Iron-Deficiency Anemia Sickle Cell Anemia Beta-Thalasemia Major (Cooley’s anemia) Hemophilia A ...

Iron-Deficiency Anemia Sickle Cell Anemia Beta-Thalasemia

Major (Cooley’s anemia) Hemophilia A Von Willebrand’s Disease ITP (Immune Thrombocytopenic Pupura)

Acute Lymphocytic Leukemia Hodgkin’s Disease Non-Hodgkin’s Lymphoma Retinoblastoma Neuroblastoma Nephroblastoma Osteogenic Sarcoma Ewing’s Sarcoma

WBC

RBC

Hgb

Hct

Platelet

WBC◦ Neutrophils- phagocytosis◦ Lymphocytes – T and B cell◦ Monoocytes – phagocytosis, antigen◦ Eosophils- allergen ◦ Basophils-inflammatory

RBC◦ MCV- volume◦ MCH◦ MCHC◦ RCW- width

Hgb Hct Platelet

◦ MPV

PT/PTT Sed Rate (ESR) Iron TIBC (Transferrin) Ferritin Bilirubin

RBC: 3.89-4.96 HgB: 10.2-13.4 Hct: 31.7-39.3% Sed: 1-8 WBC: 5,400-11,000

Platelets: 206,000-403,000

Fe: 20-105 Ferritin: 47-110 TIBC: 240-508 PT: 10-11 sec PTT: 42-54 sec Bilirubin- less than

11.7

Reduction of:◦number of red

blood cells◦the quantity of

hemoglobin◦the volume of

packed red

Iron-Deficiency Anemia

Sickle Cell Anemia Beta-Thalasemia

Major (Cooley’s anemia

)

A nutrient deficiency of inadequate dietary iron

The most common hematologic disorder of infancy and childhood

Peaks at 9 months- 2 years, adolescence

Prevented by use of iron fortified productsn

low birth weight infants

infants born to mothers with iron deficiency anemia

infants born with GI defects

chronic blood loss in older children

Dietary Fe is bloodstream binds to transferrin (TIBC) and is delivered to RBC in bone

Marrow, combines with other cells to make Hgb

Unused dietary Fe is stored in intestinal epithelial cells as ferritin

Dietary history usually shows abnormally high milk intake > 32 oz day in toddler

Ask parents specific questions

Begin the dietary history at the time the child awoke yesterday; include all activities and exactly what the child ate

Low RBC Low HGB

◦ Mild ( < 10.2), Moderate (8-9), Severe (< 7) Low HCT Low Iron High Transferrin (TIBC) Low Ferritin

Low Hgb=low O2 tissue perfusion

Hgb of 10.2 or less◦ May seem asymptomatic, not noticed by caregiver◦ Pallor/Pale mucous membranes (low hgb, not

enough red color to skin)◦ Poor muscle tone, decreased activity◦ Fatigue◦ Increased HR, RR

Hgb < 9◦ Above symptoms plus irritability, lack of interest in

play

Consume Iron-fortified formula

Limit cow’s milk to 24-32 oz/day for children >12 months

Increase age-appropriate iron-rich foods and Vit C

May be ordered to take Fe supplements- Ferrous Sulfate

Iron-Rich Foods Vitamin C Rich Foods

Meats, fish, poultry Orange juice

Vegetables Citrus fruits

Dried fruits Strawberries

Legumes Tomatoes

Enriched grain products Broccoli

Whole grain cereal Leafy Green vegetables

Iron-Fortified Cereal Potatoes

Manage side effects of Ferrous Sulfate◦ Nausea,◦ Anorexia◦ Constipation◦ Abdominal distress◦ Black stools.

Give on an empty stomach if possible

Monitor bowel movements and suggest increased fluid and fiber.

