Careplan for Sickle Cell Disease

8/12/2019 Careplan for Sickle Cell Disease

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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


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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


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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


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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

objective.

References:

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

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001554/

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


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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 http://nurseslabs.com/sickle-cell-anemia-nursing-management/


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Teacher will ask a

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

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

defectivehemoglobinmolecule

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


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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)

Pathophysiology

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


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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

adults)

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.


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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).


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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


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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

44.3%

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

15.1gm/dL

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


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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


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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


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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

ASSESSMENT

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


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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.

EXPECTED PATIENT OUTCOMES

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

NURSING DIAGNOSES FOR SICK CELLANEMIA

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


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vessels Risk for infection Risk for powerlessness related to illness

induced helplessness Decient knowledge regarding pre vention of

crisis

NURSING INTERVENTIONS

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.


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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.

LEG ULCERS

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

nosocomial infections.


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

prevention.

PRIAPISM LEADING TO IMPOTENCE

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.

CHRONIC PAIN AND SUBSTANCE ABUSE

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.


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Careplan for Sickle Cell Disease

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