Establishing an online physical exercise program for people with hemophilia

Implementing the online training program had the following requirements:

The concept of the online training program was compiled in accordance with musculoskeletal experts in the field of hemophilia from Germany. The videos were recorded separately for adults and children. The video clips were shot with a person with hemophilia and the background of studying sports science (who demonstrated the exercises for the adult videos) and a 10-year-old volunteer (who demonstrated the exercises for the children videos). During the takes a physiotherapist was present to observe the correctness of the exercise performance.

There are three options to access the webcasts:

The webcasts are structured as follows:

Table 1 presents an overview of the exercise program. It contains exercises for warm up and mobilization, coordination, strengthening and flexibility.

As the primary aim is to offer an exercise program to better stabilize the joints, training is focused on warm up and coordination, strength and flexibility exercises. Endurance training and the participation in sports are also recommended in PwH, but a detailed instruction on the choice of sports and the risk assessment of the different types of sport would have gone beyond the scope of the online training program. Regarding endurance training and sports, PwH are asked to follow the recommendations of their specialists in charge. Specific recommendations in this respect can be found in the pertinent literature [1, 47‐57].

The training should to be increased over time according to the improving fitness, which is why the presented coordination and strengthening exercises can be adapted in difficulty, intensity and volume. Coordination exercises can thereby be increased as soon as an exercise can be safely conducted. Strengthening exercises should be increased in consultation with the specialist in charge and in the following order:

Coordination and strengthening exercises should not be increased both at the same time but one after the other. In the case of swelling, pain or symptoms of a bleeding, the training should be stopped and a physician should be consulted. The training composition differs between the children and adult training program and is explained in the webcast on how to compose and increase the training. Adults are advised to focus their training on the chronically affected joint. In children who have not yet developed arthropathic joints, the training is recommended to strengthen the main deficits in children, which are coordination, flexibility of the back side of the legs and the strengthening of the hip abductors and abdominals [16, 32, 33, 48, 57, 58]. If children and adolescents already suffer from chronic joint affections, they are asked to consult their physician in order to focus the training on the affected joint similarly to the adult recommendations. In order to suggest a training program that is easily transferrable into everyday life, it is recommended to start with a training that involves an appropriate warm up and mobilization and 6 exercises consisting of coordination, strengthening and flexibility exercises to be performed 2–3 times a week on a regular base. The training can be extended after consultation with the physician in charge.

Both adults and children are advised to warm up and mobilize the joints and the spine before starting the training. It is especially important for adults to warm up appropriately with a focus on the chronically affected joints and the structures that are to be trained afterwards. Adults should take a minimum of 10 minutes for warm-up and mobilization before starting the actual training. Thereby, structures are prepared for the training, the risk of injury is reduced, and the joint range of motion is maintained. Also, PwH are advised to make use of the joint range of motion that is possible but to stay in the painless range.

Coordination exercises are important to improve balance and movement patterns and reduce the risk of injury. It is recommended to perform coordination exercises at the beginning of the training after warming up when the person is still in a resting state. In coordination as well as in strengthening exercises the main focus should rest on improving body awareness and increase the neural control of the movement according to the integrated model of joint function modified by Herbsleb et al. [59‐63], whereby a slow, focused and concentrated training is beneficial.

Strengthening exercises aim to stabilize the joints and the spine. Sufficient warm up before the training is important. As already mentioned, a slow, focused and concentrated training, starting in a small range of motion, is recommended. To properly learn the movement, few repetitions are suggested to start until the exercise can be performed confidently. One repetition should last 5–7 seconds. PwH are advised to keep breathing steadily and not to hold the breath. If the exercise is already learned properly, adults can exhale during effort and inhale during relaxation.

Flexibility exercises help to maintain or improve the joint range of motion and reduce the risk of muscle shortening/contractures. Adults should stretch after performing strengthening exercises at the end of the training, children should ideally stretch before and – if there is enough time – also after performing strengthening exercises. Stretching should be done equally on both sides with 3 repetitions per exercise in a pain-free range, each stretch lasting approximately 20–30 seconds. In general, all exercises should only be practised when they can be performed without pain. General recommendations regarding the composition of the 6 exercises are given in Table 2.

