Sickle Cell Disease

1. Sickle Cell Disease www.nursing.oman-face.com Done by: nursing institutes fourms Evaluated by: prince of nursing

2. Patient history Mr. xxxx admitted to the male medical ward on xx/xx/xxxx complaining of severe pain in the back, L leg and chest. He is 26 years old, male, Omani and single live in Bahla. He is student in the 9th class. Vital signs was taking and showed normal value T: 36 C P: 80 beats/min, BP:110/70 mmHg, R: 20 breath/min. He had no fever, cough, or truma. Patient is a known case of sickle sell anemia since he was a child . He is admitted to the hospital every 2 to 3 weeks.

3. Past medical history: The pt known case of SCD since child hood and known case of epileptic since 2 years. He had history of frequent admission with VOC and on regular exchange transfusion. Last admission in 5/2007 with ACS and treated in ICU. Present medical history: The pt was brought by his friends to the hospital on 26/9/2007 with the complaints of fits and with loss of consciousness. He is also present with back and LL pain. Given PHENERGAN and improve slightly. No H/O incontinence disturbance, no chest pain, no cough no fever and no H/O trauma.

4. Definition of the Disease Sickle sell anemia is a sever hemolytic anemia that result from inheritance of the sickle hemoglobin (HbS) gene. In hemolytic anemia, the red blood cells have shortened life span; thus, the number of the red blood cells in the circulation is reduced result in decreased available oxygen.

6. Pathophysiology The sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change; consequently, the red blood cell containing (HbS) loses its round, very pliable, biconcave disk shape and become deformed, rigid, and sickle-shaped. These long, rigid red blood cells can adhere to the endothelium of the small vessel; when they pile up against each other, blood flow to a region or an organ may be reduced. If ischemia or infarction results, the patient may have pain, swelling, and fever.

8. The deformed red blood cells obstructs blood flow to tissue (vaso-occlusive crisis) joint pain, organ infarction and fatigue results due to hypoxia eventually. Virtually any organ may be affected by this, but the primary sites involve those areas with slowed circulation, such as the spleen, lungs, and central nervous system. Complications of the sickle cell anemia include infection, stroke, renal failure, heart failure, and pulmonary hypertension.

10. According to book -Gene Disorder (HbS). -Black people. -Nutrition deficiency. -Infection. -Cardiac dysfunction. -Impaired production of RBC. -Decrease destruction of RBC. In the patient - Patient have the disease and 1 of his brother. Etiology of the Disease:

11. According to book Anemia with hemoglobin value (7-10 g/dl) Pale Tachycardia Cardiac murmurs Enlarged heart In the patient Patient has (9.54 g/dl). The pt looks pale. The patient pulse was 80 beats/min. Patient hadn’t cardiac murmur. Patient hadn’t enlarged heart. Clinical Manifestations of the Disease:

12. Dysrhythmia Heart failure Enlargement of the bone of the face and skull Joint pain Organ infarction fatigue Patient pulse was regular Patient hadn’t heart failure Patient face is normal Patient was having joint pain when admitted Patient had no organ infarction Patient was not very fatigue

13. CBC Test

15. Treatment Non-Pharmacological • Encourage the patient to use relaxation techniques. • Teach the patient breathing exercise. • Apply moist heat to painful areas. • Position the patient in comfortable position.

16. Pharmacological

17. Folic acid: • Action • -Treatment of megoblastic anemia due to nutritional deficiency, infection, and childhood • -Required for nucleoprotien synthesis and maintenance of normal erythropoisis. • Possible Side Effects • - Hypersensitivity: Allergic reaction. • - Local: pain and discomfort.

18. Nurse’s Responsibilities • Monitor patient for hypersensitivity reaction, specially if drug previously taken. • Test using schilling test and serum vitamin B12 levels to rule out pernicious anemia. • Report rash, difficulty breathing, pain or discomfort.

19. Paracetamol • Action • - Inhibits the synthesis of prostaglandis in the CNS and peripherally blocks pain impuls generation. • - Artipyresis from inhibition of hypothalamus heat regulating center. • Possible side effects • CNS: Dizziness, insomnia, fatigue. • GI: nausea, dyspepsia, GI pain, vomiting. • Respiratory: dyspnea, bronchospasm, rhinitis • Hematologic: bleeding, aplastic anemia, platelet inhibtion when higher dose.

20. Nurse’s responsibility: • Take the drug only of complaint indicated, not on anti inflammatory agent. • Avoid the use of other OTC preparation. They may contain acetaminophen and serious overdose can occur. • - Report skin rash, unusual bleeding or bruising, yeiioe of the skin or eyes.

21. Hydroxyurea • Action • - Inhibits an enzyme that is crucial for DNA synthesis. • - Use to reduce frequency of painful crises and to reduce the need for blood transfusions in adult patient with sickle cell anemia. • Possible side effects • CNS: sedation, dizziness, headache, confusing, sweating, seizures. • GI: nausea, vomiting, dry mouth, constipation, flatulance.

22. Nurse’s responsibility • Prepare a calendar for dates to return for diagnostic testing and treatment days. • Arrange for regular blood tests to monitor the drug's effect. • Inform the pt to drink at least 10-12 glasses of water while on this drug. • Report fever, chills, sore throat, unusual bleeding or severe nausea and vomiting.

23. Nursing Care Plane: Nursing diagnosis: Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels as manifested by patient verbalize of pain and discomfort. Goal: To reduce pain.

25. Outcomes • The patient: • Identifies the importance of non-pharmacological treatment and uses it. • Increases fluid intake. • Verbalizes that pain is relief after administration of analgesic.

26. Nursing Diagnosis Risk for infection related to altered immunologic response as manifested by changes in the WBC count. Goal To prevent infection.

28. Outcomes • The patient: • Has clear breath sound. • Has WBCs within normal range(5000-10000 cu mm). • Has negative result of sputum cultures. • Pt report no signs and symptoms of infection.

29. Discharge Plan: • MR. xxxx 26 years old male, single, Omani. Pt admitted to the M.M.W on xx/xx/xxxx at 17:00 PM complained of back and LL pain. He is known case of sickle cell disease brought by his friends with back and LL pain. The pt had no history of incontinence disturbance, no fever, no cough and no trauma. • Discharge condition: • - The pt discharge on xx/xx/xxxx at 11:37 AM. Case of SCD, epilepsy admitted for exchange transfusion which was done after topping up BH levels. • - Post transfusions HB levels acceptable, discharge state improved.

30. PT is on medication:

31. Discharge advice / Health education: • Follow up in medicine clinic in NPC. • Avoid exposure to very hot or very cold environment. • Use the diversion activities (breathing exercise, relaxation technique and careful movement) to minimize the pain. • Avoid stressful situation and hard activities (plying football) because it can lead to sickle crisis. • Take food that including liver, green leafy vegetables and citrus food, wheat germ and regarding supplementary vitamins like Folic acid.

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