Hallie was christened on 23.12.12

Hallie's first Christmas

video - useful to know seizures come in all different forms

Hallie's first seizure on Monday 17 December 2.20pm.  She is 7 months old. Her seizures start in her left foot and move quickly up her left leg into her torso and then into her left arm.  They last approximately 5 to 10 minutes.  Hallie only has unilateral port wine stain on her brain (the right side only) affecting the frontal and parietal lobes.

Going home!

I'm very happy to say we have managed to get out of hospital for Christmas! And Hallie's christening tomorrow : ) I had planned a Christmas going back and forth between Hallie in hospital and Isaac and Seth at home (which I would have made work as you have to work with whatever situation you are presented with) but having Hallie at home Christmas Day is something I certainly won't be taking for granted.

After being nervous about the doctors and nursing staff at the beginning of Hallie's admission, I have left feeling a lot more positive and the nurses were very caring, cheerful an attentive. I hope Hallie goes back to S2 again if she gets readmitted - although hopefully this won't be for a long while!

xxx

Hallie is hospital - photos

Our stay in hospital: part 2

After my chat with the neurologist (in which I blubbed and told him I didn't mean to offend him or question his expertise, I just wanted to fight for Hallie and ensure she was looked after properly) he apologised and said he had actually treated SWS patients before which made me feel a lot better. He agreed to treat after 5mins of a seizure starting instead of 20mins, which was concerning me. But only as a one off treatment and not repeatedly every time she had a seizure in clusters as this could send her into a deep sleep and affect her breathing.

So, Hallie was given another loading dose of sodium valproate and had her carbemazepine medication increased. The neurologist told me he was increasing Hallie's carbemazepine much faster than he normally would to try and get on top of the seizures.

Hallie's last seizure was 8.30am Thursday 20th - fingers and toes crossed (words of the neurologist) that the seizures are under control for now and Hallie can go home for her christening on Sunday and of course have her first Christmas at home!

Our stay in hospital

Well, it's been a long week of which half the time I haven't known what day it is or time! So, I haven't had the opportunity to update hallie's blog.

She was admitted on Monday 17 December to Sheffield Childrens Hospital. I had fed Hallie her usual bottle around 2pm and we were sat watching tv having a cuddle when I noticed Hallie wasn't kicking her leg normally, it was rhythmically twitching. I didn't panic as I have been waiting for this for the last 7 months (although I must admit I wasn't expecting it all to happen the week before Christmas!) I lay Hallie down on her change mat to have a proper look at her body. She was very happy in herself as she lay on her mat watching me. The twitch moved up the left side of her body and into her left arm and hand. I recorded this on my phone as evidence just in case she stopped before the ambulance arrived. I phoned 999 and explained what was happening saying my baby girl was having a seizure and that she had Sturge weber syndrome and it was her first seizure. The ambulance arrived within a few minutes and I explained all this once again to the two medics who had never heard of SWS surprise surprise and I didn't want them to think she wasn't fitting. But, even though the seizure was stopping, they could see her left side was completely paralysed so said they were going to take her in with the blue lights.

At A&E Hallie continued to fit every few minutes. I was so unbelievably worried because at this point I didn't feel they were taking the situation seriously or treating it with urgency. Hallie was fully alert and wide awake throughout her seizures - she was responding and moving her eyes following the doctor's light. Because of this, they thought she was okay and she was coming out of each seizure after a few minutes. I didn't count but I would say Hallie had approximately 10 to 15 seizures in the two hours she was in A&E. finally she was given a drug (I cannot remember which one) and she was transferred to the neuroscience ward.

Hallie was given Carbemazepine and a loading dose of sodium valproate to kick start the levels of drug in her system. The nurse explained that because little ones' livers work so efficiently they tend to get rid of the drugs really quickly out of their system so need high doses to keep levels up.

