Title: Hematologic-Oncology
1Hematologic-Oncology
2Common Hematologic Disorders in Children
- Iron-Deficiency Anemia
- Sickle Cell Anemia
- Beta-ThalasemiaMajor (Cooleys anemia)
- Hemophilia A
- Von Willebrands Disease
- ITP (Immune Thrombocytopenic Pupura)
3Common Heme-Oncology Diseases in Children
- Acute Lymphocytic Leukemia
- Hodgkins Disease
- Non-Hodgkins Lymphoma
- Retinoblastoma
- Neuroblastoma
- Nephroblastoma
- Osteogenic Sarcoma
- Ewings Sarcoma
4Complete Blood Count
5CBC with Differential
- 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
6Other Labs
- PT/PTT
- Sed Rate (ESR)
- Iron
- TIBC (Transferrin)
- Ferritin
- Bilirubin
7Pediatric Laboratory Normal Values Children age
2-12 Years
- 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
8Anemias
- Reduction of
- number of red blood cells
- the quantity of hemoglobin
- the volume of packed red
- Iron-Deficiency Anemia
- Sickle Cell Anemia
- Beta-ThalasemiaMajor (Cooleys anemia
9Iron Deficiency Anemia
10Iron-Deficiency 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
11Children at Risk
- low birth weight infants
- infants born to mothers with iron deficiency
anemia - infants born with GI defects
- chronic blood loss in older children
12Pathophysiology
- 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
13History
- Dietary history usually shows abnormally high
milk intake gt 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
14Diagnosis
- Low RBC
- Low HGB
- Mild ( lt 10.2), Moderate (8-9), Severe (lt 7)
- Low HCT
- Low Iron
- High Transferrin (TIBC)
- Low Ferritin
15Symptoms
- Low Hgblow 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 lt 9
- Above symptoms plus irritability, lack of
interest in play
16Nursing Considerations
- Consume Iron-fortified formula
- Limit cows milk to 24-32 oz/day for children gt12
months - Increase age-appropriate iron-rich foods and Vit
C - May be ordered to take Fe supplements- Ferrous
Sulfate
17Nursing Considerations
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
18Nursing Considerations
- 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.
19Nursing Considerations
- 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
20Sickle Cell Anemia
21Sickle Cell Anemia
- Autosomal recessive disorder
- Seen in African Americans
- Characterized by abnormal hemoglobin (HbS)
- Clinical manifestations caused by obstructions
due to the sickled RBCs and destruction of
sickled and normal RBCs
22Sickle Cell Anemia
- Can be diagnosed in-utero
- Symptoms may not appear until 6 months of age
- Mortality rate children lt 3 years old is 35
23Signs and Symptoms
- Pallor
- Fatigue
- SOB
- Irritability
- Jaundice
24Diagnosis
- Moderately low Hcb and Hct
- Normal Iron, TIBC, Ferritin
- Elevated Billirubin
253 Complications of Sickle Cell Anemia
- Vaso-Occlusive Crisis
- Acute Chest Syndrome
- Splenic Sequestration
26 Vaso-occlusive crisis
- 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
27(No Transcript)
28Vaso-occlusive Crisis
- 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
29Management
- Pain relief
- Prevent/Treat occulsions
- Adequate oxygenation
30Pain
- 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
31Hydration
- Prevents and treats occlusion
- Push PO fluids
- IV hydration 1.5 to 2 times normal rate
- Risk for fluid overload
- Listen for crackles
32Treat Sickling-Oxygenation
- Administer oxygen
- Maintain saturation of 95 or higher
- Semi-fowlers position
33Acute Chest Syndrome
- Sickle contents break off
- Bilateral pulmonary involvement
- Causes chest infection, embolism
34Nursing Considerations
- Symptoms
- Chest pain
- Fever
- Cough
- Wheeze
- Tachypnea
- Analgesics
- Oxygen
- Hydration
- Incentive spirometry
- Antibiotics
- PRBC
35Splenic Sequestration
- 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
36Nursing Considerations
- Symptoms
- Irritability
- Pale
- Tachycardia
- Pain to LUQ
- Enlarged Spleen
- Life Threatening- get child to ED a.s.a.p.!
