EAC Sickle Cell and Thalassemia Flashcards
define:
Sickle Cell
A hereditary condition affecting the haemoglobin contained within red blood cells
Most common in people of African and Caribbean descent.
define:
Thalassemia
There is a failure to produce red blood cells as well as premature breakdown of these cells.
This leads to an increase in the size of long bones and an increase in the size of the liver and spleen.
Mainly affects people of Mediterranean origin.
pathophysiology of:
Sickle Cell Disease
Haemoglobin binds together causing erythrocytes to change shape, a sickle shape. This is due to a genetic mutation of the haemoglobin.
The sickle cells clump together, reducing blood flow through small arteries and capillaries leading to hypoxia. sometimes occluding blood vessels completely.
pathophysiology of:
Sickle Cell Anaemia
The anaemia is due to early haemolysis (destruction of red blood cells) of the irreversibly sickled cells.
Anaemia = not enough haemoglobin available to carry sufficient oxygen from lungs to supply the needs of tissue.
pathophysiology of:
Thalassemia
failure to produce red blood cells that have effective haemoglobin. And the breakdown of erythrocytes prematurely. Leading to Anaemia, and Iron build up.
This leads to an increase in the size of the long bones AND swelling to the liver and spleen.
difference between Sickle Cell Trait and Sickle Cell Disease
Sickle cell trait: a relatively mild condition caused by the presence of a single gene for sickle-cell anaemia, producing a smaller amount of abnormal haemoglobin and conferring some resistance to malaria.
Sickle cell disease: When there are two sickle cell anaemia genes present. Causing a severe hereditary form of anaemia in which a mutated form of haemoglobin distorts the red blood cells into a crescent shape at low oxygen levels.
precipitating factors that can trigger a Sickle Cell Crisis
Altitudes above 10,000ft Extremes of temperature Infection Constrictive clothing Smoking Alcohol Vasoconstrictive drugs Exercise Dehydration
signs and symptoms of:
Sickle Cell Crisis
Dactylitis (inflammation of a digit) Severe pain, especially at the joints Difficulty breathing May show signs of jaundice May be pyrexic May be aggressive/frustrated due to the pain Dehydrated Hypotension Tachycardia Altered LoC
Management of:
Sickle Cell Crisis
Oxygen DRcABCDE If time critical - correct A and B - go to nearest ED Consider paramedic assistance Pain relief ECG Fluid Pt specific treatment plan: -Take pt to their own specialist unit.
condition specific analgesia
The pt may have their own plan for crisis and they should be able to tell you what analgesia they usually need/have.
why is it important to take pt to their specific treatment centre?
They may need a blood transfusion
They will be know to the staff at their unit who can best manage their symptoms i.e. appropriate analgesia
describe:
Acute Chest Syndrome
Chest pain and pneumonia like cough in person with sickle cell disease.
Can be fatal in paediatric pt.
A common and potentially life threatening complication of painful crisis,… often precipitated by a chest infection.
signs and symptoms of:
Acute Chest Syndrome
Dyspnoea Hypoxia - responding poorly to O2 therapy tachypnoeic Tachycardia Chest pain