Hallie kept fitting I would say 3 to 4 times a day, each fit lasting 5-10mins - the neurologist visited a lot and I had an emotional conversation with him about my concerns that he wasn't treating aggressively enough. It was so difficult because on the one hand I didn't want my baby girl to be pumped full of all these chemicals but I also wanted to ensure she was getting the treatment she needed to prevent brain damage. The nurses were a little stubborn when I kept insisting SWS seizures needed to be treated differently to other epileptic seizures. They kept telling me they had treated many patients with epilepsy over many years to which I replied "how many children have you had on this ward with Sturge weber syndrome?!"

Seizures have started

In hospital A&E - Hallie has started with seizures

She is fine in herself. The seizures started in her left foot and leg and moved rapidly into her torso and then into her left arm. They have been on and off for the past couple of hours

I will update my blog in detail later

Christmas pics of Hallie x

Marlcliffe School Christmas Fayre

Wow what an afternoon!! I haven't managed to count the money we've raised yet as every time I try my little boy Seth says he wants to "help"! But I think it's over £100 : )

We had a guess the bear's name and a lovely young boy (around 7 years old) won by guessing the name Buddy - he was very pleased

We raffled a chocolate house and I made Christmas cards and bracelets which sold well. The Marlcliffe art club Christmas cards didn't sell as well as I had thought but I will try again next week!

Hallie got sleepy half way through the Fayre and wanted a snooze bless her but the hustle and bustle was all too much. She wore her Christmas outfit especially and sat in her pram while mummy and grandma were busy on the stall.

Most importantly I managed to hand out a lot of leaflets and hopefully this will help spread awareness - I chatted to quite a few parents and explained the syndrome. The first parent I chatted to told me her son had recently passed away after having a brain tumour - this made me even more aware of how lucky I am to have Hallie in my life and how precious life is and how easily it can be taken away from us.

There were a few people who approached the stall to just give a donation without buying anything - you could see by the look on one elderly man's face that he had experienced Sturge Weber Syndrome some way in his life. All in all it was a great event and I would love to do more : )

I handed out leaflets asking parents to contact me if they would like me to organise a book party (in which I will link fundraising into the ordering of Usborne children's books)

Hallie is going on her first train journey next Tuesday, tickets are booked, we're finally off down to London to see the consultant in the specialist clinic at Great Ormond Street. I'll keep you updated on what is said.

I'm now going to have a well earned glass of wine with Graham and most likely fall fast asleep on the sofa!

X

Some recent photos of my Hallie x

Development of the central nervous system

The port wine stain - cluster of blood vessels - develops in the first few weeks of development in utero - so by the time I was 8 weeks pregnant with Hallie, her condition was already present and the blood vessels were on her tiny brain and what would be her skin eventually!

(Picture taken from google source)

Fund raising

Hello, just a quick update while I'm up with my coughing children!

My husband graham is raising money for Sturge Weber UK by doing a triathlon "iron man" event in June 2013. He has a just giving page on www.justgiving.com (type in Hallie Davidson and you'll find him) - donations welcomed please :oD

I'm also trying to do as much as I can too to raise money and awareness for the charity - I have a stall at my son's school Christmas fair on 6.12.12 - I'm going to be selling Christmas cards, raffling a very special cake donated by a very kind friend Hazel and I'm going to raffle a big cuddly bear : ) marlcliffe school have also kindly agreed to ask a few pupils to design special Christmas cards which will get printed up hopefully next week so I'll be selling those on my stall too along with the Sturge Weber UK wristbands and sweeties and children's wooden jewellery too : )

I'm also organising an Usborne book party for 1.12.12 for all the parents in my son's class YR DR and 10% of book sales will go to Sturge Weber UK - any parents who are interested please contact me ASAP as at the moment I don't have enough to hold a party unfortunately : ( - I'm planning on providing wine and food as a thank you for coming x

I've spoken to Jenny Denham this weekend just gone about my fund raising ideas - she is the first person I have chatted to from the Sturge weber Facebook support group and she is the chair of the committee of Sturge Weber UK - it was lovely speaking to her and I'm so glad there is a support group and the Internet in general these days as it provides such a useful and supportive resource for me and parents alike to share experiences and offer guidance. I know people criticise emails and texts as being impersonal but I find them a godsend for asking quick questions and getting instant answers - even from consultants!

