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Alteration in Hematologic

function

Lorraine Murphy RN PNP

October 2007

FIGURE 28–1 Types of blood cells.

Red Blood Cells

• Most abundant cellular element of blood�4.6-5.9 million/mL

• Erythropoiesis- process of forming new RBC• STIMULATED BY HYPOXIA

• Polycythemia: condition of above-normal increase in the number of RBCs

• Functions to carry hemoglobin which is responsible for cell respiration

• Newborn has a naturally occurring elevation in red blood cells due to a ligh level of erythropoietin, which stimulates red cell production

Hemolysis

• Normal survival of RBC is 120 days

Red blood cell

IRON= FERRATINBILIRUBIN EXCRETED IN

BILE AND URING

Anemia• A reduction in the number of red blood cells, the quantity of

hemoglobin and the volume of packed red cells below normal levels

• Iron deficiency anemia– Lack of iron

– Affects production of RBCs

– RBCs appear hypochromic, decreased hemoglobin synthesis– Manifestations based on severity

• Pallor occurs when hemoglobin levels are less than 7 g perdL• Fatigue• Irritability• PICA

• Thalassemia

• Aplastic Anemia

• Pernicious Anemia

• Sickle Cell Anemia

HYPOCHROMIC

RED BLOOD

CELLS

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What causes iron deficiency

• Increased iron needs

– Rapid growth of infants and toddlers require more

– Women that are pregnant

– Adolescents with heavy menstrual periods

• Decreased iron intake or absorption

– Iron from meat is absorbed 2-3X more efficiently

than iron from plants

• Excess loss

– Hemolytic anemias

Iron deficiency anemia signs and symptomsMost common type of anemia in children

Conjunctivalpallor

Difficulty maintaining body

temperature

Inflamed tongue

glossitis

Decreased immune function

Palmar crease pallor

Irritability and anorexia

Tachycardia

Systolic murmurpica

Lethargy, increased need

for sleep

Lack of interest in toys or play

activities

Iron deficiency anemia

lab values

“How is it diagnosed?”

• Hemoglobin

• Hematocrit

• Reticulocyte count

Hemoglobin 9.5-11 g/dL Mild iron deficiency anemia

Hemoglobin 8-9.4 g/dL Moderate iron deficiency

anemia

Hemoglobin < 8 g/ dL Severe iron deficiency anemia

Reticulocytes

• Helps distinguish a hypoproductive anemia

from a destructive process

Indicates bone marrow

disorders or aplastic crisis

Indicates hemolytic process or

active blood loss

Iron deficiency Anemia

Risks

Demographic factors

•Prematurity

•Teenagers

•Female

•6 month old

•Multiple gestation

Dietary factors

•Cow’s milk before age 1yr

•> 24 oz. of cow’s milk

•Low iron formula

•Breastfeeding without iron supplementation

Social/political factors

•Low socioeconomic background

•Recent immigration from a developing country

•rate of weight gain greater than averag

Risk for Iron Deficiency anemia

Introduction of cow’s

milk in the first year

of life

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Evidence-Based careprevalence of iron deficiency anemia between the ages

of 1-3 years is greater than we think

• Third National Health

and Nutrition

Examination Survey

(NHANES III)

• Third Report on

Nutrition Monitoring in

the United States

• Reported that

prevalence of iron

deficiency anemia in

one to two-year-olds to

be 3%

• Prevalence in one-to

three-year-olds to be

12%

Why does risk increase again after 1

year?

• Cow’s milk replaces breastfeeding or iron

fortified formula

• Noniron-fortified cereals enter the diet and

• juices reduce the child’s apetite for solid food

Comparison of cow’s milk and

breastmilk r/t anemia risk

Cow’s milk breastmilk

Low in iron Low in iron

Iron is poorly absorbed 50% is bioavailable to infant

It decreases the absorption of

iron from other dietary sources.

Exclusive breastfeeding after 4-

6 months increase the risk for

iron deficiency

When are children screened for Fe

deficiency anemia

• 9- 12 months

• 24 months

Table 28-4 Infectious and Inflammatory Causes of Anemia Complications of Iron Deficiency

Anemia

• Poor muscle development

• Decreased attention span

• Decreased performance on developmental

tests

• Decreased ability to process information

obtained through hearing (auditory

processing)

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Anemia treatment

• Iron supplementation

– ORAL FERROUS SULFATE at 3-6 mg/kg/day for 4

weeks, and then repeat Hgb/hct

• Increase iron in diet

Medication therapy

Ferrous Sulfate• Liquid iron should be taken through a straw to

prevent staining of teeth. For infants administer into the back of the mouth.

