Habilitation of a Child with Multiple Congenital Limb Deficiency

MARY D. BARBER, O.T.R.


Emphasis on the habilitation of a child with multiple, congenital limb deficiencies is primarily on fitting that child with one or more functional prostheses2. However, most of these children developmentally substitute available body parts for the absent upper limbs1. The lack of sensory feedback and the less dexterous function of a conventional upper-limb prosthesis may prove disconcerting and prevent the child from accepting the prosthesis as a functional and intimately associated part of his body. Therefore, treatment should emphasize an integrated system of independent body-part substitution and prosthetic use influenced by the preferences of the child.

Case Study

J.B., a Caucasian male now 12 years old, was initially seen at our clinic at 1 year 2 months of age.

History indicated that the mother's pregnancy and delivery were uneventful, although she had morning sickness from the third month through the sixth month. She received a tranquilizer for seven to ten days and was given an injection of penicillin for poison-ivy infection.

Physical examination revealed bilateral upper transverse hemimelia and a left lower transverse hemimelia. The right lower limb also presented a transverse hemimelia, with the femoral segment appearing deficient proximally. The limb was extremely foreshortened. The patient showed small hemangiomatous formations over the bridge of the nose, the forehead, and the nape of the neck. The lower jaw revealed micrognathia. The remainder of the physical examination was not remarkable.

At 14 months J.B. could roll in either direction, assume sitting position, and sit well with posterior support. He could support his upper trunk on his upper limbs when in prone position but could not assume a crawling position because of asymmetry of his lower limbs. Motor-development scores were within normal limits for his age, consistent with his disability. He could feed himself independently by holding finger foods between his upper-limb stumps. He manipulated toys between his stumps as well. Biscapular abduction was adequate for a conventional bilateral above-elbow prosthetic harness.

Therapy initiated at 14 months included body mechanics of sitting, trunk balance, mat mobility, and a general strengthening program. At 18 months J.B. was fitted with a right above-elbow prosthesis (figure-8 harness, outside elbow lock, 12P hook). Prosthetic training (three times weekly) emphasized unit placement, elbow flexion and extension, and terminal-device function. By age 3 he was fairly efficient at right upper-limb function. At 8 and 1/2 years J.B. was fitted with a conventional bilateral elbow-disarticulation prosthetic unit.

By age 1 and 1/2 J.B. was shifting weight well and beginning to establish a walking pattern, and initial fitting of bilateral lower-limb prostheses was at 30 months. At 9 years he was equipped with crutches to fit his upper-limb prostheses. Therapy was hampered by poor attendance and a move from this area for two years.

Further evaluation occurred when J.B. was about to enter junior high school at the age of 12 years. The prescription requested upper-limb prosthetic function. J.B. entered the clinic in a defiant and simultaneously threatening manner. He walked and sat independently and doffed his crutches by slipping out of the entire upper-limb prosthetic unit. His initial statement to the therapist was, "I'll use my prostheses when you make me, but they're no good." At first he refused to remove the prosthetic forearms from the crutches and preferred to justify verbally and demonstrate the superior function of his stumps over the prostheses.

Agility and dexterity with his stumps were superb. He accomplished most activities of daily living with ease. His independence in dressing was achieved by his mother's appropriate choice of pullover shirts, jockey undershorts, and trousers. His shoes remained on the lower-limb prostheses. He managed his fly with difficulty and could not independently don his lower-limb prostheses. He was dependent in toileting and had scheduled himself for times when assistance was available. He could manage his clothing and transfer to the toilet. Wiping himself was difficult but was accomplished by wrapping tissue around his left stump.

J.B.'s functional use of his upper-limb prostheses for tasks other than crutch walking is adequate but nongratifying. Attempts at independent self-care are clumsy and limited by prosthetic function. Dressing is confounded into a challenging cognitive activity. Dining technique is reliable except for positioning the knife in the dominant terminal device for cutting meat. Writing is laborious, slow, and accompanied by a plethora of verbal complaints.

School and leisure-time tasks at the time of this writing are accomplished with ease. His writing and drawing are pleasurable to him, and he is considering art as a career ( Figure 1 and Figure 2 ). He enjoys the challenges of shop skills and says the only activities he cannot actively pursue are model building and knot tying ( Figure 3 , Figure 4 , Figure 5 , Figure 6 , Figure 7 , and Figure 8 ).

Prosthetic management is satisfactory after three and one-half years of wear. His ability to position the forearm and lock the elbow in an appropriate position is excellent. However, he prefers to work with his elbows in extension (possibly because of greater sensory feedback from socket torque). Terminal-device positioning is a cognitive skill, accomplished far less readily than forearm positioning. Implement placement and manipulation in the terminal device are poor.

Functional treatment, with the aims of increasing prosthetic use and self-care independence, stressed terminal-device placement, manipulation of tools, and lower-limb dressing. Traditional upper-limb prosthetic training techniques were employed by utilizing activities in which J.B. was interested. Lower-limb dressing was accomplished by employing an adapted stationary walker and a Velcro closure on the pelvic belt of his lower-limb prostheses. The prostheses are positioned on the seat of an armchair with the knees in slight flexion and the heels on the floor. J.B. then slides into the unit and stands via the support of the adapted stationary walker. He then sits and tightens his pelvic belt, which is a wide Velcro strap looped through a D-ring.

An evaluation of prosthetic function reveals that prosthetic skills are inefficient but adequate. Appropriate terminal-device placement is hampered by an unsound body schema due to the additional length of the prostheses. J.B. exhibits an unsatisfactory gadget tolerance. His placement in a learning-disabled classroom and his threatened behavior at the lack of sensory feedback from the prostheses hint at perceptual problems which could be complicated by prosthetic use.

J.B. was encouraged to describe his feelings about the function and potential of his artificial arms. He recognizes as foremost the necessity for wearing his upper-limb prostheses for crutch walking. His figure-8 harness imparts a feeling of security in crutch placement which he prefers to the relatively insecure feeling of sockets molded directly to his crutches. Regarding future social pressure to wear his upper-limb prostheses, J.B. recognizes the importance of maintaining his skills, but he has doubts that peer pressure will come: "My friends don't care if I wear my prostheses or not."

J.B. is comfortable with his body image without arms ("My arms are too long with the prostheses."), but he prefers to have legs included ("But I'm still too short."). The lack of sensory feedback from his conventional elbow-disarticulation prostheses is the prime reason for not using his prostheses regularly. When pressured by parents, teachers, or therapists to wear his prostheses, J.B. recognizes that pressure is a powerful tool which is easily reversed ("I can make you mad by not doing a good job."). J.B. currently wears his upper-limb prostheses for walking and occupational therapy only.

The habilitation of J.B. demonstrates the importance of considering the preferences of the child when developing an integrated system of available body-part function and prosthetic use.

References:
1. Schmid, Harriet, Foot skills in children with severe upper limb deficiencies. Am J Occup
Ther, 25:159-163, 1973.

2. Sullivan, Richard A, and Felice Celikyol, Functional rehabilitation of the patient with
triple traumatic amputations. Inter Clin Inform Bull, 14:7-8:1-8, July-August 1975.