Congenital Limb Deficiencies in the Socially Disadvantaged: A Study of Two Ongoing Cases
RICHARD A. SULLIVAN, M.D. FELICE CELIKYOL, O.T.R.
Because of the close proximity of the Kessler Institute to Newark, New Jersey, we have been seeing an increasing number of disabled children and adults of Spanish-Puerto Rican origin in our Clinics. These patients combine the problems of physical disability with additional obstacles of language barrier and socioeconomic disadvantage. We have found that language difficulties and poverty combine to worsen the plight of the disabled person. Under the best of circumstances, the employability of a severely disabled person who has been rehabilitated presents a difficult problem but when the handicaps of language and educational deprivation are added the difficult becomes the impossible.
In addition, secondary to ethnic distrust and misunderstanding, there is often a complete rejection of the available social agencies and the help that they can affort the family of the severely disabled child. This rejection results in additional social, educational and financial deprivations which are totally unnecessary.
This paper presents case studies of two Puerto Rican children with severe congenital limb deficiencies who were physically, socially and educationally neglected until the full force of the Rehabilitation Center's team effort was brought to bear on their total problem.
The necessity for a full team approach to the problems of the disabled in this segment of our society cannot be overstressed. In both cases, earlier efforts at rehabilitation without a full team effort resulted in a temporary loss of both patients from the clinic, little progress in the area of prosthetics understanding and training toward independence, and educational deprivation. These problems were not fully recognized or attacked at the time of the initial clinic contacts.
Case 1.-Josephine, presently ten years of age, was born in Puerto Rico, Sept. 4, 1961. She has congenital deficiencies of all four limbs:
1. Amelia of the left upper limb.
2. Terminal transverse hemimelia of the right upper limb with no true axilla. X-rays revealed the presence of a humerus approximately 5 inches in length ( Fig. 1 ).
3. Phocomelia of the right lower limb with a rudimentary foot.
4. An intercalary deficiency of the left lower limb with a femur present, no knee joint, and the foot held in varus. The great toe and three smaller toes were present.
Josephine first came to our attention at the age of eight years ( Fig. 2 ). At that time, she was fitted with a left shoulder-disarticulation type of prosthesis and a right conventional above-elbow prosthesis. Soon after, contact was lost with the family and it was not until a year later that we were again visited by Josephine and her parents. She had not been wearing the prostheses for a number of months and had outgrown them. It was then that the decision was made to hospitalize her for an intensive evaluation.
First Admission (Sept. 16, 1971-Oct. 22, 1971)
Josephine had just turned nine years of age when she was admitted for one month's stay at Kessler Institute.
Functional Abilities on Admission
1. Ambulation was accomplished in a seated position by a sort of "waddling" movement; otherwise she was carried everywhere by family members. She had never been provided with any means of independent locomotion.
2. The left foot was used for feeding with the plate placed on the floor, or she was fed by the family.
3. Home tutoring provided during the previous year had been sporadic and obviously unsuccessful. She could neither read nor write although her spoken English was passable.
1. Josephine received a new right above-elbow prosthesis and a left shoulder cap. Contact was made with New York University Prosthetics and Orthotics unit to obtain a left Ontario Crippled Children's Centre (OCCC) coordinated electric arm.
2. Prosthetic-use training was provided and Josephine learned to feed herself using a swivel "spork" (spoon-fork combination) which was placed in the terminal device for her. The elbow unit was left unlocked and elbow flexion was achieved by pushing the forearm against the table. Josephine found this technique easier. Desk skills such as writing, typing, turning pages, and painting were practiced.
3. Self-care activities were reviewed and she was encouraged to be independent. She was able to manage limited grooming using her foot and limited dressing (overhead clothing could be donned by using trunk motions and her teeth).
4. The patient was also provided with a motorized wheelchair-foot-controlled. She was able for the first time to move about independently. A regular child-size wheelchair was also provided.
5. Contact was made with a school for the physically handicapped located near her house. They required that she be able to use at least one upper-limb prosthesis. They agreed to keep the motorized wheelchair at the school and maintain it. She was enrolled and on discharge from the Institute was able to attend school for the first time.
Josephine was discharged home and was scheduled to return for training in the use of the OCCC electric arm when it became available.
Second Admission (Jan. 4, 1972-Feb. 18, 1972)
Three months later the OCCC electric arm was received. Josephine then became a participant in a five-months' evaluation study, and at its termination she would be permitted to retain the prosthesis if she found it to be functional ( Fig. 3 ).
The prosthesis is battery-operated with three microswitches located within the shoulder cap.
1. Scapula abduction activates the anterior button to produce coordinated elbow and shoulder flexion.
2. Scapula adduction activates the posterior button to produce elbow and shoulder extension.
3. Shoulder elevation activates the button which controls terminal-device opening (the hook closes when pressure is released from the switch). The terminal device was originally a three-finger type and was to be replaced by a two-finger hook at the end of the second month as part of the study.
Program and Progress
Josephine rapidly learned how to activate the microswitches. She found object positioning, particularly writing, difficult when using the three-pronged terminal device but nonetheless persevered and did well. She preferred the Ontario Crippled Children's Centre electric arm to the conventional above-elbow prosthesis for all activities-most especially feeding. Josephine continued to use the swivel spork and also a special contoured plate. Initially, the feeding pattern was not ideal and it was necessary for the prosthetist to realign the arm by adding a wedge at the shoulder joint to provide external rotation and abduction. This allowed the terminal device to reach the mouth directly rather than bypass it as had happened previously necessitating neck motion to achieve success. After this adjustment was made, very little assistance was necessary once the food had been arranged at her place setting. Josephine was able to pick up the utensil, position it in the terminal device by using her mouth, and feed herself.
