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By Jeanie Ward

By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

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Page 1: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

By Jeanie Ward

Page 2: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during
Page 3: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Iron Deficiency Anemia

Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

growth periods

Page 4: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Iron Deficiency Anemia

Manifestations: Pallor; Pale mucus membranes Enlarged spleen and heart Poor muscle tone with decreased activity Fatigue

Diagnosis low hemoglobin (<11g/100mL) Low hematocrit (<33%) RBC are small and hypochromic Serum iron levels are low ( 30μg/mL)

Page 5: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Iron Deficiency Anemia

Treatment Oral iron supplements Ascorbic acid (vitamin C) Diet high in iron

Iron-fortified formula or supplements Limit cow’s milk to 24oz/day for children

>12 months Increase age-appropriate iron-rich foods

Page 6: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during
Page 7: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Definition

A group of genetic disorders characterized by production of elongated, crescent shaped erythrocytes, or abnormal form of hemoglobin, designated as sickle hemoglobin (HbS) in the place of the normal hemoglobin.

Page 8: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Etiology and Pathophysiology

Autosomal recessive inherited disease

Occurs almost exclusively among African Americans

Defect of beta chain of hemoglobin

Disease usually not apparent until 6 months of age – fetal hemoglobin contains a gamma, not a beta chain. Hemoglobin changes from fetal form to adult of HgbA at that time.

Page 9: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Normal vs Sickle Hemoglobin

Disc-shaped Soft (like bag of

jelly) Easily flows

through small blood vessels

Lives for 120 days

Sickle-shaped Hard (like piece

of wood) Often get stuck

in small blood vessels

Lives for 20 days or less

Page 10: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Genetics

Both Parents Have Sickle Cell Trait

25% will have Sickle cell Disease

25% will be normal

50% will have the Trait

Page 11: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

What causes the cell to sickle?

Low Oxygen tension (less than 60%-70%)

Low blood pH (acidosis)

Increased blood viscosity (dehydration, fever, hypoxia)

Page 12: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Result of Sickling ProcessThe sickled RBC’s do not move freely through

the microcirculation.

Blockage in vessel / blood flow halts

Tissue distal to the blockage becomes ischemic

Acute pain, cell destruction, tissue death

Page 13: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Assessment

Page 14: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

General Manifestations Chronic anemia (Hgb. 6-9 g/dl.), pallor,

weakness. Unable to do physical activities because of lack of stamina

Fatigue, malaise Anorexia Jaundice Possible delayed sexual maturation Marked susceptibility to sepsis Possible growth retardation – with long

bones disproportionately long.

Page 15: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Types of Sickle Cell Crisis

Page 16: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Vaso-Occlusive Crisis

Occurs from the pooling of many of the new sickled cells in vessels with resulting tissue hypoxia

May last from one day to several weeks

Page 17: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Manifestations of Vaso-Occlusive Crisis

Bone Pain Most outstanding symptom Mainly in the bones of the extremities

and joints, but can occur anywhere Abdominal and back pain is common Related to necrosis of bone tissue Parents may notice this with refusal to

move an extremity, crying out with joint movement or joint touched.

Page 18: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Hand and foot SyndromeDactylitis

Painful swelling of the hands and /or feet.

May be the first symptom of crisis

Warmth in affected areas.

Fever

Page 19: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Cerebrovascular accident

Vaso-occlusion in the brain results in cerebral infarction which causes variable degreesof neurologic damage

Hemiplegia Aphasia Seizures Vision changes Headaches Alterations in level of consciousness

Page 20: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Acute Chest Syndrome Blockage of blood vesselsin the chest leads to Pneumonia and acute chest syndrome

Chest pains Fever, cough Dyspnea, retractions

Leading cause of death in SCD.

Page 21: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Priapism

Persistent painful erection of the penis occurring when penile blood flow is obstructed.

Page 22: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Hepatomegaly

Enlarged liver with jaundice and hepatic coma.

related to damage to the kidneys

Hematuria

Page 23: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during
Page 24: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Aplastic Crisis

Diminished RBC production resulting in severe anemia

Manifested by: Malaise, lethargy Headache Pallor fainting

Page 25: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Aplastic Crisis Profound anemia- low RBC

count is lifelong. The average RBC life is down to an average of 10-20 days in SCA.

