Jennifer's Story

pffdvsg-mike — Mon, 29 Sep 2003 00:34:00 +0000

Jennifer's Story

Location: Ohio
Born: 1993
Diagnosis: Unilateral PFFD, left leg
Treatment: Foot amputation, knee fusion, prosthesis
Told by: Dad with notes from Mom
Date written: 1/22/97 - 5/15/97

In the beginning.....

Our lovely little daughter was born by C-section in 1993. Like all babies,
she was a doll. Our first hint of a problem was when the doctor told the
nurse to save the placenta for genetic tests. Jennifer went to the nursery,
mom went to recovery, and dad went to the telephone. A little while later
the obstetrician came to the recovery room and told us she was fine, BUT
there appeared to be a problem with her left hip. He called the pediatrician
and would let him check further. The pediatrician didn't know what the problem
was, so he called in an orthopaedic surgeon. The ortho surgeon came in the
next day and said he thought our baby had PFFD (the first time we heard the
term that has now become so familiar). He wasn't sure what the treatment
would be - possibly a prosthesis. Surely he couldn't mean an artificial leg?
He must mean some kind of brace while they lengthen her femur. (Denial is
a great way to make things easier to bear.) He said he had never seen or
treated a PFFD case, so he referred us to a pediatric orthopaedic surgeon.

For the next 6 weeks we just enjoyed our new baby and didn't worry too much
about her leg. She has many years ahead of her, so no sense rushing off to
the doctor - they probably wouldn't do anything until she got older anyway.
But, there always was that nagging doubt about what could they do for our
little girl....

...The first dark days

We made our first trip to a pediatric orthopaedic surgeon when Jennifer was
about 6 weeks old. He did a very thorough examination and took several X-rays.
He explained PFFD by describing each word so that it no longer seemed like
just a bizarre acronym. He said that not only was the femur short, the ball
at the top of the femur and the hip socket were also not fully developed.
He also said that in most cases the knee joint is also missing some of the
proper pieces (missing tendons, muscles, etc.). Then came the REALLY bad
news. He said the usual treatment is to amputate the foot, fuse the knee,
possibly fuse the hip, and fit her with a prosthesis. What!?! Amputate a
perfectly good foot!?! Can't we lengthen the femur? He said that if the femur
is only slightly short, they might have success with lengthening. In Jennifer's
case, he said the femur was very short, and any lengthening attempts would
probably result in a very weak bone that would be susceptible to many fractures.
He said that past clinical studies found a very low success rate with
lengthening. He suggested we think this over and call him with our questions.
(Later he would tell us that he knew that after he said amputate that we
didn't hear another word he said. How very true.)

We left his office in a state of shock. Up to this point we were sure that
Jennifer's leg could be lengthened. Now we had to face the terrible possibility
that she would have to wear an artificial leg for the rest of her life. God,
this can't be true! Why us? Why our poor little girl? She didn't do anything
to deserve this terrible fate. We just sat there in the parking lot and cried.

On the way home, the tears gradually changed to anger and denial. This doctor
doesn't know what he's talking about - he probably doesn't know enough about
PFFD. We'll search the country (or even the globe for that matter) to find
the world's greatest PFFD doctor. After all, this is our little girl we'll
talking about here. We'll do whatever it takes to get her the best treatment
available.

The fruitless search for info

Remember, 1993 was the early days of the World Wide Web and Mosaic (Mosaic
arrived in Sep 93). I didn't have a home PC, and at work we just had an old
DOS machine with no internet access. An uncle in California had internet
access. He did a Medline search and found 8 items. I took that list to the
library at a medical university and was only able to find one of the journals.
It described the Van Nes Rotation Procedure. I read it twice before I realized
it was describing turning the foot around and using the ankle as a knee joint.
This was my first experience reading a medical journal. Whew, what a rude
awakening. Lots of terms I didn't understand. Hopefully ,someday, someone
will write about PFFD in plain english.