Anyway, as the coughing seems to have died down now in our household I best try and get some sleep! Night : )

Neurology appointment finally!

After spending the last four weeks worrying about what the scans of hallie's brain might show, today we got a proper chance to view the numerous scans and put our questions to the neurologist. Although to be honest I don't feel I have learnt much more about hallie's condition other than where on her brain she has the birthmark (patch of blood vessels/stain) whatever you want to call it!

Hallie has a patch of blood vessels on the right side of her brain, underneath where the port wine stain on her scalp is. You can't really see the port wine stain on her scalp because of her beautiful mop of hair : )

The neurologist then did a few physical tests on Hallie to rest her reflexes and how she used each side of her body, particularly her hands. Hallie wasn't that co-operative but that's not surprising since she woke up with a stinking cold this morning. He said he wasn't too worried about how she was using her hands at the moment but she would need to be checked regularly as these things usually become more obvious as a child reaches 12 months.

He didn't really put a plan in place and said the usual "it's a case of waiting to see" which is extremely frustrating. I wish I knew exactly what was going to happen Hallie and when.

Now we have the Great Ormond Street appointment in December to look forward to - I am looking forwards to meeting the consultant at their Sturge Weber Clinic as my friends in the Sturge weber UK Facebook support group have sung her praises many times and I'm hoping she will provide us with a lot more information (which will hopefully make things a lot clearer as I feel a bit fuzzy at the moment)

Thanks for reading : )

Nightmare

I had my first nightmare about Hallie last night. She was sat in her high chair and I thought she was cold because she looked like she was shivering uncontrollably but then I realised she was having a seizure and her face looked really distressed and I couldn't soothe her : (

Still haven't heard from the neurologist regarding the details of Hallie's MRI scan on her brain - I'm eager to know exactly where the stains lie on her brain and what the next steps are for treatment etc. we have an appointment on 15 November to discuss the brain scan but I had actually requested that the consultant phone me before then - he's obviously too busy as I've left him three messages! Hmm might phone again today and leave another message for him..

We've got Hallie's eye appointment on 1 November so hopefully that won't be too traumatic for her and her eyes won't have worsened.

X

Sturge Weber Syndrome

I thought I'd try and write a post to say a bit more about Hallie's diagnosis (if my two mischievous sons will allow me!)

Last week Hallie was diagnosed with Sturge Weber Syndrome (SWS) - this was not a complete shock as we had been forewarned the week Hallie was born by the neonatal consultant who came to visit me at my bedside.  He had written it down on a piece of paper and told me briefly a list of possible complications Hallie may have if she did have SWS. 

For those of you that read my blog because you want to know more about SWS - here comes a detailed explanation taken from various resources (including my head)!

Sturge Weber Syndrome occurs in the first weeks of an embryo's life.  It is an abnormality of the central nervous system (I think) and is not hereditary.

Symptoms of SWS include:-

•  A Port Wine Stain of the skin (usually around the eye and forehead area as this is near the trigeminal nerve which is connected to brain activity). 
• A layer of blood vessels touching the surface of the brain called an angioma (these blood vessels can irritate the brain causing seizures).
• Glaucoma - raised pressure in the eyes caused by increased level of blood vessels in the back of the eye.
• Developmental delays and learning difficulties - this is more likely to occur when a child with SWS has the epileptic seizures.  When the blood vessels on the brain (angioma) are on both sides of the brain this is more likely to cause severe learning disability.
• Sometimes the child will have weakness of limbs on the opposite side to that which is affected by the angioma on the brain.

Hallie is due to go back for her second eye appointment on 1 November - her eye pressures measured at 16 - 19mmHg in her right eye (the one completely covered with PWS) and 12mmHg in her left eye on her first appointment aged 4 weeks old.

