Management of Congenital Deformities, Including Thalidomide Children, in Great Britain
MIROSLAW VITALI, M.D., Medical Officer
(The following report was originally presented as a lecture at the meeting of the Committee on Prosthetics Research and Development, Washington, D.C., March 28-30.)
Congenital deformities present one of the greatest and most tragic problems known to medicine. They affect the child bodily and create vast psychological difficulties for the parents. It has been noted that the standard of intelligence of the parents bears a direct relationship to their guilt feelings. The lower the standard, the more guilty they feel in having had a deformed child. The parents suffer a severe psychological shock for which they are totally unprepared.
Consideration of the parents in this situation cannot be overemphasized. They require complete understanding, assistance, explanation and encouragement. Each year a number of children are born with congenital deformities of unknown causation and these have now been compounded by the addition of the "thalidomide children". In all cases it seems appropriate to approach the problem first from the viewpoint of the parents, and then from the standpoint of the children.
Some recent statistics received from the Ministry of Health are in order here before resuming my discussion of the parents. The figures relate to the years 1960 through August, 1962, by which time it is believed that all of the babies affected by the drug thalidomide were already born. A total of 805 were born during this period. One hundred and fifty-three of these children subsequently died, including 85 cases where it was definitely established that the mother took thalidomide during pregnancy. A careful investigation revealed that there are now 302 children living who may have been affected by thalidomide. Details of the type of limb deformities are as follows:
There are certain deformities known to be attributable to thalidomide and clinically described as the thalidomide syndrome. These are:
Face: Haemangioma, which is central over forehead (sometimes eyebrows) extending down the nose to the upper lip. Saddle nose. Rarely seen: Coloboma; microtia and facial palsy; absent eyes; cleft palate; tongue-tied. Delay in cutting teeth.
Limbs: Phocomelia and/or amelia. Usually, the phocomelia is reasonably symmetrical in length although one side is sometimes slightly more developed than the other. Upper limbs are more commonly affected. Lower limbs are rarely affected on their own.
The majority of the "thalidomide children" are obviously intelligent. This is a helpful factor since it will assist them to overcome their disabilities.
The long bones are generally absent, leaving the hands directly attached tothe shoulders and having four fingers or rudimentary thumbs. In partial phocomelias of the upper limbs the defect is usually an absent radius, or on the radial side. Both sides are usually affected but one hand can be longer than the other, or have more or fewer fingers.
Complete absence of the lower limbs is rare, but sometimes major defects of both lower limbs are noted. The statistical table offers a complete, detailed picture which is self-explanatory and, since our knowledge and understanding of the subject is admittedly limited, we can only accept the situation as it is presented.
I would like to point out, however, that treatment of congenital deformities, including the thalidomide type, was known before the problem represented by thalidomide was introduced. Since a causative factor has now been recognized, it is even more important to utilize every possible means to reduce the anguish of the parents and to treat the children to the best of our ability.
At this point I return to my earlier discussion concerning the parents. Teamwork is extremely important in the treatment of the children, and the foremost member of the team is the mother, who will spend most of her time with the baby. During the first weeks afterbirth, we prefer to see the children with their parents in order to assess the existing deformity or deformities and to establish a plan of action for the near future. My first visit with the parents presents a typical pattern. The parent or parents are distressed and their eyes appear to follow my every movement, expression and action. It is my feeling that the initial visit and my attitude towards the child at that time may influence the entire result of future "habilitation" and, even more, the future of the child.
I make it a point to examine the child as I would any other infant and make certain that my staff and I act in a kind and perfectly normal way toward thechild. It is important to the mother to observe that her child, regardless of his deformities, is treated the same as any other child. We have found that this may well be the turning point in the mother?s attitude toward the entire situation. We then explain our plan of action in "habilitation" to the mother, and show her other children with similar deformities under treatment.
In many cases, the child will require exercises to ameliorate the deformity and improve the alignment of the limb or limbs. A trained physiotherapist should demonstrate to the mother how to perform the necessary manipulations, and she should continue them at home. From that point on, the mother becomes an active, not a passive, member of the team, and she will observe and appreciate every improvement resulting from the treatment of her child. This, of course, is very significant from the psychological point of view.
