New look!

The blog has had a redesign! I've had so many ideas about articles for the blog so I thought I would get started by making it look better! Watch this space..!

William's Surgery

I thought this would be an easy way to update lots of people on where we're at with William's tonsil surgery!

He was supposed to have his tonsils removed last week but the operation was cancelled last minute. We had a really awful time at Addenbrookes and have since lodged a formal complaint in the hope of improving things for other families in the future. In a nutshell, they weren't prepared for a child of William's complexity on the ward when we arrived.

I have now spoke to William's fantastic consultant at Hinchingbrooke and discussed our options with her. Having the surgery at Hinchingbrooke isn't an option because there is no PICU and they don't have specialist paediatric anaesthetists. Due to William's complications he is high risk for anaesthetic so needs these specialist services on hand.

We have discussed a referral to another hospital, particularly Great Ormond Street. She is happy to refer us but has explained this will come with it's own difficulties. We would have a longer wait as we'd be starting the process all over again, none of the drs there know William and if he were to need an intensive care stay it would be very tricky being far from home, away from any dr that knows his case.

Our current plan of action is to try and speak to William's respiratory consultant at Addenbrookes. He knows William well, is lovely and is also a PICU consultant. Between us we want to establish whether we think it's worth still pushing forward with the surgery or whether we should take a step back and look at some things first. In a child like William the tonsils would usually be removed if the child was struggling with sleep apnoeas. While we know William has apnoeas we don't know how bad they are. We might therefore look at carrying out a sleep study first at Addenbrookes to determine how much of a problem these are. This could lead us to the decision that the surgery isn't worth the risk if the sleep apnoeas aren't too severe.

We also want to speak to this consultant to see whether he can have more of a role in preparing the hospital for William if we proceed with surgery. This could involve speaking to the surgeon and anaesthetist before surgery and ensuring William has a bed on arrival while waiting for his surgery. Until we've heard from him we're not sure what the next step will be.

While we are so reluctant to return to Addenbrookes after last week's debacle, it would be the best place for William to be if something were to go wrong as he is known to a couple of drs there, they are familiar with our usual consultant and we are close to the hospice. I would also hope it would be the shortest wait for surgery given our other options.

Thank you to everyone who has sent lovely messages, dropped by with gifts to cheer us up and prayed for us. It has really meant a lot to us. In the mean time, William is thankfully completely unaware of all the worry and stress and enjoying still having his tonsils!

Last Friday

This time last week we'd just about settled William to sleep after a difficult evening. He'd become increasingly distressed with a cough, temperature and a whole load of gunk over the afternoon and spent all evening crying on our bed. The next morning we couldn't get on top of his fever, he vomited his meds and his respiratory rate was double what it should be. We decided to take him in and within 45 minutes of arriving he was in HDU on 15 litres of oxygen and not maintaining his sats very well. The nearest Paediatric Intensive Care Unit was warned. William's consultant came and chatted with us, sepsis was a real possibility and if his breathing got any worse we would be transferred to PICU. We thought this was our turning point.

We've had a lot of chats recently about what we want for William in terms of intervention should he go into respiratory failure. His lungs are starting to struggle and we need to be prepared. For William we have decided that we will intubate and ventilate on his first collapse. After that we're not so sure. Presuming he recovers our next step will be very much informed by how poorly he has been and how long it is until he becomes very poorly again. A consultant recently told us that we will learn a lot from that first PICU admission, If he's stuck on the vent for a week, suffers a lot and falls poorly again within months we might be choosing a very different path than if he bounces back within a couple of days and goes a year or so before falling unwell again. These are the sorts of choices families like ours make all the time, we all choose different paths, there isn't a right or wrong choice, you just choose what is right for you and your child.

So last Friday we thought we were on the cusp of this first major turning point. But thanks to God, some chest percussion and a bit of front lying the situation turned around. By Saturday morning William was managing well on 2 litres of oxygen, we were getting on top of his fever and his breathing was easier. 

We are now home with a whole lot of stuff to remember. William has oxygen in the home now, which we are weaning him off. He has nebulisers at least twice a day, chest physio at least twice a day, suctioning throughout the day and requires frequent position changes to stop the gunk settling in his lungs. This of course is on top of his usual medicines, managing his pretty violent seizures, calorie counting and blending 4 meals a day and all his moving and handling. It's got tough. 

