Nursing Management for patient
with blood disease
Prepared By
Dr. Zuhair Rushdi Mustafa
Lecturer at University of Duhok/
College of Nursing
The blood composed of cellular component and plasma.
The cellular component of blood consists of 3 primary cell types:
(1) Erthrocytes (RBCs) which account
for 45% of the blood.
# The remaining 1% consists of
(2) white blood cells and (3) platelets.
# plasma (a yellowish liquid containing
nutrients, hormones, minerals)
The Plasma makes up 55% of the total blood volume.
COMPOSITION OF BLOOD
Properties of blood
• In a healthy person, blood forms about 7–9% of total body
weight.
• A man has 5–6 L of blood, while a woman has 4–5 L.
• Blood is thicker, denser and flows much slower than water
due to the RBCs and proteins, such as albumin and
fibrinogen.
• The pH of blood ranges
from 7.35 to 7.45.
Functions of blood
1. transportation: RBCs in the blood transport O2 from the lungs
to body tissues and waste products of cellular metabolism from the body
tissues to the kidneys, liver, lungs and sweat glands for elimination
from the body. Blood also transports nutrients, hormones, clotting factors
and enzymes throughout the body to maintain homeostasis.
2. Regulation: Blood regulates blood clotting, body temperature,
acid–base balance , and regulates fluid and electrolyte balance through
renal function.
3. protection. Blood defends the body against bacteria and viruses
(pathogens). Some WBCs, e.g. the neutrophils, engulf and destroy
pathogens while lymphocytes produce and secrete antibodies into blood.
Plasma
• Plasma is composed of water (91%), proteins (8%; albumin,
globulin, prothrombin and fibrinogen), salts (0.9%; sodium
chloride, sodium bicarbonate and others) and the remaining
0.1% is made up of organic materials, e.g. fats, glucose,
urea, uric acid, cholesterol and amino acids.
Diagnostic tests for blood disease
I .Hematologic Studies
• The most common tests used are the complete blood count
(CBC) and the peripheral blood smear.
• The CBC identifies the total number of blood cells
(leukocytes, erythrocytes, and platelets) as well as the
hemoglobin, hematocrit (percentage of blood volume
consisting of erythrocytes. The normal hematocrit for men
is 40 to 54%; for women it is 36 to 48%.).
• Peripheral smear identifies cellular morphology
means the shape and size of the erythrocytes and platelets,
as well as the actual appearance of the leukocytes which
provide useful information in identifying hematologic
conditions.
• II. Bone Marrow Aspiration and Biopsy
• These are crucial to assess the quantity and quality
of each type of cell produced within the marrow.
• These tests are also used
to document infection
or tumor within the marrow.
• Normal bone marrow is in a semifluid state and can
be aspirated through a special large needle.
• In adults, bone marrow is usually aspirated from the
iliac crest and occasionally from the sternum.
• Biopsy samples are taken from the posterior iliac
crest. A marrow biopsy shows the architecture of the
bone marrow as well as its degree of cellularity.
Blood Disease
• Anaemia
• Anaemia, refers to a reduction in RBCs and/or
haemoglobin. This results in a reduced ability
of the blood to transport oxygen to the tissues,
causing hypoxia.
•
• The normal level of haemoglobin in an adult
male is approximately 13–17 g/100 mL of
blood and in an adult female it is
approximately 12–16 g/100 mL of blood.
• Causes of Anemia
1. excessive loss of blood through haemorrhage
2. destruction of red blood cells (haemolysis)
3. deficient RBCs production due to bone
marrow failure.
4. infections such as malaria
5. lack of intake of iron, folic acid and vitamin
B12
6. pregnancy.
Sings and Symptoms
1. Fatigue due to hypoxia
2. Weakness due to hypoxia
3. Pallor due to less oxygen being available to the
surface tissues.
4. Tachycardia as the body attempts to compensate for
less available oxygen by beating more rapidly to
increase blood supply
5. Dyspnea or shortness of breath due to hypoxia as
body attempts to get more oxygen
6. Angina as the myocardium is not getting enough
oxygen
7. Headache due to hypoxia
8. Lightheadedness due to hypoxia
9. Bone pain due to increased erythropoiesis as body
attempts to correct anemia
10. Jaundice in hemolytic anemia due to increased
levels of bilirubin as red blood cells break down
• INTERPRETING TEST RESULTS
• Hemoglobin level low.
• Hematocrit level low.
• RBC count low.
• Reticulocyte count elevated (an immature RBCs without a
nucleus, having a reticulated (net or network ) appearance) to
compensate for the anemia.
Types of Anaemia
There are three major types of anaemia:
1. Microcytic anaemia (small red blood cells)
2. Macrocytic anaemia (large blood cells)
3. Normocytic anaemia (normal-sized red
blood cells).
• Microcytic anaemia
• Microcytic anaemia is characterised by small RBCs.
• There are several types of microcytic anaemia of
which iron deficiency anaemia is the most common
cause of anaemia.
• Iron deficiency anaemia
• Iron is essential for the production of young RBCs.
As iron is a component of haem, a deficiency of iron
leads to decreased Hb synthesis.
• In iron deficiency anaemia, the RBCs are small
(microcytic) and pale (hypochromic).
Iron deficiency anaemia
Aetiology
• Iron deficiency anaemia results from:
1. dietary deficiency of iron
2. loss of iron through haemorrhage
3. poor absorption of iron from the gastrointestinal
tract after gastrectomy.
4. increased demands to Iron such as growth and
pregnancy.
AT RISK GROUPS
Infants
Under 5 children
Children of school
age
Women of child
bearing age
• Investigations
• The following investigations may be carried out to
confirm diagnosis:
1. Complete blood count
2. test for levels of ferritin, serum iron, transferring,
folate, vitamin B12
3. bone marrow examination
INTERPRETING TEST RESULTS
1. Decrease in serum hemoglobin.
2. Serum ferritin is low.
3. Mean corpuscular volume (MCV) which shows size of cell
is low—microcytic anemia.
4. Mean corpuscular hemoglobin (MCH) which shows color
of cell is low (Pale cells)—hypochromic anemia.
5. Serum iron level is low.
6. Serum iron-binding capacity is increased.
7. Peripheral blood smear shows poikilocytosis (RBCs of
different shapes).
8. Platelet count may increase.
• Signs and symptoms
1. brittle nails
2. spoon-shaped nails (koilonychias)
3. brittle hair
4. cheilosis (cracks at the corners of the
mouth)
5. dizziness
6. hypoxia
7. pica (craving to eat unusual substances such as clay, starch and coal)
8. breathlessness
9. loss of appetite, which may be due to a sore mouth.
