Topic: Audience: Date: Time: Duration: Venue:

Caring for the patient with Sickle Cell Disease 3rd year BScN. Student Nurses (NCU) September, 2013 10am-10:45am 45 Minutes 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 concrete). Though he specified that his theory applied only to reception learning in schools, it was utilized because it introduced the topic and aided the sequence of the information to be imparted. (Ormrod & Rice, 2003). Rogers: Dealt with the adult learner, he posited that learning is student centered and personalized and the educators role is that of a facilitator. Affective and cognitive needs are central and the goal is to develop self-actualized persons in a cooperative, supportive environment. This theory was used because all the participants were adult learners, thus they

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. Bruners 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 promotes autonomy, responsibility, independence, the development of creativity and problem solving skills. (Quinn et al., 2009) Bruners theory was used because it encouraged active engagement, promotes motivation, a tailored learning experience, and promotes autonomy, responsibility, independence and the development of creativity and problem solving skills for this presentation. Vygotsky: Posited that individuals learn from each other through social interaction and the teacher and the learner collaborate in a reciprocal relationship where each learns from each other through the same process of social interaction (Ormrod et al., 2003). This theory was chosen

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: Objectives: Registered nurse, lap top computer, multimedia, white board, markers At the end of 11/2 hours interactive session students should be able to 1. Define Definitions sickle cell according to Vera (2012) 2. Explain the causes/Risk Factors/Pathophysiology for sickle cell disease as cited by Smelter et al, (2010) and Vera (, 2012) 3. State the manifestations of sickle cell disease according to Smelter, et al (2010) and Rees et al., 2010) 4. Describe the types of tests used in the diagnosis of sickle cell disease according to A.D.A.M Encyclopedia, (2013)

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 Publishing. Quinn, F & Hughes, S. (2009). Quinns principles and practice of nurse education (5th ed.) Cheltenham: Nelson Thornes

Rees, D. C., Williams, T. N & Gladwin, M. T. (2010). Sickle-cell disease. Lancet. 376(9757): 2018-2031.

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. (12th ed.)Philadelphia: Wolters Klower Health/Lippincott Williams & Williams Vera, M. (2012). Sickle cell anemia nursing management and interventions. Retrieved on August 18th, 2012 from http://nurseslabs.com/sickle-cell-anemia-nursing-management/

Objectives Ice breaker

Contents

Teachers Objectives Students will be asked to relate the picture to the topic that will be discussed

Learners Objectives Students will attempt to relate the picture to the topic that will be discussed

Evaluation

Definition the term sickle cell anemia according to Vera (2012)

Sickle cell anemia is a severe hemolytic anemia resulting from the inheritance of the sickle hemoglobin (HbS) gene, which causes a defective hemoglobin molecule (Vera, 2012). Also called Sickle cell, Sickle cell disease, Hbss

Teacher will ask students to break out into three groups to define the term sickle cell anemia. Each group will identify a leader who will give the feedback on the definition of the term.

Students will break out into three groups, one person identified as group leader will give the feedback from the groups attempt to define the term sickle cell anemia in their own words. Students will sit attentively and listen as the terms group and small group discussion are defined with the aid of PowerPoint according to the

Teacher will define the term sickle cell anemia with the aid of PowerPoint presentation, markers and white board according to the content.

Student will be able to correctly define the term sickle cell anemia, using at least two key terms each from the content such as: is a severe hemolytic anemia resulting from the inheritance of the sickle hemoglobin (HbS) gene, which causes a

content. One student will correctly define the term sickle cell anemia according to the content using at least two key words such as: a severe hemolytic anemia resulting from the inheritance of the sickle hemoglobin (HbS) gene, which causes a defective hemoglobin molecule

defective hemoglobin molecule

Teacher will ask a student from one group to define the term sickle cell anemia according to the content

Explain the Causes/Risk Factors Causes/Risk Factors/Pathophysi Sickle cell anemia is caused by an abnormal type of ology for sickle hemoglobin called hemoglobin S. Hemoglobin is a

Teacher will ask one student from each of three to explain the cause, risk factor or the

One student from each of the three groups will attempt to explain in their

Students will be able to correctly explain the

cell disease as cited by Smelter et al, (2010) Vera (, 2012)

protein inside red blood cells that carries oxygen.

pathophysiology for sickle cell disease

Hemoglobin S changes the red blood cells. The red 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 blood vessels and break into pieces. This can interrupt healthy blood flow and cut down even more on the amount of oxygen flowing to body tissues.

