It is autosomal recessive genetic condition resulting from the presence of a mutated form of haemoglobin called haemoglobin S which is found in our red blood cells (haemoglobin helps to carry oxygen in our blood).
The red blood cells are now a long shape ‘sickle shape’ (moon crest shape) this is because the haemoglobin s (the part of which that carries oxygen in our blood) grows into long rods rather than a round ‘disc’ shape.
This shape then affects the shape of the red blood cells rather than being round, they are ‘sickle’ shape (like a moon crest) and often get clogged up in blood vessels causing can poor blood flow meaning oxygen cannot be easily reached to tissue and organs in the body causing tissue and organ damage and/or reduced function.
A normal red cell lifespan is 120 days. Sickled blood cells last only 10 to 20 days and there one with with sickle cell disease have long term anaemia–an abnormally low level of red blood cells resulting in anaemia.
The spleen helps to old blood cells and helps to clear infections, when one has sickle cell anaemia, the poor oxygen blood flow to the spleen cause the spleen to eventually lose its function and have a weak immunity to infections and this can be life threatening.
When blood flow is completely blocked in sickle cells disease , it is known as ‘sickle cell crisis’ and this is life threatening as tissues and organs cannot receive oxygen from the blood.
Do not take any form decongestants i.e. ephedrine, pseudoephedrine and/or Aspirin because they can constrict blood vessels and could trigger a sickle cell crisis
Cause
Family history
Both parents have a defective gene
One parent have defective gene (known as ‘sickle cell “trait” rather than sickle cell anemia, person with sickle cell trait usually have no symptoms but can pass the gene to their children)
Intramuscular injection of anti-inflammatory medications such as ketorolac or diclofenac (in the absence of any contraindications), and treatment of pain using a unit dose of a parenteral opioid such as hydromorphone, morphine, pethidine (meperidine), or fentanyl by intramuscular (IM) or subcutaneous (SQ) injection.
Spleen, kidney, skeleton (bones) and central nervous system blockages and poor function- Obstruction of the blood vessels can lead to bacterial infections and poor function.
Delayed growth during childhood and delayed puberty
If you have a sickle cell crisis, you can usually manage it at home.
The following things can help:
take over-the-counter painkillers, such as paracetamol or ibuprofen if suitable (do not give aspirin to children under 16 unless a doctor has prescribed it) – if the pain is more severe, your GP may prescribe stronger painkillers
Have plenty to drink
Use a warm towel or a wrapped heated pad to gently massage the affected body part – many pharmacies sell heat pads that you can use for this purpose
distractions to take your mind off the pain – for example, children might like to read a story, watch a film or play their favourite computer game
Please talk to your healthcare professional (i.e. Medical Doctor/Pharmacist) for further advice
Detailed Information
Please copy and paste any key words from the title: Sickle Cell Disease (Sickle Cell Crisis) in the following respective 'Medtick References and/or Sources' to find out more about the disease (this also may include diagnosis tests and generic medical treatments).
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