TREATMENT
1. Oral replacement of iron in split doses (three times a day):
• ferrous sulphate 200 mg/day
• ferrous gluconate
• ferrous fumarate RBCs.
2. Parenteral iron replacement for those who cannot tolerate or
do not respond to oral therapy, have GIT illness, or continued
bleeding; iron dextran given deep IM or IV; IM injection of
iron using Z-track method.
3. Increase dietary intake of iron.
TREATMENT (con’t)
4. The typical timeframe for oral therapy of Iron replacement
is to continue for 3 to 6 months after the anemia has been
corrected.
5. Care should be taken to avoid anaphylactic reactions to iron
dextran. Patients new to this treatment typically have a smaller
test dose initially, prior to the initiation of treatments.
6. Patients should be advised about the side effects of ferrous
sulfate, which include constipation, nausea and even
diarrhoea. They should be advised to drink 2–3 L of fluid per
day to prevent constipation.
• Macrocytic anaemia
• Macrocytic anaemia is also termed megaloblastic
anaemia. It is characterised by defective
deoxyribonucleic acid (DNA) synthesis resulting in
the production of unusually large stem cells
(macrocytes) in the circulation. In addition to an
increase in diameter, the thickness and the volume
of the cell.
• Aetiology
Macrocytic anaemia results from:
1. folate deficiency
2. vitamin B12 deficiency.
Both these are essential for DNA maturation.
3. Vegans and vegetarians are at risk of developing
macrocytic anaemia due to a lack of vitamin B12
which is found in most meat products.
• Folate deficiency
• Folic acid (folate) is an essential for the production
and maturation of RBCs.
• Folate is obtained from the diet and is absorbed from
the jejunum and stored in the liver.
• It is found in leafy vegetables, fruit (citrus fruit and
juice, avocado), cereals, dried beans, peas, nuts and
meat; most of it is lost in cooking.
Aetiology
1. malnutrition
2. malabsorption from the jejunum caused by diseases
such as coeliac disease (autoimmune
disorder primarily affecting the small intestine that
occurs in people who are genetically predisposed)
3. medications that inhibit absorption from the
jejunum, e.g. oral contraceptives and anticonvulsants
such as phenytoin
4. alcohol abuse – alcohol interferes with folate
metabolism in the liver
5. anorexia.
• Sings & Symptoms
1. fatigue
2. palpitations
3. shortness of breath
4. diarrhoea
5. progressive weakness
6. pallor
Folate Deficiency Treatment
1. Oral folate 5mg daily for 4 months or until
hematologic recovery.
2. Diet high in in leafy green vegetables such as
such as broccoli and spinach, fruit (citrus fruit and
juice, avocado), cereals, dried beans, peas, nuts
and meat especially liver.
Vitamin B12 deficiency
# vitamin B12 deficiency causes pernicious anaemia
(PA).
# Vitamin B12 is essential for the synthesis of DNA
and a deficiency impairs cellular division and
maturation.
# The absorption of vitamin B12 in the intestine
requires the presence of intrinsic factor (IF), which is
produced by the gastric mucosa.
.Intrinsic factor binds to vitamin B12 in food,
protecting it from gastrointestinal enzymes and
facilitating its absorption.
# Lack of vitamin B12 alters the structure of RBCs
and disrupts the function of the peripheral nerves,
spinal cord and brain.
Aetiology
1. total gastrectomy, partial gastrectomy or
gastrojejunostomy.
2. gastric lesions
3. carcinoma of the stomach
4. alcohol abuse
5. malabsorption due to inflammatory disease such as
Crohn’s disease ( is a type of inflammatory bowel
disease (IBD) that may affect any part of
the GIT from mouth to anus)
Sings & Symptoms
1. Pallor due to anemia
2. Weakness and fatigue due to anemia
3. paresthesias – numbness and tingling in the extremities
4. Poor balance due to effect on cerebral function
5. Dementia appears later in the disease
6. Premature graying of hair
7. Nausea may lead to anorexia and weight loss
8. Atrophic glossitis-beefy red tongue
• TREATMENT
1. Lifelong replacement with vitamin B12 will correct the
anemia and improve the neurologic changes that have
occurred.
2. Initially weekly injections of B12 to replace the deficiency,
then injections become monthly for lifelong maintenance.
3. Oral supplementation is not effective in these patients
because they cannot adequately absorb vitamin B12 due to
insufficient intrinsic factor.
4.Transfusion of packed RBC if anemia is severe.
5. Patients should be advised to eat foods that contain vitamin
B12 such as eggs, meat and dairy products.
• Normocytic anaemia
• Normocytic anaemia is characterised by RBCs that
are relatively normal in size and in haemoglobin
content, but insufficient in number.
• It is less common than microcytic and macrocytic
anaemias.
Normocytic anaemias include:
• aplastic anaemia
• haemolytic anaemia
• sickle cell anaemia.
Aplastic anaemia
• is a serious condition affecting the bone marrow. It is
characterised by a reduction of all the blood cells, i.e. the
RBCs, WBCs and platelets.
• When all three types of blood cells are low the condition is
termed pancytopenia.
Aetiology
The condition is idiopathic; however, the condition has been associated
with:
1. viral diseases, e.g. hepatitis and HIV
2. ionizing radiation
3. metastases of the bone
4. cytotoxic drugs
5. chemical compounds, e.g. benzene. (one of the chemicals used in
petrol and a solvent used in the rubber and plastic industry).
• Sings & Symptoms
1. weakness
2. fatigue
3. pallor caused by anaemia
4. petechiae – small haemorrhages
under the skin
5. ecchymoses – bruises on the skin.
6. bleeding from mucous membranes
of the nose, gums, vagina and GIT
may occur as a result of decreased platelet level
7. prone to infections as a result of a low neutrophil
count.
INTERPRETING TEST RESULTS
• Low hemoglobin.
• Low hematocrit.
• Low RBC count.
• Thrombocytopenia—low platelet count.
• Leukopenia—low WBC.
• Positive fecal occult blood test.
TREATMENT
1. Administer Erythropoietin by SC injection or IV
2. Packed RBC transfusions when anemia is symptomatic.
3. Platelet transfusion for severe bleeding.
4. Bone marrow transplant replaces functioning stem cells.
5. Administer immunosuppressive drugs, and corticosteroids.
6. Splenectomy when spleen is enlarged
7. iron supplement
8. folic acid supplement
9. vitamin B12 supplement.
