Sickle Cell Disease
What it is:
Genetic disorder causing abnormal hemoglobin → RBCs become rigid and sickle-shaped
Why it matters:
- Sickled cells block blood flow → ischemia and pain
- Repeated infarction damages organs
High-Yield Complication
Splenic infarction → increased infection risk
The spleen can also enlarge and rupture (splenic sequestration crisis)
Presentation
- Severe pain (vaso-occlusive crisis)
- Fever (infection risk)
- Signs of anemia
EMS Management
- Oxygen
- Pain control
- IV fluids
- Treat infections early
NREMT Pearl: Sickle cell → think spleen + infection risk
Marfan Syndrome
What it is:
Connective tissue disorder affecting fibrillin
Key Features (MEMORIZE)
- Tall, thin body habitus
- Long extremities
- Joint laxity
Why it matters (CRITICAL)
Weak connective tissue → aortic dilation → risk of dissection
NREMT Pearl: Marfan = risk for aortic dissection
Aortic Dissection
What it is:
Tear in the inner layer of the aorta → blood separates vessel layers
Classic Presentation (MEMORIZE)
- Sudden, severe chest pain
- “Tearing” or “ripping” pain
- Radiates to the back
Additional Clues
- Unequal blood pressures between arms
- Pulse deficits
Life Threat:
- Rapid internal bleeding → death
EMS Management
- Control BP (per protocol)
- Oxygen
- Rapid transport
NREMT Pearl: Tearing chest pain to the back = dissection
Leukemia & Infection Risk
What it is:
Cancer of blood-forming tissues → abnormal WBC production
Why patients get more infections:
Bone marrow produces immature/nonfunctional white blood cells
Result:
- Poor immune response
- High susceptibility to infection
NREMT Pearl: Leukemia ≠ strong immune system → high infection risk
Blood Transfusion Reactions
Overview:
Reactions can occur during or shortly after transfusion
Common Types
Febrile Reaction
Allergic Reaction
Hemolytic Reaction (MOST SERIOUS)
- Fever
- Back pain
- Hypotension
- Hemoglobin breakdown
CRITICAL EMS RESPONSE (MEMORIZE)
If patient develops: Dyspnea + flushing (or any reaction)
Immediate Actions:
- STOP the transfusion immediately
- Maintain IV access with normal saline
- Monitor vital signs
- Support airway and breathing
- Notify receiving facility/physician
NREMT Pearl: Any reaction → STOP transfusion FIRST
Summary
- Sickle cell → spleen damage/rupture + infection risk
- Marfan → weak connective tissue → aortic dissection risk
- Aortic dissection → tearing chest pain to back
- Leukemia → nonfunctional WBCs → infections
- Transfusion reaction → STOP transfusion immediately
NREMT Test Strategy
- Identify underlying pathology Blood disorder → infection or clotting issue Connective tissue → vascular rupture risk
- Recognize life threats quickly Dissection Hemolytic reaction Splenic rupture
- Choose priority action Stop transfusion Support airway Rapid transport