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Sickle Cell Disease by Jessica Rispoli, and Tara Ford
Transcript of Sickle Cell Disease by Jessica Rispoli, and Tara Ford
Sickle cell disease changes normal, round red blood cells into cells that can be shaped like crescent moons. Normal red blood cells move easily through your blood vessels, taking oxygen to every part of your body. But sickled cells can get stuck and block blood vessels, which stops the oxygen from getting through causing a lot of pain. It can also harm organs, muscles, and bones. Having sickle cell disease means a lifelong battle against the health problems it can cause, such as pain, infections, anemia, and stroke. But many people are able to have a very good quality of life by learning to manage the disease.
Physical Exam & Laboratory Findings
Patient appears to be weak, lethargic, and slightly pale in color . When you examine him, you note he has a temperature of 101F, BP 162/84, pulse 110 bpm, resp.rate 25/min, his oxygen saturation on 2 L/min nasal cannula is 92%. Current weight 174 lb; height 5'8''.No conjunctival pallor, no scleral icterus. Oral cavity normal, normal dentition, Lung examination reveals bronchial breath sounds in all fields.
His laboratory examinations reveal elevated white blood cell and reticulocyte counts, and a hemoglobin and hematocrit that are slightly lower than baseline. Sickle and target cells are seen on the peripheral smear
25-year-old African American male, presented to the emergency room complaining of abdominal and bilateral lower extremity pain and tingling. Besides the abdominal and leg pain, he is now complaining of chest pain, which is worse on inspiration. Patient states his pain is a 9/10 while guarding his abdomen and chest. He also states that it is hard for him to do any activities because of the pain and shortness of breath he experiences while moving around. Although he is tender on palpation of his extremities, the remainder of his examination is normal.
Over the last month, symptoms have been occurring on a daily basis, with the patient occasionally awakening at night with similar symptoms which disturb his sleep. Patient has been using over-the-counter pain medications like Advil, and Aleve, he finds they help, but only relieve symptoms temporarily.
Patient has never seen a physician for similar symptoms or used prescription medication. Has family history of hypertension and the Sickle cell trait. Patient does not report any dysphagia, change in bowel habits, melena (black/tarry stool), bright red blood with stooling, or weight loss. Non- smoker; low alcohol consumption (average two beers per week).
What is Sickle Cell Disease?
A PATIENT''S JOURNEY
Impaired Gas Exchange R/T increased blood viscosity-occlusions created by sickled cells packing together within the capillaries AEB dyspnea, elevated leukocytes, low oxygen saturation, tachycardia
Chronic Pain R/T activation of pain fibers due to deprivation of oxygen and nutrients AEB pain scale report of 9/10;guarding of the affected areas
Ineffective Tissue Perfusion R/T vaso-cclusive nature of sickling, inflammatory response AEB changes in vital signs, angina, restlessness, and tingling in extremities
Promote adequate cellular oxygenation and perfusion.
Provide information about disease process, prognosis, and treatment needs.
Useful Medications for Sickle Cell Disease
Nursing Interventions; Impaired Gas Exchange
Monitor respiratory rate and depth, use of accessory muscles, and areas of cyanosis.
Monitor vital signs; note changes and cardiac rhythm.
Rationale: Indicators of adequacy of respiratory function or degree of compromise and therapy needs and effectiveness.
Auscultate breath sounds, noting presence or absence, and adventitious sounds.
Rationale: Development of atelectasis and stasis of secretions can impair gas exchange.
Rationale: Changes in vital signs and development of dysrhythmias reflect effects of hypoxia on cardiovascular system.
Decrease Stressors/ Strengthen Lines of Defense
Assist in turning, coughing, and deep breathing exercises.
Rationale: Promotes optimal chest expansion, mobilization of secretions, and aeration of all lung fields; reduces risk of stasis of secretions and pneumonia.
Administer supplemental humidified oxygen, as indicated.
Rationale: Maximizes oxygen transport to tissues, particularly in presence of pulmonary insults or pneumonia.
Perform or assist with chest physiotherapy, intermittent positive pressure breathing (IPPB), and incentive spirometry.
Rationale: Mobilizes secretions and increases aeration of lung fields.
Encourage client to alternate periods of rest and activity.
