Catching Up

Wow, it's been a while since I last blogged! 8 months! It's been a busy time with lots of crazy family-goings-on, Warrington Wolves winning *another* Challenge Cup and a break-in, not to mention our little Bee turning 4 years old! It's strange how all of a sudden she seems so grown up, like it's happened all at once. Now we have a proper little girl with a personality and sense of humor, who likes to tell us "now" when she wants something and has started choosing her clothes for the day; a right little madam!

We've seen many changes in Bee in the last 8 months, across the board. Her development is progressing well and we think a huge part of that is increasing from two afternoons at nursery per week to five mornings. 

Her learning and cognition has improved vastly and we think her understanding educationally has really come along. Although she's not quite at an age-appropriate level (her speech therapist approximates 3 & 1/2) we know she understands a lot of colours and the numbers 1-10, a big achievement.

Speech-wise Bee now has a vocabulary of over 90 words and is gaining more by the week. Many of her words are just syllable sounds and can sound the same but she understands and attempts them. She recently said 'guitar', which her daddy was especially thrilled about!
We saw the cleft palate team at Alder Hey again in December but unfortunately she still hasn't got enough clear speech to make a decision on her submucosal cleft repair. It's not something we'd even consider unless the surgeon could guarantee us that it would benefit Bee.

 Since Bee became mobile and left her walker behind there's been no stopping her. Although she still tires fairly easily, she chooses to walk in most situations. She can even navigate curbs with a little support! She's a little unsteady on gravel or uneven surfaces but her strength improves every day.

We had concerns recently about Bee's feet turning out when she walks and the way she dragged her left foot when she became tired. We know hypotonia is still an issue for Bee so I booked another appointment with the physio to see if it's anything we should be concerned about. Now, five weeks later Bee has a lovely pink pair of Piedro orthopaedic boots which we're slowly noticing is having a beneficial effect, she's much sturdier and we've not noticed any dragging of her feet.

Bee still likes her routines and can be upset if things are different than what she's used to but she seems to be needing rigid structure much less than she did at a younger age. I've always felt that Bee used structure and familiar things to feel secure when she's lacking in confidence. As she grows so does her confidence and she seems to not need the refuge of routine as much.
Similar can be said of her fear of texture. No more does she panic when on grass and we've even witnessed her playing in a sandbox! Major progress! We feel nursery have been a massive part of this change in Bee, her confidence has soared since being there.
The biggest example of this was before Christmas at a local garden center. We'd gone for a meal & a browse and I noticed they had a table set up for facepainting. Now, we've tried this before. It was an absolute no-no. Not only does it involve someone touching Bee's face, it's them touching her with something cold and wet. She freaked. So consequently I didn't have high hopes for this time. But I asked her anyway and surprisingly she insisted she wanted something done. The girl doing it was very nice, she tried asking Bee what she would like but of course Bee just kept trying to show her her doll. I thought it was best to start small so I gave Bee a choice between a flower or a bumblebee on her cheek. She chose a "bebe" - bumblebee.

Aaaannnnnd..... voila! 


She did it! Bee actually sat still and allowed a complete stranger to not only touch her face, but touch her with something cold and wet! I was so proud! Of course I had to keep stopping her from poking at it or trying to take the brush off the girl, as you can see in the picture her hand is sneaking towards one of the paints! But overall it was hugely successful and she spent a fair amount of time afterwards admiring her new "bebe" in the mirror!


Just recently Bee had another video fluroscopy. We had one towards the end of 2012 but unfortunately Bee would not cooperate and drink the white liquid so we came away frustrated. She's understood for a long time what 'drinking' is now and we've been offering her water, which she readily accepts. We're desperate for a positive video fluro result as it will be the beginning of trying to wean her off the mic-key button. And so recently we finally received our appointment through for another. Of course we get to the appointment and Bee refuses to sit in the chair, not a good start. She initially refused the drink this time too but I managed to persuade her to take a small sip. When she realised it wasn't water she immediately spat a majority of it back out but the speech therapist was able to see that some did go down her throat and.... she didn't aspirate it!! Wonderful news! Of course this was tempered somewhat by the fact that some aspirators can only aspirate 1 in every few swallows so the team were not able to give us full permission to start her on liquids but they are happy for us to continue with water. When we and Bee feel ready to move forward onto other liquids, juice for example, we'll have to go back for another video fluro and get a comprehensive positive result. But I'll worry about that in the future, for now my girl is drinking water and it's amazing.

