Sickle cell anemia is an inherited blood disorder where red blood cells become rigid and sticky and are shaped like sickles or crescents. This causes them to get stuck in small blood vessels and block blood flow, leading to pain crises. It is caused by a genetic mutation that results in abnormal hemoglobin called hemoglobin S. Symptoms include anemia, pain crises, swelling in hands and feet, and increased risk of infections. Complications can include stroke, organ damage, pulmonary hypertension, and blindness if untreated. Treatment focuses on pain management, antibiotics to prevent infection, hydroxyurea to reduce crises, blood transfusions, and potentially a bone marrow transplant for cure. Nursing care centers on managing pain

1. SICKLE CELLANAEMIA

2. APLASTIC ANEMIA
DEFINITION
• This very rare life-threatening anemia is caused
by bone marrow hypoplasia or aplasia resulting
in pancytopenia (insufficient numbers of RBCs,
WBCs and platelets).

3. causes
Many diseases, conditions, and factors can cause aplastic
anemia, including:
Toxins, such as pesticides, arsenic, and benzene.
Radiation and chemotherapy (treatments for cancer).
Medicines, such as chloramphenicol (an antibiotic
rarely used ).
Infectious diseases, such as hepatitis, Epstein-Barr
virus, cytomegalovirus, parvovirus B19, and HIV.
Autoimmune disorders, such as lupus and
rheumatoid arthritis.
Pregnancy. (Aplastic anemia that occurs during
pregnancy often goes away after delivery.)

4. Clinical features
 Anemia with malaise, pallor and associated
symptoms such as
 palpitations
 Thrombocytopenia (low platelet counts), leading
to increased risk of hemorrhage,bruising and
petechiae
 Leukopenia (low white blood cell count), leading
to increased risk of infection
 Reticulocytopenia (low counts of reticulocytes,
that is, immature red bloodcells)

5. DIAGNOSTICEVALUATION
CBC and peripheral blood smear show
decreased RBC, WBC, platelets
(pancytopenia)
Bone marrow aspiration and biopsy: bone
marrow is hypocellular or empty with greatly
reduced or absent hematopoiesis

6. Management
1. Remove of causative agent or toxin.
2. Allergenic bone marrow transplantation
(BMT)
3. Immunosuppressive treatment
4. Androgens (oxymetholone or testosterone
enanthate) may stimulate bone marrow
regeneration.
5. Supportive treatment includes platelet and
RBC transfusions, antibiotics, and antifungals.

7. COMPLICATIONS
Untreated severe aplastic anemia is almost
always fatal, generally because of
overwhelming infection.
Even with treatment, morbidity and mortality
caused by infections and bleeding are high.
• Late complications, even after successful
treatment, include clonal hematologic diseases
such as paroxysmal nocturnal hemoglobinuria,
myelodysplasia, andmacute myelogenous
leukemia

8. NURSING
MANAGEMENT

9. 3.MEGALOBLASTIC ANEMIA:
PERNICIOUS
A megaloblast is a large, nucleated
erythrocyte with delayed and
abnormal nuclear maturation.
Pernicious anemia is a type of
megaloblastic anemia
associated with vitamin B12
deficiency because of lack of
intrinsic factor in gastric secretion

10. PERNICIOUS ANEMIA
Disease in which the production of red blood
cells (erythrocytes) is impaired as the result
of the body’s inability to absorb vitamin B12,
which is necessary for red blood cells to
mature properly in the bone marrow.
Pernicious anemia is one of many types of
anemia, a disease marked by a reduction in
red blood cells or in the oxygen-carrying
substance hemoglobin found in those cells.

11. PATHOPHYSIOLOGY AND
ETIOLOGY
Vitamin B12 is necessary for normal deoxyribonucleic
acid synthesis in maturing RBCs.
Pernicious anemia demonstrates familial incidence related to
autoimmune gastric mucosal atrophy.
Normal gastric mucosa secretes a substance called intrinsic
factor, necessary for absorption of vitamin B12 in ileum. If a
defect exists in gastric mucosa, or after gastrectomy or
small bowel disease, intrinsic factor may not be secreted
and orally ingested B12 not absorbed.
Some drugs interfere with B12 absorption, notably ascorbic
acid, cholestyramine, colchicine, neomycin, cimetidine, and
hormonal contraceptives.
Primarily a disorder of older people.

