CENTRAL OBJECTIVE:

On the completion of the class, the students acquire knowledge regarding anemia and its various aspects and acquire skill in caring for patient with anemia with desirable attitude.

SPECIFIC OBJECTIVE

At the end of the class students,

define anemia

classify different types of anemia

list down common clinical manifestations of anemia

IRON DEFICIENCY ANEMIA

define iron deficiency anemia

identify the incidence of iron deficiency anemia

listdown the cause of iron deficiency anemia

iilustrate the pathophysiology of iron deficiency anemia

recognize the clinical manifestation of iron deficiency anemia

explain the management of iron deficiency anemia

discuss the nursing management of iron deficiency anemia

enlist the complication of iron deficiency anemia

HEMOLYTIC ANEMIA

define hemolytic anemia

illustrate pathogenic mechanism of hemolytic anemia

classify hemolytic anemia

identify incidence of sickle cell anemia

enlist the cause of sickle cell anemia1

discuss the pathophysiology of sickle cell anemia

recognize the clinical manifestation sickle cell anemia

listdown complications of sickle cell anemia

describe different types of sickle cell anemia

MEGALOBLASTIC ANEMIA

define pernicious anemia

identify etiology of pernicious aemia

iiustrate the pathophysiology of megaloblastic anemia

listdown the clinical manifestation of pernicious anemia.

describe diagnostic evaluation of pernicious anemia

discuss the management of pernicious anemia

explain nursing diagnosis of pernicious anemia

define folic acid deficiency anemia

enlist the etiology of folic acid deficiency anemia

explain pathophysiology of anemia

identify clinical manifestation of anemia

discuss the diagnostic measures of anemia

describe nursing management of anemia

APLASTIC ANEMIA

define aplastic anemia

identify incidene of aplastic anemia

enlist the causative factors of aplastic anemia

illustrate the pathophysiology of anemia

discuss diagnostic measures of anemia

2

recognize the complications of anemia

3

ANEMIA

INTRODUCTION

Anemia is the lack of sufficient circulating hemoglobin to deliver oxygen to tissues. Anemia has multiple causes, and is commonly associated with other diseases and disorders (eg, renal disease, cancer, Crohn's disease, alcoholism). Anemia may be caused by inadequate production of RBCs, abnormal hemolysis and sequestration of RBCs, or blood loss. Iron deficiency anemia, pernicious anemia, folic acid deficiency, and aplastic anemia are the anemias most commonly seen in adults. Treatments for anemia range from nutritional counseling and supplements to RBC transfusions. One biotherapy treatment with significant potential is the administration of exogenous erythropoietin (Epoetin alpha or Darbepoetin alfa), a growth factor stimulating production and maturation or erythrocytes. It is used to stimulate RBC production in anemias associated with chronic renal failure, chemotherapy treatment, and HIV

DEFINITION OF ANAEMIA

• Anaemia is defined as a reduction in the haemoglobin concentration of the blood. This results in a decreased oxygen carrying capacity

CLASSIFICATION

ETIOLOGIC CLASSIFICATION

Impaired RBC production

Excessive destruction

Blood loss

MORPHOLOGIC CLASSIFICATION

Macrocytic , normochromic

Normocytic ,normochromic

Microcytic,hypochromic

Impaired RBC production

decreased erythrocyte production

iron deficiency anemia

thalassemia syndromes4

sideroblastic anemia

Defective DNA synthesis

Cobalamin deficiency

Folic acid deficiency

Decreased number of erythrocyte precursors

Aplastic anemia

Anemia of leukemia and myeliodysplasia

Anemia of chronic disease

Increased erythrocyte destruction

a) intrinsic abnormality

Membrane disorder

Hereditary spherocytosis

Hereditary elliptocytosis

Enzyme disorder

Pyruvate kinase deficiency

G6 PD deficiency

Hb c synthesis disorder

Thalassemia

Sickle cell disease

Hb c disease

Acquired membrane deficiency

Paroxysmal nocturnal hemoglobinuria.

b) extrinsic abnormality

i. Mechanical trauma-DIC

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ii. Chemical injury- lead poisoning

iii. Infection- malaria

iv. Immunologic injury- autoimmune hemolytic anemia

Drug mediated injuries.

MORPHOLOGIC CLASSIFICATION AND ETIOLOGIES OF ANEMIA

• Normocytic, normochromic anemia (MCV 80-100)

• Acute blood loss

• hemolysis

• macrocytic,normochromic anemia (MCV >100)

• cobalamine deficiency,folic acid deficiency

• Microcytic, hypochromic

Iron deficiency anemia, thalasemia, lead poisoning

IRON DEFICIENCY ANEMIA

iron deficiency is a condition in which total body iron content is decreased below a normal level, affecting hemoglobin synthesis.

ETIOLOGY

Inadequate dietary intake

Malabsorption

Blood loss

hemolysis

PATHOPHYSIOLOGY

Iron stores are typically depleted first

Decreased formation hb and decreased ability for the blood to carry oxygen

Serum iron level decreased

Cells are smaller and pallor than normal6

CLINICAL MANIFESTATION

Headache

Dizziness

Fatigue

Tinnitus

Palpitation

Dyspnea on exertion, pallor of skin and mucous membrane

smooth, sore tongue

Cheilosis(lesion at the corner of mouth)

Kolionychia(spoon shaped finger nail and thin concave nails at edges.)

