Thalidomide Children; Thirty Years Later
ERNST MARQUARDT, MD ED. JOHN R. FISK, MD
Thalidomide was developed as a sedative by the Gruuenthal Chemical Corn-Ipanyof Stulberg, West Germany and was first released on the market in October, 1957. Ten European, seven African, seventeen Asian, and eleven other countries, including Australia and Canada, approved the medication for general distribution. It was never, however, approved by the Food and Drug Administration in the United States thus forestalling in that country what was to become a major medical misfortune in those countries where it was released.
Beginning in 1960 in West Germany and elsewhere, there was an increase in infants born with severe limb deficiencies. They were primarily of the longitudinal, radial, humeral, tibial, or femoral type, or combinations of these. Some estimated that there were four thousand or more Thalidomide babies in West Germany, but the exact number attributed to the Gruuenthal product has been established at 2856. Two hundred fifty-one of them lived in other countries, sixty-five died prior to 1989 leaving 2540 thalidomide victims in West Germany. There were many other malformations in addition to the limb deficiencies; 676 external ear deformities, 262 complete deafness, 628 varying degrees of hearing deficit, 1100 were visually impaired, 12 were blind, and 193 had congenital heart defects. There were many urogenital malformations and one hundred-eleven had anal atresia. Other anomalies included maxillofacial malformations and mental retardation (Fig. 1 ).
In 1961, H. R. Wiedemann first published the occurrence of the increased numbers of aplastic malformations without knowing the cause. Later that year, an attorney in Mendeu, West Germany found that not only his wife but also another woman in the same town gave birth to very similarly malformed children. He consulted Dr. Lenz who later was to become Professor of Human Genetics in Muenster. Dr. Lenz identified Thalidomide as the causative agent.
This was felt to be the case with a high degree of probability if the medication was taken between the 34th and the 50th day after the previous menstrual period.
On November 15, 1961, Dr. Lenz expressed his suspicions to the Gruuenthal Company. Four days later he presented this material at a pediatric meeting in Duseldorph and on the 27th, twelve days after it had been called to their attention, Gruuenthal withdrew Thalidomide from the market in Germany, other countries quickly followed.
Many Thalidomide babies continued to be born in West Germany up until August of 1962. This frequency of the characteristic post Thalidomide anomalies diminished rapidly nine months following Lenz?s courageous warning. The lay press was primarily responsible for this for the medical literature lagged far behind with reports of these early findings.
The Thalidomide catastrophe attracted peoples? attention all around the world. In West Germany some of the pediatric clinics organized special dysmelia centers for the many limb deficiencies. In many cities (Berlin, Bonn, Colon, Frankfurt, Hamburg, Hanover, Munich, Heidelburg, and Muenster) parents came together in self-help groups under support from the federal government. These parent groups and the dysmelia clinics exchanged ideas and experiences. There was an interchange with established clinics in other countries as well. The United Kingdom, Sweden, and the United States already had established the team approach to the limb deficient child. This experience was drawn upon by the new clinics in West Germany.
Those affected by Thalidomide owe their thanks to individuals such as Henry Kessler, Charles Franz, Claude Lambert, Mylo Brooks, Howard Rusk, and many others. Special mention should go to Harold Wilke. Dr. Wilke, a minister and professor of practical theology, was born without arms. His experience with the healing community helped in the beginning to work out plans for the support of the parents and children.
We had to help mothers and families accept their limb deficient child as he or she is. Only those who were able to make this adjustment were able to cooperate in their care. Our goal was independence in recreational activities as well as activities of daily living appropriate to the child?s age. This approach allowed thee children to have their parents? love, have friends and joy and develop a good outlook for their future lives.
The post Thalidomide population presented a big challenge for a young occupational therapy profession of the sixties. Those without arms numbered one hundred thirty and those children having minimal residual limbs numbered 750. They needed an opportunity to learn "to use everything they have", to paraphrase Dr. Wilke. For example, one child sent to us by Dr. Kessler, prior to the Thalidomide experience, was fit with pneumatic prostheses. The child was able after just three days of training to eat, drink, and operate pronation and supination. But when later seen in his home he was found to do much better with his own feet. He had rejected his prostheses in favor of "what he had". We learned that the feet must be free and uncovered from the very beginning for an individual to adapt them for manual function.
Credit should go to many for the development of a new generation of pneumatic externally powered prostheses. There were German engineers with names such as Haefner, Krame and Emmerich. There followed the cooperation of Edward Kiessling in New York, Bo Klasson in Stockholm, David Simpson in Edinbough, and Dr. Naedes of Otto Bach in Dudestadt, West Germany. The first devices were built in our own workshop, but we could not keep up with the volume. Otto Bach made the majority of devices for us. Nevertheless, feet were preferred by most.
Very few individuals continue to use prostheses today. Some would use them in public, but behind their bedroom door they are independent with their feet. We were to learn that the majority of these with congenitally absent arms gave up the prostheses in favor of using their feet in social as well as in professional life (Fig. 2 ).
Occupational therapy in these early days helped the parents learn to facilitate the use of feet as hands. Clothing needed to be modified to permit freedom of the feet for touching, grasping, playing, and later eating, drinking, washing, dressing, and toileting (Fig. 3,4 ). Even when there were hands, short proximal segments generally made feet necessary for complete independence.
