SICKLE CELL ANAEMIA ppp.pptx./ Edward Shoko

Sickle cell anaemia is one of the leading causes of deaths around the world, gradually increasing taking lives by the day. This PowerPoint will highlight much light on it describe the management of Sickle cell anaemia

1. SICKLE CELL ANAEMIA MRS SIANKOPE

2. INTRODUCTION Sickle cell disease (SCD) is a potentially devastating condition that is caused by an autosomal (non sex chromosomal) recessive inherited hemoglobinopathy which results in the vaso -occlusive phenomena and haemolysis . 3/7/2021 2

3. Intro’ The severity of the complications that occur with this disorder are widely variable, but overall mortality is increased and life expectancy decreased when compared to the general population. Sickle-cell is a hereditary blood disorder due to the presence of an abnormal hemoglobin S ( HbS ). 3/7/2021 3

4. Intro’ The sickling occurs because of a mutation in the hemoglobin gene. Life expectancy is shortened from 120 to less than 30 days. Sickle-cell disease, usually affect people (or their descendants) from parts of tropical and subtropical regions 3/7/2021 4

5. GENERAL OBJECTIVES: At the end of the discussion, students should be able to demonstrate knowledge and understand Sickle cell disease and its management. 3/7/2021 5

6. SPECIFIC OBJECTIVES At the end of the lesson students should be able to; Define sickle cell disease Outline the types of sickle cell disease. Explain the pathophysiology of Sickle cell aneamia State the signs of symptoms of Sickle cell aneamia . 3/7/2021 6

7. SPECIFIC OBJECTIVES CONT’D Describe the management of Sickle cell aneamia . Outline the complications of Sickle cell aneamia . State the types of Sickle cell crises Mention the predisposing factors to sickling of red blood cell in Sickle cell aneamia . 3/7/2021 7

8. SICKLE CELL DISEASE It is a severe, chronic, hereditary haemolytic disorder due to the homozygous presence of haemoglobin S, usually characterized by pallor and recurrent crises. (Baliga2005) 3/7/2021 8

9. SICKLE CELL DISEASE CONT’D Sickle cell disease is a severe haemolytic anaemia occurring in persons who are homozygous for sickle cell gene, Characterized by episodes of pain caused by occlusion of small blood vessels by sickled red blood cells.( Lippincott & Wilkins 2001) 3/7/2021 9

10. TYPES OF SICKLE CELL DISEASE There are basically three, i.e Sickle cell disease (sickle cell Anaemia): There is homozygous inheritance of Hb S (Hb SS). It is symptomatic . Sickle cell trait: There is heterozygous inheritance of Hb S (Hb SA). It is asymptomatic. 3/7/2021 10

11. CONT’D Sickle cell syndromes: Associated with presence of Hb S (Hb SC sickle cell Hb C) HBSD (Sickle cell Hb D) 3/7/2021 11

12. TYPES OF HAEMOGLOBIN Adult Haemoglobin (HbA) denoted be the letter A Composed of 2 alpha and 2 beta chains Fetal haemoglobin (HbF) denoted by the letter F Composed of 2 alpha and 2 gamma chains Other abnormal Hb include: C (Hb C - lysine substituted for glutamic acid), D etc 3/7/2021 12

13. SICKLE CELL INHERITANCE Parent 1 Parent 2 Normal Trait Disease AS AA 50% 50% 0% AS AS 25% 50% 25% SS SS 0% 0% 100%   3/7/2021 13

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16. PATHOPHYSIOLOGY The Haemoglobin A consists of 4 molecules of haem folded in 1 molecule of globin. Each globin molecule consists of 2 alpha and 2 beta chains. The amino acid sequence on the beta chain is altered at the 6 th position of the 574 amino acids that makes up the globin fraction of hemoglobin (Lippincott & Wilkins, 2001). 3/7/2021 16

17. The hydrophilic amino acid glutamine is replaced by the hydrophobic amino acid valine changing the properties of the Hemoglobin . 3/7/2021 17

18. The gene defect is a known mutation of a single nucleotide of the β-globin gene, which results in glutamic acid being substituted by valine at position 6. Haemoglobin S with this mutation is referred to as HbS , as opposed to the normal adult HbA . The genetic disorder is due to the mutation of a single nucleotide, from a GAG to GTG codon mutation. 3/7/2021 18

19. This change results in change of characteristics of the haemoglobin . When the HbS is subjected to low oxygen tension the abnormal beta chain contracts and piles together within the red blood cell this distorts the shape of the red blood cell. 3/7/2021 19

20. The cells under go polymerization and clump together and form masses of RBCs called tactoids . This distorts the cell structure of the red blood cell. Usually the cell returns their normal shape, after the low oxygen conditions are removed and proper oxygenation occurs. 3/7/2021 20

21. Although the cell may appear normal at least some of the Hb remains twisted decreasing the flexibility of the cell. The repeated sickling of the cell lead to permanent distortion of the cell structure adopting a characteristic crescent (sickled) shape due to cell membrane damage. 3/7/2021 21

