Hematology and Oncology (WEDNESDAY)

Question 1

Sickle Cell Disease

What kind of disorder?
25% chance of what?
50% chance of what?
characterized by what?

Answer 1

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

Question 2

Diagnosis of sickle cell
2

Answer 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

Question 3

RBC Comparison

Normal? lifespan?
Sickle cell? lifespan?

Answer 3

Normal
-Soft, biconcave
-Lifespan: 120 days

Sickle cell
-RBC polymerize, forming stiff rods
-Lifespan: 20 days or less

Question 4

Sickle Cell Disease: Acute Pain Episodes

Diagnosis?

Answer 4

Most common acute complication

Recurrent vaso-occlusion with ischemia

Diagnosis
Standard criteria: client’s reported pain

Question 5

sickle cell management for pain

3

Answer 5

IV opioids: morphine, hydromorphone

(NSAID): ibuprofen or ketorolac

Warm compress, massage

Question 6

Sickle Cell Disease:

Acute Chest Syndrome symptoms?

Causes?

Answer 6

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

Question 7

Acute Chest Syndrome: Management

Answer 7

Frequent assessment
Chest imaging
Oxygenation
IV Fluids
Antibiotics
Analgesia (NSAIDs & Opioids)
Blood transfusion if indicated
Blood cultures
CBCD
Reticulocyte count
Incentive spirometry

Question 8

Sickle Cell Disease: Infection

Risk for what?
Spleen?
Hypoxic environment leads to what?
Bacteria?

Answer 8

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

Question 9

Sickle cell disease: Chronic Management

IMMUNIZATIONS 3

Answer 9

Pneumococcal
Meningococcal
Haemophilus influenzae type B (Hib)

Question 10

Sickle cell disease: Chronic Management

PENICILLIN PROPHYLAXIS
give to who?

Answer 10

All children with SCD from 2 months to 5 years of age

Daily dose

Question 11

Sickle cell disease: Chronic Management

HYDROXYUREA

Increases what?
Decreases risk for what?

Answer 11

Increases hemoglobin F

Hemoglobin F reduces Hemoglobin S polymerization

Decrease risk for:
ACS
Acute pain

Question 12

Sickle cell disease: Chronic Management

LIFESTYLE MODIFICATIONS
avoid what? 3

Answer 12

Dehydration
Cold temperatures
High altitudes

Question 13

Racism towards Sickle Cell Disease

Answer 13

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

Question 14

Acute Lymphoblastic Leukemia (ALL)

Answer 14

Most common

Differentiation abnormalities in the lymphoid cell lineage

Peak onset: 2-5 years

Question 15

Acute Myelogenous Leukemia (AML)

Answer 15

Differentiation abnormalities in the myeloid cell lineage

Peak onset: 1st year of life

Question 16

leukemia diagnosis?
leukemia treatment?

Answer 16

Bone marrow aspirate
Lumbar puncture

Chemotherapy
Stem cell transplant if initial treatment unsuccessful

Question 17

Wilms Tumor (tumor on kidney)

3 symptoms

Avoid what?

Answer 17

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.

Question 18

most common protozoal infection in immunosuppressed children?

What is an emergency in cancer?
Avoid what?
What hygiene?
No what?

Answer 18

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

Question 19

Complications of Cancer treatment: Sepsis

Answer 19

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

Question 20

Complications of cancer treatment

Mucositis/Esophagitis?
Treatment?
Nausea/Vomiting?
Altered Nutrition?
Altered Body Image?
Steroid Effects?
Long-term Effects?

Answer 20

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

Question 21

Social determinants of health: Cancer

Answer 21

Health insurance
Geography
Transportation
Time
Cost