A second and third opinion

We were still in shock, and couldn't believe we would have to amputate a
perfectly good foot. We sought a second opinion from another pediatric
orthopaedic surgeon in our area. He gave us the same prognosis. The grim
truth was starting to set in. An aunt in Pennsylvania did some research at
the university where she worked. She discovered that there was an experienced
PFFD doctor (Chief of Orthopaedic Staff) at Children's Hospital in Pittsburgh.
We made a trip to Pittsburgh in May of 1996. Same prognosis. Looks like we
have to face the truth now. How can I bring myself to schedule an appointment
for them to cut off my little girl's foot?

OK, we faced the truth, now what happens?

Now that we've heard the same prognosis from three doctors, it's time to
face the truth and get more information. We went back to the first doctor
to ask lots of questions.

Do we absolutely have to amputate the foot?

Yes. Since her left foot is just below the right knee, the amount of femur
lengthening required is too great. The femur would probably be too weak and
be susceptible to many fractures. Also, her knee joint appears to be missing
pieces and would also be very weak.

Fitting a prosthesis over the foot would be difficult, but it could be done.

Van Nes Rotation could be done. Turn the ankle and foot around and use the
ankle as the knee joint. This procedure usually de-rotates over time and
would probably need future surgeries too realign. Also, more importantly,
cosmetics is a big problem. Imagine a backwards foot at knee heighth.

Best course is to remove foot so that she can be fitted for a prosthesis.
Difficult and painful decision, but "a foot does you no good unless you
can get it on the floor and use it." (I would tell myself this many times
as we approached the surgery date.)

What exactly will be done while in surgery?

The foot will be amputated, and a metal rod will be pushed up through the
heel to hold the ankle immobile until the ankle fuses.

When would we amputate the foot?

No need to be in a hurry. This is usually done between 10 and 14 months.

How much would be removed?

The procedure would be a Symes or Boyd amputation. Only the front part of
the foot would be removed. The heel pad will be left as a good cushioning
surface for when she wants to crawl without the prosthesis.

Will she be in a cast?

Well sort of. Not really a cast, but a rigid sleeve to protect the incision
and to hold the ankle until the fusion process is complete. Probably be for
about 6-8 weeks.

Enter one Guardian Angel

Just as we finished asking all our questions, the doctor tells us that his
receptionist has an 18 year old son who has unilateral PFFD. Would we like
to talk to her? Sure - maybe she can help allay some of our fears. As he
went to get her, my wife and I discuss what are the odds of this happening.
We had never even heard of PFFD and now our doctor has a receptionist with
a son with PFFD. Maybe somebody upstairs is looking out for us afterall.

A woman entered the room and introduced herself as Donna. I surely didn't
know where to start and felt kind of awkward, but she was very friendly and
gradually got us talking. She seemed to know what our concerns and fears
were, and we eventually ended up talking about everything she experienced
with her son Frank. She experienced the same things - what is this PFFD?,
why me?, isn't there another way? Frank had the same course of treatment
they were proposing for Jennifer. He even had a hip surgery. She assured
us that all went well and Frank is a very active High School student. He
plays on the basketball team, wrestles, and even was on the homecoming court.
It was a major relief to know that our little girl will probably be able
to lead a fairly normal life despite this PFFD handicap.

Praying for a miracle

Ok, we have the answers, we've decided to do the foot amputation and knee
fusion, we've picked a surgery date near her first birthday, now let's hurry
up and wait. Picking a course of treatment was a very hard decision and it
was a relief to finally have a decision. We now had 3 to 4 months to wait
until the surgery date. Our main goal during this time was to just enjoy
our little girl and try to put the surgery in the back of our mind. Problem
was that sometimes we'd lie in bed at night and worry about the future...and
wonder if we were doing the right thing. My wife had her mind made up, but
I was praying for a miracle. God please make her leg grow so that we don't
have to amputate her foot - just a little growth so that we can try limb
lengthening. I caught myself many times during diaper changes straightening
her leg out and comparing it to her good leg to see if just maybe it had
grown a bit. Please God...just a small sign before it's too late.