With the aid of white board and PowerPoint presentation and white board, teacher will state the purposes for small group discussion

own words, the cause, risk factors, or the pathophysiology for sickle cell disease

causes/risk factors/pathoph ysiology for sickle cell disease according to the content

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 trait do not have the symptoms of sickle cell anemia. Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, Aboriginal tribes in India and the Caribbean (Smelter et al., 2010)

Teacher will ask one student from each group to state two purposes of small group discussions according to the contents (may solicit help from group)

Students will sit attentively, follow PowerPoint presentation and ask questions as necessary

One student from each group will state two purposes for small group discussion according to the contents

Pathophysiology
The defective hemoglobin molecule assumes a sickle shape when exposed to low oxygen tension. These long, rigid RBCs become lodged in small vessels and can obstruct blood ow to body tissue. If ischemia or infarction results, the patient may have pain, swelling, and fever. The sickling process takes time; if the erythrocyte is again exposed to adequate amounts of oxygen (eg, when

it travels through the pulmonary circulation) before the membrane becomes too rigid, it can revert to a normal shape. For this reason, the sickling crises are intermittent. The HbS gene is inherited, with some people having the sickle cell trait (a carrier, inheriting one abnormal gene) and some having sickle cell disease (inheriting two abnormal genes). Sickle cell disease is found predominantly in people of African descent and less often in people who have descended from the Mediterranean countries, the Middle East, or aboriginal tribes of India (Vera, 2012)

State the manifestations of sickle cell disease according to Smelter, et al (2010) and Rees et al., 2010)

Symptoms usually do not occur until after the age of 4 months. Varies and depends somewhat on the amount of Hbs. 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. Others have many episodes each year. The crises can be severe enough to require a hospital stay. When the anemia becomes more severe, symptoms may 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)

Teacher will ask two students at the front, two in the middle and three at back of the class to state the clinical manifestation of sickle cell disease

Two students at the front, two in the middle and three at the back of the class will attempt to state the manifestation of sickle cell disease

Students will be able to correctly state the manifestations of sickle cell disease according to the content.

Teacher will outline the manifestations of sickle cell disease with the aid of PowerPoint and screen according to the content

Students will sit, listen and following PowerPoint presentation as the manifestations of sickle cell disease are outlined Five students will randomly chosen will state the manifestations of sickle cell disease according to the contents

Teacher will randomly ask five students to state the manifestations of sickle cell disease according to the contents

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)

Over time, the spleen stops working. As a result, people with sickle cell anemia may have symptoms of infections 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, or sequestration crisis. Due to insufficient blood flow to specific 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 of sickle cell disease, and often the cause of death (Smelter et al., 2010).

Describe the types of tests used in the diagnosis of sickle cell disease according to A.D.A.M Encyclopedia, (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 blood cells are replaced by new blood cells every day. Bilirubin is left after these older blood cells are removed. The liver helps break down bilirubin so that it can be removed from the body in the stool. Large amounts of bilirubin in the blood can lead to jaundice. Jaundice is a yellow color in the skin, mucus membranes, or eyes. Jaundice is the most common reason to check bilirubin levels. Normal levels are:

Teacher will ask the previously formed three groups, to take one piece of paper that will be given and attempt explain the terms the terms that are written on them

Each of the three group will take one piece of paper each from teacher and will attempt explain the terms written on them.

Students will be able to correctly describe the types of tests used in the diagnosis of sickle cell disease according to the contents

Direct (also called conjugated) bilirubin: 0 to 0.3 mg/dL Total bilirubin: 0.3 to 1.9 mg/dL

Teacher will explain/describe the types of test used to diagnose sickle cell disease according to the content with the aid of PowerPoint presentation

Student will sit, listen, follow PowerPoint presentation and ask questions if they arise

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 the following measurements:

Average red blood cell size (MCV)

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 the CBC

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: 12.1 to 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 measures the different types of the oxygen-carrying protein (hemoglobin ) in the blood. Confirms the diagnosis Serum Creatinine Clearance - is a breakdown product of creatine, which is an important part of muscle. The

test is done to see how well your kidneys work. Creatinine is removed from the body entirely by the kidneys. If kidney function is abnormal, Creatinine levels will increase in the blood (because less Creatinine is released through your urine). Normal values - A normal result is 0.7 to 1.3 mg/dL for men and 0.6 to 1.1 mg/dL for women. Females usually have a lower Creatinine than males, because they usually have less muscle mass. Sickle cell test - A sickle cell test looks for the abnormal hemoglobin in the blood that causes sickle cell anemia This test is done to tell if there is abnormal hemoglobin that causes sickle cell disease and sickle cell trait. In sickle cell disease, a person has two abnormal hemoglobin S genes. A person with sickle cell trait has only one of these abnormal genes and no symptoms, or only mild ones. This test does not tell the difference between these two conditions

Discuss the general management of sickle cell as cited by Saunthararajah & Vichinsky (2012); Brawley, (2008)

The goal of treatment is to manage and control symptoms, and to limit the number of crises. People with sickle cell disease need ongoing treatment, even when not having a crisis. It is best to get care from health care that take care of many patients with sickle cell anemia. People with this condition should take folic acid supplements. Folic acid helps make new red blood cells. Treatment for a sickle cell crisis includes:

Teacher will randomly ask students to discuss the general management of sickle cell disease.