Jaundice, elevated bilirubin Reticulocytosis Bone marrow producing 3-4

times more RBC’s than normal

Page 26: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during
Page 27: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Acute Sequestration Crisis

Sickled cells become trapped in the spleen obstructing blood flow with pooling and enlargement of the spleen.

Leads to shock and hypovolemia

Circulatory collapse and death can occur in less than 30 minutes

Page 28: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Spleen Function

Acts as a filter against foreign organisms that infect the bloodstream.

Filters out old RBC’s from the bloodstream and recycles them

Minor Role: Manufacturers RBC’s toward end of fetal

life and after birth, taken over by bone marrow.Acts as a blood reservoir.

Page 29: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Spleen in the Infant

Soft, purplish-red organ that lies under the diaphragm on the left side of the abdominal cavity

Filters old blood and clears bacteria

Page 30: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Teach Parents How to Measure Spleen

A tongue depressor can be used to measure and track spleen size

Place tip on left nipple and make mark where the spleen tip is felt

Page 31: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Treatment for Sequestration Crisis

Intravenous fluids

Blood transfusion

Spleen removal

Page 32: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Effects of Chronic Crisis States on the Child’s Body

Spleen – more susceptible to infection. Gradual fibrosis and scarring with reduction in spleen activity. Asplenia.

Liver – enlarged, firm, tender Brain – single episode of sickling CVA,

seizures Heart – enlarges and murmurs develop Lungs – pulmonary edema and stasis Kidneys – hematuria, unable to concentrate

urine Bone Infarcts – hands and feet swell.

Page 33: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Effects of Chronic Crisis States on the Child’s Body

Eyes – bleeds in retina.

Leg Ulcers Are seen in 10-15% of older

children May start as simple insect bite

or cut that does not heal With poor circulation develops

into leg ulcer Treated with dressings, leg

elevation and elastic stockings.

Page 34: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Diagnostic Tests

CBC – low hgb and hct

Hemoglobin electrophoresis – used to determine type of hemoglobin. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition. This technique differentiates between normal hemoglobin (A), Sickle hemoglobin (S).

Page 35: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Goals in Care

The is NO CURE for the disease

Treat the Symptoms Relieve the pain

Provide oxygenation

Adequate hydration

Page 36: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Pain Management

Give analgesics as ordered Schedule nursing care to allow for

optimal rest Position joints with pillows Application of warmth Promote circulation through passive

ROM

Page 37: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Oxygenation

Assess Oxygen saturation- pulse oximetry. Should be 95% or >.

Administer Oxygen for short period of time to keep saturation levels up.

Bedrest to minimize energy expenditure and oxygen consumption

Administer blood transfusions

Page 38: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

What are Complications associated with frequent blood

transfusions?

Allergic reaction

Circulatory overload

Iron Overload Give Desferal, an iron-chelating agent,

to decrease the iron levels

Page 39: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Oxygenation

Prevent infection, dehydration Avoid emotional stress, overfatigue Avoid prolonged exposure to heat

and cold Avoid low oxygen environment

Page 40: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Hydration/ Electrolyte Replacement

Encourage fluid intake, at least the minimum amount of fluid required daily for that child’s weight. (1-2x maintenance)

Record I & O. Monitor electrolyte balance Give parents written instructions on

specific amount of daily intake needed. Assess signs of dehydration (decreased

urination, thirst) Teach parents measures to prevent

dehydration such as avoiding heat / stress.

Page 41: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Prevent Infection

With the spleen damaged, has greater chance of getting sepsis.

Avoid know sources of infection Assess Vital Signs and report elevated

temperatures. Treat with Ibuprofen, NOT ASA.

Place on prophylactic antibiotics such as Penicillin VK 250 mg twice a day, through age 5 yrs

Page 42: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Prevent Infection Immunizations

Hepatitis B series Recipient of blood and blood products.

Pneumococcal Pneumovax for now at age 24 months

and 5 years, with an improved vaccine coming on the market soon for administration to infants.