• NURSING DIAGNOSES
• Risk for infection
• Activity intolerance
• Risk for fluid volume deficient
• NURSING INTERVENTION
1. Monitor vital signs for changes.
2. Record intake and output of fluids.
3. Protect patient from falls.
4. Avoid IM injections due to altered clotting ability.
5. Explain to the patient to avoid aspirin due to effect on
platelet aggregation (clotting ability).
6. Plan to take rest periods during activities due to fatigue.
8. Only use an electric razor to decrease risk of bleeding due
to decreased platelet count.
9. Notify physician, nurse practitioner for signs of bleeding or
bruising.
• Haemolytic anaemia
• Haemolytic anaemia is a rare condition results from
the premature destruction of RBCs, leading to the
retention of iron and other products of RBCs
destruction.
• In haemolytic anaemia, the synthesis of RBCs in
the bone marrow is increased to match the number
of RBCs destroyed.
Aetiology
• The causes of haemolytic anaemia can be either
inherited or acquired and they include:
1. spherocytosis – fragility of the RBCs membrane
2. Hb defects – thalassaemia and sickle cell disease.
3. mismatched blood transfusion
4. direct cell injury from drugs, e.g. sodium chlorate
5. haemoglobinopathies: abnormalities in Hb structure.
• Signs and symptoms
1. jaundice, due to exceeds RBCs destruction
2. fatigue
3. hypoxia from impaired oxygen transport
4. dyspnoea
5. the spleen may become enlarged in patients with
congenital haemolytic disorders.
Care and management
1. advice on diet as for other forms of anaemia
2. relieving anxiety in patients and their relatives
3. blood transfusion and administration of prescribed
medication (like aplastic anaemia).
4. If patients are breathless, they must be put in upright,
supported by pillows, and oxygen administered as prescribed.
• Case study
• Mrs Nadia is a 48-year-old second-year student
nurse. Mrs Nadia has been complaining of tiredness,
breathlessness and that her ankles are slightly
swollen. She does suffer from gastritis and over 5
years ago half of her stomach was removed because
of cancer. Mrs Nadia does drink alcohol but not
excessively. Her husband convinced her to go and
see her General Practitioner (GP) to get some advice
and treatment.
Take some time to reflect on this case and then
consider the following.
1. Which type of anaemia is Mrs Nadia suffering
from?
2. Discuss the possible investigations that may be
carried out to confirm diagnosis.
3. List the medications the GP may prescribe to treat
her illness.
4. What advice will you give Mrs Nadia with regards
her diet and life style?
Leukaemia
• Leukaemia is a malignant disorder where there
is an abnormal or excessive proliferation of
immature WBCs.
• Leukaemia affect more men than women.
Aetiology
The exact cause of leukemia is unknown.
The risk factors include:
1. exposure to radiation
2. exposure to benzene
3. certain genetic conditions such as Down’s syndrome
4. smoking
5. age – chronic leukaemia is more common over the age of 40
years
6. a history of aggressive chemotherapy for a different type
of cancer
7. a family history of leukemia.
8. diseases that affect the immune system such as HIV.
• Pathophysiology
Replacement of bone marrow by abnormal cells
results in unregulated proliferation of immature WBCs
entering the circulatory system. These leukemic cells
may also enter the liver, spleen, or lymph nodes,
causing these areas to enlarge.
Leukaemic cells are immature; they proliferate
rapidly, have a long lifespan and do not function
normally, and do not become mature enough to carry
out their role in the immune system.
Classification of Leukemia
There are two principal types of Leukemia:
1. acute
2. chronic.
Acute leukaemia is more aggressive and develops rapidly. It is
more common in the younger age group and the symptoms
develop quickly; if untreated, it becomes life-threatening.
Leukemia is also classified according to the type of
cell it is derived from:
• acute myeloid leukaemia (AML)
• acute lymphoblastic leukaemia (ALL)
• chronic myeloid leukaemia (CML)
• chronic lymphoblastic leukaemia (CLL).
Classification of Leukemia (con’t)
# Lymphocytic
# Myelocytic
Lymphocytic leukemias involve immature
lymphocytes originating in the bone marrow and
typically infiltrate the spleen, lymph nodes, or central
nervous system.
Myelogenous or myelocytic leukemia involves the
myeloid stem cells in the bone marrow and interferes
with the maturation of all blood cell types
(granulocytes, erythrocytes, thrombocytes).
• Classification of Leukemia (con’t)
• Acute Myelogenous leukemia (AML) is
overproduction of immature myeloid WBCs and
Acute Lymphoblastic leukemia (ALL) is the
overproduction of immature lymphoblasts.
• Chronic Lymphoblastic Leukaemia (CLL) is more
common in men and occurs most frequently between
the ages of 50 and 70 years.
• Patients affected may live with symptoms for
several years.
• Classification of Leukemia (con’t)
• Chronic Myeloid Leukaemia (CML) has a gradual
onset, occurring primarily between the ages 30 and
50 years, and the incidence is slightly higher in men.
• In CML, there is uncontrolled production of
myeloid cells.
• These cells are abnormal and are not able to carry
out the normal functions of WBCs, such as fighting
infections.
• SIGNS AND SYMPTOMS
1. Fatigue, weakness, dyspnea and pale skin due to anemia
2. Fever due to increased susceptibility to infection
3. Bleeding, petechiae, ecchymosis (bruising), epistaxis,
gingival (gum) bleeding—due to decreased platelet count
4. Bone pain due to marrow expansion
5. Lymph nodes (lymphadenopathy) enlarged as leukemic
cells invade nodes
6. abdominal pain due to an enlarged spleen and/or liver
(hepatomegaly and splenomegaly)
7. Headache, nausea, vomiting, and weight loss
8. Papilledema, seizure if there is CNS involvement.
9. swollen lymph glands in the groin, neck and under the arms.
• INTERPRETING TEST RESULTS
• Low RBC count, low hemoglobin—anemia.
• Low platelet count—thrombocytopenia.
• Elevated WBC count—leukocytosis.
• Abnormal amount of immature WBC shown in bone
marrow biopsy.
• Pharmacological and non-pharmacological
interventions for Leukemia
• Medications and other treatments of leukaemia
include:
1. chemotherapy (use of cytotoxic drugs)
2. radiotherapy
3. stem cell and bone marrow transplants
4. opioid drugs to control pain such as Morphine .
• Care and management
1. Advice on preventative measures for bleeding
should be offered, i.e. the use soft toothbrush, use an
electric razor, and measures to prevent falls.
2. advice on measures to maintain hydration and
nutrition. Weight is monitored weekly in order to
assess weight loss.
3. daily oral hygiene to avoid Stomatitis
4. patients should be advised to take frequent rest
periods and not to over-exert themselves.
