Sickle cell disease is a genetic blood disorder that affects millions worldwide, particularly those of African, Mediterranean, Middle Eastern, and Indian descent. It is caused by a mutation in the beta-globin gene that causes red blood cells to take on a rigid, sickle shape. These sickled cells can block blood vessels and damage tissues, causing pain crises, organ damage, strokes, and early death. Treatments aim to relieve pain, prevent infections that can trigger crises, and reduce complications through medications, transfusions, hydration, and lifestyle management.

1. Mrs Thandiwe Mwanza Mwikisa
Lecturer

2. INTRODUCTION
 Sickle cell disease affect millions of people world
wide
 The highest prevalence is among people of Africa,
southern America, Arabian peninsula and
Mediterranean countries
 25 to 30% of neonates in western Africa are
carriers of sickle cell trait
 Affected patients characteristically are
asymptomatic until 4 to 6 months of age

3.  The disease originated in at least 4 places in Africa,
Mediterranean countries (such as Turkey, Greece,
and Italy), and in the Indian/Saudi Arabian
subcontinent. It exists in all countries of Africa and
in areas where Africans have migrated.
 In the United States with an estimated population of
over 270 million, about 1,000 babies are born with
sickle cell disease each year.
 In contrast, Nigeria, with an estimated 1997
population of 90 million, 45,000-90,000 babies with
sickle cell disease are born each year.

4. CONT
 Sickle cell is a genetic disorder caused by an
autosomal recessive single gene defect in the B-
globin chain of HbA
 Complete Hb molecule consist of four separate
chains of amino acids joined together
Alpha chain (2)
Beta chain (2)
Each chain is alike with similar sequence of amino
acids

6.  Hemoglobins bind oxygen in a cell, but amino
acids can switch with betaglobins making the
cell nonfunctional

7. TYPES OF HAEMOGLOBIN
 Adult Haemoglobin (HbA)
 Denoted by 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

8. CONT
 In sickle cell Hb, the two alpha chains are normal.
 The effect of mutation resides only in the Beta
chain where amino acid glutamic is replaced by
hydrophobic amino acid valine

9. Shapes of normal RBC and a sickled RBC

10. DEFINITION
 It is a severe, chronic, hereditary haemolytic
disorder due to the homozygous presence of
haemoglobin S, usually characterized by pallor
and recurrent crises.
 Is heredity blood disorder of the RBC that
assumes an abnormal rigid sickle shape
characterized by s/s of anaemia and recurrent
crises

11. Types of Sickle Cell Disorder
 There are basically three, i.e.
Sickle cell disease (sickle cell Anaemia): There is
autosomal recessive (homozygous) inheritance (Hb
SS). It is symptomatic.
Sickle cell trait: There is heterozygous inheritance
of Hb S from one parent (Hb AS). It is
asymptomatic.
Sickle cell syndromes: Associated with presence
of Hb S (Hb SC sickle cell Hb C)
HBSD (Sickle cell Hb D)
NOTE: Concentrate on Sickle cell Disease and
sickle cell trait)

12. Genetic transmission
 If one parent has sickle cell trait (HbAS) and
the other does not carry the sickle
hemoglobin at all (HbAA) then none of the
children will have sickle cell anemia.
 There is a one in two (50%) chance that any
given child will get one copy of the HbAS gene
and therefore have the sickle cell trait.
 It is equally likely that any given child will get
two HbAA genes and be completely
unaffected.

13. Genetic transmission

14. CONT
 If both parents have sickle cell trait (HbAS)
there is a one in four (25%) chance that any
given child could be born with sickle cell
anemia.
 There is also a one in four chance that any
given child could be completely unaffected.
 There is a one in two (50%) chance that any
given child will get the sickle cell trait.

15. GENETIC TRANSMISSION

16.  If one parent has sickle cell trait (HbAS) and
the other has sickle cell anaemia (HbSS)
there is a one in two (50%) chance that any
given child will get sickle cell trait and a one in
two (50%) chance that any given child will get
sickle cell anemia.
 No children will be completely unaffected.