Meningococcal vaccine H. influenzae vaccine

Page 43: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Child / Parent Teaching Assess baseline knowledge and teach

accordingly: Causes of disease and consequences, genetic

counseling Situations that cause sickling Signs of developing crisis, infection, When to call the doctor

Keep in school, promote normal g&d as much as possible

Allow for decreased endurance - let the child set his or her own pace during strenuous exercise, and to take rest breaks when fatigue

Explain some complications: CVA, anemia, swollen spleen, liver problems

Support child and family

Page 44: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Critical Thinking

Which of the following nursing diagnoses should receive priority during a vaso-occlusive crisis in a 14 year old with sickle cell anemia?A. Alteration in comfortB. Ineffective individual copingC. Decreased cardiac outputD. Ineffective airway clearance

Page 45: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Critical Thinking

Which of these instructions should the parents of a child who has recovered from a sickle cell crisis receive?A Isolate child from known sources of

infectionB Avoid contact with all childrenC Restrict child’s intake during the nightD Reinforce the basics of trait

transmission

Page 46: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during
Page 47: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Hemophilia

A group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of the blood. Hemophilia A – most common bleeding

disorder. Deficiency of factor VIII.

Hemophilia is an hereditary condition in which the mother may pass the defect to her male child.

With deficiency of coagulation factor VIII, blood does not coagulate as it should.

Page 48: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Do Hemophiliacs bleed more Profusely than people without Hemophilia?

Page 49: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

The blood of a person with hemophilia does not clot normally. He does not bleed more profusely or more quickly than other people; however, he bleeds for a longer time.

Answer:

Page 50: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Hemophiliacs bleed mainly from minor cuts

True or False

Page 51: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

False This is a myth. External wounds are usually not

serious.

Far more important is internal bleeding (hemorrhaging). These hemorrhages are in joints, especially knees, ankles and elbows; and into tissues and muscles.

When bleeding occurs in a vital organ, especially the brain, a hemophiliac's life is in danger.

Page 52: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

What Causes a Bleeding Episode? Bleeding is often caused by minor injury - a bump or a

slight twist of a joint. However, many hemorrhages, especially among severe hemophiliacs, happen for no apparent reason. This is even truer in joints that have bled often in the past. The more a joint has bled, the easier it bleeds again with no external cause.

Even hemorrhages in the brain often have no apparent cause. Brain hemorrhages are the leading cause of death from bleeding in hemophilia. Therefore it is important to recognize the symptoms of a brain hemorrhage very quickly.

Page 53: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Related to the deficiency of AHF

Page 54: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Interviewing the Child with Hemophilia – Subjective Data

Recent traumas and measures used to stop bleeding

Length of time pressure was applied before bleeding subsided

Was swelling increased after surface bleeding subsided

Did swelling and stiffness occur without apparent trauma

Page 55: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Assessment

Prolonged bleeding anywhere from or in the body.

Often diagnosed when infant is circumcised

Bleeding in the mouth from a cut, bitten tongue or loss of a tooth (especially in children)

Page 56: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Assessment Surface bruising - Bleeding from trauma into

soft tissues and muscles (the ileopsoas muscle around the hip, calf, forearm, upper arm, achilles tendon, buttocks, retroperitoneal.

Hematuria

Hemarthrosis - bleeding into joints (knees, elbows, ankles, shoulders, hips, wrists in descending order of frequency)

Page 57: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

How to Assess Bleeding in a Joint

The first sign is a feeling of tightness in the joint but no real pain. The joint feels a little puffy to the touch.

As the hours pass, the joint becomes hot to the touch. Fully flexing or extending the joint becomes painful. Weight bearing becomes difficult. By this time, the joint is visibly swollen.

As the bleeding continues and the swelling increases, all movement in the joint is lost. The joint becomes fixed in a slightly flexed position in an attempt to relieve the interior pressure in the joint. Pain at this point can be excruciating.

The bleeding slows after several days when the joint is so full of blood that the pressure inside the joint cavity is equal to the pressure inside the broken blood vessels. Slowly, the bleeding stops and the long process of absorbing the blood in the joint cavity begins.