To start, the training composition is given in order to help PwH focus on the areas that may be the most effective in improving function and joint stability over the first 6–8 weeks; however, the recommendations can always be and should be individualized by the physician in charge. Over time, PwH are asked to vary the training by keeping the focus on one or two strengthening exercises as recommended to start with (adults: focussing on the chronically affected joint, children: primarily strengthening the hip abductors and abdominals) and in addition add one or two strengthening exercises for other muscle groups and keep that scheme for another 6–8 weeks. Coordination and strengthening exercises should be increased as given above according to the increasing fitness.

In order to take account of international recommendations to individualize the training for PwH according to the individual’s fitness, joint status and needs [1, 50], the Department of Physical Medicine, Rehabilitation and Occupational Medicine will offer physical medicine consultation hours for PwH. In this setting, PwH will receive individual recommendations regarding the composition and increase of the training, following a physical examination and additional radiological or functional diagnostical evaluation as needed. PwH can thereby also receive one-on-one physiotherapy, hemophilia-adapted group therapy, aquatherapy or bicycle ergometry as well as relaxation methods in a group setting or individually through biofeedback. Additionally, the physician can recommend certain modalities of physical therapy to be carried out at an institute close to where the patient lives or at the clinic (e.g. electrotherapy, ultrasound, laser, pulsed electromagnetic fields, massage) to reduce pain and improve function. The consultation hour will be offered once a month, a referral from a specialist is needed to schedule an appointment. For PwH living in and around Vienna, the appointment at the Department of Physical Medicine, Rehabilitation and Occupational Medicine will presumably go well together with the necessary follow-up appointments at the Department of Haematology or Pediatrics and thereby ideally complements the training offer for PwH.

In German-speaking countries attempts have already been made to educate PwH in multimodal disease-specific exercises and offer instructions to continue with a home-based training throughout the year. Sport camps take place once or twice a year and last for several days which are organized by the Austrian Haemophilia Society and the Interdisciplinary Center for Motion and Sports Medicine Wuppertal in cooperation with the University of Wuppertal in Germany and Switzerland [64, 65]. For children and adolescents in Germany, the Institute for Prevention and Aftercare (IPN) in Cologne offers so-called ‚Fit for Life‘ events. At these events, PwH receive an orthopedic joint examination and are tested regarding their physical fitness, motor skills and functional impairment. Depending on the findings, the boys then receive individual advice on home-based training and the choice of sports, an app helps children and their parents and teachers to choose appropriate school sports. Some of the recommended exercises at these events are also available at the given homepages [58, 65].

The online videos presented above aim to additionally support PwH with a regular home-based training. They also offer basic information on how to start training after being evaluated through specialists regarding sufficient factor therapy and contraindications for participating in the program. Data regarding the acceptance of the online program will be collected and presented at a later date.

Earlier studies concluded that exercise interventions for PwH are associated with specific challenges, such as the risk of injury, overload and potential bleeding. However, well-managed participation in exercise with adequate factor therapy and with respect to the individual’s joint status is known to improve a number of physical parameters [18, 36, 50, 66]. Regarding the safety of exercise interventions in PwH, a Cochrane review including eight randomized controlled trials assessed various training interventions in children and adults and found no training-induced adverse effects or bleedings [2]; however, regarding to the review’s authors, the safety of the applied interventions for severely affected patients remains unclear as some study groups used prophylactic factor prior to exercise and others included only subjects with moderate hemophilia [2]. It is especially important to be cautious when training is applied in patients with severe hemophilia, limited factor supply [2] or insufficient factor therapy, in patients with an inhibitor (an antibody directed against infused factor that inhibits the function of the factor) and with certain comorbidities. Adequate factor therapy can be a limiting factor for exercise in several countries [2].

