Pediatric Hematologic Disorders

1. Anemiaa. most common hem disorder of childhood

b. child develops and adapts when anemia develops slowly
2. Iron Deficiency Anemia-most common, insufficient iron bc of
supply/intake/absorption
a. Iron need is greater in growing children bc of accelerated growth
b. Etiology- leads to smaller, reduced RBCs and quantity of hemoglobin- poor
O2 carry-capacity
i. inadequate stores during fetal development
ii. Deficient dietary intake
iii. Chronic blood loss
iv. Poor utilization of iron by body
c. Risk Factorsi. Premature baby- inadequate iron storage in latter preg
ii. Multiple Birth- inadequate iron storage in latter preg
iii. Children after age 6- intake (neonatal iron depletes)
iv. Bleeding- hemophiliacs, parasitic GI problems or females with
heavy periods(menorrhagia)
d. Assessment Findings- Pallor, paleness of mucous membranes, tiredness,
fatigue, usually seen in infants 6-24 months(time of growth spurt) toddlers
and female adolescents
i. Overweight milk baby
ii. Milk intake more than 32 oz/day
iii. Pica habit (eating non food substances)
e. Labs- decreased Hgb, Low serum iron MCV/serum ferritin, elevated total
iron binding capacity
f. Interventionsi. Dietary modifications
1. Protein and vit C- to produce new cells
2. Folic acid- converts iron from ferritin to hemoglobin
ii. Promote rest, protect from infection (bc higher risk), monitor
cardiac fn
iii. Packed RBCs given slowly(dont want to overload hrt)
iv. Restrict milk intake if necessary
v. Medications- oral iron (ferrous sulfate)
1. pill
a. Best absorbed on empty stomach btwn meals, take
with vit C
b. Milk and antacids inhibit absorption
2. Liquid
a. Stains teeth so drink with straw then brush teeth, foul
aftertaste
3. Stools tarry can be green and constipation may occur
vi. Teach1. Iron admin
a. Infants- iron formula & cereal

b. Meats, dried legumes, nuts, green leafy vegitables,

iron flours
c. Side effects, admin technique, storage(away from kids
fatal)
d. Side Effects Constipation: increase fiber & fluids
3. Sickle Cell Anemia- inherited recessive disorder of Hgb (hemogloinopathy)
a. Risk Factors- A.A, eastern Mediterranian descent, Hispanics
b. Etiology- normal hgb turns into abnormal hgb S
i. Vaso-occlusive crisis: stasis of blood (causes ischemia and
infarction)
1. s/s- fever, severe abd pain and tissue engorgement (hand
and foot), arthralgia, leg ulcers (adolescents), CVA (increased
risk of dehydration)
ii. sequestration crisis- blood pools in spleen (usually younger
children)
1. s/s- profound anemia, hypovolemia and shock
2. [life threatening, splenectomy possible]
iii. aplastic crisis-increased destruction of fragile RBC
1. S/S- profound anemia and pallor
c. Assessment Findings- frequent infections (nonfnl spleen), tired, chronic
hemolytic anemia, delayed physical growth,
i. Swelling hands and feet, protruding abd, jaundice
d. Interventions- pain management
i. Hydration, antibiotics, blood transfusion, warm compress not ice,
oxygen, hydroxyurea,
ii. Diet- high cal, high protein, folic acid supplements
iii. Pain management1. Narcotics (PCA, ATC PRN/breakthrough)
2. NSAIDs (ibuprofen-mild pain; Ketorolac svr pain)
3. DONT GIVE MEPERIDINE-can cause seizures
iv. Vaccines
1. Hep B for blood transfusions
2. Pneumococcal and meningococcal vaccine and annual
influenza vaccine
e. Educate
i. Life long disease(no contact sports)
ii. Prevention of crisis
1. Immunizations2. Manage respiratory infections
3. Avoid dehydration
iii. Importance of adequate rest, avoid heat & cold extremes, avoid
physical and emotional stress
4. Hemophiliaa. Etiology- caused by deficiency in specific clotting factor, inherited X linked
recessive
i. Hem A- factor VIII def
ii. Hem B- factor IX def
b. Risk Factors- MALES babies with heterozygous parents who carry trait
c. Assessments-abn bleeding response, nosebleeds (epistaxis),

