Design of a Myofascial Therapy Protocol for the Treatment of Hemophilic Arthropathy of the Knee and Ankle

Abstract

Hemophilic arthropathy limits daily life activities of patients with hemophilia, presenting with clinical manifestations such as chronic pain, limited mobility, or muscular atrophy. Although physical therapy is considered essential for these patients, few clinical studies have demonstrated the efficacy and safety of the various physiotherapy techniques.

Physical therapy may be useful for treating hemophilic arthropathy by applying safe and effective techniques. However, it is necessary to create protocols for possible treatments to avoid the risk of bleeding in these patients.

This article describes the musculoskeletal pathology of hemophilic arthropathy and characteristics of fascial therapy. This systematic protocol for treatment by fascial therapy of knee and ankle arthropathy in patients with hemophilia provides an analysis of the techniques that, depending on their purpose and methodology, can be used in these patients. Similarly, the protocol's applicability is analyzed and the steps to be followed in future research studies are described.

Fascial therapy is a promising physiotherapy technique for treating fascial tissue and joint contractures in patients with hemophilic arthropathy. More research is needed to assess the efficacy and safety of this intervention in patients with hemophilia, particularly with randomized multicenter clinical trials.

Introduction

Hemophilia is a congenital coagulopathy characterized by the deficit or absence of either of the coagulation factors: FVIII (hemophilia A) or FIX (hemophilia B). This disease is characterized by bleeding episodes occurring primarily in the joints (hemarthrosis) as a result of minor traumas or even spontaneously.¹ Hemarthrosis in young patients with severe or moderate hemophilia occurs mainly in the knees, ankles, and elbows.²

The perpetuation of subclinical hemorrhage or overt bleeding, toxic iron depositions, synovial inflammation, tissue wear, and mechanical instability may contribute to the progression of hemophilic arthropathy.³ Finally, as a result of chronic synovitis and cartilage damage, a joint degeneration process known as hemophilic arthropathy⁴ is established. This kind of joint disease presents with limitations in range of motion (ROM) in joints, chronic pain,⁵ loss of muscle strength,⁶ and proprioceptive disorders.⁷

The progressive loss of joint movement can be explained by three processes: (1) muscle atrophy; (2) capsular contraction; (3) and restriction of fascial tissue. Following each hemorrhagic incident, a periarticular reflex muscle inhibition appears that promotes muscular atrophy. As joint deterioration progresses, muscle deficit increases and, in this manner, the joint loses flexibility and muscle contractility. Young patients with hemophilia have been observed⁸ to have less strength in the extensor muscles of the knee and the plantar flexor of the ankle, decreasing the size of the vastus lateralis and lateral gastrocnemius muscles, compared to healthy people of the same age.

Similarly, shrinkage of the joint capsule associated with joint degeneration and bone deformities—which are typical of hemophilic arthropathy—add further restriction to joint mobility. ROM limitations have been associated with age, non-Caucasian ethnicity, and increased body mass index in patients with hemophilia.⁵

The Fascial System

The fascial system also has a major role in this process of joint deterioration. Fascia is connective tissue forming a three-dimensional network that surrounds and supports the muscular, skeletal, and visceral components of the body. The role of fascia is currently being studied by the scientific community, especially with regard to its biomechanical behavior, which is similar to that of tensegrity structures.^9

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The superficial fascia is typically in the subcutaneous tissue, mixing with the reticular dermis layer. It consists mainly of loose connective tissue and fat. This type of fascia also covers the lining of the organs, glands, and neurovascular bundles. Fascia also acts as a storage medium for fat and water, and as passages for the lymph, nerve fibers, and blood vessels.

The deep fascia is the dense fibrous connective tissue that surrounds and penetrates the muscles, bones, nerves, and blood vessels in the body. The density of collagen fibers is what gives the deep fascia its strength and integrity.¹³ The amount of elastin fibers determines the fascia's extensibility and strength.

The causes of restriction in the fascial system can vary. Mechanical stress, inflammation, repetitive poor posture or movements, or healing of wounds of surgical or traumatic origins, degenerative joint processes, and, as in the present case, repeated episodes of joint hemarthrosis can cause this limitation.

