by
Peter Helson
phelson@ozemail.com.au
Note from PFFDvsg Webmaster: Peter originally sent this reply to Beth who was having problems with her son's lengthening. I thought his advice would be helpful to future readers, and Peter graciously allowed us to post it here.
If you would like to add your own tips, please send them to pffdvsg@gmail.com and we'll post them too.
Beth,
Hi - we got forwarded your note from the PFFDvsg list. We're so sorry to hear that Justin is having such a hard time. Our 8 year old son, Luke, had the Illiazarov procedure in July 1997 through to June 1998 out here in Sydney, Australia. We wanted to share just some of our experience to try and help you and Justin.
Our son Luke was born 22 June 1990 with PFFD of his right femur . Up until June 1997 Luke was getting by with a prosthesis but had his first Illiazarov lengthening at the Westmead Children's Hospital in Sydney (an excellent location) in July 1997. At this time his leg was 15 cm shorter, primarily in the thigh, and he had a valgus foot. The surgeon decided on a full leg Illiazarov frame in which after 2 months of lengthening we achieved 5 cm in the femur and 4.5cm in the fibia and tibia - the reason for the lower limb being lengthened too was that it would be easier to make up the target 10 cm on this first lengthening using both the upper and lower limbs - 10cm from Lukes thigh was too much in one go.
I don't want to sugar-coat the whole experience as that wouldn't be doing you any favours. This was undoubtedly the toughest experience of all of our lives, particularly Lukes. But what I think you need to hear right now is some practical advice. Here's some that just comes off the top of my head:
1. Make sure you get the right balance of pain-relieving drugs as early as possible. It took us several weeks before we were able to get the right balance - he started off on morphine, then went down to Codeine which didn't seem to work. The Amyltriptyline did take some time to work (weeks) for Luke, the levels need to build up in the body, so do give it a chance. He tried Valium at one point but Luke was just so out of it all the time that we took him off it. Its important to work with the "pain team" if your hospital has one - they'll advise you on this, but experiences can vary greatly from child to child.
2. We did see a noticeable psychological change in Luke that we didn't expect and he did go to a child counsellor in the hospital (part of the Illiazarov clinic) who helped him through a lot of issues. He "lost it" on more than one occasion, became angry and talked a lot about "cutting his leg off" to end this all or "throw himself down the stairs" which was very distressing and heartbreaking for all of us. He did a lot of drawing of himself (coloring the parts of the body that were "angry", these were black; and those that were happy, yellow - interestingly he always colored his Illiazarov leg black and the top of his head yellow because, as he said "I'm still happy in my head".
Some things which helped were "imaginary" conversations between his left (good) leg and his right (Illiazarov) leg. This would go something like: Left leg: " Hi, how are you doing there right leg ? Right leg: " Its nice to know someone cares. I was beginning to think no-one loved me" Left leg: " Sure we do...." etc
There were also times when we had to let Luke just let rip. Usually he'd be yelling at himself into a mirror screaming (we called them screamathons !) "this is so unfair..this is too much for a little boy to take....I can't stand this...how would you like it if you had this frame on your leg".
It makes me sad to reflect on all this now, but we "lost" our little boy back there for a while - he wasn't the same happy--go-lucky kid for several months. I'm glad to say he's back in full spirits.
3. Pin-site cleaning does get easier. We used Nintendo and other computer games to distract Luke whilst we were doing it. We even had names for the pin-sites such as "Angel" (that one never hurt!) and "Wombat creek" ! He had over 20 in total.
4. We found Luke's appetite just disappeared and he lost a lot of weight, so its important to watch diet and ensure they're getting the right nutrients and appetite enhancers. Favorite foods got used a lot !
5. Remember you are not alone and advice is just a phone-call or e-mail away - the best account I've read of the emotional side of this whole PFFD and lengthening is by Melissa Arnold whose story "Puddles of tears" on the PFFD web-site is astonishingly similar to ours. I have tremendous respect for the courage Melissa continues to show through the face of sheer adversity - she is also a great correspondent and we've swapped several e-mails.
Beth, I hope some of this helps. We know this is not an easy time for you. I don't subscribe to the view that you may be "molly-coddling" . Each child has a very different reaction both physically and mentally to this whole procedure. I think Luke had a very tough time of it all, and we saw other children who had the frame on at the same time seemingly having a much easier experience (which didn't help things !).
I wish you all the best. If there's any way we can help out please just e-mail.
regards,
Peter & Carol Helson
phelson@ozemail.com.au
Sydney, Australia