The Long Habilitation Road For Two Children With Congenital Multiple Limb Deficiencies

Felice Celikyol, O.T.R. Ki Ho Kim, M.D. Henry H. Kessler, M.D., Ph.D.

Rehabilitation of children with multiple handicaps is a complex problem. In each case the achievement of a successful outcome necessitates the utmost effort on the part of the entire team of rehabilitation personnel, as well as the child's family. The parents, particularly, play a major role in the successful rehabilitation of these children. Their love and guidance, supported by the necessary discipline, is one of the cornerstones of a successful program.

At the Kessler Institute, our primary aim for preschool children is to help them to develop skills in dressing, feeding, and personal hygiene; and to prepare them for school attendance by teaching them to draw, write, handle books, and read. In addition, some mode of mobilization is developed, either through training in walking with the prostheses or wheelchair management. The aim is to prepare these children to attend regular rather than special schools. With the cooperation of the teachers and the family, even children with severe handicaps can usually adjust to a regular school environment and function successfully.

Two quadruple amputees who presented complicated problems are discussed in this paper.

Case 1

J.J.-P., a nine-year-old Negro male, has been treated at the Kessler Institute for Rehabilitation at two-year intervals since he was five years of age. He is a foundling and lives in a home for handicapped children in Haiti. On his first admission, in May 1963, his limb deficiencies were identified as terminal transverse hemimelias (elbow disarticulations) of both upper extremities; terminal transverse hemimelia (above knee), of the right lower extremity; and an amelia (hip disarticulation) on the left (Fig. 1 and 2 ).

Conventional bilateral upper-extremity prostheses were fitted on May 15, 1963. They incorporated bilateral plastic double-wall sockets, outside elbow locks, friction wrist units, Dorrance 10X hooks, each with one rubber band, and a figure-eight harness (Fig. 1 and 2 ).

The rehabilitation program consisted of the usual drills to develop proficiency in prosthetic operation, including the use of games and toys. J.J.-P. was soon able to operate his prostheses quite skillfully for a five-year-old. However, he did encounter difficulty in prepositioning the terminal device and in bilateral usage. He learned to feed himself with a specially curved utensil, and to brush his teeth after toothpaste was applied to the brush. He could remove his prostheses independently by adducting his shoulder girdle and shaking out of them. In all activities, J.J.-P. demonstrated exceptional balance. He was able to don his prostheses with minimal assistance. He also learned some school activities, such as turning the pages of a book and writing.

For the lower extremities, bilateral stubbies with a pelvic band were provided (Fig. 1 and 2 ). By the time the patient was discharged in October 1963, he was able to balance on them.

Early in the program it was noted that J.J.-P. was a bright child, who grasped instructions rapidly. However, maximal training results were not obtained because of the boy's uneven temperament and occasional outbursts. The success of therapy sessions therefore depended on the child's attitude on a given day. Also significant was the fact that he could accomplish numerous activities without his arm prostheses and therefore did not wholly accept them.

New Prostheses Fitted

At seven years of age, J.J.-P. returned to Kessler Institute for training with a new pair of prostheses , having outgrown the previous pair. The prescription remained the same except that ventilation holes were provided in both arm sockets. An additional half rubber band was added to each hook.

The activities of daily living previously learned were refined. A special pegboard containing hooks was constructed to enable the patient to don his stump socks and prostheses independently.

Velcro tape was used to replace fastenings on his clothing, and was also sewn vertically down the side seams of his short pants, so that he could "drop" them with a minimum of difficulty. He was taught to eat with a swivel spoon and fork, and began to learn typing. The patient continued to have difficulty in prepositioning the terminal devices and in bilateral usage.

Lower-extremity prostheses were provided at this time and consisted of a quadrilateral socket with a modified pelvic band and hip joint on the right; and a hip disarticulation socket with a silesian suspension on the left. Single-axis knee joints with elastic extension aids and single-axis ankles were used bilaterally. The patient was taught a four-point gait, with crutches, and could ambulate an unlimited distance.

J.J.-P. continued to manifest an uneven temper. At times he was extremely stubborn and demanding and would refuse to don both upper- and lower-extremity prostheses.

Current Status

On J.J.-P.'s third admission to the Kessler Institute, from February to May 1967, resections of exostoses of the left humerus and the right femur were performed. A new pair of upper-extremity prostheses was delivered on March 29th. He was now nine years of age and was provided with Dorrance 88X hooks with two rubber bands. It was noted that the upper-extremity stumps had increased considerably in strength.

He was also provided with a new pair of lower-extremity prostheses and specially adapted crutches (Fig. 3 and 4 ).

He was able to don his upper-extremity prostheses if they were slightly elevated on a pillow on his bed. He could lie on the bed and then slide into the sockets. He could slip into the lower-extremity prostheses if they were positioned against the foot of the bed or against a wall.

The patient became independent in picking up a pencil, positioning it, and writing. He learned to type with his two hooks on either a manual or an electric typewriter. He could pick up and position eating utensils unassisted, and needed no adapted utensils. He could cut most foods using both prostheses simultaneously. In addition, he learned to cut paper using adapted scissors with plastisol-covered handles. To brush his teeth, he opened the tube by holding the cap in his mouth and turning the tube with both prostheses. If the tube was full, he could squeeze paste onto the brush and hold the brush in his hook to clean his teeth. He could bring a paper cup of water to his mouth for rinsing. He was also able to feed himself and brush his teeth without his arm prostheses by using a universal cuff (FIg. 5 ) attached to his stump.

