CPN Exam Hematologic/Oncologic/Cardiovascular Flashcards
Anemia
A decrease in the serum hemoglobin– Acute or chronic
Anemia Manifestations
Acute anemia (acute tissue hypoxia) – muscle weakness, fatigue, pallor, headache, lightheadedness, increased HR
– Chronic anemia (compensated) – growth retardation, delayed sexual maturation, increased HR, heart murmur
Anemia Management Acute
Iron deficiency = give iron supplementation
– Blood transfusion is generally required with a hemoglobin
< 7.0-8.0
– Iron supplementation is generally used with a hemoglobin > 7.0-8.0
Anemia Management Chronic
Blood transfusion is generally not considered until hemoglobin is ≤ 6.0
Iron Deficiency Anemia
Most prevalent nutritional disorder in U.S.
Lack of iron leads to small RBC, that breaks apart and does not carry oxygen well
Kids at risk for Iron Deficiency Anemia
Premature infants and multiple birth infants
- Fetal transfer of Iron = Highest rate in last trimester
-Full term infant has 6 months iron stores
Iron Deficiency Anemia Assessment
Assessment of nutritional status/eating habits
– Identification of excessive milk intake or early introduction of whole milk
- Whole milk is a poor iron source
Iron Supplementation Instructions
Temporarily stains teeth – give with medicine dropper, brush teeth afterwards
– Absorption is best between meals and given with a citrus fruit or juice (i.e. orange juice)
– Stool should be a tarry green or black when iron levels are adequate
Sickle Cell Disease
Group of genetic disorders of hemoglobin production characterized by a predominance of the abnormal Hgb S
Sickle Cell Disease Pathophysiology
– RBCs sickle and get trapped/stuck in vasculature
-Caused by dehydration and hypoxia
– RBC Lifespan = 20 days (reduced from 90-120)o Chronic hemolytic anemia
Caused by vaso-occlusive crisis (VOC)
Sickle Cell Disease Assessment
Developmentally appropriate pain scale
Sickle Cell Disease Interventions
RBCs Un-sickle with adequate oxygen and hydration
-Hydration (IV/PO)
-Oxygenation
Pain Management
-Pharmacologic
-Non-Pharmacologic
Sickle Cell Disease Prevention
Promote hydration
Monitor for dehydration
Avoid/minimize risk factors
– Extreme heat/cold
– Hypoxia
Fever in Sickle Cell Disease
Fever is the first sign of bacteremia in the child with SCD (child must be medically evaluated)
Caused by splenic failure (↓ ability to fight infection)
Fever in SCD Management
History, physical, vital signs, blood cx, CBC
Prompt IV Antibiotics
Monitoring for s/s of sepsis
Patient and family education– temperature taking– when to call health care provider
Fever prevention in SCD
Penicillin prophylaxis - PCN PO BID
Pneumococcal Vaccine
Immune/Idiopathic Thrombocytopenic Purpura (ITP)
Definition: An acquired hemorrhagic disorder that results in the autoimmune destruction of platelets in the spleen
ITP Diagnosis
CBC, other lab work, bone marrow aspiration
-80% of children:
– are between 2 and 10 years of age
– recover completely within 6 months
ITP Assessment
Platelet count typically < 20,000
Assess for petechiae
Note bruises and other bleeding (urine and gums)
ITP Management
Steroids (to reduce platelet destruction; 2-3 week response) Intravenous Immunoglobulins (IVIG)– 3-6 hour IV administration; 10-12 hour response; very expensive Splenectomy for chronic, unresponsive ITP
Bleeding Precautions
– Avoid: trauma, injections, ibuprofen– Provide safety measures (no contact sports)
Hemophilia
A group of bleeding disorders in which there is a deficiency in one of the factors necessary for coagulation of blood
Hemophilia Pathophysiology
Severe: spontaneous bleeding
Moderate: bleeding with trauma
Mild: bleeding with severe trauma or surgery
Hemophilia Management
Assess for signs of bleeding
Give Factor VIII or IX
Immobilize and elevate joint/affected area
Monitoring for bleeding complications
Range of motion exercises after acute injury/bleeding subsides
Prevent bleeding/injury based on developmental risk
PRBC’s Administration
Take VS prior to starting
Check right patient, right blood type, and same type and cross match between patient and donor
IV NS to piggyback tubing
Administer 20% transfusion slowly
Use appropriate filters
Stay with patient
Assess for reaction
Transfusion Reactions
– Hemolytic - Flank pain, severe headache, dyspnea, shock and renal failure, fever/chills
– Allergic - Hives and itching– Febrile - Fevers and chills
Transfusion Reaction Interventions
Stop the transfusion
– Maintain the patent IV using normal saline
