PFFDvsg

Holly's Story



HOLLY'S STORY

This story is dedicated to our daughter Holly.

Somewhere over the rainbow
is a place where
a little girl has
two strong legs
that carry her as far as
she wants to run
and as high as
she wants to jump
Holly
we hope you
find that place someday



INTRODUCTION


This story has been 10 years in the making and may never be completed. That's because, as a parent, your child's story goes on for the rest of their life.


In our case, having a child born with PFFD thrust us into a world we would never have entered. This has been both exasperating and exhilarating at the same time. When we think about the experiences we might have missed, the places we would never have been, the people we would never have met and the inner strength we might never have gained it's almost as if you could crusade to promote it. But, in reality, it's not the ideal way to go about achieving those things.


Our story begins in 1987 in the comfort of our new home in a small town. We had done what a lot of people want to do, that is, escape the big city. Holly was born after an uneventful pregnancy and two ultrasounds which determined that everything was fine.


After the delivery, my husband and doctor approached me to tell me that something was wrong with Holly. I remember thinking at the time how helpless I felt still in the delivery room and how hushed the staff seemed. The doctor explained that they would do xrays as soon as possible and that he would get on the phone to a specialist. After that, my emotions were so up and down and I still remember the staff guardedly approaching and trying their best. One of the best things they did was send a volunteer in right away who told me about the Easter Seal Society and this was crucial, there was support from the very beginning. One of their nurses came to our home shortly after that and gave me all sorts of pamphlets and guided me through this new maze of support.


My husband had to return to work and the next morning the doctor came in with her xrays and told me he had contacted the famous Hugh MacMillan Medical Centre in Toronto where they had seen this particular problem and "treated" it. I was relieved, I didn't know the journey we were about to begin.



THE SPECIALIST


Since we'd moved to the country, all of a sudden here we were returning to the city more than ever for Holly's appointments. I remember with great clarity when the letter came to tell us our appointment date. It said to report to the Prosthetic Clinic at the Centre and I felt certain they had made a mistake! They had got Holly's problem mixed up with some other problem, prosthetics: how awful!


I really resented this whole business and I didn't want to be at the Centre. When we were approached on our first visit by a social worker I was aghast, what did that mean? Weren't social workers for the downtrodden and people who didn't have a clue? It was getting worse by the minute. Holly was six weeks old and I was still tired and upset by the whole ordeal. Family was supportive, but there was nothing they could do to change her leg.


Our first visit with the amputee team was really a genuine nightmare from our point of view. We were tired from the long trip in the car and it had been a long day before our turn came up. While we were waiting to see the doctor we asked to be shown some information and pictures about the "treatments" we had heard about. This was a big mistake in retrospect. I was feeling faint and cornered by this overload of facts and photos. The social worker commented that she saw my face go white when she mentioned "amputation", to which I responded that I "could never do that".


By now, Holly had spit up on the pretty dress I'd put her in to meet the doctor. This was not going well. After what seemed an eternity we were finally introduced to Dr. Ivan Krajbich and a team of prosthetists who were to become our friends of the future, although we had no idea about that at the time. It would have been intimidating for some but fortunately I was used to public speaking and it didn't bother me that we were at the front of a room being watched by all these people!


Doctor Krajbich alleviated some of our fears by telling us to take Holly home and enjoy her as a normal baby for a long while. He didn't need to see us again until she started to want to stand and this was a real comfort. I think it is this waiting time that saves us PFFD parents from going over the edge. We have time to adjust to the realities and time to think over any decision. This is what I tell all the "first-timers" I contact. ( In some cases, however, if the PFFD is quite severe, immediate surgery may be necessary.)



GETTING AN EXTENSION PROSTHESIS


Well, we had been through all the emotional part by the time one day I walked into Holly's room to find her standing and smiling in her crib! This was never supposed to happen (according to me) and she certainly wouldn't be happy about it! But she was, perserverance plus because she'd never known anything different than coping with two different-sized legs.


It was time to contact the Centre about getting her up and walking! Just to say: "Holly is going to walk!" was exciting. Her prosthetist spent a long day with us to get her first extension prosthesis right. Working with a 10 month old is a real challenge but Holly's gentle disposition was on our side. She was fast asleep by the end of it all, pooped out from all the attention. It took forever to fit everything just so and then we were off home.


