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Janice Summers

Objectives
At the end of the teaching session the student will be

enabled to:
Describe the hematological system structure and
function with regards to maintaining homeostasis
Demonstrate understanding of the blood groups and
safe blood transfusion knowledge applied to practice
Discuss with understanding the tests undertaken for
diagnosis in the hematological system

Bone Marrow
Fig. 30-1. Development of blood cells. RBCs, Red blood cells; WBCs,
white blood cells.

Blood cell development


begins in the bone marrow
found mainly in flat and
irregular bones (long bones,
pelvic vertebrae sacrum,
cranial etc) Two types
yellow [adipose]and red
[hematopoietic] Red bone
marrow actively produces
blood cells All three types
red, white and platelets
develop from a common
hematopoietic stem cell
within the bone marrow .
When formed it is
undifferentiated as the cells
mature several different
types are formed . Bone
marrow is able to respond
for an increase in blood cells
by a negative feedback loop.
Stimulated by several factors
e.g. erythropoietin to RBC.

Blood Structure & Functions


Transportation of O2 nutrients hormones and waste

products
Regulation of fluid electrolyte and acid base balance
Protective role ability to clot and combat infections
Two major components include blood plasma 55%
and blood cells 45%
Albumin helps maintain osmotic pressure in the
blood
Three main types of blood cells
Plasma proteins albumin, globulin and clotting
factors mostly fibrinogen

Blood Cells Type


Red Blood cells (erythrocytes) transport of
gases O2 CO2
Erythopoeisis
Hemolysis -

White Blood Cells (Leukocytes) Granulocytes neutrophil, basophil and


eosinophil, main function phagocytosis

Non granulocytes lymphocytes 20 - 40% of


WBC, B and T lymphocytes and monocytes
Platelets (Thrombocytes) main function to

facilitate the clotting process

Three blood cell types RBC (erthrocytes) , WBC

(leukocytes) and Platelets (thrombocytes)


Erythrocyte transport O2 CO2, to assist in maintaining
acid base balance . Shape and flexibility allows it to pass
through tiny capillaries. Cell wall is thin to allow diffusion
of gases Primarily composed of hemoglobin, acting as a
buffer in acid based balance. Erythropoiesis is stimulated
by cellular O2 requirements and metabolic activity , and
controlled by erythropoietin a glycoprotein growth factor
synthesized and released by the kidney . It is also
influenced by the availibility of nutrients iron, folic acid
B12, ,B2 and B6 and affected by hormones such as thyroxin
corticosteroids and testosterone.
Hemolysis (destruction of old RBC) takes place in liver
bone marrow and spleen. One component is bilirubin so
therefore an increase bilirubin results after wards which is
generally disposed of by the liver

Leucocytes two main functions several different WBC

Granulocyte neutrophil, basophil and eosinophil phagocytic


action
An increase in neutrophils for example is a common diagnostic
indicator of infection
Esinophils are able to engulf antigen antibody complexes
formed during an allergic response AN elevated level seen in
some neoplastic disorders such as Hodgkin's lymphoma as well
as in skin disorders. Able to defend against parasitic invasion
Basophils have cytoplasmic granules that contain heparin,
serotonin and histamine. It responds by releasing substances
within granules Part of the response seen in allergic and
inflammatory reactions.
Monocytes potent phagocytic cells second type to arrive at the
site of an injury. Only present in the blood for a short time before
they migrate to tissues and become macrophages . These are
then called resident macrophages examples include the kuffer
cells in the liver, osteoclasts in the bone alveolar macrophages in
the lung . They also interact with lymphocytes to facilitate
humoral and cellular responses.

Platelets Thrombocytes) main function to facilitate

the clotting process They maintain capillary integrity


by plugging openings in the capillary wall. At the site
of damage platelet activation is initiated. Also
important in clot shrinkage and retraction. Poatelets
have a span of 5 9 days

White Blood Cells

Iron metabolism
Obtained from food - spinach, beets and

molasses, liver, fish, red meat.


5 10 % ingested iron absorbed in duodenum and
Upper Jejunum
Present in red blood cells as heme which is 2/3rd
bodys iron
Other 1/3rd stored as ferritin and hemosiderin in
the bone marrow, spleen liver and macrophages.
When stored iron not replaced haemoglobin is
reduced
Iron is recycled after macrophages in the liver and
spleen ingest, destroy old and damaged RBCs

There are two types of iron: heme-iron, which is found only

in meat, fish and poultry and is more easily absorbed by


your body and non-heme iron, which is found in plant
foods and is not as easily absorbed.
Fish and Shellfish
Oysters are one of the few foods that trump beef liver when
it comes to iron content (about 5.5 mg per 3-ounce
serving). Clams, shrimp and sardines also provide a hearty
dose. White fish and salmon contain smaller amounts,
comparable to the iron content of light meat poultry
Read more: http://www.livestrong.com/article/92302-

foods-highest-iron-those-anemia/#ixzz2RDFp92t2

Clotting Mechanisms
http://www.youtube.com/watch?v=gGGAUt6N
mJg
http://users.rcn.com/jkimball.ma.ultranet/Biol
ogyPages/C/Clotting.html
Vascular response - vasoconstriction
Platelet plug formation facilitation of plasma
clotting factors
Fibrin clot formed on platelets thrombin
fibrinogen fibrin
Lysis of clot counter mechanism to clotting to
keep blood in its fluid state

In the vessel vasoconstriction it reduces leakage of

blood and presses the endothelial surfaces together


enhancing wall stickiness . Vasospasm lasts about 20
30 minutes.
At the lysis stage fibrin fibers shorten becoming

denser and stronger which approximates the edge of


the vessel wall sealing the site of injury

Blood Clotting Mechanism


Thrombin

Coagulation
factor
cascade

Fibrin
Fibrinogen

Disseminated Intravascular
Coagulation
Clinical situations that present with:
Arterial hypotension
Acidemia
Hypoxemia
Stasis of capillary blood flow
Release of tissue factor from either endothelial damage

or direct tissue damage in the presence of sepsis is


associated with DIC
Thrombin induced platelet aggregation obstruction
In an attempt to consumed platelets a
thrombocytopenia arises increasing the hemorraging

Disseminated intravascular coagulation or DIC, is a condition in

which blood clots form throughout the body's small blood


vessels. These blood clots can reduce or block blood flow
through the blood vessels, which can damage the body's organs.

In DIC, the increased clotting uses up platelets and clotting

factors in the blood. Platelets stick together to seal small cuts


and breaks on blood vessel walls and stop bleeding. Clotting
factors are proteins needed for normal blood clotting.

With fewer platelets and clotting factors in the blood, serious

bleeding can occur. DIC can cause internal and external


bleeding.

Internal bleeding occurs inside the body. External bleeding

occurs underneath or from the skin or mucosa. The tissue that


lines some organs and body cavities, such as the nose and
mouth.