God never gives us more than we can handle

One of the things that helped keep us going during these tough days was a
phrase our priest uses whenever something awful happens. He says "God never
gives us more than we can handle". I know there were many days when I thought
"I can't handle this", but then I'd think about that phrase and I'd say to
myself "if God didn't think I could handle this, he wouldn't have given it
to me". I know lots of folks will think this is corny, but it worked for
us and that's the most important thing. Use whatever tools you have to get
through these tough times.

The really dark days

The last couple weeks before the surgery were probably some of the darkest
days of my life. I still didn't know how I would get the strength to take
may little girl to the hospital so that they could cut off her foot. I'd
lie in bed some nights and just cry for her and some of the things she'll
never be able to do. Oh, I know we're not supposed to think like that. Set
no limits and let her do whatever she wants, and yes she'll probably do most
things. But, we have to face the truth too. Some things are just very hard
to do with an artificial leg - ride a tricycle or bicycle? Climb the jungle
gym with all the other kids? Run to first base? Jump rope? The list could
go on and on...I kept trying to put these thoughts aside, but as the surgery
date got closer I kept thinking about them even more. Are we sure we're doing
the right thing? Once they cut her foot off there's no turning back. Maybe
we should wait until she's older to see if her leg grows. I wish there was
some way we could be absolutely sure that this is the right thing to do.

The first surgery

The fateful day came and off we went to the hospital. I still had some doubts,
but no turning back now. The doctor met us in the waiting area and asked
us if we had any more questions. He did his best to assure us that all would
be fine. A nurse came and said that it was time for her to take Jennifer.
Oh please, not already!. We kissed our little girl goodbye and stood there
with tears in our eyes as our little girl went down the hall and around the
corner out of sight. Are we doing the right thing? I reassured myself "a
foot does you no good unless you can get it on the floor and use it." I sure
hope we're right.

The surgery took about 2 hours. The doctor came out and said everything went
very well and she was in recovery. About an hour later, the receptionist
said we could go in to see her. My first reaction was 'oh, the poor thing'.
I think everybody usually looks pretty awful after surgery, but it's alot
worse when it's your little baby. She just looked so tired and uncomfortable.
Her leg was wrapped in a big sleeve with an ace bandage wrapped around it.
God, it's just not fair that little kids have to go through this ordeal.

We spent the night in the hospital. Well actually, Jennifer and my wife did.
I stayed at the Ronald McDonald house next door. A great place and I strongly
recommend it to anyone who has kids spending the night in the hospital.

I seem to recall that the recovery process was fairly easy. I remember being
hesitant about our trip to the doctor for the first checkup. I wasn't sure
if I'd be able to watch as they took the sleeve off. Remember, I had never
seen a stump before, and I guess I had a horrible looking vision in my mind.
As they took the sleeve off I could see that the leg was wrapped in gauze
which was soaked with dried blood in the incision area. Ooh, maybe this is
going to be pretty bad. The gauze came off and revealed an incision with
stitches. Much to my surprise and relief, it wasn't as bad as I had envisioned
and the sight of my daughter without a foot didn't really disturb me. Whew,
what a relief! The wire sticking out of the heel was sort of gross, but I
knew that would be coming out soon. Everything looked ok, so the gauze, sleeve,
and bandage went back on until the next checkup. Back at home, as Jennifer
started to feel better, she started crawling around even with the sleeve
on. It didn't seem to bother her too much. The stitches were removed at about
3 weeks, and the wire and sleeve were removed at 6 weeks. Once the sleeve
was off, Jennifer still complained that the leg was sensitive. We had to
keep it wrapped in an ace bandage for several weeks. She also refused to
take a bath. She said the water hurt her leg. She was afraid to get the scab
wet and afraid to get water on the small hole in her heel where the wire
had been. No amount of coaxing could get her in the tub. Finally after many
frustrating weeks, the scab came off and we finally got her into the tub.
Hooray - one ordeal done and over with!

