The Effects of Exercise Training in Children With Hemophilia
Condition: Hemophilia
MeSH terms: Hemophilia A
Study type: Interventional

Summary: 
      This study aims to investigate the effects of an individually planned exercise program,
      counseling and family education on the followings in patients with hemophilia (PWH);

        1. To examine the joint range of motion, muscle strength and functional status.

        2. To evaluate the level of physical activity and daily living activities

        3. To aim to determine the amount of change after participation.
    

Detailed description: 
      Hemophilia is defined as bleeding in the musculoskeletal system and soft tissues as a result
      of the lack of clotting factors in the blood. Recurrent attacks of hemarthrosis cause pain,
      also because of the pain patients tend to use joints less than usual. The decrease in joint
      movement makes it harder to return to active movement again. There is a limited literature on
      the effects of exercise on children with hemophilia.

      Hemophilia is a hereditary bleeding disorder that develops as a result of factor VIII or IX
      deficiency; it is a rarely seen disease that discloses itself by hemarthrosis and
      intramuscular bleeding. Factor VIII deficiency is called Hemophilia A and Factor IX
      deficiency is called Hemophilia B. This disease is inherited by the X chromosome and passes
      on to male children through female carriers. Although Hemophilia A is observed rarely, one in
      about 5,000-10,000 male births, its frequency is still 5-6 times higher than Hemophilia B.
      Around 70% of hemophilia A patients have more severe disease, however, this rate is
      approximately 50% in hemophilia B.

      Hemophilia patients experience bleeding in the musculoskeletal system and soft tissues as a
      result of the lack of clotting factors in the blood. Recurrent attacks of hemarthrosis cause
      pain, also because of the pain patients tend to use joints less than usual. Decrease in joint
      movement makes it harder to return to active movement again in the majority of the cases,
      bleeding occurs into the joint (mostly the knee, elbow and ankle) and only a small part of
      the bleedings occurs into the muscle (especially the iliopsoas, calf and forearm muscles).

      In order to be able to participate according to the international classification of functions
      of health (ICF), it is prioritized that the activities are functional.

      Targeted treatment is a functional treatment application that conforms to ICF's "activities
      and participation among children. An activity is carrying out of a task or movement by an
      individual and the difficulties faced while carrying out the tasks or movements is called
      limitations. Participation means the individual's being in life. In life conditions, problems
      confronted are participation restrictions. Activity limitations can often lead to
      participation restrictions.

      Fear of bleeding in pediatric patients, particularly from the period of childbirth leads
      families to form an immobile lifestyle for their children compared to healthy individuals.

      The recurrent bleeding often occurs in the lower extremity joints, it causes pain and
      swelling, therefore patients need to keep the joint immobile which is particularly the reason
      why patients spend these periods mostly resting. So then, again, patients forfeit activity
      for fear of recurrent bleeding. The decrease in activity leads to a loss of strength and
      balance. Also, sports that could lead to bleeding or trauma are not suitable for hemophilia
      patients. The benefits and risks of sports activities for hemophilic individuals should be
      well known and the patient should be oriented toward suitable sports. For this reason the
      rehabilitation program should be arranged as a personalized and systematic program according
      to local and general joint and muscle conditions.

      Hematologic therapy alone is not sufficient for the treatment of musculoskeletal bleeding.
      The sedentary lifestyle that develops with hemophilic arthropathy often leads to immobility
      problems such as decreased muscle strength, weak balance and coordination, increased risk of
      obesity. These problems lead to instability and changes in joint loads, thereby instances of
      new bleeding and increased joint damages. In a similar way, after hemarthrosis, in acute
      situations, if the necessary period of resting is not accompanied by an appropriate
      physiotherapy, it causes a vicious cycle of immobility, new bleeding and eventually
      hemophilic arthropathy and loss of function.

      The key to the successful management of hemophilic arthropathy is to begin the treatment
      early. The first step of treatment for hemophilic arthropathy is physiotherapy with factor
      replacement to prevent bleeding attacks and joint damage before hemarthrosis progresses to
      chronic synovitis and joint erosion.

      Physical activity means body movement by using energy and exercise means planned physical
      activities that are personally structured. Regular exercise can prevent the progression of
      hemophilic arthropathy or it may reduce the risk. Therapeutic exercises are the main elements
      of rehabilitation for Patients with hemophilic arthropathy and muscle bleeding.