Causes
Sepsis,
Trauma (neuro)
Obstetric emergencies
Malignancy
Examples of less common causes of DIC are

bites from poisonous snakes (such as


rattlesnakes and other vipers), frostbite, and
burns.

People who have chronic DIC are more likely to have

blood clotting problems than bleeding. Cancer is the


most common cause of chronic DIC. People who have
chronic DIC may need medicines to help prevent
blood clots from forming in their small blood vessels.
Before using any over-the-counter medicines or

products, such as vitamins, supplements, or herbal


remedies, patients should speak to their health care
provider . Some of these products also can affect blood
clotting and bleeding. For example, aspirin and
ibuprofen may thin the blood too much.

Treatment
Focus on addressing the underlying disorder

Monitor vital signs,


Assess and document extent of hemorrhage and

thrombosis
Administer basic hemostatic procedures when
indicated transfusion packed red cells, platelet and
factor replacement
Heparin extensive fibrin deposition - acute only
Activated protein - sepsis with DIC (c0ntraversial)

Treatment should primarily focus on addressing the underlying disorder


DIC can result from several clinical conditions, including sepsis, trauma, obstetric

emergencies, and malignancy. Surgical management is limited to primary treatment of


certain underlying disorders.
In an emergency if the patient is bleeding vitamin K may be administered followed by
approprate supporting treatment for bleeding (haemorrhagic shock) fluids, oxygen
support, transfusion.
Platelet and factor replacement should be directed not at simply correcting laboratory

abnormalities but at addressing clinically relevant bleeding or meeting procedural needs.


Heparin should be provided to those patients who demonstrate extensive fibrin deposition
without evidence of substantial hemorrhage; it is usually reserved for cases of chronic DIC.
Heparin is appropriate to treat the thrombosis with DIC. It also has a limited use in acute
hemorrhagic DIC in a patient with a self-limited condition of acral cyanosis and digital
ischemia.
Administration of activated protein C (APC) has shown benefit in subgroups of patients with

sepsis who have DIC, with consideration given to the anticoagulant effects of this agent.
Recognition of the importance of inflammation in sepsis, coagulation, and DIC is vitally

important in directing the development of novel therapeutic strategies.

Spleen
Four major functions
Hematopoietic

Filtration
Immunological
Storage

Erythropoiesis the formation of


red blood cells. The spleen
makes red blood cells during the
time that the fetus is 9 to 28
weeks old,
Filtration by removing old and
damaged RBCs and the reuse of
iron returning it to the bone
marrow.. It also filtrates bacteria
Immunological response -As
blood passes through the spleen,
not only are old and abnormal
red blood cells removed, but
microorganisms are, as well,
macrophages will take pieces of a
microorganism to the B and T
lymphocytes while inside the
spleen.
Stores RBCs and platelets

Lymph System
Sub division of the circulatory system a

network of organs, lymph nodes, lymph


ducts, and lymph vessels
Returns excess intestinal fluids to the
blood
Lymph nodes make immune cells that help
the body fight infection, filter the lymph
fluid and remove foreign material such as
bacteria and cancer cells.

Sub division of the circulatory system a network of

organs, lymph nodes, lymph ducts, and lymph vessels


that make and move lymph from tissues to the
bloodstream.
Lymph system returns excess intestinal fluid to the
blood important therefore in the prevention of edema
Lymphatic capillaries
Thin-walled vessels with irregular diameter
Larger than blood capillaries but without valves
Primary function is filtration of pathogens and foreign
particles carried by lymph fluid.
Located both superficially and deep

Lymph nodes
Small clumps of lymphatic tissue found in

groups along lymph vessels at various sites


More than 200 lymph nodes throughout the
body
Largest concentration of lymph nodes is in the
abdomen surrounding the GI tract.
Primary function is filtration of pathogens and
foreign particles carried by lymph fluid.
Located both superficially and deep

Liver
Acts as a filter
Produces all the procoagulants essential for

hemostasis and blood coagulation


Stores excess iron
Produces hepcidin, a key regulator of iron
balance

Effects of Ageing
Red Bone marrow and number of stem cells

decreases with ageing


Reserve capacity is reduced
More vulnerability with clotting O2
transport

Laboratory studies
Complete blood count
Red blood cells - Hb, Hct, MCV, MCH,

MCHC, reticulocytes
White blood cells differentials
Platelet count and , Platelet aggregation
test
Blood Typing and Rh Factor (see table
30:10)
Iron metabolism- serum iron, Total ironbinding capacity

White Blood cells - The differential totals the number of each type and

determines if the cells are present in normal proportion to one another,


if one cell type is increased or decreased, or if immature cells are
present. This information is useful in helping to diagnose the specific
cause of an illness, such as:

Infections caused by bacteria, viruses, fungi or parasites


Inflammation
Allergies, asthma
Immune disorders (e.g., autoimmune disorders, immune deficiency)
Leukemia
Myelodysplastic syndrome
Myeloproliferative disorder

Platelets count and Platelet aggregation test The laboratory specialist

will look at how the platelets spread out in the liquid part of the blood
(plasma) and whether they form clumps after a certain chemical or drug
is added. When platelets clump together, the blood sample is more clear.
A machine measures the changes in cloudiness and prints a record of the
results.

Blood typing and cross match

Please revise this table and ensure you know the universal donor and recipient groups and that
if your blood group is O RhD negative, it can be given to anyone. That means your blood is the
only safe option when a patient's blood group is unknown or not immediately available - such
as in emergencies

Red blood cell compatibility


Blood group AB individuals have both A and B antigens on the surface of their

RBCs, and their blood plasma does not contain any antibodies against either A or B
antigen. Therefore, an individual with type AB blood can receive blood from any
group (with AB being preferable), but cannot donate blood to either A or B group.
They are known as universal recipients.
Blood group A individuals have the A antigen on the surface of their RBCs, and
blood serum containing IgM antibodies against the B antigen. Therefore, a group A
individual can receive blood only from individuals of groups A or O (with A being
preferable), and can donate blood to individuals with type A or AB.
Blood group B individuals have the B antigen on the surface of their RBCs, and
blood serum containing IgM antibodies against the A antigen. Therefore, a group B
individual can receive blood only from individuals of groups B or O (with B being
preferable), and can donate blood to individuals with type B or AB.
Blood group O (or blood group zero in some countries) individuals do not have
either A or B antigens on the surface of their RBCs, and their blood serum contains
IgM anti-A and anti-B antibodies against the A and B blood group antigens.
Therefore, a group O individual can receive blood only from a group O individual,
but can donate blood to individuals of any ABO blood group (i.e., A, B, O or AB). If
a patient in a hospital situation were to need a blood transfusion in an emergency,
and if the time taken to process the recipient's blood would cause a detrimental
delay, O Negative blood can be issued. They are known as universal donors.

Blood transfusion
Compatibility of patient and donor is essential
Human related errors
Improper labelling
Poor hand off between nurses
Person transporting blood
Method used to complete blood requisition
Complications disease
Storage continual destruction release of vit K
Human error may led to the administration of incompatible transfusion can
occur every step of the process.
In order to prevent disease transmission blood is stored at 1 6 for 35 42

days in controlled conditions.


