Physical Activity for the Child with Hemophilia

Donna Clausen Boone, B.A.


Hemophilia is a hereditary disease of blood coagulation which results from a deficiency of certain plasma factors. Because these factors are absent, the normal sequence of events leading to clot formation does not occur. The most common types of hemophilia are A (classical hemophilia), and B (Christmas disease); each type lacks a particular plasma protein. Together they account for 95 percent of the clotting disorders termed hemophilia. Genetically, both hemophilia A and B are sex-linked, recessive disorders carried on one of the female X chromosomes. Transmission occurs in a Mendelian ratio with clinical manifestations appearing only in the genetically defective male offspring. Different degrees of severity are found in each type, these being static throughout life. Manifestations of the disease relate directly to the quantitative severity. The focus of this article is on the severely affected patient.

Treatment

Treatment of the disease involves the transient replacement of the missing clotting factor through intravenous administration of plasma concentrates. These concentrates are specific for the type of hemophilia; sufficient amounts of the particular plasma protein are infused to temporarily convert the clotting status to normal.

Multidisciplinary Approach

Since 1962, the Hemophilia Rehabilitation Center at Orthopaedic Hospital, Los Angeles, has provided comprehensive management of the many-faceted problems of hemophilia. Staffing of the Center is multidiscipline in scope and includes specialists representing medicine, dentistry, nursing, physical therapy, psychiatric social work, and vocational counseling.

Complications

The most disabling complications of hemophilia result from hemorrhage into the joints and muscles of the arms and legs. The bleeding apparently occurs spontaneously or is caused by the trauma of normal daily activities. Approximately 80 percent of hemophilic patients have had joint hemorrhages. Once bleeding has occurred in a joint it tends to recur. Knees, ankles, and elbows are most frequently involved although shoulders, hips, and wrists may be affected. During the stage of acute hemorrhage, the area may become quite painful and the arm or leg incapacitated temporarily.

Administration of Concentrate

With early administration of plasma concentrate following the onset of hemorrhage, the period of incapacitation is considerably shortened. However, the child may be unable to walk his usual distance, may have to use crutches, or may not be able to write rapidly. Patients can determine when they are bleeding before swelling or other external signs appear. Even very young children can detect a change in the daily condition of their limbs. Their judgment is reliable; their complaints should be considered seriously.

Joint Damage

Following repeated bleedings into the same joint, damage to the cartilage and bony structure of the joint occurs. Arthritic problems develop including limited joint motion, stiffness when the joint is in one position for a period of time, and varying degrees of pain and discomfort. Secondary to the joint damage, the muscles weaken because of the recurrent periods of pain and immobilization. As the muscles weaken, the support of the joint lessens which predisposes the limb to additional trauma from normal activities with resultant hemorrhage and greater chronic disability. Thus, a vicious cycle is established.

Reducing Bleeding Episodes

In order to reduce or eliminate some bleeding episodes, patients are encouraged to attain and maintain good physical condition. Additionally, specific exercise instruction is given for identified areas of weakness; for example, weakness of the quadriceps muscle group following repeated episodes of bleeding into the knee ( ). When the movement of one part of the body is improper or inadequate, other areas also suffer varying degrees of weakness. The exercise program considers the total body. Swimming and water exercises are particularly important for the body with hemophilia ( ). Movement in the water is easier than on dry land and the water environment is potentially less traumatic. When exercising is initiated by a hemophiliac who has not previously engaged in such a program, the plasma concentrates are administered prior to the exercise period. Thus, any joint bleeding attributable to the exercise is anticipated. Gradually, the concentrate is discontinued prior to exercise and the boy continues to exercise without the concentrate coverage. At this point, the musculature available to provide support for his joints is becoming adequate. Boys are expected to perform their exercises at home on a daily basis. Frequently, the physical-education period at school can be utilized with the cooperation of the teacher or coach ( ). When the boy is participating regularly in activities, such as swimming, bicycling, tennis, or golf, the specific individual exercise program may be unnecessary.

Types of Activities

Often, school personnel have questions concerning the kinds of activities or sports endeavors in which the hemophilic boy can engage. Many youngsters are banned arbitrarily from playgrounds while others are encouraged to participate in the regular physical-education program of the school. Each hemophilic child differs in his physical abilities, in the severity of his joint problems, and in the frequency and kinds of bleeding episodes he has incurred. His uniqueness should be considered when planning a physical activity program. For example, a boy with severe ankle or knee problems is unable to run well; hence, track and field activities may keep his joints chronically irritated. However, he will be able to swim, to bicycle, to play golf, and perhaps to play tennis. In establishing his program, counseling with the child and his parents is encouraged. He can then describe the activities he performs away from school; this information will be of help in planning school activities. In general, certain sports are encouraged; tennis, golf, badminton, cycling, and swimming are pursued successfully by a large number of hemophilic boys ( ). They are not encouraged to engage in vigorous contact sports such as football or basketball. A number of our patients have played Little League ball, some participate in intramural basketball, soccer, and other sports. Each child is encouraged to learn his own physical limitations and not to accept passively those superimposed by his parents, his doctors, his teachers, or his physical therapist. Younger children should be allowed to participate in playground activities so that they can have the opportunity to join in the usual play activities of their peers. Again, common sense and consultation with the boy and his parents can be useful guides to the appropriate type and amount of activity.

