Sickle cell disease is a hereditary condition characterized by abnormal hemoglobin, primarily affecting individuals of African, Mediterranean, and Arab descent. It manifests in several forms, with sickle cell anemia being the most severe, leading to painful crises and various complications. Diagnosis involves laboratory tests like CBC and hemoglobin electrophoresis, while treatment focuses on pain management, hydration, and addressing infections.

1. SICKLE CELL
BY
AMEYAW COLLINS

2. SICKLE CELL DISEASE
• Definition: Sickle cell disease is associated
with an inherited defect in the synthesis of
hemoglobin. It is the name of a group of
disorders of red blood cells in which the
predominant haemoglobin in the red blood
cell is haemoglobins, also known as sickle cell
haemoglobin.

3. Incidence
• The disease is found predominately in Africans
and Black Americans. It also occurs in people
from Mediterranean and Arab countries. It
affects both males and females.
Causes
• It is a genetic condition caused by the transfer
of haemoglobin S gene from parents to their
offspring

4. Forms of sickle cell disease
• SS (Sickle Cell Anemia)
• SC (Sickle-Hemoglobin C Disease)
• S beta-plus thalassemia
• S beta-zero thalassemia

5. SICKLE CELL TRAIT
• A trait is a common word for a condition where a person
gets an abnormal gene from one parent and the normal
type from the other parent. If he gets the abnormal
genes from both parents he is said to have the disease.
• Sickle cell trait is described as a heterozygous form
of the disease. Here, the individual inherits one
haemoglobin C or S from one parent. Such persons
seldom experience sickle cell crises and have life
expectancy and their frequency of hospitalization do not
differ from those with normal haemoglobin A.

6. SICKLE CELL ANAEMIA
• Sickle anaemia is a severe haemolytic anaemia
that results from inheritance of the sickle
haemoglobin S gene from both parents. The
gene causes the haemoglobin molecule to be
defective.
• That is, it causes the red blood cell to assume
crescent of sickle shape. These red bloods impair
circulation resulting in chronic ill health period
cries, long term complication and premature death.

7. Types of Sickle Cell Disease
• There are two types of the disease
• The severe type and
• Asymptomatic condition.

8. SEVERE TYPE
• In the severe type there is a persistent hemolytic anemia
with periodic episodes of painful crises. This appears in
homozygous form (inherited from both parents).
ASYMPTOMATIC TYPE
• This is when the defect is heterozygous (inherited from
only one parent), thus having a sickle cell trait.
• This is much more frequent among Negroes in the United
States than is the true disease. If both parents are
heterozygous, one of their children will have sickle cell
disease, on the average, two will have the trait, and one
will be hematologically normal.

9. • Pathologically, hemoglobin in both forms is
abnormal (hemoglobin S). Under reduced oxygen
tension this hemoglobin is responsible for changing
red blood cell into a sickled shape.
• In sickle cell disease these malformed cells clump
together and obstruct capillaries, thereby causing
what is known as a crisis in the disease. Capillary
obstruction leads to anoxic changes which cause
further sickling and thus still further obstruction in
the blood vessels resulting in pain in the area.

10. FACTORS THAT PRECIPITATE SICKLE CELL CRISIS
• Exposure to extreme of temperature either cold or
hot
• High altitude
• Stress
• Dehydration
• Malaria
• Extremes of emotion either over excitement or
depression
• Anxiety

11. CLINICAL MANIFESTATIONS
• In children with sickle cell disease the onset
may be as early as the second or third month
of life, when normal fetal hemoglobin is
replaced by the abnormal hemoglobin
which causes sickling.
• Approximately half of all children affected by
this condition have symptoms by one year of
age.

12. CLINICAL MANIFESTATION CONT
• The first clinical manifestation the infant
may have is sickle cell crisis.
• The child may also have an extreme pallor
• fever
• vomiting
• severe flank pain
• with hematuria

13. • convulsions
• stiff neck
• coma or paralysis
• Two to three minutes after the crisis, Jaundice
may appear. Between crises the
manifestations are
• pallor
• anemia

14. LABORATORY FINDINGS AND DIAGNOSES
• CBC: Reticulocytosis (count may vary from 30%–50%);
leukocytosis (especially in vaso-occlusive crisis), with counts
over 20,000 indicate infection, decreased Hb (5–10 g/dL) and
total RBCs, elevated platelets, and a normal to elevated MCV.
• Stained RBC examination: Demonstrates partially or
completely sickled, crescent-shaped cells; anisocytosis;
poikilocytosis; polychromasia; target cells; Howell-Jolly bodies;
basophilic stippling; occasional nucleated RBCs (normoblasts).
• Sickle-turbidity tube test (Sickledex): Routine screening test
that determines the presence of hemoglobin S (HbS) but does
not differentiate between sickle cell anemia and trait.

15. DIAGNOSIS CONT
• Hemoglobin electrophoresis: Identifies any abnormal
hemoglobin types and differentiates between sickle cell trait and
sickle cell anemia. Results may be inaccurate if patient has
received a blood transfusion within 3–4 mo before testing.
• ESR: Elevated.
• Erythrocyte fragility: Decreased (osmotic fragility or RBC
fragility); RBC survival time decreased (accelerated breakdown).
• ABGs: May reflect decreased Po2 (defects in gas exchange at the
alveolar capillary level); acidosis (hypoxemia and acidic states in
vaso-occlusive crisis).
• Serum bilirubin (total and indirect): Elevated (increased RBC
hemolysis).

16. TREATMENT
• Treatment during crises includes codeine and aspirin for
relive of pain and maintenance of hydration.
• Antibiotic therapy may be necessary if a bacteria
infection such as pneumonia or meningitis is present
such as penicillin when they're about 2 months of age
and continue taking it until they're at least 5 years old.
• Transfusion of erythrocytes may be required
• Oxygen is given if the hemoglobin level falls rapidly to as
low as 4gm. Per100ml. and the child has symptoms of
hypoxia

17. TREATMENT CONT
• Recently research has shown that the
administration of urea dissolve in a sugar
solution relieved acute sickle cell crises by
dissipating the electrochemical bounds that hold
the hemoglobin sickle cell in rod – shaped
aggregations.
• Investigation is now being done to determine
whether these crises can be prevented by given urea
orally in a sweat soft drink daily.

18. TREATMENT CONT
• Folic acid is administered daily for increased
marrow requirement.
• Supportive care involves pain management
(aspirin or NSAIDs, morphine, and patient
controlled analgesia), oral or IV hydration,
physical and occupational therapy,
physiotherapy, cognitive and behavioral
intervention, and support groups.

19. NURSING CARE
• Assisting the physician with the treatments mentioned such as
checking of vitals especially assessing the level of pain and
administering prescribed analgesics
• Helping other members of the health team to educate the parent
and the child about the disease.
• The clinical manifestations of the disease can be explained to
the parent so that they know when to bring the child to the
hospital and when it is necessary only to call the physician for
information.
• The parents need to know that the child should consume huge
quantities of liquids, especially in harmattan, and should also
have well balanced meals.

20. • They also need to know that the child should
be treated immediately when infections
occurs.
• Enhance tissue perfusion.
• Provide nutritional and fluid needs.
• Other children in the family should be tested
to find those who may similarity afflicted.

21. Complications
• Congestive heart failure
• Leg ulcers
• Renal failure
• Periodic bone pain
• Anaemia
• Splenomegaly
• Hepatomegaly
• Opoiod tolerance
• Osteomyelitis
• Priapism and infection of the penis
• Stroke
• Decreased immune reactions