Conditions are controlled as it is a living tissue therefore anticoagulants and
preservatives are used to maintain the shelf life. Caution is needed when
transfusing older blood

Blood transfusion

AB can receive any blood - universal recipient


O can only receive O however is universal donor
A+ or can receive A
B+ or can receive B
AB AB+ -, A+ or B+ or O+ Check the doctor has the patients consent signed
Ensure there is venous access and start N Saline 0.9%
Initiation Obtain blood product from blood bank
blood must be given within 30 minutes after release
from laboratory (INS 2011)
Check bag for signs of contamination

Blood Transfusion
Check the blood unit is correctly labelled
Check against the patients identification
Two nurses should verify the correct unit

and correct patient before commencing


transfusion
Please review the clinical nursing skills book

Blood transfusion complications


Most Common occurrence - Febrile non hemolytic
reaction temperature, flushing chills and headache

30 mins after initiation to 6 hours post transfusion.


Nursing Action?
Acute haemolytic reaction ABO Rh
incompatibility - severe pain in kidney area and
chest ,temperature significantly 41c or 105f Heart
rate increased, chills, lower back pain dyspnoea,

bronchospasm, anxiety .hypotension Extreme cases vascular collapse possible DIC. Nursing Action?
(Perry Potter & Ostendorf 2010)

Raise in temperature with a fever greater than 1degree centigrade

above baseline seen frequently in patients with


immunosuppressed patients stop the transfusion and
administer prescribed antipyretics 4 hourly temperature.
Prevention - use leukocyte reduced products for future
transfusions

Acute haemolytic reaction ABO Rh incompatibility causes

intravascular destruction of transfused RBCs as antibodies in


recipients plasma stoantigens on donor RB.

Most Common occurrence - Febrile non hemolytic reaction

temperature, flushing chills and headache 30 mins after initiation


to 6 hours post transfusion STOP transfusion immediately.
Acute haemolytic reaction ABO Rh incompatibility - severe pain
in kidney area and chest ,temperature significantly 41c or 105f
Heart rate increased, chills, lower back pain dyspnea,
bronchospasm, anxiety .hypotension Extreme cases - vascular
collapse possible DIC STOP transfusion immediately ,remove
blood product and tubing, maintain IV access notify health care
provider, start 15 minute VS, administer ordered therapy (Perry
Potter & Ostendorf 2010)

Drugs affecting hematological function!

Anti seizure agents Phenytoin Carbamazepine - anemia


Anti hypertensive Methyldopa hemolytic agent
Isoniazide (INH) - TB treatment - Neutropenia
Anti-microbals Chloramphenicol anemia neutropenia,
thrombocytopenia
Corticosteroids Dexamethasone bone marrow suppression
Diuretics loop (Furosemide) and thiazide (Bendroflumethiazide)
Interfere with platelet formation
Histamines ( ranitadine) - Interfere with platelet formation
NSAID anemia, leukopenia, inhibit platelet aggregation
Salicylates interfere with platelet formation
Aggregation - The clumping together of platelets in the blood.
Platelet aggregation is part of the sequence of events leading to the
formation of a thrombus (clot).

Biopsies
Bone Marrow extraction

Other biopsies /tests


Lymph node biopsy

Molecular Cytogenetics

(study of the structure and


function of the cell ),and
Gene Analysis
Why would they do gene

analysis?

Bone marrow extraction undertaken by going in through hip bone or sternum (less common) to obtain a specimen of bone
marrow.
Potential problems bone pain, infection, swelling Therefore a persistent fever, worsening pain or discomfort, swelling at
the procedure site
Or increasing redness or drainage at the procedure site should be reported immediately
Lymph biopsy - Sentinel node biopsy is a surgical procedure that doctors use to stage (determine the extent of spread of)
certain types of cancer in patients who have been recently diagnosed with cancer. Sentinel node biopsy is most commonly
associated with staging breast cancer; however, the procedure is also commonly used to stage malignant melanoma (a type
of skin cancer).
Methods
Fine-needle aspiration biopsy. The doctor inserts a thin needle into a lymph node and removes a sample of cells.

Core needle biopsy. Your doctor inserts a needle with a special tip and removes a sample of tissue about the size of a grain of rice.

Open (surgical) biopsy. The doctor will make a small cut in the skin and remove a lymph node. If more than one lymph
node is taken, the biopsy is called a lymph node dissection. Open biopsy and lymph node dissection let your doctor take a
bigger sample than a needle biopsy. An open biopsy of a lymph node is done by a surgeon. For a lymph node near the
surface of the skin, the biopsy site is numbed with local anesthetic. For a lymph node deeper in the body or for lymph node
dissection, you may have general anesthesia.

or 1 to 2 days after an open lymph node biopsy, you may feel tired. You may also have a mild sore throat if a tube was used to help you
breathe during the biopsy. Using throat lozenges and gargling with warm salt water may help with the sore throat.

After an open biopsy, the area may feel tender, firm, swollen, and bruised. Fluid may collect near the biopsy site. Fluid may
also leak from the biopsy site. You can use an ice pack or take an over-the-counter pain medicine (not aspirin) to help
relieve swelling and mild pain. The tenderness should go away in about a week, and the bruising usually fades within 2
weeks. But the firmness and swelling may last for 6 to 8 weeks. Patients should not do any heavy lifting or other activities
that stretch or pull the muscles around the area.
Your pain lasts longer than a week.
You have redness, a lot of swelling, bleeding, or pus from the biopsy site.
You have a fever.
Report any fluid buildup in the area where the lymph node was taken out. This occurs most often when removing the
lymph nodes that run in a line from under the arm to the collarbone (axillary lymph nodes). This can happen immediately
after surgery or even months or years later. Most people who have a lymph node biopsy do not have a problem with fluid
buildup.
Numbness in the skin near the biopsy site. This may be caused by nerve damage.

Nursing Assessment & Review


Past health history
Medicines
Surgery and other treatments What specifically would we

be looking for here?