A child grows through experience. He learns by trying and by succeeding or failing. For the hemophilic child, some of this experience may produce bleeding episodes; however, these can be treated when they occur. The treatment of severe emotional and physical problems occurring in an adult who was not permitted to grow through experience is much more difficult. Some hemophilic boys have been and are overly restricted by their parents from participation in the usual boyhood activities. When school personnel encounter such a situation, the staff of a hemophilia treatment center may be helpful to the school in planning appropriate events. A child should not be excluded from playground activity or forced to spend recess in the principal's office simply because he has hemophilia.

Flexibility Needed

Hemophilia is a variable disease; i.e., frequency of bleeding episodes varies greatly even in the same child. Hence, his physical capabilities will vary with the frequency and severity of his bleeding. A school program should be flexible enough to accommodate for this disease variability. For example, the boy may not be able to play tennis, or swim, or run on the playground the day he is recovering from a bleeding episode into his knee. However, as soon as the knee has recovered, he should resume his usual activities.

Assistive Devices

Certain assistive devices may be used temporarily to support a joint in which bleeding has recurred frequently or to correct a deformity. Fewer crippling deformities are found in younger children since these bleeding problems are now controlled more effectively by the plasma-concentrate program. However, residuals from the preconcentrate era are still evident. The simplest devices encountered in school are crutches or canes. When he is using these aids, a student may have difficulty in carrying loads of books, in getting from class to class rapidly, and in going up and down steps. When crutches are used for an extended period, some arrangements should be made to assist the student in carrying books. Scheduling his classes on one level is an additional aid.

An elbow splint may be worn to protect the joint following hemorrhage; the elbow is prevented from overuse but the hand can be used for writing and other activities. Ankle supports may be used to protect arthritic ankles from everyday trauma and decrease discomfort. These supports fit into the shoes and are relatively undetectable by classmates and others. Occasionally, walking splints of a thermoplastic material or plaster are worn to provide support for the knee until muscle strength is adequate. These splints do not allow the knee to bend; therefore, some seating modification should be made for the student wearing one since he may be unable to properly place his splinted leg under his desk. The majority of patients, particularly those under ten years of age, use these devices only rarely. Older boys may require devices more frequently because of chronic joint problems incurred prior to better control of bleeding.

Summary

Bleeding episodes in patients with hemophilia can be treated. Therefore, crippling joint problems can be reduced through prompt control of the hemorrhage by administration of plasma concentrate. Boys with hemophilia are extremely knowledgeable about their disease. They know when bleeding starts into a joint or soft tissue area; their judgment is reliable and should be trusted so that prompt treatment can be instituted. They can also explain reasons for certain treatment measures such as why they are using a particular splint or crutches. Many hemophilic boys have elected to tell their class or the school's health classes about hemophilia as a special project. The frequency and severity of bleeding episodes vary in the individual boy and between them. The school program should take this variability into consideration.

Excessive body weight places stress on the knees, ankles, and hips. When chronic arthritis is present, this stress is intensified. Since school personnel have more frequent contacts with hemophilic boys than do personnel at treatment centers, they may observe the weight gains of patients more readily and be able to undertake appropriate counseling measures.

Areas of particular muscle weakness should be identified and specific exercise programs instituted to correct the weakness. The exercise program may be supervised by treatment center physical therapists, or by those in the boy's community. Contact between the physical therapist and the physical education teacher is helpful, so that specific exercises can be incorporated into the hemophiliac's physical-education program. Boys with hemophilia should be encouraged to establish patterns of physical activity which will maintain them in the best possible physical condition. Routine swimming is especially recommended. Types of recreational sports will vary from person to person because of individual preferences and extents of physical capability, as well as limitations imposed by chronic joint problems. Each boy, however, should be intimately involved in the selection of appropriate sports activities.

A hemophilia treatment center can serve as a useful aid to school personnel if questions arise about hemophilic students. Additional information can be provided about either general or specific problems related to hemophilia.

Finally and emphatically, boys with hemophilia should be regarded as normal boys who happen to have a chronic, variable, and sometimes inconveniencing disease.

Hemophilia Rehabilitation Center Orthopaedic Hospital, Los Angeles, California