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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