I'll never know what she really thought of the whole process at the beginning, but after only a short time she was up in her walker and walking with help all over the place! One day, at around 18 months, we had brought home an exercycle from shopping. She wanted to touch it in the worst way but we kept holding her back not wanting her to touch it for safety reasons. She fussed and whined and pulled at my hand until I realized that if I let go she would walk by herself and not even realize it. And she did!!


Well, as a proud parent what would you do? Of course I called the media in to announce that our daughter had walked! They embraced the story and the timing couldn't have been better as the Easter Seal campaign was gearing up and we received all kinds of positive feedback from the community.



THE BIG DECISION


The dreaded "decision visit" was upon us. Holly was getting close to 3 years old and was scooting around in her extension leg and doing well. Pants were hard to fit over the bottom of the leg and she would get frustrated from her lack of range of mobility and she had quite a lurch as well.


By now we knew the routine with Dr. Krajbich and her prosthetist. We had sought a second opinion and agonized over lengthening her leg. We know from the reports that it was hard for the team to agree on a course of treatment and that they gave it just as much thought as we did. There was no turning back.


We had initially thought of the Van Nes rotationplasty as extreme and radical. The pictures we had seen were clinical and depressing. Nobody had any real answers. Nobody knew a parent to contact to talk about it. There was no Internet. I was against the Symes amputation because it involved removing a part of my child and I knew I would always wonder where her leg and perfect little foot had gone: in the hospital garbage? How could we remove something so perfect and just let it go like that?


So, for me and my husband, if we didn't want to remove something and lengthening was too agonizing and risky, we pumped ourselves up to become comfortable with choosing the Van Nes. In the meantime, we had met a wonderful family whose teenage son had had the Van Nes performed because of cancer. I think it really helped us see the possibilites with this surgery. This guy was a sports nut and was already back swimming and walking very well.


We told Dr. Krajbich and the team that we had decided. I don't know why, but at the last moment with the team I had to rush out of the room and cry. I think they understood, I just couldn't believe in my life that I had been asked to make this decision, even though I knew it was right and had to be done. I left my poor husband with Holly and a room full of people.



THE SURGERY


Here we were in July in Toronto in 90 degree heat on a long weekend. Holly's surgery was scheduled for first thing in the morning and we tried to tell her as best as you can tell a 3 year old that she wouldn't be so happy come morning. She was enjoying checking out the hospital, the nurses, the bed, her toys, etc.


We were in cramped quarters, but I won't go into the accomodation part of the story. I wanted to know what kind of weekend the doctor had had. I wanted to speak to him just before they took her away. I wanted out! He arrived jovial and fresh and it made me feel a whole lot better. He was obviously ready for the 4 hours ahead even if we weren't. And then she was gone.


At the hospital they have an excellent volunteer network. Throughout our hours in the waiting room they would update us on the progress of the surgery and were ready to help in any way. I truly believe that volunteers everywhere make the world go round!


And then they said we could see her for 5 mins in the recovery room. What an awful place, we had to wade through other children in various states until we saw Holly in a tiny bed at the back! And her eyes were open! We brought her a stuffed animal and she reached up and took it. I was speechless. Then I said out loud: "This is it, we've done it. I can't believe it."


And so began a gruelling journey of 10 days of hell at the hospital. The heat was terrible and Holly had fever from the anaesthetic and couldn't seem to get comfortable. Finally after about 2 days the doctor was concerned that she really wasn't herself, not the Holly he knew. I couldn't believe it when he said that she really shouldn't be in as much pain as she was. I thought, c'mon, you just turned her leg around, how would you feel?? They had us sign another consent form and this made me extremely upset, what if they had to amputate?, if there was a complication?, we had just signed her life away? So, they took her back to the OR, removed her cast and found that it had simply been too tight, and that she was healing well enough to just wrap in bandages. She was back in her room and smiling! The doctor was right!


That's not to say there weren't problems. She still had the fevers, but the worst was recovering from the anaesthetic again and nerve palsy in her foot and toes. This caused her no end of trouble and I had to hold her toes for her to go to sleep. She had lost the feeling in them and it was probably excruciating. They monitored this closely and the doctor was concerned. In the end, however, she has regained all the feeling as the nerves have healed over time. In some cases this does not happen.



