Doctors in Calgary have developed a procedure that appears to cure this crippling genetic disease. Sickle cell anemia affects people of sub-Saharan Africa, parts of India and the Arabian Peninsula. It is caused when two copies of the recessive sickling gene are inherited. When one inherits a single copy of the gene, it is an advantage; sickling isn't as severe and the sickle cells are less susceptible to the malaria parasite. In patients with two recessive genes, the normal doughnut-shaped red blood cells are turned into elongated sickles as seen in the picture. Instead of carrying oxygen around and delivering it via the capillaries (blood vessels with a diameter of one red blood cell), the cells deform and plug capillary.
The results of blocked capillaries are show here. As well as the anemia, pain, bacterial infections and strokes may occur. During a sickling crisis, there will be pain, necrosis (death of tissue) and ischemia (where blood supply is blocked.) If the crisis is severe, organ damage may occur. Often the spleen is affected. Sufferers of sickle cell anemia have a life span of 55-60 years but they may die during a crisis.
Researchers at Alberta Children's Hospital in Calgary have cured 7 boys and 2 girls of sickle cell anemia with the technique they have developed. The blood system of the child with sickle cell anemia is destroyed and re-established using stem cells from a relative who must be an immune match. The new blood system lacks the pair of sickling genes and the patient is free from sickle cell disease. This procedure may become the treatment standard and at this time is only performed in Calgary. Dr. Greg Guilcher, who is leading the program and is also an assistant professor in the departments of Oncology and Pediatrics at the University of Calgary’s Cumming School of Medicine says the team has received phone calls and emails from doctors all around the world.
Until this technique (first performed in 2009), treatment consisted of analgesics for pain, infection prevention with vaccination and antibiotics, high fluid intake, and folic acid supplementation. Other measures may include blood transfusion, and the medication, hydroxycarbamide (hydroxyurea). Patients lived in pain and were often in and out of hospital. This procedure is a cure and frees people from the tyranny of sickle cell anemia.
I've been writing on and off for years and this is where my more serious pieces will be.