- PRBC
- Remove spleen
37Post-Splenectomy
- 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
-
38Beta-ThalasemiaMajor
39Beta-ThalasemiaMajor (Cooleys anemia)
- Hereditary anemia due to abnormal synthesis of
hemoglobin - Life long disorder
- Mediterranean descent
- Life threatening symptoms
40Diagnosis
- Low RBCs
- Extremely low Hgb lt 5
- Increased serum iron
41Symptoms
- 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
42Management
- 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
43Nursing Considerations
-
- 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
44Compare and Contrast
Iron Deficiency Sickle Cell Thalasemia
Low RBCs Low HCT Low Hgb Low iron Low ferritin High TIBC Low RBCs Low HCT Mod low Hgb Normal iron Normal ferritin Normal TIBC Inc Bilirubin Low RBCs Low HCT Very low Hgb Increased iron Normal ferritin Normal TIBC
45Bleeding Disorders
46Clotting
- Host of factors
- Platelets aggregation at site of injury
- Tested by coagulation time (PT/PTT)
47Types of Bleeding Disorders
- Hemophilia A
- Von Willebrands Disease
- ITP (Immune Thrombocytopenic Pupura)
48Hemophilia A
49Hemophilia A
- Hereditary blood coagulation deficiency (factor
8) - Ability to clot is slower
- X-linked recessive (white, males)
50If Suspected ask about
- 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
51Diagnosis
- Above History
- Suspected by Labs
- Platelet level Normal
- PTT Prolonged (elevated number) gt 60
- Confirmed by genetic testing for missing factor
52Symptoms
- 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
53Management of Bleeding
- 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)
54Parental Education
- Primary Goal is Injury Prevention
- Promote oral hygiene, up to date immunizations
- No aspirin
- Avoid activities that induce bleeding
- Provide activities for normal GD
- Administration of factor replacement prn
55Von Willebrands Disease
56Von Willebrands Disease
- 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
57Diagnosis
- Platelets is normal
- PT/PTT is normal
- Confirmed by genetic testing for VWF
58Signs Symptoms
- Can be so mild that disease is undiagnosed
- Bleeding from gums
- Epitaxis
- Prolonged bleeding from cuts
- Excessive bleeding following surgery
59Management
- Primary Goal Injury Prevention
- Prophylactic therapy
- Replace dysfunctional factor in blood when
bleeding with DDAVP
60ITP (Immune Thrombocytopenic Pupura)
61ITP
- 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
62Signs and Symptoms
- Excessive bruising and petechiae
- Epitaxis
- Bleeding into joints
- Tourniquet test shows many petechiae after
inflation of BP cuff
63Diagnosis
- Platelets lt 150 (Marked thrombocytopenia)
- PT and PTT is Normal
64Management
- 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
65Compare and Contrast
Hemophilia A VWF ITP
Normal Platelets Elevated PT/PTT Normal Platelets Normal PT/PTT Very Low Platelets Normal PT/PTT
66Oncology
67Oncology
- 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
68Oncology Treatment
- Surgical intervention
- Removing the entire cancerous tumor
- Most ideal and frequently used treatment method
69Oncology Treatment
- Chemotherapy
- destroy tumor cells by cause cell death
- normal cells that have rapid growth are also
affected, such as hair growth - toxic side effects
70Oncology Treatment
- Radiation therapy
- Least preferred treatment in children
- Interrupt cellular growth by breaking the DNA
stands, leading to cell death
71Types of Cancer in Children
- Small percentage Carcinoma (opposed to large
percentage in adults) - Mostly Leukemia
- Followed by Lymphoma
- The rest is solid or soft tissue tumors
72Clinical Manifestations
- 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
73Common Clinical Manifestations
- Pain
- Anemia
- Anorexia, weight loss
- Infections
- Bruising
- Neurological symptoms
- Palpable mass
74Psychosocial Concerns
- 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
75Effects on Child
- 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
76General Nursing Considerations
- 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)
77Risk for infection r/t Immunosuppressed state.
