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Sickle Cell Disease :recycle: (Clinical Manifestations: Pain is the most…
Sickle Cell Disease :recycle:
Patho:
A genetic disorder that results from in chronic anemia, pain disability, organ damage, increase risk of infection, and death.
This is a result from a formation of abnormal hemoglobin chains found in Hemoglobin S molecules normally seen in sickle cell disease. In SCD, > 40% of total hemoglobin is composed of an abnormal beta chain.
HgS are sensitive to low oxygen content of the RBC's. When a large amount of HgS is exposed to low oxygen, the chains pile up together inside the RBC and deforming the cell into the sickle cell shape becoming rigid and sticky. The sickled RBC travel through the blood vessels and clump up together blocking the blood flow known as "vaso-occlusion event, which lead to tissue hypoxia. More sickle cell shaped RBC's are formed due to the hypoxia which lead to more blood vessel occlusion and leads to ischemia.
Repeated ischemia episodes progress to organ damage from anoxia and infraction. :red_flag:
Conditions that causes sickling :warning:
hypoxia
dehydration
infection
venous stasis
pregnancy
alcohol consumption
high altitudes
low and high environmental /body temps
acidosis
strenuous exercise
emotional stress
anesthesia
Healthy adults have normal hemoglobin A (HgA) molecules which have 2 alpha chains and 2 beta chains of amino acids. The normal RBC's contain 98-99% HbA with small percentage of Hemoglobin F (HgF) :smiley:
When hypoxia is corrected by normalizing the oxygen level, the sickle cells go back to their normal RBC shape and proper tissue perfusion resumes. :star:
Although the cells appear normal, some hemoglobin remains twisted preventing the RBC sickle shaps from taking its original form and eventually stays in it sickled shape. At this point, the membrane of the sickle shaped RBC is more fragile and more easily broken down. :red_flag:
The average normal RBC lifespan is about 120days, compared to the lifespan of an RBC with HbS lifespan of 10-20 days. The destruction of a large amount of sickled RBC's leads to hemolytic anemia. :red_flag:
Crisis:
Periodic episodes of extensive cellular sickling are called "crisis". Occurring only in response to conditions that causes local or systemic hypoxemia (deficient oxygen in the blood) :explode:
Assessment:
People with the SCD trait will not notice about the presence of the disease until they have a hematologic problem where HbS is detected due to an illness, or when they are exposed to anesthesia during a procedure or surgery
Ask about previous crisis
what led to the crisis, severity, and management
manifestations of infections
activities during the last 24 hours
nutrition and fluid intake
exposed to temperatures
drugs
exercise
trauma
stress
recent airplane travel
alcohol use
changes in sleep patterns
fatigue and SOB
:frowning_face:
SCD is a genetic disorder with an autosomal recessive pattern of inheritance.
A mutation in the hemoglobin gene allele on chromosome 11 leads to the formation of HbS instead of HbA.
Clinical Manifestations:
Pain is the most common manifestation including other manifestations due to tissue damage.
Cardiovascular Manifestations:
SOB
General fatigue/weakness
murmurs
presence of S3 heart sounds
JVD and pulsations
compare peripheral pulses, temps, and cap. refill {Ext. distal to vaso-occlusion are cool to touch with slow cap. refill and reduced or absent pulse}
rapid heart rate
low blood pressure to average anemia :<3:
Priapism:
Prolonged penile erection due to excessive vascular engorgement in erectile tissue, very painful and lasts for hours and unable to urinate. :pencil2:
Skin changes:
pallor
cyanosis due to low gas echange from decreased perfusion and anemia.
gray lips, tongue, soles, palms, conjunctivae
bluish tinged bed nails
jaundice due to the destruction of RBC's and release of bilirubin. Usually causing intense itching.
ulcers in lower extremities due to low perfusion
assess for necrotic tissue in darker areas of the skin :silhouette:
Abdominal changes:
damaged spleen and liver due to ischemia episodes of hypoxia. Liver and spleen may feel firm and enlarged with lumpy texture in later stages. :no_entry:
Kidney/urinary changes:
anoxic damage to kidney nephrons
led effective filtration & absorption
proteinuria causing no output and kidney failure :recycle:
Musculoskeletal changes:
occurs due to blood vessel occlusion
joint damage due to hypoxic episodes and might have necrotic degeneration
Swelling to joints
temperature changes or pallor
ROM with pain on movement :explode:
Central nervous system (CNS):
low grade fever
seizures
manifestations of stoke
-B/L hand grasp strength, gait, and coordination "pronator drift" :silhouettes:
Diagnostic Studies:
CBC {detection of percentage of HbS on electrophoresis
