Physiotherapeutic Treatment of Dysmelic Children
In 1962 the drug Contergan, which contained thalidomide, was taken off the market, but not before three to four thousand dysmelic children had been born in Germany. As a result, special children's habilitation and rehabilitation units were formed in Hamburg, Hanover, Münster, München, Frankfurt, Heidelberg, and Tübingen.
While some dysmelic children were treated in one orthopaedic clinic of the University of Heidelberg as early as 1957, it was not until 1965 that the department for "Dysmelien and technische Orthopaedie" was instituted by Prof. Dr. Ernst Marquardt.
Teamwork is a necessity for optimum habilitation results and Dr. Marquardts staff includes an assistant orthopaedic physician, a psychologist, a special teacher for handicapped children, a social worker, six nurses, five kindergarten teachers, four graduate occupational therapists, four physical therapists (with two additional students at different levels), and three secretaries. Two fitting workshops, one for the lower and the other for the upper extremities, are available. One physicist and some research technicians, as well as a photographer responsible for documentation, are also part of the team which takes care of some 750 outpatients and 24 inpatients, ranging in age from a few days to 12 years.
Typical findings for these "thalidomide children" show that more were born with a lack of the upper than of the lower extremities and that the deformities are more symmetrical than asymmetrical.
The typical upper-extremity deformity is the loss of or damage to the radius combined with a "clubhand" without a thumb. Correspondingly in the lower extremities the most frequent defect is of the tibia, with an equinovarus foot (Fig. 1 ).
Other frequently encountered problems include: poorly functioning joints due to impaired articulations, alterations in the spine, hip joints, kidneys, heart, and viscera; and, very often, ear and eye problems.
The children receive physical therapy, occupational therapy, and surgical treatment, and are fitted with prostheses. In the ward there are four kindergartens, each with six children. In each kindergarten at least one nurse and one kindergarten teacher or pre-school teacher are responsible. The children do not have to stay in bed for the whole day as in the usual clinic since they are disabled but not sick. If a child is really sick or has had surgery, rest in bed, and, if necessary, a separate room is prescribed, but as soon as possible he will return to the kindergarten.
The philosophy behind the habilitation program at Heidelberg is to use the defective extremities and to increase their strength and function; and to compensate for missing or weak function by improving, building up, and teaching the use of other parts of the body. Thus, an armless child has to learn how to use his feet for playing and eating; later he uses these extremities for writing and other activities (Fig. 2 ). A child handicapped in all four extremities is taught to manage many things with his hands, his feet, his mouth, or by a grip between his jaw and chest.
Children are fitted with prostheses primarily for improved function and later for cosmetic appearance as well as function. The child must also learn how to manage if the prostheses become broken.
We have learned that for successful habilitation the parents must also be part of the clinic team. Without their cooperation and confidence the efforts of the therapist will be less effective.
Treatments given to each child individually emphasize postural training, functional training of the deformed extremities, mobilization of the hips, balance training, orthopaedic bracing, gait training with and without prostheses, postoperative training after plastic surgery of the thumbs and hips, selection of the correct means of locomotion, and swimming.
Training of the whole body as well as the extremities for more skillful use is also indicated, plus the use of proper aids such as braces and prostheses. Advice concerning special clothing for children who use their feet and short hands for dressing and undressing can be obtained by the parents in the Occupational Therapy Department.
Mat sessions, each usually lasting an hour, are especially important in the individualized programs. Treatment is given in the form of play so that the child enjoys himself and is not forced to perform exercises which are too complicated. Before and after treatment children between the ages of four and five years who can dress and undress themselves are supervised in these activities.
Toys are used for each activity to provide motivation. Thus a child can be led to train himself without knowing it. The therapist tells a child to pick up a toy with his toes and bring it to his hand, and the child can do this with his leg in the desired abduction position. Obviously, a child would only be confused if the therapist were to instruct him to abduct his hip. Sticks, balls, cloth animals, building blocks, a red ball, rocking board, stairs and rung ladder, the parallel bars, or the trampoline, are all used in the program. The desire of young children to play ensures that the sessions are fun as well as being of therapeutic value.
In physical therapy, attention has been drawn to the importance of the psychological and pedagogic aspects of training. In her treatment, the physical therapist must use her knowledge and understanding of child psychology to motivate the child. The program of physiotherapeutic treatment will be determined by the level of the child's deficiencies, and by his psychological, mental, and intellectual development.