- Monitor vital signs q4h
- Instruct parents how to measure temp at home
- Proper handwashing
- Inspect childs skin for breakdown
- Inspect childs mouth for ulcers
- Teach child and parents meticulous oral hygiene
- No live virus administration
78Leukemia
79Leukemia
- 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
80Acute Lymphocytic Leukemia
- Occurs in childrenlt 15 years old (peak 2-6 years
old) - Distorted and uncontrolled proliferation of
immature WBCs (lymphoblasts) - Causes decreased RBCs, platelets, and mature
WBCs production - Invasion of body organs by rapidly increasing
lymphoblasts
81Signs and Symptoms
- Fever
- Bone or joint pain
- Bruising
- Lymphadenopathy
- Hepatosplenomegaly
- Decreased RBCs
- Decreased PLTs
- Very high WBC
82Diagnosis
- Signs symptoms
- CBC changes
- Confirmed with bone marrow aspiration (gt 25 of
lymphoblast cells present)
83Management
- High dose chemotherapy administered for 2-3 years
- Returns blast cells in bone marrow to less than
5 - Physical assessment findings are normal
84Tumor Lysis Syndrome
- Metabolic emergency
- Lysis (dissolving or decomposing) of tumor cells
- Rapid release of their contents into the blood
85Tumor lysis syndrome
- Rapid cell destruction releases high levels of
- uric acid
- potassium
- phosphates
- Uric acid overloads the kidneys
- Leads to cardiac arrhythmias and renal failure
86Nursing Considerations
- Children receiving chemotherapy monitor for
- Hyperuricemia
- Hyperkalemia
- Hyperphosphatemia
- Hypocalcemia
87Nursing Considerations
- Administer vigorous hydration (24 times rate for
maintenance fluid) - Administer allopurinol or urate oxidase
(rasburicase) - Reduce conversion of metabolic by-products to
uric acid
88Soft Tissue Tumors
- Hodgkin's
- Non Hodgkin's
- Retinoblastoma
89Lymphomas
- A malignancy that arises from the lymphoid system
- Two types
- Hodgkins
- Non Hodgkins
90Hodgkins Lymphoma
91Hodgkins Lymphoma
- Neoplasm of the lymph tissue
- Affects adolescents to late 20s
- Males gt females
- Etiology unknown- infectious agent likely
92Signs and Symptoms
- Begins as a single painless enlarged cervical
node - Spreads predictably to nonnodal sites
- spleen, liver, bone, marrow, lungs, mediastinum
93Signs and Symptoms
- As cancer progresses
- Unexplained weight loss
- Unexplained fevers
- Night sweats
94Diagnosis and Treatment
- Diagnosis
- Biopsy of enlarged lymph node
- Treatment
- Chemotherapy
- Radiation-low doses, higher if physiologically
mature - Good Prognosis-single origin
95Non-Hodgkins Lymphoma
96Non-Hodgkins Lymphoma
- Aggressive neoplasm of many lymph nodes
- No single origin
- Rapid in onset
- Affects younger children ages 5-15
- Males gt females
- Cause unknown-infectious agent likely
97Signs and Symptoms
- Multiple enlarged painless lymph nodes
- Acute abdominal and chest pain, constipation,
cramping - Anorexia, weight loss
98Signs and Symptoms
- As cancer progresses
- CNS symptoms,
- Headache
- Nausea
- Vomiting
- Mediastinal mass
- Petichaie
- Bruising
- Bone pain
99Diagnosis and Treament
- 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
100Compare and Contrast
Hodgkins Non Hodgkins
MalesgtFemales Late adolescent-20s Single origin of cervical gland Good Prognosis Males gt females Ages 5-15 No single origin wide-spread involvement Aggressive treatment- may have poorer progrosis
101Retinoblastoma
102Retinoblastoma
- Malignant tumor of retina
- Immature retinal cells become malignant
- Affects children 6 weeks of age to preschool age
- May be unilateral or bilateral
103Signs and Symptoms
- Absent red reflex
- Whitish glow to pupil
- Strabismus
- Eye pain
104Treatment
- Small retinoblastoma
- Cryosurgery
- Partial vision
- Large retinoblastoma and/or metastases brain/ 2nd
eye - Chemo
- Radiation
- Enucleation
- Eye prosthesis
105Solid Tumors
- Neuroblastoma
- Nephroblastoma
- Osteoscaroma
- Ewings Sarcoma
106Neuroblastoma
107Neuroblastoma
- 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