Fascial Therapy

In the therapeutic field, the different approaches of the fascial system manuals suggest removal of the restrictions present in both the superficial fascia and deeper fascial structures. Fascial therapy or myofascial release (MFR) therapy includes various therapeutic modalities and methods of manual and instrumental treatment. In general, the goal of these techniques is to eliminate restrictions of the fascial system with the aim of restoring tissue health. Researchers, such as Schelip,¹⁴ hypothesized that fascial restrictions in a region of the body cause excessive stress in more distant regions because of the continuity of the fascial system. These restrictions in the fascial system may result in increased pressure and stress on the body through the soft tissue, causing pain and dysfunction. MFR therapy usually involves working on restricted fascial layers, either directly (direct MFR) or indirectly (indirect MFR).

Direct techniques are designed to act on the fascia against the restriction barrier. The therapist uses hands, knuckles, elbows, or other therapeutic tools to slowly manipulate the fascia. The physical therapy approach of this technique involves application of a pressure of a few kilograms of force in the direction opposed to the restriction.

Indirect MFR techniques involve mild stretching guided in the direction of least resistance (toward the easier side). The pressure applied is a few grams of force and the hands tend to follow the direction of fascial restrictions until perceiving clearly that movement stops—this is known as the “barrier zone.” The position acquired must be maintained by the physiotherapist without forcing the tissues to allow self-liberation of the fascia.¹⁵ The rationale behind these techniques is defined by the plasticity, and the viscoelastic and piezoelectric properties of connective tissue described in several studies.^16,17

The connective tissue often densifies as a result of excessive and inappropriate use, after traumatic injury, or as a result of chronic musculoskeletal pathology. This densification response may be the result of an altered composition of collagen fibers, fibroblasts, or ground substance. Altering fascial flexibility could be a source of poor alignment of the body and could lead to deterioration of the biomechanics of the soft tissue, favoring postural misalignment, and a decrease in strength and motor coordination.¹⁸

Background of Manual Therapy for Treating Hemophilia

Although hemophilia presents with a predominantly orthopedic clinical pattern, manual therapy for treating the physical consequences of this disease is limited. Reviewing the literature revealed that there were few studies in which the effectiveness of the manual techniques for treating hemophilic arthropathy was assessed.

Heijnen and de Kleijn ¹⁹ were the first researchers to conduct a study using articular traction techniques on 20 patients with hemophilic arthropathy. The researchers observed improvement in the patients' ROM without bleeding episodes during the intervention period. Recently, the effectiveness and safety of orthopedic manual therapy techniques^20,21 was noted following Kaltenborn's criteria.²² Physiotherapy intervention over 6 and 12 weeks improved mobility and reduced joint pain in 9 and 11 patients with ankle arthropathy, respectively. As in the study conducted by Heijnen and de Kleijn,¹⁹ no episodes of hemarthrosis or muscle bruising were observed as a result of the intervention in either study. To date, no study has examined the safety and effectiveness of a physiotherapy intervention using fascial therapy in patients with hemophilic arthropathy. Currently, this technique is contraindicated in physiotherapy treatment of patients with hemophilia, although no complications or adverse effects have been found when applying fascial therapy to these patients.

This article presents a systematic protocol for treating hemophilic arthropathy with fascial therapy, based on the published evidence. Similarly, the methodology used in the development of clinical studies is presented, confirming the safety and efficacy of this technique in patients with hemophilia, and ankle- and knee-joint disease.

A Systematic Protocol for Physiotherapy Treatment of Hemophilic Arthropathy with Fascial Therapy

Treatment Sequence

The treatment program consists of 3 fascial-therapy sessions conducted over a period of 15 days, using a protocol designed ad hoc for this study (Table 1). The estimated treatment time per session is 45–60 minutes.

Table 1.

Protocol for Myofascial Therapy for Treating Hemophilic Arthropathy of the Knee and Ankle

Type	Maneuver	N	TP	TA	Comments	Time
	Superficial sliding of the anterior part of the leg	3	1	2	The strokes are performed, assisted by slight movements of dorsiflexion and plantar flexion by the patient.	2 min
	Superficial sliding of the anterior part of the thigh	3	3	—	None.	2 min
	Superficial sliding of the popliteal fascia	3	4	—	Movements are applied to the popliteal region. The maneuver is repeated 3 times.	2 min
Superficial	Superficial sliding of the posterior part of the leg	3	1	2	The strokes are performed assisted by slight movements of dorsiflexion and plantar flexion.	2 min
	Superficial sliding of the posterior part of the thigh	3	3	—	The 1st line is on the region of ease. The 2nd is on the outer thigh. The 3^rd is on the inner thigh.	2 min
	Superficial sliding of the side part of the lower limb	3	3	—	The maneuver starts at the trochanteric region and ends in the posterior region of the lateral malleolus.	2 min