Common questions include:
dietary intake, nausea and vomiting, unusual bleeding or
bruising, weight changes
Lumps or swelling
Elimination patterns blood in stools , urine, changes in
bowel or urinary habits
Lack of energy or excessive tiredness
Numbness or tingling

You need to ask about skin anomalies excessive purpura and

bruising, nose bleeds, pruritus Hodgkin's lymphoma,


cutaneous lymphoma, leukemia. Leg ulcers especially on ankles
could indicate sickle cell disease
Blurred vision anemia, thrombocytopenia
Nose bleeding epistaxis, may occur with low platelet counts
Smooth red beefy tongue pernicious anemia
Enlarged lymph nodes - infection, leukemia Hodgkins
lymphoma
Heart rate and rhythm raise or palpitations in anemia to increase
output - Hypotension
Low O2 saturation
Abdomen Hepatomegaly leukemia, cirrhosis , Splenomegaly
anemia thrombocytopenia, leukemia, lymphoma, malaria,
mononucleosis, distended abdomen lymphedema for example
may present as an abdominal mass or bowel obstruction.
CNS - numbness and tingling of extremities

Assessment - Physical
Lymph node assessment by light palpation
Palpation of liver and spleen not normally

detectable by percussion or palpation


Skin assessment abnormal bruising, spider
nevus, nodular lesions, petechiae can
indicate bleeding disorders

Use pads of finger and lightly palpate lymph nodes in adults

these would not normally be palpable A node which is tender


hard fixed or enlarged is an abnormal finding the tenderness
suggesting inflammation and hard fixed nodes suggest
malignancy This finding however needs further investigation
and should be considered in light of the whole examination and
history. If the node is palpable should be samll 0.5 1 cm. Start
with the head and work down the body

Liver - degree of liver enlargement measured by number of

finger breaths it extends below rib boarder.

Skin RBC disorders pale or pasty skin tones, erthrocytosis often

produce small vessel occlusions purple mottled appearance of


skin face nose fingers and toe s, abnormal bruising, spider nevus,
nodular lesions, petechiae can indicate bleeding disorders
Skin and mucosal bleeding indicative of a platelet disorder,
spontaneous bleeding into joints or muscles a coagulation factor
disorder
Clubbing of fingers seen with chronic anemia e.g. patients with
sickle cell disease

Acute idiopathic thrombocytopenic


purpura
Acute idiopathic
thrombocytopenic
purpura commonly
manifests with
purpuric lesions of
this kind, although
they may often be
widespread by the
time medical
attention is sought.
From Forbes CD,
Jackson WF: Color
atlas and text of
clinical medicine, ed
3, London, 2003,
Mosby

Severe bruising in a patient

following a fall
Called ecchymosis a purplish
patch caused by extravasation
of blood into the skin,
differing from petechiae only
in size
Medical term for a
subcutaneous purpura larger
than 1 centimeter or a
hematoma

References
Ignatavicius D. D & Workman M.L (2013) Medical Surgical

Nursing Patient Centred Collaborative Care, 7th Edition,


USA, Elsevier.

Lewis SL, Dirksen SR, Heitkemper M, Bucher L, Camera

IM (2009) Medical Surgical Nursing Eighth edition, USA,


Elsevier.

Perry AG, Potter PA & Ostendorf WR (2010) Clinical

Nursing Skills and Techniques 8th Edition St Louis Missouri,


Elsevier.

Janice Summers

Objectives
At the end of the teaching session the student will be

enabled to:
Discuss the different types of nutritional linked

anemias including cause, diagnosis and treatment


Illustrate knowledge and understanding of the
nursing management and collaborative care
interventions for anemia

What is Anemia?
A deficiency in the
Number of erythrocytes
(red blood cells [RBCs])
Quantity of hemoglobin

Volume of packed RBCs (hematocrit)

Classifications:
Morphologic

Cellular characteristics

Descriptive, objective laboratory information

Etiologic

Underlying cause

Not a specific disease rather a manifestation of a

pathologic process
Identified through history and physical exam and
classified by laboratory review
Can result from a primary hematological problem or
develop as a secondary consequence of a disease
process
Diverse causes such as
Blood loss
Impaired production of erythrocytes
Increased destruction of erythrocytes

Mild states of anemia (Hb 10 to 14 g/dL [100 to 140

g/L]) may exist without causing symptoms. If


symptoms develop, it is because the patient has an
underlying disease or is experiencing a compensatory
response to heavy exercise. Symptoms include
palpitations, dyspnea, and mild fatigue.
In cases of moderate anemia (Hb 6 to 10 g/dL [60 to
100 g/L]), cardiopulmonary symptoms are increased
and the patient may experience them while resting, as
well as with activity.
The patient with severe anemia (Hb less than 6 g/dL
[60 g/L]) has many clinical manifestations involving
multiple body systems.

Caused by the
bodys
response to
tissue hypoxia
Hemoglobin
(Hb) levels
are used to
determine the
severity of
anemia.

Table 31.2
Decreased RBC

Blood loss

Decreased hemoglobin synthesis


Iron deficiency Thalassemia's, Siderblastic
anemia

Acute
Due to trauma and blood vessel rupture

Defective DNA Synthesis


Colbalimin (B12) deficiency
Folic Acid

Chronic
Gastritis
Menstrual flow
Hemorrhoids

Decreased Number of RBC Precursors


Aplastic Anemia
Anemia (related to leukemias)
Chronic Diseases or Disorders
Medications (Chemotherapy

Increased RBC Destruction


Hemolytic Anemias
Abnormal HB due to sickle cell anemia
Enzyme deficiency
Membrane abnormalities
Extrinsic
Physical trauma eg heart valves
Antibodies
Infectious Agents

Clinical Presentation
Skin Pallor
Hemoglobin
Blood flow to the skin

Jaundice
Concentration of serum bilirubin

Pruritus
Serum and skin bile salt concentrations

Cardiopulmonary Manifestations
Cardiac output maintained by increasing the heart rate

and stroke volume

Clinical manifestations are caused by the bodys response to


tissue hypoxia. In addition to the skin, the sclerae of the eyes
and mucous membranes should be evaluated for jaundice
because they reflect the integumentary changes more
accurately, especially in a dark-skinned individual.
Additional attempts by the heart and lungs to provide
adequate O2 to the tissues increased heart rate results in an
increased cardiac output
In extreme cases, or when concomitant heart disease is
present, angina pectoris and myocardial infarction (MI) may
occur if myocardial O2 needs cannot be met.
Heart failure (HF), cardiomegaly, pulmonary and systemic
congestion, ascites, and peripheral edema may develop if the
heart is overworked for an extended period of time.

Gerontologic Considerations
Healthy older men modest decrease in Hb because of

decreased production of androgens


In women minor decrease in Hb (Marks & Gladder

2009)
40% admitted to hospital and 47% in long term care

facilities found to have anemia (Gaskell,Derry & Moore


et all 2008)
Signs and symptoms can go unrecognized put down to

normal ageing

Androgen- androgenic hormone or testoid, is the generic term for any

natural or synthetic compound, usually a steroid hormone, that


stimulates or controls the development and maintenance of male
characteristics. Most common one is testosterone.

Signs and symptoms can go unrecognized but include pallor,

confusion, ataxia, fatigue worsening angina, and heart failure, put


down to normal part of ageing or be overlooked because of another
health problem

Gaskell,Derry & Moore et all (2008)


40% admitted to hospital and 47% in long term care facilities found to
have anemia Forty-five studies contributed data. Thirty-four studies (n
= 85,409) used WHO criteria to define anaemia. The weighted mean
prevalence was 17% (3-50%) overall, and 12% (3-25%) in studies based
in the community (27, n = 69,975), 47% (31-50%) in nursing homes (3,
n = 1481), and 40% (40-72%) in hospital admissions (4, n = 13,953).
Anaemia prevalence increased with age, was slightly higher in men
than women, and was higher in black people than white. Most
individuals classified as anaemic using WHO criteria were only mildly
anaemic.