Despite last Friday turning out a lot better than we were expecting I can't now shake the feeling that this monster is just a round the corner. The 10-15 years I'd always hoped we'd have with William is suddenly feeling ridiculously ambitious. Last week we were looking at getting a bigger car for William and his accessories. I was looking at holiday homes for next year after our incredible holiday two weeks ago. Now all of a sudden I daren't. My head is filled with timescales. Two days ago I'd convinced myself he wouldn't make it past 6. Today I can't shake the feeling we've only got 6 months.

The reality is we don't know. He has deteriorated but it's not dire. When he was 6 months old we thought he wouldn't live to see his first birthday and now we've had 4! Maybe 15 is still possible but our hope is shaken.

I think we're going through a period of grief. We've had a lot of illness this winter, our longest hospital stay in a couple of years, our worst chest infection thus far, a lot of new medical equipment and a lot of "those" chats. Hopefully the summer months will bring less infections and we can all have some time to rest, recuperate and take stock.

To end on a happier note...today Eliza was playing a rattle type toy and I started talking to William asking if he could hear it. Without prompting Eliza walked over to William, uncurled two of his fingers and places the rattle in his hand just as Steve or I would do. It brought happy tears to my eyes. I love that she takes him just as he is and is so thoughtful towards him. Having a child like William in our lives is an incredibly profound and wonderful experience and I love that I'm starting to see glimpses of her loving him.

And breathe...

Today William met with a couple of the respiratory team drs at addenbrookes hospital. William has always had some odd ways with his breathing owing to his brain malformation, but recently we've run into more problems.

William has been struggling with very thick copious secretions for the last few months. We now have a suction machine for him to help him clear it from his mouth which is very useful. William often sounds very bubbly and gurgly and uses up a lot of energy breathing through thick gunk then trying to cough it out. He has also developed more of a stridor where he kind of squeaks sometimes when he's breathing. We've also seen the reappearance of some apnoea episodes. A couple of years ago William started having some apnoea episodes but after a while they subsided. They only occurred when he slept sitting up so we could fairly easily stop them. We've now had a couple when he's been asleep lying down so they are more tricky to stop. A few nights ago I happened to check in on him and noticed he wasn't really breathing and then he did a big gasp and repeated. I checked his oxygen saturations and they were dropping very low during the not breathing times. We ended up having to turn him over to get him out of it. 

Due to these ongoing concerns we were referred to the team at addenbrookes for a review. We met with two brilliant consultants, a physio and a speech and language therapist.

We started off discussing the secretions. The dr felt there was a chance the excessive secretions were being worsened by an ongoing infection so we have a two week course of a particular type of antibiotic and William is being started on longer term prophylactic antibiotics. The physio wants us to start giving daily chest physio and has talked us through what she wants us to do. We also discussed maybe trying a special vibrating vest like this http://www2.hill-rom.com/medicraft/TheVest.htm to help keep everything moving in his lungs. We may also try some stronger saline nebulisers if the antibiotics don't help. We have already tried 0.9% and 3% saline nebs and will try 7% next to see if they help loosen everything up. 

William has previously tried two different medicines to treat excess secretions but they haven't really helped him. We could also look at having surgery to remove part of his saliva glands or having botox infections into his saliva glands to help control secretions. For this reason we are being referred to Addenbrookes' ENT team to discuss this further. 

It was also thought there was the possibility the secretions were linked to some kind of sinus infection. They are going to try some nasal flushes to see if this helps clear anything out stuck at the back of his nose. The antibiotics we have will also help treat this if this is contributing. 

We have recently started William on a blended diet. Instead of giving him medical formula we blend normal food and give it through his tube. One of the reasons we made the switch was to see if it might help reduce his secretions as it has done with other children. We are yet to see any improvements but will continue with the diet to see what happens. The drs thought it was good that we were trying this so we felt encouraged to continue.

We then moved on to discuss William's generally noisy breathing (see video below). There is still a possibility that he has large adenoids so the ENT team will review these to see if they need removing. William has very low tone because of his condition so probably has floppy muscles in his airways. The dr described his breathing as uncoordinated which all contributes to the noise. 

