Providing Armless Babies With Active Prostheses In The Second Year Of Life
This article is dedicated to Henry H. Kessler, M.D., Ph.D., Director of the Kessler Institute for Rehabilitation, West Orange, New Jersey, in appreciation of his work for armless children.
(The following article by Dr. Ernst Marquardt, of the University of Heidelberg's Orthopedic Clinic, appeared in a booklet, Information on Measures for the Rehabilitation of Children with Dysmelia, published by the German Association for Rehabilitation at the University of Heidelberg. It is reprinted by permission of Dr. Marquardt.)
Stephanie S. was born on October 17, 1959 near Heidelberg, Germany, with a complete amelia of the left upper extremity and a phocomelia on the right side, with a small finger-hand attached to the shoulder (Fig. 1 ).
The parents contacted us shortly after birth. Our plan was to fit the child, after she was six months old, with passively adjustable prostheses (American baby mitts), so she could become accustomed to artificial arms at a very early age; and also that she might be able to include them into her "body image". We also thought of using rollers fixed to the artificial elbow joints to help her to crawl.
In Stephanie's second year of life, we planned the first active prosthesis function, namely, the coupled internal rotation of the upper arms. This construction would enable the child to hold dolls, toys, balls, etc. between its baby mitts. The uniform movement of both hands, as in the game "pat-a-cake", gives children a great deal of pleasure. It is an adequate grasping function and one of the simplest for babies. To my knowledge, it has never been utilized before in the construction of a prosthesis. A wrist joint adapted to this grasping procedure was planned from the beginning.
Stephanie was treated in the outpatient clinic of a children's hospital for several months because of a continued high temperature of unknown origin. Orthopaedic measures could not be initiated, therefore, until she was one year old. On November 21, 1960, Stephanie was brought to our clinic for the first time. From her medical record we learned that her parents and a four-year-old sister were healthy; and that there was no history of deformities in her family.
During the first weeks of pregnancy the mother had had no virus infections nor had any X-rays been taken. Subsequently, however, she reported that before conception and during the first months of pregnancy she had regularly taken the drug thalidomide. The following is a summary of the medical findings concerning Stephanie :
Good general physical condition; amelia of the left upper extremity; a loose, walnut-shaped rudimentary hand emanating from the right shoulder, ending in a little finger without a nail; between shoulder and hand-finger a deep amniotic strangulation into which the hand was drawn even more deeply during adduction; shoulder blades well developed and able to move vigorously; spinal column and pelvis normal; feet used for eating and playing, though the child did not yet stand or walk.
Our first step was to remove the amniotic strangulation by plastic surgery. This was followed by systematic training in grasping and touching with the hand-finger rudiment. At first Stephanie did not seem aware of this digit, which until then had been hidden under her clothes and had not been observed or used for any activity. The clothes were changed in such a way that the finger remained uncovered and could contact its surroundings by touching and grasping. Stephanie could not perceive why she should use this finger for grasping, since she could hold things and play much better with her feet. Also, she could observe the activities of these limbs.
By mid-January 1961 (age one year, three months), she could hold small or light objects, such as pencils, spatulas, or little play animals between finger and chest, by adduction. It was extremely interesting to observe Stephanie at this time. She finally became conscious of the grasping procedure and took increased advantage of its possibilities. I was delighted to note that, after three weeks training, she tried to use the finger to pick up dropped objects by bending her chest sideways.
It became apparent at the training sessions that the sense of touch in Stephanie's finger-hand was developing steadily. At first objects were held only by adduction, but she was soon able to embrace them if their shape permitted this. Palpating, clutching, and grasping became more differentiated ([popoup2]).
At the end of 1961, we made a cast for a plastic shoulder cap and a dual pressure valve that was easily operable. This was not used initially for control of the prosthesis, but to move a jumping jack pneumatically. Our aim was to give Stephanie an additional device that could be used between treatment sessions for training the finger in grasping against resistances. We also wished to change the training process in order to find out how much time she would need to become aware of the relationship between finger movement, valve operation, and movement of the jumping jack. How soon would an unconscious urge to move the finger (resulting from play and other experiences) be transformed into conscious valve control?
The little boys in our children's department showed immediate pleasure in using the jumping jack, but Stephanie found the toy more weird than interesting. This feeling was intensified because a cold draft escaped from the valve at every movement due to faulty valve adaptation. The boys also had the advantage from the beginning of being able to watch the operating mechanism of the limb and the jumping jack simultaneously and could thus perceive much more quickly how it functioned. Stephanie, however, could watch either the digit and valve, or the jumping jack, but not both at the same time.
On February 10, 1961, Stephanie was temporarily discharged from the hospital. Highly favorable home conditions guaranteed a conscientious continuation of training.
Fitting of Shoulder Cap
The first fitting of the shoulder cap resulted in a minor catastrophe. She opposed us completely and kicked and cried in frustration. In order that she might become accustomed to the change gradually, we used only soft sockets at first, held together by soft bandages, but withheld the prostheses. At home the caps were put on every morning when Stephanie was being dressed, and were removed at night before she was bathed. This routine was adhered to rigorously and she soon accepted the caps as though they were part of her clothing.