Nursing Assessment Subjective Data

Important health information


Past health history
Medications
Surgery or other treatments
Dietary history
So what would be considered important health information? Recent
blood loss, underlying disease problems involving kidney, liver,
endocrine system , Crohns disease, smoking, exposure to toxins , recent
travel suggesting exposure to infection angina history
Family history
Elimination patterns hematuria, diarrhoea, tarry black stool
Medications iron supplements, vitimins, aspirin, anticoagulants,

phenobarbitol, quinine, phenytoin

Nursing Management Goals


Assume normal activities of daily

living.
Maintain adequate nutrition.
Develop no complications related to
anemia.

Nursing Implementation
Blood or blood product transfusions
Drug therapy
Volume replacement
Dietary and lifestyle changes
Correcting the cause of the anemia is the

ultimate goal of therapy.


Examples of drug therapy include
erythropoietin and vitamin supplements.
Dietary and lifestyle changes

Thalassemia
Inherited blood disorder
Makes an abnormal form of hemaglobin
Occurs when there is a defect in the gene which makes

alpha and beta globulin


Thalssemia major severe anemia during first year of
life
Bone deformities, fatigue, growth failure, shortness of

breath, jaundice skin

Thalassemia minor small red blood cells


no symptoms

Thalassemia is a blood disorder passed down through families

(inherited) in which the body makes an abnormal form of hemoglobin,


the protein in red blood cells that carries oxygen. The disorder results in
excessive destruction of red blood cells, which leads to anemia.
There are two main types of thalassemia:
Alpha thalassemia occurs when a gene or genes related to the alpha

globin protein are missing or changed (mutated).


Beta thalassemia occurs when similar gene defects affect production of
the beta globin protein.
Alpha thalassemias occur most commonly in persons from southeast
Asia, the Middle East, China, and in those of African descent.
Beta thalassemias occur in persons of Mediterranean origin, and to a
lesser extent, Chinese, other Asians, and African Americans.
You must inherit the defective gene from both parents to develop
thalassemia major.
Thalassemia minor occurs if you receive the defective gene from only one
parent. Persons with this form of the disorder are carriers of the disease
and usually do not have symptoms.

Signs and symptoms


Swollen enlarged spleen
Red blood cells small and abnormally

shaped
CBC anemia
Hemoglobin electrophoresis abnormal
form of hemoglobin
Mutational analysis alpha thalessemia

Treatment
Regular blood transfusions or Iron

supplements
Patients should not receive both!
Chelation therapy to remove excess
iron from blood
Bone marrow transplant may treat
disease especially children

Sideroblastic anemia
Bone marrow produces ringed siderblasts
Causes:
Genetic disorder
Defect in an X-linked recessive gene.
Acquired sideroblastic anemia is the result of prolonged
exposure to certain toxic substances ,drugs, disease
processes
Body has iron available but can not convert to

hemaglobin
http://www.rarediseases.org/rare-diseaseinformation/rare-diseases/byID/351/viewFullReport

It may be caused either by a genetic disorder or indirectly as part of


myelodysplastic syndrome, which can evolve into hematological
malignancies (especially acute myelogenous leukemia). In
sideroblastic anemia, the body has iron available but cannot
incorporate it into hemoglobin.

Acquired sideroblastic anemia is the result of prolonged exposure to


certain toxic substances or drugs, or an unpleasant effect of other
disorders such as immune disorders, granulomatous disease,
tumors, or metabolic disorders.
The signs and symptoms can range from mild to severe, and

include fatigue, breathing difficulties, and weakness. Enlargement


of the liver or spleen may also occur. In severe cases, the increased
levels of iron in the blood may lead to heart disease, liver damage,
and kidney failure. The sideroblastic anemias are characterized by
fatigue, breathing difficulties, and feelings of weakness. On
exertion, persons with this disorder may feel angina-like chest
pains.

Siderblastic Anemia Treatment


If the disorder is acquired, the responsible agent must be
identified and avoided.
Vitamin B6 (pyridoxine) therapy may be helpful in some

cases.

Excess iron removed from the from the body


To remove excess iron from the body of someone with

sideroblastic anemia, the drug desferrioxamine


(Desferal) is infused under the skin (subcutaneously) or
injected into a muscle (intramuscular), often with good
results. Desferrioxamine binds excess iron and promotes
its excretion from the body.

Iron Deficiency
Found in up to 30% of worlds population
In 2002, iron deficiency anaemia (IDA) was considered
to be among the most important contributing factors
to the global burden of disease (WHO 2002)

In the US occurs in 5 -10% of over 45s


Other susceptible groups include:
Babies and children

The poor
Women in their productive years (;Brittenham

2009)

Causes

Iron deficiency anemia occurs because of:


Poor diet
Malabsorption problems
Blood loss
Hemolysis (separation of the hemoglobin from the red cells)

As iron absorption occurs in the duodenum, malabsorption syndromes may

involve disease of the duodenum in which the absorption surface is altered or


destroyed.
Major sources of chronic blood loss involve the GI and genitourinary (GU)
systems. Common causes of GI blood loss are peptic ulcer, gastritis, esophagitis,
diverticuli, hemorrhoids, and neoplasia. GU blood loss occurs primarily
through menstrual bleeding.
In addition to anemia of chronic renal failure, dialysis treatment may induce
iron-deficiency anemia as the result of blood lost in the dialysis equipment and
frequent blood sampling.

Presentation
Glossitis - Inflammation and burning

of the tongue (Smooth red tongue)


Chelitis -Inflammation of the lips
Tiredness and fatigue
Headache
Paresthesis

Nursing Management
Recognising at risk groups premenopausal and

pregnant women, older adults, poor socioeconomic


background
Teaching re diet - Iron-rich foods include dark green

vegetables, meat, apricots, prunes, raisins and ironfortified bread


Consider referral to a dietician if diet is thought to be

the cause.

Drug Treatment
Ferrous sulphate 150 -200 mg two to three times
per day.
Common side-effects usually reduce with time and
include:
Constipation, Black stools, Diarrhoea,

Heartburn, Nausea, Abdominal/epigastric pain


Factors to consider - Best absorbed as ferrous
sulphate in an acidic environment
Liquid iron should be diluted and ingested
through a straw.
Iron should be taken about an hour before
meals

Iron is absorbed best from the duodenum and proximal jejunum.

