Q: I am a 34 year old with sickle cell anaemia. I often have episodes of severe chronic pain. I make numerous trips to my local emergency clinic when my doctor's office is close. How should sickle cell anaemia patient should be treated in emergency?

A:The defective haemoglobin (Hb S) synthesized in sickle cell anaemia has a tendency to aggregate into large polymers whenever there is a decrease in oxygen tension. This leads to distortion of the red blood cells, which obstruct the flow of blood in small capillaries resulting in tissue hypoxia. This can affect any organ in the body and is referred to as vaso-occlusive crisis. It usually presents as severe pain and if it occurs repetitively may result in irreversible damage to the affected organ. The usual affected organ is the bone but it may also present with pain in abdomen or chest, symptoms related to the brain (like seizure, stroke, coma etc.), skin ulcers or vision disturbance. Most often the painful crises are brought about by physical or emotional stress, dehydration due to physical exercise or warm weather, exposure to cold, alcohol ingestion, prolonged air travel, infection etc. The treatment for it is bed rest, plenty of fluids and use of pain killers. It is best to avoid alcohol, smoking and use of medicines without consulting your doctor. Keep yourself well hydrated by taking plenty of fluids. Even though morphine and morphine-like (opioid) drugs are most effective for pain control, they are addictive and patients may often become dependent on them. Use drugs like acetaminophen, ibuprofen, aspirin, and codeine for mild pain and in case it is moderate, take oxycodone or methadone. Taking folic acid supplement, a regular exercise schedule, yoga, acupuncture and being part of a support group often help.