– Monitor vital signs
– Contact MD/PNP
Leukemia
1 type of cancer in children
Is an abnormal proliferation of white bloods cells invading the bone marrow causing:
– Bone pain (from bone marrow hypertrophy)
– Anemia
– Bleeding Disorders (Petechiae and Ecchymosis)
– Lymphadenopathy
– Immunosuppression
Acute Lymphoid Leukemia
Acute Lymphoid Leukemia (ALL)
– Most common type of leukemia and cancer in children
– Peak age is 2-5 years
– > 85% cure rate
– Death usually follows overwhelming infection
Acute Myelogenous Leukemia
More common than ALL in adolescents
– 60-70% cure rate
– Death usually follows overwhelming infection
Brain Tumor
Most common childhood solid tumor and 2nd most common childhood cancer
Most commonly seen in kids 5 to 10 years old
Brain Tumor Symptoms
Cause increased intracranial pressure (ICP) and CNS side effects including
– Most common symptoms
Headache upon awakening
Seizures
– Other Symptoms
Vomiting
Abnormalities in coordination, gait, reflexes, speech
Brain Tumor Management
Prepare child for CT or MRI
Prepare for radiotherapy prior to tumor removal
Prepare for chemotherapy
Prepare for surgery
Post-surgical care
– Monitor neurologic status, ICP, and seizure activity
– Provide skin care and prevent infection
– Administer chemotherapy
Hodgkin’s Lymphoma
Begins in lymph node(s)o Characterized by giant, multi-nucleated Reed-Sternberg Cells
Non-Hodgkin’s Lymphoma
Proliferation of B or T Lymphocytes
Hodgkin’s Management
Painless enlargement of lymph nodes (most commonly in supraclavicular region)
– Systemic symptoms = weight loss, fever, night sweats
Non-Hodgkin’s Management
Depend on site and extent of involvement
– Swelling in abdomen or neck
– Congestion/URI Symptoms
– Systemic symptoms = same as for Hodgkin’s
Wilm’s Tumor (Nephroblastoma)
Embryonal cancer of the kidney originating during fetal life
Average age at diagnosis is 2-4 years
Tumor is usually unilateral
Prognosis is good if there is no metastasis
Wilm’s Tumor Assessment
Assess for nontender mass, usually midline near liver
Note abdominal pain, hypertension, hematuria, anemia, or constipation
Wilm’s Tumor Management
Cautious palpation of the abdomen as to avoid mass rupture and bleeding
Prepare child and family for a nephrectomy within 24-48 hours of diagnosis followed by chemotherapy
Tumor Lysis Syndrome
Serious metabolic disorder occurring within 72 hours following starting of chemotherapy (occasionally prior to) resulting in elevated uric acid, potassium, and phosphorous and decreased calcium
Tumor Lysis Syndrome Assessment
CV: dysrhythmias (due to hyperkalemia); can cause sudden death
GU: renal impairment/failure (b/c of ↑ uric acid); may need dialysis
Neuro: lethargy, muscle spasms, cramps, seizures
GI: nausea and vomiting
Tumor Lysis Syndrome Management
Electrolyte/Fluid Management
Restrict potassium intake, fluid administration
Diuretic
Strict I & O and Daily Weight
Cyanotic Heart Defect S/S
Cyanosis of lips and digits and/or a “dusky look” with abnormal oxygen saturations
Breathlessness, tachypnea
Feeding difficulties
Failure to thrive
Acyanotic Heart Defect S/S
Pink baby with normal oxygen saturations
– May have oxygen desaturations with exertion
-May be asymptomatic; symptoms may emerge
-Activity intolerance/fatigue
-Feeding difficulties
-Recurrent respiratory infections
-Respiratory distress (nasal flaring, retractions, tachypnea, grunting)
Tetralogy of Fallot
Cyanotic
involves the mixing of oxygenated and deoxygenated blood for transport to the systemic circulation
Tetralogy of Fallot Disorders
Pulmonary artery stenosis, ventricular septal defect (VSD), hypertrophy of the right ventricle, and an overriding aorta (dextraposition of the aorta)
Tetralogy of Fallot Pathophysiology
The increased pressure in the right ventricle causes blood to shunt from the right to left ventricle forcing deoxygenated blood into the aorta and then to the systemic circulation
Tetralogy of Fallot Assessment
Acute episodes of cyanosis and hypoxia called “tet spells” or “blue spells”
– Polycythemia
– Signs of chronic hypoxia
Dyspnea, clubbing of digits, failure to thrive, exercise
intolerance
Tetralogy of Fallot Management
Decrease oxygen demands - time limited activities
Knee to chest positioning during tet spells
Oxygen
Surgical repair around 12 months
Ventricular Septal Defect
Acyanotic
The most common congenital cardiac anomaly
Occurs when the septum between the ventricles fails to completely form, resulting in left-to-right shunting of blood.