5. Patients should be protected from infections, e.g.
washing hands before and after attending to them and
discouraging unnecessary visitation by relatives.
• Leukaemia
Case study
Mr Tate is a 44-year-old policeman. Over the past 2–3 months
Mr Tate has been feeling excessively tired. Although he sleeps
well at night, he does not feel rested in the morning. Over the
past 2 weeks, Mr Tate has been complaining of a sore throat,
persistent colds and mouth ulcers. His wife persuaded him to
see his GP.
After a thorough physical examination, it was decided that Mr
Tate should be seen by a specialist at the hospital. Mr Tate is
seen by the haematology consultant at the local hospital and
after some blood tests and investigations a provisional
diagnosis of acute myeloid leukaemia is made. The consultant
decided to admit Mr Tate for further investigations and
treatment.
• Take some time to reflect on this case and then
consider the following.
1. Discuss the possible tests that may be done to
confirm diagnosis.
2. What are the risk factors associated with acute
myeloid leukaemia?
3. What advice would you give Mr Tate if he is to
commence chemotherapy?
4. Outline Mr Tate’s care during his stay in hospital.
• Multiple Myeloma
• A malignancy of the plasma cells causes an excessive
amount of plasma cells in the bone marrow.
• Masses within the bone marrow cause destructive lesions in
the bone.
• Normal bone marrow function is reduced as the abnormal
plasma cells continue to grow. Immune function is
diminished and the patient develops anemia.
• Is the second-most common cancer of the blood.
• The disease typically affects older adults.
Bone marrow angiogenesis in multiple myeloma.
A, Normal bone marrow
B, Bone marrow from a patient with multiple myeloma. New blood
vessel formation is noted
SIGNS AND SYMPTOMS
1. Severe bone pain in back or ribs
2. Anemia due to invasion of the bone marrow.
3. Skeletal fractures due to loss of normal bone
structure (osteoporosis)
4. Increased risk of infection due to bone marrow
failure to produce WBCs
5. Spinal cord compression as mass enlarges
6. Renal failure due to protein effect in renal tubules.
INTERPRETING TEST RESULTS
1. Presence of the Bence Jones protein in urine.
2. CBC shows anemia.
3. Abnormal plasma cells in bone marrow biopsy.
4. . X-rays of bone show lytic lesions.
5. Elevated calcium in blood (hypercalcemia).
6. Protein in urine (proteinuria).
7. Elevated erythrocyte sedimentation rate (ESR).
TREATMENT
Combination therapy is common in treatment of
multiple myeloma.
1. Pain management.
2. chemotherapy:
3. Diet high in protein, carbohydrates, vitamins, and
minerals.
4. Small frequent meals.
5. Transfusion of packed RBCs if anemia is severe.
6. Bone marrow transplantation.
hemorrhagic disorder
Thrombocytopenia
Is the most common hemorrhagic disorder,
thrombocytopenia is characterized by a deficiency of
circulating platelets.
Because platelets play a vital role in blood clotting,
this disorder seriously threatens hemostasis.
• Aetiology
Many disease processes can cause thrombocytopenia:
1. anaemia as a result of vitamin B12 or folic acid
deficiency
2. systemic lupus erythematous
3. sepsis, systemic viral or bacterial infections
4. chemotherapy
5. radiation
6. heparin-induced thrombocytopenia (white clot
syndrome)
• HIV.
Signs and symptoms
1. unexpected bruising
2. petechiae (small red spots under
the skin)
3. bleeding from the GIT
4. epistaxis (bleeding from the nose)
5. pain in the joints and muscles
6. heavier than usual menstrual
periods in women.
Care and management
1. Monitor vital signs – heart rate, respiratory rate and
blood pressure – every 4 hours.
2. Observe for bleeding from other parts of the body,
such as in the urine (haematuria), GIT, nasal
membrane and vagina.
3. Observe the skin for petechiae.
4. Advise the patient about the use of safety measures
to minimize the risk of bleeding, such as use of a soft-
bristled toothbrush and an electric razor for shaving.
5. Encourage the patient to rinse the mouth with salt
solution every 2–4 hours to maintain oral hygiene.
6. Advise the patient to take 2–2.5 L of fluid over 24
hours to prevent dehydration and infection.
7. Advise the patient to avoid medications that
interfere with platelet function such as aspirin.
8. A healthy diet high in fiber should be encouraged
to prevent constipation. Straining to have a bowel
movement could increase the risk of internal bleeding
from the GIT.
• Pharmacological and non-pharmacological
interventions
1. Platelet transfusion
2. Oral glucocorticoids such a prednisolone to
suppress the autoimmune response.
3. Splenectomy may be performed.
Hemophilia
Hemophilia
• Hemophilia is a hereditary bleeding disorder that
results from lack of specific clotting factors as a
result the blood does not clot when the patient
bleeds. It occurs in two main forms:
• Hemophilia A (classic hemophilia), seen in more
than 80% of hemophilia cases, results from deficiency
of factor VIII.
• Hemophilia B (Christmas disease), which accounts
for roughly 15% of hemophilia cases, results from
deficiency of factor IX.
• Etiology
• Hemophilia A and B are inherited as X-linked Gene.
• female carriers have a 50% chance of transmitting
the gene to each son or daughter.
• Daughters who receive the gene are carriers; sons
who receive it are born with hemophilia.
• SIGNS AND SYMPTOMS
• Tender joints due to bleeding
• Swelling of knees, ankles, hips,
and elbows due to bleeding
• Blood in stool (tarry stool) due to GI blood loss
• Blood in the urine (hematuria)
• INTERPRETING TEST RESULTS
• PTT prolonged.
• PT normal.
• Bleeding time normal.
• Fibrinogen level normal.
• Decrease in clotting factor VIII found in blood serum
in Hemophilia A.
• Decrease in clotting factor IX found in blood serum
in Hemophilia B.
TREATMENT
1. Avoid aspirin.
2. For hemophilia A administer factor VIII
concentrates.
3. Cryoprecipitate.
4. Fresh frozen plasma also can be given.
5. For hemophilia B administer factor IX concentrates.
NURSING DIAGNOSES
• Acute pain
• Impaired gas exchange
NURSING INTERVENTION
1. No IM injections.
2. No aspirin.
3. To stop bleeding:
A. Elevate site.
B. Apply direct pressure to the site.
4. Explain to the patient:
A. Contact physician for any injury.
B. Avoid situations where injury might occur.

Blood disease

  • 1.
    Nursing Management forpatient with blood disease Prepared By Dr. Zuhair Rushdi Mustafa Lecturer at University of Duhok/ College of Nursing
  • 2.
