1. SEMINAR ON NURSING MANAGEMENT OF SICKLE
CELL
ANEMIA,POLYCYTHEMIA,THROMBOCYTOPENI
A AND HEMOPHILIA
Presented by,
Umadevi.k
The oxford college of nursing
bengaluru

2. SICKLE CELL ANEMIA

3. 
Sickle-cell disease (SCD), or sickle-cell
anaemia (SCA) or drepanocytosis, is an
autosomal recessive genetic blood disorder
with overdominance, characterized by red
blood cells that assume an abnormal, rigid,
sickle shape. Sickle cell anemia is caused by
an abnormal type of hemoglobin called
hemoglobin S. Hemoglobin is a protein
inside red blood cells that carries oxygen.

6. 
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Sickle cell disease is prevalent in many parts
of India, where the prevalence has ranged
from 9.4 to 22.2% in endemic areas.
Sickle cell anemia affects millions throughout
the world.
It is particularly common among people
whose ancestors come from sub-Saharan
Africa; Spanish-speaking regions (South
America, Cuba, Central America); Saudi
Arabia; India; and Mediterranean countries
such as Turkey, Greece, and Italy.

7. 
Sickle cell anemia is inherited disease caused
by an abnormal type of hemoglobin called
hemoglobin S.

8. 
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A point mutation in the β-globin chain of
haemoglobin
Amino acid glutamic acid replaced with the
hydrophobic amino acid valine at the sixth
position.
The β-globin gene is found on the short arm
of chromosome .The association of two wildtype α-globin subunits with two mutant βglobin subunits forms haemoglobin S (HbS).

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Under low-oxygen conditions (being at high altitude,
for example), the absence of a polar amino acid at
position six of the β-globin chain promotes the noncovalent polymerisation (aggregation) of
haemoglobin,
Distortion of red blood cells into a sickle shape
Repeated episodes of sickling damage the cell
membrane
Decrease the cell's elasticity.
Rigid blood cells are unable to deform as they pass
through narrow capillaries
Vessel occlusion and ischaemia.

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Symptoms usually do not occur until after age
4 months.
Almost all patients with sickle cell anemia
have painful episodes (called crises), which
can last from hours to days. These crises can
cause pain in the bones of the back, the long
bones, and the chest.
Younger children with sickle cell anemia
have attacks of abdominal pain.

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When the anemia becomes more severe,
symptoms may include:
Fatigue
Paleness
Rapid heart rate
Shortness of breath
Yellowing of the eyes and skin (jaundice)

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The following symptoms may occur because
small blood vessels may become blocked by
the abnormal cells:
Painful and prolonged erection (priapism)
Poor eyesight or blindness
Problems with thinking or confusion caused
by small strokes
Ulcers on the lower legs (in adolescents and
adults)

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Over time, the spleen no longer works. As a
result, people with sickle cell anemia may
have symptoms of infections such as:
Bone infection (osteomyelitis)
Gallbladder infection (cholecystitis)
Lung infection (pneumonia)
Urinary tract infection

14. 
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Delayed growth and puberty
Painful joints caused by arthritis

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Tests commonly performed to diagnose and
monitor patients with sickle cell anemia
include:
Bilirubin
Blood oxygen
Complete blood count (CBC)
Hemoglobin electrophoresis
Serum creatinine
Serum potassium
Sickle cell test

16. 
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Treatment for a sickle cell crisis includes:
Blood transfusions (may also be given
regularly to prevent stroke)
Pain medicines
Plenty of fluids

17. 
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Other treatments for sickle cell anemia may
include:
Hydroxyurea (Hydrea), a medicine that may help
reduce the number of pain episodes (including
chest pain and difficulty breathing) in some
people
Antibiotics to prevent bacterial infections, which
are common in children with sickle cell disease
Bone marrow or stem cell transplants can cure
sickle cell anemia.