She has continued to improve her facility in self-feeding, which is the primary thrust of the OCCC coordinated electric arm ( Fig. 4 ).
We are awaiting delivery of the UCLA Child Amputee Prosthetics Project (CAPP) cart as a replacement for the standard motorized wheelchair which she now operates with her (left) toe. This cart will provide the additional feature of allowing her to elevate the seat level for feeding and desk work, and offer a more functional operating and turning pattern.
Her family accepts the child and her disability totally. Their cooperation has been greatly enhanced by her admission to the Institute and, secondary to this, their total involvement in the child's prosthetic training. Social Service contact has eased their problems with the community. We have changed the pattern of Josephine's education by enrollment in the local school for the handicapped rather than continuance of the ineffective tutorial guidance she had received at home. Prior to this program the family had seemed unaware of the social agencies available to assist them. They had never been completely involved in the rehabilitation program. Weekly social service contacts had opened up to them new avenues of help to solve their many problems related to this child, her disability and its impact on their lives.
The Vocational and Psychological services of the Institute had fully investigated the child's vocational potential. They then sought out and directed the parents to the local school for the handicapped resulting in Josephine's acceptance by her peers at a fully accredited school. This acceptance ended years of isolation at home where there had been little stimulation for education and self-improvement.
The result has been an increasingly more independent child receiving the full support of a cooperative and involved family and community.
Case 2.-Samuel, age 3 1/2 years, was born in New Jersey, Dec. 11, 1968, of Puerto Rican parents. He has deficiencies of all four limbs:
- Right terminal transverse partial hemimelia (short below elbow).
- Left terminal transverse partial hemimelia (long below elbow).
- Bilateral terminal transverse hemimelias (essentially constituting knee disarticulations, although some soft tissue redundancy was present below the femurs).
This child was one month old when he was admitted to Babies Hospital in Newark for a complete work-up. The only additional finding other than his amputations was that he had ankyloglossia (tongue-tie), but this did not interfere with feeding. During his stay in the hospital he developed complications of severe bilateral pneumonia and was therefore not discharged until he was 3% months of age. It was at this hospital that we first examined Samuel when he was six weeks old. Recommendations were made that he be seen for the application of upper-limb prostheses at the age of four to six months and that at eight to ten months he receive lower-limb prostheses without knee joints.
He was not seen again by us, however, until he was ten months old. At this time recommendations were made for pylon-type lower-limb prostheses with quadrilateral sockets, Silesian belts, and posterior platforms (stubbies). A Muenster prosthesis was also prescribed for the right short-below-elbow stump. The decision was made not to prescribe a prosthesis for the long left stump so as to allow him to retain sensation in at least one extremity. These prostheses were fabricated and delivered.
Subsequent Follow-up Visils
After the prostheses were received, the parents were instructed in a home program. No social service or social agency was involved at that time. It was obvious at the next clinic visit that the family had not followed through on the home prosthetics program.
The next visit took place when Samuel was 18 months old. He was not using his prostheses. He had not yet developed standing balance without support and was "shrugging" out of the Muenster socket. We noted that he had 15-20 deg. of hyperextension at the elbow which seemed to enhance his quick removal of the arm. We then decided to prescribe a conventional below-elbow prosthesis. In order to encourage standing balance and endurance, a standing table was also prescribed. We hoped that, by using this standing table, Samuel would also be more inclined to use both upper limbs.
Home Health Services
There was obviously poor family understanding and cooperation in prosthetics use. In addition, transportation to the Institute was a persistent problem and, because of this and the fact that Samuel and his parents were not cooperating in any attempts at training, we notified the Home Health Services Agency and our Social Service Department became involved with the family and its problems.
The visiting nurse was instructed in the care and use of the child's prostheses and the home program was reviewed with her. She was to visit the home on a regular basis to provide guidance to the mother-encourage her to apply the prostheses, place the boy in the standing table, encourage him to walk with the lower-limb prostheses and encourage upper-limb prosthetic use. Through the combined effort and close cooperative agency-clinic contact, success was achieved for the first time with the patient and with his family ( Fig. 5 ).
Samuel is now ambulating independently and showing good balance with his bilateral stubbies. He has been provided with a left below-elbow prosthesis in preparation for eventual ambulation with crutches. He has a conventional right below-elbow prosthesis ( Fig. 6 ).
Recent clinic visits showed that Samuel was using his upper-limb prostheses more and that the mother was more diligent in applying the prostheses and following the home program. By educating the parents and enhancing their understanding of the disability and the child's needs, great strides have been made over the past year.
We also do not want the boy's English language deficiency to develop into a further handicap. His parents continued to speak only Spanish at home resulting in little understanding of English by the child. As an adjunct to his prosthetic rehabilitation, a nursery school to provide exposure to his peers and stimulation in the English language is now being investigated by our psychological-vocational services in addition to our social service efforts. In this way we hope to avoid the educational problem encountered by Josephine.
Two cases of children with multiple congenital handicaps have been presented. The prosthetic problems and their solutions have been discussed. In addition, the problems of social disadvantage and language barrier have been considered with emphasis on the ways in which these problems can hinder the rehabilitation process. The need for a full team approach to the habilitation of these children has been stressed. Involvement of the social services of the hospital, the psychological and vocational services of the Institute, and the local service agencies of the community working together with the medical therapeutic team is essential for successful habilitation. Failure to enlist the help of any part of the team can result in failure of the effort, both with the patient and the patient's family.