GOING HOME


We had had enough after 10 days in the heat and couldn't wait to go home. Not that home would be any cooler, but it would be home! I sat in the back of the car with Holly's head in my lap and we didn't stop until we hit our driveway. I vaguely remember grocery shopping later that day and feeling like a zombie from lack of sleep and really not knowing what I was doing in the store. Scary! People I knew came up to me and talked to me but I don't remember what they said, I was in another world.


My husband ended up physically sick with a migraine from all the sleepless days and nights and tension. Our older son was going to be coming home the next day. Who is ever ready for all this?


Holly had a few temper tantrums before she realized she couldn't get all day and all night service at home, like at the hospital. You feel guilty getting angry with a kid after all she's been through, but it had to be done! Then we got into a good routine and she was a model patient! We devised many methods to cope with the little problems that came up, like toileting with a body cast on and underwear and sock fittings. That too is another story. She would scoot around the house with her walker, to which we had attached a bicycle basket so she could carry her things.


Fortunately I wasn't working outside the home all these years, somebody must have known it wasn't to be. I was able to spend a lot of time with both of our children this way. Our son took everything in stride, although we did take him to see Holly in the hospital one day and she was in considerable fuss. This made him cry and so we felt the best thing to do was have someone bring him back the very next day and see her doing much better, which seemed to work. She had a steady parade of visitors and probably a trunkful of presents we had to bring home. Everyone was most generous and it made for many different things she could do to occupy her time during recovery.


Her recovery was swift and simple. She didn't need much physical therapy as she was so athletic to begin with and dove right back into everything as soon as she could. After about 8 weeks she was fitted with and walked in her new Van Nes prosthesis in one day! The prosthetist was amazed and so was Holly as she had a "knee" that bent instead of having to move her whole leg forward. She was excellent at it! Dr. Krajbich's star patient! It was then we knew for sure that we had made the right choice!



RIGHT NOW


Over the years, there have been various crises with the leg and with dealing with the whole Van Nes idea. Not what you might think though.


With the leg it is the hinge putting holes in clothing that causes no end of annoyance! Holly can't really wear girls tights or the new leggings they have out as they get caught in the hinge. I have worked repeatedly with her prosthetist to come up with "hinge covers" but most Holly find too restrictive. However, I insist she wear one when she has on her expensive figure skating tights.


Oh yes, did I tell you that she figure skates? And she does the splits, and cartwheels and skipping and basketball and swimming and was on the school soccer team. There is no stopping her! After some hip surgery in 1993, you'd be hard pressed to see what was different about Holly, especially when she is wearing pants. She certainly has been an inspiration.


With the Van Nes, it has taken some getting used to, for everyone, especially upon first seeing the leg after the cast comes off. For Holly, her schoolmates knew from the start and small children are very accepting. As the years went by, with each new teacher and class, together we would do a presentation about her leg and I believe it helped put everyone at ease. It was also good for her confidence and communication skills. It made her feel special.


At the beach is the hardest. People will stare, but then we think a backwards leg is normal-looking now. We forget how frightful it must be for some.


And what about that athletic teenager we had seen with the Van Nes? This past summer, Holly came home one day to say she had seen a boy with a Van Nes. He was swimming at the resort we live next to. Guess who? Our families rekindled our friendship and caught up on 10 years. Sometimes it's a small world!


Time has made me forget some of the details I know I found very profound at the time they were happening! Here in Canada, in addition to Easter Seals, we have the War Amps of Canada and their CHAMP (Child Amputee) Program with a network of parents helping parents to cope, so if someone forgets a detail there is sure to be another to remember it. (Check out their website)



HERE TO HELP


I purposely did not go into heavy detail about the actual surgery because I am very prepared to help, one on one, those parents who may need to consider the Van Nes as their course of treatment. My husband and I have networked with many PFFD parents over the years and I know that it's best to absorb the information a little at a time. There are helpful hints specific to this surgery and everyone's hospital experience is different.


Most organizations have booklets and people available for you to talk to about what will happen next. Make sure you use those services! Isn't life just full of surprises.....


If you have questions or want to talk, just send an e-mail to Laurie and Steve at sdsmith@nexicom.net