An appointment has been pencilled in for Hallie to be seen at the specialist SWS clinic at Great Ormond Street in December.  We will hopefully get a chance to chat to Dr Aylett, the specialist who is very knowledgeable with the syndrome. 

Hallie is also pencilled in for her first laser treatment in January at Leeds.  I haven't changed this as a few parents on the Sturge Weber Facebook Support Group have reassured me that Hallie would be treated in a children's ward which made me a little happier.

I have found it invaluable having the SWS support group on Facebook and am so thankful for modern technology - I would feel so alone and helpless without the internet and Facebook and the numerous people I have become connected to via support groups.  The same can be said for the autism support groups I am connected to on Facebook too (that's another story though!)  Contacting the consultants is so quick and easy and I can get in touch with a few of them via email and get reassuring replies a few hours later.  All this support and information helps keep me calm and collected which allows me to continue fighting for Hallie's referrals and appointments etc.

I still haven't seen the neurologist regarding the results of Hallie's MRI brain scan.  He has been away.  I am hoping to contact him this week and ask where on the brain Hallie has the angiomas.  I am guessing it isn't extensive otherwise her behaviour wouldn't be as it is - but I don't know how it all works.  I am under the impression the parts of the brain where the angioma touches are the only parts that are affected when the child is having an epileptic seizure - which is why sometimes a child can carry on playing normally whilst having a seizure... this worries me and makes me anxious I will miss a seizure or not notice her first one straightaway (if she has any at all that is)  The symptoms above do not all occur in every child with SWS. 

Most symptoms occur in the first year of life.

Epilepsy:

approximately 80% of people with SWS have epilepsy - in most cases the epilepsy starts in the first two years of life and if by age 5 Hallie has not had a seizure she isn't likely to.

I am to watch out for twitching or jerking of the limbs on one side of the body, or jerking involving all limbs with loss of awareness.  Absence seizures (where child appears "not there"), drop seizures where the child will drop to the floor and episodes of limbs becoming stiff. 

The seizures I am worried about are the subtle ones where the child may just have a change of breathing or just a slight twitch of the hand or foot for example.

{I have taken a lot of this information from a booklet on SWS written by Dr Sarah Aylett from GOSH}

PLEASE SEE www.sturgeweber.org.uk FOR MORE INFORMATION ON SWS AND HOW TO SUPPORT THE CHARITY.

Diagnosis

Today Hallie was diagnosed with Sturge Weber Syndrome. We have had this in the back of our minds since she was born but it was confirmed today after her MRI brain scan last week - have just about stopped shaking now.

I am yet to speak to the neurologist regarding the details but will post again once I know more x

Give your support :)

www.changingfaces.org.uk

Hallie before theatre, after and at home with Bearlinda

An emotionally tiring week

Hallie had her laser appointment on 3rd October - it was her initial appointment and we went armed with a big list of questions expecting a thorough consultation and a plan of treatment put in place for Hallie so we could get the ball rolling with her treatment early.

This did not happen.  We saw a registrar who spent time making notes about Hallie and saying things like "well, it looks like a port wine stain" DER!  I was more than a little displeased and altogether unimpressed with the consultation.  I had to strip her down to her nappy on what looked like a dentist chair which was very awkward.  There were no nursing staff on hand to help and the clinic did not have a single picture on the wall (I think the way a clinic is presented has a huge effect on the patient and the way they feel) I did not feel comfortable or welcome here.  I requested to see the consultant (since that was who we had been referred to!) and he came and spoke to us briefly, looking at Hallie's face and talking about laser treatment as though it was not a necessity rather a cosmetic procedure and therefore did not have any urgency or importance.  I didn't like the manner of the consultant (informal and relaxed but not in a friendly caring way) He never sat down with us and talked through how he would go about treating such an extensive port wine stain as Hallie's - he was actually quite negative and said extensive birthmarks such as Hallie's quite often did not respond to treatment.  At one point I asked him about the inner ear (as Hallie has deep stains all over both her ears which go right down into her ear canal) the consultant just replied "oh well most patients choose not to have their ears done because you can't see them as much as the face".  He really did not seem to understand our concern, not for Hallie's appearance, but for the thickening of her birthmark in the future and the effect this may have on her hearing, sight, mouth\speech and nose\breathing!  I was not impressed at all and we went away feeling perplexed and very disappointed. 