    The blood composedof cellular component and plasma. The cellular component of blood consists of 3 primary cell types: (1) Erthrocytes (RBCs) which account for 45% of the blood. # The remaining 1% consists of (2) white blood cells and (3) platelets. # plasma (a yellowish liquid containing nutrients, hormones, minerals) The Plasma makes up 55% of the total blood volume. COMPOSITION OF BLOOD
  • 3.
    Properties of blood •In a healthy person, blood forms about 7–9% of total body weight. • A man has 5–6 L of blood, while a woman has 4–5 L. • Blood is thicker, denser and flows much slower than water due to the RBCs and proteins, such as albumin and fibrinogen. • The pH of blood ranges from 7.35 to 7.45.
  • 4.
    Functions of blood 1.transportation: RBCs in the blood transport O2 from the lungs to body tissues and waste products of cellular metabolism from the body tissues to the kidneys, liver, lungs and sweat glands for elimination from the body. Blood also transports nutrients, hormones, clotting factors and enzymes throughout the body to maintain homeostasis. 2. Regulation: Blood regulates blood clotting, body temperature, acid–base balance , and regulates fluid and electrolyte balance through renal function. 3. protection. Blood defends the body against bacteria and viruses (pathogens). Some WBCs, e.g. the neutrophils, engulf and destroy pathogens while lymphocytes produce and secrete antibodies into blood.
  • 5.
    Plasma • Plasma iscomposed of water (91%), proteins (8%; albumin, globulin, prothrombin and fibrinogen), salts (0.9%; sodium chloride, sodium bicarbonate and others) and the remaining 0.1% is made up of organic materials, e.g. fats, glucose, urea, uric acid, cholesterol and amino acids.
  • 6.
    Diagnostic tests forblood disease I .Hematologic Studies • The most common tests used are the complete blood count (CBC) and the peripheral blood smear. • The CBC identifies the total number of blood cells (leukocytes, erythrocytes, and platelets) as well as the hemoglobin, hematocrit (percentage of blood volume consisting of erythrocytes. The normal hematocrit for men is 40 to 54%; for women it is 36 to 48%.). • Peripheral smear identifies cellular morphology means the shape and size of the erythrocytes and platelets, as well as the actual appearance of the leukocytes which provide useful information in identifying hematologic conditions.
  • 7.
    • II. BoneMarrow Aspiration and Biopsy • These are crucial to assess the quantity and quality of each type of cell produced within the marrow. • These tests are also used to document infection or tumor within the marrow. • Normal bone marrow is in a semifluid state and can be aspirated through a special large needle.
  • 8.
    • In adults,bone marrow is usually aspirated from the iliac crest and occasionally from the sternum. • Biopsy samples are taken from the posterior iliac crest. A marrow biopsy shows the architecture of the bone marrow as well as its degree of cellularity.
  • 9.
    Blood Disease • Anaemia •Anaemia, refers to a reduction in RBCs and/or haemoglobin. This results in a reduced ability of the blood to transport oxygen to the tissues, causing hypoxia. • • The normal level of haemoglobin in an adult male is approximately 13–17 g/100 mL of blood and in an adult female it is approximately 12–16 g/100 mL of blood.
  • 10.
    • Causes ofAnemia 1. excessive loss of blood through haemorrhage 2. destruction of red blood cells (haemolysis) 3. deficient RBCs production due to bone marrow failure. 4. infections such as malaria 5. lack of intake of iron, folic acid and vitamin B12 6. pregnancy.
  • 11.
    Sings and Symptoms 1.Fatigue due to hypoxia 2. Weakness due to hypoxia 3. Pallor due to less oxygen being available to the surface tissues. 4. Tachycardia as the body attempts to compensate for less available oxygen by beating more rapidly to increase blood supply 5. Dyspnea or shortness of breath due to hypoxia as body attempts to get more oxygen
  • 12.
    6. Angina asthe myocardium is not getting enough oxygen 7. Headache due to hypoxia 8. Lightheadedness due to hypoxia 9. Bone pain due to increased erythropoiesis as body attempts to correct anemia 10. Jaundice in hemolytic anemia due to increased levels of bilirubin as red blood cells break down
  • 13.
    • INTERPRETING TESTRESULTS • Hemoglobin level low. • Hematocrit level low. • RBC count low. • Reticulocyte count elevated (an immature RBCs without a nucleus, having a reticulated (net or network ) appearance) to compensate for the anemia.
  • 14.
    Types of Anaemia Thereare three major types of anaemia: 1. Microcytic anaemia (small red blood cells) 2. Macrocytic anaemia (large blood cells) 3. Normocytic anaemia (normal-sized red blood cells).
  • 15.
    • Microcytic anaemia •Microcytic anaemia is characterised by small RBCs. • There are several types of microcytic anaemia of which iron deficiency anaemia is the most common cause of anaemia. • Iron deficiency anaemia • Iron is essential for the production of young RBCs. As iron is a component of haem, a deficiency of iron leads to decreased Hb synthesis. • In iron deficiency anaemia, the RBCs are small (microcytic) and pale (hypochromic).
  • 16.
    Iron deficiency anaemia Aetiology •Iron deficiency anaemia results from: 1. dietary deficiency of iron 2. loss of iron through haemorrhage 3. poor absorption of iron from the gastrointestinal tract after gastrectomy. 4. increased demands to Iron such as growth and pregnancy.
  • 17.
    AT RISK GROUPS Infants Under5 children Children of school age Women of child bearing age
  • 18.
    • Investigations • Thefollowing investigations may be carried out to confirm diagnosis: 1. Complete blood count 2. test for levels of ferritin, serum iron, transferring, folate, vitamin B12 3. bone marrow examination
  • 19.
    INTERPRETING TEST RESULTS 1.Decrease in serum hemoglobin. 2. Serum ferritin is low. 3. Mean corpuscular volume (MCV) which shows size of cell is low—microcytic anemia. 4. Mean corpuscular hemoglobin (MCH) which shows color of cell is low (Pale cells)—hypochromic anemia. 5. Serum iron level is low. 6. Serum iron-binding capacity is increased. 7. Peripheral blood smear shows poikilocytosis (RBCs of different shapes). 8. Platelet count may increase.
  • 21.
    • Signs andsymptoms 1. brittle nails 2. spoon-shaped nails (koilonychias) 3. brittle hair 4. cheilosis (cracks at the corners of the mouth) 5. dizziness 6. hypoxia 7. pica (craving to eat unusual substances such as clay, starch and coal) 8. breathlessness 9. loss of appetite, which may be due to a sore mouth.
  • 22.
