Jahna’s Story: At-Knee Leg Disarticulation

At knee disarticulation is a procedure sometimes chosen to address a characteristic of tightly bent, fixated knee joints with webbing between calf and thigh. It may also sometimes be used to address straight leg knee joint fixation (when the knees cannot bend)  For considerations of this surgery, see this listing of reasons why it might be chosen or not chosen as an  option.

Here is a story of an adult with SA/CRS looking back on her experiences with at-knee disarticulation.

Jahna’s Story

This is just a peek into the life of an adult born with sacral agenesis/caudal regression syndrome. Mine. My experience. My opinions. I am not a doctor. I don’t know the specifics of you or your child’s medical history. I can only offer you a peek into my life as a person with SA/CRS who had at-knee leg disarticulation at age 4.

When I was born in 1975 my parents were told that I was 1 of 60 known children/ adults alive in the world who were born with sacral agenesis/caudal regression syndrome (That probably wasn’t accurate information). This began a journey for them, a blind journey of making important, and at times hard (almost impossible) decisions for me, that would impact me immediately, and, for the rest of my life. In 1975 there were no support groups for SA/CRS and no internet to surf. Even today most doctors have only read about this condition. Back then it was virtually unknown.

One of those major decisions made by my parents focused on my legs. When I was born my legs were crossed permanently in a “Buddha” or “frog like” position. They were extremely small, (my grandmother knit all my booties and socks for many years) and they had extremely poor circulation. I had feeling all the way down into my toes but I couldn’t move my legs or flex my feet or toes. Essentially my legs were nothing but dead weight.

As an infant, my parents had difficulty diapering me, putting on pants or tights, etc. It was difficult to get the pants up and over/between the crossed legs. They couldn’t imagine how I’d do it alone. Their ultimate goal raising me was for me to be 100% independent as an adult. As I got older mobility became an issue. I couldn’t quite figure out how to crawl forwards, initially I scooted backwards. Though, eventually, I did figure out how to crawl forwards while pulling my legs behind me.

This presented another set of problems. Rug burn on my knees and feet as well as wearing down of the knees of my pants and holes in my socks and shoes. I also ran the risk that if any sores occurred because of the rug burns it could take twice, sometimes three times as long to heal because of the poor circulation issue.

When I was 3 years old I was fitted for my first wheelchair. This presented yet another issue. Seating. The width of the chair had to be made wider because my legs stuck out to the sides. My parents realized that as I grew, so would the width of my legs and that would cause issues with proper fitting of my wheelchairs, seating on buses, planes, a car when I learned to drive, etc. They finally decided to consult a doctor about what they could do with/for my legs, and my future.

My parents first consulted a “specialist in SA/CRS” here in Michigan. He suggested amputating my legs at my hips, and when my mother refused outright he was less than professional in his treatment of her. She left his office in tears.

My parents then sought out a second opinion at Shriner/s Hospitals for Children in Chicago. Doctors there suggested going right up between my femur (upper leg bone) and my tibia and fibula (lower leg bones), keeping my femurs intact so that I’d have something to sit and balance on. I would keep the upper part of my leg, but the lower part below the knee would be removed.

My parents faced three choices:

  1. Do nothing and let me decide (or not) as an adult, and deal with any cuts, infections, seating and dressing issues, etc. that may occur over time.
  2. Disarticulate at the hips.
  3. Disarticulate at the knees.

In the end, my parents chose disarticulation at the knees. Before sending me into a surgery that could have affected the way I looked at myself and my body, my parents had me see a child psychologist so she could get to know me and my personality. On the off chance that I didn’t do well emotionally or mentally after the surgery they wanted someone to understand who I was beforehand to help guide me back to myself. She also taught my parents hypnosis techniques to use as a way of managing any pain.

My knee caps never grew in so it was an easy surgery. It took about 30 minutes and recovery was relatively easy with little to no pain afterwards. By all accounts the surgery itself had no impact on my personality or my mental and emotional health.

The choice whether to disarticulate (amputation without cutting of bone) your child’s, or even your own, legs, is a difficult one, and one filled with anxiety and apprehension. So many questions: “Is this the right decision for my child, or me”, “Will my child have negative feelings towards me when they grow up because I made this decision for them?”, “How will this impact their life in the future?”

These were questions my parents faced. I asked them how they felt at the time, they told me they had decided that it was the right decision for me, that, in their minds, it would impact me in nothing but positive ways, but yes, there was some apprehension. They were concerned that I’d be upset later in life. They were trusting that I’d understand that (to quote my mother) “the ends justified the means.” I’ve never questioned their choice. Not once in my entire life.

There are no real “right” or “wrong” answers to those questions. I know adults with “Buddha” legs that kept theirs and can’t imagine NOT having them. By comparison, I can’t imagine HAVING them. I have no memory of the surgery or even having legs before the surgery. I don’t miss my legs either. I’ve often told people if I was offered a new body I’d just take a “Jahna 2.0”. I’d be me, just with newer parts. I wouldn’t wish to be “typical”. This is me. This is what I know and who I am. I’ve never regretted the surgery, or that my parents chose to have it done for me. I’ve thanked my parents many times over the years for making this decision for me. It’s given me greater freedom, mobility and independence.

Because I had the surgery I had the opportunity to try artificial legs. Ultimately, they weren’t for me. I was slow, clumsy and it took three times the energy than hand walking or using my wheelchair. I was also 100% dependent on others to carry things, open doors, etc. I hated it. But, I had the chance to try them and decide for myself, which I preferred. I’m grateful for that chance, and for being allow to make that choice.

I am glad that walking wasn’t a goal that my parents had for me. For them, if I did walk, with the use of prosthetic legs, then great. If I chose a wheelchair or some other mode of transportation, then that was great too. To them it was all about quality of life and independence by whatever means. I think I would have thought and felt very differently had they pursued multiple surgeries to “fix” my legs so I could walk or look more “typical”. Their goals for me were happiness, independence and mobility in whatever way that happened to manifest itself. I feel like my wheelchair is an extension of me. It is my independence. It is my freedom. It is my legs. I’m not “bound” to it. I’m not “confined” to it. Without it, I would be bound or confined.

Because my parents ultimately chose disarticulation surgery for me, I can dress easily. My legs are better protected. My chair is the proper width for my shoulders. I adjusted to my new body seamlessly. I’ve traveled, and when I do, I fit easily in planes, trains and buses, I fit in a booster seat so I can drive. There are so many advantages to not having to accommodate the ever-growing width of “Buddha” legs.

Is this the right path for every child or adult who has “Buddha” legs? Maybe not. I can just tell you that in the years since my surgery I’ve never once regretted it. It’s one of the best things that ever happened to me. If I could, I would go back to 1979 and somehow tell my parents that it was all going to be OK. That I would adjust so well and that I’d have a wonderful life. Anything to save them the anxiety and apprehension that they went through making this one decision. I’d give them that peace of mind.