One dysmelic child with birth deficiencies of all four extremities began treatments for the first time at the age of six years. She was not able to speak, eat by herself, or sit up without help. After medical and psychological tests and evaluations jn physical and occupational therapy, it was discovered that her true development age was two years, not six. In cases such as this treatments must be adapted accordingly.
An attempt is made to give every child the same therapist each time so that good rapport is established. Changing therapists requires that the child make a new orientation and establish another relationship, thus increasing his difficulties.
In physical therapy treatments, as well as the fitting of the lower extremities with special braces and prostheses, are prescribed by the physician.
The most important individualized treatment in the physical therapy program is postural training (Fig. 3 ). The poor posture of the thalidomide child is a result of:
- Repeatedly assuming special habitual positions needed for balance while playing, eating, writing or painting. These children easily develop kyphoses, lordoses, and scolioses. If they are not able to compensate actively for the poor posture by straightening the spine, the conditions become pathological.
- Differences in the length of the extremities.
- Weak function of muscles and misplaced and asymmetrically developed muscles.
- Congenital defects of the spine visible only in x-rays.
Many poor postural conditions can be avoided by daily systematic exercises and swimming if these activities are started early (Fig. 4 ). Therefore, an attempt is made to have the mother or father exercise with the child at home for half an hour each day.
The physical therapist has to plan an individual home program for each child and then show it to the parents. They are given special exercise forms and the exercises are checked and new ones added each time the child is brought in for therapy.
Before entering school, the child's level of achievement should be such that the program of exercises can be reduced to avoid overloading him.
Special Training of Hands and Arms
Postural training is given to almost every dysmelic child (see above) and takes the most time. However, special exercises must also be added to improve the function of the defective extremities. All of these exercises seek to increase the range of motion of the limbs. In the first year after birth and in early childhood, passive range-of-motion exercises are applied in order to avoid or decrease clubhands or other contractures. Later, active exercises will follow to increase the range of movement of clubhands, to improve pronation and supination, and to achieve better function of the elbow and the shoulder joints. Special attention must be paid to the underdeveloped biceps and deltoid muscles. Activities which require skillful coordination, such as the game of quoits, combine play with effective training.
Special Training of Feet and Hips
Children without any arms have to use their feet instead. Children with phocomelia of the upper extremities have to use their feet in addition to their arms in order to become independent in the activities of daily living. The occupational therapist is responsible for developing skill in one or both feet, while the physical therapist has to effect special mobilization of the hips in order to obtain a greater range of motion (Fig. 5A , Fig. 5B , and Fig. 5C ). The shorter the arms the more important it is to improve the range of motion in the hips in abduction, with external rotation, together with about 90 deg. flexion in the knee and hip joints.
Passive and active exercises to correct club-, valgus and pes planus feet, and resistive exercises for each part of the lower extremities are given. The more strength there is in the legs the better control the child will have of his prostheses, and the better his balance and gait will be.
Balance Activities to Prepare the Child for Gait Training
Initially balance has to be achieved in the sitting position without artificial limbs and, if possible, in standing and walking situations. Exercises on the rocking board are preferable. Children who are not able to stand on their own feet will be fitted with orthopaedic boots or, if that is impossible, with rockers or stubbies at the proper maturational time. At the time of the first fitting, gait-training will be necessary.
Fitting with Prostheses and Braces, Gait Training, and Activities of Daily Living
The best possible fit of prostheses and braces can be achieved only if there is close cooperation between the physician who prescribes them, the pros-thetist or orthotist who fits them, and the physical therapist who trains the child and knows him best.
A special functional test of the lower extremities in the supine and prone positions, and if possible with the child on his own feet, can provide useful information. Children with phocomelia of one or both lower extremities have best muscle power for external rotation. Thus, most of the children are given a device called an "external buildup," which requires that the cast be taken with the legs in the abducted position. Sometimes this procedure means less cosmesis, but it increases function. The uprights of the device follow the flexed angulation of the hips but recurve to bring the artificial feet under the body (Fig. 8A and Fig. 8B ).
After the insurance company has agreed to pay for the prostheses or the braces, the cast is applied by the prosthetist with the help of the physical therapist who accompanies the often frightened child to the fitting sessions.
Between fittings the child will have gait training, and every defect that appears will be corrected immediately; for instance, faulty fit of the socket, pressure spots, or insufficient adaptation of the socket buildup to the functional position of the legs. While the child is being fitted, an attempt is made to compensate for kyphotic, lordotic, or scoliotic spinal deviations.