    TREATMENT 1. Oral replacementof iron in split doses (three times a day): • ferrous sulphate 200 mg/day • ferrous gluconate • ferrous fumarate RBCs. 2. Parenteral iron replacement for those who cannot tolerate or do not respond to oral therapy, have GIT illness, or continued bleeding; iron dextran given deep IM or IV; IM injection of iron using Z-track method. 3. Increase dietary intake of iron.
  • 23.
    TREATMENT (con’t) 4. Thetypical timeframe for oral therapy of Iron replacement is to continue for 3 to 6 months after the anemia has been corrected. 5. Care should be taken to avoid anaphylactic reactions to iron dextran. Patients new to this treatment typically have a smaller test dose initially, prior to the initiation of treatments. 6. Patients should be advised about the side effects of ferrous sulfate, which include constipation, nausea and even diarrhoea. They should be advised to drink 2–3 L of fluid per day to prevent constipation.
  • 24.
    • Macrocytic anaemia •Macrocytic anaemia is also termed megaloblastic anaemia. It is characterised by defective deoxyribonucleic acid (DNA) synthesis resulting in the production of unusually large stem cells (macrocytes) in the circulation. In addition to an increase in diameter, the thickness and the volume of the cell.
  • 25.
    • Aetiology Macrocytic anaemiaresults from: 1. folate deficiency 2. vitamin B12 deficiency. Both these are essential for DNA maturation. 3. Vegans and vegetarians are at risk of developing macrocytic anaemia due to a lack of vitamin B12 which is found in most meat products.
  • 26.
    • Folate deficiency •Folic acid (folate) is an essential for the production and maturation of RBCs. • Folate is obtained from the diet and is absorbed from the jejunum and stored in the liver. • It is found in leafy vegetables, fruit (citrus fruit and juice, avocado), cereals, dried beans, peas, nuts and meat; most of it is lost in cooking.
  • 27.
    Aetiology 1. malnutrition 2. malabsorptionfrom the jejunum caused by diseases such as coeliac disease (autoimmune disorder primarily affecting the small intestine that occurs in people who are genetically predisposed) 3. medications that inhibit absorption from the jejunum, e.g. oral contraceptives and anticonvulsants such as phenytoin 4. alcohol abuse – alcohol interferes with folate metabolism in the liver 5. anorexia.
  • 28.
    • Sings &Symptoms 1. fatigue 2. palpitations 3. shortness of breath 4. diarrhoea 5. progressive weakness 6. pallor
  • 29.
    Folate Deficiency Treatment 1.Oral folate 5mg daily for 4 months or until hematologic recovery. 2. Diet high in in leafy green vegetables such as such as broccoli and spinach, fruit (citrus fruit and juice, avocado), cereals, dried beans, peas, nuts and meat especially liver.
  • 30.
    Vitamin B12 deficiency #vitamin B12 deficiency causes pernicious anaemia (PA). # Vitamin B12 is essential for the synthesis of DNA and a deficiency impairs cellular division and maturation.
  • 31.
    # The absorptionof vitamin B12 in the intestine requires the presence of intrinsic factor (IF), which is produced by the gastric mucosa. .Intrinsic factor binds to vitamin B12 in food, protecting it from gastrointestinal enzymes and facilitating its absorption. # Lack of vitamin B12 alters the structure of RBCs and disrupts the function of the peripheral nerves, spinal cord and brain.
  • 32.
    Aetiology 1. total gastrectomy,partial gastrectomy or gastrojejunostomy. 2. gastric lesions 3. carcinoma of the stomach 4. alcohol abuse 5. malabsorption due to inflammatory disease such as Crohn’s disease ( is a type of inflammatory bowel disease (IBD) that may affect any part of the GIT from mouth to anus)
  • 33.
    Sings & Symptoms 1.Pallor due to anemia 2. Weakness and fatigue due to anemia 3. paresthesias – numbness and tingling in the extremities 4. Poor balance due to effect on cerebral function 5. Dementia appears later in the disease 6. Premature graying of hair 7. Nausea may lead to anorexia and weight loss 8. Atrophic glossitis-beefy red tongue
  • 34.
    • TREATMENT 1. Lifelongreplacement with vitamin B12 will correct the anemia and improve the neurologic changes that have occurred. 2. Initially weekly injections of B12 to replace the deficiency, then injections become monthly for lifelong maintenance. 3. Oral supplementation is not effective in these patients because they cannot adequately absorb vitamin B12 due to insufficient intrinsic factor. 4.Transfusion of packed RBC if anemia is severe. 5. Patients should be advised to eat foods that contain vitamin B12 such as eggs, meat and dairy products.
  • 35.
    • Normocytic anaemia •Normocytic anaemia is characterised by RBCs that are relatively normal in size and in haemoglobin content, but insufficient in number. • It is less common than microcytic and macrocytic anaemias. Normocytic anaemias include: • aplastic anaemia • haemolytic anaemia • sickle cell anaemia.
  • 36.
    Aplastic anaemia • isa serious condition affecting the bone marrow. It is characterised by a reduction of all the blood cells, i.e. the RBCs, WBCs and platelets. • When all three types of blood cells are low the condition is termed pancytopenia. Aetiology The condition is idiopathic; however, the condition has been associated with: 1. viral diseases, e.g. hepatitis and HIV 2. ionizing radiation 3. metastases of the bone 4. cytotoxic drugs 5. chemical compounds, e.g. benzene. (one of the chemicals used in petrol and a solvent used in the rubber and plastic industry).
  • 37.
    • Sings &Symptoms 1. weakness 2. fatigue 3. pallor caused by anaemia 4. petechiae – small haemorrhages under the skin 5. ecchymoses – bruises on the skin. 6. bleeding from mucous membranes of the nose, gums, vagina and GIT may occur as a result of decreased platelet level 7. prone to infections as a result of a low neutrophil count.
  • 38.
    INTERPRETING TEST RESULTS •Low hemoglobin. • Low hematocrit. • Low RBC count. • Thrombocytopenia—low platelet count. • Leukopenia—low WBC. • Positive fecal occult blood test.
  • 39.
    TREATMENT 1. Administer Erythropoietinby SC injection or IV 2. Packed RBC transfusions when anemia is symptomatic. 3. Platelet transfusion for severe bleeding. 4. Bone marrow transplant replaces functioning stem cells. 5. Administer immunosuppressive drugs, and corticosteroids. 6. Splenectomy when spleen is enlarged 7. iron supplement 8. folic acid supplement 9. vitamin B12 supplement.
  • 40.
    • NURSING DIAGNOSES •Risk for infection • Activity intolerance • Risk for fluid volume deficient
  • 41.