We then talked about William's apnoea episodes. I was able to show the dr a video (see below) of one of these episodes from a couple of years ago. In it you can see William making very little effort to breathe for a while before taking a big gasp. There are two types of apnoea, central and obstructive and the dr thought William's looked more likely to be central as he makes very little effort to breathe before the gasp. He explained it is difficult to treat a central apnoea as it's a problem with the brain sending out the signals to breath. We have discussed the possibility of William having some breathing support for the apnoea but if it is central they feel he would probably need a ventilator. If we are still having problems in 6 months they might look to do a proper sleep study to get more information. We discussed in some depth having CPAP but don't feel it will really benefit William or add to his quality of life. It won't help to extend his life and would just be more equipment to worry about. I asked whether there was the possibility of one of these episodes just causing William to stop breathing and not start again but they said that was very unlikely and you would wake up. We saw this the other night when William desatted to 57 then woke himself up to breathe. 

We will now be getting some oxygen for home for palliative use. At times William seems to work so hard just to breathe and it exhausts him. We are hoping have oxygen for occasional use will help to keep him more comfortable and provide us with a bit of reassurance when we are on long car journeys with him and can't reposition him if he starts tiring. 

On top of all this we had a lot of discussion today regarding William's ongoing lung health. Both the drs we saw today work a lot in the paediatric intensive care unit and see a lot of children like William when they are very poorly. We know that William is most likely to die from lung problems caused by increasing chest infections. We talked a lot about how this is most likely to unfold and what sort of treatments we would want for William. This is not new to us but it was useful to revisit these topics and get a clearer understanding of what to expect. Obviously things can always take a different turn and we can't know what will happen but it's good to be as prepared as possible. We felt quite concerned after our initial appointment at how concerned the dr was with the current state of William's chest, particularly with all his secretions and the amount he is likely to be aspirating. He was surprised that given William's current state he had not already had a PICU stay for a chest infection. In the second appointment I asked the dr outright what her thoughts were on William's life expectancy given our current situation. I was concerned they might say a couple of years max but thankfully she was more positive. She explained that most children like William make it to their mid teens before they start running into serious difficulties. While she can't obviously guarantee what's going to happen for William she also didn't rule out the possibility that William could still make it into his mid to late teens. Whereas we might consider a healthy person to live into their 80s, we have always known that surviving past his teenage years is very unlikely for William. We don't know what's around the corner, we don't know when William's first major chest infection will hit and how bad it will be but so long as we can keep hoping for more years then we're ok. 

Below are two videos, one showing William's apnoea and one showing William's stridor noises. I thought these might be helpful in explaining what I'm talking about.

 

Just to end on a lighter note...these are the smiles that keep us going. The smiles that let us peek at William's incredible personality and that show us we have to keep doing everything we can to keep him healthy and happy. 

22nd September 2014

There's been a lot going on in William's world recently so I thought I'd do a little blog update for all of William's loyal fans!

The past few months have been difficult ones for William. Something is bothering him but we're not sure what. He had a cold about three months ago and hasn't been himself since. He wants to sleep a lot and when he's not sleeping he's often whinging because he wants to be asleep. We've also noticed that he's getting recurrent mouth ulcers at the back of his mouth which might be causing or contributing to the unhappiness. It's been difficult to see him so unsettled and I'm now thinking we need to take more action to see what might be causing it.

William is now attending school four full days a week. Reports from school have been mostly positive so far this term and William seems to be enjoying himself. The staff are brilliant with him and very in tune to his needs. He has a lovely new teacher this year who we really like. The extra days at school seem to be making him extra tired and he often comes home very tired. We had wondered whether this increase in school time was contributing to his unhappiness but it lasted over the summer holidays so that doesn't really add up.

We recently met with William's orthopaedic surgeon following William's latest hip x-ray. Children like William are at high risk of hip dislocation because they don't weight bare so he has had his hips x-rayed regularly from an early age. His hips have suddenly moved quite a lot between his last two x-rays, one is about 50% out of socket and the other is about 70% out of socket. The surgeon was lovely and explained that the sockets in his pelvis have formed well so she would only need to break his femurs to re-set them, rather than his femurs and pelvis. However, it's a major surgery to go through and she believes it takes a year to fully recover from it, so she will only operate with good reason. At the moment William is still tolerating standing well in his frame and isn't in any obvious hip related pain so for the time being she will monitor him and reassess the situation in 6 months. Of course we are concerned that his current bout of unhappiness may be hip related but we aren't seeing obvious patterns of discomfort linked with standing/being moved/being left in one position for a long period so we can't be sure.