Page 58: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Assessment CNS bleeding - Major cause of death Signs and Symptoms of CNS bleeds

Persistent or increasing headache Repeated vomiting Sleepiness or a change in normal behavior Sudden weakness or clumsiness of an arm or leg Stiffness of the neck or complaints of pain with neck

movement Complaints of seeing double The development of crossed eyes Poor balance when walking, a lack of coordination Convulsions or seizures

Page 59: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Assessment of Child

Hemarthrosis

Bleeding in the mouth

Surface bruising

Page 60: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Diagnostic Tests

DNA testing for the trait Blood Tests

1. Partial Thromboplastin Time (PTT) – Prolonged

2. Bleeding time – Prolonged > 2 hrs.

3. Platelet count and Prothrombin time – Normal

4. Low levels of Factor VIII

Page 61: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Goals

Prevent and control Bleeding

Prevent crippling effects / Decrease pain

Child/ Parent Teaching

Page 62: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Interventions to Prevent or Control Bleeding

The basic treatment to stop or prevent bleeding in people with hemophilia A is:

Give Factor VIII replacement therapy

via IV.

These concentrates come from two sources: human plasma (a component of blood), Fresh whole

blood, fresh or frozen plasma may be used. a genetically engineered (artificial) Factor VIII blood

product made by DNA technology.

Page 63: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

In both cases, the Factor VIII protein is nearly identical to the protein which is lacking in the blood of hemophiliacs. After an infusion of the concentrate, all the proteins needed for clotting are in place. A hemophiliac's blood becomes "normal", at least for a few hours. This allows the time for a clot to form at the site of the damaged blood vessel.

Unfortunately, the replacement of the missing clotting factors is not permanent. Half of the clotting factor activity which was infused is removed by the body every 24 hours. This means that within 3 days almost none is left. The hemophiliac's blood is again unable to clot normally.

Page 64: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

•They are very concentrated. A small amount contains enough Factor VIII to control bleeding, even in major surgery. Thus, they are very effective. •They are convenient. The concentrates can be stored in a home refrigerator for up to a year or kept at room temperature for 3 to 6 months. •They are easy to mix. Five to 10 ml. of sterile water is added to the powdered concentrate. One minute later the mixture is ready to be infused. •They are quick to infuse. From beginning to end, the infusion takes about 15 to 20 minutes. •They are very safe. Hepatitis is rarely, if ever, transmitted by the concentrates made from human plasma and the concentrates made by genetic engineering are even safer because they are not made from human plasma, which can carry blood-borne infections.

Advantages of Factor VIII concentrate

Page 65: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Additional Interventions to Prevent and Control Bleeding

Administration of DDAVP (desmopressin acetate) nasal spray used to stimiulate release of factor VIII. Used with mild hemophilia

Apply local pressure for 10-15 minutes Elevate joint above level of heart Apply cold compresses to promote

vasoconstriction Prophylactic administration of factor VIII

Page 66: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

What is prophylactic vs. on-demand therapy?

In prophylaxis therapy, hemophiliacs receive factor concentrates several times a week to prevent bleeding. The goal is to keep the levels of Factor VIII or IX in the blood high enough that bleeding does not happen. This therapy is common with children.

On-demand therapy is the infusion of factor concentrates immediately after the beginning of a bleed. The goal is to stop the bleeding quickly, before any damage is done to the joint or muscle.

Research has shown that prophylaxis therapy gives children the best chance to reach adulthood without damage to their joints.

Page 67: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Interventions to Prevent Crippling effects / Decrease Pain

During bleeding episode—elevate joint and immobilize

ROM after acute episode Exercise unaffected joints and

muscles. Give analgesics before Physical therapy. Do NOT give Aspirin.

Watch diet/ weight – excessive weight stresses the joints

Page 68: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

What is the crippling Effect of Repeated bleeds into a Joint?

Bleeding in joints, especially knees, ankles and elbows can lead to: loss of range of motion muscle loss destruction of the joints themselves

Page 69: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Child / Parent Teaching

Measures to prevent injury/ providing a safe environment

Early recognition of bleeding episodes

Keep current on immunizations Hepatitis B – because recipient of blood

and blood products

Page 70: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Child / Parent Teaching

How to administer factor VIII. Children often learn how to infuse

themselves at the age of eight or ten. Then, the hemophiliac is able to treat himself at home, at school, at camp or on vacation.