22. The sickled cell increases the viscosity of blood and the cells easily attach to the endothelial cell causing occlusion of the red blood cell. These cells block the blood flow leading to sickling of other RBCs with more obstruction of blood vessels and ischemia of the affected tissues. 3/7/2021 22

23. Repeated episodes of ischaemia leads to progressive damage from infarction of the affected tissues The cell also becomes more fragile and easily hemolysed due to structural change. Its life span reduces from 120 to less than 30 days. The reduced life span of the RBC and increased destruction of red blood cells causes haemolytic anaemia. 3/7/2021 23

24. The patient also experiences periodic episodes of cellular sickling called crises, characterized by high fever, general body pain 3/7/2021 24

25. SIGNS AND SYMPTOMS OF SICKLE CELL DISEASE Attacks of abdominal pain due to tissue ischaemia Bone pain due to ischaemia Breathlessness due to cardio pulmonary involvement and intra pulmonary sickling Delayed growth and puberty because energy demands of the bone marrow for red blood cell production compete with the demands of a growing body. Fatigue due to tissue hypoxia 3/7/2021 25

26. Fever due the inflammatory reaction caused by tissue infarction and sometimes co-existing infections Jaundice due to increased levels of bilirubin as a result of increased haemolysis Pallor due to low hemoglobin Tachycardia as the heart attempts to compensate for hypoxia Susceptibility to infections due to low immunity 3/7/2021 26

27. Bloody urine (haematuria) due to renal damage following repeated tissue infarction Chest pain due to pulmonary infarction or cardiac ischemia Restlessness especially during crises due to pain Painful erection (priapism; this occurs in 10 - 40% of sufferers of the disease) due to blood being trapped by occluded blood vessel 3/7/2021 27

28. TYPES OF CRISES There are four (4) types of sickle cell crises ie ; Vaso-occlusive crisis Aplastic crisis Sequestration crisis Haemolytic crisis. 3/7/2021 28

29. VASO-OCCLUSIVE CRISIS The vaso -occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries causing ischaemia and infarction. This results in ischaemia , pain, necrosis and often organ damage. The frequency, severity, and duration of these crises vary considerably. 3/7/2021 29

30. Painful crises are treated with hydration, analgesics, and blood transfusion; pain management requires opioid administration at regular intervals until the crisis has settled. For milder crises, a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen). 3/7/2021 30

31. SPLENIC SEQUESTRATION CRISIS Splenic sequestration crises : are acute, painful enlargements of the spleen. The sinusoids and gates would open at the same time resulting in sudden pooling of the blood into the spleen and circulatory defect leading to sudden hypovolaemia . The abdomen becomes bloated and very hard. Splenic sequestration crises are considered an emergency. 3/7/2021 31

32. If not treated, patients may die within 1–2 hours due to circulatory failure. It is the commonest cause of death in children with sickle cell.   Management is supportive, sometimes with blood transfusion. This crises is transient, it continues for 3–4 hours and may last for one day. 3/7/2021 32

33. Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle-cell anaemia. This autosplenectomy increases the risk of infection from encapsulated organisms (such as Haemophilus influenza and streptococcus pneumoniae), therefore, preventive antibiotics and vaccinations are recommended. 3/7/2021 33

34. APLASTIC CRISIS Aplastic crises occur when there is acute worsening of the patient's baseline anaemia. This crisis is triggered by parvovirus B19, which directly affects erythropoiesis by invading the red cell precursors and multiplying in them and destroying them. Parvovirus infection nearly completely prevents red blood cell production. 3/7/2021 34

35. In normal individuals, this is of little consequence, but the shortened red cell life of sickle-cell patients’ results in an abrupt, life-threatening situation. Reticulocyte counts drop dramatically during the disease (causing reticulocytopenia ), This leads to low oxygen tension precipitating a crisis 3/7/2021 35

36. HAEMOLYTIC CRISIS The HbS is very fragile leading to a high red blood cells break down. This leads to acute accelerated drops in haemoglobin level. The result is low oxygen tension there by precipitating a crisis. Management is supportive, sometimes with blood transfusions 3/7/2021 36

37. PREDISPOSING FACTORS TO A CRISIS Dehydration Infection Strenuous exercises Severe trauma Exposure to cold Change of altitude from low to high with low oxygen concentration 3/7/2021 37

38. MANAGEMENT OF A PATIENT WITH SICKLE CELL ANAEMIA HEALTH HISTORY When taking specific history of sickle cell from the caretaker, ask questions that are more specific to the condition. 3/7/2021 38

39. CONT’D PHYSICAL EXAMINATION Physical examination includes: - Direct observation / inspection of the entire body to rule out the clinical features of sickle cell eg. Anaemia, growth retardation,etc 3/7/2021 39

40. CONT’D INVESTIGATIONS Full blood count will reveal haemoglobin levels in the range of 6–8 g/ dL with a high Reticulocyte count (as the bone marrow compensates for the destruction of sickle cells by producing more red blood cells). Sickling test will show sickling of cells (This test does not tell you if patient is trait or disease) 3/7/2021 40