Our first prosthesis

On the day the sleeve was removed, we took her for her first prosthetic
fitting.We had talked to Dave the prosthetist before the surgery, but we
never got any details about the steps involved. The first step was molding
a plaster cast around her leg. Imagine trying to put a cast on a wiggly,
screaming 1 year old - not fun. Jennifer wasn't very happy, especially when
it came to step 2 - cutting the cast off. Poor Dave sure didn't make friends
with her that day. Dave took several measurements and said he would use the
plaster cast as a mold to make the prosthesis. He said it should be ready
in a few weeks.

The exciting day came when we finally got her first leg. I don't know who
was more excited - Jennifer or mommy and daddy. Dave brought out the leg
and I must admit that I was disappointed. I had visions of a nice natural
looking flesh colored leg. Instead, it was a white plastic socket with a
wooden foot. It had a small plastic door with a hinge made of brown webbing
which was attached to the plastic sleeve with rivets. A velcro strap held
the door closed. Definitely not very appealing looking, but I guess function
is the key.

Dave showed us how to put it on. First, 2 or 3 socks, then a brown fiber
type sleeve, then the leg, and finally close the door and secure it with
the velcro. Dave had to make some minor adjustments - adjust the length,
add some padding here, take some padding off there. Finally we were on our
way.

Our first problem appeared a day or two later. We could not get the prosthesis
all the way on. No matter what we did there always seemed to be a gap between
the end of her stump and the bottom of the leg socket. Back to Dave we went.
He almost died laughing. This prosthesis is not an end bearing type and the
leg is not supposed to touch the bottom of the socket. Boy did we feel stupid.
The point here is to ask lots of questions before you leave the prosthetist's
office. If we had we could have saved ourselves alot of time and trouble.

Our next problem lasted for several weeks. Jennifer just plain refused to
wear her leg. Sometimes she would put it on for brief periods, but would
never try walking. Finally one day when she was about 18 months old, the
babysitter got her to put it on and she took her first steps. Oh what a happy
day!!!

The next year and half were typical for a growing child - with a few exceptions.
I think the learning to walk process was probably very similar to what
you would experience with a 'normal' child. Jennifer seemed to adjust to
the prosthesis pretty well. Still had times when she wouldn't wear it, and
lots of times she preferred to just leave her leg off and crawl around the
house. At times it was very frustrating because she can be a stubborn little
girl who does what she wants when she wants.We just tried to encourage (and
cajole), but the final decision was always up to her. We had our share of
fitting and adjustment problems. Sometimes 2 socks were needed, other times
1 was enough. I think we probably made at least 4 or 5 trips back to Dave
for minor adjustments and lengthening.

The second (and hopefully final) surgery

The knee fusion surgery was scheduled for October 5, 1995 when Jennifer was
2 1/2. This surgery will fuse the femur to the fibia/tibia and shut down
the growth plate at the bottom end of the femur. The goal of this surgery
is to create a long single bone which will act as a long lever arm to fit
inside the prosthesis. The growth plate is shut down so that eventually the
stump will be above the other knee so that the prosthesis can have a knee
joint level with the other knee. The biggest problem is that she'll have
to be in a lower body cast for 3-4 months. OUCH! How will an active 2 yr
old accept being immobile for 3 months? We're definitely not looking forward
to this. We're much more comfortable with this surgery than the first one,
but this body cast sounds like a nightmare. How do we change diapers? How
will she ride in her car seat? How do we bath her? I guess we'll learn as
we go.

As we approached the surgery we recalled how much we hated watching a stranger
carry our little girl off to the operating room. Somebody told us that other
hospitals allow a parent to accompany the child into the OR until they go
to sleep - excellent idea! Surgery day came and the asst anesthesiologist
came out to ask the typical questions. We told him we wanted to accompany
her into OR. He said he'd see what he could do. A short time later, the chief
anesthesiologist came out and said that they normally don't allow it due
to some past problems, but if we wanted to he would allow it. My wife suited
up in scrubs, I kissed Jennifer goodbye, and off they went. It was definitely
much easier watching her head off to surgery in the arms of my wife rather
than in the arms of a stranger. My wife came out about 10 minutes later and
said she hugged Jennifer until they put the mask on her and put her to sleep.
She said it was tough watching her own child be put to sleep and she would
not look forward to having to do it again.