Page 71: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Critical Thinking

A 12 year old hemophiliac child has been admitted to the hospital for hemarthrosis. Which of these expected outcomes should receive priority in the child’s care?A. Family will receive genetic counselingB. Child will participate in appropriate

activities for present conditionC. Child and family will seek support from

National Hemophilia FoundationD. Maximum function of the joint will be

restored

Page 72: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Review

Following administration of factor VIII to a nine year old child admitted to the hospital with hemarthrosis, the nurse’s next action would be to: A. Elevate and immobilize the

affected jointB. Institute passive range of motion to the affected joint during the acute phaseC. Apply warm compresses to the affected jointD. Apply pressure to the area as needed

Page 73: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during
Page 74: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Leading cause of death from disease in children

Almost all childhood cancers involve blood or blood-forming tissues

CancerCancer

Page 75: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Types and Incidence of Cancers

Leukemia

Brain Tumors

Lymphomas

Neuroblastoma

Wilms

Rhabdomyosarcoma

Retinoblastoma

Othes

Page 76: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Brain tumors – second leading cause of death from childhood cancer. Most are cerebellar and brain stem tumors

Lymphomas Non-Hodgkins lymphomas—one-third present with a

mass in the neck or mediastinal area. Also have dyspnea, wheezing, abdominal mass or pain and lymphadenopathy.

Hodgkin’s disease – arises in single lymph node with painless nodal enlargement, followed by extension to adjacent nodes and into spleen, liver, lungs, bone marrow.

Neuroblastoma – malignant tumor arising from sympathetic NS ganglion cells outside the cranium and and can arise from anywhere along the sympathetic nervous system chain. Can also occur in retroperitoneal area, pelvis, neck.

Childhood Cancers

Page 77: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Wilms Tumor – solid tumor of kidney.

Rhabdomyosarcoma—malignant tumor of the striated muscle cells. occur in muscles around eye, head, neck,

extremities, GU system. Retinoblastoma – intraocular malignancy of the

retina of eye. Usually unilateral. If bilateral , hereditary. First sign is white pupil.

Others – osteogenic sarcoma/ Ewings sarcoma – tumor of bones of the trunk. Often seen in adolescence growth spurt. Found in distal femur, proximal tibia.

Page 78: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Intrauterine carcinogens

Physical carcinogens

Viruses

Immune defects

Genetics Discovered

gene for leukemia on chromosome 22

Theories of Etiology

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C = continual unexplained weight lossH = headaches with vomiting (early morning)I = increased swelling of pain in jointsL = lump or massD = development of whitish appearance in

pupilR = recurrent or persistent fevers, night

sweatsE = excessive bruising or bleedingN = noticeable paleness or tiredness

Warning Signs of Childhood Cancer

Page 80: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Biopsy Blood Tests

CBC Uric Acid

Bone Marrow Aspiration PET, SPECT MRI, CT, ultrasound

Diagnostic Tests

Page 81: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Radiation therapy

Chemotherapy

Surgery

Bone Marrow and Stem cell transplantation

Interventions

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Radiation Therapy

Changes the DNA component ofChanges the DNA component of a cell nucleus a cell nucleus The cell cannot replicate which The cell cannot replicate which Inhibits further cell division and growthInhibits further cell division and growth

Page 83: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

Radiation sickness- anorexia, nausea, vomiting Treated with antiemetics (Zofran or Anzimet).

Cool cloth to forehead, provide distraction, accurate I&O.

Fatigue allow for naps an rest periods (coordinate

care), encourage parent to cuddle in bed with child, pillow, blankets, favorite toys

Skin reactions –erythema, tenderness

Effects of Radiation Therapy

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Bone marrow suppression – anemia, neutropenia, thrombocytopenia May be on reverse isolation

Mucositis- inflammation of mucus membranes mainly the mouth

Offer soft foods, and cold foods. Frequent mouth care. Lidocaine oral to

swish in mouth (older child)

Long term – depends on part of body receiving radiation

Page 85: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

There are several categories of antineoplastic drugs used in treating childhood cancers.

Scheduled at set times and days and by different predetermined routes.

May remain in hospital for few days at first, then later report on specific day for therapy.

Children and Parents must be taught about what to do and not to do during therapy.