Clinical treatment was continued during the summer of 1961, at which time our workshops made various alterations in the pneumatic rotation joint (its weight had proven too great). The problem was eventually solved in the simplest manner by transmitting the movements from a cylinder containing a piston through two Bowden cables to the artificial arms, essentially effecting a coupled internal rotation (Fig. 3A and Fig. 3B ). At the appropriate valve operation, springe on each side cause a uniform external rotation with the elbows passively flexed. Objects could now be grasped and held. This was possible, however, only when hand palms and both wrist joints operated against elastic resistance to dorsi-flexion.
Since the entire construction was experimental in character, I had simple spiral springs built in on both sides, as provisional wrist connections. These always returned to their starting points, and allowed the baby mitts to adapt to the object being grasped. There were some undesirable side effects, since the springs would yield in all directions, but this appeared to be the best solution to the problem (Fig. 4 ).
After the prosthesis was completed on July 20, 1961 (age one year, nine months), we were able to initiate training of this highly intelligent little girl. The change from home instruction to the clinic again evoked resistance, which became quite violent at times. Just to wear the prostheses seemed to agitate the child greatly.
Work of Therapist
Before training with the artificial limb could commence, it was necessary for the occupational therapist to win Stephanie' s affection and confidence. There is a widespread concept these days that as much work as possible must be done in the shortest possible time, so that the efficiency of one's work will impress the agency underwriting the project. However, careful and considerate treatment, which would create confidence in the patient, would often be more appropriate and in accordance with the better judgment of the worker.
In our special set of circumstances, the therapist recognized that it would be psychologically unwise to initiate treatment at once, and revised the procedures on her own initiative. Her program of play and informal activities involved the expenditure of far more time and patience than is customary in the training process. Her method was the correct one in this situation because the success of the entire undertaking depended upon winning Stephanie's affection and confidence before formal training was initiated. Basic also was the fact that, though the therapist had to gain the child's trust, at the same time she had to be recognized as the person in authority.
Limiting instruction to one or two daily therapeutic sessions proved to be insufficient, no matter how diligently these were conducted. It became necessary for the occupational therapist to participate completely in the child's daily activities, including eating, playing, singing, telling stories, going for walks, toileting, dressing, etc.; she almost became a "mother substitute". These routines sometimes overlapped those supervised by the nurse, but they were the ones performed at home by the mother.
The regularity of routines, with all activities conducted by the adult in a spirit of determination, calmness, and assurance, was of equal importance. Theoretically, this sounds simple, but in practice difficulties arose because of Stephanie's rebelliousness. At the start it was almost impossible to get any work done with her for she nearly shouted her head off. She continually wanted to go away, so the therapist took her for walks to the fountain containing goldfish at the aquarium, and to many other places which the child found interesting. Fairy tales and other stories were told to her. Much of the time Stephanie and the therapist just played together. Gradually, after a fortnight of such procedures, the confidence of this very obstinate little girl was gained.
Use of Arm Prostheses
Since Stephanie's feet were covered by shoes on these trips, the opportunity was taken to introduce the use of the arm prostheses during play, although the therapist had to operate the valve herself for almost a month. The little digit lying above the touching lever of the valve was manipulated to teach the movement necessary for control. Subsequently, with help, Stephanie learned to hold a shovel, small hammers, and other toys and to use her chest to maneuver them into position for grasp.
At first, she found the prostheses very disagreeable. They were removed after play and before bedtime, and she soon learned that when the devices were taken off, play time was over. After two and a half weeks of this procedure, she cried when it was time to remove the limbs. However, she gradually achieved a sense of the prostheses and their possibilities, even though she was not yet able to operate their active functions. Nor could she manage the flexion-extension of the elbow joints or the forward extension of the shoulder joints.
We noted with interest that, during the period between two and a half and four weeks after training began, Stephanie constantly attempted to manage the grasping procedure herself. When these efforts failed, she would cry with rage and deep frustration. Then the occupational therapist had to operate the touching lever of the valve. Often the therapist simply pushed the toy between Stephanie's mitts, as, for example, the wooden hammer to play the xylophone, the pencil for painting (Fig. 5A and Fig. 5B ), or the shovel for sand play. The chest movements necessary to obtain different sounds on the xylophone, to move the pencil from the paper into a glass of water, or to lift the shovel from the sand pile to a car required practically no training.
At this stage of development, the pleasure derived from walking helped greatly to encourage Stephanie to use the mitts. In addition, the child now began to realize that she had hands, too. Once I observed her looking at the mitts very carefully and, after comparing them thoroughly with the hands of a normal child, laugh happily. Although at this time she was still unable to control the movements of the artificial arms actively, she would ask for the mitts early every morning. I then realized for the first time that the additional hours we had devoted to her, despite our busy schedule, had not been in vain.
A devoted friendship developed between Stephanie and a five-year-old armless girl who had recently been fitted and was already able to operate her prostheses skillfully. This helped Stephanie to become accustomed to her own devices. Using her own artificial limbs as an example, the older girl demonstrated the use of the finger-hand to obtain grasp and encouraged Stephanie to imitate her. This sharing of experiences was, I am sure, of inestimable value in the child's recognition of the processes involved.
