Rehabilitation of Joint Surgery in Hemophiliacs

Fig. 12.1

Sixteen-year-old hemophilia patient with musculoskeletal impairment in several areas. Note the kyphoscoliosis, pelvic obliquity, arthropathy in both elbows and the left knee with reduced extension in these joints, and muscle hypotrophy. He also has arthropathy of the left ankle with flat foot and valgus deformity and acute hemarthrosis in the right knee with genu valgus deformity

Table 12.1

Overall assessment of the patient with hemophilia

Hemodynamic status of the patient

Static postural

Pain focused on bony structures or soft parts

Joint mobility ranges

Trophism and muscle strength

Deformities or pathologies associated in the kinetic chain

Osteoarticular problems in other limbs or vertebral column

Neurovascular status of member

Bimanual skill and walking if possible

Cardiorespiratory impairment, concomitant pathology, age, previous immobility, cognitive status, sequelae of previous injuries, medications, etc.

It is advisable to use rating scales to unify criteria, measure the effectiveness of the operation carried out, and monitor the patient’s clinical progress throughout the various stages of treatment. For adults, Gilbert is the most widely used scale [1–3]. On the other hand, in order to be able to objectively quantify these patients’ functional level, it is essential to use assessment tools that take into account the patient’s psychological recovery and their social integration, such as the functional rating scales. Although no specific scales are available for hemophilia patients, the generic ones most commonly used in daily clinical practice are: FIM (Functional Independence Measure), Barthel index, and HAQ (Health Assessment Questionnaire) [1–3].

12.4 Objectives of Rehabilitation

Once the patient’s clinical and functional status has been evaluated through clinical and functional assessments and image tests, the course of action can be determined. There is no doubt of the effectiveness of rehabilitation for hemophiliac patients undergoing joint surgery, and there is virtually unanimous agreement on the benefit of applying it throughout the process.

The ultimate objectives are to: prevent complications, improve the functionality of the limb operated on, and prepare the patient for carrying out their everyday activities independently. This applies, in the case of surgery on joints in the upper limbs, to improving the manual dexterity and skill necessary for everyday activities, and, in the case of lower limb surgeries, to gait reeducation and training in walking up and down stairs. These overall objectives can be broken down into more specific objectives, which are shown in the Table 12.2.

Table 12.2

Objectives of rehabilitation in hemophiliacs undergoing joint surgery

Relieve pain

Regain range of joint motion

Improve muscle strength and trophism

Regaining proprioception and muscle balance

Prevent muscle atrophy, deformities, demineralization, and other sequelae

Improve functional capabilities

Maintain an appropriate pattern of movement when walking

Improve quality of life

It is essential to establish realistic therapeutic goals and determine the program and treatment techniques that will be used to achieve these goals. The therapeutic program should be individualized, adapted to the situation of each patient [10].

Ideally, the rehabilitation treatment should be started early, even if the segment involved needs to be immobilized, as this does not have to mean that the patient is completely immobilized.

The success in treating these patients will also depend on the work done by a multidisciplinary team of professionals collaborating on a common goal (orthopedic surgeon, hematologist, rehabilitation physician, nurses, physiotherapists, occupational therapists, orthopedic technicians).

12.4.1 Preoperative Rehabilitation

The first preoperative visit should take place 4–6 weeks prior to the operation. In it, the doctor responsible for the rehabilitation treatment must evaluate the patient and inform him or her on the postoperative process and the need to be receiving hematological treatment while the physical treatment is ongoing [11]. Clear information allows reducing patient’s anxiety, analgesic use and the hospital stay after surgery. It is also important to determine how motivated the patient is to participate in a postoperative rehabilitation program; those patients who are not motivated enough may not be accepted [2, 12].

A physical and functional assessment of the patient will identify any mobility problems and specific needs to assist in moving about after the operation. As a result, it will be possible to order any equipment that may be required during the postoperative period to assist with adaptation, in order to ensure that it is delivered to the patient on time. This type of equipment may include wheelchair, crutches, orthotics, footwear, technical aids, or adaptations for home [2].

Preoperative physiotherapy programs are not indicated in all cases, because for some conditions they have not been shown to improve the results over the long term [2, 12]. It must be borne in mind that if a patient with hemophilia is to undergo surgery on a joint, it is because other more conservative therapeutic interventions, including rehabilitation, have previously been tried and failed to solve the problem.

Patients should always be instructed to do the rehabilitation exercises learned in prior therapeutic programs daily at home [2].

If it is decided that new preoperative rehabilitation treatment should be prescribed, the objective will be the same. These programs may include respiratory therapy techniques, postural standards, spinal hygiene, global kinesitherapy of all four limbs, balance techniques, learning transfers, and use of external aids. Table 12.3 reflects the contribution of medical rehabilitation during the preoperative period.

Table 12.3

Contribution of rehabilitation physician in the presurgical phase of the hemophiliac patient

Explain the postoperative process

Inform as to realistic expectations about the results to be expected in the long term

Determine the patients’ degree of motivation to participate in a rehabilitation program

Perform a physical and functional assessment of the patient before the operation

Identify possible mobility difficulties that could affect postoperative gait

Prescribe the equipment that may be required for adaptation after the operation (crutches, orthosis, footwear, technical aids,..)

