November 15, 2004 update on Michele

Well, it’s been another four years since I wrote my last update on Michele who is now 22 years old. I felt compelled to write this today as Michele is sitting on the couch eating popcorn with her mom, Bonnie, beside her watching “Seventh Heaven”. Bonnie tells me that when Michele heard the popcorn popping, she said “yes! Yes!”. Michele speaks very few words now – the only one that is pretty clear is “yea, yea, yea” when go through a list of what she wants (usually something to eat). It’s very hard to figure out the “no” so we rely on her to shake her head sideways to communicate “no”. Michele can still eat food that is small enough to be picked up with her fingers and while it may take a long time, she eats and drinks fluids very well.

We have all adapted pretty well to moving her around using a wheelchair. We have to lift her from the wheelchair to the potty, to the bed, to the shower seat, and so on. It keeps her mom and I in pretty good shape. Seizures are still pretty well controlled (using Depakote and Keppra) with only about one per month.

One of Michele’s favorite activities is going to free music concerts in town where she can clap her hands along with the rest of he audience. In the winter, we spend most of her time listening to music or watching TV. She especially likes tapes of “Hey Dude” that we taped from Nickelodeon back in the late 1980’s when she could still see. This is something to keep in mind for those parents of Batten children who are still young, can see, and have favorite shows on TV. Michele also loves musicals such as “Meet me in St. Louis” and Grease”.

All in all, Michele is still very happy and in good health. It would be hard to ask for more.

Steve Sheridan

It has been 4 years since we wrote our first story about Michele who is now
18 years old. She has lost all of her eyesight and can no longer walk
without assistance. She still loves going to school each day where she is
learning "life skills" at a skill center. She spends most of her time in a
wheelchair while at school. This is preparing her for the transition to a
wheelchair at home where we get by now by helping her get from one chair to another by holding both of her hands - much the same as when she was learning to walk (at 9 months of age). She still loves to get in a pool and swim.

We had our first experience with a seizure in the pool just yesterday - a
much less serious event than one would expect. One of us is always with her
while she is in the pool and we keep her out of the deep end. The grand-mal seizure lasted only a few minutes and she was able to climb out of the pool (with lots of assistance) within 30 minutes. Seizures are well controlled by medication and are still pretty infrequent - only one per month on average. We are still hoping that the future may bring some treatment or a cure.

Steve & Bonnie Sheridan

Original entry dated January 10, 1997

Michele Sheridan is our 14 year old daughter. She was diagnosed with Juvenile Batten Disease in February of 1996. She lives at home with us and her 16 year old sister who does not have Batten's. Michele is almost totally blind but is still able to walk, run (with a guide) and swim. Her verbal ability, while reduced, is still good. She loves animals (especially horses) and wants to be a Veterinarian when she "grows up". Michele's first symptom was some vision loss at the age of 7. Her Ophthalmologist originally thought that the vision loss was due to RP or cone-rod dystrophy. The vision loss, however, became worse at increasing rate.

The first grand-mal seizure at age 9 and a change in behavior and some learning problems told us that the problem was much worse than just vision loss. No one, however, seemed to have a clue. The next seizure at age 12 brought a diagnosis of Epilepsy and anti-seizure medication. Two more grand-mal seizures and letters from teachers about declining cognitive ability finally resulted in a skin biopsy at age 14. A few months later she was conclusively diagnosed as having the Juvenile form of Batten Disease. The long delay in diagnosis and early misdiagnosis is typical of this form of Batten Disease. The long delay made life very difficult for Michele - especially in school. Low-vision aids were of little use as her vision was declining so rapidly.

She was slow to learn Braille. She would complain "what is wrong with me? I used to be able to do this!" as she tried to complete her math homework using big print books along with a lighted magnifier. Returning home from school with severe headache was a daily event. Every evening was spent with many hours of homework. Now that she has been diagnosed, however, she has been placed in a program (still in her public school) for children with reduced cognitive abilities. Homework has been eliminated. She is much happier now, she enjoys going to school and headaches are rare. Her self-confidence has improved now that she can perform at or above the level of her peers.

A few people have asked us if we would rather not know that Michele has this terrible, fatal disease. Our answer is we wish we knew it sooner. We can now make changes to reduce the stress in Michele's life and, through the BDSRA, learn from other parents how to cope with the related problems. We are very thankful that medical research has allowed us to get a diagnosis. I hope that any doctors or parents reading this will consider the quality of life of their patients or children when they consider additional testing. While the diagnosis of disease that has no treatment or cure may seem non-productive to some, it can dramatically improve the quality of life for the child. We feel fortunate that we now have the knowledge to make the most of Michele's remaining years. We pray that medical research will someday offer a future for children like Michele.

Steve & Bonnie Sheridan
Plymouth, Michigan
E-mail: sheridan@wideopenwest.com

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