The Habilitation Of A Congenital Quadruple Amputee

Edward T. Haslam, M.D. Joan Hayden, O.T.R. Jean Dutro, L.P.T.


This colored female born on November 23, 1962, was first seen in the Juvenile Amputee Clinic at the Crippled Children's Hospital in New Orleans on November 22, 1963. The patient was the eighth of nine children delivered at term to a mother then 30 years of age. There were no unusual illnesses during pregnancy, and the only medication taken was Gantrisin, during the last trimester. The child was said to weigh four pounds at birth and was noted to have absence of both arms at or above elbow level and absence of both lower extremities.

Our initial impression was that this patient had a transverse hemimelia (elbow disarticulation type) of the right upper extremity, a transverse hemimelia of the distal third of the left humerus, and bilateral amelia of the lower extremities. A rudimentary toe was present on each buttock. The child was otherwise in excellent health but could not be hospitalized for prosthetic treatment until she was weaned.

At 16 months of age she was admitted to the hospital and was fitted with a plastic laminate socket attached to a wooden platform with four wheels, and with bilateral upperextremity prostheses. A special two-position outside-locking elbow hinge was used on the longer right side (Fig. 1 ). Training objectives at this point were to establish the patient's mobility in the wheeled socket, using the prostheses for propulsion, with the elbows in nearly full extension, and to accustom her to eating "finger foods" with the right elbow in maximum flexion.

Mobilization Training

The initial phase of mobilization training consisted of passive movements of the upper-extremity prostheses by the therapists to bring the arms through the range of motion necessary to contact the floor and propel the cart. This introductory exercise was followed by active motion on the part of the patient. As a result of this activity, it was found necessary to drill air holes in all of the sockets to increase the rate of heat dissipation. The tip of the left mitten terminal device was too soft to permit propulsion and was promptly replaced with a Dorrance 10AW split wafer hook similar to the one on the right. Since the right elbow unit was lighter than the left, the child was pulled off balance when she attempted to propel the cart. This difficulty was corrected by filling the right forearm shell with a plastic laminate material.

Initially the child preferred to push herself backward; therefore the wheels of the cart were reversed, the swivel wheels being shifted to the "front" and the straight wheels to the "rear." This arrangement made it easier for her to turn corners and propel herself forward. A tendency of the cart to tip over when the child pushed rapidly was controlled by attaching a strap from the back of the pelvic socket to the elastic back strap of the upper-extremity shoulder saddle harness.

Attempts to teach the child to swing through on the prostheses without the cart were unsuccessful because of pain in the axillae. After three and a half months in the hospital, the patient was discharged, being able to feed herself "finger foods" when the right prosthesis was positioned for her and to push herself about in all directions in the wheeled cart.

Terminal Device Training

At 21 months, both Dorrance 10AW split wafer terminal devices were activated, and control training was initiated on the right with the elbow in extension. The child independently initiated terminal device operation on the left, this initiative being encouraged. Training techniques consisted of simple play activities appropriate to her age. Some attempts were made at self-feeding, but they were not completely successful. She was discharged home for one month, but apparently the prosthetic equipment was not worn while she was away from the hospital.

At 23 months of age, the child was readmitted to the hospital. The above-elbow sockets were replaced, and the right elbow was changed to the standard Hosmer unit and activated for voluntary operation. Because she had not used the equipment at home, retraining was necessary. Control training was promptly accomplished. Self-feeding was reinstituted and accomplished with ease.

Since the child lived in a rural area more than 200 miles from the hospital, numerous readmissions for replacement of the equipment and additional training were necessary. During the interim periods at home, however, the prostheses were used and invariably required cleaning and repairs on read-mission.

Replacement Devices

At two years nine months, the terminal devices were changed to Dorrance 99X hooks and the left elbow was activated. A new plastic socket on a larger platform was constructed, with a cutout for the rudimentary digits on the buttocks. The child developed the ability to propel herself over rough ground, grass, and cement, as well as on smooth surfaces, and showed increasing skill in bimanual terminal device use.

At the age of three years two months, further training in the activities of daily living was given, but it was found that the patient was unable to don or doff the above-elbow prostheses unassisted because of difficulty in fastening the buckle closure. The buckle was replaced with Velcro (Fig. 2 ), and she learned to apply her own equipment lying supine on the floor to don the prostheses and then securing the Velcro closure with her teeth. Velcro was also used to hold the pelvic socket door open (by an attachment to the front left corner of the platform) when she transferred in and out of it.

At this time the patient developed low back pain, but X-rays did not indicate any pathology. However, she was apparently not getting good ischial weight-bearing. The socket

was redesigned to solve this problem, and her symptoms cleared up. As she became increasingly active, crossed safety straps were added to keep her in the cart (Fig. 3 ).