    • NURSING INTERVENTION 1.Monitor vital signs for changes. 2. Record intake and output of fluids. 3. Protect patient from falls. 4. Avoid IM injections due to altered clotting ability. 5. Explain to the patient to avoid aspirin due to effect on platelet aggregation (clotting ability). 6. Plan to take rest periods during activities due to fatigue. 8. Only use an electric razor to decrease risk of bleeding due to decreased platelet count. 9. Notify physician, nurse practitioner for signs of bleeding or bruising.
  • 42.
    • Haemolytic anaemia •Haemolytic anaemia is a rare condition results from the premature destruction of RBCs, leading to the retention of iron and other products of RBCs destruction. • In haemolytic anaemia, the synthesis of RBCs in the bone marrow is increased to match the number of RBCs destroyed.
  • 43.
    Aetiology • The causesof haemolytic anaemia can be either inherited or acquired and they include: 1. spherocytosis – fragility of the RBCs membrane 2. Hb defects – thalassaemia and sickle cell disease. 3. mismatched blood transfusion 4. direct cell injury from drugs, e.g. sodium chlorate 5. haemoglobinopathies: abnormalities in Hb structure.
  • 44.
    • Signs andsymptoms 1. jaundice, due to exceeds RBCs destruction 2. fatigue 3. hypoxia from impaired oxygen transport 4. dyspnoea 5. the spleen may become enlarged in patients with congenital haemolytic disorders.
  • 45.
    Care and management 1.advice on diet as for other forms of anaemia 2. relieving anxiety in patients and their relatives 3. blood transfusion and administration of prescribed medication (like aplastic anaemia). 4. If patients are breathless, they must be put in upright, supported by pillows, and oxygen administered as prescribed.
  • 46.
    • Case study •Mrs Nadia is a 48-year-old second-year student nurse. Mrs Nadia has been complaining of tiredness, breathlessness and that her ankles are slightly swollen. She does suffer from gastritis and over 5 years ago half of her stomach was removed because of cancer. Mrs Nadia does drink alcohol but not excessively. Her husband convinced her to go and see her General Practitioner (GP) to get some advice and treatment.
  • 47.
    Take some timeto reflect on this case and then consider the following. 1. Which type of anaemia is Mrs Nadia suffering from? 2. Discuss the possible investigations that may be carried out to confirm diagnosis. 3. List the medications the GP may prescribe to treat her illness. 4. What advice will you give Mrs Nadia with regards her diet and life style?
  • 48.
    Leukaemia • Leukaemia isa malignant disorder where there is an abnormal or excessive proliferation of immature WBCs. • Leukaemia affect more men than women.
  • 49.
    Aetiology The exact causeof leukemia is unknown. The risk factors include: 1. exposure to radiation 2. exposure to benzene 3. certain genetic conditions such as Down’s syndrome 4. smoking 5. age – chronic leukaemia is more common over the age of 40 years 6. a history of aggressive chemotherapy for a different type of cancer 7. a family history of leukemia. 8. diseases that affect the immune system such as HIV.
  • 50.
    • Pathophysiology Replacement ofbone marrow by abnormal cells results in unregulated proliferation of immature WBCs entering the circulatory system. These leukemic cells may also enter the liver, spleen, or lymph nodes, causing these areas to enlarge. Leukaemic cells are immature; they proliferate rapidly, have a long lifespan and do not function normally, and do not become mature enough to carry out their role in the immune system.
  • 51.
    Classification of Leukemia Thereare two principal types of Leukemia: 1. acute 2. chronic. Acute leukaemia is more aggressive and develops rapidly. It is more common in the younger age group and the symptoms develop quickly; if untreated, it becomes life-threatening. Leukemia is also classified according to the type of cell it is derived from: • acute myeloid leukaemia (AML) • acute lymphoblastic leukaemia (ALL) • chronic myeloid leukaemia (CML) • chronic lymphoblastic leukaemia (CLL).
  • 52.
    Classification of Leukemia(con’t) # Lymphocytic # Myelocytic Lymphocytic leukemias involve immature lymphocytes originating in the bone marrow and typically infiltrate the spleen, lymph nodes, or central nervous system. Myelogenous or myelocytic leukemia involves the myeloid stem cells in the bone marrow and interferes with the maturation of all blood cell types (granulocytes, erythrocytes, thrombocytes).
  • 53.
    • Classification ofLeukemia (con’t) • Acute Myelogenous leukemia (AML) is overproduction of immature myeloid WBCs and Acute Lymphoblastic leukemia (ALL) is the overproduction of immature lymphoblasts. • Chronic Lymphoblastic Leukaemia (CLL) is more common in men and occurs most frequently between the ages of 50 and 70 years. • Patients affected may live with symptoms for several years.
  • 54.
    • Classification ofLeukemia (con’t) • Chronic Myeloid Leukaemia (CML) has a gradual onset, occurring primarily between the ages 30 and 50 years, and the incidence is slightly higher in men. • In CML, there is uncontrolled production of myeloid cells. • These cells are abnormal and are not able to carry out the normal functions of WBCs, such as fighting infections.
  • 55.
    • SIGNS ANDSYMPTOMS 1. Fatigue, weakness, dyspnea and pale skin due to anemia 2. Fever due to increased susceptibility to infection 3. Bleeding, petechiae, ecchymosis (bruising), epistaxis, gingival (gum) bleeding—due to decreased platelet count 4. Bone pain due to marrow expansion 5. Lymph nodes (lymphadenopathy) enlarged as leukemic cells invade nodes 6. abdominal pain due to an enlarged spleen and/or liver (hepatomegaly and splenomegaly) 7. Headache, nausea, vomiting, and weight loss 8. Papilledema, seizure if there is CNS involvement. 9. swollen lymph glands in the groin, neck and under the arms.
  • 56.
    • INTERPRETING TESTRESULTS • Low RBC count, low hemoglobin—anemia. • Low platelet count—thrombocytopenia. • Elevated WBC count—leukocytosis. • Abnormal amount of immature WBC shown in bone marrow biopsy.
  • 57.
    • Pharmacological andnon-pharmacological interventions for Leukemia • Medications and other treatments of leukaemia include: 1. chemotherapy (use of cytotoxic drugs) 2. radiotherapy 3. stem cell and bone marrow transplants 4. opioid drugs to control pain such as Morphine .
  • 58.
    • Care andmanagement 1. Advice on preventative measures for bleeding should be offered, i.e. the use soft toothbrush, use an electric razor, and measures to prevent falls. 2. advice on measures to maintain hydration and nutrition. Weight is monitored weekly in order to assess weight loss. 3. daily oral hygiene to avoid Stomatitis 4. patients should be advised to take frequent rest periods and not to over-exert themselves. 5. Patients should be protected from infections, e.g. washing hands before and after attending to them and discouraging unnecessary visitation by relatives.
