Problem Sqlving For The Multiply Handicapped Child Two Interesting Case Studies

William E. Gazeley M. D. Robert S. Hoffman, M. D. Mildred C. Ey, O.T.R. William Sampson, C.P.


Most of the children served by our clinic need prosthetic replacement for only one extremity. While these unilateral fittings sometimes tax the ingenuity of our prosthetist they are relatively straightforward compared to the treatment of the multiply handicapped child, particularly when the involvements are severe.

Our experience in working with two children, each wearing three prostheses, has been both interesting and challenging because of the multiplicity and severity of their involvement. They are of approximately the same age and both have been followed in our clinic for nearly three years. They are reported on herein.

We hope that our presentation of these cases will help others who may encounter similar cases for the first time. We will also appreciate comments and/or suggestions regarding the future handling of these patients from persons who have had prior experience with comparable situations.

Case 1

Initial Observations

A. L., a female, was born December 13, 1961. She was first seen in our clinic on January 9, 1963 at the age of 13 months. Her skeletal limb deficiencies were classified by the Frantz-O'Rahilly system as:

1. Upper Extremities:
a. Right - Partial aphalangia with absence of rays 2, 3, and 4, and reduplication of rays 1 and 5. She also presented a non-articulated elbow, the radius and ulna being continuous with the humerus.
b. Left - Visually and prosthetic-ally this is a complete amelia,although X-ray reveals a short humeral stump imbedded in the flesh.
2. Lower Extremities:
Basically, these are both proximal femoral focal deficiencies with accompanying absence of the fibulae, the distal rows of metatarsals, and the 5th rays of the phalanges. Femoral elements are evident bilaterally, with slightly more present on the right side than on the left.

The child has no other known anomalies.

First Fitting

When first seen the child had a-chieved good sitting balance but was not yet attempting to stand. It was decided to fit her with a passive upper extremity prosthesis so that she would become accustomed to having something on the deficient side. Because of her age and the severity of the deficiency, it was believed that a functional prosthesis would be of little benefit to her at that time.

A. L. received her prosthesis on April 10, 1963 when she was one and a half years of age. It consisted of a shoulder disarticulation "banana"-type of prosthesis with anterior and posterior wings. It had been fabricated in slight forward flexion at the shoulder, with the elbow flexed at 45°. The terminal device was a passive mitt. The shoulder harness consisted of a chest strap with an ancillary elastic shoulder strap.

The training period consisted of three sessions, during which the mother was shown how to help A. L. use her prosthesis to assist in gross bilateral activities and as a balancing aid. By the end of the training sessions, A. L. was wearing her prosthesis during all walking hours, using it for the purposes intended.

Follow-Up Visits And Lower Extremity Fitting

A. L. was next seen in our clinic on May 22, 1963. After evaluation of her progress, the decision was made to attempt to fit her with bilateral lower extremity prostheses. A careful investigation was made to determine how this should be done, as this was our first experience with this type of problem.

At this time A. L. was also referred to a hand specialist, Dr. William Littler of New York City, concerning her right hand. He recommended that any attempt at surgery be delayed for a few years.

The lower extremity prostheses were completed and checked out on November 27, 1963. These were pylon-type limbs with SACH feet and funnel shaped sockets for gluteal weight bearing. They had anterior windows covered with molded leather pads, which were fastened in place with Velcro after the prostheses were on. A. L. had exceedingly good balance and almost immediately was able to stand independently for at least one minute.

Early Problems

At the next clinic visit, on April 22, 1964, it was found that Mrs. L. had been experiencing difficulty in getting her daughter to wear the prostheses. A. L. was using her upper extremity prosthesis to help her crawl around the floor, but she was apparently not interested in standing. A special discussion was held with the mother and the importance of having the child become accustomed to her prostheses was stressed. It was suggested that Mrs. L. place A. L. at a "standing table" with some favorite toys for at least 15 minutes twice daily. When next seen, she was wearing her prostheses and standing two hours daily. The upper extremity prosthesis was used to assist in balancing when she was playing at her standing table.

A Functional Arm Provided

A prescription for a functional upper extremity prosthesis was written, but fabrication was delayed because the parents were reluctant to accept it. However, on September 9, 1964 the parents were ready to accept the functional upper extremity prosthesis. It was completed and approved on October 21, 1964 at which time A. L. was 2 years and 10 months of age. This prosthesis consisted of a shoulder disarticulation socket and harness, friction elbow, laminated forearm, friction wrist, 12P hook, and single control cable. (The "friction elbow" is the E-50F Hosmer child friction elbow. Because this is manually positioned, there is no need for elbow lock or flexion control; the "single control cable" serves only to operate the terminal device.) Mrs. L. was instructed in procedures for helping her daughter use the upper extremity prosthesis actively.

