Research Projects Funded in 2015
- HR-pQCT: a novel imaging technology detects microarchitectural skeletal pathology in hemophilia patients
- DDAVP vs exercise in patients with mild hemophilia A – which is better and do they work synergistically in improving hemostasis?
- Hemophilic arthropathy of the ankle: at what age can it be detected?
HR-pQCT: a novel imaging technology detects microarchitectural skeletal pathology in hemophilia patients
Dr. Adrienne Lee
University of Calgary (Alberta)
Second year funding
Dr. Man-Chiu Poon, University of Calgary
Dr. Steven Boyd, University of Calgary
Dr. Gregory Kline, University of Calgary
Comprehensive care of hemophilia patients has resulted in significant improvements in survival, preservation of joint function, and quality of life. Thus, the focus is now shifting to issues of the aging hemophilia patient. Osteoporosis is emerging as a unique concern in persons with hemophilia (PWH) and several studies demonstrate a significant proportion of PWH with low bone mineral density (BMD) developing at an early age. Low BMD predisposes to bone fractures, a consequence that would be difficult and expensive to manage in PWH due to the underlying bleeding disorder. Why hemophilia persons are developing low BMD is unclear since this is normally a disease of postmenopausal women. The goal of this project is to utilize a new imaging technology called HR-pQCT (high resolution peripheral quantitative computed tomography) that visualizes the bone structure in 3D. This information along with bone metabolism markers can tells us about bone strength, the changes in bone density, and potentially the reason why these changes occur in PWH. We hope the results of our study will help identify aging PWH individuals with bone quality deficits who are at risk of fracture and may benefit from early preventative measures.
DDAVP vs exercise in patients with mild hemophilia A – which is better and do they work synergistically in improving hemostasis?
Dr. Manuel D. Carcao
Hospital for Sick Children – Toronto
First year funding
Vanessa Bouskill, MN, RN(EC) Nurse Practitioner, Hospital for Sick Children – Toronto
Dr. Michelle Sholzberg, St. Michael’s Hospital – Toronto
To reduce the risk of bleeding, persons with hemophilia benefit from increasing their factor level in their blood. For persons with mild hemophilia A (MHA) this can be done by receiving DDAVP. DDAVP temporarily (≈12 hrs) increases the amount of FVIII in a person’s blood by shifting FVIII from sites of storage into the blood. DDAVP can be given as a nasal spray.
Our group showed that exercising for about 15 min on a stationary bicycle can increase FVIII in a similar way/extent as DDAVP. Thus exercise by itself may protect persons with MHA from bleeding and make taking DDAVP not necessary.
We wish to study this by testing 40 males (ages: 12-40) with MHA. They will be divided into two groups: Group 1 will receive DDAVP while Group 2 will undergo exercise. After that all subjects will have their blood rechecked. Then they will receive the other intervention to check if the combination of DDAVP plus exercise results in better clotting ability than just exercise or DDAVP alone.
We predict that exercise and DDAVP will result in similar effects on improving a person’s clotting ability. If successful the implications of our study will be that persons with MHA may not need to receive DDAVP but can feel reassured that they can exercise with less risk of bleeding provided that they appropriately warm up and thus raise their FVIII before engaging in sports activities that pose a risk of bleeding.
Hemophilic arthropathy of the ankle: at what age can it be detected?
Kathy Mulder, PT
Health Sciences Centre – Winnipeg
One year funding.
Dr. Kristy Witmeier, Centre for Health care Innovation – Winnipeg Regional Health Authority
Matthew Thiessen, PT, Health Sciences Centre – Winnipeg
The aim of the project is to review the joint health scores for the ankles of all subjects who participated in the Canadian Escalating Dose Prophylaxis Study. Ankle joints are often affected in people with hemophilia. Chronic pain and loss of mobility can begin at an early age. Degeneration usually progresses quickly and necessitates the use of special footwear, mobility aids and joint surgery.
We have observed that there may be two key periods when children are more likely to show signs of ankle involvement. The purpose of this project is to analyze the joint scores in detail to determine if this is so.
If there is a discernible pattern, it may be possible to tailor interventions, such as factor prophylaxis, physiotherapy, footwear modifications and activity selection, to provide better protection for the ankles during critical periods of growth and development with an aim to delaying or preventing chronic joint disease in ankles.