We are still waiting the arrival of William's new wheelchair. We are hoping it will arrive sometime in October and we are very excited about it's arrival. Here's a couple of photos of William in it from his assessment.

We are also hoping to soon purchase the specialist buggy that our good friends raised £1700 for. We have applied for the additional funding from a local charity so are waiting to hear back from them before ordering. William is also waiting the arrival of a new headrest for his home chair. We've been having increasing problems with head supports as shown in the photo. We recently trialled a headrest called the Rollercoaster and William's OT has now ordered one for his home chair. If this works for him we may look at getting one for his school chair, wheelchair and standing frames. We are also hoping to get William a P-Pod style bean bag seat (http://www.specialisedorthoticservices.co.uk/product/p-pod/).

Rollercoaster headrest

Because William is so exhausted when he gets home from school he is unable to tolerate his therapy chair. Currently we lie him on a mat on the kitchen floor while we eat tea but we'd rather not put him on the floor while we are eating. The p-pod is a more relaxed informal seat which William can relax in after school. It's not particularly liked by his physios but we need to balance his postural needs with all his other needs. Tonight we had a call from William's OT to say she has located a p-pod in their stores so she is getting it delivered to us to try to see if it's the right size. We are really hoping it is so we don't have to purchase one ourselves.

The final major thing going on for William and us at the moment is we are have been considering putting William onto a blended diet. Currently we get a monthly delivery of nutritionally complete prescribed milk which we feed William through his feeding tube. A blended diet would mean eventually taking William off this feed and giving him pureed 'normal' food. This would be a fairly big undertaking (at least initially) and would involve buying a fancy very expensive blender so it's not a decision we want to rush into. The idea was put to us by William's consultant when we had a discussion regarding the risk of gut failure in someone like William. There are no proven benefits of a blended diet but the children I know on it thrive on it, and common sense tells you proper food is better for you than formula. In the first instance I would hope to be able to manage William's secretions better and to manage his constipation using more natural remedies rather than relying on medicine. I am due to meet with the dietician this week to discuss this further. She advised me today that we would need a multi disciplinary meeting with all professionals involved in William's care before I undertake the diet, which seems crazy given it was William's consultant's suggestion. I will see how the meeting goes this week but am hoping to start very soon giving him regular food. We would start very slowly to watch out for any intolerances and to see how well William tolerated it. I love the idea that I can feed my own child with food I have prepared and chosen for him. His feeding is so regimented, he gets the same thing at the same time at the same speed every day. I think this diet could bring us a bit of normality.

Check back soon for updates!

New Chair

I wrote a little while ago about the sponsored walk our friend Michelle is doing and what we were planning on putting the money towards.

We've had a bit of a change of plan so I thought I'd write to update.

William in his therapy chair

We were considering putting the money raised towards a specialist double buggy or a specialist relaxing chair for William. We have now decided against both of these, at least for the time being. We had a second trial with the double buggy and decided it was too big and heavy to use frequently. We therefore felt it was a waste of money buying it. We do still anticipate needing a more informal home chair for William at some point in the future but he has been tolerating his therapy chair well recently and we have been shown a few things we can adjust on it when he is struggling more with his breathing.

Here you can see how William keeps his head locked to the left.

Since I last wrote we have been to back to wheelchair clinic with William to begin assessing him for a wheelchair. He is currently using a specialist buggy called a Kimba Spring but is on the verge of outgrowing it. It is also no longer offering him the postural support her needs. His head is not well positioned and he keep his head locked to one side and he needs a better harness. We are awaiting a follow up appointment to try out some different chairs. Once we receive the wheelchair the Kimba Spring buggy will have to be returned to wheelchair services. Wheelchairs tend to offer more postural support options which is why we switching from a buggy to a wheelchair.

We have a three wheel off road base that we bought ourselves to go with the Kimba Spring seat. This also turns into a bike trailer allowing William to join us on bike rides. Once the Kimba Spring buggy is returned we won't be able to continue using the three wheel base and bike trailer as we won't have a seat unit. Therefore we have decided to put the money towards another Kimba buggy.