12. MANIFESTATIONS
Pallor, fatigue, dyspnea on exertion, palpitations.
May be angina pectoris and heart failure in the
elderly or those predisposed to heart disease.
GI dysfunction: sore mouth, glossitis, anorexia,
nausea, vomiting, and loss of weight, indigestion,
epigastric discomfort, recurring diarrhea or
constipation.
Neuropathy (occurs in high percentage of untreated
patients): paresthesia that involves hands and feet,
gait disturbance, bladder and bowel dysfunction,
psychiatric symptoms caused by cerebral
dysfunction

13. DIAGNOSTIC EVALUATION
A diagnosis of PA requires several different tests.
It requires a:-
• complete blood count (CBC) test
• vitamin B-12 deficiency test- Schilling test for absorption of
vitamin B12 uses small amount of radioactive B12 orally and
24-hour urine collection to measure uptake—decreased
• IF deficiency test
• Proof of stomach destruction
CBC measures the amount of:
• Hemoglobin - protein bound to oxygen to carry it
throughout the blood
• Hematocrit - used to measure how much space red blood
cells use within the blood
• .

14. • MANAGEMENT
• Parenteral replacement with
hydroxocobalamin or cyanocobalamin (B12) is necessary by
I.M. injection from health care provider, generally every
month.
• COMPLICATIONS
• Neurologic: paresthesia, gait disturbances, bowel and
bladder dysfunction, and cerebral dysfunction may be
persistent.
• Nursing Assessment
• Assess for pallor, tachycardia, dyspnea on exertion, exercise
intolerance to determine patient's response to anemia.
• Assess for paresthesia, gait disturbances, changes in
bladder or bowel function, altered thought processes
indicating neurologic involvement.
• Obtain history of gastric surgery or GI disease

15. • Nursing Diagnoses
• Disturbed Thought Processes related to neurologic
dysfunction in absence of vitamin B12
• Impaired Sensory Perception (kinesthetic) related to
neurologic dysfunction in absence of vitamin B12.
• Nursing Interventions
• Improving Thought Processes
• Administer parenteral vitamin B12 as prescribed.
• Provide patient with quiet, supportive environment;
reorient to time, place, and person if needed; give
instructions and information in short, simple sentences
and reinforce frequently.

16. • Minimizing the Effects of Paresthesia
• Assess extent and severity of paresthesia,
imbalance, or other sensory alterations.
• Refer patient for physical therapy and
occupational therapy as appropriate.
• Provide safe, uncluttered environment; make
sure personal belongings are within reach;
provide assistance with activities as needed

17. Sickle cell anemia
Sickle cell anemia is an inherited form of anemia
.Sickle cell anemia is a severe, chronic . Incurable haemolytic
aneamia resulting from inherited defective haemoglobin
molecule (sickle haemoglobin gene Hbs) and marked by
episodic painful crisis.
— In this condition there aren't enough healthy red blood
cells to carry adequate oxygen throughout your body
Normally, red blood cells are flexible and round, moving
easily through blood vessels. In sickle cell anemia, the red
blood cells become rigid and sticky and are shaped like
sickles or crescent moons. These irregularly shaped cells
can get stuck in small blood vessels, which can slow or
block blood flow and oxygen to parts of the body.

18. • Sickle cell crisis –occurs when the client
experience decreased oxygen resulting in the
enlargement of rigid sickle shaped cells with
one another causing increased blood viscosity
this result in blood stasis with enlargement
and engorgement of organ, infraction with
ischaemia and destruction of RBCs and
replacement of fibrous tissue.

19. Sickle Cell disease: is a genetic disorder that
affects erythrocytes (RBC) causing them to
become sickle or crescent shaped.
people with sickle cell disease have blood cells
that contain mostly haemoglobin S an
abnormal type of haemoglobin.. Sometime
these blood red blood cells become more sickle
shape(cresent shape)mand have difficulty
passing through small blood cellls.