The surgery lasted about 3 hours. Halfway through, a nurse came out and said
everything was going fine and asked us what color cast we wanted. Blue it
is. A short while later the doctor came out and said everything went fine
and the surgery team was finishing up with the cast. He said they tried to
hold the knee stable by pushing a rod up through the heel and tibia and through
the knee joint and into the femur. (OUCH!) He said the bones were too soft
and the rod wouldn't stay, so instead they put two rods criss-crossed across
the knee joint. These rods would be removed when the cast came off. When
we finally got to see her in recovery she looked terrible, but not as bad
as after the first surgery. We spent the night and got to go home the next
day.

Pain didn't seem to be too bad. She had a pain killer for about the first
4-5 days, but never complained about very much pain.

Life with a lower body cast

The cast was made of blue fiberglass with internal padding and a 'Tyvek'
type liner material. It completely enclosed her left leg and extended down
to just above the knee on the right leg. At the top it went to just above
her belly button. The legs were fixed in a sitting position and spread open.
The groin area was open so that we could change diapers - had to just kind
of stuff the diapers in and push them up in the front and the back. No real
way to get in there to fasten them.

Our first problem showed up when we went to get into the car for the trip
home. Lower body casts don't fit into child car seats. My wife ended up holding
Jennifer on a pillow on her lap in the back seat. Illegal for sure, but I
didn't see any better way. Did it that way for the entire time that she had
the cast.

Problem two was a cleaning problem. Since it was difficult to get the diapers
on properly, we frequently had leaks, especially at night. The inside of
the cast would get soaking wet, even though the liner material was supposed
to be waterproof. It even got soaked to the point that the inside plaster/padding
became mushy and soggy. We came up with the idea of using a hair dryer to
dry the inside of the cast. It dried it well, but it left a urine smell behind.
My wife did her best to try to clean the inside, but it quickly became discolored
and smelly and by the time it came off it looked pretty disgusting.

Entertaining a 2 year old who is immobile in a lower body cast wasn't as
bad as we feared. Luckily for all of us, Jennifer always was the calm, reserved
type child who leans towards games, puzzles, coloring, TV watching, etc.
Most of these activities were not restrained by the cast. Some of them required
strange sitting positions with pillows as props, but generally she did OK.
I'm sure she missed being able to crawl around, but she wasn't too cranky.

The stinky cast finally comes off (in surgery again!!!)

After only 6 weeks the doctor gave us the great news that the cast could
come off and be replaced by a small leg cast. The bad news was that she would
have to go into surgery to have this done. Oh boy - here we go again. These
'nothing to eat or drink after midnight' ordeals are getting to be a pain.
At least this surgery was brief and we got to come home the same day. The
prosthetist took a cast mold of her leg while she was out during the surgery,
so that was one less ordeal to deal with later on. Plus, now her new leg
would be ready and waiting for her when she got the leg cast off. We were
all very happy to be rid of that stinky body cast. Jennifer was now able
to crawl around and life returned to fairly normal. The leg cast was on for
about 6 weeks, so the total time with the two casts was about 12 weeks.

The NEW leg

The surgery timing was ideal because she had just about outgrown her first
leg and was due for a new one. She got her new leg about 2 weeks after the
cast came off. The new leg was basically the same as the first one - plastic
socket and plastic door with web hinge and velcro strap latch. One change
was the foot was now soft, rubbery feeling and tan colored. Another change
was that we no longer had the separate sleeve - this sleeve is permanently
attached to the inside of the plastic socket. One big addition is a strap
that goes around her waist to help pull the leg inward and apparently try
to offer some hip support. The belt is a 5/8 inch wide nylon strap with velcro
on one end. It is riveted to the back of the socket, wraps around her waist,
slips into a D-ring riveted to the front of the socket, then folds back on
itself for the velcro latch. It generally seems to work pretty well, but
Jennifer can't put it on by herself and that is currently causing problems
as we try to get her potty trained. The doctor and the prosthetist seem to
think the strap can probably be removed, but Jennifer insists on keeping
it on.