The mixture of emotions gushing through both me and Graham (daddy) were exhausting and the worse was yet to come as Friday 5th October was MRI scan day.

Friday morning started (for me) at 3am, Hallie's last feed, I had to starve her after then for the general anaesthetic procedure.  We got to the hospital for 7.30am and sat waiting with what looked like another 30 children with their parents, it was packed in the waiting area.  The children were there for a variety of operations and procedures, not just MRI scans.  We didn't have to wait too long and at 9am Hallie was taken from me by a male nurse and they disappeared through some double doors into theatre.  I wasn't allowed to sit with her while she was put to sleep as she had to be put to sleep in theatre where she was having her endoscopy.  The ENT consultant performed the endoscopy as he was concerned Hallie had a blockage in her airway that may have been caused by swollen birthmarks growing on her tongue.  Straight after this procedure in theatre, Hallie had to be put under general anaesthetic and transferred to the x-ray department for her MRI scan which took 90 minutes!  The MRI scan was on her brain (to look for stains related to Sturge Weber Syndrome) and her throat and chest to look for malformations and generally check the size and shape of things to check everything was okay.

After just over 2 hours away from us she was brought out of the x-ray department on a large bed, crying.  Her voice was croaky and she was understandably groggy.

The ENT consultant explained that he found Hallie had a floppy larynx and a narrow windpipe - which he had operated on and made wider with a balloon.  "You may notice an improvement with her breathing now" he told us.  Hallie is always very congested and makes loud grunting and snorting noises quite often when breathing.  He also confirmed there were no birthmarks on her tongue although it was quite large.  He needs to do an examination of her throat and vocal chords while she is awake and crying which he will do over the next few weeks.  I imagine this will be very traumatic for Hallie but better to do it when she is a baby I suppose.

We now have to wait for the MRI results (7 to 10 days) (will feel like years) and we have an appointment with the ENT consultant next week to discuss his findings in more detail.

Phew! A lot to talk about but all good news up to now and Hallie is back to her normal self today, smiling and feeding but still has her croaky voice x

All wrapped up for the chilly weather

more photos!

not the most flattering of pics but these show Hallie's pws clearly

big bro hitching a lift in Hallie's pram

jj

trip to the supermarket

"I don't want to go back to sleep"

you can see Hallie's tongue and gus on this pic - both of which I feel are large

Hallie's first visit to the seaside, and dip in the sea! aged 4 months

Hallie's new chair :o)

big week ahead of us

Since I last posted, things seem to have moved forwards with Hallie's hospital appointments.  On Wednesday 3rd October she is going to see the laser specialist at Leeds General Infirmary to discuss treatment and hopefully put some sort of plan in place for her first laser treatment.  I need to put together a list of questions, there's so much I want to know!  Friday 5th October will be a difficult day for Hallie (and mummy and daddy) as this is when she is having her MRI scan on her brain to check for stains - clusters of blood vessels touching the brain which can lead to seizures.  I am no expert and I find all this very confusing myself so feel free to comment and correct anything I get wrong!

