Training A Patient With Bilateral Upper-Extremity Phocomelia And A Foreshortened Left Lower Extremity In Self-Care Activities
Felice Celikyol, O.T.R. Hildegard Boynton, R.N. Alice Stymacks, R.N. Ki Ho Kim, M.D.
D.G., a white male, presently 11 years of age, was born in September 1957 with skeletal limb deficiencies of both upper extremities and the left lower extremity. His condition was classified as proximal phocomelia of both upper extremities with syndactyly of two digits on the left (Fig. 1 ). X-rays revealed that the radius and ulna were underdeveloped bilaterally. The femur of the left lower extremity was foreshortened.
The patient's capabilities were evaluated when he was first brought to the clinic in 1962 at the age of five years. It was found that trunk and hip flexion were moderately limited so that the boy was unable to reach his mouth with either foot. Although he did have some skill in using his toes for prehension, self-care activities had been carried out by his family. He was unable to feed himself, or even partially dress or undress himself. Hence he was quite dependent on the help of others at this time.
Application of First Prosthesis and Training
Following evaluation, D.G. was provided with a right shoulder-disarticulation prosthesis which contained a nudge control for elbow lock and unlock; a turntable, and a peroneal strap for terminal-device control.
Training included exercises to increase the range of motion and strength of the hip and trunk, bilateral and unilateral use of the feet, and prosthetics training.
Status Upon Discharge
D.G. could successfully feed himself with his prosthesis using a small fork and a swivel spoon. He was able to use the prosthesis for pre-writing activities and certain play activities. He was now using his feet more frequently, but he still found feeding himself to be quite difficult.
D.G. demonstrated good intellectual capacity in that he was quick to learn and retain instructions. In addition, he demonstrated ingenuity in devising new ways to use his prosthesis without prompting. Prior to discharge he was provided with a wedge build-up for the left shoe.
Appointments for periodic examinations were not kept, and D.G. was not seen for reexamination until he was eight years of age at which time it was noted that he had definitely outgrown his prosthesis. X-rays were taken and revealed a slight scoliosis of the lower thoracic spine with the convexity of the curve toward the left and the right iliac crest one-half inch higher than the left. In view of these findings, it was decided that D.G. should be fitted with bilateral arm prostheses and plans were made to provide him with American Institute for Prosthetic Research (AIPR) CO2 prostheses.
In June 1965 the patient received his bilateral AIPR pneumatic prostheses and attended clinics for training. These prostheses consisted of bilateral shoulder caps with openings through which his phocomelic hands could operate the valves. The right prosthesis consisted of a power-lock shoulder joint and humeral turntable, a special feeder component, a wrist unit and a 12P hook. Elbow movement was controlled by the front portion of the rocker valve while the posterior portion controlled terminal-device operation. The left prosthesis was essentially the same except that a peroneal strap was used for operation of the terminal device; while buttons located within the shoulder cap and actuated by the tip of the acromion controlled elbow movements.
In the summer of 1966 the patient received a new pair of revised AIPR prostheses (Fig. 2 ). The right prosthesis contained an automatic shoulder lock, power-lock turntable, anterior and posterior valve buttons located within the shoulder cap and activated by the acromion (used to control elbow flexion and extension), and a wrist-rotation unit (controlled by applying pressure on the anterior portion of a rocker valve with his fingers). The terminal device was operated by pressure applied by his appendage on the posterior portion of the valve.
The left prosthesis was essentially the same except that a feeder component was included on that side and no wrist-rotation unit was provided.
Although the new prostheses provided excellent function when they operated properly, a number of persistent problems were encountered in their use. These problems were:
Leakage of CO2 at the valves.
Instability of the shoulder units.
Loosening of turntables.
Malfunctioning of the wrist-rotation unit.
Self-Help Equipment and Training
When the patient received his revised AIPR prostheses in June 1966, he was readmitted to the Institute for training with emphasis placed on developing maximum independence in self-care. During the course of this training, it was found that the pneumatic prostheses were of most value for feeding and school activities. Tasks for which the prostheses were particularly useful were:
Writing - he was able to pick up and position a pencil using his wrist-rotation unit.
