Hello, as an adult with PFFD and after seeing many, many doctors and doing my own research I feel I have learned a great deal on the subject of Amputation, Van Nes Rotation, Limb Lengthening, and no surgical intervention for children born with PFFD, Coxa Vara and other conditions that result in a lower limb length difference. I admit that I do have some strong opinions on all these choices as many of us do but I will try and set these aside to try and provide some information on what these procedures involve, what the results may be, what the doctors are really saying, why they give you certain options, and what the doctors aren't telling you.
Before I begin let me say that I am not against amputees or prosthetics. My grandfather was an amputee. He had a below the elbow amputation due to a logging accident at work. He didn't use an "artificial arm" and was a remarkable man. His wife died giving birth to their eleventh child and he raised the family alone. My mother told me he could mix, shape, and bake homemade biscuits with just his one hand, something my mother still can't do, she needs both hands. He never thought of himself as handicapped and being the tall, strong man that he was, no one ever dared to treat him as "disabled". I admired him greatly and miss him terribly.
I, also, had a friend from college who was born with small flipper like appendages instead of legs. He wore heavy artificial legs that were held on by a belt around the waist. He would swing a hip up and forward to swing his "leg" out to take a step. I later learned that he had had feet and when they went into the prosthesis that he sort of sat in, it would hurt his feet since they were stuck inside the prosthesis and would cause ingrown toenails and other pain so he agreed to have his feet amputated to relieve the pain. I agreed that he made the right choice since he didn't have "legs" per say that he could walk with so his feet would never be usable, it didn't change how he got around and he didn't need to be in pain. Now lets begin...
WHAT IS AMPUTATION: Doctors consider amputation as a form of failure. If
a limb is damaged beyond repair or that doctors expertise in reconstruction
then the limb is amputated. If gangrene has set in or part of the limb is
dead then amputation is necessary. For some like my friend with severe birth
defects then amputation may be necessary to relieve pain and enhance mobility.
(Note: I will be talking only about lower limbs for the rest of this paper
unless otherwise stated.)
Doctors will admit that the best solution for the body's weight bearing for walking are the feet. The rest of the leg or hip was not designed to bear the body's weight in walking and that is where the problems can occur. Fortunately, there have been advances in prosthetics or artificial legs as they are more commonly known. Prosthetics can now closely imitate the flexing of the foot or the workings of the knee. We've all seen the T.V. specials or read the stories of amputees running marathons or climbing mountains and they are truly inspirational. However, you don't have their whole story and for a majority of amputees they can't afford those advanced prosthetics which can cost thousands of dollars. An amputee must also deal with pressure sores, watch for infections of the stump which could cause even more problems even further amputation, and deal with the stump changing shape due to changes in weight, muscle atrophy and so forth which means a costly refit of the prosthesis or more surgery.
AMPUTATION AND PFFD/HIP DEFORMITIES: This is a whole different ball game
in my opinion. Those amputees you read about or see on T.V. have had amputations
due to accident, illness or due to a defect of the foot or lower leg. The
difference is they have a "normal" hip and femur. With PFFD, especially,
you have a shortened femur which is usually angled incorrectly and usually
have a deformity with the hip and this will make one knee higher than the
other. This is the difference. Prosthetics work best with people who have
no defects of the hip or femur.
AMPUTATION IS COSMETICALLY BETTER: Chances are your definition of "cosmetically
better" is vastly different than your doctors definition. You're picturing
a nice shaped leg that looks like the real thing that your child will wear
all the time as if it was their real leg and will allow your child to walk
and run normally. Your doctor is saying, if your daughter wears long skirts
people will only see two feet on the ground, or if your son stands with long
pants on people will see two feet on the ground. People won't know there
is a prothesis so they won't stare as much when he/she walks with the limp
that comes with having PFFD or other hip problems. The point is your doctor
thinks that having one foot higher than the other is so traumatic on the
child and society that removing the foot is necessary to make your child
"look normal". The main reason doctors urge amputation is for "cosmetic"
reasons, not health reasons.
HOW MUCH TO AMPUTATE: Depending on the degree of the deformity of the limb,
especially the degree of shortening of the femur and the condition of the
knee and hip, doctors can offer options in how much to amputate. A Symes
amputation is removing a portion of the foot, leaving the heel for weight
bearing. That usually leaves the knee free to work normally. However, this
does not solve the problem of the hip or femur deformity or the difference
in the level of the knees. If one does decide to amputate a Symes amputation
might be the lesser of the evils.
If the knee isn't "normal" then some doctors fuse the knee which leaves the leg stiff and unable to bend or some doctors amputate above the knee. In an above-the-knee amputation a full leg prosthesis which is held on by a belt around the waist is used. A person mainly uses their hips in order to move their prosthesis since the remainder of the limb completely atrophies and is usually to small and weak to move the prosthesis. A former chief of orthopedics of a major American hospital once told me that when you amputate there is always total muscle atrophy. Also, remember if a person does amputate, there is a risk throughout life of pressure sores from weight bearing on the stump, or even further amputations being needed. Instead of asking how much to amputate you might consider asking "Are there other options?"