Students will attempt to discuss the general management of sickle cell disease Students will sit and listen attentively, participate by asking questions as the general management of sickle cell disease is discussed Two students will discuss two general management modalities for sickle cell disease according to the content

Teacher will discuss the general management of sickle cell disease using PowerPoint according to the content

Students will be able to discuss the general management of sickle cell disease according to the content

Blood transfusions (may also be given regularly to prevent stroke) Analgesics pain reduction Plenty of fluids make blood less viscous (Saunthararajah et al., 2012)

Other treatments for sickle cell anemia may include:

Teacher will ask two students to discuss two general management modalities for sickle cell disease according to the content

Hydroxyurea (Hydrea). Helps reduce the number of pain episodes (including chest pain and breathing problems) in some people Antibiotics. Help prevent bacterial infections, which are common in children with sickle cell disease (Brawley, 2008)

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

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

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 sickle cell anemia, but this treatment is not an option for most people. Sickle cell anemia patients often cannot find well-matched stem cell donors. People with sickle cell disease should have the following vaccinations to lower the risk of infection:

Haemophilus influenzae vaccine (Hib) Pneumococcal Conjugate vaccine (PCV) Pneumococcal polysaccharide vaccine (PPV) (Saunthararajah et al., 2012)

Discuss the nursing management of patient will sickle cell disease utilizing the nursing process

ASSESSMENT

Question patients in crisis about factors that could have precipitated the crisis and measures used to prevent crisis. Assess all body systems, with particular emphasis on pain (0to10 scale, quality, and frequency), swelling, fever (all joint areas and abdomen). Carefully assess respiratory system, including breath sounds, oxygen saturation levels. Assess for signs of cardiac failure (edema,

Teacher will ask one of the previously formed groups to look at the assessment, one the interventions and rationales and the other the patient outcomes

One of the previously formed groups will look at the assessment, one the interventions and rationales and the other the patient outcome Students will sit, listen, follow PowerPoint

Students will be able to discuss the nursing management of sickle cell disease according to the content

Teacher will discuss the nursing management of clients with sickle cell

increased point of maximal impulse, and according to the content cardiomegaly [as seen on chest xray]). with the aid of Elicit symptoms of cerebral hypoxia by careful PowerPoint presentation neurologic examination. Assess for signs of dehydration and history of uid intake; examine mucous membranes, skin turgor, urine output, serum creatinine, and BUN values. Assess for signs of any infectious process (examine chest and long bones and femoral head, because pneumonia and osteomyelitis are common). Monitor hemoglobin, hematocrit, and reticulocyte count and compare with baseline levels. Assess current and past history of medical management, particularly chronic transfusion therapy, hydroxyurea use, and prior treatment for infection.

presentation, ask questions as is necessary

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 CELL ANEMIA

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

vessels Risk for infection Risk for powerlessness related to illness induced helplessness Decient knowledge regarding prevention of crisis

NURSING INTERVENTIONS Managing Pain

Use patients subjective description of pain and pain rating on a pain scale to guide the use of analgesic agents. Support and elevate any joint that is acutely swollen until swelling diminishes. Teach patient relaxation techniques, breathing exercises, and distraction to ease pain. When acute painful episode has diminished, implement aggressive measures to preserve function (eg, physical therapy, whirlpool baths, and transcutaneous nerve stimulation).

Preventing and Managing Infection

Monitor patient for signs and symptoms of infection. Initiate prescribed antibiotics promptly. Assess patient for signs of dehydration. Teach patient to take prescribed oral antibiotics at home, if indicated, emphasizing the need to complete the entire course of antibiotic therapy.

Promoting Coping Skills

Enhance pain management to promote a therapeutic relationship based on mutual trust. Focus on patients strengths rather than decits to enhance effective coping skills. Provide opportunities for patient to make decisions about daily care to increase feelings of control.

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 stressful situations). If hydroxyurea is prescribed for a woman of childbearing age, inform her that the drug can cause congenital harm to unborn children and advise about pregnancy prevention.

Monitoring and Managing Potential Complications Management measures for many of the potential complications are delineated in the previous sections; additional measures should be taken to address the following issues. LEG ULCERS

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

Refer to a woundostomycontinence nurse, which may facilitate healing and assist with prevention.

PRIAPISM LEADING TO IMPOTENCE

Teach patient to empty the bladder at the onset of the attack, exercise, and take a warm bath. Inform patient to seek medical attention if an episode persists more than 3 hours.

CHRONIC PAIN AND SUBSTANCE ABUSE

Emphasize the importance of complying with prescribed treatment plan. Promote trust with patient through adequate management of acute pain during episodes of crisis. Suggest to patient that receiving care from a single provider over time is much more benecial than receiving care from rotating physicians and staff in an emergency department. When a crisis arises, emergency department staff should contact patients primary health care provider for optimal management. Promote continuity of care and establish written contracts with patient.