Blackboard - he could pick up and position chalk in essentially the same manner as a pencil.
Typing - he could use an electric typewriter without difficulty.
Feeding - he used the right prosthesis which incorporated the wrist-rotation unit rather than the feeder component on the left arm.
Utensils were provided with metal loops for added stability. The boy was also provided with a sandwich-holding device (Fig. 3 ).
Initial evaluation showed that without his prostheses D.G. required help in such activities as cutting meat, trimming his toenails, washing his back, adjusting his clothes for toilet use, cleaning himself after toilet usage, putting on and removing his T-shirt, putting on and removing a dress shirt, managing buttons, removing and putting on briefs and slacks.
At the time of discharge, he continued to need assistance in cutting meat and trimming his toenails.
A frame device was made for D.G. and fastened to his own bed at home (Fig. 4 ). With the use of this frame, he was capable of putting on and removing his shirt (Fig. 5 and Fig. 6 ) and pants. Velcro replaced buttons and D.G. used elastic laces in his shoes.
He was provided with a special grasp shoehorn to prevent breakage of the shoe counters when he put on his shoes (Fig. 7 ).
A special device for holding Kleenex and for nose-blowing was also attached to the frame. Kleenex was transferred by mouth from the holder to the forked "nose blower" (Fig. 8 and 9 ).
In Fig. 10 D.G. shows how he is able to lower and raise his pants and underpants. The double hook on this frame has been fastened to his home bathroom wall so that he is able to manage pants for toilet procedures. It has also been installed in the school bathroom.
A special device permits D.G. to be independent in cleaning himself following use of the toilet (Fig. 11 and 12 ). A similar device was provided for the school bathroom. He is able to carry the device to the toilet between his chest and chin. He places it on the rim of the toilet bowl and inserts the paper with his toes or prosthesis. To dispose of the used paper, D.G. applies his foot to the clean underside of the paper and kicks it into the toilet.
Conventional Prostheses Reapplied
Because the pneumatic prostheses were too frequently in need of repair, D.G.'s last admission in August 1967 was for training in the use of bilateral conventional prostheses. These prostheses contained shoulder caps, bilateral turntables, chin nudges on both sides for control of elbow lock and unlock, peroneal straps bilaterally for terminal-device operation, and two voluntary-opening Dorrance 88X hooks. D.G. was taught to don and remove his prostheses by lying on his bed. Putting on the prostheses required that he first slip into the peroneal straps and then the shoulder caps (Fig. 13 ). The sequence is reversed in removing the prostheses. Velcro tape has been sewn onto the chest straps to eliminate the need for a buckle. This method of fastening and unfastening can be managed by D.G. using his feet and teeth (Fig. 14 ).
D.G. continues to use his dressing frame at home. The bathroom hooks and wiping device are used both in school and at home. He uses his prostheses primarily for feeding and school activities. High vamp loafers have been ordered since the oxford shoes were never laced snugly enough.
A soaping device has been built and installed in D.G.'s shower stall at home so that he is able to soap his body independently. This soaping device was made of Kydex plastic and was molded around a large can to produce a convex surface to which a towel is taped. It has been attached to the corner of the shower-stall wall (Fig. 15 ). The towel is soaped and D.G. can then soap his back and chest by rubbing against it.
This boy has achieved a reasonable degree of independence through the use of his feet and conventional prostheses. However, it is obvious that he could use much more prosthetic help than we have yet been able to provide him. It is our opinion that this additional help must come through the medium of externally powered components with greatly increased reliability. These devices should provide greater functionability while reducing the patient's expenditure of energy. We urge developers to expedite their efforts to this end.
Felice Celikyol, O.T.R., Hildegard Boynton, R.N., Alice Stymacks, R.N., and Ki Ho Kim, M.D. are associated with the Kessler Institute for Rehabilitation West Orange, New Jersey