Rationale: Protects from excessive fatigue and reduces oxygen and degree of hypoxia.
Demonstrate and encourage use of relaxation techniques, such as guided imagery and visualization.
Rationale: Relaxation decreases muscle tension and anxiety and, hence, metabolic demand for oxygen.
Educate client about adequate fluid intake, such as 2-3 L/day within cardiac tolerance.
Rationale: Sufficient hydration is necessary to provide for mobilization of secretions and to prevent hyper-viscosity of blood with associated capillary occlusion.
Nursing Interventions: Chronic Pain
Assess reports of pain including location, duration, and intensity (scale of 0-10).
Observe nonverbal pain cues, such as gait disturbances, body positioning, reluctance to move, facial expressions, etc.
Rationale: Nonverbal cues may aid in evaluation of pain and effectiveness of therapy.
When assessing pain, have client help differentiate current pain from typical or usual pain problems.
Rationale: Client may also have acute pain from another cause (ulcers, appendicitis), chronic pain from sickle cell damage, or other causes (old injuries, arthritis).
Rationale: Vaso-occlusive pain is the most common manifestation of sickle cell crises, where sickling potentiates cellular hypoxia, resulting in severe pain.
Provide support for and carefully position affected extremities.
Rationale: Reduces edema, discomfort, and risk of injury, especially if osteomyelitis is present.
Apply local massage gently to affected areas.
Rationale: Helps reduce muscle tension.
Apply warm, moist compresses to affected joints or other painful areas. Avoid use of ice or cold compresses.
Rationale: Warmth causes vaso dilation and increases circulation to hypoxic areas. Cold causes vasoconstriction and componds the crisis.
Discuss with the client/SO what pain relief measures were effective in the past.
Rationale: Involves client/SO in care and allows for identification of remedies that have already been found to relieve pain.
Encourage ROM exercises.
Rationale: May reduce joint stiffness and possible contracture formation.
Explore alternative pain relief measures, such as relaxation techniques, biofeedback, yoga, meditation, and distraction.
Rationale: Cognitive-behavioral interventions may reduce reliance on pharmacological therapy and enhance clients sense of control.
Nursing Interventions: Ineffective tissue Perfusion
decrease moderate to severe pain
(Morphine, Dilaudid; patient controlled analgesia (PCA), oral form
Non-steroidal Anti-Inflammatory Agents (NSAIDS) ;
decrease mild to moderate pain/reduce inflammation
(Advil, Aleve, Aspirin )
reduction of painful crisis in sickle cell anemia
Drugs to help prevent sepsis; pt more prone to bacterial infection (spleen function decreased);
Penicillin; reduces number of pneumonia/streptococcal infections);
Patient and family will be educated about current condition, side effects, prescribed medication, and lifestyle changes that need to be made. Generic hydroxyurea will be prescribed and a follow-up visit will be arranged in four weeks’ time.
After four weeks, the patient returned and reported improved relief of his symptoms. He did not report any side effects related to the medication. His physical exam was improved from the previous visit, vital signs were within normal limits. He reports having less pain and no shortness of breath with activity, also no longer has trouble sleeping at night.
Monitor vital signs carefully. Assess pulses for rate, rhythm, and volume.
Assess skin for coolness, pallor, cyanosis, diaphoresis, and delayed capillary refill.
Assess lower extremities for skin texture, edema, and ulcerations.
Rationale: Changes reflect diminished circulation and hypoxia potentiating capillary occlusion.
Rationale: Reduced peripheral circulation often leads to skin and underlying tissue changes and delayed healing.
Rationale: Sludging and sickling in peripheral vessels may lead to complete or partial obliteration of a vessel with diminished perfusion to surrounding tissues.
Maintain environmental temperature and body warmth without overheating. Avoid hypothermia.
Rationale: Prevents vasoconstriction, aids in maintaining circulation and perfusion.
Administer IV solutions, such as 0.45% normal saline, via an infusion pump.
Rationale: Hydration lowers the hemoglobin S concentration, which decreases the sickling tendency and also reduces blood viscosity, which helps to maintain perfusion.
Administer hydroxyurea (Droxia) or experimental anti-sickling agents.
