Download - Careplan for Sickle Cell Disease

  • 8/12/2019 Careplan for Sickle Cell Disease


    Topic: Caring for the patient with Sickle Cell Disease

    Audience: 3 rd year BScN. Student Nurses (NCU)

    Date: September, 2013

    Time: 10am-10:45am

    Duration: 45 Minutes

    Venue: Lecture Room

    Methodology: Lecture/Discussion

    Number of participants: xxxxxx

    Learning Theories: Ausubel: Emphasized the use of advance organizers which he said was

    different from overviews and summaries. His use of an advance organizer acted as a bridge the

    chasm between learning material and existing related ideas. The advanced organizer used; sought

    to bridge new knowledge with what was known (sometimes what is known is uncertain and not

  • 8/12/2019 Careplan for Sickle Cell Disease


    were responsible for their learning experience and the teacher sought to guide them through this

    experience. (Quinn & Hughes, 2009).

    Bruner: Believed that learners were not blank slates but bought past experiences to a new

    situation, he also stated that new information was linked to prior knowledge, thus mental

    representations are subjective. Bruner s Discovery learning is an inquiry-based, constructivist

    learning theory that takes place in problem solving situations where the learner draws on his or

    her own past experience and existing knowledge to discover facts and relationships and new

    truths to be learned. (Quinn et al., 2009). Students interact with the world by exploring and

    manipulating objects, wrestling with questions and controversies, or performing experiments. As

    a result, students may be more likely to remember concepts and knowledge discovered on their

    own (in contrast to a transmissionist model). (Quinn et al., 2009). Models that are based upon

    discovery learning model include: guided discovery, problem-based learning, simulation-based

    learning, case-based learning, incidental learning, among others. The advantages of this theory

    are: it encourages active engagement, promotes motivation, a tailored learning experience, and

    ibili i d d h d l f i i d bl

  • 8/12/2019 Careplan for Sickle Cell Disease


    since it lays the overall foundation for human behaviours that of interaction, it coincides with the

    topic and the overall mode of delivery of the topic

    Aim of the activity: To educate/show students how to care for individuals with sickle disease.

    Scientific Principle: production rate of Red Blood Cells (RBCs) = destruction rate of

    RBCs: This ensures a steady state always exists in the number of RBCs

    in our body. That is, there is always a constant number of RBCs in our

    blood stream on Earth. As RBCs become old, they are destroyed by the

    body to make room for new RBCs that are born to replace the old ones. If

    destruction of RBCs occurs before they are old, however, then not only is

    the destruction rate increased but the survival rate of healthy RBCs is

    reduced (Smelzter, Bare, Hinkle & Cheever, 2010). This scientific

    principle was utilized since it covers the topic to be discussed.

    Resources: Registered nurse, lap top computer, multimedia, white board, markers

    Objectives: At the end of 11/2 hours interactive session students should be able to

  • 8/12/2019 Careplan for Sickle Cell Disease


    5. Discuss the general management of sickle cell as cited by Saunthararajah

    and Vichinsky (2012) and Brawley et al, (2008)

    6. Discuss the nursing management of patients with sickle cell disease

    utilizing the nursing process

    Evaluation: Formative and Summative. Questions will be asked before and after each



    A.D.A.M. Medical Encyclopedia. (2013). Sickle cell anemia. Retrieved from

    Brawley, W., Cornelius, L. J., Edwards, L. R., Gamble, V. N., Green, B. L & Inturrisi C, et al.

    (2008). National Institutes of Health consensus development conference statement:

    hydroxyurea treatment for sickle cell disease. Ann Intern Med . 148: 932-938.

