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Orthoses in the Treatment of Amyotrophic Lateral Sclerosis
Gene Bernardoni, CO President and Owner, Ballert Orthopedic of Chicago

Patients suffering from ALS often experience distal weakness in their arms and legs, and in the muscles of the neck. Additionally, generalized muscle weakness is common.  The ALS patient can benefit from a variety of symptomatic and supportive treatment modalities, including the use of orthotic braces.

In designing orthotic intervention it is important to understand the course of the disease and some of the common complaints and weaknesses that the patient may experience. Fig. 1. As the tibia becomes more vertical, active ankle dorsiflexion is required to avoid toe drag. One of the most common problems is lower leg weakness. Often the patient presents with a loss of the ability to dorsiflex his or her foot. Dorsiflexion is the ability to flex the ankle, raising the forefoot in order to clear the ground when that leg is swinging forward. (See Fig. 1.)

Fig. 1. As the tibia becomes more vertical, active ankle dorsiflexion is required to avoid toe drag.

Because human beings are normally very efficient walkers, the forefoot is not raised high in order to clear the ground. Consequently, even the slightest weakness can result in catching the toe and tripping on uneven ground or a misgauged curb. Since one typically flexes the leg at three places (the hip, the knee and the ankle), the ALS patient can sometimes compensate for dorsiflexion weakness by exaggerated flexing at the knee and the hip. Such high stepping, however, is inefficient and fatiguing.

It is usually better to treat the dorsiflexion problem and maintain a more efficient gait for the patient. A brace called an Ankle Foot Orthosis (AFO) is usually all that is necessary to enable the patient to manage this problem. An AFO is a plastic orthotic brace that fits inside the shoe and extends up the back of the leg to just below the knee, where a strap wraps around the lower leg to hold the brace in place. AFO’s can be customized to provide additional support for the knee if there is weakness in the quadiceps; however, because the weakness is usually only below the knee, I will focus on AFO’s that are used to correct for ankle dorsiflexion weakness.

Fig. 2. AFO, modified posterior leaf spring

Although there are a number of different kinds of AFO’s, the two most common types in this application are a Posterior Leaf Spring AFO (PLSAFO) (Fig. 2) and an Articulated AFO (AAFO).

The PLSAFO takes advantage of the fact that plastic tends to return to its original shape after being deformed (energy storing) by person’s weight. By carefully trimming the width of the plastic strip directly behind the patient’s ankle, we can customize the AFO to the weight and strength of the patient (see above, Fig. 2). This AFO works as follows: When the patient steps on his foot the part behind the heel will bend, allowing the foot to become flat on the ground as it absorbs the patient’s weight and allows the other leg to swing through. When the patient steps down on the other foot and takes his weight off the foot with the AFO, the AFO returns to its original shape and picks up the toe to allow the patient to swing through without catching the toe and tripping.

	The Articulated AFO (AAFO) is hinged at the ankle with any one of a number of different kinds of joints (Fig. 3). Typically we use a joint that is contoured to provide the same dorsiflexion assist as described above. The AAFO will allow free dorsiflexion (elevating the forefoot), and if the contoured joint is used it will assist dorsiflexion with a spring-like action (see joint in Fig. 3).
Fig. 3. Articulated AFO

The part of the brace behind the heel provides a stop to prevent the forefoot from dropping when that leg is lifted. The jointed AAFO has the advantage that the joint of the brace is more closely aligned with the joint of the ankle, thereby preventing a pistoning effect as the ankle bends. This means that the heel can move up and down within the brace as the patient walks with the PLSAFO described above. The AAFO can also provide better side-to-side support. The disadvantage of the AAFO is that it is more bulky and is more difficult to conceal (for those patients who wish to conceal the fact that they are wearing a brace). Furthermore, the plastic at the anklebones sometimes causes discomfort.

A custom-made AFO is made in the following way.
At the patient’s first visit the orthotist will take measurements and take a plaster cast of the leg to use as a model from which to fabricate the AFO. This plaster cast is cut off and brought to the laboratory where it is filled with a plaster slurry into which a pipe is inserted. When the plaster slurry is hard the plaster cast that was taken of the leg is removed and discarded. What remains is an exact model of the lower leg, foot and ankle with a pipe sticking out the top like a sewing manikin of the leg.

