J.C. Just Keeps on Going! (Feb. 1994)

Our son, Jason (JC) was 18 months old when we noticed he had a problem with his feet. Not long after this first observation his feet were so out of shape he couldn't even wear shoes. He was so tough and a happy wee boy everyone was amazed at what he achieved. He had his first operation at two years with transferring of muscles and lengthening the Achilles tendon. We were led to believe this operation in Christchurch would correct Jason's feet. We were not told he had CMT. Cliff and I also had electro studies to check our reflexes but all was okay. JC was then put int those horrible boots and calipers (braces).

Not long after we moved to Nelson, we were seen by another pediatrician and orthopedic surgeon. It was decided to put JC in plasters to straighten his feet, which was followed by splints. This went on for nearly a year but was unsuccessful. We were then sent to Dunedin Hospital to have more tests under the care of Professor Martin Pollock, a neurologist, and only then was JC's condition confirmed – the rare autosomal recessive form of HMSN Type II.

Cliff and I were devastated knowing the outcome of Jason's future. We try hard not to show really how we feel. I think that is why JC has accepted the way he is.

As JC's feet had deteriorated again, the surgeon, Mr. Panting, decided to operate again to fuse the bones below the ankles which prevented him rolling over and also he could walk without support. JC was so brave after the operation, all he said was, "Don't get blood on the sheets!" He can't stand blood after having blood tests. It was a trying time.
JC was so excited when he was able to wear normal shoes without splints or calipers He was five then and hated being different and overcomes this by his personality and charm!

Jason is just like you when you were a child; we let him have a go! He is an outdoor boy. He runs everywhere (in his own way), climbs, bikes, skateboards and loves swimming. He just keeps going. Our wee man is eight now and doing very well considering the condition of his hands, feet, and legs, which have wasted considerably below the knee. We decided against splints/calipers to give JC the quality of life. His feet are so deformed we are unable to move them during stretching exercises. Jason's hands and fingers are practically closed, yet I am amazed what he can do.

We have our moments, too! It has taken more than a year to finally get a computer for school and home, so hopefully he will be able to keep up with his peers during his school work. He still uses pens/pencils, etc., but finds he gets a little tired after a while. JC is bright and will achieve self-satisfaction and self-esteem by having the computer because now he can teach the other children how to use it.

I'm not sure what can be done for his hands, also the next step for his feet. The thought we have deep down of JC not walking scares us.

I have enclosed photos of his hands and feet which were taken over a year ago; as you will probably realize, his condition is much worse There is no record of anyone in our family having CMT.
Cliff, Dianne & Jason Corey (J.C.) Potts, New Zealand

(And an update from Dianne)

Early this year Jason had his third operation to straighten his feet After five months in plasters, he had them removed and is now learning to walk again with his feet flat on the ground. It has taken a lot of determination tokeep on his feet and to strengthen his bones but he is now walking normally like his peers. Jason (and of course mum and dad) are over the moon because it is unbelievable what the surgeon has performed to make Jason mobile. He has become more independent and his self-esteem has improved immensely. The surgeon is quite confident his feet will stay straight this time and we are keeping our fingers crossed!

Jason's hands have deteriorated and his fingers have closed up and he now uses his knuckles.

We had problems with Jason's placement at school this year because of his physical disability (they won't admit that it was because of his disability). Academically he is an average student and it is very difficult to accept that he is being held back because of it.

We would also like to thank Gaile Fornusek (CMT International New Zealand director) for putting us in touch with CMT International concerning Jason's disability because we need all the support we can get and we appreciate hearing about how other people are coping.

Cliff had polio when he was eight. Could this have any relation to Jason and CMT? Cliff and I do not have CMT.

Linda here - CMT is genetic, polio is a virus. Don't think so.