Wednesday, 18 April 2012


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.


  1. Are her hands and feet normally cold? I haves 4 month old baby w/ Beals and her hands and feet are always cold and often purple.

    Thank you for this blog! My little one has aducted thumbs and they will be starting her on braces soon. It's just nice to see someone else whose child had the same issues a mine. I've only met one specialist out of a dozen who had even heard of Beals.

    Christine Chicoine

    1. Hi.
      I am a Mazda with your problem finding doctors and therapists know in BEALS
      I'm from Israel and I am 14 years old and I was diagnosed with Syndrome beals at the age of 5 months.
      And since I'm dealing successfully with the challenge that God blessed me with him.
      To my knowledge I am the only child in Israel with the syndrome BEALS.
      I would be happy to contact you by e-mail: ODET65@GMAIL.COM

  2. Eeeek! The photo in the hat at the top is soooooo cute!

    It's always lovely seeing how you advocate for Evelyne.

    Would love to hear an update about the recent international convention you attended.

    ~ Jamie Boros

  3. Hi Christine,
    I do remember them being cold and purplish as a baby. The photo above of her as a baby shows that they were quite purple. I haven't noticed it in recent times though. Maybe it has changed as she has started using them more?

    Jamie, Thanks! I'll give an update once I get some feedback from the specialist.


  4. Love this post Lou. I never get sick of looking at photos of our Evelyne but the background you've given on the hands should be helpful for others. I did notice that you didn't mention the Ian Thorpe feet? Dad