Pica(craving to eat unusual substances)

DIAGNOSTIC MEASURES

History collection

Physical examination

Inspection may reveal a red swollen, smooth, shiny& tender tongue(glossitis)

The corners of mouth may be eroded, tender and swollen(angular stomatitis)

Inspection may also reveals spoon shaped, brittle nails.

A patient with advanced iron deficiency anemia may develop tachycardia because decreased oxygen perfusion causes the heart to compensate with increased cardiac output.

Lab investigation: CBC and iron profile, decreased Hb,hematocrit,serum iron and ferritin, elevated red cell distribution with normal or elevated total iron binding capacity(transferrin)

Determination of source of chronic blood loss may include sigmoidoscopy, colonoscopy,upper and lower GI studies,stool and urine for occult blood examination.

MANAGEMENT

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MEDICAL MANAGEMENT

MODALITIES OF MANAGEMENT

• Oral iron

100mg elemental iron it increase 0.18%day(300mg ferrous sulphate contain 60 mg elemental iron).iron preparations are ferrous gluconate, ferrous fumerate,ferrous succinate, ferrous sulphate.

Side effects: GIupset common. Ineffective in patient with worm infestations

• Parentral

Injectable iron and human recombinant erythropoietin

• Blood transfusion

NURSING MANAGEMENT

Nursing Diagnoses Imbalanced Nutrition: Less Than Body Requirements related to inadequate intake of iron Activity Intolerance related to decreased oxygen-carrying capacity of the blood

Ineffective Tissue Perfusion related to decreased oxygen-carrying capacity of the blood

Interventions

Promoting Iron Intake Assess diet for inclusion of foods rich in iron. Arrange nutritionist referral as appropriate. Administer iron replacement as ordered. Monitor iron levels of patients on chronic

therapy.

Increasing Activity Tolerance Assess level of fatigue and normal sleep pattern; determine activities that cause fatigue. Assist in developing a schedule of activity, rest periods, and sleep.

Encourage conditioning exercises to increase strength and endurance.

Maximizing Tissue Perfusion Assess patient for palpitations, chest pain, dizziness, and shortness of breath; minimize

activities that cause these symptoms. Elevate head of bed and provide supplemental oxygen as ordered.

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Monitor vital signs and fluid balance.

PATIENT TEACHING Reinforce the physician’s explanation of the disorder and answer any

questions. Be sure the patient understands the prescribed treatments and possible complications.

Ask about possible exposure to lead in the home (especially for children) or on the job. Teach the patient and his family about the dangers of lead poisoning, especially if the patient reports pica.

Advise the patient not to stop therapy even if he feels better because replacement of iron stores takes time.

Inform the patient that milk or an antacid interferes with absorption but that vitamin C can increase absorption. Instruct him to drink liquid supplemental iron through a straw to prevent staining his teeth.

Tell the patient to report any adverse effects of iron therapy, such as nausea, vomiting, diarrhea, and constipation, which may require a dosage adjustment or supplemental stool softeners.

Teach the basics of a nutritionally balanced diet—red meats, green vegetables, eggs, whole wheat, iron-fortified bread, and milk. However, explain that no food in itself contains enough iron to  treat iron deficiency anemia; an average-sized person with anemia would have to eat at least 10 lb (4.5 kg) of steak daily to receive therapeutic amounts of iron.

PERNICIOUS ANEMIA (ADDISON’S ANEMIA)

Pernicious anemia is a type of megaloblastic anemia associated with vitaminB 12

deficiency.or it is characterized by decreased production of hydrochloric acid deficiency of intrinsic factor,a substance normally secreated by the parietal cells of gastric mucosa that is essential for vitamin B 12absorption

ETIOLOGY Vitamin B12 is necessary for normal deoxyribonucleic acid synthesis in maturing RBCs. Pernicious anemia demonstrates familial incidence related to autoimmune gastric

mucosal atrophy.

Normal gastric mucosa secretes a substance called intrinsic factor, necessary for absorption of vitamin B12 in ileum. If a defect exists in gastric mucosa, or after gastrectomy or small-bowel disease, intrinsic factor may not be secreted and orally ingested B12 not absorbed.

Some drugs interfere with B12 absorption, notably ascorbic acid, cholestyramine, colchicine, neomycin, cimetidine, and hormonal contraceptives.

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Primarily a disorder of older people

PATHOPHYSIOLOGY

CLINICAL MANIFESTATION Of anemia—pallor, fatigue, dyspnea on exertion, palpitations. May be angina pectoris

and heart failure in the elderly or those predisposed to heart disease. Of underlying GI dysfunction—sore mouth, glossitis, anorexia, nausea, vomiting, loss of

weight, indigestion, epigastric discomfort, recurring diarrhea or constipation.

Of neuropathy (occurs in high percentage of untreated patients)—paresthesia that involves hands and feet, gait disturbance, bladder and bowel dysfunction, psychiatric symptoms caused by cerebral dysfunction.