THE DRAWBACKS OF AMPUTATION: For a person with a shortened leg there are
benefits to keeping the foot. Most people can walk barefoot with a short
leg so in cases of emergencies such as a fire a person can escape without
looking for their prosthesis or other walking aids. Prosthetics cost a great
deal and government funds won't pay for prosthetics or braces for adults.
Many people with prosthetics only wear them when they must make an "acceptable"
appearance. Prosthetics are uncomfortable and can even be painful. Many don't
even bother with them and end up using crutches and/or a wheelchair. My friend
that I mentioned earlier only wore his "legs" to school and even then he
relied on a motorized wheelchair. He rarely walked in his prosthesis because
they were difficult to walk in. Sometimes a "knee" would unlock while he
was walking causing him to fall which if it didn't injure him physically,
it injured his pride.
In cases of PFFD or other birth defects amputation doesn't repair the hip, femur or knee to restore normal function. It increases the disability by adding a stump and the problems associated with amputation to the other problems and there is the loss of the use of what could be a working foot.
Amputation is usually offered while the child is still very young so that it's easier for them to accept their stump. The older a child becomes the more the child adapts to and accepts their shortened limb. They, also, are psychologically upset at the loss of their limb and the sight of their stump. Also, the older the patient becomes there is a greater chance the patient will refuse amputation. Remember that amputation can't be undone. It is permanent and will leave the person disabled for life. Amputation will never "fix" your child or make him/her "normal". Even just removing the foot will end any hope of possible reconstruction of the leg as medical science continues to improve in the field of orthopedics.
WHY DOCTORS OFFER AMPUTATION: Amputation for PFFD and shortened lower limb
conditions is now mainly offered only by American doctors. Other countries
use more advanced measures in orthopedic reconstruction and limb lengthening
in order to save the limb and restore use. In the U.S., doctors tend to reject
techniques that come from other countries. Doctors in the U.S. tend to want
to do what is the quickest and easiest solution for themselves. They are,
also, now driven by "hollywood" or society's idea of what is an acceptable
appearance. They seem to believe a platform shoe or a foot higher than the
other is to traumatic for the child, however, that simply isn't the case.
Doctors also know a child accepts and adjusts to a great deal which is why
doctors urge amputation as quickly as possible so that the child doesn't
miss their foot or leg. A prosthetic leg can give the "appearance" of what
is normal but only if the prosthetic is covered so that all that the "world"
can see are two shoes on the ground.
Also, many doctors simply aren't trained in the more advanced specialized techniques and tend to rely on the same decades old procedures. Finances, fear of malpractice lawsuits, and patient load are other factors, a quick amputation is less expensive, less complicated and time consuming than two or more operations and intensive physical therapy and a doctor has too many patients to want to spend the extra time to help just one patient. Simply put, doctors don't want to learn new procedures and it's easier and more convenient for them to amputate. Pride or greed can also keep them from sending their patient to a doctor who may be able to save the leg.
VAN NES ROTATION, THE SECOND OPTION: In the early 1990's I was in my early
twenties when a doctor first told me about this option. This procedure is
used usually as an option to amputation of the foot, however sometimes the
toes may be amputated for better fitting of the stump. The entire leg is
rotated backwards so that the foot is backwards. The foot is made to point
downward and will go into a prosthesis easier than if the foot was in the
natural forward position and sticking out of a prosthesis. Again, doctors
are considering the "cosmetic effect". The doctor showed me a photo of a
child with a Van Nes Rotation from a big, old medical book. I think the photo
was taken in the sixties or earlier. What he didn't tell me is that many
times the body derotates the leg in an order to go back to its natural position
and the surgery has to be done one or two more times. This usually happens
when Van Nes Rotation is done on a child. The leg can derotate because the
child is growing. Van Nes Rotation still does not correct the hip, femur
or knee deformity. This is an old, antiquated option for this condition,
in my opinion.
WHAT IS LIMB LENGTHENING: Limb lengthening was accidentally discovered in
1951 by a Russian physician, Dr. Ilizarov, for whom the procedure was named
for. He went on to further research and refine the technique and to teach
other physicians the procedure mainly in the European countries. Today, limb
lengthening is commonly done in European countries instead of the older options
of amputation and Van Nes rotation.
Limb lengthening involves in basic terms breaking the bones and attaching pins through the leg into the bone. The pins pull the bones apart a millimeter each day and the bone grows new bone to try and mend back together. It takes about a month to grow an inch. It is a time consuming and difficult process but when done by a specially trained physician a limb can be restored to equal length for normal use. The only drawback is a slightly stiff knee. When complete the patient has two equal and working legs for the rest of their life.
Walking aids such as a prosthesis, braces, platform shoes, crutches and wheelchairs are no longer necessary. As with any medical procedure there can be complications but a specially trained physician would be aware of these complications and know how to deal with them. This procedure is usually done in conjunction with reconstruction of the limb deformities of the hip, knee, or foot. The results could eliminate deterioration of the hip or other joints which could lead to the development of arthritis, back problems or hip or joint replacements later in life and can allow walking without a limp or perhaps only a slight limp.