Monitor development, sleep, and activity/fatigue patterns

Monitor hemoglobin to measure effectiveness of therapy

Instruct families to keep Ferrous Sulfate locked and out of reach of children; poisoning is a serious risk

Autosomal recessive disorder Seen in African Americans Characterized by abnormal hemoglobin

(HbS) Clinical manifestations caused by

obstructions due to the sickled RBC’s and destruction of sickled and normal RBC’s

Can be diagnosed in-utero

Symptoms may not appear until 6 months of age

Mortality rate children < 3 years old is 35%

PallorFatigueSOB Irritability

Jaundice

Moderately low Hcb and Hct

Normal Iron, TIBC, Ferritin

Elevated Billirubin

1. Vaso-Occlusive Crisis

2. Acute Chest Syndrome

3. Splenic Sequestration

Severe, sudden onset of sickling where many new sickled cells pool in a vessel and cause pain and tissue hypoxia

Caused by: infection, dehydration, anxiety, cold

Most common from hypoxia secondary to rapidly destroyed RBC

Lasts for hours to weeks

Early Signs◦ fever◦ tachycardia◦ pallor

Late Signs◦ abdominal pain◦ back pain◦ extremity pain

First Crisis in infants ◦ Dactylitis (hand &

foot syndrome) swelling of hands and

feet joints may be warm &

swollen

Pain reliefPrevent/Treat occulsionsAdequate oxygenation

Assess pain every 1-2h or more frequently

Use pain scale appropriate for age

Non-pharmacological pain methods

AROUND THE CLOCK PAIN MEDS

Tylenol for mild pain

Narcotics for mod-severe pain

Prevents and treats occlusion

Push PO fluidsIV hydration 1.5 to 2 times normal rate

Risk for fluid overload◦Listen for crackles

Administer oxygen Maintain saturation of 95% or higher

Semi-fowler’s position

Sickle contents break off

Bilateral pulmonary involvement

Causes chest infection, embolism

Symptoms Chest pain Fever Cough Wheeze Tachypnea

Analgesics Oxygen Hydration Incentive

spirometry Antibiotics PRBC

Sickled cells block the spleen

Blood pools in spleen and/or liver and enlarges

Pooled blood leads to a decrease in circulating volume

Can lead to hypovolemic shock

Symptoms◦ Irritability◦ Pale◦ Tachycardia◦ Pain to LUQ◦ Enlarged Spleen

Life Threatening- get child to ED a.s.a.p.!

PRBC Remove spleen

Risk for Infection r/t Chronic Immunosuppression

Administer PCN everyday Up-to-date vaccines Educate parents

◦ Signs of infection & respiratory distress◦ possible triggers◦ treat pain immediately◦ adequate fluids

(Cooley’s Anemia)

Hereditary anemia due to abnormal synthesis of hemoglobin

Life long disorder

Mediterranean descent

Life threatening symptoms

Low RBC’s

Extremely low Hgb < 5

Increased serum iron

Facial anomalies ◦ Frontal bossing

(prominent and protruding forehead)

◦ Maxillary prominence◦ Wide-set eyes with a

flattened nose Bronze skin color

(Greenish yellow skin tone)

Growth and maturation retardation

RBC transfusions q 2-4 weeks

Iron Chelation therapy ◦ Desferal (deferoxamine) SQ

Splenectomy

Cure isbone marrow stem cell transplant

Estimated 70% do not find a suitable donor

◦ Observe for complications of transfusion- iron

overload

◦ Supporting the child and family in dealing with a chronic life-threatening illness

◦ Monitor Growth and Development

◦ Refer the family for genetic counseling

Iron DeficiencyIron Deficiency Sickle CellSickle Cell Thalasemia Thalasemia

•Low RBC’sLow RBC’s•Low HCTLow HCT•Low Hgb Low Hgb •Low ironLow iron•Low ferritinLow ferritin•High TIBCHigh TIBC

•Low RBC’sLow RBC’s•Low HCTLow HCT•Mod low Hgb Mod low Hgb •Normal ironNormal iron•Normal Normal ferritinferritin•Normal TIBCNormal TIBC•Inc BilirubinInc Bilirubin

•Low RBC’sLow RBC’s•Low HCTLow HCT•Very low Hgb Very low Hgb •Increased ironIncreased iron•Normal Normal ferritinferritin•Normal TIBCNormal TIBC

Host of factors

Platelets aggregation at site of injury

Tested by coagulation time (PT/PTT)

Hemophilia A

Von Willebrand’s Disease

ITP (Immune Thrombocytopenic Pupura)

Hereditary blood coagulation deficiency (factor 8)

Ability to clot is slower

X-linked recessive (white, males)

Recent traumas and measures used to stop bleeding

Length of time pressure was applied before bleeding subsided

Whether swelling increased after surface bleeding subsided

Whether swelling and stiffness occurred without apparent trauma

Above History

Suspected by Labs:◦ Platelet level: Normal◦ PTT: Prolonged (elevated number) > 60