DIAGNOSTIC EVALUATIONCBC and blood smear—decreased hemoglobin and hematocrit; marked variation in size and shape of RBCs with a variable number of unusually large cells.

Folic acid (normal) and B12 levels (decreased). Gastric analysis—volume and acidity of gastric juice diminished.

Schilling test for absorption of vitamin B12 uses small amount of radioactive B12 orally and 24-hour urine collection to measure uptake—decreased.

MANAGEMENT

Early I.M. vitamin B12 replacement can reverse pernicious anemia and may prevent permanent neurologic damage. An initial high dose of parenteral vitamin B 12 causes rapid RBC regeneration. Within 2 weeks, hemoglobin should rise to normal, and the patient’s condition should markedly improve. Because rapid cell regeneration increases the patient’s iron requirements, concomitant iron replacement is necessary to prevent iron deficiency anemia. After the patient’s condition improves, vitamin

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an inherited autoimmune response may cause genetic

Decreas hydrochloric acid and intrinsic factor productionImpairs vitB12 Absorption

Inhibits growth of all cells particularly RBcInsufficient and deformed RBcs with poor oxygen carrying capacity.

B12 doses can be decreased to maintenance levels and given monthly. Because such injections must be continued for life, patients should learn self-administration.If anemia causes extreme fatigue, the patient may require bed rest until hemoglobin rises. If he’s critically ill, with severe anemia and cardiopulmonary distress, he may need blood transfusions, cardiac glycosides, a diuretic, and a low-sodium diet for heart failure. Most important is the replacement of vitamin B 12 to control the condition that led to this failure. Antibiotics help combat accompanying infections, and topical anesthetics may relieve mouth pain .

NURSING DIAGNOSIS

Nursing Diagnoses Disturbed Thought Processes related to neurologic dysfunction in absence of vitamin B12

Impaired Sensory Perception (kinesthetic) related to neurologic dysfunction in absence of vitamin B12

Nursing InterventionsImproving Thought Processes

Administer parenteral vitamin B12 as prescribed. Provide patient with quiet, supportive environment; reorient to time, place, and person if

needed; give instructions and information in short, simple sentences and reinforce frequently.

Minimizing the Effects of Paresthesia Assess extent and severity of paresthesia, imbalance, or other sensory alterations. Refer patient for physical therapy and occupational therapy as appropriate.

Provide safe, uncluttered environment; make sure personal belongings are within reach; provide assistance with activities as needed.

Warn the patient to guard against infections, and tell him to report signs of infection promptly, especially pulmonary and urinary tract infections. The patient’s weakened condition may increase his susceptibility to infection.

Caution the patient with a sensory deficit to avoid exposure of his extremities to extreme heat or cold.

If neurologic involvement is present, advise the patient to avoid clothing with small buttons and ADLs that require fine motor skills.

Teach family members to observe for confusion or irritability and to report these findings to the physician.

Stress that vitamin B12 replacement isn’t a permanent cure and that Cthese injections must be continued for life, even after symptoms subside. If possible, teach the patient or his caregiver proper injection techniques.

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To prevent pernicious anemia, emphasize the importance of vitamin B12 supplements for patients who have had extensive gastric resections or who follow strict vegetarian diets.

MEGALOBLASTIC ANEMIA: FOLIC ACID DEFICIENCY

hronic megaloblastic anemia caused by folic acid (folate) deficiency. Etiology

associated with alcoholism. Impaired Dietary deficiency, malnutrition, marginal diets, excessive cooking of foods;

commonly absorption in jejunum (eg, with small-bowel disease).

Increased requirements (eg, with chronic hemolytic anemia, exfoliative dermatitis, pregnancy).

Impaired utilization from folic acid antagonists (methotrexate) and other drugs (phenytoin, broad-spectrum antibiotics, sulfamethoxazole, alcohol, hormonal contraceptives).

Clinical Manifestations Of anemia: fatigue, weakness, pallor, dizziness, headache, tachycardia. Of folic acid deficiency: sore tongue, cracked lips.

Diagnostic EvaluationHistory collection and assessmentPatient’s history may reveal severe, progressive fatigue, the hallmark of folic acid deficiency.associated findings include ,shortness of breath,palpitations,diarrhea,nausea,anorexia,headaches,forgetfulness and irritability.INSPECTION: may reveal generalized pallor and jaundice.Cheilosis and glossitis may be present.LAB INVESTIGATION

Vitamin B12 and folic acid level—folic acid will be decreased. CBC will show decreased RBC, hemoglobin, and hematocrit with increased mean

corpuscular volume and mean corpuscular hemoglobin concentration.

MANAGEMENT

Folic acid 5mg per day,oral

Continue routine oral therapy for hemolytic anaemia.

Parentral therapy for severe deficiency.

Worm infestation

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Common cause of anemia developing countries

Oral iron therapy becomes ineffective

Treatment by antihelminthics is a must.

TREATMENT

Mebendazole: 100mg twice daily for three days

Albendazole:400 mg once a day for three days.

DIET THERAPY

Well balanced diet,foods high in folic acid .folic acid is water soluble and heat liable,it’s easily destroyed by cooking. foods are beef liver,mush rooms,oatmeal,peanut butter,red beans.