  • 59.
    • Leukaemia Case study MrTate is a 44-year-old policeman. Over the past 2–3 months Mr Tate has been feeling excessively tired. Although he sleeps well at night, he does not feel rested in the morning. Over the past 2 weeks, Mr Tate has been complaining of a sore throat, persistent colds and mouth ulcers. His wife persuaded him to see his GP. After a thorough physical examination, it was decided that Mr Tate should be seen by a specialist at the hospital. Mr Tate is seen by the haematology consultant at the local hospital and after some blood tests and investigations a provisional diagnosis of acute myeloid leukaemia is made. The consultant decided to admit Mr Tate for further investigations and treatment.
  • 60.
    • Take sometime to reflect on this case and then consider the following. 1. Discuss the possible tests that may be done to confirm diagnosis. 2. What are the risk factors associated with acute myeloid leukaemia? 3. What advice would you give Mr Tate if he is to commence chemotherapy? 4. Outline Mr Tate’s care during his stay in hospital.
  • 61.
    • Multiple Myeloma •A malignancy of the plasma cells causes an excessive amount of plasma cells in the bone marrow. • Masses within the bone marrow cause destructive lesions in the bone. • Normal bone marrow function is reduced as the abnormal plasma cells continue to grow. Immune function is diminished and the patient develops anemia. • Is the second-most common cancer of the blood. • The disease typically affects older adults.
  • 62.
    Bone marrow angiogenesisin multiple myeloma. A, Normal bone marrow B, Bone marrow from a patient with multiple myeloma. New blood vessel formation is noted
  • 63.
    SIGNS AND SYMPTOMS 1.Severe bone pain in back or ribs 2. Anemia due to invasion of the bone marrow. 3. Skeletal fractures due to loss of normal bone structure (osteoporosis) 4. Increased risk of infection due to bone marrow failure to produce WBCs 5. Spinal cord compression as mass enlarges 6. Renal failure due to protein effect in renal tubules.
  • 64.
    INTERPRETING TEST RESULTS 1.Presence of the Bence Jones protein in urine. 2. CBC shows anemia. 3. Abnormal plasma cells in bone marrow biopsy. 4. . X-rays of bone show lytic lesions. 5. Elevated calcium in blood (hypercalcemia). 6. Protein in urine (proteinuria). 7. Elevated erythrocyte sedimentation rate (ESR).
  • 65.
    TREATMENT Combination therapy iscommon in treatment of multiple myeloma. 1. Pain management. 2. chemotherapy: 3. Diet high in protein, carbohydrates, vitamins, and minerals. 4. Small frequent meals. 5. Transfusion of packed RBCs if anemia is severe. 6. Bone marrow transplantation.
  • 66.
    hemorrhagic disorder Thrombocytopenia Is themost common hemorrhagic disorder, thrombocytopenia is characterized by a deficiency of circulating platelets. Because platelets play a vital role in blood clotting, this disorder seriously threatens hemostasis.
  • 67.
    • Aetiology Many diseaseprocesses can cause thrombocytopenia: 1. anaemia as a result of vitamin B12 or folic acid deficiency 2. systemic lupus erythematous 3. sepsis, systemic viral or bacterial infections 4. chemotherapy 5. radiation 6. heparin-induced thrombocytopenia (white clot syndrome) • HIV.
  • 68.
    Signs and symptoms 1.unexpected bruising 2. petechiae (small red spots under the skin) 3. bleeding from the GIT 4. epistaxis (bleeding from the nose) 5. pain in the joints and muscles 6. heavier than usual menstrual periods in women.
  • 69.
    Care and management 1.Monitor vital signs – heart rate, respiratory rate and blood pressure – every 4 hours. 2. Observe for bleeding from other parts of the body, such as in the urine (haematuria), GIT, nasal membrane and vagina. 3. Observe the skin for petechiae. 4. Advise the patient about the use of safety measures to minimize the risk of bleeding, such as use of a soft- bristled toothbrush and an electric razor for shaving.
  • 70.
    5. Encourage thepatient to rinse the mouth with salt solution every 2–4 hours to maintain oral hygiene. 6. Advise the patient to take 2–2.5 L of fluid over 24 hours to prevent dehydration and infection. 7. Advise the patient to avoid medications that interfere with platelet function such as aspirin. 8. A healthy diet high in fiber should be encouraged to prevent constipation. Straining to have a bowel movement could increase the risk of internal bleeding from the GIT.
  • 71.
    • Pharmacological andnon-pharmacological interventions 1. Platelet transfusion 2. Oral glucocorticoids such a prednisolone to suppress the autoimmune response. 3. Splenectomy may be performed.
  • 72.
    Hemophilia Hemophilia • Hemophilia isa hereditary bleeding disorder that results from lack of specific clotting factors as a result the blood does not clot when the patient bleeds. It occurs in two main forms: • Hemophilia A (classic hemophilia), seen in more than 80% of hemophilia cases, results from deficiency of factor VIII. • Hemophilia B (Christmas disease), which accounts for roughly 15% of hemophilia cases, results from deficiency of factor IX.
  • 73.
    • Etiology • HemophiliaA and B are inherited as X-linked Gene. • female carriers have a 50% chance of transmitting the gene to each son or daughter. • Daughters who receive the gene are carriers; sons who receive it are born with hemophilia. • SIGNS AND SYMPTOMS • Tender joints due to bleeding • Swelling of knees, ankles, hips, and elbows due to bleeding • Blood in stool (tarry stool) due to GI blood loss • Blood in the urine (hematuria)
  • 74.
    • INTERPRETING TESTRESULTS • PTT prolonged. • PT normal. • Bleeding time normal. • Fibrinogen level normal. • Decrease in clotting factor VIII found in blood serum in Hemophilia A. • Decrease in clotting factor IX found in blood serum in Hemophilia B.
  • 75.
    TREATMENT 1. Avoid aspirin. 2.For hemophilia A administer factor VIII concentrates. 3. Cryoprecipitate. 4. Fresh frozen plasma also can be given. 5. For hemophilia B administer factor IX concentrates.
  • 76.
    NURSING DIAGNOSES • Acutepain • Impaired gas exchange NURSING INTERVENTION 1. No IM injections. 2. No aspirin. 3. To stop bleeding: A. Elevate site. B. Apply direct pressure to the site. 4. Explain to the patient: A. Contact physician for any injury. B. Avoid situations where injury might occur.