To Bianca (sorry, quite long)

[Guest guest]

----- Original Message -----

From: Bianca Jansen

....... info on who you are and what your children have.

..... I realize your kids have PMG, but what handicaps do they have.

Hi Bianca

Our little Hannah is 5½ years old and she has PMG wide spread on both sides of

her brain. Portions at the back of her brain are smooth. She was diagnosed by

MRI scan when seizures were found to be uncontrollable at 2 months of age.

Hannah began to have noticeable seizures at 10 weeks, and to this day still has

them. She has not responded well to any of six medications, but the ketogenic

diet helped her enormously. Unfortunately she could not continue the diet

because of other medical reasons, and her seizures have become much more severe

and far more frequent. She has a few different seizure types.

At about 12 months she was regarded as having cerebral palsy, although her

injury is congenital and not from around the time of her birth. She cannot move

herself at all, does not grasp anything, cannot point etc, and she cannot sit or

stand. Because she has poor posture and twists herself a lot, she has developed

an S-shaped curve in her spine, and one leg has become shorter and tighter than

the other. She had surgery to lengthen the tendons and to relax a muscle in that

leg, but it has returned to the way it was. They tell me she needs bone surgery,

but we are not at all sure that we will allow that. Other muscle issues have

lead to poor swallowing ability, and as a result she has also had trouble with

respiratory issues when she chokes on her saliva. Because she cannot take enough

food and fluid orally without great risk, she is totally tube fed via a

gastrostomy tube. This takes the worry out of feeding her and we know she will

be well nourished and hydrated, and it is the easiest way to get meds into her

too.

Hannah has cortical vision impairment, which means her eyes are structurally

perfect, but her brain does not interpret the visual messages into meaningful

information for her. She will rarely focus on anything, or track any movement,

but she does sometimes turn her head to sounds and to touch, a bit like the

primitive baby reflexes. Her hearing is possibly affected, although it is

difficult to determine just how much she misses hearing. She does respond to

sounds, and shows pleasure in some sounds and irritation with others. She is non

verbal and has the intellect of about a 2 month old, but she responds very well

to people, with smiles and giggles. She absolutely thrives on physical contact

and movement.

I'd say that Hannah is one of the most severe cases of PMG that I've heard of,

but in spite of all her problems she is a gorgeous child who gives us much

pleasure. It is very difficult to care for her, and becoming more so, but with

plenty of help we hope to manage for much longer. Take heart in the fact that

most children have many more skills than Hannah, and each child takes their own

time to develop to their full potential. Unfortunately the doctors cannot really

predict with any certainty how a child will be affected. We were told that

Hannah would not have a normal life and were offered a social worker. In fact,

none of our family have a " normal " life and we have needed many more

professionals than just a social worker. On the other hand, many positive things

have happened to us as a result of Hannah's condition, and we have met some

wonderful people and made some good friends. We have seen another side of life

which, although difficult, is also rewarding in many ways.

(Hannah's mum, Australia)