    Ormrod, J & Rice, F. (2003). Lifespan development and learning. Boston MA: Pearson

  • 8/12/2019 Careplan for Sickle Cell Disease


    Saunthararajah,Y & Vichinsky, E. Sickle Cell Disease: Clinical Features and Management. In:

    Hoffman, R., Benz , J Jr., Silberstein, L., Heslop, E &Weitz JI (2012). Hematology:

    Basic principles and practice . (6th ed). Philadelphia, Pa: Saunders Elsevier

    Smelter, C., Bare, B., Hinkle, J & Cheever, K. (2010). Brunner & Suddarths textbook of

    medical surgical nursing. (12 th ed.)Philadelphia: Wolters Klower Health/Lippincott

    Williams & Williams

    Vera, M. (2012). Sickle cell anemia nursing management and interventions. Retrieved on August

    18 th, 2012 from

  • 8/12/2019 Careplan for Sickle Cell Disease


  • 8/12/2019 Careplan for Sickle Cell Disease


    Teacher will ask a

    student from one groupto define the term sicklecell anemia according tothe content


    One student will

    correctly define theterm sickle cellanemia according tothe content using atleast two key wordssuch as:a severe hemolyticanemia resultingfrom the inheritanceof the sicklehemoglobin (HbS)gene, which causes adefectivehemoglobinmolecule


    Explain theCauses/RiskFactors/Pathophysiology for sickle

    Causes/Risk Factors

    Sickle cell anemia is caused by an abnormal type ofhemoglobin called hemoglobin S. Hemoglobin is a

    Teacher will ask onestudent from each ofthree to explain thecause, risk factor or the

    One student fromeach of the threegroups will attemptto explain in their

    Students will be able tocorrectlyexplain the

  • 8/12/2019 Careplan for Sickle Cell Disease


    cell disease ascited by Smelter etal, (2010) Vera (,2012)

    protein inside red blood cells that carries oxygen.

    Hemoglobin S changes the red blood cells. Thered blood cells become fragile and shaped like

    crescents or sickles. The abnormal cells deliver less oxygen to the body's tissues.

    They can also easily get stuck in small bloodvessels and break into pieces. This can interrupthealthy blood flow and cut down even more onthe amount of oxygen flowing to body tissues.

    Sickle cell anemia is inherited from both parents. If a person gets the sickle cell gene from only one parent,

    they will have only the trait. People with sickle cell traitdo not have the symptoms of sickle cell anemia.

    Sickle cell disease is much more common in people ofAfrican and Mediterranean descent. It is also seen in

    people from South and Central America, Aboriginaltribes in India and the Caribbean (Smelter et al., 2010)


    The defective hemoglobin molecule assumes a sickleshape when exposed to low oxygen tension. These long,rigid RBCs become lodged in small vessels and cano bstruct blood ow to body tissue. If ischemia orinfarction results, the patient may have pain, swelling,and fever.

    The sickling process takes time; if the erythrocyte isagain exposed to adequate amounts of oxygen (eg, when

    pathophysiology forsickle cell disease

    With the aid of white board and PowerPoint presentation and white board, teacher will statethe purposes for smallgroup discussion

    Teacher will ask onestudent from each group

    to state two purposes ofsmall group discussionsaccording to thecontents (may solicithelp from group)

    own words, thecause, risk factors,or the

    pathophysiology for

    sickle cell disease

    Students will sitattentively, followPowerPoint

    presentation and askquestions asnecessary

    One student fromeach group will statetwo purposes forsmall groupdiscussion accordingto the contents

    causes/riskfactors/pathophysiology forsickle cell

    diseaseaccording tothe content

  • 8/12/2019 Careplan for Sickle Cell Disease


  • 8/12/2019 Careplan for Sickle Cell Disease


    State themanifestations ofsickle cell diseaseaccording to

    Smelter, et al(2010) and Rees etal., 2010)

    Symptoms usually do not occur until after the age of 4months. Varies and depends somewhat on the amount ofHbs. Almost all people with sickle cell anemia have

    painful episodes called crises. These can last from hours

    to days. Crises can cause pain in the lower back, leg, joints, and chest.

    Some people have one episode every few years. Othershave many episodes each year. The crises can be severeenough to require a hospital stay.