	Maneuver	Comments	Time
	Ankle joint complex	Overcome between 3 and 5 restriction barriers.	4–6 min
Deep	Anterior compartment of the knee	Hands crossed technique is applied to the anterior compartment of the knee.	4–6 min
	Thoracolumbar fascia	Hands crossed technique is applied to the thoracolumbar region.	4–6 min
	Telescopic maneuver	This involves degravitation and slight traction of the lower limb.	5 min

All maneuvers except No. 9 (thoracolumbar fascia) are performed in both lower limbs.

Type, Type of maneuver; N, number of strokes (total sliding maneuvers to be performed); TP, passive stroke (stroke made passively over the superficial fascias without assistance by the patient and usually applied in the sense of restraint); TA, assisted stroke (assisted strokes with the patient's cooperation); min, minutes.

Protocol Description

The manual therapy protocol applied to the fascial system was specifically designed for use in patients with hemophilic arthropathy of the knee and ankle. The treatment sequence consists of 10 maneuvers: 6 of longitudinal superficial sliding through direct techniques (direct MFR) and 4 of indirect-type deep myofascial induction (indirect MFR). The fascial therapy protocol is freely available online by the Universidad Católica San Antonio of Murcia, Spain.²³

Longitudinal Superficial Sliding Techniques

In this kind of maneuver, the therapist performs a longitudinal slide over the superficial fascia to identify and release restrictions that are present. This is a direct and structural type technique (direct MFR), because, by identifying the restriction zone, the maneuver provides a stimulus to overcome the restriction barrier.

Each maneuver comprises 3 strokes with which the physiotherapist, through manual action on the tissue, promotes release of the superficial fascia. In maneuvers 1 and 4, sliding is applied to the anterior and posterior regions of the leg, but it should be kept in mind that both the second and third sliding stroke are performed in combination with a contraction assisted by the patient through slight movements of dorsiflexion and plantar flexion of the ankle.

Deep Sliding Techniques

Generically speaking, deep techniques are framed within the so-called functional techniques (indirect MFR). Working with deep maneuvers does not mean applying more force to the techniques, but rather changing the orientation of the manual stimulation. Unlike sliding maneuvers, in deep maneuvers the physiotherapist acts as a facilitator. Thus, treatment is performed based on the response of the patient, through feedback of the tissues, as they form the barriers and thus the progress or completion of the procedure.

During the interactive process of information exchange in these maneuvers, the restrictions of the myofascial system are removed. This action allows the release of the restriction bands and possible sore spots that restrict movement or hinder the normal movement pattern that creates restrictions, dysfunction, and pain.

Through this maneuver, the spontaneous movement of the patient's body is used to reach the deep restrictions. Subsequently, using the properties of the fascial system (piezoelectricity, thixotropic reaction, and tensegrity system), the therapist helps the patient's body to allow the releases.

In the deep techniques—7, 8, and 9 of the protocol—the following sequence should be considered:

(1) Respiratory synchronization—The therapist places hands gently on the patient and takes several simultaneous breaths (3–5 breaths).

(2) Load the fascia—The therapist induces a slow and progressive pressure on the patient (varying the type of stimulus depending on the region of treatment), respecting the tissue adaptation to the stimulus. If the stimulus is adequate, a slow process of energizing the structure in question begins (usually achieved by a gentle longitudinal stretching and a vertical thrust).

(3) Continuation of maneuvers—The therapist continues the fascial “pull up” to the first restriction barrier (usually a stop in motion is identified). At that time, the therapist must sustain the stimulus without forcing the tissues awaiting a new perception of fascial movement, indicating that a restriction barrier has been overcome.

Three to five consecutive barriers must be overcome in each maneuver. The physiotherapist should follow the fascia in the small micro-movements and consider that this is a three-dimensional type of movement.