Rationale: Hydroxyurea, a cytotoxic agent, dramatically decreases the number of sickle cell episodes, and is given to prevent crises.
Teach client to report changes LOC.
Rationale: Changes may reflect diminished perfusion to the CNS due to ischemia or infarction (stroke).
Teach client to maintian adequate fluid intake, and monitor urine output.
Rationale: Dehydration not only causes hypovolemia but increases sickling and occlusion of capillaries.
Teach client to report change in character of pain, or development of bone pain, angina, tingling of extremities, and eye pain or vision disturbances.
Rationale: Changes may reflect increased sickling of cells and impaired circulation with further involvement of organs.
Evaluation of Care
Definition: Recognize the patient or designee as the source of control and full partner in providing compassionate and coordinated care based on respect for patient's preferences, values, and needs.
*Patient's presence and extent of pain and suffering will be evaluated, physical and emotional comfort will be assessed, and patient and family will be expected to learn about relief of pain, and comfort for patient. Effective treatments to relieve pain will will be initiated.
Definition: Minimizes risk of harm to patients and providers through body system effectiveness and individual performance.
*Organizational error reporting systems for near miss and error reporting will be utilized. Observations or concerns related to hazards and errors to patients, families, and health care team will be communicated.
Definition: Use data to monitor the outcomes of care processes and use improvement methods to design and test changes to continuously improve the quality and safety of health care systems.
*Tools (such as flow-charts, cause-effect diagrams) to make processes of care explicit will be utilized. Measures and changes involved in improving care will be practiced.
Definition: Integrate best current evidence with clinical expertise and patient/family preferences and values for delivery of optimal health care.
*Effective appropriate data collection and other research activities will be participated in. Individual care plan will be based on patient values, clinical expertise, and evidence.
Teamwork and Collaboration
Definition: Function effectively within nursing and inter-professional teams, fostering open communcation, mutual respect, and shared decision-making to achieve quality patient care.
*Requests for help will be initiated when appropriate to situation. The contribution of others who play a role in helping patient/family achieve health goals will be integrated.
Definition: Use information and technology to communicate, manage knowledge, mitigate error, and support decision making.
*Technology and information management tools to support safe processes of care will be applied. Patient care will be planned and documented in an electronic health record.
American Pain Society
Web Address: www.ampainsoc.org
The American Pain Society is a group of scientists, clinicians, and others. This group seeks to advance pain-related research, educate people about pain, and change public policy and clinical practice to reduce pain-related suffering.
Centers for Disease Control and Prevention (CDC): National Center on Birth Defects and Developmental Disabilities (NCBDDD)
Web Address: www.cdc.gov/ncbddd
NCBDDD aims to find the cause of and prevent birth defects and developmental disabilities. This agency works to help people of all ages with disabilities live to the fullest. The website has information on many topics, including genetics, autism, ADHD, fetal alcohol spectrum disorders, diabetes and pregnancy, blood disorders, and hearing loss.
March of Dimes
Web Address: www.marchofdimes.com
The March of Dimes tries to improve the health of babies by preventing birth defects, premature birth, and early death. March of Dimes supports research, community services, education, and advocacy to save babies' lives. The organization's website has information on premature birth, birth defects, birth defects testing, pregnancy, and prenatal care.
National Heart, Lung, and Blood Institute (NHLBI)
Web Address: www.nhlbi.nih.gov
The U.S. National Heart, Lung, and Blood Institute (NHLBI) information center offers information and publications about preventing and treating:
Diseases affecting the heart and circulation, such as heart attacks, high cholesterol, high blood pressure, peripheral artery disease, and heart problems present at birth (congenital heart diseases).
Diseases that affect the lungs, such as asthma, chronic obstructive pulmonary disease (COPD), emphysema, sleep apnea, and pneumonia.
Diseases that affect the blood, such as anemia, hemochromatosis, hemophilia, thalassemia, and Von Willebrand disease.
Sickle Cell Disease Association of America (SCDAA)
Web Address: http://www.sicklecelldisease.org
The Sickle Cell Disease Association of America is a national membership organization that provides education, research updates, and support around sickle cell disease problems. Its Web site has the Sickle Cell Disease Forum for people of all ages to discuss concerns about sickle cell disease.