17. GENETIC TRANSMISSION

18. CONT
 If one parent has sickle cell anaemia (HbSS)
and the other is completely unaffected
(HbAA) then all the children will have sickle
cell trait.
 None will have sickle cell anemia.
 The parent who has sickle cell anemia (HbSS)
can only pass the sickle hemoglobin gene to
each of their children.

19. GENETIC TRANSMISSION

20. PATHOPHYSIOLOGY
 The Hb A consists of 4 molecules of haem folded
in 1 molecule of globulin.
 Each globulin molecule consist of 2 alpha and 2
beta chain
 The amino acid glutamine is replaced by the
amino acid valine changing the properties of the
normal Hb .
 The absence of glutamic acid changes the
structure of the Hb molecule. This includes loss of
elasticity, distorts the shape and easily polymerises

21. PATHOPHYSIOLOGY CONT
 When the HbS is subjected to low oxygen tension
the abnormal beta chain contracts and piles
together within the red blood cell. This also
occurs when the RBC releases their load of
oxygen
 This distorts the shape of the red blood cell.
 The cells assume a sickle shape, become rigid,
clump together and form masses of RBCs.
 The sickled cell produces a sticky patch on the
surface membrane when they are not compressed
with oxygen

22. CONT
 Other molecule of Hb with sickled cells also
develop the sticky patch and adhere to each other.
 This causes the cells polymerise into long fibres
inside the blood vessels.
 The sickled cell tend to get stuck in narrow blood
vessel blocking the flow of blood.

23. PATHOPHYSIOLOGY CONT
 This lead to Sickling of other RBCs with more
obstruction of blood vessels and ischemia of the
affected tissues.
 Repeated episodes of ischaemia leads to
progressive damage of tissues or organs from
infarction
 Usually the cell return their normal shape after the
low oxygen conditions are removed and proper
oxygenation occurs

24. PATHOPHYSIOLOGY CONT
• 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.

25. Red blood cells, multiple sickle cells

26. RED BLOOD CELLS, MULTIPLE SICKLE
CELLS

27. PATHOPHYSIOLOGY CONT
 The cell becomes more fragile and easily
haemolysed
 The life span reduces from 120 to less than 30
days.
 The sickle shaped cell increase the viscosity of
blood thereby increasing the chances of infarction
causing further sickling of cells.

28. PATHOPHYSIOLOGY CONT
 After recurrent episodes of sickling, membrane
damage occurs and the cells are no longer
capable of resuming the biconcave shape upon
reoxygenation.
 Thus, they become irreversibly sickled cells
(ISCs). From 5-50% of RBCs permanently
remain in the sickled shape.

29. PATHOPHYSIOLOGY CONT
 The reduced life span of the RBC causes
haemolytic anaemia
 The patient also experiences periodic episodes of
cellular sickling called crises, characterized by high
fever, general body pains,etc.
 Severity of symptoms varies from person to person

30. PATHOPHYSIOLOGY CONT
 Some people have mild symptoms while others
are frequently hospitalised for serious
complications
 Most manifestations are caused by the sickling of
the RBC
 Sickled RBC breaks down frequently and
prematurely leading to anaemia
 Anaemia causes shortness of breath, fatigue,
delayed growth and development in children

31. PATHOPHYSIOLOGY CONT
 Rapid breakdown of RBC in the spleen may
cause jaundice
 Painful episodes may be due to tissue
infarction caused by ischaemia as a result of
blocked blood vessels.