Strap problems....and how to fix them with carpet tape

One problem that has nagged us since her first day with the waist strap is
that it tends to slide down off her hip and constantly has to be unhooked
and repositioned. We asked our prosthetist about this and he tried to attach
it higher on the socket but the problem still persisted. Finally, after a
year of frustration, I said we've got to come up with some way of keeping
that darn strap in place. The basic problem is that the nylon strap is too
slippery and slides on her underpants. The fix would be to make it less slippery
or maybe even sticky - EUREKA! Double stick carpet tape is sticky on both
sides! I cut a piece as wide as the strap and about 4-5 inches long and stuck
it to the strap at the spot where it rests on her hip bone. Works great -
no more sliding strap. It does tend to loose it's stickiness and peel off
the strap after a week or so, but it's very cheap and easily replaced. The
moral of this story is don't be afraid to improvise. Just because your
prosthetist doesn't know how to fix a problem doesn't mean you can't come
up with something that works. I'm sure others can share similar stories.

Some of God's greatest gifts are unanswered prayers

I can still vividly recall those long days and nights when I prayed for
a miracle. Oh God, please make her leg grow so that we won't have to
amputate her foot! Please just do this one thing for me and I'll never ask
for anything again. Well, the miracle didn't happen and we had to amputate
our little girl's foot. To this day, that is still one of the hardest things
I've ever had to do in my life. But, it was something that had to be done,
and now I'm glad that we did it. If I had gotten my miracle and saw some
leg growth, we'd still be dealing with a bulky prosthesis over her foot,
an unstable knee joint, and years of uncertainty praying for yet another
miracle - eventually probably ending up with the same course of treatment
but at a much older and more difficult age. Instead, today, all of our surgeries
are already behind us and Jennifer walks just like any other 4 year old.
Sure, she has problems with rough terrain and she can't run as fast as other
kids, but she walks quite well and her handicap is not a very big problem.
We fully expect that she'll lead a fairly normal life - with only minor
adjustments to deal with an artificial leg. I never thought I'd feel this
way - especially when I remember those dark days when we first learned of
her birth defect and that we would have to amputate her foot.

As I look back on it all now, I think of a song I heard by Garth Brooks.
He was singing about a high school love that never happened, but the words
ring very true for me in this experience too.

And each night I'd spend prayin' that God would make her mine

And if he'd only grant me this wish I wished back then

I'd never ask for anything again

Sometimes I thank God for unanswered prayers

Remember when you're talkin' to the man upstairs

That just because he doesn't answer doesn't mean he don't care

Some of God's greatest gifts are unanswered prayers

I guess the Lord knows what he's doin' after all

And as she walked away and I looked at my wife

And then and there I thanked the good Lord

For the gifts in my life

Children are great gifts and a child with a birth defect is an even more
special gift. Jennifer is our special little gift, and I wouldn't want to
change a thing about her. In fact, she just wouldn't seem like Jennifer if
she had two 'normal' legs. I just can't picture her any other way. We're
all looking forward to the future when she'll get a leg with a knee and ankle
joint which will allow her to walk even better and do more things.

...the happy ending....or is it really the happy beginning?

If you're reading this because you just found out that your child has PFFD,
I hope we were able to help and give you some encouragement. PFFD has caused
us our share of heartaches and sadness, but today is a happy day and I expect
the future will be even happier.

If you have questions or want to talk, just send an e-mail to Mike and Cindy
at pffdvsg@nls.net

pffdvsg-mike's blog

Jennifer's Story

Enter one Guardian Angel

The first surgery

Our first prosthesis

The second (and hopefully final) surgery

Life with a lower body cast

The stinky cast finally comes off (in surgery again!!!)

The NEW leg

Strap problems....and how to fix them with carpet tape

Some of God's greatest gifts are unanswered prayers

...the happy ending....or is it really the happy beginning?