Confirmed by genetic testing for missing factor

Vary according to concentration of factor 8

Soft tissue bleeding and painful hemorrhage into joints

Severe bleeding may occur in GI tract, peritoneum or CNS

Acute therapy Acute bleeding stopped by IV

administration of factor 8 Pressure to laceration

Prophylactic therapy PO factor 8 replacement on a regular

schedule if frequently symptomatic (prior to surgery, dental work)

Primary Goal is Injury Prevention Promote oral hygiene, up to date

immunizations No aspirin Avoid activities that induce bleeding Provide activities for normal G&D Administration of factor replacement

prn

Most commonly inherited bleeding disorder, autosomal dominant (Males and Females)

Lacks production of VWF

Platelets are normal in number

Inability of platelets to aggregate

Varying degrees of disease◦VWF is deficient to defective

Platelets is normal

PT/PTT is normal

Confirmed by genetic testing for VWF

Can be so mild that disease is undiagnosed

Bleeding from gums

Epitaxis Prolonged

bleeding from cuts

Excessive bleeding following surgery

Primary Goal: Injury Prevention

Prophylactic therapy◦Replace dysfunctional factor in blood when bleeding with DDAVP

Autoimmune disorder (antiplatelet antibody) or cause is unknown (idiopathic)

Occurs most commonly at age 2-4 years

Reduction in and destruction of platelets

Typically seen 2 weeks after a febrile, viral illness

Excessive bruising and petechiae

Epitaxis Bleeding into

joints Tourniquet test:

shows many petechiae after inflation of BP cuff

Platelets < 150 (Marked thrombocytopenia)

PT and PTT is Normal

PLT transfusion (only a temporary solution) Injury Prevention Avoid when possible:

◦ administering intramuscular injections◦ aspirin, aspirin-containing products, and

nonsteroidal antiinflammatory medications (e.g., ibuprofen)

◦ taking temperatures rectally

Hemophilia AHemophilia A VWFVWF ITPITP

Normal PlateletsNormal Platelets

Elevated Elevated PT/PTTPT/PTT

Normal PlateletsNormal Platelets

Normal PT/PTTNormal PT/PTT

Very Low Very Low PlateletsPlatelets

Normal PT/PTTNormal PT/PTT

Cancer in adults ◦ abnormal cell is transformed by genetic

mutation of its DNA◦ usually as a result from exposure to a tetragon

Cancer in children◦ usually arises from chromosomal abnormalities,

genetic mutations and proliferation of embryonic cells

Surgical intervention

◦ Removing the entire cancerous tumor

◦ Most ideal and frequently used treatment method

Chemotherapy

◦destroy tumor cells by cause cell death

◦normal cells that have rapid growth are also affected, such as hair growth

◦toxic side effects

Radiation therapy

◦ Least preferred treatment in children

◦ Interrupt cellular growth by breaking the DNA stands, leading to cell death

Small percentage Carcinoma (opposed to large percentage in adults)

Mostly Leukemia

Followed by Lymphoma

The rest is solid or soft tissue tumors

Differ based on type of cancer Many symptoms are similar to common

childhood illnesses Symptoms may be in site other than the

cancer =delay in diagnosis Often diagnosis made when cancer is

advanced

Pain Anemia Anorexia, weight loss Infections Bruising Neurological symptoms Palpable mass

Parents in disbelief Health child suddenly becomes ill Potentially life-threatening Treatment decisions, can last months-years Travel for treatment, heavy financial

responsibilities Effects of siblings

Infants- unaware of diagnosis Toddlers- aware they do not feel well Preschoolers-beginning understanding of

illness, not cancer School-age-understand cancer, benefit from

talking about it Adolescents-mature understanding, benefits

from other adolescents with cancer

Provide optimal nutrition- high metabolic rate of cancer depletes stores

Ensure adequate hydration-ice pops, jello Manage pain Promote growth and development Prevent Infection (next slide)

Monitor vital signs q4h Instruct parents how to measure temp

at home Proper handwashing Inspect child’s skin for breakdown Inspect child’s mouth for ulcers Teach child and parents meticulous

oral hygiene No live virus administration

Broad term describing a group of malignant diseases

Normal bone marrow is replaced by abnormal immature cells

Develops from a variety of agents thought to increase risk (virus, toxins, drugs) combined with genetics