      The main objective of therapy should be the following: restoration or preservation of range
      of motion, strengthening of muscles, prevention or treatment of joint contracture, pain
      management, increasing exercise tolerance, improvement of balance, coordination, and
      proprioception. To prevent patients from giving up on exercising earlier than required, İt is
      important to assist patients, encourage them during exercises, support them and when
      necessary give them advice and explain the exercises in a way that patients can easily and
      clearly understand. There is a fine line between beneficial and harmful activities, this fine
      line leads many patients with bleeding disorder to avoid exercise for fear of causing
      bleeding. Paradoxically, when people with bleeding disorders avoid exercising this causes
      decreased function, range of motion, and quality of life because of muscle weakness.

      In Turkey, the cost of joint health complications in patients with hemophilia and other
      inherited bleeding disorders is significantly high however most significant point is that
      these costs can be avoided. Physical activity which is a non-pharmacologic treatment method,
      plays an important role in reducing the risks, alleviating the symptoms and improving the
      results in hemophilia. For this reason, it is very important to develop strategies to
      increase the level of physical activity.

      In the relevant literature there are very few studies on the effects of exercise in pediatric
      patients with hemophilia. With this study, we plan to show, in an objective manner, that the
      personally planned exercise program leads to change in the level of physical activity,
      activities of daily life and participation.

      The Methods of the Study

      Before the study the patients and their families will be informed about the evaluations,
      treatments that will be made. They will be also informed about outcomes and evaluations after
      the treatment, and the risks that they may face during the the treatments. They will also be
      asked to sign the "Informed Consent Form" for their voluntary participation in the study.

      An "Assessment Form" will be prepared in order to keep the record of demographic and disease
      information of all the cases included in the study. Hemophilia types, patients biographic
      information, family history, the time when the disease was diagnosed, treatments taken,
      medications used, body mass index, range of motion and muscle strength will be recorded in
      this assessment form.

      Randomly patients will be divided into two groups as physical activity counseling and
      individually planned exercise group and only physical activity counseling group. We plan that
      the exercise will be done for 50 minutes a day, twice in a week and will last for 8 weeks.

      Before and after the 8-week intervention program of all patients physical activity levels
      with International Physical Activity Questionnaire (IPAQ), activity performance exercise with
      Children's Functional Independence Scale (Wee-FIM) and Canadian Occupational Performance
      Measurement (COPM), joint health with hemophilia joint health scoring (HESS), exercise
      capacity with 6-minute walking test (6MWT) and sit-and-stand test, hand function speed and
      dexterity with Jebsen-Taylor Hand Function Test (JTHFT) and quality of life with Pediatric's
      Quality of life Scale (PedsQL) will be evaluated.

      Assessment methods, exercise and counseling practices will be carried out in the Turkey
      Hemophilia Society of Physiotherapy Unit.Only the participants in the hematologists follow-up
      will continue their routine doctor checks and recommendations.

      All analyzes will be performed using Statistical Package for Social Sciences (SPSS) for
      Windows 22. The compliance of the data to normal distribution will be evaluated by drawing
      the Kolmogorov-Smirnov test and histogram. Parametric tests will be used for data suitable
      for normal distribution. Data that are not suitable for normal distribution will be analyzed
      with non-parametric tests. Variables will be defined by their mean and standard deviation
      values. Analyzes and correlations between groups will be examined separately with appropriate
      tests. A value of p<0.05 will be considered statistically significant.
    

Intervention name(s): Experimental group 1; Experimental group 2
Intervention type(s): Behavioral; Behavioral
Intervention description(s): It is planned to do exercises for 50 minutes a day, 2 days a week during 8 weeks with physiotherapist. It will be planned individually according to the needs of the patients. Exercises will progress in the following weeks by changing the number of repetitions, the amount of resistance and the amount of time. Counseling about physical activity and the suggested sports activity will be provided. In daily living activities ergonomics and joint protection principles will be taught. Informing Family.; Counseling about physical activity and the suggested sports activity will be provided.Patients make aerobic and strengthening exercises by himself based on physiotherapist's suggestions. Informing Family about; Why are the exercises necessary To what extent the families should be protective The harmful effects of inactivity There will be information about the resting-activity cycle when there is bleeding.
Other intervention name(s): exercise
Allocation: Randomized
Intervention model: Factorial Assignment
Masking: None (Open Label)
Primary purpose: Prevention
Keywords: hemophilia; exercise; participation; physical activity; muscle strength