32. SIGNS AND SYMPTOMS OF SICKLE CELL
DISEASE
• Attacks of abdominal pain due to tissue ischaemia
• Bone pain due to ischaemia
• Breathlessness cardio pulmonary involvement and
anaemia
• 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
• Fever due the inflammatory reaction caused by
tissue infarction

33. SIGNS AND SYMPTOMS OF SICKLE CELL
DISEASE CONT
 Jaundice due to increased levels of bilirubin as a
result of increased haemolysis
 Pallor due low Hb
 Rapid heart rate as a compensatory mechanism to
hypoxia
 Susceptibility to infections due to low immunity
 Ulcers on the lower legs (in adolescents) due to
ischaemia

34. SIGNS AND SYMPTOMS OF SICKLE CELL
DISEASE CONT
• Bloody urine (hematuria) due to renal
damage following repeated tissue
infarction
• Chest pain due to pulmonary infarction or
cardiac ischaemia
• Restlessness especially during crises due
to pain

35. SIGNS AND SYMPTOMS OF SICKLE CELL
DISEASE CONT
Painful erection (priapism; this occurs in
10 - 40% ) due to blood being trapped by
occluded blood vessel
Poor eyesight/blindness due to impaired
blood supply to the retina
Cyanosis due to hypoxia

36. SICKLE CELL CRISIS
 Sickle cell disease results in Anaemia and crises
that could be of many types
Vaso-occlusive
Aplastic
Sequestration
Hemolytic
 Most episodes of sickle cell crisis last between 5
to 7 days

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

38. HAEMOLYTIC CRISIS
 This is an acute accelerated drop in the
haemoglobin level
 The HbS is very fragile and easily haemolysed.
 Severe haemolysis leads to low oxygen carrying
capacity
 This will lead to low oxygen tension there by
precipitating a crisis

39. THROMBO EMBOLITIC / VASO OCCLUSIVE
CRISIS
 Sickle shaped cells obstruct the capillaries and
restrict blood flow to distal organs resulting in
ischaemia, pain ,necrosis and often organ
damage
 Also changes is the structure of HbSS tends to
increase the viscosity of blood
 This causes obstruction of the blood vessel due
to thrombosis

40. THROMBO EMBOLITIC / VASO OCCLUSIVE
CRISIS
 This later leads to low oxygen tension in the
area distal to the point of occlusion
 There by causing a crisis
 This may lead to ulcer formation and stroke
 Frequency ,severity and duration of the crisis
vary considerably
 Vaso occlusive crisis involving the penis and
lungs are considered medical emmergency

41. SPLEENIC SEQUESTRATION CRISIS
 This is acute painful enlargement of the spleen
caused by intrasplenic trapping of the RBC
 Large amount of blood is trapped in the spleen
because of its narrow vessels and function in
clearing defective RBC
 This leads to a fall in Hb level resulting in a
potential development of hypovolaemic shock
 The crisis is transient, continue 3 to 4 hours and
may last for a day

42. SPLEENIC SEQUESTRATION CRISIS
 This cause circulatory collapse in general
circulation which may lead to low oxygen level
 Low oxygen level will further trigger a crisis

43. APLASTIC CRISIS
 Acute worsening of patient’s baseline anaemia
producing pallor, tachycardia and fatigue
 It is normally triggered by parvovirus B19
which directly affects erythropoesis by invading
the red cell precursor.
 The bone marrow is unable to produce enough
red blood cell
 The infection prevents production of RBC for 2
to 3 days

44. APLASTIC CRISIS
 In normal individuals, this is of little
consequence, but the shortened life span of a
sickle cell patient results in an abrupt life
threatening situation.
 There is rapid drop in RBC and the rapid
turnover of RBC leads to the drop in Hb

45. APLASTIC CRISIS
 The result is low oxygen tension due to
inadequate red blood cells
 This will enventually causes a crisis

46. DIAGNOSIS
 Full blood count will show low HB and RBC
count
 Sickling test will show Sickling of cell
 Haemoglobin electrophoresis will confirm the
diagnosis
 History may reveal sickle cell in the family
 The clinical feature of sickle cell may be present
e.g. anaemia, growth retardation