Occurs in children< 15 years old (peak 2-6 years old)

Distorted and uncontrolled proliferation of immature WBC’s (lymphoblasts)

Causes decreased RBC’s, platelets, and mature WBC’s production

Invasion of body organs by rapidly increasing lymphoblasts

Physical Exam CBC Changes

Fever Bone or joint pain Bruising Lymphadenopathy Hepatosplenomegal

y

Decreased RBCs Decreased PLTs Very high WBC

Signs & symptomsCBC changesConfirmed with bone marrow aspiration (> 25% of lymphoblast cells present)

High dose chemotherapy administered for 2-3 years

Returns blast cells in bone marrow to less than 5%

Physical assessment findings are normal

Metabolic emergency

Lysis (dissolving or decomposing) of tumor cells

Rapid release of their contents into the blood

Rapid cell destruction releases high levels of◦ uric acid◦ potassium◦ phosphates

Uric acid overloads the kidneys

Leads to cardiac arrhythmias and renal failure

Children receiving chemotherapy monitor for◦Hyperuricemia◦Hyperkalemia◦Hyperphosphatemia◦Hypocalcemia

Administer vigorous hydration (2–4 times rate for maintenance fluid)

Administer allopurinol or urate oxidase (rasburicase)

Reduce conversion of metabolic by-products to uric acid

Hodgkin'sNon Hodgkin's

Retinoblastoma

A malignancy that arises from the lymphoid system

Two types◦Hodgkins◦Non Hodgkins

Neoplasm of the lymph tissue

Affects adolescents to late 20’s

Males > females

Etiology unknown- infectious agent likely

Begins as a single painless enlarged cervical node

Spreads predictably to nonnodal sites◦spleen, liver, bone,

marrow, lungs, mediastinum

As cancer progresses◦Unexplained weight loss◦Unexplained fevers◦Night sweats

Diagnosis Biopsy of enlarged lymph node

Treatment Chemotherapy Radiation-low doses, higher if

physiologically mature Good Prognosis-single origin

Aggressive neoplasm of many lymph nodes

No single origin

Rapid in onset

Affects younger children ages 5-15

Males > females

Cause unknown-infectious agent likely

Multiple enlarged painless lymph nodes

Acute abdominal and chest pain, constipation, cramping

Anorexia, weight loss

As cancer progresses◦ CNS symptoms,◦ Headache◦ Nausea◦ Vomiting◦ Mediastinal mass◦ Petichaie◦ Bruising◦ Bone pain

Diagnosis Biopsy from bone marrow or lymph

node

Treatment Aggressive multi-agent chemo for 2

years Risk for tumor lysis syndrome May need crainal radiation

Hodgkins Non Hodgkins

Males>Females

Late adolescent-20’s

Single origin of cervical gland

Good Prognosis

Males > females

Ages 5-15

No single origin wide-spread involvement

Aggressive treatment- may have poorer progrosis

Malignant tumor of retina

Immature retinal cells become malignant

Affects children 6 weeks of age to preschool age

May be unilateral or bilateral

Absent red reflex

Whitish glow to pupil

Strabismus

Eye pain

Small retinoblastoma◦Cryosurgery ◦Partial vision

Large retinoblastoma and/or metastases brain/ 2nd eye◦Chemo ◦Radiation◦Enucleation◦Eye prosthesis

NeuroblastomaNephroblastoma

OsteoscaromaEwing’s Sarcoma

Solid tumor usually in abdomen

Affects infants to pre-school age children

Cancer cells arise from sympathetic nervous system called crest cells◦ Embryologic cells of adrenal glands

◦ Etiology: unknown

Depend on◦ extent of disease◦ location of tumor

65% of neuroblastomas ◦ protuberant, firm, irregular abdominal mass

that crosses midline

impaired ROM and mobilitypain & limpingrespiratory symptoms

Management◦depends on the presence and extent of metastasis

Malignant tumor of the kidneys

Peak age 3-4 years

Girls > boys

Cause is unknown

Parents usually notice a large, mobile abdominal mass while bathing or the diaper doesn’t fit anymore