18. 
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Treatments that may be needed to manage
complications of sickle cell anemia include:
Dialysis or kidney transplant for kidney disease
Counseling for psychological complications
Gallbladder removal in people with gallstone
disease
Hip replacementfor avascular necrosis of the hip
Surgery for eye problems
Treatment for overuse or abuse of narcotic pain
medicines
Wound care for leg ulcers

19. 
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People with sickle cell disease must reduce
their risk of infections. This includes
receiving certain vaccinations, including:
Haemophilus influenzae vaccine(Hib)
Pneumococcal conjugate vaccine(PCV)
Pneumococcal polysaccharide vaccine(PPV)

20. 
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Acute pain related to tissue hypoxia due to
agglutination of sickle cells with in blood
vessels
Risk for infection related to
immunocompromised state
Risk for powerlessness related to illness
induced helplessness
Deficient knowledge regarding sickle crisis
prevention

21. 
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Managing pain
Preventing and managing infection
Promoting coping skills
Minimizing deficient knowledge
Monitoring and managing potential
complications

22. POLYCYTHEMIA

23. 
Polycythemia(also known as polyglobulia) is
a disease state in which the proportion of
blood volume that is occupied by red blood
cells increases.

24. 
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ABSOLUTE (The overproduction of red blood cells may
be due to a primary process in the bone marrow (a socalled myeloproliferative syndrome), or it may be a
reaction to chronically low oxygen levels or, rarely, a
malignancy)
PRIMARY
(Polycythemia vera (PCV), polycythemia rubra vera (PRV),
or erythremia, occurs when excess red blood cells are
produced as a result of an abnormality of the bone marrow
)
SECONDARY
Secondary polycythemia is caused by either natural or
artificial increases in the production of erythropoietin,
hence an increased production of erythrocytes

25. 
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ALTITUDE RELATED
Secondary polycythemia can be induced directly by
phlebotomy (blood letting) to withdraw some blood,
concentrate the erythrocytes, and return them to the
body.
HYPOXIC DISEASE-ASSOCIATED
Cyanotic heart disease where blood oxygen levels are
reduced significantly
POLYCYTHEMIA CAUSED BY ALTERED OXYGEN
SENSING
Inherited mutations in 3 genes which all result in
increased stability of Hypoxia Inducible Factors
(HIFs), leading to increased erythropoietin
production, have been shown to cause erythrocytosis:

26. 
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IATROGENIC
(phlebotomy)
GENETIC
(This includes patients who have a special form
of hemoglobin known as Hb Chesapeake)
RELATIVE
(Relative polycythemia is an apparent rise of the
erythrocyte level in the blood; however, the
underlying cause is reduced blood plasma)

27. 
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Polycythemia vera is a disorder of the bone
marrow. It mainly causes too much
production of red blood cells, although the
numbers of white blood cells and platelets
are also increased.
It is a rare disease that occurs more often in
men than women, and is rare in patients
under age 40. It is usually associated with a
gene mutation .The cause of this mutation is
unknown.

28. 
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Breathing difficulty when lying down
Dizziness
Excessive bleeding
Fullness in the left upper abdomen (due to
enlarged spleen)
Headache
Itchiness, especially after a warm bath
Red coloring, especially of the face
Shortness of breath
Symptoms of phlebitis

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Other symptoms that may occur with this
disease:
Bluish skin color
Fatigue
Red skin spots
Vision problems

30. 
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History
Physical exam
Tests that may be done include:
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Bone marrow biopsy
Complete blood count with differential
Comprehensive metabolic panel
Erythropoietin level
Genetic test for mutation
Oxygen saturation of the blood
Red blood cell mass
Vitamin B12 level

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This disease may also affect the results of the
following tests:
ESR
Lactate deydrogenase
Leukocyte alkaline phosphatase
Platelet aggregation test
Serum uric acid

32. 
A method called phlebotomy is used to
decrease blood thickness. One unit of blood
(about 1 pint) is removed weekly until the
hematocrit level is less than 45 (males) or 42
(females). Then therapy is continued as
needed.

33. 
Some patients are advised to take aspirin to
reduce the risk of blood clots, though it
increases the risk for stomach bleeding.
Ultraviolet-B light therapy can reduce the
severe itching some patients experience.

34. 
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Acute myelogenous leukemia (AML)
Bleeding from the stomach or other parts of
the intestinal tract
Gout
Heart failure
Myelofibrosis
Thrombosis (blood clotting, which can cause
a stroke, heart attack, or other body damage)

35. 
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Educator
Risk factors for thrombotic complications are
assessed
Patients with history of bleeding is advised to
avoid aspirin
Instruct for minimal alcohol intake
For pruritis advice for bath in tepid or cool
water along with applications of cocoa butter
based lotions and bath products

36. 
Occasionally, chemotherapy (specifically
hydroxyurea) may be given to reduce the
number of red blood cells made by the bone
marrow. Interferon may also be given to
lower blood counts. A medicine called
anagrelide may be given to lower platelet
counts

37. HEMOPHILIA

38. 
Haemophilia is a group of hereditary genetic
disorders that impair the body's ability to
control blood clotting or coagulation, which is
used to stop bleeding when a blood vessel is
broken.