Bee turned four years old in September 2012. Four. I still find it hard to believe that I have a four year old. She was spoilt for her birthday and got two lovely days out. The first we spent at Eureka, the national children's museum in Halifax. It's a wonderful place with absolutely tons to do and Bee had a blast even though much of it was beyond her. She's not ready for leaning about how science works and what our blood vessels do just yet but the 'mini town' section was well worth the entrance money! There's a bank, shop, post office, garage and a house. One of Bee's favourite games is pretend play so she had a ball playing 'shops' and posting letters. We would definitely go back there!

Eureka!

Our second day out took place on Bee's actual birthday, we went to Blackpool Sealife Center and then down the front to see Blackpool Lights. the Sealife didn't turn out to be the best choice, Bee was not impressed by the fish and spent most of the time wanting to leave. We got an equally indifferent reaction to the lights, I think by the time we started (when night fell) she was too tired to really be bothered. She did enjoy the flashing light stick we bought her off the front though! I think her favourite part of the day was the meal we went for between the Sealife and the lights. She got to eat chips (her favourite!) with ice cream and a share of daddy's chocolate brownie for dessert! Winner! 

 Over the last few months one of Bee's favourite things to do is bake with her daddy on his days off. She's getting very good at mixing and I've been treated to some very yummy fairy cakes, biscuits and cookies recently!





Our biggest change is that Bee will be starting school in September. School. I can't believe it. It's been a long and confusing process and we still haven't chosen our final school. There are 6 to choose from: 2 special needs schools and 4 mainstream schools with developmental units, which is the fancy way of saying special needs class. We started this process knowing that Bee wouldn't be in a mainstream class but we've kept our minds open as to the other possibilities. We've now had a handful of meetings with the Educational Psychologist and copies of reports from a few of Bee's therapists, we have a good idea of what will be going in her statement: that Bee has complex learning needs and significant developmental delay. She will need a lot of support in an educational environment and ongoing intervention from her therapists. But we know that Bee is a happy and very sociable child, she thrives on attention from others and benefits greatly from interaction with her peers. Her nursery have said she loves being in the 'garden (mainstream) room' and plays brilliantly with the children in there who are a little ahead of Bee developmentally and can model behaviour for her and help support her play.

Of the 6 schools we ruled out two immediately, one for being a faith school and we're not a family of faith, the other as we believe it provides for children with more significant physical and learning needs than Bee. That left us with 4 to choose from; one special needs school and three primaries with development units (or 'designated provision'). We've been encouraged by everyone to view, view, view the schools and find the one we're happiest with so that's what we've done.

We started with the special needs school, which has an outstanding Ofstead report and is located close to Bee's Nana & Grandad's house. The school is great and one of the biggest positives for us is that it caters for children aged 5-19, which means if Bee was to attend there she wouldn't have to move again until she was ready to end her education. This is a big plus for us as Bee really struggles with settling at new places and we're already very stressed about what September will bring. They use Makaton and the PECS system and the teacher/pupil ratio is great. I really couldn't pinpoint any fault with them at all.
But it just didn't feel right.

Over the following few weeks we visited the 3 primary schools with designated provision. one of our main concerns about this choice for Bee is that she would struggle to cope. We loved all three of the primary schools we visited but all the children seemed so much more advanced than Bee. Still, that was something that could be counted as a plus as well as a minus. Bee really benefits from watching and interacting with others, especially children who are ahead of her developmentally.

We came away from the viewings no closer to a decision and decided to go back and look at them all again, but take Bee with us this time. In part this was because we hoped seeing Bee in the environments would help us get that feeling about 'the right one' that everyone said we would get. In was also in part because we wanted staff at these schools (especially the mainstream primaries) to meet Bee and reassure us that they could support her needs and help her thrive. 