    When the anemia becomes more severe, symptomsmay include :

    Fatigue Paleness

    Rapid heart rate Shortness of breath Yellowing of the eyes and skin (Jaundice) Younger children with sickle cell anemia have

    attacks of abdominal pain (Smelter et al., 2010 &Rees et al., 2010)

    The following symptoms may occur because small

    blood vessels become blocked by the abnormal cells:

    Painful and prolonged erection (priapism) Poor eyesight or blindness Problems with thinking or confusion caused by

    small strokes Ulcers on the lower legs (in adolescents and


    Teacher will ask twostudents at the front, twoin the middle and threeat back of the class to

    state the clinicalmanifestation of sicklecell disease

    Teacher will outline themanifestations of sicklecell disease with the aidof PowerPoint andscreen according to thecontent

    Teacher will randomlyask five students to statethe manifestations ofsickle cell diseaseaccording to thecontents

    Two students at thefront, two in themiddle and three atthe back of the class

    will attempt to statethe manifestation ofsickle cell disease

    Students will sit,listen and followingPowerPoint

    presentation as themanifestations ofsickle cell diseaseare outlined

    Five students willrandomly chosenwill state themanifestations ofsickle cell diseaseaccording to thecontents

    Students will be able tocorrectly statethe

    manifestationsof sickle celldiseaseaccording tothe content.

  • 8/12/2019 Careplan for Sickle Cell Disease


    Over time, the spleen stops working. As a result,people with sickle cell anemia may have symptoms ofinfections such as :

    Bone infection (osteomyelitis) Gallbladder infection (cholecystitis)

    Lung infection (pneumonia) Urinary tract infection

    Other symptoms include:

    Delayed growth and puberty Painful joints caused by arthritis

    Sickle cell crisis: sickle crisis, aplastic crisis, orsequestration crisis. Due to insufficient blood flow tospecific region of tissue or organ

    Acute chest syndrome: fever, cough, tachycardia,and new inltrates seen on the chest xray

    Pulmonary hypertension is a common sequela ofsickle cell disease, and often the cause of death (Smelteret al., 2010).

  • 8/12/2019 Careplan for Sickle Cell Disease


    Describe the typesof tests used in thediagnosis of sicklecell disease

    according toA.D.A.MEncyclopedia,(2013)

    Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:

    Bilirubin - is a yellowish pigment found in bile, a fluid

    made by the liver. A small amount of older red bloodcells are replaced by new blood cells every day.Bilirubin is left after these older blood cells areremoved. The liver helps break down bilirubin so that itcan be removed from the body in the stool. Largeamounts of bilirubin in the blood can lead to jaundice.Jaundice is a yellow color in the skin, mucusmembranes, or eyes. Jaundice is the most commonreason to check bilirubin levels. Normal levels are:

    Direct (also called conjugated) bilirubin: 0 to 0.3mg/dL

    Total bilirubin: 0.3 to 1.9 mg/dL

    Blood oxygen saturation -

    Complete blood count - A complete blood count(CBC) test measures the following:

    The number of red blood cells The number of white blood cells The total amount of hemoglobin in the blood The fraction of the blood composed of red blood

    cells (hematocrit)

    The CBC test also provides information about thefollowing measurements:

    Average red blood cell size (MCV)

    Teacher will ask the previously formed threegroups, to take one pieceof paper that will be

    given and attemptexplain the terms theterms that are written onthem

    Teacher willexplain/describe thetypes of test used todiagnose sickle celldisease according to thecontent with the aid ofPowerPoint presentation

    Each of the threegroup will take one

    piece of paper eachfrom teacher and

    will attempt explainthe terms written onthem.

    Student will sit,listen, followPowerPoint

    presentation and askquestions if theyarise

    Students will be able tocorrectlydescribe the

    types of testsused in thediagnosis ofsickle celldiseaseaccording tothe contents

  • 8/12/2019 Careplan for Sickle Cell Disease


    Hemoglobin amount per red blood cell (MCH) The amount of hemoglobin relative to the size of

    the cell (hemoglobin concentration) per red blood cell (MCHC)

    The platelet count is also usually included in theCBC

    normal results are:

    RBC count : Male: 4.7 to 6.1 million cells/mcL, Female:4.2 to 5.4 million cells/mcL

    WBC count : 4,500 to 10,000 cells/mcL

    Hematocrit: Male: 40.7 to 50.3%, Female: 36.1 to


    Hemoglobin: Male: 13.8 to 17.2 gm/dL, Female: 12.1to


    Red blood cell indices : MCV: 80 to 95 femtoliter,

    MCH: 27 to 31 pg/cell, MCHC: 32 to 36 gm/dL

    Hemoglobin electrophoresis - is a test that measuresthe different types of the oxygen-carrying protein(hemoglobin ) in the blood. Confirms the diagnosis