Therefore enteric-coated or sustained-release capsules, which release


iron farther down the GI tract, are counterproductive and expensive.
The daily dosage should provide 150 to 200 mg of elemental iron. This
can be ingested in three or four daily doses, with each tablet or capsule
of the iron preparation containing between 50 and 100 mg of iron.
Explain common side-effects and how to reduce them
Advise about storage of iron supplements out of the reach of children.
Ferrous fumarate or ferrous gluconate are alternatives if ferrous sulphate
isn't tolerated.
When the underlying cause is known, this should be managed
appropriately.
Iron is best absorbed as ferrous sulfate (Fe2+) in an acidic environment.
For this reason and to avoid binding the iron with food, Iron should be
taken about an hour before meals , when the duodenal mucosa is most
acidic. Taking iron with vitamin C (ascorbic acid) or orange juice, which
contains ascorbic acid, also enhances iron absorption.
Side effect example: Many individuals who need supplemental iron
cannot tolerate ferrous sulfate because of the effects of the sulfate base.
However, ferrous gluconate may be an acceptable substitute.

Parenteral iron
Indicated for malabsorption, oral iron intolerance, need

for iron beyond normal limits, poor patient compliance


Can be given IM or IV
IM may stain skin.
An iron-dextran complex (INFeD) contains 50 mg/mL of
elemental iron in 2 mL. Sodium ferrous gluconate and iron
sucrose are alternatives, and may provide less risk of lifethreatening anaphylaxis.
Because IM iron solutions may stain the skin, separate
needles should be used for withdrawing the solution and
for injecting the medication. A Z-track injection technique
should be used.

Laboratory findings
Hb, Hct, MCV, MCH, MCHC, reticulocytes,

serum iron, TIBC, bilirubin, platelets


Stool guaiac test
Endoscopy
Colonoscopy
Bone marrow biopsy

The MCV reflects the size of red blood cells


Hemoglobin amount per red blood cell (MCH)
The amount of hemoglobin relative to the size of the cell (hemoglobin
concentration) per red blood cell (MCHC)
Hemocrit is the percentage of blood volume containing RBC
TIBC -Total iron binding capacity (TIBC) is a blood test to see if you may have too much
or too little iron in the blood. Iron moves through the blood attached to a protein called
transferrin. It tests how well that protein can carry iron in the blood.
The MCV reflects the size of red blood cells. The MCH and MCHC reflect the
hemoglobin content of red blood cells. These RBC measures are used to diagnose
types of anemia.
Anemias are defined based on cell size (MCV) and amount of Hgb (MCH).

MCV less than lower limit of normal: microcytic anemia


MCV within normal range: normocytic anemia
MCV greater than upper limit of normal: macrocytic anemia
MCH less than lower limit of normal: hypochromic anemia
MCH within normal range: normochromic anemia
MCH greater than upper limit of normal: hyperchromic anemia
Normal Results
MCV: 80 - 100 femtoliter
MCH: 27 - 31 picograms/cell
MCHC: 32 - 36 grams/deciliter

Megaloblastic Anemias
Group of disorders caused by impaired DNA
synthesis
Characterized by the presence of large RBCs
(megaloblasts)
Easily destroyed because of fragile cell membrane
Majority result from deficiency in
Cobalamin (vitamin B12)
Folic acid
Drug-induced suppression of DNA synthesis
Inborn errors
Erythroleukemia

Pernicious Anemia
Lack of Intrinsic factor (IF) a protein secreted by the

parietal cells of the gastric mucosa


Required for cobalamin absorption in the small
intestine.
Causes

Pernicious anemia
Insidious onset
Gastrectomy
Nutritional deficiencies
Chronic alcoholism
Hereditary enzymatic defects

The most common cause is pernicious anemia, a disease in which

the gastric mucosa is not secreting IF because antibodies are


being directed against the gastric parietal cells and/or IF itself.
Pernicious anemia begins in middle age or later (usually after 40),
with 60 being the most common age.
Pernicious anemia occurs frequently in persons of Northern
European ancestry (particularly Scandinavians) and African
Americans. In African Americans, the disease tends to begin early,
occurs with higher frequency in women, and is often severe.
Examples not related to ancestry and race : GI surgery

(gastrectomy, gastric bypass, small bowel resection involving the


ileum) and chronic diseases of GI tract (Crohns disease, ileitis,
celiac disease, diverticuli of the small intestine, chronic atrophic
gastritis)
Absence of IF can be due to gastric mucosal atrophy or to
autoimmune destruction of parietal cells. This results in a decrease
in hydrochloric acid secretion by the stomach. An acidic
environment in the stomach is required for the secretion of IF.
Long-term users of H2-histamine receptor blockers

Presentation

Sore tongue (Red, beefy tongue)


Anorexia
Nausea
Vomiting
Abdominal pain
Neuromuscular manifestations
Weakness
Paresthesias of the feet and hands
Vibratory and position senses
Ataxia
Muscle weakness
Impaired thought processes
Understand that these are important in the assessment ofn
the patient presenting with pernicious anemia

Laboratory
RBCs appear large with abnormal shapes.
Serum cobalamin levels are decreased.
Normal serum folate levels and decreased cobalamin levels

suggest megaloblastic anemia due to cobalamin deficiency.


Schilling test
Serum methylmalonic acid
Serum homocysteine
Because the potential for gastric cancer is increased in patients
with pernicious anemia, gastroscopy and biopsy of the gastric
mucosa may also be done.
Schilling test: After radioactive cobalamin is administered to the
patient, the amount of cobalamin excreted in the urine is
measured. An individual who cannot absorb cobalamin excretes
only a small amount of this radioactive form.

Treatment
Parenteral or intranasal administration of cobalamin
The dosage and frequency of cobalamin administration may vary. A

typical treatment schedule consists of 1000 mg of cobalamin IM daily


for 2 weeks and then weekly until the hematocrit is normal, then
monthly for life.
Increase in dietary cobalamin does not correct the anemia.
Still important to emphasize adequate dietary intake
The dosage and frequency of cobalamin administration may vary. A
typical treatment schedule consists of 1000 mg of cobalamin IM daily
for 2 weeks and then weekly until the hematocrit is normal, then
monthly for life.
High-dose oral cobalamin and sublingual cobalamin are also available
for those in whom GI absorption is intact.
As long as supplemental cobalamin is used, the anemia can be
reversed. However, if the person has had long-standing neuromuscular
complications, they may not be reversible.

Specific Nursing management


Familial disposition
Early detection and treatment can lead to

reversal of symptoms.
Ensure that injuries are not sustained
because of the patients diminished
sensation to heat and pain.
Ensure patient compliance with treatment.
Frequently evaluate patient for gastric
carcinoma.

Folic Acid Deficiency


Also a cause of megaloblastic anemia

Folic acid is required for DNA

synthesis.
RBC formation and maturation

Common causes
Dietary deficiency
Malabsorption syndromes
Drugs
Increased requirement
Alcohol abuse and anorexia
Loss during hemodialysis
Drugs that interfere with absorption/use of

folic acid include methotrexate and antiseizure


drugs (e.g., phenobarbital, diphenylhydantoin
[Dilantin]).

Clinical Manifestations and Treatment


Similar to those of cobalamin deficiency.
Insidious onset
Absence of neurologic problems
Treated by replacement therapy
Encourage patient to eat foods with large

amounts of folic acid.


