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.
Deep Brain Stimulation or DBS was introduced in 1987 and involves surgery to implant a neurostimulator into the brain of the individual who is suffering from movement or neuropsychiatric disorders. The operation involves drilling into the brain and inserting the neurostimulator into the specific area of the brain that may help to provide therapy when a disorder has been "treatment-resistant." The electrodes target the brain part and are stimulated by a pulse generator that is placed in the chest, much like a pacemaker for the heart.
Conditions that have been helped with DBS are such things as Parkinson's disease, dystonia, chronic pain, major depression and obsessive-compulsive disorder.
CTV's W5 aired a program on DBS and its use as a treatment for anorexia nervosa on March 7, 2015. Dr. Andres Lozano heads the research team at Toronto's University Health Network and has used the technique to help desperate victims of the eating disorder. The two women showcased were so severely affected that the decision to have DBS was one of life or death. The surgery, itself, doesn't come with guarantees and someone in the weakened state that a sufferer of anorexia is, may die on the operating table.
Electrodes are inserted into the part of the brain that regulates mood and anxiety. The patient is awake for this part of the procedure and her feelings and observations help to make sure the neurostimulator is correctly located. For the second part, there is a general anaesthetic while the pulse generator is implanted into the chest.
DBS isn't magic and its results aren't immediate. The two young women still had to overcome their obsession with food and work at becoming "normal." For both of them, the eventual outcome was very positive and they "got their lives" back.
The pulse generator is turned on after about two weeks and the placement of the electrodes and the "dosage" of electricity act to modify brain circuits, either turning off those that are too active or stimulating those that are too slow. When all goes well, the errant brain is "normalized" and those patients who had all but lost hope were able to start to function again.
Just what the DBS is doing for sure isn't understood, but as more research is conducted this technique from 1987 may prove more and more helpful to people in untenable situations.
I've been writing on and off for years and this is where my more serious pieces will be.