Sickle Cell Disease

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

2. Patient’s Demographic data:Name of patient: xxxxAge: 23 yearsAddress: xxxxReligion: MuslimMarital status: SingleLevel of education: class 11Occupation: Doesn’t workNationality: xxxxxData of hospitalization: xx-xx-xxxxDuration of hospitalization: 2 days

3. Health History • The patient came to the hospital because she complaints of severe joint pain’ pain in the arms, back and complete fatigue. • Vital signs: • BP:120/70mmHg • Temperature:37C • RR:20 per min • P:80 bate/ min

4. Medical Data: • Diagnosis • SCD with VOC with pain upper limb and lower limb.

5. Past medical History • The patient doesn’t have any significant illness but she has known case of SCD since she was child.

6. Definition of the disease: • Sickle cell anaemia is the most severe from of sickle cell disease. It is severe haemolytic anaemia that results from inheritance of the sickle haemoglobin gene. When the oxygen level in venous blood is low it to causes change in RBC containing haemoglobin HBS. Loses its round, biconcave disk shape, deformed and sickle shaped. The abnormal HGB turns RBCs in to sickle shaped. This decrease the oxygen carrying capacity of the RBC. If deformed RBC obstructs blood flow to tissue, ischemia or infarction results. Sickling crises are intermittent because RBC can revert to normal shape if it takes adequate oxygen.

8. According to books: Genetic factor. Increase destruction of RBC. Nutritional deficiency. Infection. Cardiac dysfunction. Impaired production of RBC. Undergoing anesthesia with out proper oxygenation. Climbing to high altitude. Excessive exercising Pulmonary dysfunction. In the patient: Genetic factors Nutrional deficiency Etiology Of Sickle-cell Disease

9. According to books: Jaundice , pale. Dysrhythmias and heart failure. Sudden change in temperature. There is sever pain in various part of the body. Loss of appetite. Weakness. Fever. Clinical Manifestations

10. According to books: .Ischemic necrosis or hypoxic damage. Bone pain: painful and swollen large joints. Cardiomegaly Splenomegaly. Decreased splenic function Clinical Manifestation

11. In the patient Pale Decrease in hgb 9 QIDC Loss of appetite Weakness Sudden change in temperature .Increase when she has pain 37.5C. Severe pain in various part of body. L and R lower and upper limbs. Pain score 8. Clinical manifestation:

12. PATHOPHYSIOLOGY Abnormal hemoglobin turns in to sickle shaped ↓ Decrease oxygen carrying capacity of the RBCs ↓ The deformed RBCs obstruct blood flow to tissue ↓ Vaso occlusive crisis ↓ Hypoxia ↓ Joint pain and organ infraction

13. Blood investigation:

14. Pharmacology:

15. Non pharmacologic: • Massage • Heat application

16. Tramadol: • Action: • Bind to mu- opioid receptors and inhibits the reuptake of norepinphrine and serotonin causes many effects similar to the opioids, dizziness, somuolence, nausea, constipation but does not have the respiratory depressant effects.

17. Tramadol: • Possible side effects: • CNS: Headache, fatigue. • CV: Tachycardia, angina pectoris. • CHF: hypotintion in salt or volume depleted pt. • Dermatologic: rash • GI: glupset, dirrhea. • GU: renal insufficiency, renal failure, polyuria, oliguria. • Others: cough, dizziness, malaise and dry mouth.

18. Tramadol • Nurse’s Responsibilities: • Administer once a day at same time each day.

19. Mefenamic Acid [ponstan ]: • Action: • Anti inflammatory, analgesic and antipyretic activities related to inhibition of prostaglandin synthesis exact mechanisms of action are not know.

20. Possible side effects: • CNS: headache, dizziness, insomnia, fatigue, tiredness and ophthalmic effect. • Dermatologic: rash, sweating, dry mucous membrane and stomatitis. • GI: nausea, dyspepsia, GI pain, dirrhea, vomiting and constipation. • GU: dysuria and renal impairment. • Hematologic: bleeding, platelets inhebition with higher doses and neutropenia. • Respiratory: dyspnea, hemoptysis, rhinitis. • Perepheral edema: anaphylactic shock..

21. Nurse’s responsibilities: • Give with milk or food to decrease GI upset. • Arrange for periodic ophthalmologic examinations during long term therapy.

22. Folic Acid: • Action: • Required for nucleoprotein synthesis and maintenance of normal erythropoiesis. • Possible side effects: • Hypersensitivity. • Local pain and discomfort at injection site.

23. Nurse’s Responsibility: • Administer orally if it all possible with severe GI malabsorpation or very severe disease give IM, IV or subcutaneously. • Test using schilling test and serum vitamin B12 levels to rule out pernicious anemia apy may mask signs of pernicious anemia while the neurologic deterioration continues.

24. Paracetamol: • Action: • Inhibits the synthesis of prostaglandins in the CNS and peripherally blocks pain impulse generation. • Artipyresis from inhibition of hypothalamus heat regulating centre.

25. Possible Side Effects: • Endocrine and metabolic: • May increase chloride, uric acid and glucose. • Hematologic:Anemia, blood dyscrasia. • Hepatic: may increase bilirubin. • Renal: nephrotoxiaty with over dosage.

26. NURSING CARE PLAN

30. Evaluation: • Report that pain is relieved promptly and lower discomfort. • Identifies effective pain relief strategies. • Use pain medication as prescribed . • Experiences minimal side effects of analgesia.

33. EVALUATION • Exhibits improve nutritional status by increasing weight and improve laboratory data. • States rationale for dietary modification • Identifies foods high in carbohydrates. • Report improved appetite. • hydration.Give him written instructions.Regarding the minimum amount of fluid required each day. • Instruct him to avoid strenuous physical activity. • Teach him to avoid undue emotional stress. • Instruct him to avoid areas of low oxygen concentration.

34. Nursing Diagnosis:Activity intolerance related to illness as manifested by pain, loss appetite and patient looks pale.GOAL:Encourage power.

36. Out come Criteria: • Expresses improved sense of control. • Participates in goal setting and in planning and implementing daily activity. • Participates indecisions about care.

37. Nursing Diagnosis: • Knowledge deficient regarding sickle crisis prevention related to lack of exposure/ recall, information misinterprtation and unfamiliarity with resources as manifested by in adequate nutrition in take and regular admition in the hospital. • Goal: • Verbalizes understanding of disease process and therapeutic needs, initiates behaviours/ lifestyle changes.

40. Out come Criteria: • Increases knowledge about disease process. • Identifies situations and factors that can precipitate sickle cell crisis. • Describes the importance of warmth, adequate hydration, and prevention of infection in preventing crisis.

41. Discharge Plan: • xxxx 23years old. She is from Nizwa. She came to NH because she complaints from arms and back pain. She was very tired, weakness, looks pale, fever. Her temperature 37.5C. BP 120/80mmHg. P 70bate/min. RR 20per/ min.

42. Health Education: • Exercise regularly but not so much that became really tired. • When do exercise, drink a lots of water. • Drink at least 8 glasses of water a day, specially during warm weather. • Avoid stress . • Treat any infection as soon as it occurs. • Wear warm clothes out side in cold weather. And don’t swim in cold water. • Tell the doctor if might has as sleep problem like snoring or some time during sleep- apnea. • If has any another medical condition like diabetes get treatment and control the condition. • Take food that including liver, green leafy vegetables, citrus fruits and wheat germ and regarding supplementary vitamins such as folic acid.

43. THE END