The special needs school was first and Bee spent some time with the reception class while we toured the playground/outside space with the headteacher. She had a blast and didn't want to leave but we were not keen on the classroom facilities and felt that the structure of the session we saw was a little too like a nursery. Of course we only saw a snapshot of the day but we came away unhappy.

Next was the primaries. Foz's favourite was first. It's hard to judge this school on it's facilities as it's in the process of being knocked down and rebuilt before September, so will have brand-new everything. Although I suppose that counts as a plus but it doesn't help us imagine Bee attending! We both really liked the teacher at this school, she talked very passionately about her pupils and her job. Plus instead of spending the entire time talking about the school (which others did) she asked us tons of questions about Bee and told us how she (and the school) could support her needs and make the setting suitable for her.
Bee spent about an hour with the class in this school. The minute we walked into the classroom she was gone. She did us a beautiful painting while we spoke to the teacher (which is currently adorning our fridge) and had a ball with all the toys.

The second primary we visited again had been my favourite on our first viewing, I liked the facilities and the staff and with all the schools being so similar in lots of ways it had come to down to minute things like whether or not we liked the teacher. On this visit Bee spent about an hour with the class, while we stood in the background and watched. She sat at the table with them and had some cake and water (they were having some kind of celebration - we timed that well!) and, although she didn't say anything and just watched she didn't look lost or left out. Many of the children went out of their way to try and include her, asking her questions and offering her cake or more water and she didn't seem she or withdrawn. She looked tiny in comparison but not out of place. After cake she followed the other children outside for a 10 minute playtime. She even, I saw it with my own eyes, played throw-and-catch with another little girl! It was a struggle not to cry!

After that second visit Foz and I decided that our final selection was between one of those two. We've decided on a primary with designated provision (rather than a special needs school) as we feel that Bee will really benefit from being among others who she can model behaviour and learning on. She thrives on interaction with others, especially children who can help support her play by leading. Basically, we want to give her the chance to cope in slightly tougher environment. Plus we have the reassurance of knowing she'll have 6-monthly progress reviews and if she's not coping as well as we hope she'll be able to move to the special needs school.

Now all we need to do is decide between our final two!

Hello Again B11 Cubicles!

After being discharge from hospital 2 months passed fairly uneventfully. We still struggled with Bethany's feeding, she would cry and choke on the milk, and we were always told she was fine and it was normal. We were once told she was a 'fussy' baby if you can believe it. Still we struggled on with the feeding and Bee struggled on with the weight gain. She cried most of the time and life got very stressful. By the time our second hospital stay rolled around in May Bee still only weighed 12lbs at 7 months old.

Bethany started with a cough, high temperature and vomiting which meant she was getting down very little food. For a baby of Bethany's weight that was worrying so I made an emergency appointment at the GP's surgery. I took her down and she was examined. After listening to her chest the Doctor made a few notes on the records in front of him. Out of concern of looking like a paranoid parent, which was how I'd so often been treated, I broke the silence with: "I just wanted to get her checked out, I was worried about the cough and her throwing up. She's so little to begin with. Last time this happened we spent a week in hospital". To my relief the Doctor replied: "that's where I'm sending you now." Bethany's chest had sounded 'crackly' and with the constant vomiting and her low weight it was his opinion that she should be admitted to the children's ward for possible Bronchiolitis. As worried as I was I was also really pleased that the Doctor had taken me seriously and we were being seen immediately. Luckily the hospital is only a 5 minute walk from my GP's surgery.

And so we were admitted back on to B11 Cubicles for the second time on Thursday 7th May 2009. For the first few days Bethany was extremely poorly. Her temperature was sky high and she couldn't keep any food down. She coughed so much and so severely that her throat was hoarse and when she cried hardly any sound came out. She needed quite a bit of oxygen during the course of the illness as her breathing was very laboured and she would desaturate, meaning the percentage of oxygen in her blood would drop. It was heartbreaking. A chest x-ray had confirmed bronchiolitis but unfortunately the only treatment was to wait it out. We fell back into the old routine. I would stay there 24/7, sleeping on a camp bed on the floor and Foz would visit in the evenings and on his days off. Thankfully the bronchiolitis eased after 4 days, although Bethany's cough remained. Unfortunately she lost a lot of weight, 8ozs in total, which is a lot for such a tiny baby. She went from 12lbs 4oz to 11lbs 8oz.