21. • Hemoglobin
Each hemoglobin molecule is made up of four heme
groups surrounding a globin group.
Heme contains iron and gives a red color to the
molecule.
Globin consists of two linked pairs of polypeptide chains

23. Mechanism
When sickle hemoglobin (HbS) gives up its
oxygen to the tissues, HbS sticks together
Forms long rods form inside RBC
RBC become rigid, inflexible, and sickle-shaped
Unable to squeeze through small blood vessels,
instead blocks small blood vessels
Less oxygen to tissues of body
RBCs containing HbS have a shorter lifespan
Normally 20 days
Chronic state of anemia

24. CAUSES OF SICKLE CELL
ANEMIA
a mutation in the gene that tells body to make hemoglobin —In
sickle cell anemia, the abnormal hemoglobin causes red blood
cells to become rigid, sticky and misshapen.
AUTOSOMAL RECESSIVE INHERITANCE. -The sickle cell gene is passed from
generation to generation in a pattern of inheritance called autosomal
recessive inheritance. This means that both the mother and the father
must pass on the defective form of the gene for a child to be affected.
If only one parent passes the sickle cell gene to the child, that
child will have the sickle cell trait. With one normal hemoglobin
gene and one defective form of the gene, people with the sickle
cell trait make both normal hemoglobin and sickle cell
hemoglobin. Their blood may contain some sickle cells, but they
generally don't experience symptoms. However, they are
carriers of the disease, which means they can pass the defective
gene on to their children.

28. • Symptoms
They vary from person to person and change over
time, include:
Anemia. Sickle cells break apart easily and die,
leaving you without enough red blood cells. Red
blood cells usually live for about 120 days before
they need to be replaced. But Sickle cells usually
die in 10 to 20 days, leaving a shortage of red
blood cells (anemia). Without enough red blood
cells, your body can't get the oxygen it needs to
feel energized, causing fatigue.

29. Episodes of pain. Periodic episodes of pain, called crises,
are a major symptom of sickle cell anemia.
Pain develops when sickle-shaped red blood cells block blood flow through
tiny blood vessels to your chest, abdomen and joints. Pain can also occur in
bones.
The pain varies in intensity and can last for a few hours to a few weeks. Some
people have only a few pain episodes.
Painful swelling of hands and feet. The swelling is caused by sickle-shaped red
blood cells blocking blood flow to the hands and feet.

30. Frequent infections. Sickle cells can damage an
organ that fights infection (spleen), leaving more
vulnerable to infections.
Delayed growth. Red blood cells provide body with
the oxygen and nutrients you need for growth. A
shortage of healthy red blood cells can slow
growth in infants and children and delay puberty
in teenagers.
Vision problems. Tiny blood vessels that supply
eyes may become plugged with sickle cells. This
can damage the retina
— the portion of the eye that processes visual
images, leading to vision problems.

31. Complications
Sickle cell anemia can lead to a host of complications, including:
Stroke. A stroke can occur if sickle cells block blood flow to an area of
brain. Signs of stroke include seizures, weakness or numbness of
arms and legs, sudden speech difficulties, and loss of
consciousness.
Acute chest syndrome. This life-threatening complication causes
chest pain, fever and difficulty breathing. Acute chest syndrome can
be caused by a lung infection or by sickle cells blocking blood vessels
in lungs.
It might require emergency medical treatment with antibiotics and other
treatments.
Pulmonary hypertension. People with sickle cell anemia can develop
high blood pressure in their lungs (pulmonary hypertension). This
complication usually affects adults rather than children. Shortness of
breath and fatigue are common symptoms of this condition, which can
be fatal.

32. Organ damage. Sickle cells that block blood flow through blood
vessels immediately deprive the affected organ of blood and
oxygen. In sickle cell anemia, blood is also chronically low on
oxygen. Chronic deprivation of oxygen-rich blood can damage
nerves and organs in your body, including
kidneys, liver and spleen. Organ damage can be fatal.
Blindness. Sickle cells can block tiny blood vessels that Supply eyes.
Over time, this can damage the portion of the eye that processes
visual images (retina) and lead to blindness.
Leg ulcers. Sickle cell anemia can cause open sores, called
ulcers, on legs.
Gallstones. The breakdown of red blood cells produces a substance
called bilirubin. A high level of bilirubin inbody can lead to
gallstones.

33. Diagnosis
A blood test can check for hemoglobin S — the defective
form of hemoglobin that underlies sickle cell anemia.
Genetic counseling
Two tests can be used to help expectant parents find out if
their child is affected.
1. Amniocentesis, done usually at 14-16 weeks of pregnancy,
tests a sample of the amniotic fluid in the womb for genetic
defects (the fluid and the fetus have the same DNA). Under
local anesthesia, a thin needle is inserted through the
woman's abdomen and into the womb. About 20 milliliters
of fluid (roughly 4 teaspoons) is withdrawn and sent to a lab
for evaluation. Test results often take 1-2 weeks.

