Message from our President - Ms. Doreen Alexander

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1 Summer Edition: June-August 2012 Volume 1 Issue 6 Inside Youth Corner Save The Date World Sickle Cell Day Event Hope Gala & Awards 2012 Avascular Necrosis Contributor Message from our President - Ms. Doreen Alexander I bring you greetings on behalf of the Board of Directors at Sickle Cell Awareness Group of Ontario. We thank everyone that supported our Hope Gala & Awards 2012 and look forward to hosting you all next year. We are especially grateful to those that helped to sell tickets for the events, and while space will not permit me to mention all, I must recognize Dianne and Kemisha Johnson for their commitment to the Hope Gala year in year out. This year, we have a lot more volunteers that helped out. Kwabena Boateng did a wonderful job in coordinating all volunteers and we recognize this effort. I also thank the chair, Global Sickle Cell Network, Dr. Isaac Odame that delivered the Key Medical Speech as well as Hon. Jean Augustine that brought greetings from her office. The Deputy chiefs and Inspectors of Police, the Black Beauty Canada pageant, the Gala hosts- Ms. Traci Melchor etalk Canada and Nathan Downer CP24 must also be appreciated. Lastly, we must appreciate Mr. Don Moreland, Mr. Carlos Ferguson and Mr. Joseph Williams for helping with the Video and Photography. I would also like to use this medium to advise you all that SCAGO has joined the Sickle Cell Disease Association of Canada as a member Organization. We hope to work collaboratively with every member of SCDAC. I also encourage you to look at the Save the Date below to see our activities for the Summer Months Thanking and wishing you a happy, relaxed Summer time. Dr. Richard Ward Youth Corner SCAGO S Youth Group welcomes Mr. Kwabena Boateng. With background in Public Relations and dedication to Sickle Cell Disease, we believe to see amazing results in 2012 Welcome on Board, Mr. Boateng Here is a glimpse of our World Sickle Cell Day Event SCAGO led World Sickle Cell Day event took place at the Tree of life, Toronto General Hospital200 Elizabeth Street. SCAGO s representatives also supported the Sickle Cell Association of Ontario and Camp Jumoke led events SAVE The DATE June 23rd, 2012 SCAGO Youth Meeting Location: SCAGO office June 29th, 2012 SCAGO Volunteer Meeting Location: SCAGO office July 7th, 2012 East End Parents Support Meeting Location: Rouge Valley Health Systems- Centenary location Time: Call for more information July 7th, 2012 CACD Event Location: Mississauga Valley Community Centre (Parks A & B) Tme: 11AM-4PM July 8th, 2012 Dr. Cyrus Picnic - Location: Masaryktown Park- 450 Scarborough Golf Club Rd July 14th, 2012 SCAGO S Monthly Board of Directors Meeting Location: SCAGO Office July 28th, 2012 Learning for Life Session Topic: Strokes in Sickle Cell Disease Location: Toronto General Hospital To attend: RSVP- secretary@sicklecellanemia.ca August 2nd Awareness at Toronto Caribbean King & Queen Event Location: Allan Lamport Stadium Time: 3PM-&PM