During this clinic visit it was noted that A. L. was doing considerable walking without her lower extremity prostheses. Although her gait was unstable, her balance was excellent. She had also shown improvement in her ability to walk with her prostheses, needing only to hold onto someone's hand.

By January 20, 1965, A. L. had adapted well to her new upper extremity prosthesis and wore it during all waking hours. She still did not use the terminal device actively, but limited its use to gross motions.

Growth Problems

When seen in January 1965 the patient had not been wearing her lower extremity prostheses. They had become tight and difficult to manage in view of the mother's pregnancy. It was decided to have the sockets enlarged, and the mother was urged to have A. L. wear the prostheses daily. The importance of encouraging their use was again stressed.

The child was next seen on April 21, 1965. The sockets had been enlarged and A. L. was again wearing the lower extremity prostheses for short periods each day. However, her growth indicated the need for a longer pair of legs. These were completed and seen for checkout on August 18, 1965. They were ischial bearing and very satisfactory (Fig. 1 and Fig. 2 ). Triple wall sockets and one and a half inches of elongation were incorporated to allow for circumferential and longitudinal growth. Further training to help the child become accustomed to her new prostheses was discussed with her mother. Mrs. L. preferred to attempt to work with her daughter at home and bring her in for training only if this method failed.

Social Problems

A. L. is a child of average intelligence or better. Because of her disability, her two older brothers cater to her. Therefore, she does not feel the need to be independent. She is a demanding child who controls both her family and the neighborhood children.

Clinic personnel have kept close contact with the family between visits. The mother has always been most cooperative and willing to seek help whenever needed. Mr. L. has never been to the clinic. He is frequently out-of-town, but he feels that his wife should be able to train A. L. at home without help from the clinic. However, Mrs. L. now believes that she must bring A. L. in for training sessions. She was supposed to bring the child to the clinic on November 11, 1965, but was unable to do so because of illness. A. L. was seen on December 1, 1965 and a training schedule established.

It is hoped that A. L. will cooperate during her training sessions. She will soon be old enough to start school, and it is important that she learn to be as independent as possible. Also, because of growth, it is becoming increasing difficult for her to reach her mouth with her right hand. It is, therefore, going to be necessary for her to learn the use of assistive devices and her upper extremity prosthesis.

The clinic felt that a training program should have been instituted for both upper and lower extremity prostheses when they were first fitted, but it was not until recent months that the mother was able to accept this requirement.

Future Plans

A training program will be conducted during the next few months. We will then continue to see A. L. in the clinic at three to four month intervals.

Case II

Initial Observations

B. S., a male, was born on January 8, 1962 and was first seen in our clinic on November 7, 1962 at approximately 10 months of age. According to the Frantz-O'Rahilly system his skeletal limb deficiencies were classified as:

1. Upper Extremities:
a. Right - Terminal longitudinal partial aphalangia, ray 2.
b. Left - Terminal transverse partial hemimelia, the below elbow portion being extremely short.
2. Lower Extremities:
Both are terminal transverse partial hemimelias.

In addition to the skeletal limb deficiencies it was noted that B. S. had partial facial paralysis.

First Fitting

B. S. received his first prostheses on May 21, 1963 at 16 months of age and was admitted to Sunnyview Rehabilitation Center for in-patient training. Because the below elbow portion of his left arm was so short, the initial fitting consisted of a triple wall laminated above-elbow "banana"-type prosthesis with the elbow preflexed to 45 degrees. He was fitted with a Northwestern University ring harness, and a passive mitt. His lower extremity prostheses were of the patellar tendon bearing type; but knee hinges, thigh cuffs, and SACH feet were used.

The training period lasted until June 28, 1963. During this time B. S. was seen four times daily in the Physical and Occupational Therapy Departments. He adjusted well to all three prostheses and could wear them without irritation during all waking hours. He was able to use his upper extremity prosthesis in performing gross bilateral activities. If supported from behind, he was able to take a few steps on his lower extremity prostheses. He could creep independently using all three prostheses and could roll over from a prone position. Prior to discharge, his parents were instructed in the care of the prostheses and in working with B. S. so that he would continue to improve.

