Hands

One of the characteristics of Beals Syndrome is long, slender fingers. The joints in the fingers can have contractures or be hyperflexible depending on the individual. In fact, in Evelyne's case she has some joints in her hands that are hyperflexible, and others that are fixed or contracted. It is also common for the thumb to be adducted, or tucked into the palm.

I have been informed that the main issue in the future will be her hands getting tired or sore with use, as well as weakness in the joints. Those I have had contact with who have hand contractures report that they still have full functional use, although their hands may not look 'normal'. One adult wrote that he only has 40% use of his fingers, but works on computers with an 'adapted style'. Another who has hyperflexible joints reports of her hands getting 'tired' easily and not being able to cope at times.

When Evelyne was born, her hands were one of the first 'out of the ordinary' things we noticed. She had tightly clenched little fists and was very relucatant to bring her thumb out. As she got older and wanted to use her hands we discovered she could open her palm and extend (abduct) her thumb, however, she often still tried to use her hands without her thumb. We had a soft neoprene splint that we used at this age to encourage her thumb to stay out of her palm. As she got older, she began functional use of her thumb.


For the past 2 years or more, we have not worried about her hands, as she has had full use of them. However in recent months, since she started pre-school, her Occupational Therapist has wanted her to start using her pen or pencils with a better grip, in preparation for hand-writing. Her natural hand position is to tuck her thumb in and draw with a fist. We tried strapping, by taping her thumb into a fixed position, but Evelyne was not very impressed with this option. We then tried a thermoplastic splint (pictured below) to be worn when she is drawing. Again, Evelyne is not keen to wear the splint, but says "I'll do it myself", showing me the correct hand position. Even with the splint she does not last as long colouring in as she used to, as she complains of her hands being sore. It is something we are still working on. Other things we have been using to make it easier include: a slope board, pencil grips, and scissors with springs in them. These are all meant to reduce fatigue in her hands.

I am yet to find out if her flexible joints may in fact become fixed in the future. From what I have read, I believe this may be the case.

Flower girl

Evelyne was a flower girl at a close friend's wedding a couple of weeks ago.


We took her brace off for the afternoon so she could wear the pretty dress and move more freely. She did a great job, especially at staying still for photos (I think it's the practice from x-rays). By the end of the afternoon, after a few hours out of her brace, she was visibly struggling with tiredness. She could barely hold herself up and collapsed into bed once home (thanks Nanna and Grandad!)


It was a special day and I think you'll agree she looked gorgeous! 

Preschool

This year Evelyne has started preschool! She is attending our local preschool one day a week. It is a wonderful parent run preschool with great staff and facilities. Samuel has attended there for the last 2 years, so Evelyne is very familiar with the environment and the teachers. 


We managed to get funding for a full-time teachers aide which has taken some of the worry out about how she will cope. And I think it will give us a good idea about how she will go in a couple of years at school and how much assistance she will need.


At the moment she needs some assistance with safety on the outdoor equipment (due to risk to her spine), help with her clothing for toileting (due to the brace).  The preschool has a special cut out table and slope board which makes it easier and less strain to do work at the tables. We also need to start working on her hand position for pencil grip.


She loves painting, glueing, reading and singing and proudly displays her craft and sings us the songs once she gets home. The only problem is she finds it hard to wait until the next Friday comes around!


The photos are of Evelyne's first day. She is grumpy because she just wanted to get out the door!

New specialist, new brace

A few months ago we had an assessment with a new specialist. We discovered a spinal surgeon in Sydney that has hands on training in Mehta casting and wanted his option on Evelyne's spine and the possibility of casting in order to postpone surgery.

He informed us that bracing is not very effective in controlling the curve once it's over 40 degrees (as we knew). When he examined her the curve was over 50 degrees in the brace, and so he suggested the best way forward was to try Mehta casting to attempt to get the curve under control.  We decided that over the summer before using Mehta casting that we would have one more attempt at bracing with a new design methodology. 

The new brace has been more uncomfortable and restrictive with numerous complaints from Evelyne when putting it on and more when traveling in the car. It also has a tendency to put holes in her clothing. However after 4 weeks in this new brace, today we had an x-ray. The x-ray showed a curve of only 41 degrees! It is encouraging to see that the brace has reduced the curve, however we will still have to monitor how well she is tolerating the discomfort and watch for any pressure areas. The next few years will be a juggle between wanting treatment to be effective, but making sure it is able to be tolerated by Evelyne.

Brace shirts

Evelyne has had frequent problems with rubbing from her brace. This has significantly worsened in the new higher design, in particular under her arms. Through a couple of my online contacts, I heard of shirts specially made for under casts and braces. There are different types, one brand called 'knit-rite' which make a cast t-shirt (to be worn under a plaster cast), and another 'the Boston T' or 'the Boston Silver T' (made for boston braces). They reduce perspiration, bacteria, are cooler in summer, and can be changed to reduce smelling and itching. 


I tried to find the shirts online and always hit brick walls. The main being that they would not ship to Australia. In a last attempt to find one I posted a request on an online forum for infantile scoliosis. Success! Within a week I had an offer to post some to me from the US and not long after they arrived. The shirts we got sent are the Boston Silver T and are specifically designed for a brace. They even have flaps under the arms to reduce rubbing in that area. They have made a phenomenal difference to Evelyne's comfort levels. She is kept cooler, less sweaty and smelly, and most importantly - no sores under the arms or on her spine!


Here is a picture of her showing off her new singlet.

Magnetic growing rods

There is a new technique being trialled in Melbourne which uses magnetic growing rods. My specialists here in Sydney are also trialling a similar type of system. They have done one surgery at present, with another to be completed soon. This technique may be an option for Evelyne. The benefit is the rods can be extended without 6 monthly surgeries. There is a recent article below on a 7 year old boy who recently had the surgery.

http://www.theage.com.au/victoria/magnetic-attraction-gives-matthew-chance-to-stand-tall-20111023-1memn.html

Holidays

In the recent school holidays we had the opportunity to use a friend's time-share unit for a week. It was wonderful to have a restful family holiday. We do spend quite a bit of time visiting family etc but it is usually related to doctors visits and various clinics.

Samuel loved fishing, going out on a boat, swimming in the pool. Evelyne enjoyed the beach and the pool. It's hard to imagine her being in a cast soon and having to avoid some of these things she loves so much!

Here are some pics.