In order to be independent with their feet technical aids were necessary. The correct working surface for secretaries with adaptations of typewriters for phocomelic hands were developed (Fig.5,6 ). The French developed foot controls and the English developed a system for the quadremembral person. Those without either arms or legs cannot go to work without a car. It is impossible for them to get on a bus or a train with an electric driven wheelchair, but with a joystick and a computer they can operate a normal automobile. Such mobility is most important for an individual to find employment and to find fulfillment in a social sense.
Therapists were very helpful in improving function. Muscles needed to be strengthened to better assist with balance. There often was seen an imbalance between the vastus lateralis and the vastus medialis resulting in patellar subluxation. Appropriate exercise frequently prevented the need for surgery. Swimming was an activity which the therapists assisted with. Most of the children learned to do this before they started school. It helped not only with strength, but also safety and improved body image. Group sports under the supervision of occupational therapists were fun and helped with self confidence. Such activities improved their sense of well-being and their attendance at school.
The role of the orthopaedic surgeon as a team member was new in Germany. Certainly with limb deficiencies the orthopedist was considered the team leader, but he had to learn the roles of all of the other team members and appreciate the importance of all of their contributions. A person with club hands, for example, would develop by himself independent recreation and activities of daily living, but if a hearing deficit was overlooked in time for optimal treatment the child?s future could be destroyed. This was indeed the case with some of the 261 persons having deafness following Thalidomide. The orthopedist needed to recognize this and make proper referrals. Many children had hip dysplasia (Table 1 ). Abduction splints were used initially which lead to avascular necrosis. Until this was recognized and the Pavlik Harness substituted, many children developed stiffness that was particularly detrimental to flexibility needed for independent foot use since they were missing arms. When hips were found that were complete teratologic dislocations, reductions were not performed. It was feared that each attempt would jeopardize hip flexibility and thus reduce the independence of an upper amelic person. On some occasions angulation osteotomies at a later age could improve leg position. The spine frequently developed degenerative disease later in life or the knees would become stiff, but rarely did hips that were left dislocated cause problems (Table 2 ).
Club hands remained a problem for the patient, the orthopedic surgeon, and the clinic team. Approximally 1500 Thalidomide patients had predominantly bilateral symmetrical radial club hands. Half had significant deficiencies of their humerus. Surgery is contraindicated in this situation because the radial deviation is necessary in the shortened limb for bimanual activity. Those with normal humeral length frequently have dysplastic elbows or shoulders. Experience taught us that they too did better without surgery and acquired adaptive techniques best without risking loss of motion due to post surgical stiffness. The little finger generally functioned as a thumb. If grasp is needed the little finger is always the first at the object and develops a wonderful function. Surgical corrections of the radial club hand is only indicated if the individual has normal elbow flexion.
In situations when it is uncertain whether surgery will benefit the function of a patient, a careful occupational therapy evaluation is needed. The ability to perform activities is evaluated with the hand as it normally is and with the hand splinted in the corrected position (Fig. 7a,b ). When a reduced position is functional I prefer not to do a complete arthrodesis. It is necessary to shorten the extremity because many of the tendons are severely shortened. The radius is fused to the proximal row of carpals in order to preserve midcarpal and carpalmetacarpal motion. Because of the reduced position of the hand, frequently the over-all length is no shorter than the original extremity (Fig. 8a,b ).
Patients with synostosis of the radius and ulna are corrected only if they have excessive pronation or supination. They are brought to a neural position. Pollicization procedures are frequently very helpful. Care must be taken when planning procedures for the lower extremities especially when the patients are lacking upper extremities. Frequently the individuals will not let you touch their feet because they realize the importance of them to their independence. Procedures such as amputation for longitudinal deficiency of the tibia should be avoided because of the functional importance of the feet. The same is true for those having PFFD. Club feet can sometimes be more functional left uncorrected than if stiff following surgery. Function as a hand must be balanced against function as a foot.
On December 18, 1970 a significant lawsuit was settled between the Gruuenthal Chemical Company and the West German government. Each was to make available one hundred million Deutsche Marks to establish a foundation, Hilfswerk fur behindeste Kinds (Help for the disabled child). This was relief work for the Thalidomide victims. Eligible people had to register using medical history, x-rays, and photographs and present them to a commission representing the medical profession and the parents organization. This offered an excellent opportunity for gathering data on all of the Thalidomide children.
The Foundation in cooperation with the Labor Market Research Institute looked at the professional and vocational success of this group of patients in 1987. Many individuals were still in school so some of the unemployment figures are misleading, but the Thalidomide victims fared well compared with the controls. Most entered professional fields; juris prudence, social or economic sciences. A higher percentage, thirty-seven versus twenty-nine in the general population, entered Universities. Fifty-four percent of the Thalidomide group are gainfully employed in a trade versus sixty-one percent of the general population. This is in spite of the fact that sixty-nine percent of the Thalidomide group are classified as 100% disabled.
Even though the majority of the Thalidomide population are severely disabled, one's profession cannot be the only important fact for the quality of life. Marriage, children, hobbies and special interests are important. There are many examples of excellent ways of life, but there are also some tragic circumstances. Those having severe limb deficiencies combined with deafness have frequently been found to have severe psychological problems. All of the victims are now beginning to fear secondary degenerative changes in their joints. They want to know how will these changes impact on daily life. This will be the challenge for the clinic team in the future. All of the Thalidomide victims who are now parents have had normal children. I end on this obvious optimistic note for the future.
Journal of the Association of Children's Prosthetic-Orthotic Clinics
Vol. 28, No. 2, Spring 1994