Classification of Lower Limb Deficiencies

Classification of the various types of limb deficiencies has always been difficult. In the past a combination of Greek and Latin has been used and words like dysmelia, phocomelia and amelia may appear to describe individual deficiencies; but these definitions tend to confuse rather than clarify.

It is now agreed by international standard ISO 8548/1 that there are two types of deficiency:

The Transverse in which the limb has developed normally to a particular level, beyond which no skeletal elements exist though there may be digital buds. (This is sometimes erroneously referred to as a congenital amputation because of the resemblance to an amputation, but it is a failure of formation rather than a failure to persist).

Such segments are described by naming the segment at which the limb terminates and then describing the level within the segment beyond which no skeletal elements exist eg. 'Transverse thigh, upper third' means the thigh is present down to the upper third section.

The Longitudinal in which there is a reduction or absence of an element or elements within the long axis of the limb. There may be skeletal elements distal to the affected bones or bone.

Affected bones are named in sequence starting from the bone nearest to the trunk of the body. Any bone not named is present and of normal form. Affected bones are described as totally or partially absent and in the case of partial deficiencies the approximate fraction and position of the absent part is stated. The number of digits (toes) are stated, starting from the big toe, sometimes these are referred to as rays, eg. 'Longitudinal, fibula total, tarsus partial, rays 4 & 5 total', means that there is no fibula, the ankle bone is abnormal and the two outer rays (metatarsals and toes) of the foot are missing.

The Birth of a Child With Lower Limb Deficiency

If you have given birth to a baby with a lower limb deficiency, you will know of the shock that you experience. My initial reaction at the time of Laura's birth was one of rejection. Grief is commonly felt; grief for the loss of the "normal" baby expected. Common feelings also include: depression, anxiety, isolation and a feeling of alternating guilt and anger.

Some parents ask "why us?", unable to understand why their baby is physically imperfect when the rest of the world seems full of perfect healthy children. Endlessly searching for an explanation of their role in events, parents can easily feel like victims. A mother expresses her painful feelings;

"I had no knowledge of reactions to birth, and it seemed as in some way I had rejected her because of her handicap."

Feelings are often contradictory and feelings of rejection and guilt were for me relatively short lived. As I began to know my child her condition became less predominant in my thoughts; although the shock, at the time of her birth, had been great.

Parents sometimes believe they are responsible for their baby's condition and question and examine every aspect of pregnancy. This search for an explanation is understandable for parents feel vulnerable and are desperate to comprehend. To make matters worse, doctors and other health professionals may ask pregnancy-related questions, in a sense confirming parents anxieties. However in the majority of cases, medical staff are genuinely unable to provide a suitable explanation.

The baby's (dis)ability dominates parents' thoughts at this time. Because of the complexity of the problem it is not unusual for parents to feel isolated and unable to communicate their fears and anxieties. A steps member voiced the question uppermost on every parent's mind,

"Will my child be able to walk and if so, how?"

An overwhelming need for good information and emotional support exists. Parents may well be under pressure from relatives as well as professionals to make decisions they feel ill equipped to reach. In such cases it may be constructive for them to share their thoughts and feelings with others who have experienced a similar occurrence in their lives. Early referral to a specialist centre is also desirable.

The shock and anxiety at the time of birth gradually recedes. Acceptance takes a little longer, but most parents are able to cope once they see possibilities of their child learning to walk. As M. Phillips & D. Duckworth say in their book "Children with Disabilities and Their Disabilities",

"As far as the child is concerned the most important factor in the child's early development is the families response to the adjustments that face it."

The Child's Perception

As the child develops, so too does the child's image of him/herself. Society is organised on the basis of a two legged, two armed, walking model. It is, therefore, inevitable that people who diverge from this model may well experience problems. The child's awareness of the condition is, to a large extent, rooted in the way the family deals with the situation.

Pre-school children only seem to notice their hard leg when they play with it, such as using it to hit a drum, or at the Disablement Services Centre when a new limb is being fitted. Few questions arise before children enter the school environment and become aware of others attitudes.

When the child begins to ask questions, honest, informative and sensitive responses should be offered. If the child is not ready to understand the answers then the questions would not have arisen in the first place.

Communication is vital and encouragement should always be given.

Siblings

Siblings should not be pushed aside to make way for a brother of sister who appears to make more demands than the normal arrival. Many emotions can be experienced by siblings. The range of emotions are obviously varied: jealousy and feelings of guilt are probably the most common.

Many issues have to be lived with and therefore understood. It is of great importance to explain the nature and cause of their siblings' (dis)ability brothers and sisters will be asked questions by other children and can help the affected child by giving an explanation to those who ask questions. My son often explains to people what has happened to his sister's leg, and this takes away some of the pressure that would otherwise completely lie with her.

If hospitalisation is deemed necessary, it is advisable to keep a special eye on the siblings who are left out of the parents main focus.

Adolescence

This phase may raise specific problems for the teenager. Physical appearance becomes especially important. Particular attention should be paid to a good cosmetic appearance by the professionals at the Disablement Services Centres.

Stigma and prejudice may also take on new dimensions, as the young person moves out of the school environment into either further education or employment. The individual's positive self image will help to provide a firm foundation and few problems persist.

Management of Lower Limb Deficiencies

None - Some deficiencies are so mild as to require no treatment or support. Minor leg length differences can be accepted at least for the first few years of life.

Orthotic Support
A sole and heel raise may be required to equalize the two leg lengths. 'This is acceptable if it is not so much as to make the shoe cumbersome, heavy and ugly. A specially made shoe may be needed.

A splint or calliper may be necessary with or without a special shoe.

An extension prosthesis is required when the leg is deformed and too short to be corrected by a shoe raise.

Surgical Correction
A single surgical procedure, or a programme of procedures may be required to correct a deformity, lengthen a leg or both. This programme can be combined with orthotic support between procedures. A close co-operation between the surgeon, the prosthetic specialist and the orthotist is necessary. Physiotherapy may also be needed.

Amputation and Prosthetic Replacement
Where amputation is advised, again close co-operation between the surgeon, the prosthetic specialist and the orthotist is essential to determine the time and level of amputation and to start rehabilitation on the artificial leg (prosthesis) as soon after surgery as possible.

The above considerations will have to be planned and explained carefully to the parents, preferably in joint consultative meetings with the various specialists in a Disablement Services Centre or hospital unit. It is advisable to anticipate the child's milestones so that a prosthesis or orthosis is provided when the child is ready to stand or walk, ie about the 9th or 10th month onwards.

We gratefully acknowledge the steps Medical Panel for their assistance in putting this information together.