47. TREATMENT
 Antibiotics e.g. e.g. Crystapen Penicillin 1-2mu
qid for 5/7
 Oxygen therapy to relieve hypoxemia e.g.
5l/minute
 Narcotic analgesics like pethidine 0.5-2mg/kg for
3/7
 Folic acid 5-10 mg od for 14/7
 Blood transfusion with packed cell may be given

48. TREATMENT CONT
IV fluid with normal saline to relieve
dehydration
Hydroxurea used to reduce number of
episodes

49. COMPLICATIONS
 Opioid tolerance, which can occur as a normal,
physiologic response to the therapeutic use of
opiates leading to addiction
 Stroke, which can result from a progressive
narrowing of blood vessels, preventing oxygen
from reaching the brain.
 Chronic renal failure due to Sickle cell
nephropathy
 Acute papillary necrosis in the kidneys.

50. COMPLICATIONS
 Cholelithiasis (gallstones) and cholecystitis,
which may result from excessive bilirubin
production and precipitation due to prolonged
haemolysis.
 Priapism and infarction of the penis.
 Leg ulcers.
 In eyes, background retinopathy, proliferative
retinopathy, vitreous haemorrhages and retinal
detachments, resulting in blindness.

51. COMPLICATIONS
 Avascular necrosis (aseptic bone necrosis) of the
hip and other major joints, which may occur as a
result of ischaemia.
 Pulmonary hypertension (increased pressure on
the pulmonary artery), leading to strain on the
right ventricle and a risk of heart failure
 Decreased immune reactions due to
hyposplenism (malfunctioning of the spleen)

52. PREVENTION
 Identify what can trigger the “Crisis” such as
stress, avoid extremes of heat and cold weather,
don’t travel airplane that is not cabin pressurized
 Maintain healthy lifestyle habits
Eating healthy
Avoid dehydration
Exercise regularly
Get enough sleep and rest
Avoid alcohol and don’t smoke
 Regular medical checkups and treatment are
important

53. NURSING CARE
 AIMS:
To relieve pain
To prevent complications
To promote comfort
To relieve anxiety

54. ENVIRONMENT
 The patient will be nursed in a general ward,
however reverse barrier nursing will be used to
prevent nosocomial infection.
 Patient will be nursed at the acute bay for close
observation
 The room should be warm because the cold
weather can precipitate to a crises.
 The room should be well ventilated to promote air
circulation and prevent R.T.I.

55. ENVIRONMENT CONT
Oxygen apparatus will be made available
for use when in time of dyspnoea
The room should be well lit for easy
observation

56. PAIN RELIEF
Offer prescribed analgesics for pain relief
hence promote comfort and rest
I will do warm compresses on the painful
areas to relieve pain
I will provide a bed cradle to relieve the
weight of the linen their by prevent pain
due to pressure

57. POSITION
The patient will be propped up to
relieve dyspnoea.
As the condition improves I will
allow the patient to adopt any
position of comfort

58. PSYCHOLOGICAL SUPPORT
 I will explain the disease process in order to
raise the knowledge levels and thereby alley
anxiety
 I will encourage the patient/care taker to ask
question and I will answer accordingly those I
cant answer I will refer to the physician
 I will explain all procedures to my patient/care
taker in order to allay anxiety

59. PSYCHOLOGICAL CARE CONT
 I will involve a successfully managed case to
come and talk to my patient in order to allow
the patient ask pressing question and get
answer this will improve the patients out look
on his condition
 I will explain to him that as the health care
team we are doing everything possible to
ensure that he get better in order to promote co-
operation.

60. PSYCHOLOGICAL SUPPORT CONT
 I will involve the loved ones in the plan of his
care in order for him not to feel neglected.
 I will provide diversional therapy in order to shift
the patient’s mind from the hospital routine and
his condition
 I will involve him in planning of his own care.