By August 18, 1961 (age one year 10 months), Stephanie was able, for the first time, to turn the arms inward and outward of her own volition. She could now grasp large objects, such as dolls, teddy bears and big balls; hold them and release them. This success was not accidental. Smaller objects, such as pencils, hammers, shovels, and the biscuits she liked so much, still were handled by the therapist. Stephanie was also able to push her doll's pram through the corridors and garden-an activity she loved (Fig. 6A , Fig. 6B , and Fig. 6C ). The therapist or the nurse accompanied her on these excursions, since walking was not quite safe as yet. When holding or shaking hands, she could rotate the arms externally and hold out the right hand.
On August 25, 1961, we sent Stephanie home. Her mother had learned our methods of training during this time and had received some instruction in the use and care of the prostheses. It was necessary that she know which activities could be performed with the support of the devices and which could not, and when special help was required to avoid disruption of the training process.
Stephanie, in the meantime, had learned to do many new things with the devices (Fig. 7A , Fig. 7B , Fig. 7C , Fig. 8A , Fig. 8B , Fig. 8C , Fig. 9 ). She even knew when to tell her parents to refill the carbon dioxide container. On my visit to the home at the end of January 1962, the widely-opened arms suddenly ceased to function during play. Instead of becoming angry or anxious, Stephanie ran to her mother and said, "Go to the cellar and refill". Since walking was basically unsafe, and it was impossible to cushion a fall by using the hands, her parents made a ring of soft rubber which was useful in this situation.
Our future plans involve the fitting of Stephanie with an active pneumatic hand on the right side to permit direct grasping and holding. Internal rotation will be actuated by a scapula abduction, external rotation by a backward movement of the shoulders. This procedure is well-based psychologically. Initially, the rudimentary hand-finger would operate the grasping function of the pneumatic right hand (or of the pneumatically-powered hook) by a dual pressure valve connected with the touching lever This lever would later control grasping of the left prosthetic hand by a new pressure valve. By spatial division, the right and left hands would be controlled separately, thus providing greater security than if the movements of both hands derived from a four-way pressure valve. This development was planned for the summer of 1962. Furthermore, the spiral springs which serve as wrist joints must be replaced with more stable connections. These should provide dorsi-flexion only, but be otherwise passively adjustable, so that there may be a greater degree of security in grasping and holding.
The next step in our plan is to install an active pneumatic wrist-rotation unit, which will eventually be controlled through a transformer with two valves. I shall continue to report on this new provision. Even at this developmental stage, however, the case presented here indicated that it is proper and even necessary to initiate the application of prostheses and active training in the second year of a child's life.
It will not be possible to secure the same measure of success with every baby in the same relatively short period of time as was the case with Stephanie. Much depends upon the level of intellectual and physical development. Before provision for a prosthesis can be initiated, an available phocomelic finger must be trained for touching and grasping early in the baby's development. If there are no stumps or digits attached to the shoulder, training with the shoulder-girdle should be started at an equally early stage.
On the basis of our experience we must warn urgently against amputating rudimentary hands or fingers for aesthetic reasons, or because they might hinder the application of a prosthesis later. Such amputations were common practice until recently. A skillful orthopedic technician will discover means of adapting the prosthesis without disturbing or diminishing the mobility of such fingers or hands.
A weak phocomelic finger can be utilized to actuate a pneumatic prosthesis and provide the decisive functions of an artificial arm, even at the present level of development. Its use will be extended substantially in the future when it becomes possible to combine electronic controls with pneumatic systems. In 1960, for instance, at Rancho Los Amigos, the poliomyelitis center in Los Angeles, I saw a man whose arms were almost completely paralyzed bilaterally. The only functional muscle was the supinator on one side. With this he was able to control the pneumatic apparatus on the opposite side, by amplification of muscle-action potentials and an electronic switching device. At the present time, this combination is too expensive for a prosthesis; but we must bear in mind that technical progress continues to be made.
Even without a prosthesis, such a vestigial finger can be of great value to an armless person. For example, I can visualize a situation where the finger could directly control automated equipment in a factory by means of an ingenious electronic system. This might be achieved through a window in the prosthesis or even without any artificial limb. A development of this type is entirely feasible from a technical point of view.
We have noted that a short stump or hand-finger, attached to one or both shoulders, can operate proficiently, even in the present stage of development of the pneumatic prosthesis. Weak movements of the finger can produce strong motions in the artificial arm. This can be of vital importance to an armless child. On the other hand, the success of prosthetic techniques should not lead us to limit training to such devices only, to the neglect of the development and utilization of the feet in grasping. Even with the most efficient artificial devices, the more an armless person can learn to use his feet skillfully the less dependent he will be upon others for help. This applies particularly to dressing, undressing, and caring for his bodily needs. Such dexterity must be acquired in infancy (Fig. 10A and Fig. 10B ). It is essential to balance prosthetic instruction and pedal training in order that the patient acquire as far-reaching a degree of independence as possible in his daily routines and occupational activities.