COMPLICATIONSFolic acid deficiency has been implicated in the etiology of congenitally acquired neural tube defects.NURSING MANAGEMENTNursing Assessment

Obtain nutritional history. Monitor level of dyspnea, tachycardia, and development of chest pain or shortness of

breath for worsening of condition.

Nursing Diagnosis

Imbalanced nutrition: Less than body requirements related to adverse GI effects .:

identify foods high in folic acid consume a diet high in folic acid

no longer show signs and symptoms of folic acid deficiency.

Assist alcoholic patient to obtain counseling and additional medical care as needed.

Fatigue related to folic acid deficiency: express an understanding of the relationship between fatigue and folic acid

deficiency employ measures to prevent or lessen fatigue

regain his normal energy level as folic acid deficiency resolves.

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Impaired physical mobility related to weakness caused by reduced hemoglobin levels.

• maintain functional mobility not develop complications due to impaired physical mobility

regain normal physical mobility as hemoglobin levels return to normal and folic acid deficiency resolves.

PATIENT EDUCATION

To prevent folic acid deficiency anemia, emphasize the importance of a well-balanced diet high in folic acid. Identify alcoholics or other high-risk people with poor dietary habits, and try to arrange for appropriate counseling.

Teach the patient to meet daily folic acid requirements by including a foo d from each food group in every meal. If the patient has a severe deficiency, explain that diet only reinforces folic acid supplementation and isn’t therapeutic by itself. Urge compliance with the prescribed course of therapy. Advise the patient not to stop taking the supplements when he begins to feel better.

Warn the patient to guard against infections, and tell him to report signs of infection promptly, especially pulmonary and urinary tract infections, because his weakened condition may increase susceptibility.

APLASIC ANEMIA

DEFINITION:aplastic anemia is a disorder characterized by bone marrow hypoplasia or aplasia resulting in pancytopenia(insufficient numbers of RBc’s,WBc’s and platelets.

INCIDENCE:it is rare disorder contributing to 1-2% of total number of anemias.Males suffer twice as many as females.In 75% idiopathic.

ETIOLOGY:Primary:acquired idiopathic; congenital(fanconis anemia)

Secondary:chemicals and toxins including drugs.

1.agents which regularly produce bone marrow hypoplasia

a. anticancer drugs(cyclophosphamide)

b. ionizing raditation.

2.agents which occationally produce bone marrow hypoplasia

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(antidiabetic agents,anticonvulsants etc)

3. infections: viral hepatitis,chicken pox&miliary tuberculosis.

4.pregnancy

5.thymoma

PATHOPHYSIOLOGY

1. Loss of pluri potent stemcells2. Dysfunction of progenitor cells

3. Defects in the microenvironment of the marrow

4. Abnormalities of humoral regulators of hematopoiesis

5. Autoimmune inhibition of hematopoiesis

Due to factors

CLINICAL MANIFESTATIONS

From anemia: pallor, weakness, fatigue, exertional dyspnea, palpitations. From infections associated with neutropenia: fever, headache, malaise; adventitious

breath sounds; abdominal pain, diarrhea; erythema, pain, exudate at wounds or sites of invasive procedures.

From thrombocytopenia: bleeding from gums, nose, GI or GU tracts; purpura, petechiae, ecchymoses.

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Reduced bone marrow volume

Formed elements are reduced

Megakaryocytes, myeloid elements,and erythroblasts are progressively wiped out

Resulting thrombocytopenia, neutropenia, anemia

DIAGNOSTIC MEASURES

History collection and physical examination

The patient may report signs and symptoms of anemia(progressive weakness and fatigue,shotness of breath and headache or signsof thrombocytopenoia.

Inspection: may reveals pallor if patient is anemic.

Auscultation: reveals basilar crackles bilaterly,tachycardia and gallop rhythm

Lab investigations:RBC count of 1 millon/mm 3

platelet count less than 130,000/mm3

neutrophills count less than 47.6%

bone marrow biopsies performed at several sites

MANAGEMENT

Removal of causative agent or toxin. Allogeneic bone marrow transplantation (BMT)—treatment of choice for patient with

severe aplastic anemia (see page 1009). This treatment option provides longterm survival for 75% to 90% of patients, depending on the age of the patient, history of prior blood transfusions, and source of marrow.

Immunosuppressive treatment with corticosteroids, cyclosporine, cyclophosphamide, antithymocyte globulin or antilymphocyte globulin as single treatments or in combinations. This treatment option provides long-term survival for 70% to 80% of patients.

Androgens (oxymetholone or testosterone enanthate) may stimulate bone marrow regeneration; significant toxicity encountered. They may be used when other treatments have failed.

Supportive treatment includes platelet and RBC transfusions, antibiotics, and antifungals.

NURSING DIAGNOSIS

Nursing Diagnoses Risk for Infection related to granulocytopenia secondary to bone marrow aplasia Risk for Injury related to bleeding

Nursing InterventionsMinimizing Risk of Infection

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Care for patient in protective environment while hospitalized —private room with strict hand washing and avoidance of any contaminants (see Patient Education Guidelines).

Encourage good personal hygiene, including daily shower or bath with mild soap, mouth care, and perirectal care after using the toilet.

Monitor vital signs, including temperature, frequently; notify health care provider of oral temperature of 101°F (38.3°C) or higher.