Follow-Up Visits

B. S. was next seen on July 24, 1963. He showed some progress in standing balance and in walking with assistance. Further progress was evident when the child was seen on September 18, 1963. Because of the multiplicity of the involvement, it was decided not to give the child an active terminal device for the upper extremity prosthesis until he had become more proficient in walking.

By the time B. S. was again seen on January 8, 1964, he had developed an independent gait but walked with knees and hips in marked flexion. It was felt that he would soon be ready for an active upper extremity prosthesis, and this was prescribed.

An Active Arm Provided

B. S. was next seen on April 22, 1964. His gait showed some improvement, although he still walked with flexed knees and hips. State Aid approval for a new upper extremity prosthesis was finally received, and the prosthesis was completed on July 13, 1964. This prosthesis consisted of a triple wall laminated socket with an outside elbow lock; laminated forearm; constant friction wrist unit; 10P hook; manual control cable for elbow lock; and a figure "8" harness with dual control cable system for elbow flexion and hook operation. This elbow-disarticulation type prosthesis was decided upon at this time because the below-elbow stump was so extremely short that it disappeared completely at 90 degrees of elbow flexion. Due to the child's age, a stump activated elbow lock did not seem feasible.

The training program consisted of four outpatient sessions, during which the mother was present so that she could continue to work with B. S. at home. At the termination of the program on August 26, 1964, B. S. was able to operate his terminal device fairly well when he wanted to, either actively, or by using the right hand.

Growth Problems

At the next clinic visit on September 30, 1964, the patient's gait pattern showed some improvement, and he was making more assistive use of his upper extremity prosthesis. However, B. S. had outgrown the lower extremity prostheses and his stumps were being irritated. New prostheses were ordered and were completed. During checkout on December 1, 1965, the child's walking posture showed immediate improvement. He adapted to his new legs quickly and demonstrated his ability to walk, run, and to get up from the floor independently. He was also using his upper extremity prosthesis more actively.

When next seen on April 21, 1965, B. S. was outgrowing his upper extremity prosthesis. His mother requested that fabrication of a prosthesis be delayed as long as possible, because B. S. was growing so rapidly. Therefore, a new harness was supplied and the hook recovered, so that the prosthesis could be used a while longer.

Muenster-Type Arm Fitted

During the succeeding months, the prosthetist had an opportunity to take the course on the fabrication of the Muen-ster-type below-elbow prostheses at New York University, and it was decided that an attempt should be made to fit B. S. with a modified Muenster below-elbow socket. In fitting the patient with this type of socket, it was necessary to take the cast with the elbow flexed at 45 degrees only, since the stump disappeared and could not be fitted at 90 degrees. A socket which allowed about 30 degrees of motion in the elbow was successfully made. To get satisfactory suspension, it was necessary to utilize a triceps cuff and Northwestern University ring type harness. The prosthesis was completed with a 10X hook and a single control cable, and checked out at the clinic on November 17, 1965 (Fig. 3 and Fig. 4 ). B. S. has readily adapted to his new prosthesis and is already using his natural elbow motion. Mrs. S. is very pleased with the new arm because of its simplicity of application compared to the child's previously worn above-elbow type prosthesis.

Future Plans

The clinic will continue to see B. S. at three to four month intervals throughout his growth period. It is not anticipated that additional training will be necessary.

Summary

Two children, both with congenital involvement of all four extremities, have presented an interesting challenge to our clinic. One has been treated as a "normal" child by his family and has learned to use all three prostheses well. When he first started walking, and frequently during the succeeding two years, we questioned whether or not we should do something to correct his marked hip and knee flexion. The possibility of a knee lock for one leg was considered. There were no contractures evident in hips or knees. We did note some slight progress each time he was seen. At the last clinic visit he was walking erect.

The other child has been "catered to" by her family and has persuaded others to do things for her; therefore, it has not been necessary for her to become independent. She will soon be of school age, and independence is necessary if she is to be in a class with "normal" children. It is hoped that this goal will be achieved through the proposed training program.

William E. Gazeley, M. D. Robert S. Hoffman, M. D. Mildred C. Ey, O.T.R. and William Sampson, C.P. are associated with Child Amputee Clinic Sunnyview Rehabilitation Center Schenectady, New York

William Sampson, C.P. is associated with the LaTorre Orthopedic Laboratory, Schenectady, New York