Replacement therapy: The usual dose is 1
mg per day by mouth. In malabsorption
states, up to 5 mg per day may be required.

GI disturbances include dyspepsia and a smooth,


beefy red tongue.
During diagnostic studies, the serum folate level is

low (normal is 3 to 25 mg/mL [7 to 57 mol/L]), the


serum cobalamin level is normal, and the gastric
analysis is positive for hydrochloric acid.
Replacement therapy: The usual dose is 1 mg per day
by mouth. In malabsorption states, up to 5 mg per
day may be required.
See Table 31-5 for foods high in folic acid.

Diagnostic studies
During diagnostic studies, the serum folate

level is low (normal is 3 to 25 mg/mL [7 to


57 mol/L]), the serum cobalamin level is
normal, and the gastric analysis is positive
for hydrochloric acid.

References
Gaskell H, Derry S, Andrew Moore R, McQuay HJ

(2008) Prevalence of anaemia in older persons:


systematic review BMC Geriatr. 14;8:1
Marks and Gladder (2009)
World Health Organization (2002) The World Health
Report 2002: Reducing risks, promoting healthy life.
Geneva, World Health Organization.
WHO (2008) Worldwide prevalence of anaemia 1993
2005:WHO Global database on anaemia, Geneva.
WHO Press

Objectives
At end of the session the student will be enabled to:
Describe what sickle cell anemia is with reference to

the pathophysiology
Recognise and appropriately treat patients in sickle
cell crisis using appropriate nursing management
Discuss with understanding the importance of
education to enable individuals with sickle cell anemia
to avoid crisis situations

Sickle cell disease is an inherited condition that causes

red blood cells to become sickle shaped when they lose


water. This leads to a high risk of the blood vessels
becoming blocked.
Types of SCD
Sickle cell anemia

Most severe

Homozygous for hemoglobin S (HbSS)


Sickle cell thalassemia
Sickle cell HbC disease
Sickle cell trait (HbAS)

Sickle cell disease is an inherited condition that causes red blood


cells to become sickle shaped when they lose water. This leads to a
high risk of the blood vessels becoming blocked. Such blockages can
cause pain, stroke and damage to organs.
Sickle cell disease (SCD) is a chronic hemoglobinopathy of clinical
relevance because of its significant morbidity and mortality,
particularly in people of African and Mediterranean ancestry
Carriers of the sickle cell trait (HbAS, heterozygotes) have some
resistance to the often-fatal malaria caused by Plasmodium
falciparum.
Ever since carriers of the mutated gene survived the deadly

malaria epidemics that were thought to occur thousands of years


ago, the gene has continued to survive in malaria-endemic areas.
However, in areas such as the US, where malaria is not a problem,
the trait no longer provides a survival advantage and instead poses
the threat of sickle cell disease if the carrier's children inherit the
sickle cell gene from both parents (ie, HbSS)

Sickled RBCs become rigid and take on an elongated, crescent shape. Sickled cells
cannot easily pass through capillaries or other small vessels and can cause vascular

occlusion, leading to acute or chronic tissue injury. The resulting hemostasis promotes a
self-perpetuating cycle of local hypoxia, deoxygenation of more erythrocytes, and more
sickling. Circulating sickled cells are hemolyzed by the spleen, leading to anemia.

When the sickle hemoglobin molecule loses its oxygen, it forms rigid rods called
polymers that change the red blood cells into a sickle or crescent shape.
These sickle-shaped cells stick to the walls and cannot squeeze through the capillaries.
Blood flow through tiny blood vessels becomes slowed or stopped in many parts of the
body. This deprives tissues and organs of oxygen.
When this blood flow slows or stops suddenly in a certain part of the body, the decrease
in oxygen (hypoxia) can cause severe pain (the sickle cell crisis). Over time, it leads to
gradual destruction in organs and tissues throughout the body.
The higher the concentration of sickle hemoglobin and the more acidic the
environment, the faster the sickle cell process.
When blood cells dry out (dehydrates), the density of hemoglobin S within the cell
increases, thereby speeding the sickling process.
Sickle cells also have a shorter life span (10 - 20 days) than that of normal red blood cells
(90 - 120 days). Every day the body produces new red blood cells to replace old ones, but

sickle cells become destroyed so fast that the body cannot keep up. The red blood cell
count drops, which results in anemia. This gives sickle cell disease its more common
name, sickle cell anemia. http://adam.about.net/reports/Sickle-cell-disease.htm

Clinical Manifestations
Sickling episodes
Sickle cell crisis
Severe painful acute exacerbation of

sickling causes a vaso-occlusive crisis.

Symptoms may include


Pain and swelling
Pallor of mucous membranes
Jaundice

Typical patient is asymptomatic, except during sickling episodes.

Sickling episodes are most commonly triggered by low oxygen tension


in the blood.
Infection is the most common precipitating factor. Other events that
can trigger or sustain a sickling episode include dehydration, increased
hydrogen ion concentration (acidosis), increased plasma osmolality,
decreased plasma volume, and low body temperature.
Shock is a possible life-threatening consequence of sickle cell crisis
caused by severe oxygen depletion of the tissues and a reduction in
circulating fluid volume. Sickle cell crisis can begin suddenly and can
persist for days to weeks.
Sickling episodes can affect any area of the body or several sites
simultaneously, with the back, chest, extremities, and abdomen being
most commonly affected. Pain severity can range from trivial to
excruciating.
Pain episodes are often accompanied by objective clinical signs such as
fever, swelling, tenderness, tachypnea, hypertension, nausea, and
vomiting.
If experiencing nausea, vomiting, and diarrhea; losing a lot of fluid;
and unable to drink and keep it down, the person with sickle cell
disease is in danger of becoming dehydrated. This is a serious concern
with sickle cell disease. Which will require IV fluids to replace the lost
fluids.

In sickle cell disease, the hemoglobin clumps together, which causes red blood cells to become stiff and develop a
C-shaped (sickle) form. These sickled red blood cells can block blood vessels, reducing blood flow in many parts
of the body. This process results in tissue and organ damage.

In the respiratory system - Acute chest syndrome, which is triggered by infection or blockage of blood vessels in
the lungs, is also a common and serious occurrence. Infection. Infection from viruses or small atypical organisms
(Chlamydia and Mycoplasma) is the most common cause of the oxygen deprivation that leads to acute chest
syndrome. About 30% of patients with sickle cell disease have pulmonary hypertension. Gladwin MT, Vichinsky
E. (2008) Pulmonary complications of sickle cell disease. N Engl J Med ;359(21):2254-65.

Heart - Over time, pulmonary hypertension may cause a condition called cor pulmonale, in which the right side
of the heart increases in size. In some cases, this enlargement can lead to heart failure.