2 Avascular Necrosis and Sickle Cell Disease By Dr. Richard Ward What is Sickle Cell Disease (SCD)? SCD is a condition whereby the body makes an abnormal form of Hemoglobin, the protein inside the Red Blood Cells (RBCs) that carries oxygen around the body. As a result, under certain conditions the RBCs change shape and become sickle-like in appearance. These abnormal RBCs breakdown and also cause blockage of the blood flow through the blood vessels of the body (vaso-occlusion). The consequence of this is to starve the tissues and organs of oxygen that they need to function normally. As the bone is the factory for producing all blood cells, this is the site that is most commonly affected. Without oxygen, the bone and other organs cannot work properly and will eventually die. About Bones The body s bony skeleton is one of the largest organs, and has many roles. It acts as a scaffold for skin, protects vital organs such as the heart and liver, produces blood cells in the bone marrow, and also regulates calcium and other minerals and hormones. Bone must be stiff and able to resist deformation, thereby weight bearing, but also be flexible and able to absorb energy by deforming without cracking. They must also be light to facilitate movement. To do all of this, bone comes in different compositions and shapes. How Does SCD Affect Bones? Sickle Cell affects the bones in many ways. Due to the increase in demand for new RBC production from the anemia, there is an increase in the amount of bone marrow in the middle of the bones. Sometimes, there can also be lumps of blood-forming tissue that grow elsewhere in the body, such as along the spine. This is termed extramedullary hematopoiesis, EMH. These very rarely cause any problems, but can show up on x-rays and scans. The most common problem with the bones in SCD is from vaso-occlusion, resulting in death of the marrow & bone, causing the typical bone pain episodes seen in SCD. Occasionally, little pieces of dead bone marrow can circulate around the body and end up in other organ s blood vessels, termed fat emboli. As the spleen is shrunken and not working well in most patients with SCD, there is an increased risk of infection of the bone (osteomyelitis) and joints (septic arthritis). Finally, it is believed that all these changes to the bone increase the risk of Osteoporosis, weakening of the bone that can result in fractures later in life. What is Avascular Necrosis? Avascular Necrosis (AVN) is also called Osteonecrosis, Aseptic necrosis, Ischemic necrosis or Osteochondritis dissecans. It results from reduced blood supply to bone leading to the death of bone and the bone marrow cells, with eventual mechanical failure. Reduced blood supply to the area of bone causes an increase in pressure in the bone, loss of minerals, and thinning of the bone. Increase in mechanical stress on the weakened bone leads to collapse or fracture. Is AVN Common in SCD? AVN has been reported in 1 in 4 children and in upto 40% of patients by the time they reach 35 years, but depends on what is used to detect it, as MRI is more likely to find it than a normal x-ray. What May I Notice if I Have AVN? AVN usually affects the shoulders or hips. However, half of patients with an affected hip will have both hips involved, and 3 in 4 patients with an affected shoulder will also have an affected hip. 50% of patients with hip AVN have no symptoms, and even less so if the shoulder is involved. AVN may cause pain around the joint on movement, or when weight bearing (hips). Hip pain can also be felt in the groin, thighs or buttocks. You may also find it less easy to move the joint than previously. How is AVN Diagnosed? There are several stages to AVN that represent progressive damage to the joint. Plain x-rays, CT and MRI can all be used to make the diagnosis. It is suspected when a patient reports chronic pain or reduced movement in a joint. 0 - no symptoms, normal x-ray and MRI 1 - normal x-ray, abnormal MRI 2 - abnormal x-ray, cysts and sclerosis 3 - subchondral collapse, crescent sign 4 - flattening of the head 5/6 - degenerative joint disease - arthritis How Do You Treat AVN? The process is progressive, resulting in joint destruction within 3 to 5 years if left untreated. Unfortunately, the treatment options are not very effective apart from joint replacement surgery. The following may reduce or control the pain, but do not affect the joint itself: bed rest, physiotherapy, using crutches, pain relief. A structured physiotherapy program can be prescribed by orthopedic surgeons. Due to the chronic nature of the pain form AVN long acting opiates can be useful analgesia. Because artificial joints last less than 10 years, and patients are often very young, doctors have looked at alternative treatments that may delay the need for joint replacement.core decompression involves removing small bits of bone from the hip to try and reduce the pressure in the bone to improve blood flow and relieve pain. It does not seem to delay the progressive damage and time to collapse of the joint. It has also not been shown to be any better than physiotherapy. New techniques include injecting bone marrow cells into the bone at the time of the procedure to encourage new bone growth. Another option is Osteotomy, whereby a wedge shaped bit of bone is removed from the joint. This is meant to redistribute weight bearing forces to the healthy bone in the joint. What is the Best Treatment? Replacement of the joint gives the best results, but is a major surgical procedure to undertake. It is usually necessary to have a blood transfusion pre-operatively. It is a good option for: older, less active patients, patients with lots of damage to the joint, and when the joint has totally collapsed. Unfortunately, 30% of patients will need further surgery within 5 years. Will Treating the Sickle Cell Help the AVN? We do not have good scientific evidence to answer this question. A study of patients taking hydroxyurea for their SCD, showed less patients developing AVN compared to patients not taking the medication. A smaller study of patients with AVN showed that those patients taking Hydroxyurea had better pain control, and the x-ray appearances of the joint improved. However, the benefit was only seen in patients with early stage AVN. There is no data to support the use of blood transfusions to prevent or treat AVN. Some patients who have a very high Hb level get pain relief from regular removal of a unit of blood on a monthly basis. It is thought that lowering their Hb may reduce the vaso-occlusion. Summary AVN is a common complication of Sickle Cell and is very difficult to treat. At present there are no good alternatives to hip replacement surgery, and it can be difficult to follow through with prescribed exercise programs. The chronic pain from AVN can also be challenging to manage. Future research in this area is needed. Article is supplied by: Dr. Richard Ward Toronto General Hospital. Sickle Cell Awareness Group of Ontario appreciates Dr. Ward s contribution to the SCAGO summer Newsletter. 2

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4 Meet our Working Board of Directors Term Ms. Doreen Alexander - President Ms. Dotty Nicholas - Vice President Ms. Gloria Chukwu - Treasurer Committee Coordinators Patient Coordinator - Bimpe Daniells Parents Support Coordinator - Isatu Bah Youth Support Coordinator - Neenh-Iye Bundu Youth Support P.R - Caroline Dankwah Ms. Doreen Alexander Ms. Dotty Nicholas Ms. Gloria Chukwu Featured Article. As we all look forward to a cure for Sickle Cell Disease, it is exhilarating to see the advances on SCD. Please read the New Hope for Sickle Cell Disease Treatment at the link below: cell anemia drug.pdf Hope Gala & Awards Thank you for making it happen 4

5 Nigerian Canadian News Supports Sickle Cell Awareness Know your Sickle Cell Status. Get tested today