Gait training includes several series of exercises. First, the child is taught to stand safely by himself in his prostheses. Balance training is given in the upright position as well as on the rocking board and ambulation training indoors as well as out (Fig. 9A and Fig. 9B ). Later, stair climbing is added.
Training in activities of daily living is very important in teaching the use of the limbs and should include rising from a chair and sitting down, falling down and getting up. In occupational therapy these children learn to become independent with their limbs when using the toilet.
Means of Locomotion
Development of the child to achieve the fullest independence is only possible when the upper extremities are normal, or when there is only a slight defect. The more severe the upper-extremity disability the more the child has to relinquish his independence in using the prostheses. His mobility and his ability to explore the environment are also more limited. With these considerations in mind, it will definitely be necessary to supply him with a means of locomotion such as a bicycle with training wheels (Fig. 10 ). It is necessary to discover the most suitable means of locomotion for each child.
In children with defects of the upper as well as the lower extremities, surgery is often necessary to improve function or to increase stability. The occupational and physical therapists are responsible for the postoperative training, especially after plastic surgery of the thumbs and hips. The permanent success of these operations depends upon the correct method of treatment and the proper fit of splints and braces.
The children begin to learn to swim between the ages of two and three years. Initially lessons last approximately ten minutes. Later the children are in the water for half an hour. The water temperature is maintained between 84 and 86 deg.
Each child has his own physical therapist who helps him become used to the water by playing and plunging (Fig. 11 ). Experience has shown that swimming lessons should begin in the supine position, in which these children feel safer (Fig. 12 ). Breast or prone swimming follows later. The "Heidelberger Halskrause" is used and special floats are applied to the arms and around the waist. The children, however, are gradually weaned away from this apparatus so that they will not become overly dependent on it. Children with defective upper extremities are taught normal motions with the legs, and, if possible, circular motions of the short arms.
For the child who is handicapped in all four extremities, the physical therapist tries to discover the best means of getting him active in the water. Very often these children use a dog paddle in learning how to swim. The children must be encouraged to enter and to leave the swimming pool by themselves, whether by walking in, sliding over the floor, or crawling. Jumping into deep water should also be part of the exercises.
Other types of sports should be permitted according to the functional capacity of the child. Horseback riding, skiing, roller-skating, trampoline (Fig. 13 ), and group athletic exercises are typical activities.
Group exercises are held for one hour once during the week for children with upper-extremity disabilities and at another time for children with defects in the lower extremities or in all four extremities (Fig. 14 ). Group exercises, although important from the psychologic and pedagogic viewpoints, do not take the place of the individual treatments. Without any appliances the children can move about and they easily learn to participate in group play. In the performance of skillful exercises they are encouraged by watching others. They exert greater efforts to accomplish the activity and use all their resources to do so.
Instructions and Advice to Parents
After a child has been discharged from the ward, he is seen in the dysmelic outpatient clinic.
The doctor informs the parents about the child and about the necessity for him to do exercises regularly. Each exercise in the home program is demonstrated and written up for the mother or father (Fig. 15 ). If necessary, on the next visit, the child will be examined in the outpatient clinic, and the medical report and x-rays will be reviewed. Then the exercises will be repeated and improved upon, and new ones added.
The physical therapist can determine whether the exercises are being done correctly at home by supervising the exercises as they are demonstrated by the mother or father. However, supervision is not enough. If the child has been fitted with prostheses the parents must know:
- how to put them on and take them off
- the length of time the child can wear them during the day
- when the special helmet is to be worn
- what to do in case pressure spots develop while the prostheses are being worn
- how to recognise when the child has outgrown his prostheses.
Often the parents have many questions and the physical therapist must take time to answer them in detail. The counseling process may be discouraging if the parents are not cooperative. However, the majority of the parents do make continuance worthwhile. These parents are willing to make every effort to improve the lot of their child. The therapist learns from the child's progress, or lack of it, and the clinic is brought closer to the purpose of its work-the broad habilitation of the dysmelic child.
The author is grateful to Prof. Dr. Ernst Marquardt and to her colleagues in physical therapy at the University of Heidelberg for their contributions to the article; and thanks Miss Hazel Wong, R.P.T., of the Institute for the Crippled and Disabled, New York, for her help in the preparation of the manuscript.
Aus der Orthopädischen Klinik und Poliklinik der Universität Heidelberg, Germany (Director: Prof. Dr. H. Cotta); Abteilung für Dysmelein und technische Orthopaedie, Leiter: Prof. Dr. Ernst Marquardt.