i. joint bleeding(hemarthrosis)- cause pain, tenderness, swelling, limit

ROM
ii. tendency to bruise easy, hematuria
d. Complications: extensive bleeding from
i. Circumcision, tooth extractions, lesser injuries and minor surgical
procedures
e. Lab findingsi. Normal: PT, thrombin time, fibrinogen a/nd platelet count
ii. Abnormal: Prolonged aPTT (PTT low), low factor VIII/IX
f. Interventions- Control bleeding, (RICE: rest ice compression elevation)
i. Check neuro for neuropathies
ii. Fresh whole blood- for severe critical areas with bleeding
iii. Fresh or fresh frozen plasma, not platlets
iv. No rectal temps
v. Med- desompressin, whole blood, fresh frozen plasma, factor VIII
concentrate
1. No platelets bc not needed
g. Teachi. No contact sports
ii. Wear medic-alert bracelet
iii. Protective devices
1. while learning to walk
2. Helmets, knee and elbow pads during sports like bicycling
and skating
iv. Toddlers: wear clothes with extra padding as they learn to walk
v. Pad furniture corners, clean environment
vi. Swimming/golf ok non contact sports
vii. AVOID ASPRIN* or ASPRIN like meds
viii. Medication administration techniques
1. Replacement factor may be stored in refrigerator until
needed
2. Teach proper dilution of factor; swirl factor gently until
completely mixed
3. Teach IV admin
4. Use plastic syringe not glass
5. Once replacement factor is reconstituted administer within 3
hours
5. Thalassemia Major- severe anemia req transfusion (Cooleys anemia)
a. Etiology-autosomal recessive condition RBCs are fragile and shorter life
spans
b. Risk factors- Mediterranean descent- Italians, Greeks, Syrians
c. Complications: anemia and hypoxia, excessive iron storage causing
i. Splenomegaly, cardiac complications, gallbladder disease, liver
enlargement and cirrhosis, growth retardation and endocrine
complications, jaundice, skeletal changes- enlarged head, thinkened
cranial bones, enlarged maxilla
d. Assessment- frontal bossing, maxillary prominence, wide-set eyes with
flattened nose, greenish yellow skin tone, hepatosplenomegaly, severe
anemia, microcytic, hypochromic RBS
i. Early sign- pallor and failure to thrive

ii. Osteoporosis, heart murmurs, CHF

iii. Folic acid deficiency may be present
e. Interventionsi. Give blood transfusion
ii. Monitor for iron overload
1. Chelation therapy with Exjade/deferoxamine may be
prescribed to treat iron overload and to prevent organ
damage
iii. If splenectomy- monitor for infection and teach parents bc of sepsis
risk
f. Teach- importance of receiving pneumococcal and meningococcal
vaccines in addition to annual flu vaccine and reg scheduled vaccines
6. Von Willebrands Disease-hereditary bleeding disorder caused by
deficient/defective protein
a. An increased tendancy to bleed from mucous membranes
b. Assessment: epistaxis, gum bleeding, easy bruising, menhorria
c. Interventions: treat and care similar to hemophila including admn of
clotting factors
i. Wear medic-alert bracelet (all bleeding disorders)

HIV/AIDS
1. Care of Child
a. Multidisciplinary approach
i. Prophylaxis
b. Immunizations if child has symptomatic HIV
i. Only inactivated influenza vaccine (IM)
ii. Measles vaccine not give- immunoglobulin may be prescribed
after exposure
iii. Only inactivated polio vaccine (IM)
iv. Rotavirus vaccine not given
v. Varicella zoster maybe after chickpox exposure
vi. Tetanus immunoglobulin for tetanus-prone wounds

Oncologic Disorders
Acquired Health Problems
1. Leukemia- proliferation of immature, abn WBC that depress bone marrow
causing
a. Anemia- from decreased erythrocytes
b. Infection- from neutropenia
c. Bleeding- from decreased platelet production (thrombocytopenia)
d. Cause unknown genetic mutation