After 6 days we were transferred to a larger room with a cot for Bee and a bed for me! Joy! Normally Bee would probably have been allowed home once her bronchiolitis symptoms has eased. But with her weight loss and reluctance to feed normally again we had to stay. Of course being in-patients had it benefits: we were seen every day by a Doctor, sometimes more than one. Bethany had more blood tests and examinations, trying to diagnose a cause for her failure to thrive. Bethany has also always had a rattle when she breathes. It sounds like she needs a really good cough and it's been there since she was born. One doctor once told us that it was milk in her throat. What 24 hours a day, 7 days a week?? I don't think so. After complaining to the umpteenth doctor about Bethany's difficulties with feeding and her rattle one finally suggested that Bee be checked out by ENT, or the Ears, Nose and Throat department. Hooray! Progress! Someone who listens!

So I wrapped Bethany in a blanket, the Children's Ward is always stifling but the corridors off the ward are cold, and headed off to the ENT department. We were accompanied by a nurse, as if I couldn't be trust not to run away with her. She dropped us in the waiting room, informed the receptionist that we were there and told me she'd be back to collect us. I tried to reassure her I was very familiar with the hospital by that point and the hospital wasn't that big to begin with but she insisted. So there we sat, waiting, amongst the eldery men and women. Eventually Bethany's name was called and we followed the nurse into the room. This was mine and Bethany's first experience of a throat examination and I was quite prepared for how unpleasant it would be. I had to sit Bee on my knee, facing me, then lie her down so her head was resting upside down on the Doctor's knee. He then used a large wooden stick, like a giant lollipop stick, to push down Bethany's tongue and see right down her throat. My poor girl gagged and retched and I honestly thought she was going to throw up but she was a trooper and persevered. It only took 20 seconds and it was all over. I lifted her up and she sat on my knee, blinking and taking us all in. I murmured some reassuring words to her and she gave me a big smile. That's my girl. I turned to the Doctor for the verdict and it was here we got our first clue on the way to getting Bethany's final diagnosis. Our little Bee has a bifid uvula. My first reaction was probably pretty much what yours is: "A what??". Well, according to Wikipedia a uvula is "a conic projection from the posterior edge of the middle of the soft palate." In laymans terms it's a piece of tissue that hangs down like the tonsils. Bifid means split. So a bifid uvula means it is split. I asked about the ramifications of that and was told that although it can cause trouble swallowing, it's not a huge problem in and of itself and many people live with them with no effects whatsoever. He referred us to the ENT department at Alder Hey Children's Hospital with the recommendation Bethany have an endoscopy (camera) down her throat. Of course it's never pleasing that a Doctor does find a problem with your child but if there is something to be found we are always relieved when a Doctor does finally find it. We were pleased with the Alder Hey referral. They have a great reputation and are the nearest children's hospital to our home. It is also a regional centre for so many specialist units we knew we'd be in safe hands.