Regarding strength training, static, dynamic and isokinetic exercises can be a part of a rehabilitation program for PwH as also recommended by Pietri et al. [31]. Exercising with relatively maximum resistance loads and roughly 6–10 repetitions to increase muscle mass and peak strength in healthy adults would cause a substantially higher risk of injury in PwH [38]. To strike a balance between strength improvement and a low risk of joint injury, several authors suggested reducing the risk by initially learning to use the accurate technique, training at submaximum loads, at a lower velocity, in limited joint ranges, or even isometrically at various joint angles [38, 67‐72]. Therefore, the recommendations regarding strength training focus on slow repetitions and a concentrated performance. The authors advise starting with a low number of repetitions in a limited range of motion and increasing the training as ability improves. These recommendations are consistent with Negrier et al. [50].

The recommendations presented in the videos can always be adapted and increased individually by the physician in charge. According to the literature, strength training recommendations should be used as a guide only. The appropriate exercise should always be adapted to the individual’s needs and should be prescribed by healthcare professionals trained in hemophilia care who ensure the appropriateness, sufficient factor therapy and protective gear if necessary [1, 50]. Therefore, each webcast points to the necessity of a sufficient factor protection and an approval of the training through specialists prior to starting the training (hematologist, pediatrician, specialist for internal medicine, orthopedist or a specialist in physical medicine and rehabilitation) and highly recommends an ongoing supervision throughout the training. For PwH living in and around Vienna, the physical medicine consultation hour at the Department of Physical Medicine, Rehabilitation and Occupational Medicine at the Medical University of Vienna offers an individual adaption and supervision of the exercises.

The cited Cochrane review on the safety and effectiveness of exercise in PwH assessed many study interventions that trained several motor skills [2]. Regarding the effectiveness of the included training interventions, the authors found that in most exercise interventions one or more of the measured outcome parameters including pain, range of motion, strength and walking tolerance improved [2]. Functional exercises, such as treadmill walking and partial weight bearing exercises seemed to be more effective than static or short arc exercises regarding improvements in muscle strength [2]. The findings were consistent with several non-controlled intervention reports in the haemophilia literature [2].

According to the American College of Sports Medicine (ACSM) guidelines for resistance training for healthy adults, dynamic training with intensities of at least 40–50% of the one-repetition maximum (or the appropriate correlate in maximum isometric force) with ≤ 15 repetitions is required to improve muscle strength. The existing literature suggests that in children and adults with hemophilia not only training in intensities according to the ACSM resistance exercise criteria but also low resistance dynamic, isokinetic and partly also isometric combined with dynamic training of sufficient frequency and duration seem to be able to increase muscular strength [17, 18, 37, 73‐79]. Regarding training frequency, ACSM guidelines for healthy adults recommend to train 2–3 times a week with a rest period to promote cellular adaptations [34]. In PwH, the training should be performed in accordance with the factor replacement regimen and the individual recovery time needed.

Increased physical fitness is known to have multiple benefits in PwH. Sufficient strength, flexibility and coordination are important to improve joint stability and function, increase bone mass and reduce the risk of injury which might possibly reduce the risk of bleeding. Therefore, a regular multimodal training is important for PwH. As the distance to hemophilia treatment centers with professionally guided exercise training may be a challenge for PwH who live too far away to participate regularly, the online video program was implemented to close the gap and offer easily accessible and free of charge training recommendations that can be applied at home on a regular base. It is important that the patients participating in the training are under sufficient factor therapy according to their hematologist or pediatrician. To ensure safety, the training should be prescribed and supervised through musculoskeletal specialists with experience in the field of hemophilia and should be adapted individually according to the individual’s needs. One possibility to do so is the consultation hour at the Department of Physical Medicine, Rehabilitation and Occupational Medicine at the Medical University of Vienna. Further information and access to the online videos is available through the clinic’s homepage: http://​www.​meduniwien.​ac.​at/​physmedrehab-videos.