous IVF maintained adequate hydration via PO intake (water) Thursday, February 26, 2009

SOCIAL/FAMILIAL IMPACT Patient lives in an apartment, 1 bedroom, alone in N. Charleston No pets. Unemployed, covered under disability. Medicare A & B, Medicaid Emergency contact is mother who lives in Summerville reports visiting/speaking to mother and sister “often” Has large support network consisting of primarily friends and church people During hospitalization, was seen talking on cell at least once an hour Thursday, February 26, 2009

NCLEX QUESTION 1 OF 5 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 Thursday, February 26, 2009

NCLEX QUESTION 2 OF 5 A 30-year-old male from Haiti is brought to the emergency department in sickle cell crisis. What is the best position for this client? A. Side-lying with knees ﬂexed B. Knee-chest C. High Fowler's with knees ﬂexed D. Semi-Fowler's with legs extended on the bed Thursday, February 26, 2009

NCLEX QUESTION 3 OF 5 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 Thursday, February 26, 2009

NCLEX QUESTION 4 OF 5 Which of the following foods would the nurse encourage the client in sickle cell crisis to eat? A. Peaches B. Cottage cheese C. Popsicle D. Lima beans Thursday, February 26, 2009

NCLEX QUESTION 5 OF 5 The test used to differentiate sickle cell anemia and sickle cell trait is: A. Sickle cell preparation B. Peripheral smear C. Sickledex D. Hemoglobin electrophoresis Thursday, February 26, 2009

SICKLE CELL: TAKE AWAY SCHEDULED pain medication - NOT PRN. Keep environment warm/comfortable for patient. Oxygenate, humidify. Encourage PO ﬂuids and to conserve energy during crises. For prevention, patient education is key! Thursday, February 26, 2009

Grand Rounds Presentation

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