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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
                     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
STRUCTURE




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
                     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
                     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
                     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
                     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
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
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 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 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 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 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
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

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Grand Rounds Presentation

  • 1. 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
  • 2. 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
  • 3. 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
  • 4. 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
  • 5. 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
  • 6. 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
  • 7. 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
  • 8. 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
  • 12. 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
  • 13. 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
  • 14. DIAGNOSIS Abnormal labs elevated %age of HbS reticulocytes WBC count decreased H/H platelet count Thursday, February 26, 2009
  • 15. 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
  • 16. 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
  • 17. 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
  • 18. 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
  • 19. 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
  • 20. 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
  • 21. 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
  • 22. 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
  • 23. 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
  • 24. 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
  • 25. 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
  • 26. 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
  • 27. 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
  • 28. 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
  • 29. 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
  • 30. 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
  • 31. 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
  • 32. 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
  • 33. PROGRESSION OF INTERVENTIONS Level of Pain Coumadin (mg) PT (sec) Thursday, February 26, 2009
  • 34. 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
  • 35. 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
  • 36. 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
  • 37. 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
  • 38. 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
  • 39. 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
  • 40. 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
  • 41. 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
  • 42. 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
  • 43. 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
  • 44. 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
  • 45. 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
  • 46. 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
  • 47. 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
  • 48. 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
  • 49. 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
  • 50. 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
  • 51. 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
  • 52. 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

Editor's Notes