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Signs & Symptoms
- Generally covered under the term 'Sickle Cell Crisis'
- Two main types of Crisis
- These Crisis' vary in severity but typically worsen with age.
Cardic issues can arise as a complication of SCD due to the disease itself and the lack of flexibility within RBC's.
Due to the low levels of oxygen able to be carried by the blood in patients with chronic Anemia this can put extra pressure on the heart, increasing the risk of heart failure.
An immune response can sometimes be triggered when a sickle cell bursts which can also cause inflammation and heart problems.
Sickle Cell and the Heart
Vaso-occlusive Crisis
When there is a reduction in Oxygen or the demand is high sickle cell haemoglobin deforms when the Oxygen is released. This causes the cell to form a "stiff rod-like structure that bends the RBC into its characteristic sickle (crescent) shape. ( Tortora 2017)
Because of the change in shape the RBC has a reduced ability to carry Oxygen via its Haemoglobin. (Hb).
This type of crisis is can be triggered by and internal or external factor, such as cold, dehydration, infection of hypoxia, but can be others.
There may also be swelling and pain in the hands and feet of children under 3 years of age.
Depending on where the occulsion is will depend on where the pain is felt.
Can lead to Priapism in males.
If CNS is affected patient can present with stroke or convulsions.
What is Sickle Cell?
Sickel Cell Disease (SCD) is a gentic mutation that affects one of the four globin chains within Red Blood Cells.
This disease is inherited from either one or both parents. This will determine whether a person has Sickle Cell Anemia, from both parents or Sickle Cell Trait, from a single parent.
SCD is common in people with an African or Caribbean family background.
Acute Chest Syndrome
Sickle Cell
Can be likened to a pneumonia like illness.
Signs & Symptoms
- Shortness of Breath
- Tachypnoea ( Fast Breathing)
- Hypoxia
- Cough
- Fever
This could be caused by; infection or fat embolism.
Treatments
- Follow and ABC approach
- Follow patients care plan if they have one.
-Time Critical signs require prompt transport to ED with pre-alert.
- Place patient on supplemental Oxygen.
- May benefit from broncodialating medications if indicated.
- Pain relief
- 12 Lead ECG in patients with chest pain.
- Entonox can be used as analgesia, however use should be limited to 60 mins or less.