Minimize invasive procedures or possible trauma to skin or mucous membranes.

Obtain cultures of suspected infected sites or body fluids

Minimizing Risk of Bleeding Use only soft toothbrush or toothette for mouth care and electric razor for shaving; keep

nails short by filing. Avoid I.M. injections and other invasive procedures.

Prevent constipation with stool softeners as prescribed.

Restrict activity based on platelet count and active bleeding.

Monitor pad count for menstruating patient; avoid use of vaginal tampons.

Control bleeding by applying pressure to site, using ice packs and prescribed topical hemostatic agents.

Administer blood product replacement as ordered; monitor for allergic reaction, anaphylaxis, and volume overload.

PATIENT EDUCATION AND HEALTH MAINTENANCE Teach patient how to minimize risk of infection

o Wash hands after contact with possible source of infection.

o Immediately clean any abrasion or wound of mucous membranes or skin.

o Monitor temperature and report fever or other sign of infection immediately.

o Avoid crowds and people with illnesses.

o Avoid raw or undercooked foods.

o Use condoms and other safer sex practices.

Teach patient how to minimize risk of bleeding

o Avoid falls or other injury.

o Use electric razor rather than plain razor.

o Use nail clippers or file rather than scissors.

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o Avoid blowing nose.

o Use soft toothbrush or toothette for mouth care.

o Use water-soluble lubricants, as needed, during sexual activity.

Advise patient to avoid exposure to potential bone marrow toxins: solvents, sprays, paints, pesticides.

Teach patient to take only prescribed medications; avoid aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs), which may interfere with platelet function. As some vitamins and herbs may also affect platelet function, instruct patient to check with physician before using any supplements.

COMPLICATIONS Untreated severe aplastic anemia is almost always fatal, generally because of

overwhelming infection. Even with treatment, morbidity and mortality caused by infections and bleeding are high.

Late complications, even after successful treatment, include clonal hematologic diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplasia, and acute myelogenous leukemia

HEMOLYTIC ANEMIAS

MEANING:in hemolytic anemias ,the erythrocytes have a shortened life span,thus their number in the circulation is reduced.

INCIDENCE

Hemolytic anemia contributes 4% of the total anemias seen in south India.

PATHOGENIC MECHANISM

Intravascular :Hemolysis occur intravascularly and occurs with in systemic circulation.hemoglobin is released in to plasma.

Hemoglobin is lost through kidneys or catabolized in the liver.

Extravascular: trapping of red cells in spleen or liver siuses.

Lyses of trapped red cells.

CLASSIFICATION OF HEMOLYTIC DISORDERS18

1. INHERITED HEMOLYTIC DISORDERS

a) Red cell membrane defects eg:hereditary spherocytosis, hereditary elliptocytosis

b) Red cell enzyme deficiencies eg: deficiencies of pyruvate kinase,G6PD deficiency.

c) Defects in aminoacid configuration of globin chain eg:sickle cell anemia

d) Failure to switch over of beta or alpha globin chainsin post natal life eg:thalassemias

2)ACQUIRED HEMOLYTIC ANEMIA

a) Immunologically mediated hemolytic anemia

Eg:incompatible blood transfusioniseas, hemolytic disease of the newborn,autoimmune hemolytic anemia

b) Microangiopathic hemolytic anemia

Eg: disseminated intravascular coagulation

c) Caused by infections:malaria

d) Caused by drugs: chemicals and toxins.

Eg: sulphonamides, formic acid poisoning

e)paroxysmal nocturnal hemoglobinuria

SICKLE CELL ANEMIA

Sickle cell disease is a group of inherited autosomal recessive disorders characterized by the presence of an abnormal form of hemoglobin in the erythrocyte to stiffen and elongate taking on a sickle shape.

INCIDENCE

1 in 350-500 live birth in African Americans.

1 in 1000-1400hispanic Americans

Affects 8 of every 1 lakh people

TYPES OF SICKLE CELL DISEASE

Sickle cell anemia

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Sickle cell thalassemia

Sickle cell Hbc disease

Sickle cell trait

Sickle cell anemia is the severe form of SCD syndromes. It occurs when person is homozygous for hemoglobinS(Hbss);the person is inherited Hbs from both parents.

Sickle cell thalassemia and sickle cell Hbc occur when the person inherits Hbs from one parent and another type of abnormal haemoglobin(such as thalassemia or Hbc) from other parent . it is less common and less severe.

sickle cell trait occurs when a person is heterozygous for hemoglobin S(Hb AS);the person has

inherited Hbs from other parent. It is mild to asymptomatic condition.

ETIOLOGY AND PATHOPHYSIOLOGY

It is generally determined, inherited disease. The basic abnormality lies with in the globin fraction of the haemoglobin,where a single aminoacid(valine) is substituted for glutamic acid in the 6th position of the beta chain.this single aminoacid substitution profoundly alters the properties of hemoglobin molecules(Hbs HbA

Hbs has normal oxygen carrying capacity. However, when the oxygen tension of the RBC’s decreases,Hbs polymerizes causing hemoglobin to distort and realign the RBC into a sickle shape.

organs.the affected cells have shortened life spanof 7-20 days,because they are identified as abnormal and destroyed by the spleen.

