Meya Pettersson

Sjukdomen

This is how the disease is described on wikipedia

The condition mostly affects children, with an average age of 6 years. However, one in ten people with the condition develops it in adulthood. There are two main stages, sometimes preceded by a ‘prodromal stage’ of a few months. In the acute stage, lasting four to eight months, the inflammation is active and the symptoms become progressively worse. These include weakness of one side of the body (hemiparesis), loss of vision for one side of the visual field (hemianopia), and cognitive difficulties (affecting learning, memory or language, for example). Epileptic seizures are also a major part of the illness, although these are often partial. Focal motor seizures or epilepsia partialis continua are particularly common, and may be very difficult to control with drugs. In the chronic or residual stage, the inflammation is no longer active, but the sufferer is left with some or all of the symptoms because of the damage that the inflammation has caused. In the long term, most patients are left with some epilepsy, paralysis and cognitive problems, but the severity varies considerably.

Meya first showed symptoms at the age of 2,5 years, which is unusual. Because the disease itself is also unusual, and due to the fact that she has an atypical form, she was not actually diagnosed with Rasmussens until after about two years. They suspected it earlier, but it wasn’t until she had her callosotomy (procedure were they sever the link between the brains hemispheres) infebruary 2011, that they could do a biopsy and finally say for sure. What makes it atypical is that her seizures would seemingly manifest on either side of the body and not in ways that are very typical for the disease.

The disease came sneaking in spring 2009. It started with some really weird symptoms. Meya would complain about strange, nasty smells that no one else could smell. She wanted to sit in our laps  and would rub her nose grimacing. Then she wanted to lay down and usually fell right to sleep, sweating profusely while sleeping. We have later noticed that after big seizures, Meya would get fever spikes that came and went in a relatively short time, so this could be the reason for sweating so much back then.

She had her first ”real” seizure in June and shortly after she was diagnosed with epilepsy. They could not say why she had it though. In the beginning she had a few seizures per week, but slowly this increased. At most she has had about 30 per day that we know of. I say ‘know of’, because EEG has shown her to have internal seizures that we don’t see–some of which she tells us and some she don’t.

EEG has also shown us that she has almost continuous , what we call spark or static, i.e. epileptic activity in the brain that never ”release” and turn into a seizure.

When first the doctors mentioned Rasmussen, we of course did not know what it was. Searching for info on it on the net (something we had stayed away from earlier) we came across a clip from the today show about a little girl called Cameron Mott. She had gone through pretty much exactly the same thing Meya was going through.

Cameron’s seizures and life in general was exactly like Meya’s. The girls even look eerily much like each other at times 🙂

Those big and awful seizures that strike like lightning are so painful to see. Luckily for Meya she only got them once or a few times a day.

(This is the original clip from the today show)

There is also a facebook page and a blog that, even if it has not been updated for a long time, has a lot worth reading. It helped us tremendously, especially going forward with Meya’s hemi.

Rasmussen’s is a long term disease and the effects are permanent. It’s a very aggressive form of encephalitis that basically kills brain cells in the affected hemisphere. During the long period its active it causes a lot of damage. It is the areas with dead cells that cause the epilepsy but it also effects other functions of the brain.

Treatment

They have tried treating Meya’s seizures with a number of different medicines over the years. There is no cure for the disease itself, but some medicines have had at least some effect. Its hard to say exactly how much of an effect a certain medication has had, but if she doesn’t get her pills, she will have an increase of big seizures. It has been an endlesss try-fail cycle. It is slow because they add a medicine carefully and take it out even more carefully. Some have had bad side effects.

The doctors don’t really know and they depend a lot on us parents to give good reports on how seizures start and look and on what kind of effects we see from the medicine they are trying out.

She did a Callosotomy in 2011. They did that operation mainly because of those big seizures she had every day that were really powerful and which made her fall uncontrollably and, usually, slam her head into whatever was in front of her. The greatest benefit was of course that she really stopped falling like that. She still had the big seizures, but as they only affected half her brain, she usually managed to catch herself somehow and not slam into the floor. It was such a huge difference for her and for us and really took a lot of pressure of off us.

One period they tried giving Meya a really really high dose of cortisone. She first got a three day ”pulse” via IV, then through pills for about 8 months. It was a really high dose, basically as much as you would give a grown person (Meya weighed 18kg when they started). It had some good effects and some bad side effects.

Probably the most important benefit was that it took away the ”static”, allowing Meya to concentrate/relax and really grow as a person for a few months. It was a wonderful time in that respect. The mood and food-craze.. not so much 🙂

After those 8 months, they tried a long period with only those IV ”pulses” . It had good effect in the beginning, but over time the side effects grew so large, they totally eclipsed any positives. They tried giving her Takrolimus during this period, which would hopefully have some of the benefits of the cortisone, but the effect were hard to trace.

After trying basically everything, and because of Meya getting older, it was decided that the only thing left to do was what is  historically the only solution to this horrible disease.

hemispherectomy

Due to the nature of the disease and the damages it cause, its only through a so called hemispherectomy that you can successfully ”treat” it. What this means is that they either turn off or completely remove the whole diseased hemisphere. This is, at the time of writing this, still the only way to get rid of the very tough epilepsy that come from the damages cause to the brain. Traditionally removing the hemisphere has been the way this is done, but lately this has changed. Basically the reason for this is that, because the brain is such an important part of metabolism, its better for the body to still have the hemisphere in the blood-system. So instead of removing it, they disconnect it completely, but leave it physically in the brain, connected to the bloodstream. There are more reasons for this and there are also advantages to complete removal, which is why that procedure is still used.

There is more information on the net, and if you want to know more I suggest the Hemifoundation site. They help children and families that have to go through with this (its not only Rasmussens that lead to hemispherectomy). They are also on facebook