Haemophilia care has steadily improved over the years and especially so during the last decade. The routine use of prophylactic treatment has undoubtedly resulted in a significant improvement in the life-style, quality of life and life expectancy of these patients. However despite our best efforts there is still a group of young adults who have a severe degree of knee joint destruction as a result of repeated articular bleeding episodes during their early years.

The knee is the most common joint affected in haemophilia (50%). The repeated articular bleeding episodes during the patients' early years leads to the onset of pain and significant functional disability at a time when they require the best possible quality of life. The major objective of total joint replacement is to reduce the level of pain in the affected joint and, in addition, a significant reduction in the frequency and number of joint bleeds, which improves both function and mobility.

The results of 60 primary total knee replacements performed in 42 patients with severe haemophilia between 1983 and 2003 were reviewed retrospectively. Functional results were assessed using the Hospital for Special Surgery (HSS) knee score both pre- and post-operatively. Kaplan-Meier survivorship analysis was used to calculate prosthetic survival.

The mean age of patients was 43.35 (range 25-70yrs). The overall prevalence of infection was less than 2%. The HSS clinical score was excellent or good for 95% of the knees.

We believe that total joint replacement is a safe and effective procedure in the management of haemophilic joint arthropathy. The latest techniques using continuous infusion and recombinant factor replacement have gone a long way to reducing the complications rate and to achieving results that match those of the general population.

On 7 July 2005, four bombs were detonated on the London transport system. Three of these bombs exploded almost simultaneously at 08:50h affecting the underground tube network at Aldgate, King's Cross and Edgware Road stations. The fourth bomb exploded at 09:47h on a double-decker bus in Tavistock Square. There were 54 deaths in total at the scenes and over 700 injured.

194 patients were brought to the Royal London Hospital. 167 were assessed in a designated minor injuries unit and discharged on the same day. 27 patients were admitted of whom 7 required ITU care, 1 died in theatre and 1 died post-operatively. The median Injurity Severity Score (ISS) in this group of patients was 6 (range 0-48) and the mean ISS was 12. The general pattern of injury in the critically ill patients was of mangled lower limbs and multiple, severely contaminated fragment wounds. Hepatitis B prophylaxis was administered to those patients with wounds contaminated by foreign biological material. 11 primary limb amputations were performed in 7 patients. 9 limb fasciotomies, 5 laparotomies and 1 sternotomy were carried out. 3 patients had blast lung injury. All patients who underwent primary amputations and debridement received further regular inspections in theatre. These inspections formed the majority of our theatre work. Under no circumstance was initial reconstructive surgery attempted. Delayed primary closure and split skin grafting of all wounds was completed by the end of the second week. There have been no sepsis-related deaths.

Our experience at The Royal London has allowed us to revisit the principles of blast wound management in a peacetime setting. A number of lessons were learned regarding communication and resource allocation. A multi-disciplinary approach with the successful execution of a major incident plan is the key to managing an event of this magnitude.

Haemophilia care has steadily improved over the years and especially so during the last decade. The routine use of prophylactic treatment has undoubtedly resulted in a significant improvement in the life-style, quality of life and life expectancy of these patients, and bodes well for the future.

The knee is the most common joint affected in patients with severe haemophilia (approx 50%) and despite best efforts there is still a group of young adults who have a severe degree of knee joint destruction as a result of repeated articular bleeding episodes during their early years.

The indications for operation are primarily disabling pain that is unresponsive to medical treatment. Deformity and poor functional range of motion, particularly a severe flexion contracture of the knee, are relative indications and may in themselves justify joint replacement. Equally joint contractures and flexion deformity pose various surgical challenges for the surgeon. The introduction of continuous replacement clotting factor has facilitated the operation and in our experience has reduced the complications of TKR. We have found that it permits earlier rehabilitation and in our present series the outcome in this group of patients almost comparable to TKR performed in the general population.

Patients with severe haemophilia have a tendency towards recurrent haemarthroses resulting in chronic synovitis and leading to end stage haemophilic arthropathy. From 1997 to 2001 five patients underwent sequential bilateral total knee replacement. We compared these patients with 13 haemophilic patients undergoing primary unilateral total knee replacement. One senior surgeon performed all surgery using an identical prosthesis under similar surgical and haematological conditions.

We reviewed information regarding pre-operative medical condition, antibiotic prophylaxis, blood replacement requirement and tourniquet time were all recorded. The rate of post-operative complications and economic evaluation between the two cohorts was calculated. Functional results were assessed using the Hospital for Special Surgery knee scoring system both pre and postoperatively.

We have shown that complication rates following bilateral and unilateral total knee replacements are comparable and that there are no differences in the functional outcomes or complication rates between the two groups. Furthermore, we found that bilateral procedures were advantageous with respect to total rehabilitation times, length of in-patient stay clotting factor usage and cost efficiency.