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DECREASED OXYGEN TENSION

SICKLING OF RBc

INCREASED BLOOD VISCOSITY

DECREASED OXYGEN SUPPLY

ORGAN INFARCTION

PLUGGING OF SMALL VESSELS

CLINICAL MANIFESTATION

Clinical manifestations are sporadic; the child may be asymptomatic for several months.periods

of crisis occurs in variable intervals.

SIGNS OF ANEMIA

Hb level 6-9gm/dl

Loss of appetite

Paler

Weakness

Fever

Irritability

Jaundice.

SICKLE CELL CRISIS

Distal ischemia and infarction

Extremities :bony destruction,bone pain(painful,swollen large joints)

Dactylitis(hand foot syndrome) aseptic infarction of metacarpals and metatarsals causing

symmetrical swelling and pain.

SPLEEN:abdominal pain,spleenomegaly,splenic vasoocclusion,decreased splenic function.

CEREBRAL OCCLUSION:strokes,hemiplegia,retinal damage lead to blindness,seizures.

PULMONARY INFARCTION

ALTERED RENAL FUNCTION(enuresis,hematuria)

IMPAIRED LIVER FUNCTION:hepatomegaly,gall stones)

PRIAPISM-abnormal recurrent prolonged painfulpenile erection.

SPLENIC SEQUESTRATION CRISIS21

Large amout of blood pooled in spleen.

Massive enlargement of spleen.

Great decrease in RBc mass

Signs of circulatory collapse(shock and hypotension)

APLASTIC CRISIS

Bone marrow ceases production of RBc’s only

Low reticulocyte count

CHRONIC SYMPTOMS

Gall stones, renal failure, fibyotic spleen ,growth retardation ,delayed puberty, chronic anemia,heart failure,decreased life span,altered bony structures.

DIAGNOSTIC EVALUATION

History collection,physical examination

Peripheral blood smear(reveal sickled cell and abnormal reticulocytes)

Sickling test/sickle cell prep

Oxygen is removed from drop of blood and is observed under microscope for sickle shaped cells.

Sickledex

A small amount of blood is placed in a solution containing a chemical reducing agent.sickle hemoglobin is indicated if the solution turns cloudy.

Hemoglobin electrophoresis

Hemoglobin is subjected to an electric current that separates the various types and determines the amounts present.

complete blood count

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Liver and renal parameters

Antenatal diagnosis

Amneocentesis

Gene mapping

COMPLICATIONS

Chronic hemolytic anemia

Risk of death in children<5years

Sepsis or/low sequestration

Hemolysis and vasocclusion

MANAGEMENT

PREVENTION OF SICKLING

Promote oxygenation and hemodilusion

150ml/kg/day fluid intake

Avoid high attitude/low oxygen environment

Avoid physical exertion

Avoid extreme heat or cold

Oxygen therapy if spo2 <90%

Routine blood transfusion

ACUTE MANAGEMENT

1. APLASTIC EPISODE

Blood transfusion starting at10ml/kg

2. SPLENIC SEQUESTRATION

Blood transfusions to release trapped RBC’S.

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Plasma volume expanders(hypovolemia)

3. Hemolytic episodes

Requires hydration and transfusions

4. Vasoocclusive painful episode

a) Hydration

b) Monitor electrolyte Ph balance

c) Analgesics

IV opiods morphine

NSAIDs and acetaminophen

Behaviour modification program

Relaxation therapy

Hypnosis

Music therapy

Massage

Transcutaneous electrical nerve stimulation

d)blood transfusion

Fe overload-complication

e)new research on butyrate and hydroxyurea which increase HbFand pevent sickling. Bone marrow transplantation is under investigation

5. Infection

Antibiotic prophylaxis-prevent bacterimia

Cefuroxime 100mg/kg/day

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Prevention through vacinatoon

6. Exchange blood transfusion.

THALASSEMIA

Thalassemia is defined as an autosomal recessive genetic disorder associated with defective hemoglobin chain synthesis ,characterized by hypochromia( an abnormal decrease in the hemoglobin content of RBCs),extreme microcytosis(smaller RBCs than normal),hemolysis(destruction of blood elements) and variable degrees of anemia.

INCIDENCE

Pediatric population is commonly affected

Over 9000 thalasemia children are born every year in India and treatment is very expensive.

CLASSFICATION AND PATHOLOGY

Thalassemias are classified according to which chain of hemoglobin is affected. The predominant type of hemoglobin from soon after birth until death is HbA.all hemoglobin consists of two parts heme and globin. The globin part of HbA has 4 protein secreations called polypeptide chains.two of these chains are identical and re designated as alpha chains. The other two chains are identical to one another ,but differ from alpha chains and are termed as beta

chains. thalassemia produces “deficiency of alpha or beta globin.

i. Alpha thalassemis

Involve the genes HBA,andHBA2

Connected to the detection of 16 p chromosome

Associated with decreased alpha globin production

Result in excess beta chains in adults and excess y chains in newborn

Excess beta chains form unstable tetramers(called hemoglobin H or HbH of 4 beta

chains) which have abnormal oxygen dissociation curve.

ii. Beta thalassemias

Involve mutations in HBBgene on chromosome 11

Severity of disease depend on the nature of mutation.

