Sickle Cell
what is sickle cell disease?
RBCs become hard and sticky and look like a C shape sickle. These cells die early which causes a shortage of RBCs and therefore a shortage of O2 in blood
What organ is vulnerable in SCT since it promotes sickling of RBCs?
spleen
What is Rhabdomylosis?
ana cute breakdown of skeletal muscle causing release of intracellular contents into circulatory system
What causes rhabdo?
excessive muscle strain or activity
extreme unaccustomed, or novel physical exercise
crush syndrome/traumatic compression of msucles (MVAs, earthquakes, torture, abuse)
obstruction of blood supply to muscles
electrical shock / lightning
prolonged seizures, epilepsy, cramping
extremes in body temp
What is the purpose of hemoglobin?
transports O2 in the blood
What are the two main characteristics of SCD?
anemia and recurrent vaso-occlusion
If an athlete has SCT can they compete in NCAA?
Yes, having SCT does not disqualify them. They need proper awareness and precautions as well as counseling from physicians/counselor certified in sickle cell trait
What increases the risk of Rhabdo?
dehydration and electrolyte disturbances
SCT
extreme heat and humidity
exercise-induced asthma
fatigue
within 2-3 minutes of all out vigorous exercise
often early in season/ pre season
symptoms can occur 24-48 hours post conditioning
What is hemolytic anemia?
RBCs are destroyed faster than they are replaced
what is the difference between sickle cell and sickle cell trait?
sickle cell trait is an individual who has one of the genes for sickle cell, but not both needed to display symptoms
sickle cell is present in those individuals who have both copies of the altered hemoglobin gene and display the symptoms of sickle cell
What are 3 symptoms of a sickling crisis?
muscle cramping
muscle weakness
difficulty breathing or inability to catch breath
fatigue
leg or low back pain
What are the three key symptoms of rhabdo?
muscular aches, weakness, and tea-colored urine
What is the diagnostic criteria for exertional rhabdo?
rule out the 3 most common non traumatic causes of injury (heart, heat, stroke, asthma)
look at urine: dark urine without other symptoms
look at CK levels: 5x the higher limit of normal CK (>5,000 U/L)
What is end organ damage?
damage to the major organs that are fed by circulatory system - heart, kidneys, brain
What four things put an individual with SCT at risk?
1. heat - affects hydration levels
2. dehydration - causes shrinkage in RBCs
3. altitude - less O2 available
4. asthma - bronchospasm which decreases O2
How do you treat ECAST?
check vitals
administer high flow O2
cool if necessary
call 911, attach AED, start an IV, and transport ASAP
What are 3 clinical presentations of exertional rhabdo?
dark tea colored urine
myalgia
elevated serum muscle enzymes
acute muscle necrosis with swollen, tender, and weak muscles
changes in color of skin compatible with compartment syndrome
When would you hospitalize someone with rhabdo?
highly increased CK activity
decreased creatinine clearance
elevated serum creatinine
myoglobinuria
metabolic abnormalities
signs of compartment syndrome
Your patient slumps to the ground with muscle weakness, can talk at first, and has a temp of 99. What is your diagnosis?
sickling
What are the primary differences to look for with SCT vs heat related illnesses?
core temp is not elevated
sudden collapse precipitated by exercise with intravascular sickling
often occurs within 30 minutes of on field wind sprints
onset is hasty; no warning
pain is ischemic rather than lactic acidosis
What does sickling have a similar presentation to?
rhabdo or heat cramps/stroke
Explain the pathophysiology of rhabdomylosis.
an increased intracellular calcium leads to activation of proteases and production of reactive oxygen species. Necrosis of skeletal muscle cells releases intracellular contents (K, myoglobin, CK, and LDH). This causes pain, swelling, and potential end-organ damage.
What other conditions do you have to use differential diagnosis for with rhabdo?
myoptahies
periodic paralysis
polymyoitis or dermaatomyoitis
guillain-barre syndrome
Your patient suddenly collapses with no warning, is unconscious, and has no cramping in their muscles. What is your diagnosis?
cardiac issue