2. Risk Factors- Genetic, viral, immunological and environmental factors and

exposure to radiation, chemicals and medications
a. More common in boys than girls after 1 yr
3. Assessment- fever, pallor, fatigue, anorexia, hemorrhage (usually petechiae)
bone and joint pain
a. Pathological fractures can occur
b. Signs of infection common- bc neutropenia
c. Hepatosplenomegaly and lymphadenopathy
d. Normal/elevated/low wbc can be present
e. Increased Intercranial Pressure
i. Infants- poor feeding/high pitched cry/ Macewens sign, tense
fontanel
ii. Children- headache nausea, forceful vomiting, diplopia(blurred
vision) seizures
iii. Personality and Behavioral Signs- irritable, restless, indifference,
drowsiness, inability to follow simple commands
iv. Late Signs- bradycardia, papilledema, decrebrate/decorticate
posturing, alt in pupil size and reaction, dec sense response to
painful stimuli, coma
f. Infection-any break in skin can cause it
g. Lab Test-Bone marrow biopsy specimen
h. Interventionsi. Well balanced diet with meals that req little chewing not irritating to
oral mucosa
1. Tpn or enteral feedings if not eating
ii. Allow adequate rest- dont perform care unless necessary
iii. Provide gentle rectal hygiene
iv. Watch oral mucosa for mucositits- give oral anesthetics
4. Bone Tumorsa. Osteogenic Sarcoma- tumor from bone cell, osteoblast
i. Etiology- most common bone cancer in children, most frequently
affects metaphysis of long bones, esp lower extremities. (femur)
ii. Risk Factors- Adolescent boys, can be genetics
iii. Assessment- pain/swelling,
iv. Interventions- radical resection or amputation, thoracotomy may be
needed for lung
1. Elevate stump for no more than 24 hrs
b. Ewings Sarcoma- malignant small round cell tumor
i. Diaphysial (shaft) portion of long bones1. found: femur, pelvis, tibia, fibula, ribs, scapula, humerus and
clavicle
ii. Etiology- tumor arises in marrow space of bone, often metastasizes
to lung
iii. Risk factors- age 4-25
iv. Assess- pain, soft tissue mass, anorexia, malaise, fever, fatigue, wt
loss, x-ray of affected area,
v. Lab test- radionuclide bone scans and CT scans of chest-assess
metastasis

vi. Intervention1. Encourage mobility as tolerated

2. Allow open communication
5. Neuroblastoma-CONGENTIAL HEALTH PROBLEM
a. Tumor that originates from embryonic neural crest cells- normally grow to
adrenal medulla and sympathetic ganglia
i. Adrenal/retroperitoneal sympathetic chain are common sites
(head/neck/chest/pelvis)
b. Risk factor- under 10 years
c. Assessment- firm, nontender, irregular mass in abd that crosses midline,
urine changes, bone pain, supraorbital ecchymosis, perorbital edema and
exophthalmos
i. Pallor, weakness, irritability, anorexia, wt loss
ii. Respiratory impairment(thoracic lesions)/ neuro impairment
(intercranial lesions)
iii. Paralysis
d. Preop Interventions-s/s of tumor and emotional support
e. Postop- monitor organ complications, and support
6. Brain Tumors
a. Infratentorial- below tentorium cerebelli tumor posterior third of the brain
i. Primarily cerebellum/brainstem
b. Supratentorial tumor- anterior 2/3 of brain- mainly cerebrum
c. Etiology- cause unknown but links to radiation and environmental factors
d. Assessment- headache when waking and get better throughout day.
i. Vomiting unrelated to food
ii. Ataxia
iii. Seizures, behavioral changes, clumsiness (awkward gait/difficulty
walking)
iv. Diplopia, facial weakness
e. Interventions- monitor for signs of increased ICP
i. PREOP-prepare child, assess neuro q4, seizure precautions, shave
head
ii. Postop- monitor for colorless drainage of ears/nose (CSF)
1. Position of head and degree of flexion
2. Complications: hydrocephalus, seizures, sensorimotor
deficits, and endocrine disorders may also need management
3. Maintain nutritional support: vomiting reduces ICP (its ok if it
happens)