Hematology Blueprint Flashcards
What causes hemophilia?
X-linked recessive chromosomal mutation characterized by coagulation factor deficiency
Hemophilia A is a deficiency in what?
Factor VIII
Hemophilia B is a deficiency in what?
Factor IX
What is the most common type of hemophilia?
A
List the symptoms of hemophilia (hint: 5)
- Frequent nosebleeds (epistaxis)
- Bruising: shins, knees, thighs, forearms
- Excessive bleeding with teeth brushing
- Joint swelling
- Prolonged bleeding after minor injuries
Differentiate between normal vs abnormal bruising in hemophilia
Bony prominence → is a NORMAL bruise
Soft tissue → NOT NORMAL bruise
List the common signs and symptoms of iron deficiency anemia on physical examination (hint: 8)
- Tachycardia
- Pallor
- Brittle, spoon- shaped fingernails
- Fatigue
- irritability
- muscle weakness
- Systolic heart murmur
- Cravings for non-nutritive substances (ice, dirt, paper) aka PICA
What activities are safe for a pt with hemophilia?
- Swimming (still monitor child & avoid risky collisions w pool walls and other swimmers)
- Track (but NO hurdles, shot put, or pole vaulting)
- Cycling
- Walking
- Golf
- Playing with non-contact toys or activities
Why is swimming safe for hemophilia?
It minimizes risk of muscle and joint injuries
What sx following a head injury in someone with hemophilia requires urgent attention? (hint: 4)
Persistent or worsening headache
Vomiting
Confusion
Changes in behavior
List the sx of sickle cell disease (hint: 7)
Pain
Fatigue
Jaundice
Hand & foot swelling & joint pain (dactylitis)
Skin rash
SOB
Priapism
List the severe complications of sickle cell disease (hint: 7)
Vaso-occlusive crisis
splenic sequestration
hyper hemolytic crisis
aplastic crisis
acute chest syndrome
CVA
infection
What factors can cause a sickle cell crisis? (hint: 10)
- Infections
- Low oxygen tension
- Concomitant medical conditions → sarcoidosis, diabetes mellitus, herpes
- Dehydration
- Acidosis
- Extreme physical exercise
- Physical or psychologic stress
- Alcohol
- Pregnancy
- Cold weather (extreme temperature)
What is a vaso-occlusive crisis?
A “painful episode”
Ischemia causes mild to severe pain & can last minutes to days
Any pain can be a sign of crisis
What is a splenic sequestration?
pooling of a large amount of blood, usually in the spleen… rarely in the liver
In a splenic sequestration, what can the decrease in blood volume lead to?
Shock!!
what is acute chest syndrome? what are the signs and sx? (hint: 6)
Similar to pneumonia; the presence of new pulmonary infiltrate
S&S:
- Chest pain
- Fever
- Cough
- Tachypnea
- Hypoxia
- Wheezing
What is important about acute chest syndrome that she said to know for the test?
Fever is a medical emergency !!
What are the tx options for sickle cell? (4)
- opioid analgesics
- hydration: oral and IV
- hydroxyurea
- vaccinations
how does hydroxyurea help tx sickle cell?
it increases fetal Hgb
which does NOT sickle or crescent
it leads to less clumping and better blood flow
helps to reduce the frequency of VOCs
why are vaccinations necessary for pts with sickle cell?
to prevent infections
list the mechanisms for managing sickle cell disease and preventing complications (hint: 7)
Prevent the sickling that is responsible for pathologic sequelae
- Provide rest
- Hydration → oral & IV
- Electrolyte replacement
- Blood transfusions
- Hemaphresis
- Analgesics
- Antibiotics
In sickle cell disease, prevention is key!! list the preventative mechanisms (hint: 4)
Routine vaccinations
Oral penicillin prophylaxis
Daily hydroxyurea
Oxygen PRN if hypoxic
What are key assessments for sickle cell disease?
monitoring vital signs and assessing for pain
If someone with sickle cell disease is experiencing CVA, what are the signs and sx? (hint: 6)
Severe, unrelieved headaches
Jerking of extremities and face
Seizures
Strange, abnormal behavior
Inability to move arm &/or leg
Stagger / unsteady gait
In a pt with SCD, the nurse should monitor for VOC. List the signs they should monitor for (hint: 4)
Localized pain
Swelling
Decreased mobility
Limb pain (early sign)
In a pt with SCD, the nurse should monitor for stroke. List the signs they should monitor for (hint: 3)
weakness
slurred speech
visual changes
When should a patient with Sickle Cell Disease contact a physician or go to the ER?
If they have signs of infection, crisis or have a fever!
What should someone with SCD avoid?
- Cold
- Aspirin
- Traveling where atmospheric oxygen is decreased
What pharmacological pain management is given for someone in an acute SCD crisis?
Narcotics / opioids IV / Codeine PO
For someone with SCD, what can be given parenterally?
NSAID → ketorolac / toradol
what mild analgesic is given to someone with SCD?
PO NSAIDs
What does IV methylprednisolone do for someone with SCD? (hint: 2)
Decreases duration of severe pain in children
Decreases inflammatory response