Chemotherapy

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Bone Marrow Suppression – neutropenia, anemia, thrombocytopenia Place in reverse isolation, keep anyone

exposed to a virus away from patient. Monitor temperature Should not receive live-virus vaccines

Bleeding Tendency (thrombocytopenia) Apply pressure to puncture site No contact sports Check urine and stool for blood Give stool softeners. WHY? Soft objects in mouth

Side effects and Toxic Reactions to Chemotherapy

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Malaise and fatigue Encourage video games, movies, etc Allow visits from friends

Nausea, vomiting, diarrhea, anorexia Give anti-emetics Small frequent meals Monitor for dehydration

Altered mucous membranes Stomatitis Rectal ulcerations

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Renal involvement Uric acid levels rise as a result of breakdown of

cells. The renal tubules causing renal failure. If kidney affected/damaged- chemo drugs will

not be excreted as usual and may limit drugs given.

Body Image changes Alopecia

Pain

Side effects of chemotherapy

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Know OSHA guidelines for administering antineoplastic agents

Confirm all measurements and calculations

Double-check ordered dosages Obtain complete blood count within 48

hours of chemotherapy administration Note white blood cell and platelet levels

before chemotherapy begins Know side effects of chemotherapeutic

agents and ways to alleviate these effects

Nursing Responsibilities Related to Chemotherapy

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Ensure patency of IV tubing by checking for blood return

Ensure needle placement for implantable infusion device

Give vesicants (agents that can cause tissue necrosis) only through a fresh IV site

Have emergency drugs available

Nursing Responsibilities Related to Chemotherapy (Cont’d)

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Chemotherapy Bone marrow

suppression Alopecia Malaise/fatigue Nausea Vomiting Anorexia Stomatitis

Radiation side effects Skin reactions Fatigue Bone marrow

suppression Nausea Vomiting Anorexia Mucositis

Review of Common Side Effects of

Chemotherapy and Radiation

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CurativeRemove the tumor and

cancerous tissue

PalliativeRelieve complications due to

the cancer

Surgery

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The goal of therapy is to administer a lethal dose of chemotherapy and radiation therapy that will kill the cancer and then re-supply the body with bone marrow and stem cells to reconstitute immunologic function.

Healthy bone marrow or stem cells are infused into the bloodstream and migrate to the marrow space to replenish the patient’s immunologic function and help kill remaining cancer cells.

Bone Marrow and Stem Cell Transplantation

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Syngeneic bone marrow comes from identical twin

Allogeneic bone marrow comes from matched sibling (one in

four chances) or someone who is histocompatible.

Autologous own bone marrow. May be harvested at time of

remission in preparation for relapse or when bone marrow is free of malignant cells. Also being used so toxic doses of chemotherapy and radiation can be administered and the bone marrow rescued.

Types of Transplantations

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First --All potential donors are typed for HLA (human leukocyte antigen) compatibility.

Collection of bone marrow is a surgical procedure. The donor undergoes anesthesia for aspiration of the

bone marrow The bone marrow is then processed and frozen

When patient ready - it is infused into the recipient.

Procedure

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Where is the most common site for bone marrow aspiration in children?

Iliac crest

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1. Graft-Versus-Host Disease (GVHD) – potentially lethal immunologic response of donor T cells against the tissue of the recipient. Signs and symptoms – rash, malaise, high

fever, diarrhea, liver and spleen enlargement. Because there is no cure, prevention is

essential. Careful tissue typing, irradiation of blood products which helps to inactivate mature T lymphocytes.

2. Rejection of the transplant

Side effects of Transplantation

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CNS – cognitive disorders, seizures, headaches, coordination problems

Bone – asymmetric growth of bones, easy fractures, scoliosis, kyphosis

CV – cardiomyopathy (pericardial thickening) , pericardial damage

Respiratory – pneumonitis, pulmonary fibrosis GI – enteritis, bleeding, hepatic fibrosis Urinary – hemorrhagic cystitis, reflux Endocrine – decrease in growth, thyroid and

gonadal dysfunction Reproductive – decrease sperm Dental - caries Secondary malignancies 

Post Therapeutic Disabilities

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What is the most common form of childhood cancer?a. leukemiab. brain tumorsc. lymphomad.osteosarcoma

Ask Yourself?

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Leukemia

A malignancy of the blood forming tissues/ bone marrow in which normal bone marrow is replaced by malignant immature WBC’s.

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The WBC's are produced so rapidly that immature cells (blast cells) are released into the circulation.

These blast cells are nonfunctional, can't fight infection, and are formed continuously without respect to the body's needs

The blasts cells then invade other organs and interfere with metabolism / function. The production of red blood cells and platelets decreases leading to anemia and thrombocytopenia.