108Signs and Symptoms
- Depend on
- extent of disease
- location of tumor
- 65 of neuroblastomas
- protuberant, firm, irregular abdominal mass that
crosses midline
109Signs and Symptoms
- impaired ROM and mobility
- pain limping
- respiratory symptoms
- Management
- depends on the presence and extent of metastasis
110Nephroblastoma
111Nephroblastoma
- Malignant tumor of the kidneys
- Peak age 3-4 years
- Girls gt boys
- Cause is unknown
112Nephroblastoma
- Parents usually notice a large, mobile abdominal
mass while bathing or the diaper doesnt fit
anymore - Grows extremely quickly, in a matter of days
113Other Signs Symptoms
- Hematuria
- Hypertension
- Abdominal pain
- Fatigue
- Anemia
- Fever
114Staging 1 through 5
- tumor confined to the kidney and completely
removed surgically - tumor extending beyond the kidney but completely
removed surgically - regional spread of disease beyond the kidney with
residual abdominal disease postoperatively - metastases to lung (primary site), liver, bone,
distant lymph nodes - bilateral disease
115Treatment
- 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
116Bone Cancers
117Osteosarcoma
118Osteosarcoma
- Bone cancer from osteoblasts
- Affects adolescents
- Attributed to extremity injury or growth spurt
- 40-50 occur at distal femur and knee
119Signs and symptoms
- Progressive pain at site of tumor
- Palpable mass swelling
- Limping
- Limited range of motion
- Pathological fractures
120Management
- Remove tumor, prevent spread of disease
- Combination of surgery chemo
- Amputation my be necessary
- Limb salvage operation
121Nursing care Post-Op
- Comfort
- Infection
- Potential hemorrhage
- Phantom limb pain
- Prosthesis
- Changes in body image and functioning
122Ewings Sarcoma
123Ewings Sarcoma
- Highly malignant tumor in bone marrow
- Can present in any bone
- Spreads longitudinally through bone
- Affects older children and young adolescents
124Signs and Symptoms
- 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)
125Treatment
- Surgery
- Multi agent chemo
- Risk for tumor lysis syndrome
- Radiation
126Compare and Contrast
Osteogenic scaroma Ewings 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
127Care of the Chronically Ill Child
128Chronically Ill ChildNursing Diagnosis
- Fear
- Death Anxiety
- Anticipatory Grieving
- Hopelessness
129Goals for Care of the Chronically Ill Child
- 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
130Nursing Care for Children with Chronic
Conditions and Their Families
- Attend to the needs of the family system
- Revise goals frequently to meet the childs
changing developmental needs - Listen carefully to the child's perception of the
condition
131Nursing Care for the Dying Child and the Childs
Family
- 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
132Practice Questions!
133- A child is being admitted to the unit with
thalassemia major (Cooleys anemia). In preparing
client assignments, the charge nurse wants to
assign a nurse to this child who can - Teach dietary sources of iron
- Administer blood infusions
- Work with a dying child
- Monitor the child for bleeding tendencies
134- 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 - Prevent further sickling
- Prevent respiratory complications
- Increase O2 capacity of RBCs
- Decrease the potential for infection
135- 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? - Promotion of skin integrity
- Promotion of hydration
- Promotion of nutrition
- Conserving energy
136- 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 - Risk for disuse syndrome
- Disturbed body image
- Self-care deficit
- Activity related intolerance
137The nurse is reviewing the lab work on a child
admitted for fatigue
- 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
138- After analyzing the results, the nurse suspects
the child may have - 1. Fe Deficiency Anemia
- 2. Cooleys Anemia
- 3. Sickle Cell Anemia
- 4. Aplastic Anemia
139- 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