As a result of the ENT diagnosis and because of Bethany's feeding difficulty we also had a Speech and Language Therapist come and see us. We were pretty confused when we were told this as Bethany wasn't yet old enough to be speaking and she'd only just started baby babbling. When Claire the Speech Therapist arrived we felt immediately at ease with her. She was one of the nicest and approachable health professionals we'd met with so far and explained everything very clearly, instead of just assuming we understood the long medical terms. She explained that a speech therapist didn't just do the obvious of encouraging and developing speech. It was her job to look at the function of the mouth and throat, see how the mechanics were working and if there were any problems. We'd had to starve Bethany before the appointment to make sure she would eat while Claire was examining her. Consequently she was irritable and moaning so we started with the examination pretty quickly. Whilst Bethany had some solid food and then her bottle Claire listened to her neck with a stethoscope. She let Bethany finish eating then had a quick examination of her throat as the ENT Doctor had done, I then settled her in the cot with some toys so we could have a chat. She asked me lots of questions about Bethany's feeding, breathing and general health and finally gave me the verdict. Claire confirmed what the ENT Doctor had told us about the bifid uvula and gave us some rather bigger news. She suspected Bethany had a sub-mucous cleft of the soft palate. A cleft palate?? But she was fine, there was no split in her lip or the roof of her mouth. How could she have a cleft palate? I just didn't understand. Claire explained that the mucousal layer is the layer of skin and tissue that forms the roof of your mouth and cleft means split. So basically the sub (underneath) of Bethany's mucousal layer is split. That's why we couldn't see a split, it's underneath the roof of the mouth. The physical evidence was a high arch and a faint silver line running down the center of her roof. What this means is the muscles haven't come together and fused after the tongue descended during development. When a regular person swallows those muscles lift up the soft palate at the back of the mouth, to close off the passageway to the nose. As this hasn't happened in Bee's mouth it means when she swallows she gets fluid coming back up into her nose. This means effectively she has to swallow twice for each mouthful of food, doing twice the work, and she's got a constant amount of liquid sitting in the back of her nose, making her rattle as it doesn't drain properly. The second major effect that a sub-mucous cleft can have is difficulty sucking. To get a decent suck a baby needs to create a vacuum with it's mouth. If the soft palate isn't closing off the nose passage then air can still circulate and sucking becomes difficult. Suddenly all those hours struggling to breastfeed popped into my head and things started falling into place. Of course the little we knew about cleft palates involved surgery, speech therapy and all kinds of drastic procedures. Claire reassured us that in a lot of instances no surgery was required. As long as special bottles were used and weaning encouraged Bethany shouldn't have a problem. Usually surgery was only performed if the child developed nasal-sounding speech as they got older. That was reassuring. Bethany just seemed to tiny to go through surgery and it was the last thing we wanted to put her through. Claire gave us some special squeezy bottles so Bethany wouldn't have to struggle sucking, we could help push the milk into her mouth and promised to inform Alder Hey Cleft Palate department, who would send a specialist nurse to come and see us.

Trish, the Cleft Palate Nurse Specialist, came the following day. She confirmed everything Claire had told us and referred us on to the Cleft Palate Surgeon, Mr. Van Eden for a surgical consultation. Trish expressed concern during her examination of Bethany that her fontanelle (soft spot on her skull) appeared to have closed. We already knew Bee's was small but it having closed so early in life was a cause for concern. Trish explained that normally, as babies brains grow their skulls grow with it, closing the gap between the skull plates, known as the fontanelle. As Bethany's has closed so early their was concern that whilst her brain would continue to grow, the skull would not be able to accommodate the growth. This would result in the skull essentially squashing the growing brain and causing inter-cranial pressure leading to brain damage and an abnormally shaped skull. The correct term for this is Craniosynostosis. She told us she'd put a referral through to the Cranio-Facial Unit at Alder Hey and they would contact us in due course. Of course when she left I panicked. I imagined every minute going by was causing more and more brain damage to my beautiful little girl. As you can imagine we were completely freaked.

Bethany was finally discharged, after gaining 6 of the 8ozs she'd lost, on the 13th day. It was the day before my 26th birthday and the best present I could have hoped for, as cliched as that is. This time round we felt like we'd made progress. We had an answer as to why Bethany struggled to feed and I had the comfort of knowing the difficulty with breastfeeding wasn't my fault. But the up-shot of all this was it becoming increasingly obvious the Geneticists had something to find when they started testing. Now not only did Bethany have failure to thrive, developmental delay and hypotonia, she also had a cleft palate and bifid uvula to contend with as well as possible craniosynostosis. Trish actually asked us if we'd be surprised by a genetic diagnosis of something and we told her we wouldn't be. Her reaction was "if I'm brutally honest I wouldn't be surprised either". That just said it all.

We again couldn't have got through this period without the support of some very wonderful people: my Mum and Ken, Foz's parents and our good friends Lynne and Mike, even when it was just a pint of milk for a cuppa in the parent's room! :)