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Haemophilia A (clotting factor VIII
deficiency) is the most common form of the
disorder, present in about 1 in 5,000–10,000
male births.
Haemophilia B (factor IX deficiency) occurs in
around 1 in about 20,000–34,000 male births.
Like
most
recessive
sex-linked,
X
chromosome disorders, haemophilia is more
likely to occur in males than females

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Haemophilia A is a recessive X-linked genetic
disorder involving a lack of functional clotting
Factor VIII and represents 80% of haemophilia
cases.
Haemophilia B is a recessive X-linked genetic
disorder involving a lack of functional clotting
Factor IX. It comprises approximately 20% of
haemophilia cases.
Haemophilia C is an autosomal genetic disorder
(i.e. not X-linked) involving a lack of functional
clotting Factor XI

42. 
Internal or external bleeding episodes, which are
called "bleeds".
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Patients with more severe haemophilia suffer more
severe and more frequent bleeds, while patients
with mild haemophilia usually suffer more minor
symptoms except after surgery or serious trauma.
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In both haemophilia A and B, there is spontaneous
bleeding but a normal bleeding time, normal
prothrombin time, normal thrombin time, but
prolonged partial thromboplastin time.

43. 
Internal bleeding(JOINT BLEED) is
common in people with severe haemophilia
and some individuals with moderate
haemophilia.
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If not treated promptly, joint bleeds can
lead to permanent joint damage and
disfigurement
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Heavy bleeding from a dental procedure, an
accident, or surgery.

44. 
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Deep internal bleeding, e.g. deep-muscle bleeding,
leading to swelling, numbness or pain of a limb.
Joint damage from Haemarthrosis(haemophilic
arthropathy),
potentially
with
severe
pain,
disfigurement, and even destruction of the joint and
development of debilitating arthritis.
Transfusion transmitted infection from
transfusions that are given as treatment.
blood
Intracranial haemorrhage is a serious medical
emergency caused by the buildup of pressure inside
the skull. It can cause disorientation, nausea, loss of
consciousness.

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Haemophilia A can be mimicked by von
Willebrand disease.
von Willebrand Disease could significantly
affect as many as 1 in 10,000 people.
von Willebrand Disease type 2A, where
decreased levels of von Willebrand Factor can
lead to premature proteolysis of Factor VIII.
In contrast to haemophilia, VWD type 2A is
inherited in an autosomal dominant fashion.

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von Willebrand Disease type 2N, where von
Willebrand Factor cannot bind Factor VIII,
autosomal recessive inheritance. (i.e.; both
parents need to give the child a copy of the
gene).
von Willebrand Disease type 3, where lack of
von Willebrand Factor causes premature
proteolysis of Factor VIII. In contrast to
haemophilia, vWD type 3 is inherited in an
autosomal recessive fashion.

47. 
Commercially produced factor concentrates
such as "Advate", a recombinant Factor VIII,
come as a white powder in a vial which must
be mixed with sterile water prior to
intravenous injection.

48. 
If a patient becomes refractory to replacement
coagulation factor as a result of circulating
inhibitors, this may be partially overcome
with recombinant human factor VII
(NovoSeven), which is registered for this
indication in many countries

49.  PROPHYLAXIS
OR ON-
DEMAND
 GENE THERAPY
 PREVENTIVE EXERCISES
 ALTERNATIVE MEDICINE

50. 
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Patients with hemophilia is instructed to avoid
any agents that interfere with platelet
aggregation such as
aspirin,NSAIDS,herbs,nutritional supplyments
and alcohol.
If pt had rescent surgery the nurse frequently
and carefully assess surgical site for bleeding
Frequent vital sign monitoring is needed until
the nurse is certain than there is no excessive
postoperative bleeding.

51. 
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Analgesics are administered to allevate pain
Advice for dental hygiene
All injections and invasive procedures should be
avoided
Patients with hemophilia should be encouraged
to carry or wear medical identification
During bleeding heat application should be
avoided
Apply pressure to control bleed if factor
deficiency is not much severe.

52. CONCLUSION

53.  THANK
UUUUUU……