In some cases, because of the strong emotions involved in the situation, the parents can be convinced that their love may serve as a catalyst in the future rehabilitation of the child. They should be observed and guided to assure that the necessary assistance they can render is forthcoming.
There are normal milestones in the development of a child with no deformities and one cannot expect a deformed child to achieve standards of development before his or her age group. This is so obvious that I would not have made reference to it but for the fact that it is often forgotten by the parents and others.
There are definite rules which should be observed in dealing with congenital deformities, and I have listed them hronologically:
- Surgery is not advised unless we find that we are completely unable to fit the child.
- Do not amputate any member hastily because, in later years, you may alter your decision about the level or even the necessity for amputation.
- Any segment of the body left by nature may be utilized later as a source of power, i.e., to press buttons in pneumatic arms or to pull the cords in prostheses.
- Attempt to fit the child by all means, provided that it can move and grow in the right alignment, and that you are not creating additional deformities such as scoliosis, etc.
- Always think, plan and calculate on the premise that what you give to a child should be more than what you take away.
- The child may lead a double life in the future-one at home and one in the outside world. Do not discourage an armless child from using his feet as a replacement for arms and hands-a function which is natural and effortless. Children must learn the feeling of texture, weight, temperature, etc., which an artificial arm will not provide.
- If you are pressed, usually by the parents, for an amputation or partial amputation of a deformed limb, attempt to persuade them that you would like the child, when he is older, to take an active part in the final decision about amputation. (An example of this is a girl with a congenital deformity who was fitted satisfactorily as a child. Later, when she married, she requested amputation of her rudimentary foot.)
These seven points should not be regarded as dogma. The door should not be closed to reconstructive surgery such as elongation of stumps; reconstruction of the thumb to ensure better apposition and grip; producing lobster claws from a partially malformed hand; or even cineplastic surgery.
It is possible that a good functional result may be achieved but the outcome not be psychologically acceptable to the patient. This is especially true in the case of a young woman who is motivated by cosmetic considerations. (A Kruckenberg amputation is an example of a surgical measure which may not be acceptable to a young woman.)
Each child should be seen at the Limb Centre on a regular three-month basis so that the original limb may be altered according to anatomical changes. The child presents a happy picture when he has been fitted properly, is able to undertake activities and play with other children. The question of education is also very significant. Unless a child is severely handicapped, every effort should be made to have him attend normal school and mingle with other chil-dren. In this regard, I again refer to the fact that the majority of the children are intelligent.
A discussion of the prosthetic management of congenital deformities in children is also in order. I will divide the deformities in to two groups, upper and lower limbs:
Upper Limbs: The first manual function of an infant is to grasp large objects between his hands and forearms. It is obvious that an armless child is unable to perform this simple action and it is advisable to provide a simple prosthesis to achieve the function and ensure that cortical control will develop accordingly. The simplest type of prosthesis is a shoulder cap with a fixed upper and lower arm piece flexed at about 1200 to achieve better grasp. There would be soft dome ends rather than a hand attachment. When the child changes to a sitting period of life, we have found that it is desirable to fit a simple elbow joint operated by a physiotherapist or the parents. The next stage is an artificial arm with a split hook which could be removed and replaced by an artificial hand. An intelligent child can use a split hook as early as six months of age. This type of artificial arm may later change to a more complicated mechanism, usually at the school age.
Lower Limbs: The lower limbs are generally provided later than the arms as it is an obvious natural development of a child. At the time of sitting stage, a special plaster cast can be made to assist the child to sit in an upright position. This is followed at the later stage, usually corresponding with natural demand, by a pair of short rocker pylons in case of a bilateral deformity of the limbs. The rockers help the child to move and also correct malalignment of the deformed limbs for later fittings. The rockers are later replaced by artificial limbs, usually platform ones, to use natural weight bearing. Every possible effort is made to correct the deformity and to give a natural-looking appearance to the artificial leg.
Ministry of Health Limb Fitting Centre Roehampton, London
Vol. 28, No. 2, Spring 1994
Journal of the Association of Children?s Prosthetic-Orthotic Clinics