Pathophysiology of Leukemias

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Leukemia

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Bone marrow Depression results in:1. Decrease in mature WBC’s - fever2. Decrease RBC’s, Anemia- pallor, lethargy, anorexia3. Decreased Platelets/ thrombocytopenia- bleeding4. Increase cell metabolism which deprives cells of

nutrients 5. Enlargement of organs infiltrated with blast cells

results in: Bone pain Spleenomegaly. Hepatomegaly, Nephromegaly Lymphadenopathy CNS infiltration – increased ICP

Signs & Symptoms

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Diagnostic Tests History and Physical

Blood Work Leukocytes are > 10,000 Platelet count, Hgb and Hct low Blast cells appear (where they normally don’t)

Bone Marrow Aspiration Used to identify the type of WBC involved,

therefore, type of leukemia

X-rays of long bones Show lesions caused by invasion of abnormal

cells

Lumbar Puncture – blast cells in the CSF

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Page 109: By Jeanie Ward. Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during

A combination of antineoplastic drugs are given for about a month

A different combination is given for about 2-3 years

Advantages of using a combination of drugs: Decrease resistance to one drug Lessening of severe side effects of massive

doses of one drug Breakdown of the tumor cell cycle at multiple

sites

Chemotherapy

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1. Remission induction- most intense treatment. Large doses of antineoplastic drug administered in an effort to destroy as many proliferating cells as possible. Lasts 4-6 wks. About 95% respond.

2. Consolidation- method of destroying leukemic cells in the CNS- for children who have CNS involvement or are high risk. Given Intrathecal.

3. Remission maintenance- drugs given at specific intervals. If remain in remission for 3 yrs, treatment is discontinued. Approx 80% of children who sustain remission for 2-3 yrs continue to remain in remission and appear to be cured.

Chemotherapy Phases

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Administration of Chemotherapy

When is intrathecal administration of chemotherapeutic medications

required?

Intrathecal chemotherapy is instilled in to the spinal canal for

cancers that have metastasized to the brain

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Bypass the blood brain barrier

What is the rationale for the use of cranial radiation in addition to chemotherapy?

What is the rationale for the use of cranial radiation in addition to chemotherapy?

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Prevent infection (neutropenia, anemia) Pain Relief Nausea and vomiting Mouth discomfort-mucositis / stomatitis Fatigue Alopecia Prevent blood loss-platelet low- nose bleed most

common kind of bleed Support child and family Assist with referrals to social services, home

health agency, chaplain

Nursing Care

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Community Resource Candlelighters Childhood Cancer Foundation® (CCCF)

is a national non-profit membership organization whose mission is to educate, support, serve, and advocate for families of children with cancer, survivors of childhood cancer, and the professionals who care for them.

http://www.candlelighters.org/ American Cancer Society Make a wish Foundation Leukemia Society Church and Schools

Nursing Care

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Caused by the breakdown (lysis) of malignant cells which release intracellular contents into the blood.

Intracellular electrolytes overload the kidneys and can lead to kidney failure.

Further severe electrolyte imbalances cause metabolic acidosis, hyperkalemia, increase uric acid levels, hypocalcemia and cardiac arrythmias.

Treat with IV fluids, electrolyte replacement, allopurinol.

Tumor Lysis Syndrome

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An embryonic tumor of the kidney.

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Cause is unknown

Originates from immature renoblast cells

Tumor is vascular

Etiology and Pathophysiology

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Palpable abdominal mass Firm and smooth

**The abdomen should not be palpated once the diagnosis is made. Avoid palpating the tumor mass during assessment because of the risk of rupturing the protective capsule. Excessive manipulation can cause seeding of the tumor and spread of cancerous cells

Abdominal distention Fever Fatigue Late signs

Anemia Hematuria, dysuria Hypertension

Assessment

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Abdominal ultrasound

CT, MRI

Biopsy

Diagnostic Tests

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Nephrectomy and removal of lymph nodes

Post-op chemotherapy and / or radiation therapy

CT every 6 months for 3 years

Chest x-ray every 3 months for 3 years

Interventions

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Pre-op Sign on bed ”Do Not Palpate Abdomen” Child / Parent teaching

Post-op Monitor kidney function, Strict I & O Monitor vital signs—B/P and temperature Monitor GI function– assess bowel sounds and

stool production NG tube to drainage. Measure abdominal girth.