41. TREATMENT Antibiotics e.g. e.g. crystalline penicillin 1-2mu qid for 5/7 Oxygen therapy to relieve hypoxemia e.g. 5l/minute Narcotic analgesics like pethidine 1mg/kg body weight for 3/7 or morphine Folic acid 5-10 mg od for 14/7 Blood transfusion with packed cell may be give 3/7/2021 41

42. TREATMENT CONT’D If malaria is present antimalarials will be given Non steroidal anti inflammatory drugs such as aspirin 150-600mg tds for 3/7 or diclofenac or B rustan . Iv fluid with normal saline to relieve dehydration e.g. 150ml /kg in 24hours 3/7/2021 42

43. NURSING CARE Problem/need J oint/abdominal pain Nursing Diagnosis Pain due to tissue ischaemia and infarction manifested by verbalization and restlessness Objective To relieve pain within 1 hour 3/7/2021 43

44. CONT’D Nursing intervention/ rationale I will:- Offer prescribed pethidine 1mg/kg body weight PRN in order to relieve pain and comfort I will do warm compresses on the painful areas to relieve pain I will give prescribed normal saline to rehydrate patient thereby relieve ischaemia and subsequently pain 3/7/2021 44

45. CONT’D Expected outcome Pain relieved within1 hour evidenced by patient verbalisation or being calm. 3/7/2021 45

46. CONT’D Problem/needs identified I neffective breathing pattern Nursing diagnosis Ineffective breathing pattern due to intrapulmonary sickling resulting in impaired gaseous exchange manifested by use of accessory muscles of respiration and flaring of nares Objective To relieve improve breathing pattern within 30 minutes 3/7/2021 46

47. CONT’D Nursing interventions/ rationale I will offer oxygen therapy 2-3l/minute If patient is old enough I will prop him up to promote lung expansion and thereby relieving dyspnea Expected outcome Breathing pattern improved within 30 minutes evidenced by reduced use of accessory muscles and no flaring of nares 3/7/2021 47

48. Other problems Activity intolerance due to pain Anxiety due to repeated crises Knowledge deficit if older child Impaired tissue oxygen perfusion Risk for infection Risk for cerebral tissue damage 3/7/2021 48

49. POSSIBLE COMPLICATIONS Sickle-cell anaemia can lead to various complications, including: Post Auto splenectomy infection (OPSI), this is due to functional asplenia (absence of spleen function), caused by encapsulated organisms such as Streptococcus pneumoniae and Haemophilus influenzae .. Priapism due to trapping of blood in the penis 3/7/2021 49

50. CONT’D Stroke, which can result from a progressive narrowing of blood vessels, preventing oxygen from reaching the brain. Cerebral infarction occurs in children. Cholelithiasis (gallstones) and cholecystitis, which may result from excessive bilirubin production and precipitation due to prolonged haemolysis 3/7/2021 50

51. CONT’D Avascular necrosis (aseptic bone necrosis) of the hip and other major joints, which may occur as a result of ischaemia . Decreased immune reactions due to hyposplenism (malfunctioning of the spleen). 3/7/2021 51

52. CONT’D Acute papillary necrosis in the kidneys. Chronic renal failure due to Sickle cell nephropathy Leg ulcers due to ischaemia . In eyes, background retinopathy, proliferative retinopathy, vitreous haemorrhages and retinal detachments, resulting in blindness. 3/7/2021 52

53. CONT’D During pregnancy (in adolescence stage) intrauterine growth retardation, spontaneous abortion, and pre-eclampsia . Cardiomyoparthies due to pulmonary hypertension leading to strain on the right ventricle and a risk of heart failure. Typical symptoms are shortness of breath, decreased exercise tolerance and episodes of syncope. 3/7/2021 53

54. PREVENTING CRISES The following steps can help prevent a sickle cell crisis : M aintain good oxygen levels and prevent dehydration. Avoid strenuous activities, stress, smoking, high-altitudes, no pressurized flights, and other events that reduce your oxygen level. Always have plenty of fluids with you Avoid too much sun exposure Consider having the child with sickle cell anemia wear a Medic Alert bracelet. Share the above information with teachers and other caretakers, when necessary . 3/7/2021 54

55. REFERRENCES Lewis S.M., Colier I. C. and Heitkemper , M.M. (1996) Medical and Surgical Nursing: Assessment and Management of clinical practice , 4 th edition, Mosby – Year Book Inc , st Louis. Marlow D. (1973) Text book of Paediatric Nursing , 4 th edition, Saunders comp, Philadelphia. 3/7/2021 55

56. Smeltzer S.C. & Bare B.G.(2004), Text book of Medical and Surgical Nursing , 10 th edition, Lippincott Willam & Wilkins, Philadelphia. Wong L. D. (1995), Nursing care of Infants and Children . 5 th edition, Mosby Year Book inc. st Louis, USA. Roper N., Logan W.W. & Tierney A.J. (1980). The Elements of Nursing . Churchill Livingstone 3/7/2021 56

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