VSD Assessment
Signs of congestive heart failure (CHF) with right ventricular hypertrophy
– Tachycardia
– Diaphoresis
– Tachypnea
– Fatigue
Failure to thrive
Exercise/activity intolerance
Recurrent respiratory infections
VSD Management
Digoxin - decrease pulse, strengthen cardiac contractions
-Monitor for bradycardia, dysrhythmias, anorexia, nausea, vomiting
Monitor fluid status
-Strict I&O
Administer diuretic as ordered
-Monitor potassium loss
High Calorie food
Coarctation of the Aorta
Obstructive Defect
Narrowing of aortic arch = decrease blood flow to trunk and lower extremities, increase to head and arms
Risk for stroke
CoA Assessment
Full, bounding pulses in the arms with weak or absent pulses in the legs
Increased blood pressure on the arms and decreased blood pressure in the legs
Warm upper body and cool lower body
Rheumatic Fever
Acquired autoimmune inflammatory disorder
Occurs 1-6 weeks after a group A betahemolytic infection– Related to inadequate treatment
Antibodies are produced against the toxin of streptococci and attack and destroy the heart valves
Rheumatic Fever Assessment
Fever
- Fatigue
- Joint pain with swelling, redness, and warmth
– Chorea (sudden involuntary movement of the extremities)
– Erythema marginatum (temporary, disk-shaped red macules that are non-pruritic and faded in the center
Rheumatic Fever Management
Prevention is KEY
– Administer penicillin with strep throat infections
Treatmen
– Promote bed rest until ESR normalizes (about 5 weeks)
– Medicationso Anti-inflammatoryo Penicillin prophylaxis to prevent future attacks
Kawasaki Disease
Acute systemic vasculitis of unknown origin lasting 6-8 weeks; coronary arteries most at risk
Leading cause of acquired heart disease in kids
– Untreated, 25% develop permanent cardiac damage– With treatment, permanent cardiac damage is rare
Kawasaki Disease Complications
Myocardial infarction caused by occlusion of a coronary artery as a result of
-Thrombosis
-Stenosis
Fluid overload and CHF
Acute Phase Kawasaki Disease
High, persistent fever unresponsive to antipyretics
– Swelling of the conjunctiva without drainage
– Inflammation of the mouth, lips, tongue
Strawberry tongue
-Rash – varies from child to child
– Swollen, red hands and feet
– Cervical lymphadenopathy
Subacute Phase Kawasaki Disease
Begins with resolution of fever and ends when all outward symptoms are gone.
– Hands and feet peel during this phase.
– Irritability persists
Convalescent Phase Kawasaki Disease
Begins when all clinical signs of KD have resolved and ends when all blood values (ESR, C-Reactive protein) return to normal.
– ESR and C-Reactive protein = remain elevated
Thrombocytosis still present
Arthritis still present
IV Immune Globulin
Reduces incidence of coronary artery abnormalities when given within 10 days of infection/symptoms
Salicylate Therapy (aspirin)
Anti-inflammatory dose (80-100 mg/kg/day divided Q 6 hours)
Maintenance Treatment Aspirin
Low, Anti-platelet dose (3 – 5 mg/kg/day)* In kids with normal echocardiograms – continued 6-8 weeks until PLT count has returned to normal* In kids with abnormal echocardiograms – continued indefinitely
Kawasaki Disease Treatment
Cardiac Monitoring
– Assessment for S/S fluid overload and CHF
– Administer fluids judiciously
Symptom Relief
– Minimize skin discomfort
- Mouth care
- Cool cloths and lukewarm baths
Medication Administration
– IVIG– Complications: anaphylaxis, headache
* Patient/Family Education– Support family particularly regarding child’s Irritability
Offer support
Offer time away/breaks
Fluid Maintenance
Kg 1-10 X 4ml/hour +
Kg 11-20 X 2ml/hour +
Kg >20 X 1 ml/hour