Hallie went into hospital last week for a sleep study to monitor her saturation levels (oxygen levels in blood).  Not a nice experience for myself, Hallie on the other hand seemed indifferent, I don't think she cares where she is as long as she is with her mummy :o)  Six beds in a bay, each sick child with a parent = 12, yes 12 bodies in a small bay!  To say it was hot would be an understatement - all the children were naked with just pants or nappies on, including Hallie.  The parents and staff were sweating too!  I felt so claustrophobic in there, especially as I was surrounded by poorly children crying and wailing in pain and discomfort, It was quite unbearable.  Hallie fed and went straight to sleep at 7pm, was hooked up to the monitor and checked on every three hours for her obs.  I thought her stats had remained quite high (in the 90s) but the next morning the respiratory consultant came to let me know that the pattern on the trace showed there could be some kind of blockage which was causing Hallie's oxygen saturation levels to dip every few minutes - not dangerously - but enough to cause her concern.  She arranged for the ENT consultant to visit Hallie in hospital that day.  He came, stared at her for a while, watching her large tongue (another concern) and the way she was breathing.  He asked my permission to take a photo on his mobile phone and send it to his colleague in the US (the speed at which we can communicate these days comes in very handy when trying to make a diagnosis!)

To cut a very long story (and long sweaty wait in hospital) short, the ENT consultant wants to perform an endoscopy on Hallie to see if there is any kind of blockage in her airways causing her saturation levels to drop - one suggestion was that it may be her large tongue causing the problem.  The respiratory consultant came to see me (looking very concerned) and said she had been chatting to the dermatologist who initially saw Hallie and he was also very worried and wanted to discuss Hallie with his friends at Great Ormond Street as if Hallie had a vascular malformation (birthmark) on her tongue which was making it swollen and large, it would be something for the specialists at GOSH to look at and treat - I'm guessing because it will cause complications and be difficult to treat.  We were sent home that evening none the wiser as to what was going to happen.

I got a phone call the next morning from Dr Jay (respiratory consultant) and she confirmed they had decided to do the MRI scan not only on Hallie's brain but on her throat and chest, to see the exact construction of her tongue and throat and check for abnormalities.  While she is under general anaesthetic they will do the endoscopy as well and take her bloods to test.  After these investigations are complete they will then be in a better position to decide whether she needs urgent referral down to GOSH.

So, big week ahead of us - I'm trying not to think about it - writing it all down on this blog then pushing it to the back of my mind until Friday.  Hallie is as ever smiling and playing most of the day, hitting her toys and grabbing them now.  She has also started chatting to me (this started last week in hospital, she could obviously tell I was bored and needed cheering up!) she makes the most beautiful sounds when she gurgles and smiles at me.  Oh and her weight seems to be picking up - she has put on nearly a pound in the last 10 days! x

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In hospital for saturation levels monitoring

Hallie went to see a consultant in respiratory medicine last week.  She was referred by our GP due to concerns that the port wine stain covers the whole of her neck and part of her chest and this can sometimes affect the heart apparently.  The consultant was a very kind warm young woman, different to most consultants, and tried to reassure me that Hallie would be okay and she would get all the treatments/appointments she needed without delay.  She sent Hallie for a chest x-ray - Hallie was a very good girl and stayed still for it!

Hallie also has to stay the night in hospital tomorrow so her oxygen saturation levels can be monitored to make sure her breathing is okay.  Then, she is having her bloods and urine taken - they are being thorough, mainly due to her slow weight gain I think.

Hallie was weighed last week (aged 18 weeks) and weighed 9lb 14oz - I was pretty shocked, disappointed and worried, I felt disappointed that I hadn't managed to make her bigger with my milk and worried that it might be something serious linked to her PWS.  She was born on the 50th centile and is now on the 2nd so has dropped significantly.  I have asked the health visitor to refer her to a dietician so fingers crossed this is not a big problem and something that can be fixed with a bit of special fatty formula - she has been referred to ENT to investigate her large tongue and gums and her congestion problem she has had since 2 weeks old which could be making it more difficult for her to breathe resulting in her using all her calories up breathing rather than growing.

Anyway, I'll stop there as I didn't want this blog to be all doom and gloom!!  I shall post some cute pics of my beautiful Hallie instead x

 2 days old

 first bath at home

 daddy cuddle

 hallie aged 5 weeks

 bath time with her big bros

 O-lay!

 little ballerina xxx