    Serum Creatinine Clearance - is a breakdown productof creatine, which is an important part of muscle. The

  • 8/12/2019 Careplan for Sickle Cell Disease


    test is done to see how well your kidneys work.Creatinine is removed from the body entirely by thekidneys. If kidney function is abnormal, Creatininelevels will increase in the blood (because less Creatinine

    is released through your urine). Normal values - Anormal result is 0.7 to 1.3 mg/dL for men and 0.6 to 1.1mg/dL for women. Females usually have a lowerCreatinine than males, because they usually have lessmuscle mass.

    Sickle cell test - A sickle cell test looks for theabnormal hemoglobin in the blood that causes sickle cellanemia This test is done to tell if there is abnormalhemoglobin that causes sickle cell disease and sicklecell trait.

    In sickle cell disease, a person has two abnormalhemoglobin S genes. A person with sickle cell trait hasonly one of these abnormal genes and no symptoms, oronly mild ones. This test does not tell the difference

    between these two conditions

  • 8/12/2019 Careplan for Sickle Cell Disease


    Discuss thegeneralmanagement ofsickle cell as cited

    by Saunthararajah& Vichinsky(2012); Brawley,(2008)

    The goal of treatment is to manage and controlsymptoms, and to limit the number of crises. Peoplewith sickle cell disease need ongoing treatment, evenwhen not having a crisis. It is best to get care fromhealth care that take care of many patients with sicklecell anemia.

    People with this condition should take folic acidsupplements. Folic acid helps make new red blood cells.

    Treatment for a sickle cell crisis includes:

    Blood transfusions (may also be given regularlyto prevent stroke)

    Analgesics pain reduction Plenty of fluids make blood less viscous

    (Saunthararajah et al., 2012)

    Other treatments for sickle cell anemia may include:

    Hydroxyurea (Hydrea). Helps reduce the numberof pain episodes (including chest pain and

    breathing problems) in some people Antibiotics. Help prevent bacterial infections,

    which are common in children with sickle celldisease (Brawley, 2008)

    Treatments that may be needed to managecomplications of sickle cell anemia include:

    Dialysis for kidney transplant for kidney disease Counseling for psychological complications Gall bladder removal in people with gallstone

    Teacher will randomlyask students to discussthe general managementof sickle cell disease.

    Teacher will discuss thegeneral management ofsickle cell disease usingPowerPoint according tothe content

    Teacher will ask twostudents to discuss twogeneral managementmodalities for sickle celldisease according to thecontent

    Students willattempt to discussthe generalmanagement ofsickle cell disease

    Students will sit andlisten attentively,

    participate by askingquestions as thegeneral managementof sickle cell diseaseis discussed

    Two students willdiscuss two generalmanagementmodalities for sicklecell diseaseaccording to thecontent

    Students will be able todiscuss thegeneralmanagement ofsickle celldiseaseaccording tothe content

  • 8/12/2019 Careplan for Sickle Cell Disease


    disease Hip replacement for avascular necrosis of the hip Surgery for eye problems Treatment for overuse or abuse of narcotic pain

    medicines Wound care for leg ulcers

    Bone marrow or stem cell transplants can cure sicklecell anemia, but this treatment is not an option for most

    people. Sickle cell anemia patients often cannot findwell-matched stem cell donors.

    People with sickle cell disease should have thefollowing vaccinations to lower the risk of infection:

    Haemophilus influenzae vaccine (Hib) Pneumococcal Conjugate vaccine (PCV) Pneumococcal polysaccharide vaccine (PPV)

    (Saunthararajah et al., 2012)

    Discuss thenursingmanagement of

    patient will sicklecell diseaseutilizing thenursing process


    Question patients in crisis about factors thatcould have precipitated the crisis and measuresused to prevent crisis.

    Assess all body systems, with particularemphasis on pain (0to10 scale, quality, andfrequency), swelling, fever (all joint areas andabdomen).

    Carefully assess respiratory system, including breath sounds, oxygen saturation levels.