WHY SOME DOCTORS ARE AGAINST LIMB LENGTHENING: There are many reasons for
this. One is prejudice against medical procedures from other countries. I'm
not kidding. One doctor told me "they" didn't believe in anything from "foreign
countries". To make things worse limb lengthening came from the former Soviet
Union, remember the cold war thing? Add to this the arrogance that American
medicine is superior because it's American. When the Ilizarov Method was
first discovered there was some excitement and doctors quickly began trying
the procedure in the 1960's without extensive training in the method. When
they didn't get the results they wanted, American doctors seemed to give
up on the technique and decided it didn't work. Limb lengthening is a difficult
and complicated procedure and when done by an unskilled doctor or untrained
in the procedure or in how to deal with the complications, the results can
be disastrous. Some doctors are too arrogant, full of pride, or jealous to
admit that another doctor can help their patient and they can't.
Doctors are human after all but, unfortunately, a few tend to think they are more superior than the average human. Some doctors may be prejudiced against the procedure. Another reason is they may believe that you or the child's particular case may have complications that the doctor can't surgically repair and if he can't fix it then it can't be fixed, right? They may also be too skeptical or unsure of the limb lengthening procedure that they won't refer the patient to a doctor experienced in limb lengthening. One important reason may be you as a parent or the patient. Many doctors will follow your lead in how you are dealing with the situation. It can depend on you. If you are demanding to know all of your options they will give you that, some will do so reluctantly. If you seem upset and can't deal with the situation, they will offer you the quickest and easiest solution possible. For example, in an unrelated situation, a doctor was giving a young boy who had very severe mental retardation a stronger dosage of seizure medication than was needed in order to keep him heavily sedated. The boys' mother was complaining to the doctor that she could never keep him awake. He finally told her that he was giving the child more medication than was necessary to keep him sedated because some parents wanted their child kept quiet and asleep. He assumed it would be easier on her if her child was kept sedated and that she would want it that way as well. By the way, judging by her raised voice, he assumed wrong. Doctors not only consider the patients needs, they, also, consider the family's needs as well and they sometimes make assumptions. The point is they could be following your lead on deciding what treatment they will offer.
Not all doctors are bad, like not all mechanics are bad. Some do wonderful work, care about the job and try to do what's best for the individual. Some try to get as much money as they can from you and fix it where you have to return again and again. Some simply do mediocre work because they don't really care about the job or you, and yes, some are bad and shouldn't be in business.
WHAT IF WE WAIT AND SEE: You are not a negligent parent or a negligent patient
if you want to wait and study your options or if you don't want them to operate,
after all this is something that will affect your child for the rest of their
life or your life if you are the patient. Not only are parents dealing with
the shock and grief of having a newborn with a condition resulting in limb
length difference but then they are bombarded by the doctors dire predictions
and bleak prognosis. It can be overwhelming and parents can make hurried
decisions out of fear and blinded with emotions of the moment before being
able to educate themselves and being able to weigh the alternatives.
Unfortunately, parents may make decisions that they may later regret. If there isn't a life threatening condition then there really is no rush to make any decisions. A good physician will not pressure you, use guilt, or scare tactics to push you into doing what THEY think is right for YOUR child or for you. If you feel uncomfortable or have an uneasy feeling in your gut then trust your instincts and find another doctor. If what they tell you doesn't make sense or feel right then find another doctor and another until you feel that what a doctor is offering makes sense and is in the best interest of of you as the patient or your child and your child's overall health for the rest of their life.
My parents and I have seen so many doctors that I don't even remember them all. Doctors told my parents I would never walk, they were positive of that and they were wrong! Walking, swimming, horseback riding were all things that not only I but many of us with a limb length difference can do and enjoy. Doctors can be wrong and can make mistakes. Many children with a limb length difference do walk perhaps with only the aid of a platform shoe. Waiting can allow medical science to improve so that the old, drastic, and sometimes permanent, disabling measures of amputation or disfiguring the limb further won't be necessary. Waiting can also allow you the time to learn about the condition, research your options, talk to doctors, get referrals from others affected by this condition and see how your child progresses without surgery.
That's about it. These are just the main options for treating and dealing with lower limb difference conditions. There are some other less known procedures offered which also call for surgically disfiguring the limb in order to accommodate the use of a prosthesis.
Remember, I am not a doctor but I had a slightly annoyed doctor tell me I should have been since I knew about as much as he did about my condition, does that count? :-) I guess I tend to get pushy when it comes to finding help for my medical problems. It is my life after all, doctors don't have to live with my decision, I do. I hope this has helped answer a few of your questions and educate you a little bit.
I dedicate this paper first to my Lord, Jesus Christ who has loved me through all this and provided for all my needs. Secondly, to those affected by limb length difference and those who love them. Thirdly, to the doctors and researchers who do care and try their best to offer quality medical care. Lastly, to my parents who did the best they could with what they had and to the MCLLR who will save my leg and make all my waiting worthwhile.