• Administer on an empty stomach

• Administer with source of vitamin C

• Side effects– Black tarry stools

– Constipation

– GI discomfort

– Foul aftertaste

Dietary management of prevention of

iron deficiency anemia

newborn

breastfed

Before 4 months nothing

After 4 months

Add sources of iron

After 12 months

Add iron rich food

Formula fed

High iron

Do not give additional iron

Low iron

Not recommended

Iron rich foods

• Enriched bread and cereal

• Iron-fortified dry cereal

• Dark green vegetables

• Beans

• Meats, fish, poultryADD VITAMIN C WHICH HELPS THE

BODY ABSORB IRON

Evaluation

• With treatment, reticulocyte count increases within

3-5 days. Indicates a positive therapeutic response

• Hemoglobin should return to normal within 4-8

weeks

• If Hemoglobin has increased by more than 1 g/dL or

hematocrit has increased by more than 3%, continue

treatment for 2 more months, repeat labs,

• When labs are normal, wean from Fe supplements,

• Repeat labs in 6 months, monitor wt/ development

September is sickle

cell awareness

month. More than

70,000 people in

the US have sickle

cell disease and

more than 2

million people

carry the gene that

allows them to

pass it on to their

children

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Anemia (continued)

• Sickle cell anemia

– Most common single gene disorder in African

americans

– Genetic mutation autosomal recessive disease

– Hemoglobin S replaces normal hemoglobin

– RBC’s loose doughnut shape become sickle-shaped

– Manifestations appear in multiple body systems

– Severity based on pathologic changes

FIGURE 28–5 The clinical manifestations of sickle cell anemia result from pathologic changes to structures and systems throughout the

body.

FIGURE 28–5 (continued) The clinical manifestations of sickle cell anemia result from pathologic changes to structures and systems

throughout the body. Sickle Cell Anemia

Sickling

• Triggered by fever, emotional stress,

physical stress

• States of hypoxia

– High altitudes

– Poorly pressurized airplanes

– Hypoventilation

• Dehydration

• Cold

Sickle cell crisis

• Acute exacerbations of disease

• Vary in severity and frequency

• Three types

– Vaso-occlusive crisis

– Sequestration crisis

– Aplastic crisis

Vaso-occlusive crisis

“Painful crisis”• Happens when sickled red blood cells block small

blood vessels that carry blood to tissues or bones, causing pain that can begin suddenly and last several hours to several days.

• Thrombosis and infarction of tissue may occur if crisis is not reversed

• Fever, pain, swelling of joints in hands and feet

• Severe abdominal pain

• priapism

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Splenic sequestration

• Blood is trapped in spleen leads to vasomotor collapse and shock

• Life threatening complication, second only to infections as a cause of death in infants with sickle cell disease.

• Occurs between the ages of 4 months and 3 years

• Treated with aggressive blood transfusions

• Emergent splenectomy

Aplastic crisis

• Diminished erythropoiesis and increased

destruction of red blood cells

• Triggered by viral infection or depletion of folic

acid

• Clinical manifestations include profound

anemia, pallor and fatigue

Pain r/t sickling of RBC

• Can be reversed

• OXYGENATION

• HYDRATION

• PAIN MANAGEMENT

• rest

Risk for infection

• Daily prophylactic penicillin VK 125 mg bid

starting at 2 months of age

• Erythromycin for children with penicillin

allergies

• Vaccines to prevent pneumonia, haemophilus

influenzae type B (HIB)

Medications for SS anemia

• Hydoxyurea 15-20 mg/kg/day to start and increased

until therapeutic response (not more than 35 mg/kg)

– Decreases the production of abnormal blood cells and

leads to a lesser amount of pain.

– Side effects include bone marrow suppressin, headaches,

dizziness, nausea, and vomiting.

Anemia (continued)

• Hereditary spherocytosis

– Congenital hemolytic anemia

– No abnormality of hemoglobin

– Cells have unusual structure

– Manifestations appear in neonatal period or infancy

– Severity varies

• Thalassemias

– Groups of hereditary disorders

– Hemoglobin synthesis abnormal

– Range mild to severe

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Anemia (continued)

• Thalassemias

– Three types based on which chain in hemoglobin

molecule is defective

– Clinical manifestations based on type and severity

– Severe anemia leads to chronic hypoxia

• Autosomal recessive

• Symptoms include pallor, poor wt. gain,

hepatosplenomegaly, severe anemia

FIGURE 28–6 Red blood cell appearance in ß-thalassemia. What characteristic abnormalities can be seen on this microscopic view?

Courtesy of Dr. Ed Wong, Laboratory Medicine, Children’s National Medical Center, Washington, DC.

B-thalassemia

• To compensate for decreased HbA, production of

Hb F increases. The RBCs are fragile and are

easily destroyed, shortening their lifespan

• As hemolysis increased, hemosiderin is deposited

in the skin, causing a bronze appearance.