With continued growth and maturation the child wished to participate in outdoor play activity with her peers, and found that it was difficult to push the wheeled cart in grassy areas. A leather diaper was fabricated to encourage play activity, protect the buttocks, and increase pelvic and abdominal mobility and strength. The diaper was subsequently replaced with a molded leather socket (Fig. 4 and 5 ), which she used for "scooting" activities, propelling herself with her prostheses. Additional X-rays (Fig. 6 ) suggested that both humeri were complete and suitable for end-bearing, and Silastic end-bearing pads were placed in the above-elbow sockets. This addition greatly improved the patient's ability to bear weight on the upper-extremity stumps. Incidentally, these later X-rays indicated that the child's upper-extremity deficiencies were bilateral transverse hemimelias, elbow disarticulation type, with one humerus somewhat hypoplastic. Fig. 7 depicts the condition of the pelvis at this time.

Digits Removed

When the child was three years six months of age, additional training was given. It was determined at this time that the rudimentary digits on the buttocks needed to be removed, since the skin around them was becoming irritated and retained secretions with a foul odor. The operation was accomplished uneventfully, and after convalescence at approximately three years ten months of age (Fig. 8 ), lower-extremity prostheses were fitted and equipped with red shoes at the request of the patient. Initial training in stand balance was instituted with the assistance of the therapist for support, and balance was rapidly attained. However, some instability was noted, apparently due to the fact that the socket was made in two sections. Hinges were applied posteriorly to increase stability while still allowing the socket to be opened for ease of application. To encourage tolerance to the prostheses, as well as balance, a stand table was used. A wand (a simple wood stick or dowel), which was held in the terminal devices with the elbows flexed and locked at waist level and supported in the middle by the therapist, was used in the early gait-training phase. With this assistive device the child rapidly attained an erect position and was able to keep her head over her shoulders, hips, and feet when ambulating.

At this stage of treatment, mat activities and scooting in the leather socket were continued to increase trunk mobility and strength, and the wheeled cart was discontinued.

When sitting activities were initiated (Fig. 9 ), it was noted that the slippery surfaces of the sockets caused the child to slide forward out of the chair. Kemblo patches were added to each buttock shell to alleviate this problem.

Gait Training

The attainment of independent gait proved to be difficult and was achieved with the aid of conventional infant Canadian crutches with padded cuffs. As the child exerted force to pull the legs through in the four-point gait pattern, the terminal devices would open. To insure a firm, safer grip, therefore, the crutches were applied in reverse (Fig. 10 ). Moreover, the crutches were applied with the thumbs of the terminal devices in the neutral position and with the hand pieces of the crutch pointed laterally. The terminal devices were then rotated medially 90 degrees, thus putting the hand pieces and the cuff of the crutches in the correct alignment. The upper band of the cuff rested just under the forearm lift loop. It was necessary to pad the cuffs to prevent scarring of the forearm shells, as well as to gain purchase on the laminated surface. A four-point gait was then readily attained.

When attempting a swing-through gait for speed, two major problems arose: the elbows unlocked with bilateral forced extension of the arms, and at heel strike the hip joints would flex, causing the child to sit down forcibly. A swing-to gait for speed was adapted as being safer at this time. The child then learned to walk slowly without support (Fig. 11 ).

Other Training Techniques

Additional training has consisted of falling techniques both forward and backward. Thicker Kemblo has been added on the posterior shells to reduce the impact when the child fell backward. Anterior bumpers have been added above the hip joints to prevent the feet from striking the child's face when she rolled or sat (Fig. 12 ). She developed the ability to sit up unassisted from the supine position with full equipment by rolling over on her side and pushing up with the right elbow flexed at 90 degrees and the left extended. In the event of an emergency, such as fire, the child could now roll to safety even with full equipment.

Continued training in the activities of daily living, including such activities as getting in and out of conventional furniture, bathroom activities, getting up from the floor, and ambulating on rough ground, etc., will be emphasized. At this time, the child cannot put on her own crutches or rotate the terminal devices unassisted. At a later date some other type of crutch may have to be adapted if she continues to require these aids.

Conclusion

We have related our experiences with an intelligent but severely handicapped child living in a rural area under circumstances where the use of sophisticated equipment activated by external power appeared to be impossible. The ages at which this patient achieved the various functions described do not necessarily represent the earliest ages at which she might have done so. However, for the guidance of others who may be confronted with a similar problem, the timetable of events in this case are summarized on pages 10-11. This child has become totally independent insofar as eating is concerned (Fig. 13 ) and has acquired considerable independence in other areas. To achieve this result required close cooperation between the therapists, the prosthetist, and the orthopedist, since numerous minor modifications in prostheses and training procedures were necessary. Numerous additional problems remain to be solved if we are to achieve our goal-making this patient totally independent.

Acknowledgements

The authors wish to express their appreciation to Thomas L. Maples, CP., and Leo P. Marcotte, CP., who made valuable suggestions concerning the prosthetic prescriptions and fabricated the prostheses; and to Ruppert J. Warner, CO., who designed and built the two-position, drop-lock elbow hinge. We are also indebted to Roy Trahan, photographer, for the excellent pictures of our patient.

Table 1 , Table 2

Edward T. Haslam, M.D., Joan Hayden, O.T.R., and Jean Dutro, L.P.T. are associated with the Juvenile Amputee Clinic Crippled Children's Hospital New Orleans, Louisiana