Nursing Care

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Clinical manifestations

Treatment sequence How does this treatment differ

from osteosarcoma?

Why is metastases more of a concern with this cancer?

Ewing’s Sarcoma

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Clinical manifestations-

Treatment sequence-

Chemotherapeutic agents (VAC)

Rabdomyosarcoma

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Clinical manifestations

Treatment sequence

Why is the sequence of treatment important?

Osteosarcoma

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Pre-operative nursing goals for the child diagnosed with cancer- Disturbed sensory perception Anticipatory grieving Risk for infection

Post-operative nursing goals- Risk for infection Impaired skin integrity Impaired adjustment- disturbed body image Pain

Review of Nursing Care:

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What is the rationale for treating some cancers with chemotherapy prior to removal of the tumor?

What specific teaching should the nurse include as priority regarding chemotherapy?

What lab values does the nurse monitor carefully in the client undergoing chemotherapy?

Review of Nursing Care:

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What are the priority psychosocial needs of the child/family diagnosed with cancer

What are nursing interventions to assist with stressors that influence the child/family’s response to the diagnosis and treatment of cancer.

Review of Nursing Care:

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Caring for a child who is dying can be one of the hardest tasks in nursing

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Children under 3 Have no understanding of own impending death May perceive family anxiety, sadness

Preschool to 5 years More afraid of separation from parents than of

thought of dying. Greatest fear is separation. Envision death as temporary, and have little of

adults’ fear of it Think of it as a long sleep, not a final process.

Nightmares increase. May feel pain / illness is a punishment for

misdeeds or thoughts May ask questions about death In long term illness – may simulate adult

response with depression, withdrawal, fearfulness, anxiety

Understanding of Death

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School Age 5-9 Begin to understand that death is

permanent May think it is something that only happens

to adults Become aware of what is happening to them

when their disorder has a fatal prognosis. Concerns center around fear of pain, fear of

being left alone and leaving parents and friends.

May associate death with sleep and may be afraid to go to sleep without someone near them.

May associate death with darkness—want light left on in room

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Adolescent, older school age By age 10 have an adults concept of death,

realizing that it is inevitable, universal, and irreversible. Have more understanding than adults realize.

Understand that death is the cessation of life. Emotional outbursts may reflect anger View death as fearsome and fascinating

(increase in adolescent suicide). May feel immune to death and deny symptoms

for longer than usual because they believe it is impossible that anything serious could happen to them.

Some adolescents consider themselves alienated from their peers and unable to communicate with their parents for emotional support feeling alone in their struggle.

Understanding Death

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1. Elicit child's understanding of death before discussing

2. Encourage children to express feelings in own way through play, drawings, or verbalization to promote free expression.

3. Provide a safe, acceptable outlet for expressions of feelings

4. Structure care of child to allow child choices and participation in process within constraints of physical condition

5. Help child maintain independence and control; normal ADL as much as possible (set realistic goals)

6. Realize that they will go through the stages of dying: denial, bargaining, anger, depression, acceptance

Nursing Care Child

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1. Spend time with family to listen, answer questions, and provide information. Discuss issues with parents before discussing with child.

2. Provide opportunities for family to express their emotions and deal with their feelings. Parental reactions: continuum of grief process and usually depend on previous experiences with loss, intellectualization.

3. Reactions may depend on relationship with child and circumstances of illness or injury

4. Reactions depend on degree of guilt felt by parents-help them sort out

Nursing Care Parents

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5. Assist parents in expressing fears, concerns, grief to enable them to appropriately support child

6. Assist parents to understand sibling' possible reactions to terminally ill child·   Guilt- believing they caused the problem or

illness·  Jealousy- wanting equal attention from

parents·   Anger- feelings of being left behind

7. Support, enhance parent-child communication, enhance parents' ability to support child

8. Refer to parent, family support groups- not alone, help focus, open communication, provide information

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Nurse needs to care for self.

Care of the caregiver is imperative if the nurse is to provide physical and psychosocial care for families at such a difficult time.

Caring for dying children and their families can be stressful and emotionally demanding.

Nursing Care Nurse