Consider the patient’s social environment, family support and the need for adaptations to the home

Advise that the exercises learned in previous therapeutic programs must be followed daily at home

Prescribe additional sessions of physiotherapy/occupational therapy to work on specific techniques, make transfers and use external aids under the supervision of a therapist

Bring the patient to the best possible condition in general and functional terms in preparation for the operation

12.4.2 Postoperative Rehabilitation

Postoperative rehabilitation programs are fundamental to optimizing the results of surgery and are nowadays considered essential to ensure proper improvement after surgery. That is to say, there are no doubts as to the benefits of such programs. It is important to note that surgery can improve hemarthrosis and pain, but not all the associated problems that have evolved over the years. In this respect, arthroscopy makes it possible to perform a synovectomy and perform chondral debridement but will not solve the muscle atrophy or the reduced joint mobility, the instability, or loss of proprioception. The osteotomies and the arthroplasties usually improve mobility, joint stability, and deformity correction [5].

The patient should be reassessed as soon as possible after surgery, in order to ascertain their clinical and physical status and to be able to establish goals for rehabilitation. The orthopedic surgeon can describe the assessment made during surgery, any problems encountered, and the plan to have the patient kept immobile or non-weight-bearing, if necessary, and so must be contacted to obtain all this information. Additionally, the hematologists must be in touch, since good postoperative hemostatic coverage is vital during rehabilitation. All members of the team should have experience in handling people with hemophilia [13].

If there were no complications with the operation, the therapeutic rehabilitation program should begin early, during the period of hospitalization, and then continue as an outpatient program.

During the first 24–48 h after surgery it is paramount to control hemostasis and pain; also to follow some postural recommendations that will allow the patient to maintain the operated limb in good alignment and in good articular, muscular, vascular and neurological state.

Immediately after this period, a more demanding therapeutic program can begin, in which an experienced therapist will apply the physiotherapy techniques appropriate to each case. One or two treatment sessions per day are usually recommended for a period of about 6 weeks, depending on the clinical evolution [2, 14]. Rehabilitation of a patient with hemophilia has some specific characteristics that must be borne in mind and are described in Table 12.4. To reduce the risk of infection through the surgical wound, the highest possible hygiene standards must be maintained. Pain medication will be used throughout the whole time the patient is undergoing rehabilitation treatment. Hemostatic coverage is essential; if clotting factor infusion is not continuous, physiotherapy and occupational therapy should be scheduled on the basis of the timetable for clotting factor infusion [2]. According to the latest published guides: during the first three days after the operation, patients must maintain clotting factor concentrations of 60–80 IU/dl (in hemophilia A) and 40–60 IU/dl (in hemophilia B); for the fourth to sixth days, these levels should be 40–60 IU/dl (in hemophilia A) and 30–50 IU/dl (in hemophilia B); during the second week, the recommended level is 30–50 IU/dl (in hemophilia A) and 20–40 IU/dl (in hemophilia B) [13], in order to maintain concentrations of at least 40 %, and patients may begin to receive one infusion per day. During the third and fourth weeks after the operation, patients receive 20–40 IU/kg/day, depending on the clinical condition of the joint that has been operated [2]. Therefore, if there is any increase in inflammation or pain during the rehabilitation period, it is recommended that an immediate treatment of a full dose of clotting factor concentrate is administered for several days, until symptoms disappear. Before removing the sutures, factor concentrate should be administered if it has not yet been administered on a regular basis [2]. The general guidelines for a rehabilitation program after joint surgery without complications in patients with hemophilia are shown in the Table 12.5.

Table 12.4

Specific aspects of the postoperative rehabilitation program in patients with hemophilia

Maximize hygiene standards and the treatment during assessment

Maintain proper hemostatic coverage during the rehabilitation period, to reduce the risk of bleeding

Control pain with the use of multimodal analgesia and avoiding NSAIDs

Schedule physiotherapy and occupational therapy sessions to coincide with clotting factor infusion

Close monitoring of possible complications, which usually require hematological and rehabilitation therapy to be adjusted immediately

In those joints for which there is a significant risk of developing a flexion contracture, a hinged orthosis is very useful

To achieve maximum functional recovery, any preexisting musculoskeletal problems must be considered and treated

Table 12.5

The general guidelines for a rehabilitation program after joint surgery without complications in patients with hemophilia

1–3°day	Postural control. Full joint extension. Passive or assisted mobilization of 0–40°. Start isometric muscular work^a. Sedestation. CPM can be used for arthroplasties
4–7°day	Increase the movement of the joint (progressing at a rate of 5–10°per day). Isotonic muscle work^a. Bed-chair transfers. Progressive increase in bipedal standing and walking with partial weight-bearing^b
2–4°weeks	Release of soft tissues. Progress in ROM, muscle strength^a, and joint balance. Reeducation of the gait pattern and recovery of functional skills
>4°week	Walking around obstacles, ramp, stairs. Reeducation in everyday activities (personal hygiene, clothing.)

Progression in the treatment will depend on postoperative time and each patient’s own progress

CMP continuous passive motion, ROM range of motion

^aIn the case of myotendinous operations, active muscle work must be avoided for at least 3 weeks

^bIn the case of osteotomy or arthrodesis, the discharge time stated by the orthopedic surgeon must be observed

12.5 Most Commonly Used Rehabilitation Techniques

The most commonly used rehabilitation techniques are described below.

12.5.1 Prevention of Venous Thrombosis

In addition to strict hematological control, early mobilization, intermittent pneumatic compression therapy, and the use of compressive elastic stockings have been shown to be effective in preventing deep vein thrombosis. Other physical measures such as voluntary muscle contraction and massage therapy to improve circulation may also be used.

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