• Chronic anemia leads to hyerplasia of bone

marrow cavity and thinning of the bone marrow

cortex as the bone marrow attempts to

compensate for the anemia, leading to fractures

Thalassemia clinical manifestations

RBC

Hypochromic and microcyticchanges

Folic acid deficiency

Frequent epistaxis

Skeletal changes

Osteoporosis

Risk of fractures

Delayed growth

Facial deformities

Heart

Chronic congestive heart failure

Myocardial fibrosis

murmurs

Liver/gallbladder

Hepatomegaly

Hepatic insufficiency

Spleen

splenomegaly

Endocrine system

Delayed sexual development

Fibrotic pancreas

Diabetes risk

Skin

Darkening of the skin

Treatment goal

• Maintain normal hemoglobin levels

• Frequent transfusions

– Transfusion reactions

– Hemochromatosis

• Chelation to prevent iron overload

• Bone marrow transplants

– Now improved with stem cells from siblings

Problems of White Blood Cells

Leukemia

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Leukemia

• Most commonly diagnosed malignancy in

children under 14

• Cancer of the blood or bone marrow,

characterized by an abnormal proliferation of

blood cells, usually white blood cells

Childhood Leukemia

• Acute Lymphocytic Leukemia (ALL) 75% of

leukemias in children

– Peak age of onset is 2-4 years

– Rapid proliferation of immature blood cells which

makes the bone marrow unable to produce

healthy blood cells.

• Acute nonlymphocytic (ANLL) or Acute

Myelomonodcytic leukemia

Leukemia

Initial presentation• Symptoms are related to infiltration of blasts in th bone marrow, lymphoid

system, and extramedullary sites (CNS)

• Fever (60%)

• Fatigue (50%)

• Pallor (25%)

• Weight loss (26%)

• Bone pain (23%)

• Disruption of normal hematopoiesis

• Thrombocytopenia (<100,000) 75%

• Hemoglobin less than 7 g/dL (40%)

• WBC can be either elevated (>50,000) in 20% or – Decreased (< 500)

• Lymphadenopathy, splenomegaly

• CNS involvement (<5%)– Headache, vomiting, papilledema

Leukemia: Treatment

• Induction chemotherapy: kill as many

leukemic cells as quickly as possible (over a 4-

6 week period = attain remission

– vincristine

– prednisone

– L-asparaginase

– daunomycin

Leukemia: Chemotherapy phaseII

CNS Prophylaxis:

• Since leukemic cells sometimes infiltrate the CNS and

accumulate in the brain, and drugs used for

induction chemotherapy do not penetrate the

“blood-brain” barrier in sufficient quantities to

destroy leukemic cells in the brain, most ALL patients

undergo radiation and/or “intrathecal”

chemotherapy .

• ARA-C

• Methotrexate

Leukemia Chemotherapy: Phase III

Consolidation Chemotherapy

• Given to reduce the risk of relapse

• One to six courses of chemotherapy

– L-asparaginase

– Doxorubicin

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Leukemia Treatment:

Delayed Intensification

• Chemotherapy

– Vincristine

– ARA-C

– Cyclophosphamide

• Targets the leukemic cells that have survived.

Leukemia Chemotherapy: Phase IV

Mainenance Chemotherapy

• Begins after the completion of remission

induction and consolidation therapy.

• Combined drug therapies are used

• CBC’s are measured and periodic bone

marrow aspirates are performed to be sure

the child remains in remission

• Prognosis after relapse depends on timing of

relapse (after 3 yrs= good prognosis; less than

18 months < 5% chance of survival)

Follow-up therapy

• Supportive care may include

– IV nutrition

– Antibiotic prophyllaxis

Leukemia:

Bone Marrow Transplantation

• Option for patients who achieve a complete

remission following standard chemotherapy

and subsequently relapse.

• Three types of BMTs:

– Allogenic donor

– Syngeneic identical twin

– Autologous pt’s own bone marrow

Bone Marrow Transplant:

Complications• Infection (CMV)

– special air filters

– screening blood products

– staff and visitors use protective clothing

– no live plants, fruits, vegetables in pt’s room

– prophylactic antibiotics

• graft vs host disease (20-50%)

– donor’s bone marrow begins attacking organs

and tissues in pt’s body

Nursing Management

• Difficult due to multisystem effect

• Long period of treatment required

• Assessment complete and thorough

– Observe signs of bleeding

– Signs of infection

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Nursing Management (continued)

• Monitor for toxic side effects of chemotherapy or tumor cell lysis

– Renal function

• Specific gravity– I & O

– Daily weight

– Special attention for children on cyclophosphamide

• Nutrition

– Daily intake, nausea and vomiting,

– constipation

• CNS infiltration

Nursing Management (continued)

• Pain

• Bone marrow suppression

– Isolation and transmission precautions

• Education of family and child

– Careful handwashing

– Prevention of spread of infection

– Oral care

• Nurses are vital to the multidisciplinary team

Bleeding disorders

• Hemophilia A (factor VIII deficiency)