We will get the next size up which means it should last William several more years. The Kimba Spring is no longer in production so we think we will need to get a new model Kimba Neo unless we can source a Kimba Spring. Having had a brief look at prices, weighing up all the postural accessories William will need, we predict needing about £2500 for the buggy. We will put any money Michelle raises towards this and fund the rest either ourselves or through grants from other charities.

Thank you so much to Michelle for agreeing to walk so far for our little William! And a big thank you to everyone that's already sponsored her. We are incredibly humbled and grateful. If you would like to sponsor her and help encourage her on you can at http://www.gofundme.com/69jr58.

Kimba Neo buggy

William using the three wheel base set up as a bike trailer with his current Kimba Spring seat

Brixworth Country Park

So this weekend we decided we needed a nice quiet family day out as we've had lots of visitors over the last few weeks. We did some research into where might be nice to go and decided to just go for a walk around our local reservoir. While I was looking on the website I noticed a link to a country park on a different reservoir about an hour away from us.

This is how it was described on the website:

"Developed with Millennium Commission funding, the park is a national showpiece for accessible countryside – providing access for all irrespective of physical or mental ability and social or cultural background."

"Centred on a seven-mile surfaced track, suitable for cycles and pushchairs, are a number of well maintained access routes servicing our activity centres and attractions. Brixworth Country Park was designed specifically to meet the needs of visitors with disabilities."

I read up some more online and found the park had a sensory garden, footpaths suitable for wheelchairs and buggies, a playground with some accessible equipment and a changing places bathroom (this is a bathroom designed for someone like William equipped with hoisting and a full size changing bench).

We were very excited to discover somewhere that would be a good day out for all our family so headed off yesterday with great expectations. Unfortunately we were left very disappointed. I'd been all prepared to write a great review recommending it to all my other SN mum friends but  it soon became apparent I wouldn't be recommending it at all.

First of all the sensory garden:

Rusty musical chimes

Was this supposed to be a chess board?

Mostly dead looking plants

Water fountain wasn't working

We thought the sensory garden would be a lovely colourful garden which would have lots to see, hear and smell for William. Sadly it was dreary, neglected and dead looking. It probably didn't help that we went at the end of the winter but you could just tell it wasn't well maintained at all. The hospice where William goes has a lovely sensory garden with lots of different smelling and colourful plants. There are also some lovely colourful outdoor musical instruments in the playground which would have made a great addition to this garden. 

Secondly we were attracted by the promise of wheelchair friendly paths. While there weren't steps etc the paths were actually quite bumpy which meant William's head was bobbing around as he has no head control and half way round our little walk he was sick because he had been jolted so much in the buggy.

Thirdly, while we know better than to expect any playground to be fully accessible, we were encouraged by the promise of some accessible equipment. The playground was brilliant for mobile children but there was only one swing William could have used. This would have involved us lifting him out of his buggy though so would not be suitable for bigger non mobile children. It looked like they'd given it some thought, for example there was a roundabout with more full supportive seats. However they'd completely bypassed the point that some children cannot get out of their wheelchairs or support themselves in any way. Again, the hospice where William goes has a fabulous playground. It is really accessible to someone like William (it has a wheelchair swing and a level access roundabout for pushing wheelchairs on to) but is also completely usable by mobile children. The playground was heaving and there was no way we could have put William on the swing without another child just climbing on aswell. 

Finally, we had seen there was a changing places bathroom accessed via a Radar key. While the bathroom was as promised the equipment was well overdue a service (as identified by the service label on the change bench) and there were so many spiders on the ceiling that I couldn't stay in there. Would it really be such an effort for someone to give it a sweep out once a week? 

We had been so excited that someone had given some thought into the design of this country park, but as is usually the case we found it was poorly maintained and poorly executed. How was this a park "providing access for all irrespective of physical or mental ability"? It wasn't "accessible" to William at all. It has become apparent that we as a nation we are terrible at understanding what the term 'disability' can include. At a local shopping centre to us they have around 10 disabled toilets, if not more. Not one of those toilets had a change bench for someone like William meaning he would have to be changed on the toilet floor. We seem to think all disabled people are capable of sitting on a toilet and of getting onto that toilet with just the help of a handrail. 