61. OBSERVATIONS
 I will do vital sign and BP to act as the base
line data in order to know if the condition is
improving or deteriorating
 I will observe for cyanosis if improving or
getting worse and give oxygen therapy when
necessary
 I will observe dyspnea if present will prop up
the patient to promote lung expansion and there
by relieve dyspnea

62. OBSERVATIONS CONT
 I will observe the patient’s facial expressions to
detect pain and administer prescribed
analgesics like Panadol
 I will observe the feeding pattern of my patient
and take measures like giving small frequent
meals
 I will observe the respirations to detect
dyspnoea and report accordingly

63. OBSERVATIONS CONT
 I will observe the pressure areas to detect on
set of pressure sore development
 I will observe the IV fluids to prevent fluid
over load
 If on Blood Transfusion I will observe the
transfusion to detect transfusion reaction such
as high temperature.

64. ELIMINATION
 I will provide a lot of fluids and roughage to
prevent constipation
 I will give more fluids in order to promote
renal wash out and there by prevent renal
complications
 I will offer a bed pan if he is confined to bed to
ensure bowel movement

65. REST AND ACTIVITY
 The patient will be on bed rest in in the acute
phase in order to reduce the demand for oxygen.
 During a crisis the patient will be on complete
bed rest in order to promote recovery.
 I will plan my nursing care in such a way that
periods of rest are allowed in order to conserve
patients energy.
 I will provide a quiet environment to promote
rest.

66. REST AND ACTIVITY CONT
 I will nurse the patient in a quiet room to
promote rest
 I will do related procedures in blocks of nursing
care to avoid disturbing the patient hence
promote rest
 I will ensure that squeaking trolleys are oiled to
prevent noise and there by promote rest

67. NUTRITION
 I will provide energy giving foods to provide
the energy needed for the metabolic processes
 I will provide protein foods like fish and beans
to promote replacement of worn out tissues
 Vegetables and fruits will be provided to raise
the immunity and promote skin and mucous
membrane

68. NUTRITION CONT
 I will provide a lot of oral fluids to prevent
dehydration due to excessive sweating
 I will serve small frequent meals to promote
appetite
 I will provide Iron rich food such as liver,
meat, green leafy vegetables to promote blood
formation

69. NUTRITION CONT
 I will allow care taker to prepare food preferred
by the patient in order to promote appetite
 I will do regular mouth washes in order to
promote appetite

70. HYGIENE
 I will encourage the patient to take baths in
order to remove dead epithelium and promote
comfort
 I will do hair care to promote self esteem and
also prevent pediculosis
 I will do nail care to prevent auto infection and
bruising self which can lead to bleeding

71. HYGIENE CONT
 I will do mouth care to improve appetite
 Any soiled linen and clothes will be changed to
promote comfort

72. MEDICATION
 I will administer prescribed analgesic like
pethidine or morphine at the right time to
promote rest
 I will ensure that the drugs are swallowed in
my presence to promote recovery.
 I will the patient/caretaker on the side effects of
the drugs
 I will sign the treatment chart upon
administering the drug

73. MEDICATION CONT
 I will ensure that I sign for the drug to prevent
over dosing the patient
 I will give the drug at the right time and
frequency to ensure required plasma level are
maintained thereby promoting recovery

74. HEALTH EDUCATION
 I will educate the patient about his condition in
order to create awareness and prevent
recurrence of the condition
 I will educate the patient about the
predisposing factors in order to prevent crises
 I will talk to the patient about the need to take a
balanced diet using locally available foods in
order to boost the immunity and blood
formation.

75. HEALTH EDUCATION CONT
 I will educate the patient about the need keep the
review dates so that his progress is monitored to
ensure full recovery
 I will advise the patient to ensure that he is
dewormed at least twice a year
 I will advise my patient to have malaria promptly
treated to avoid haemolysis which can lead to
anaemia
 I will explain the need for taking the medication
in order to promote compliance and recovery.

76. THE END
THANK
YOU!
76