Merlin Syndrome

There is so much to work out and understand.

It became obvious really early that there is no manual for this, and the considerable experience of the eminent consultants and specialists produced only the sadly pronounced advice that every case was different. I rapidly became not just the expert on R's condition but also a conduit for data that were enthusiastically hoovered up.

I began to look more closely at what was actually happening in the hope that this would give some pointers on how to deal with the problem.

It is evident that a lot of the things we take for granted, and were learned with great pains in our childhood, are being steadily forgotten as the disease progresses.

Major among these is the deterioration in spatial awareness. This sometimes manifests as poor balance but the problem goes deeper.  R has no internalised image of where her body is in relation to surrounding furniture, walls, doors and other objects. If something is in her field of view she may be able to recognise it and define her own position relative to it, but the moment anything is out of her sight it ceases to exist or she has no concept of where it is.

So the simple act of sitting in a chair is a major undertaking. If she is looking at it and can see it then she will sometimes start sitting where she is (and where the chair isn't). If she turns so her body is in a position to sit safely she can't see the chair and panics that there is nothing behind her. Reversing her until the backs of her knees touch the chair may work, but that sensation is not necessarily associated with the certainty in her mind that what she is feeling is part of a chair and it is safe to sit. A structure of rails has made sitting on the toilet less of a heart stopping adventure but there are still moments where it all goes wrong.

She also has major problems with holding things. You and I can hold a mug full of tea and carry on a conversation or watch TV and a small part of our brain is monitoring the mug and keeping it level. That is a skill which was learned, and can be forgotten. Even two handled mugs do not help. Similarly with spoons full of food. There is an added complication that a spoon or fork is recognised to some extent for what it is, but the use of it is not information that is immediately available. I have seen R pick up a fork by the prongs and attempt to use the handle to pick up food. She knows something is wrong but gets very distressed trying to work out what.

Other previously reliable tools are disappearing fast. Pointing does not work. I point at something and she will grab my finger. Concepts such as 'over there', 'behind you' or 'here in my hand' mean nothing, and I can hold something directly in front of her face and she cannot see it. I am developing an entire routine for identifying something and guiding her to it. It is like trying to learn a new language in the middle of a battle.

Awareness of self and other is also disappearing. When I am doing up her jacket she will be aware of what is going on and seem to want to help, but she grabs my sleeve or my jacket and starts manipulating it.

I also think one side of the brain is more affected than the other, although the results of the last MRI scan did not identify any differences. Anything in the left hand side of her field of vision is much more difficult to see, sometimes to the extent of being invisible.

Such spaces as are visible seem to be defined by their edges. So, for instance, if she has a mug in her hand and is putting it down on the table where there is a plate, she will inevitable try to put it down on the edge of the plate. If the table is clear she will often try to put it down frighteningly near the edge of the table.

This is all an enormous challenge to handle. Sometimes I am lucky enough to find a form of words that communicates as an offer of help without the implication she can't manage, but this is very rare. She is acutely aware of what is happening and understandably she has a limited threshold of being able to accept someone pointing out when things are going wrong.

The best I can hope for is to be able to think far enough ahead so that my help and support come across as a natural part of the universe rather than an intervention.

There is also the entire topic of language, but that deserves a post on its own and must wait for next time.