The pipe serves two purposes. First, it is a handle to be inserted into a vise to allow the orthotist to make corrections to the mold of the foot and leg by using such tools as a sure form file and drawknife. The model is then smoothed and moved to a different vise in the oven room. A vacuum hose is attached to the pipe. If a joint is to be added it will be nailed to the plaster cast at this time. A piece of plastic of the appropriate size and thickness is heated in the oven set to 350 degrees until it becomes moldable. The plastic is picked up by technicians using special gloves and draped over the mold of the leg. The vacuum is turned on and the edges of the hot plastic are pushed together and sealed. The vacuum then causes the plastic to draw in and conform exactly to the mold of the leg.

When the plastic has cooled it is cut off the mold at the trimlines ordered by the orthotist. The edges are sanded and buffed and a buckle and strap are added. If a joint is to be included it is screwed into place. At the next appointment with the orthotist, the AFO is fitted and adjusted to the specific needs of the patient. One or more further appointments with the orthotist may be necessary to fine-tune the AFO so as to accommodate the patient’s daily activities.

Another common complaint of those affected with some neuromuscular diseases including ALS is cervical weakness. As the neck muscles fatigue, it becomes difficult to hold one’s head up without using one’s hands for support. I have tried many variations of cervical collars and cervical orthoses (braces) to aid my patients with this complaint. The standard cervical orthoses are designed to limit or stop the motions in the neck because they produce pain or could produce further injury. Since patients with muscle weakness alone are not being treated for either of these conditions, the standard cervical orthoses are usually lacking in one area or another. The Soft Cervical Collar made of foam covered with cloth is the least restrictive and seems to work well in allowing motion while providing a degree of support. One drawback of these collars is that they are hot and bulky. Another is that they do not provide enough support with more severe weakness.

The standard firm collars such as the Aspen Collar and the Miami J Collar do provide the support needed but they too are hot and restrict motion too much. Some “frame” type collars have been developed such as the Headmaster which provide good support and allow air circulation around the neck but they too can be too restrictive for the patient. The three most common complaints heard about the cervical orthoses worn by ALS patients is that they are too hot, not supportive enough and overly restrictive of movement.

I was challenged by a neurologist to find or invent a design that would better address the needs of patients with ALS. The cervical orthosis we designed based on our observations of many collars in use was a frame type cervical orthosis. It consists of a wire frame covered with foam for comfort and a spring action mandibular piece (chin). It has a leather adjustable strap which fits behind the neck (Fig. 4).

Fig. 4. Wire Frame Cervical Orthosis

The purpose of this Wire Frame Cervical Orthosis is simply to help to balance the patient’s head on his shoulders and prevent the neck from flexing (chin going down on chest). It is only worn for certain tasks such as reading or watching television or for other times when patients become fatigued. The results have been good for most patients.

Our goal in providing orthotic devices to our patients is to improve function without getting in the way of the activities of daily living. Finding and fitting the appropriate orthoses to our patients is an on-going learning experience. We get our best ideas from the feed-back given to us by our patients.

Profile: Gene P. Bernardoni, RPh., C.O.
Gene P. Bernardoni is the owner and President of Ballert Orthopedic of Chicago. A 1962 graduate of the University Illinois College of Pharmacy with a BS degree in pharmacy, Bernardoni owned and operated pharmacies in Chicago and its suburbs for over two decades.

Bernardoni’s interest in orthotics dates from the early 1980’s.  After completing the NARD Orthotic fitter’s examination (1983) and becoming a NARD Master Certified Orthotist (1987), Bernardoni purchased Ballert Orthopedic in October 1986.

Bernardoni completed the orthotics program at Northwestern University in 1989, and, after a year of internship, was certified by the American Board for Certification. In 1991, Bernardoni was certified by the Board for Orthotic Certification of the University of Maryland.

Bernardoni has completed more than 20 courses in continuing orthotic education. He has traveled throughout the United States to further his knowledge of specialized regional orthotic techniques. In 1998, Bernardoni was invited to participate in the annual conference of the Japan Association of Prosthetics and Orthotics held in Ehime, Japan.

Bernardoni is licensed to practice orthotics and pedorthics by the State of Illinois. He has privileges at most Chicago area and suburban hospitals, and is the exclusive consulting orthotist at Northwestern Memorial Hospital, where he is also on the faculty of their School of Orthotics and Prosthetics. For seven years, he was the staff orthotist at Cook County Hospital in Chicago.

Bernardoni has lectured and written on the subjects of spinal orthotics and cranial molding helmets. With regard to the latter, in 2000 Bernardoni sought and won clearance from the FDA to manufacture molding helmets. Today, under Bernardoni’s leadership, Ballert is in the forefront of orthotics firms nationwide practicing this specialized discipline, manufacturing not only for direct referrals, but also for other O&P firms in the United States.