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Mutations are characterized as

-severe form due to prevention of any form of beta chain

-mild,allow some beta chain formation

Donot form tetramers, rather bind to RBc membrane,producing membrane damage and at high concentrations they form toxic aggregates.

There are two forms of thalassemia

Thalassemia minor

Thalassemia major(cooley’s anemia)

THLASSEMIA MINOR

Has only one type of thalassemia gene together with one perfectly normal beta chain gene.

The person is said to be heterozygous for beta thalassemia.

Mild anemia with slight lowering of Hb.

Have normal blood iron level unless they are iron deficient for other reasons.

No treatment is necessary.

Iron is neither necessary, nor advised.

THALASSEMIA MAJOR(COOLEY’S ANEMIA)

child born with thalassemia major has 2 gene for beta thalassemia and no normal beta chain gene.

Child is homozygous for beta thalassemia. This cause a striking deficiency in beta chain production and in the production of Hb A.

The clinical picture is associated with thalassemia major was described in1925 by the American pediatrician cooley.

At birth the baby with thalassemia major seems entirely normal. This is because the predominant Hb at birth is still fetal hemoglobin.(HbF).HbF has has two alpha chains(like HbA)and two gamma chains(unlike HbA).it has no beta chains so the baby is protected at birth

from the effects of thalassemia major.

DELTA THALASSEMIA

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As well as alpha and beta chains, being present in Hb about 3% of adult hemoglobin is made of alpha and beta chains. Just as with beta thalassemia mutations can occur which affect the ability of this gene to produce delta chains.

ETIOLOGY

Inherited, autosomal recessive

Absent or defective synthesis of HbA

CLINICAL FEATURES

Tiredness –due to anemia

Anemia(moderate- beta thalassemia intermedia;severe-beta thalassemia major)

Slow growth(physical and mental)

Enlarged internal organs- spleen, liver, heart(due to rapid destruction of abnormal RBc’s extramedullary production of RBc’s and fibrotic changes caused by hemochromatosis- excessive iron storage in tissues with resultant cellular damage.

Bone problems (appearance resembling down syndrome):wider bones than normal,mangoloid eyes,

Frontal bossing and maxillary prominence.( huge erythroid production is in effective because most cells produced are so poorly hemoglobinized that they never leave marrow), nasal bridge depression,malocclusion(protrution of upperlip and incisors)

Jaundice

Fetal death:alpha thalassemia major also known as hydrops fetalis, result in death of fetus before it has developed to the point of viability.

Skin discolouration (absorption of iron higher than normal amount from GIT as compensatory mechanism for ineffective marrow expansion.(greenish brown or bronzed skin with fine freekles)

Cardiacfailure(if Hb falls<6gm/dl)

Cirrhosis of liver(fibrosis of hepatic cell by chemochromatosis)

Insulin dependent diabetes mellitus(fibrosis of pancreas)

Lymph node enlargement in abdomen.

DIAGNOSTIC MEASURES27

Hematologic studies: immature erythrocytes, low HbB,low hematocrit, decreased RBC count.Hemoglobin electrophoresis (the movement of charged suspended particles through liquid medium in response to change in an electrical field)( a test to identify various abnormal hemoglobin in blood including certain genetic disorders such as sickle cell anemia.

PARAMETERS FINDINGS

Hemoglobin Decreased

Hematocrit Decreased

MCV Normal

Reticulocytes Increased

Serum iron Increased

Bilirubin Increased

TREATMENT

MEDICAL MANAGEMENT

THALASSEMIA MINOR: requires no treatment because body adapts to the reduction of

normal hemoglobin.

CHELATION THERAPY: iv deferoxamine binds to iron to reduce the iron overloading that some times occurEto foster the patient’s own erythropoiesis with out spleen enlargement .

SPLENECTOMY(RBC s sequestered in enlarged spleen)

BLOOD TRANSFUSION

Cost effective

Maintain Hb between 9-9.5gm/dl

Pretransfusion workup RBC phenotype, hepatitis vaccination.

Iron and folate level should be checked.h transf

Transfuse blood with less leukocyte.

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10-15ml/kg packed RBC at rate of 5ml/kg/hr every 3-5 weeks

Administer acetaminophen and diphenhydramine hydrochloride before each transfusion to minimize febrile and allergic reaction.

CHELATION THERAPY

Chelation therapy can delay the onset of cardiac disease and in some patients, even prevent its occurance.

Many def ,agents tested only deferoxamine mesylate proven effective and safe.

Adult 35mg/day iron is removed.

Oral less effective

Can be administered parenterally(IM,IV,S/C)

30-40 mg/kg/day administered over 8-12 hrs for 5 days/week by

Syringe pump.

6-10 g administered iv in reversing serious iron overload complication.

SURGICAL MANAGEMENT

SPLENECTOMY

Spleen is known to contain large amount of labile nontoxic iron. Spleen also increases RBC distruction and iron distribution.

PLACEMENT OF CENTRAL LINE

It is for easy and convenient administration of blood transfusion ,chelation therapy or both.

DIET THERAPY

Normal diet is recommended.emphasis on following supplements

Folic acid to be included, avoid iron intake.

