|Alice and her brother David|
Our first child, Alice, was born in January 1997. It was a normal delivery; she weighed 9 lb 8 oz, and the significance of her reddish skin colour was not apparent to us until one of the midwives just mentioned that she thought the paediatrician should look at it. However, we were advised that Alice seemed fine, the paediatrician at the hospital would review her at her 6 week check and we went home with our beautiful little girl.
We had five weeks of treating Alice as a baby unaware of Sturge Weber Syndrome (SWS) and its associated problems which subsequently seemed to completely take over or even take away "normal" new baby things. For this we will always be grateful and "glad" that we did not know.
Alice's port wine stain is extensive, over both sides of her face and the right side of her body. To me, and I am ashamed that it is so, the hardest thing to deal with is the staring that people do.
Apparently, to some people, if a child or adult does not look quite the same as what the apparent "normal" is, they make judgements about the cause. Anyone who reads this and knows a child or adult who has a port wine stain or other visible marking can probably identify with this.
On good days you can pass off people's stares or comments, on bad days it hurts you or you are ready to attack them. People have suggested that Alice was sunburnt (in February?); she had been scalded (after laser treatment which blistered); she was choking; or even was blind??
Alice is obviously unconcerned and I hope we can help her stay like that as she gets older.
It has improved as we have adjusted and got used to it, Alice's sociability helps as she insists on being talked to and being noticed but often it is still painful. When I wander round the supermarket with our second child, David, who does not have SWS, I am very aware that people don't stare at him and it is a completely different experience.
As children grow older I think that it is very important that they learn the right way for themselves to deal with the reactions they receive and the reactions that they then want to make.
But back to my story, Alice stopped moving her right arm just before her hospital check though this was a red herring because she now has a marked weakness on her left side; doctors later decided that she probably had an unobserved seizure which caused this temporary result. It is the right side of Alice's brain which is badly damaged and the left which appears normal. Her epilepsy, which in SWS is frequently difficult to control, has not been a problem to Alice. Her anti-convulsants have recently been stopped. This shows that there really is no typical pattern even in the syndrome itself and that each child is an individual who should be treated as such.
I find that I have to try very hard not to dwell on what problems may occur as they might not and it is enough for anyone to deal with what problems actually arise. Looking too far into the future is not a useful occupation either.
I realised that Alice had a definite seizure while we were waiting for tests following the hospital appointment and she was readmitted to our local maternity hospital where staff were lovely and we as a family came to terms with the diagnosis. There seemed to be so much news, much of it difficult, which we had to take in. No time to adjust to Alice being a "different" baby to the one we thought we had and because of the practical issues of watching for further seizures or attending hospital or even informing people, everything went so fast and hazy.
The Easter after she was born, following an MRI scan, we were advised about brain surgery being appropriate for Alice and this happened abruptly, out of the blue. This seems the bleakest time we ever had, feeling we had been given such drastic news but then hearing nothing for weeks and ultimately hemispherectomy was not deemed appropriate after all our anxiety and worry.
Alice has had no evidence of glaucoma so far, but as the port wine stain is on both her eye lids she is regularly checked by the specialist. Coming with the syndrome comes numerous hospital appointments and it is hard work and tiring attending them so frequently. With SWS the possibility of brain problems, eye problems, laser treatments, etc., a child can be seeing numerous consultants each about different things. To begin with Alice seemed to be at hospital so regularly, but it has got better.
To be honest, Alice's babyhood is a bit of a blur. David, our son, who is just over one now, has made me realise just how much rushing around we actually did with Alice, to this appointment or that assessment. He has passed a few assessments now without difficulty and it does feel good. I think this is what it should have been like for Alice.
Please do not misunderstand me, a child like Alice who has special needs, no matter how complex their condition, I think brings such joy to their family. Their achievements, which so often cost so much to themselves and their family, mean so much.
Currently we view Alice's biggest problem as being her nose bleeds. When the ENT medical staff investigated Alice's nose under anaesthetic, they unexpectedly discovered there was no obvious reason for her nose bleeds; the inside of her nose was clear of birthmark. However, a second MRI scan performed recently, showed that Alice had dilated blood vessels and the extra pressure was causing the nose bleeds. We can find no other person with SWS having this particular problem. They happen without warning and are heavy, meaning that blood transfusions are often required. Each time is a traumatic time to us all as a family,not least to Alice as she gets older and repeated blood tests or even worse, insertion of nose packs, frighten and upset her. She has a sociable nature and soon recovers her general enjoyment of meeting people, but is made nervous for a time.This is what I mean about not dwelling on what might happen, as this happening to Alice could not have been anticipated at all.
Another problem is that Alice also reacts very badly to minor bumps to her head. She becomes drowsy, vomits, her blood pressure rises and generally she shows signs of major head injury.This was dreadful the first time it happened, but having occurred more than once, it becomes less frightening. Again, this seems to be atypical, so is not likely to present in other children with SWS. We know what the pattern is likely to be and deal with it. Any child bumps their head frequently and one who has a weakness and is unable to control their movements very well bumps more frequently, so trying to watch Alice more carefully is essential.
And so having worried about seizures (which influenced decisions on baby sitting, holidays, nursery school, etc.) we then went on to worry about nose bleeds occurring whilst we weren't with Alice. It can make life a little harder than normal. Our expectations have needed to change or adjust but our greatest joy is still Alice herself and though I say it myself she spreads happiness when being with people. She attends our local mainstream school nursery each morning where a teaching assistant enables her to join in. Alice loves being with the other children which made the many assessments and hassle involved in receiving the statement of educational need for her worthwhile.
In closing my motive for writing these words is that I think every person needs to be aware and accept each individual, whether they have a disability or not. Do we see a person as different to the "norm" (what is the norm??) or do we respond as positively as we can and try to encourage each other. I would like to take this opportunity to thank Jenny, the Committee and the Foundation for its continuing support and all the hard work that they do for each family or person who contacts them. When we sometimes feel as if there is only us with these problems, it helps to know that we are not alone and many other people struggle with the same things, or even worse sometimes. And I look at Alice who is usually smiling and I think "Yes, it is all worth it".
By the way, if anyone reading this knows anyone or of anyone with, SWS who suffers the type of nose bleed which Alice has with the cause being the brain angioma, I would be so grateful if they could let Jenny know to pass on to us and our local ENT department.