When the rigid erythrocytes jam in the arterial and venous sinusoids of skeletal tissue, the resultant effect is
intravascular thrombosis, which leads to infarction of bone and bone marrow. Repeated episodes of these crises
eventually lead to irreversible bone infarcts and osteonecrosis, especially in weight-bearing areas. Patients with
sickle cell disease can have a variety of growth defects due to the abnormal maturation of bone. Growth defects
are often seen in sickle cell disease: bone shortening (premature epiphyseal fusion)

Spleen Damage - The spleen of most adults with sickle cell anemia is nonfunctional due to recurrent episodes of
oxygen deprivation that eventually destroy it. Injury to spleen increases the risk for serious infection. Acute
splenic sequestration crisis (sudden spleen enlargement) can occur when the spleen suddenly becomes enlarged
from trapped blood.

Leg Sores and Ulcers - Leg sores and ulcers occur in up to 10% of sickle cell patients and usually affect patients
older than 10 years.

Eye Problems - Sickle cell disease can damage blood vessels in the eye and cause scarring and detachment of the
retina, which can lead to blindness.

Complications
Gradual involvement of all body

systems
Usually fatal by middle age from renal
and pulmonary failure
Prone to infection
Pneumonia, most common infection
Acute chest syndrome

Organs that are most commonly affected are the

spleen, lungs, kidneys, and brain.


One reason that patients are susceptible to infection is
failure of the spleen to phagocytize foreign substances,
as it becomes infarcted and dysfunctional (usually by 2
to 4 years of age) from the sickled red cells.
Acute chest syndrome is characterized by fever, chest
pain, cough, pulmonary infiltrates, and dyspnea.
Pulmonary infarctions may cause pulmonary
hypertension, MI, HF, and ultimately cor pulmonale.

Diagnostic studies
Peripheral blood smear
Sickling test
Electrophoresis of hemoglobin
Skeletal x-rays
Magnetic resonance imaging (MRI)

Peripheral blood smear looking at size and shape of cells


Sickling test - This test detects if a red blood cell inappropriately changes into

a sickle shape (crescent shape) after a blood sample is mixed with a chemical
that will reduce the amount of oxygen it carries. This test is used to screen for
an abnormal type of hemoglobin called Hemoglobin S in blood.
Electrophoresis of hemoglobin -Hemoglobin electrophoresis is a test that
measures the different types of the oxygen-carrying protein (hemoglobin) in
the blood. HbS is an abnormal form of hemoglobin associated with sickle cell
anemia.
Skeletal x-rays -For imaging the skeletal manifestations of sickle cell disease,

MRI is the best method for detecting early signs of osteonecrosis, followed by
nuclear imaging. Radiography is excellent for identificatin of complications of
bone infarction. The skeletal manifestations of sickle cell disease are the result
of changes in bone and bone marrow caused by the chronic tissue hypoxia
that is exacerbated by episodic occlusion of the microcirculation by the
abnormal sickle cells. The main processes that lead to bone and joint
destruction in sickle cell disease are infarction of bone and bone marrow,
compensatory bone marrow hyperplasia, secondary osteomyelitis, and
secondary growth defects.

Magnetic resonance imaging (MRI) -

Emergency management
O2 for hypoxia and to control sickling
Pain management

Acute chest syndrome


Antibiotics

O2 therapy
Fluid therapy
Transfusions, if needed

In a crisis O2 for hypoxia and to control sickling as respiratory


failure is the most common cause of death
the nurse should therefore be aware of changes in respiratory
status.
Fluids and electrolytes are administered to reduce blood
viscosity and maintain renal function, more especially if he
patient is severely dehydrated.
During an acute crisis, optimal pain control usually includes
large doses of continuous (rather than as-needed) opioid
analgesics along with breakthrough analgesia, often in the
form of patient-controlled analgesia. Morphine and
hydromorphone are the drugs of choice.
Rest may be instituted to reduce metabolic requirements

and deep vein thrombosis prophylaxis (using anticoagulants)


may be prescribed.

Drug Treatment
Hydroxyurea: Antisickling agent
Erythropoietin in patients

unresponsive to hydroxyurea
Hematopoietic stem cell transplant
Can cure some patients with SCD

Hydroxyurea induces fetal hemoglobin production, increases the red cell mean

corpuscular volume, and reduces the number of dense cells and irreversibly
sickled cells in the circulation (Goldberg et al., 1992) Hydroxyurea increases the
production of hemoglobin F (fetal hemoglobin), decreases the reactive
neutrophil count, increases erythrocyte volume and hydration, and alters the
adhesion of sickle erythrocytes to the endothelium.
Although thrombocytopenia and/or neutropenia are relative contraindications,
some patients can tolerate the medication despite these pre-existing factors
with close monitoring. Bimonthly blood counts are required when patients are
started on hydroxyurea. In some patients on hydroxyurea, the hematocrit rises
to the high 30's or even low 40's.
Erythropoietin (EPO) is a hormone produced by the kidney that promotes the
formation of red blood cells by the bone marrow. produced to a lesser extent by
the liver.
Patients take 2 hydroxyurea tablets a day until their fetal hemoglobin levels

stabilize, usually over 2 to 4 months. They have blood tests every 2 weeks to
monitor hemoglobin and fetal hemoglobin levels. At some time during this
period, they undergo a test to measure kidney function, in which they are
injected with an iodine-containing dye and wear a small pump for 1 day that
injects a small amount of dye under the skin over 24 hours. They come to the
clinic for 2 or 3 blood tests collected over 4 hours.
http://clinicaltrials.gov/show/NCT00270478

When assessing a patient's nutritional-metabolic pattern

related to hematologic health, the nurse would:


a. Inspect the skin for petechiae.
b. Ask the patient about joint pain.
c. Assess for vitamin C deficiency.
d. Determine if the patient can perform ADLs.

A is Correct
Inspect the skin for petechiae. Any changes in the skin's texture
or color should be explored when assessing the patient's
nutritional-metabolic pattern related to hematologic health.
The presences of petechiae or ecchymotic areas could be
indicative of hematologic deficiencies related to poor
nutritional intake or related causes.

A blood type and cross-match has been ordered for a

male patient who is experiencing an upper


gastrointestinal bleed. The results of the blood work
indicate that the patient has type A blood. This means
that
a. The patient has A antigens on his red blood cells
(RBCs).
b. The patient may only receive a type A transfusion.
c. The patient can be transfused with type AB blood.
d. Antibodies are present on the surface of the patients
RBCs.

Correct A The patient has A antigens on his red blood cells

(RBCs).
Donors with blood type A... can donate to recipients with blood
types A and AB
Donors with blood type B... can donate to recipients with blood
types B and AB
Donors with blood type AB... can donate to recipients with blood
type AB only
Donors with blood type O... can donate to recipients with blood
types A, B, AB and O (O is the universal donor: donors with O
blood are compatible with any other blood type)
So,
Recipients with blood type O... can receive from blood type O only
Recipients with blood type A... can receive from blood types A and
O
Recipients with blood type B... can receive from blood types B and
O
Recipients with blood type AB... can receive from blood types A, B,
AB and O (AB is the universal recipient: recipients with AB blood
are compatible with any other blood type)