Hematology and Oncology (WEDNESDAY) Flashcards
Sickle Cell Disease
What kind of disorder?
25% chance of what?
50% chance of what?
characterized by what?
Autosomal recessive inherited blood disorder
25% chance will inherit two genes for sickle cell or two genes for Hemoglobin A
50% chance will inherit one gene for sickle cell and one normal gene, carrier state
Sickle Cell Trait
Characterized by the formation of long chains of hemoglobin when deoxygenated
Diagnosis of sickle cell
2
Newborn screening
Hemoglobin electrophoresis
Sickle cell Disease: Pathophysiology
in times where O2 is lower (dehydration, infections, physical exertion, cold temps… can cause hemoglobin becomes hard and sickled
RBC Comparison
Normal? lifespan?
Sickle cell? lifespan?
Normal
-Soft, biconcave
-Lifespan: 120 days
Sickle cell
-RBC polymerize, forming stiff rods
-Lifespan: 20 days or less
Sickle Cell Disease: Acute Pain Episodes
Diagnosis?
Most common acute complication
Recurrent vaso-occlusion with ischemia
Diagnosis
Standard criteria: client’s reported pain
sickle cell management for pain
3
IV opioids: morphine, hydromorphone
(NSAID): ibuprofen or ketorolac
Warm compress, massage
Sickle Cell Disease:
Acute Chest Syndrome symptoms?
Causes?
Pulmonary vascular vaso-occlusion
New infiltrate on chest imaging
Plus 2 of the following
*Chest pain
*Decreased oxygen saturation
*Tachypnea
*Fever
Causes:
Infection
Acute pain episode
Acute Chest Syndrome: Management
Frequent assessment
Chest imaging
Oxygenation
IV Fluids
Antibiotics
Analgesia (NSAIDs & Opioids)
Blood transfusion if indicated
Blood cultures
CBCD
Reticulocyte count
Incentive spirometry
Sickle Cell Disease: Infection
Risk for what?
Spleen?
Hypoxic environment leads to what?
Bacteria?
Risk for pneumonia, sepsis, and osteomyelitis!!!
*Spleen infarction
(Spleen plays a role in preventing infection)
*Hypoxic environment leads to spleen infarction during the first 6-12 months of life
*Unable to produce specific immunoglobulin antibodies to certain bacteria
*Streptococcus pneumoniae
Sickle cell disease: Chronic Management
IMMUNIZATIONS 3
Pneumococcal
Meningococcal
Haemophilus influenzae type B (Hib)
Sickle cell disease: Chronic Management
PENICILLIN PROPHYLAXIS
give to who?
All children with SCD from 2 months to 5 years of age
Daily dose
Sickle cell disease: Chronic Management
HYDROXYUREA
Increases what?
Decreases risk for what?
Increases hemoglobin F
Hemoglobin F reduces Hemoglobin S polymerization
Decrease risk for:
ACS
Acute pain
Sickle cell disease: Chronic Management
LIFESTYLE MODIFICATIONS
avoid what? 3
Dehydration
Cold temperatures
High altitudes
Racism towards Sickle Cell Disease
Marginalized and dismissed while seeking medical care for life threatening complications
Stigmatized as drug-seeking and exaggerating their pain
Inadequate treatment and increased suffering
Often avoid medical care because of the perceived racial stigma
Acute Lymphoblastic Leukemia (ALL)
Most common
Differentiation abnormalities in the lymphoid cell lineage
Peak onset: 2-5 years
Acute Myelogenous Leukemia (AML)
Differentiation abnormalities in the myeloid cell lineage
Peak onset: 1st year of life
leukemia diagnosis?
leukemia treatment?
Bone marrow aspirate
Lumbar puncture
Chemotherapy
Stem cell transplant if initial treatment unsuccessful
Wilms Tumor (tumor on kidney)
3 symptoms
Avoid what?
Asymptomatic abdominal mass
Malaise, hematuria, and hypertension
Avoid palpating the abdomen. Wilms tumor is often encapsulated and palpating the abdomen could cause the tumor to rupture and spread cancer cells.
most common protozoal infection in immunosuppressed children?
What is an emergency in cancer?
Avoid what?
What hygiene?
No what?
Neutropenia
*Definition: ANC < 500/mm3
Severe infection risk
Pneumocystis jiroveci pneumonia (PCP): most common protozoal infection in immunosuppressed children
Prophylaxis: trimethoprim-sulfamethoxazole (Bactrim):
*Fever is a medical emergency
*Avoid large crowds
*Hand hygiene
*No fresh flowers
Anemia
*Decreased RBCs
Thrombocytopenia
*Decreased platelets
Complications of Cancer treatment: Sepsis
Systemic inflammatory response syndrome
*Fever > or = 100.9 F (38.3 C)
*Tachycardia, tachypnea, hypotension, prolonged capillary refill, decreased urine output, extremities cool to touch
Management
*Head-to-toe assessment
*Blood cultures
*Antibiotics
*IV resuscitation bolus if clinically unstable
*20 mL/kg of normal saline
*Reassess lung sounds
Complications of cancer treatment
Mucositis/Esophagitis?
Treatment?
Nausea/Vomiting?
Altered Nutrition?
Altered Body Image?
Steroid Effects?
Long-term Effects?
Mucositis/Esophagitis
*Inflammation/ulceration of mucous membranes of oral cavity/esophagus
Treatment:
*Pain management
*Oral hygiene
*Avoid acidic food/drinks
Nausea/Vomiting
*Poor control leads to dehydration, electrolyte imbalance, weight loss, psychological stress
*Anti-emetics scheduled or as needed
*Ondansetron (Zofran)
*Promethazine (Phenergan)
*Lorazepam (Ativan)
Altered Nutrition
*High calorie diet
*TPN/IL
Altered Body Image
*Alopecia
*Altered growth
*Delayed sexual maturation
Steroid Effects
*Cushingoid appearance, facial swelling
*Behavioral changes
Long-term Effects
*Family dynamics
*Risk for relapse
*Multi-system complications
Social determinants of health: Cancer
Health insurance
Geography
Transportation
Time
Cost