34. 2. Chorionic villus sampling, or CVS, involves
the removal and testing of a very small sample
of the placenta during early pregnancy. The
sample, which contains the same DNA as the
fetus, is removed by catheter or a fine needle
inserted through the cervix or by a fine needle
inserted through the abdomen. The tissue is
tested for genetic changes identified in an
affected family member. Results are usually
available within 2 weeks.

35. • Treatment
• Treatment is usually aimed at avoiding
crises, relieving
• symptoms and preventing complications.

36. Antibiotics. Children with sickle cell anemia
may begin taking the antibiotic penicillin when
they're about 2 months old and continue
taking it until they're at least 5 years old.
Doing so helps prevent infections, such as
pneumonia, which can be lifethreatening
to an infant or child with sickle cell anemia.

37. • Pain-relieving medications. To relieve pain
during a sickle cell crisis.
Hydroxyurea (Droxia, Hydrea). When
taken daily, hydroxyurea reduces the
frequency of painful crises and might reduce
the need for blood transfusions and
hospitalizations.
Hydroxyurea seems to work by stimulating

38. • Blood transfusions
In a red blood cell transfusion, red blood cells are removed
from a supply of donated blood, then given intravenously to
a person with sickle cell anemia.
Blood transfusions increase the number of normal red blood
cells in circulation, helping to relieve anemia. In children with
sickle cell anemia at high risk of stroke, regular blood
transfusions can decrease the risk. Transfusions can also be
used to treat other complications of sickle cell anemia, or
they can be given to prevent complications.

39. Nitric oxide. People with
sickle cell anemia have low
levels of nitric oxide in their
blood.
Nitric oxide is a gas that helps
keep blood vessels open and
reduces the stickiness of red
blood cells. Treatment with
inhaled nitric oxide might
prevent sickle cells from
clumping together

40. Bone marrow transplant, also known as stem
cell transplant, offers the only potential
cure for sickle cell anemia. It's usually
reserved for people younger than age 16
because the risks increase for people older
than 16. Finding a donor is difficult, and
the procedure has serious risks associated
with it, including death.
A bone marrow transplant involves
replacing bone marrow affected by sickle
cell anemia with healthy bone marrow
from a donor. The procedure usually uses a
matched donor, such as a sibling, who
doesn't have sickle cell anemia. For many,
donors aren't available. But stem cells
from umbilical cord blood might be an
option.

41. Gene therapy. Researchers are
exploring whether inserting a normal gene into
the bone marrow of people with sickle cell
anemia will result in normal hemoglobin

42. • NURSING MANAGEMENT
• NURSING DIAGNOSIS
• Acute pain related to tissue hypoxia due
• to agglutination of sickle cell with in
• blood vessels
• Risk for infection
• INTERVENTIONS……
• Managing pain
• Prevention and managing infection
• Promoting coping skills
• Minimizing Deficient knowledge
• Promoting home and community basedcare

43. NURSING MANAGEMENT OF ANEMIA
1. Nursing diagnosis - Imbalanced nutrition less then
body requirement related to inadequate intake of
essential nutrients as evidenced by skin integrity,
color and body weight.
• Goals: Improve nutrition level
• Intervention:
• A healthy diet should be encouraged.
• Avoid alcoholic beverages.
• Dietary teaching session should be individualized
including culture aspect related to food preference
and food preparation.

44. 2. Nursing diagnosis – activity intolerance related
to low level of Hb in body as evidenced by the
weakness, fatigue and malaise.
• Goal: Improve the activity intolerance
• Intervention:
• assist the patient to prioritize the Activities and a
establish balance between the activity and rest
that is realistic and feasible from the patient
perspectives.
• Patient with chronic anemia need to maintain
some physical activity and exercise to prevent
the deconditioning that results from the
inactivity.

45. 3. Nursing diagnosis – ineffective tissue perfusion
related to less blood volume as evidenced by skin
color (pallor).
• Goal: Improve tissue perfusion.
• Intervention:
• The nurse monitor the vital sign closely.
• lost volume replaced with blood transfusion or IV
fluids.
• Supplemental oxygen may be necessary but it is
rarely needed on a long term basis.
• Other medication such as antihypertensive agent
may be needed to be adjusted