There is no time limit for the folded-hands technique, but 3–5 minutes is the minimum time to consider. Once the maneuver is completed, the hands should be removed slowly and gradually. The times used in this protocol for deep maneuvers are slightly longer than those described by Barnes,²⁴ who identified MFR response times between 90 and 120 seconds.

For the development of maneuver 10 (the telescopic technique), the therapist is guided by the response to the stimulus of stretching when straining the patient's lower limb. The maneuver starts with a slight degravitation of the treatment area and gentle traction. After three telescopic release barriers from this movement, the therapist must be aware of any intention of movement or activity by the patient. Adjustment movements in rotation, adduction and abduction, flexion, or extension should be allowed. Meanwhile, the physiotherapist must maintain constant traction without getting ahead of, or staying behind, the patient's movement.

Discussion

Fascial therapy does not replace any conventional therapy. However, it is offered as an excellent addition to work within a multidisciplinary approach for treating a chronic disease. MFR therapy can be useful for patients who have chronic pain.

Hemophilic arthropathy, in which functional limitations and chronic joint pain are characteristic,²⁵ seems to be a suitable condition for the application of this physiotherapy technique.

Deep Maneuvers

Deep maneuvers have some difficulty in terms of their justification by scientific evidence, as they are mainly based on the therapist–patient interaction that develops during treatment. It is not easy to complete a neutral treatment; therefore, the subjectivity of the interaction cannot be underestimated when trying to determine the outcome.²⁶

Regarding the justification of deep maneuvers, particularly for the “unwinding” maneuver, Minasny²⁷ offered a theoretical proposal based on the ideomotor model theories by Dorko and Carpenter; neurobiologic type theories proposed by Schleip; and a theory based on the psychology of consciousness proposed by Halligan and Oakley.

From a neurobiologic point of view, the various justifications are oriented toward the existence of a close relationship between the fascial system and the autonomic nervous system.^14,16 The fascial tissue is richly innervated by mechanoreceptors that respond to manual pressure stimuli, the information of which is processed by the central nervous system and the autonomic nervous system (ANS). Indirect stimulation of the ANS in its parasympathetic area favors greater muscle relaxation and overall mental calmness. Gentler fascial maneuvers and cranial techniques favor a parasympathetic response.²⁸ Fascial stimulation also favors a vasodilatory response, as well as changes in the viscosity of the tissue and smooth-muscle fibers in the fascial system. In addition, the stimulus conducted through the nervous system produces a change in the tone of some striated muscle fibers. Although researchers such as Schleip et al.²⁹ refer to certain fascial contractility, the ability to “unwind” has not been demonstrated and its mechanisms are not entirely clear.

Recommendations for Future Research

It is necessary to conduct experimental pilot tests and randomized clinical trials to confirm the safety and efficacy of fascial therapy for treating hemophilic arthropathy. The safety assessment measured by a bleeding record before surgery and during its development, indicates the applicability of this physiotherapy technique without a risk of bleeding. By performing a pre–post-treatment analysis, the effectiveness of this intervention can be quantified, valuing dependent variables such as joint ROM, pain perception, and clinical joint condition (e.g., specifically using the Hemophilia Joint Health Score [HJHS]).³⁰

During the development of pilot testing, an advisable patient inclusion criterion would relate to patients receiving prophylactic replacement therapy with FVIII/FIX. This prophylaxis favors the safety of the intervention and decreases the chances of developing bruises or hemarthrosis.

Use of this technique in patients with hemophilia by a physiotherapist skilled in fascial therapy is imperative. The expertise of the physiotherapist is key when performing the technique correctly, thereby avoiding unsuitable maneuvers that pose a risk of bleeding.

Conclusion

Fascial therapy is a physiotherapy technique that does not require application of untimely or aggressive maneuvers to treat hemophilic arthropathy. As a result of its increasingly widespread use in physical therapy, fascial therapy has become a promising technique for treating joint, muscle, and fascial restrictions that are typical of hemophilic arthropathy.

This technique can be used as a complementary treatment for addressing arthropathy. Clinical studies testing the safety and effectiveness of fascial therapy for treating hemophilic arthropathy should be conducted. It is hoped that this standardized program of fascial therapy for addressing hemophilic arthropathy will facilitate the implementation of this technique in the management of this condition.

Footnotes

Author Disclosure Statement

The authors state that they had no interests that might be perceived as posing a conflict or bias. ■

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