Small doses of ascorbic acid (vit-c) and alpha tocopherol(vit-E)

Coffee or tea (help to decrease iron absorption in gut.)

COMPLICATION

Iron overload

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Cardiac failure

Infection

Bone deformities

Spleenomegaly

Slow growth/growth retardation

NURSING CARE PLAN FOR THALASSEMIA

ASSESSMENT

Signs of anemia

Problem associated with vital functions like respiration cardiac function.

Oxygen saturation level

Activity level

Genetic predisposition/familial predisposition

Laboratory values.

NURSING DIAGNOSIS

Fatigue related to decreased oxygen carrying capacity of the blood secondarty to reduced hemoglobin level.

Imbalaced nutrition less than body requirement related to treatment effect, disease effect

Risk for infection related to malfunction

REVIEW OF LITERATURE

ORIGINAL ARTICLE     

Year : 2008  |  Volume : 33  |  Issue : 4  |  Page : 243-245

 

A study of anemia among adolescent females in the urban area of Nagpur

Sanjeev M Chaudhary, Vasant R Dhage

Department of Preventive and Social Medicine, Government Medical College and Hospital, Nagpur, Maharashtra,

India

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Abstract  

Objectives: To estimate the prevalence of anemia among adolescent females and to study the socio-demographic factors associated with anemia. Materials and Methods: A cross-sectional survey was conducted in an urban area under Urban Health Training Center, Department of Preventive and Social Medicine, Government Medical College and Hospital, Nagpur. A total of 296 adolescent females (10-19 years old) were included in this study. The study took place from October 2002 to March 2003 (6 months). Statistical analyses were done using percentage, standard error of proportion, Chi-square test, and Student's 't' test. Results: The prevalence of anemia was found to be 35.1%. A significant association of anemia was found with socio-economic status and literacy status of parents. Mean height and weight of subjects with anemia was significantly less than subjects without anemia. Conclusions: A high prevalence of anemia among adolescent females was found, which was higher in the lower socio-economic strata and among those whose parents were less educated. It was seen that anemia affects the overall nutritional status of adolescent females.

Grand Rounds | August 8, 2001

Runner's AnemiaChi V. Dang, MD, PhD

JAMA. 2001;286(6):714-716. doi:10.1001/jama.286.6.71

Macrocytic anemia occurring in patients with fatigue suggests numerous diagnoses, ranging from nutritional deficiencies to a myelodysplastic syndrome. A careful history-taking is critically important for recognition of runner's anemia, which is due to plasma volume expansion, with hemolysis from the pounding of feet on pavement, and hemoglobinuria. Gastrointestinal blood loss may also contribute to anemia in long-distance runners. Early recognition of runner's anemia in patients with a complex presentation of anemia is important in circumventing many diagnostic tests. Runner's anemia should be considered when, amidst a constellation of signs and symptoms, mild anemia is well tolerated by an avid runner

. CONCLUSIONRunner's anemia is largely due to plasma volume expansion with elements of hemolysis, hemoglobinuria, and in long-distance runners, gastrointestinal blood loss. The recognition of these symptoms is extremely important in patients—such as the one reported here—with a complex presentation of anemia and chronic fatigue. In particular, reaching this diagnosis at an earlier stage would have circumvented many of the diagnostic tests discussed above. Furthermore, recognition of this syndrome is important in patient management, which should include reassurance and avoidance of unnecessary therapies. Nevertheless, it was the methodical evaluation of the differential diagnoses that led to the correct diagnosis. Amidst the constellation of signs and symptoms, mild anemia that is tolerated by an avid, devoted runner should raise the possibility of a diagnosis of runner's anemia.

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CONCLUSION

lifespan; thus, their number in the circulation is reduced. Fewer erythrocytes result in decreased available oxygen, causing hypoxia, which in turn stimulates an increase in erythropoietin release from the kidney. The erythropoietin stimulates the bone marrow to compensate by producing new erythrocytes and releasing some of them into the cir-culation somewhat prematurely as reticulocytes. If the re cell destruction persists, the hemoglobin is broken down ex-cessively; about 80% of the heme is converted to bilirubin, conjugated in the liver, and excreted in the bile.

The mechanism of erythrocyte destruction varies, but allM types of hemolytic anemia share certain laboratory features: The reticulocyte count is elevated, the fraction of indirect (unconjugated) bilirubin is increased, and the supply of haptoglobin (a binding protein for free hemoglobin) is de-pleted as more hemoglobin is released. As a result, the plasma haptoglobin level is low. If the marrow cannot com-pensate to replace the erythrocytes (indicated by a de-creased reticulocyte count), the anemia will progress.

REFERENCE

Joyce m black.medical surgical nursing .volume ii .elseiver : Missouri.2005 Lewis , heitkemper etc .medical surgical nursing .7th edition .mosby. Philadelphia .2007

Monachan sancks , Phipps .medical surgical nursing .8th edition. Elseiver t :Philadelphia. 2007.

Suzanne. C. sneltzer .Brunner & suddharts text book of medical surgical nursing .10 th

edition .lippincott: Philadelphia.2004

Sandra m neltima. Lippincott manual of nursing .practice .8th edition. Medical publishers.2006.

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