• Hemophilia B (factor IX deficiency)

• Von Willebrand Disease

• Others

Hemophilia

• Hereditary bleeding disorder

• Deficiency in clotting factors (factor VIII is most common)

• X-linked, most expressed in males, females carrier status

• Manifestations range mild to moderate to severe and is consistent between family members

• About 30% are new mutations

Degrees of severity of Hemophilia

• Normal factor VIII level= 50-150%

• Mild hemophilia

– Factor VIII or IX level is 6-50%

• Moderate hemophilia

– Factor VIII or IX level is 1-5%

• Severe hemophilia

– Factor VIII or IX is < 1%

Clinical manifestations

• Spontaneous bleeding

• Hemarthrosis: Joint bleeding

• Deep tissue hemorrhage

• Nosebleeds

• Hematuria

• Easy bruising

• Life-threatening bleeding

• Head/ intracranial

• Neck and throat

• Abdominal/ GI

• Iliopsoas muscle with decrease hip range of motion

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Complications of bleeding

• Flexion contractions

• Joint arthritis

• Chronic pain

• Muscle atrophy

• Compartment syndrome

• Neurologic impairment

Hemophilia treatment

• Goal control bleeding by replacing the missing clotting factors

– On demand

– prophyllaxis

• IV infusions consist of

– Plasma

– Fresh frozen plasma

– Cryoprecipitate

– Factor VIII

Prophylaxis

• Scheduled infusions of factor concentrates to

prevent most bleeding

• Frequency is 2-3 times per week to deep

trough factor VIII or IX levels at 2-3%

DDAVP (Desmopressin acetate)

not to be confused with DDAVP for

nocturnal enuresis• Synthetic vasopressin

• Method of action

– Release of stores from endothelial cells raising

factor VIII and vWD serum levels

• Administration

– IV

– Subcutaneously

– Nasally (stimate)

• Side effects

Amicar

(epsilon amino caproic acid)

• Antifibrinolytic

• Uses

– Mucocutaneours bleeding

– 50-100mg/kg q 6 hours

• Contraindications

– hematuria

Complications of treatment

• Inhibitors/antibody development

– IgG antibody to infused factor VIII concentrates which occurs after exposure to the extraneous VIII protein

– 20-30% of patients with severe hemophilia A

• Hepatitis A

• Hepatitis B

• Hepatitis C

• HIV

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Nursing considerations

• Factor replacement to be given on time

• Lab monitoring as ordered

• Increase metabolic states will increase factor requirements

• Factor coverage for any invasive porcedures

• Document- infusions, response to treatment

• NO NSAIDS

• NO HEAT

• NO IM INJECTIONS

• Utilize Hemophilia Center staff for questions

Psychosocial issues

• Guilt

• Challenge of hospitalizations

• Control issues

• Financial/insurance challenges

• Feeling different/unable to do certain

activities

• Counseling needs

Von Willebrand Disease

• Autosomal dominant trait

• Equal expressivity in males and females

• Manifestations

– Easy bruising

– Epistaxis

Clotting Disorders and Bleeding

Tendencies (continued)

• Idiopathic thrombocytopenic purpura (ITP)

– Autoimmune disorder

– After a viral illness

– Manifestations

• Ecchymoses

• Petechiae

• Purpura

• Bleeding from gums

• Nosebleeds

• Blood in urine

• Blood in stools

Nursing Care of a Child with a

Hematologic Disorder Is Based on

the Disorder• RBCs

– Oxygenation

– Circulation

– Fluid

– Nutrition

– Pain management

Nursing Care of a Child with a

Hematologic Disorder Is Based on

the Disorder (continued)

• WBCs

– Infection

– Oxygenation

– Nutrition

• Platelets and bleeding disorders

– Bleeding

– Oxygenation

– Circulation

– Injury prevention

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Collaborative Care Approach for a

Child with a Hematologic Disorder

• Team approach

• Family involved

– Decisions with family and child

Hematopoietic Stem Cell

Transplant (HSCT) • Treatment for disorders unresponsive to other

theapy

• Pretransplant phase

– Total body irradiation

– Strict isolation

• Transplant phase

– Intravenous transfusion of donor stem cells

– Transplant starts to grow two to four weeks

Hematopoietic Stem Cell

Transplant (HSCT) (continued)

• Posttransplant phase

– Lasts several weeks

– Major risk is infection

– Immunosuppressive agents prevent graft-versus-host disease

• Nursing care

– Disease prevention

– Prevent infections

– Injury prevention

Hematopoietic Stem Cell

Transplant (HSCT) (continued)

• Nursing care

– Growth and development

– Nutrition

– Physical activity limits

– Oral health

– Mental and spiritual health

– Family and social relationships