    Assess for signs of cardiac failure (edema,

    Teacher will ask one ofthe previously formedgroups to look at theassessment, one theinterventions andrationales and the otherthe patient outcomes

    Teacher will discuss thenursing management ofclients with sickle cell

    One of the previously formedgroups will look atthe assessment, onethe interventions andrationales and theother the patientoutcome

    Students will sit,listen, followPowerPoint

    Students will be able todiscuss thenursingmanagement ofsickle celldiseaseaccording tothe content

  • 8/12/2019 Careplan for Sickle Cell Disease


    increased point of maximal impulse, andcardiomegaly [as seen on chest xray]).

    Elicit symptoms of cerebral hypoxia by carefulneurologic examination.

    Assess for signs of dehydration and history ofuid intake; examine mucous membranes, skinturgor, urine output, serum creatinine, and BUNvalues.

    Assess for signs of any infectious process(examine chest and long bones and femoralhead, because pneumonia and osteomyelitis arecommon).

    Monitor hemoglobin, hematocrit, andreticulocyte count and compare with baselinelevels.

    Assess current and past history of medicalmanagement, particularly chronic transfusiontherapy, hydroxyurea use, and prior treatment forinfection.


    Reports control of pain Is free of infection Expresses improved sense of control Increases knowledge about disease process Experiences absence of complications


    Acute pain related to tissue hypoxia due toagglutination of sickled cells within blood

    according to the contentwith the aid ofPowerPoint presentation

    presentation, askquestions as isnecessary

  • 8/12/2019 Careplan for Sickle Cell Disease


    vessels Risk for infection Risk for powerlessness related to illness

    induced helplessness Decient knowledge regarding pre vention of



    Managing Pain

    Use patients subjective description of pain and pain rating on a pain scale to guide the use ofanalgesic agents.

    Support and elevate any joint that is acutelyswollen until swelling diminishes.

    Teach patient relaxation techniques, breathingexercises, and distraction to ease pain.

    When acute painful episode has diminished,implement aggressive measures to preservefunction (eg, physical therapy, whirlpool baths,and transcutaneous nerve stimulation).

    Preventing and Managing Infection

    Monitor patient for signs and symptoms ofinfection.

    Initiate prescribed antibiotics promptly. Assess patient for signs of dehydration. Teach patient to take prescribed oral antibiotics

    at home, if indicated, emphasizing the need tocomplete the entire course of antibiotic therapy.

  • 8/12/2019 Careplan for Sickle Cell Disease


    Promoting Coping Skills

    Enhance pain management to promote atherapeutic relationship based on mutual trust.

    Focus on patients strengths rather than decitsto enhance effective coping skills.

    Provide opportunities for patient to makedecisions about daily care to increase feelings ofcontrol.

    Increasing Knowledge

    Teach patient about situations that can precipitate a sickle cell crisis and steps to take to prevent or diminish such crises (eg, keep warm,maintain adequate hydration, avoid stressfulsituations).

    If hydroxyurea is prescribed for a woman ofchildbearing age, inform her that the drug cancause congenital harm to unborn children andadvise about pregnancy prevention.

    Monitoring and Managing Potential Complications Management measures for many of the potentialcomplications are delineated in the previous sections;additional measures should be taken to address thefollowing issues.


    Protect the leg from trauma and contamination. Use scrupulous aseptic technique to prevent

    nosocomial infections.

  • 8/12/2019 Careplan for Sickle Cell Disease


    Refer to a wound ostomy continence nurse,which may facilitate healing and assist with



    Teach patient to empty the bladder at the onsetof the attack, exercise, and take a warm bath.

    Inform patient to seek medical attention if anepisode persists more than 3 hours.


    Emphasize the importance of complying with prescribed treatment plan.

    Promote trust with patient through adequatemanagement of acute pain during episodes ofcrisis.

    Suggest to patient that receiving care from asingle provider over time is much more

    benecial than receiving care from rotating physicians and staff in an emergencydepartment.

    When a crisis arises, emergency department staffshould contact patients primary health care

    provider for optimal management. Promote continuity of care and establish written

    contracts with patient.

  • 8/12/2019 Careplan for Sickle Cell Disease