Grows extremely quickly, in a matter of days

HematuriaHypertensionAbdominal painFatigueAnemiaFever

1. tumor confined to the kidney and completely removed surgically

2. tumor extending beyond the kidney but completely removed surgically

3. regional spread of disease beyond the kidney with residual abdominal disease postoperatively

4. metastases to lung (primary site), liver, bone, distant lymph nodes

5. bilateral disease

DO NOT PALPATE ABDOMEN ◦ can rupture the tumor and cause spreading of

cancerous cells State 1 and 2

◦Nephrectomy◦Chemotherapy

Stage 3-5◦Nephrectomy◦Radiation◦Chemotherapy

Bone cancer from osteoblasts

Affects adolescents

Attributed to extremity injury or growth spurt

40-50% occur at distal femur and knee

Progressive pain at site of tumor Palpable mass & swelling Limping Limited range of motion Pathological fractures

Remove tumor, prevent spread of disease

Combination of surgery & chemo Amputation my be necessary Limb salvage operation

ComfortInfectionPotential hemorrhagePhantom limb painProsthesisChanges in body image and functioning

Highly malignant tumor in bone marrow

Can present in any bone

Spreads longitudinally through bone

Affects older children and young adolescents

Intermittent pain attributed to injury Swelling at tumor site Pain becomes constant Progresses into

◦ Weight loss◦ Fever ◦ Increased sed rate

Metastases is usually present at time of dx (lungs, bone, CNS, lymph nodes)

Surgery Multi agent chemo

◦ Risk for tumor lysis syndrome Radiation

Osteogenic scaroma Ewing’s Sarcoma

Affects long bones

Older adolescents

Intermittent pain Palpable mass & swelling

Limping, progressive limited range of motion

Pathological fractures

Metastases not as likely

Surgery and chemo

Affects any bone

School-age and adolescents

Intermittent pain becomes constant

Swelling at tumor site

Progresses into systemic symptoms

Metastases likely

Aggressive treatment

Fear Death Anxiety Anticipatory Grieving Hopelessness

Goals for the child

◦ Achieve and maintain normalization

◦ Obtain the highest level of health and function possible

Goals for the family

◦ Remain intact

◦ Achieve and maintain normalization

◦ Maximize function throughout the illness

Attend to the needs of the family system Revise goals frequently to meet the

child’s changing developmental needs Listen carefully to the child's perception

of the condition

Be available to assist both child and family Avoid imposing personal beliefs and expectations Provide time and attention to the dying child Recognize the need to talk about illness

and death Provide adequate pain control, oral care, privacy,

and information about the signs of imminent death After death, allow family members as much time

as they desire with the child

A child is being admitted to the unit with thalassemia major (Cooley’s anemia). In preparing client assignments, the charge nurse wants to assign a nurse to this child who can:

1. Teach dietary sources of iron2. Administer blood infusions3. Work with a dying child4. Monitor the child for bleeding tendencies

A 14-year-old boy with sickle cell anemia is admitted with severe pain in his abdomen and legs. He asks why the doctor ordered oxygen when he is not having any breathing problems. The nurse states the therapeutic action of O2 is:

1. Prevent further sickling2. Prevent respiratory complications3. Increase O2 capacity of RBCs4. Decrease the potential for infection

A 10-year old in the ER has a CBC results that include a Hgb of 8, and Hct of 24. The nurse determines that based on the lab results which nursing action has a high priority?

1. Promotion of skin integrity2. Promotion of hydration3. Promotion of nutrition4. Conserving energy

A 17-year old is being admitted for an amputation related to a bone tumor. The nurse is developing a nursing care plan and determines the most appropriate age related diagnosis is:

1.Risk for disuse syndrome2.Disturbed body image3.Self-care deficit4.Activity related intolerance

◦ WBC 7,200◦ RBC3.01◦ Hgb 9.1◦ Hct 29.3◦ Platelets 371,000

Iron 64 Ferritin 70 Transferrin 250

• Bilirubin 18.2• PTT 45 seconds

After analyzing the results, the nurse suspects the child may have:

1. Fe Deficiency Anemia 2. Cooley’s Anemia 3. Sickle Cell Anemia 4. Aplastic Anemia

The nurse is admitting a child for a swollen elbow. The history indicated multiple bruising. Which of the following laboratory results heightens the nurses suspicion for Hemophilia?

1. Hbg 12,000 2. WBC 9,000 3. Platelets 356,000 4. PTT 73 seconds