J.J.-P. is now able to preposition the terminal devices by using his chin, the other hook, or the underside of a table. He continues to prefer unilateral usage and is right dominant.

The child's uneven temperament persists. His obstinacy is seen as an attempt to assert his independence and to control others by making them yield to his demands. He will respond to very personal attention and affection.

J.J.-P. continues to ambulate using a four-point gait with his revised lower-extremity prostheses and adapted crutches. He can ascend and descend 4-inch steps and ambulate on grass and pavement. He can sit on and stand from a 17-inch stool and can go up and down a 2-inch curb.

Plans for the Future

Projected plans for future admissions include (1) providing functional hands, since he will be more concerned with cosmesis as he grows older; (2) developing independence in toilet activities; and (3) continuing emphasis on the refinement of performance with all four prostheses.

J.J.-P. is a bright boy and can be quite pleasant and gay (Fig. 6 Fig. 7 ), but most of the time he is stubborn and uncooperative. He has often been seen sitting by himself on the floor in a very pensive mood, although the nuns and staff of the orphanage are warm, kind, and good to him. Apparently, he misses the special love and discipline that can only come from parents. The success of his rehabilitation program will depend on the refinement and development of his personality.

Case 2

M.O., a white female, is now five years of age. She was first seen at our Congenital Amputee Clinic in November 1962 at the age of six months and has been followed since then. She is the oldest of three children. Her anomalies were identified as right amelia and left terminal transverse hemimelia (above elbow) of the upper extremities; and bilateral proximal femoral focal deficiencies with associated complete paraxial fibular hemimelias (Fig. 8 ).

In April 1963, at the age of 11 months, M.O. received a pair of stubbies. Six months later "kyacks" were attached to allow her to rock and balance. M.O. adjusted well to the rocker mechanism and could balance herself for long periods.

In January 1964, at the age of one and a half years, M.O. received her first arm prosthesis. This consisted of a left above-elbow socket, an outside elbow lock, a friction wrist unit, and a modified Northwestern ring harness. In place of an axilla loop, posterior and anterior stabilizing straps were used, with attachments to her right leg prosthesis.

Pneumatic Prosthesis Fitted

In June 1965, M.O. was selected as a pilot wearer for the American Institute for Prosthetic Research (AIPR) pneumatic arm. The prosthesis was a shoulder-disarticulation type for the right amelia, with a passive friction lock universal shoulder joint, a power lock turntable, and a pneumatic hook with a "feeder" * component with cable control attachment to the left lower-extremity prosthesis (Fig. 9 and 10 ). Elbow flexion and extension were produced by applying pressure on buttons located anteriorly and posteriorly inside the shoulder cap. The terminal device was body powered and controlled by means of lateral trunk flexion, the control cable being attached to the left leg prosthesis. The "feeder" mechanism (at the wrist unit) could be locked in or out by sliding the medial elbow button up or down. Thus the coupled wrist action could be used or not as desired.

*Wrist flexion coupled to elbow flexion so that a spoon held in the terminal device remains level and turns into the mouth for feeding.

The training program consisted of play activities appropriate for the child's age, with emphasis placed on feeding activities. M.O. did not completely accept the prosthesis for many activities, as she demonstrated adept prehensile skills with her toes. She did, however, accept it for feeding.

M.O. was readmitted in September 1965 for continued training. The control cable for the terminal device previously attached to the left leg was now connected to a left axilla loop, which proved more satisfactory. The upper-extremity prosthesis was now completely separated from the lower-leg prostheses, and the CO2 tank was attached to a waist strap.

New leg prostheses were fitted, conforming to her own legs and containing a support inside the sockets for her feet. Each socket contained a flap which allowed her toes to protrude slightly (Fig. 9 and 10 ). There were no articulating components for her knees, and the feet were modified SACH.

Problems encountered by M.O. were that she was unable to preposition the shoulder joint and hook unassisted; was unable to sit unassisted; and could not safely walk alone unless the floor was smooth and even.

M.O. is a personable and charming child who is obviously brighter than normal for her years. Her parents have been most cooperative in the treatment program. We have observed that she is treated with warmth and respect but without overindulgence .

Future Plans

Plans are presently under way to provide M.O. with bilateral upper-extremity conventional prostheses, as she is no longer eligible for the pneumatic arm study. Her prostheses will consist of a right shoulder disarticulation prosthesis with a passive abduction unit, a friction elbow, a friction wrist unit, and a voluntary-opening Dorrance hook; plus a left above-elbow prosthesis with internal elbow lock, wrist flexion unit, voluntary-opening Dorrance hook, and bilateral harness attachment.

M.O. is approaching school age and has been enrolled in a regular kindergarten for September 1967. Her ambulation is precarious, and a battery-operated wheelchair has been provided. The direction control box has been relocated so that M.O. can control the chair with her foot, thereby freeing her arms for school activities (Fig. 11 ).

As soon as the arm prostheses are received, M.O. will be trained in their use, with emphasis on school activities.

M.O.'s parents are very understanding and cooperative, and provide her with adequate love and discipline, which is reflected in her charming personality and willingness to improve herself. Although she has multiple handicaps, her future seems to be brighter than her physical disabilities would presuppose.

Felice Celikyol, O.T.R., Ki Ho Kim, M.D. and Henry H. Kessler, M.D., Ph.D. are associated with the Amputee Clinic Kessler Institute for Rehabilitation West Orange, New Jersey