It makes me so sad that on Children in need night the country is so compassionate towards children like William, giving generously to support charities such as the hospice which supports our family, and yet the rest of the time we don't give a second thought to meeting the needs of people like William. We spend so much time trying to think of fun places to take William to and always come up with a blank. It would be too dangerous to take him to a soft play centre, local swimming pools are too cold with poor change facilities, he can't sit on any rides at the theme park, he can't go on any of the equipment at the local park, he can't see any of the animals at the zoo, it's near impossible to push his buggy on the beach etc etc etc. 

It's just so frustrating.

Equipment

You might have seen on facebook that one of our good friends, Michelle, is planning on doing a sponsored walk in order to raise money for some equipment for William. She will walk from her home near Kettering to ours near Huntingdon, a distance of around 35 miles.

We thought it would be good to write a little about what we would put any money raised towards. There are currently two main pieces of kit we are considering.

The first is a specialist double buggy (as we are expecting our second child any day now). William is too big and too floppy to use a standard high street buggy of any kind. We've had a good look around and considered many options but there isn't anything suitable on the high street. We then found a buggy called the Activate tandem which is the only thing like it available in this country. It is a double inline buggy with a specialist fully supportive seat at the front for a child like William and a 'normal' seat at the back for another child (http://www.activateforkids.co.uk/tandem.htm). 

We had a trial with the buggy a couple of months back and really liked it (despite it being bus like in proportions). However because it's a specialist piece of kit it comes with a hefty price tag of £3500 (including all extra bits we need). We have applied to a charity for funding and are currently waiting for a response but even if we are successful in securing funding we will still be required to self fund 20% of the cost. We are therefore unsure at the moment whether we will proceed with this depending on several factors.

One of the factors affecting whether we will proceed with the buggy is that it has become apparent that there is another piece of kit we need more. At home William has a specialist chair by JCM called a Triton.

Here you can see William in his chair. It offers a lot of postural support and is a great chair but we have two main problems with it. Here you can see William with his head flopped forward. He doesn't usually sit this badly but on certain days it is a big problem and we are left with no option than to take him out the chair. We are currently starting to look at some other head support options and will be starting with a trial of a neck support called the Hensinger. If that doesn't work we may need to revisit the possibility of getting an i2i head support but these are expensive and complex pieces of kit. Any additional head support items needed will be funded through our OT.

The second problem we have with the chair is regarding William's breathing. In the last few months we've noticed more and more that William can struggle with his breathing when sitting up, especially when he sleeps sitting up. A few months back we had an appointment with the respiratory paediatric specialist at our local hospital who advised that as William's difficulties are for relatively short times during the day (as he is usually fine when asleep at night in bed) and given that he has many other problems there was no need to take further action at this time. What it seems is happening is that William has an obstructive apnea occasionally when he sleeps sitting up, probably caused by a floppy airway due to his low tone. 

We would really like an alternative chair for William on days where he is having more difficulties with his breathing or when is head control is poorer. At the moment the only other alternative is putting him on the floor, which he does like but which isn't great when he's stuck there all day.

There is quite a big market for specialist chairs for people like William which are more like living room arm chairs. They offer a more comfortable relaxed type of seating while still offering good postural support. If we had a chair like this we could recline it more to help William on his more difficult days. A greater recline would help him with his breathing and reduce the need for him to support his head.

We currently have a short list of five chairs that we are looking into and possibly going to trial. If you are interested they are the Kirton Duo Mini, Leckey Comfee, JCM Jupiter, Symmetrikit chair and P-Pod beanbag seat. Each offer different advantages and disadvantages so we will need to trial them to see which works best for William.

At this stage we do not know if we will need to pay for this new chair ourselves. As we already have a home chair which has been funded through the OT we are not guaranteed funding for this one. The chairs vary wildly in cost with the most expensive coming in at £3500. The P-Pod is the cheapest chair by far coming in at around £500. However this is probably going to be the worst option posturally and therefore not necessarily the best option. Deciding which will be best will depend on what suits William, what fits in our living room and what we can realistically afford if we don't get it funded. 

We plan to put any money Michelle raises towards one of these two pieces of equipment. In the case that the chair is funded through the OT and we don't get the buggy (because we are turned down for funding) then we will have a think what would be the next thing William needs. Rest assured there are always pricey pieces of kit that we will need to pay for just around the corner. As we begin to think about moving William from his specialist buggy to a wheelchair we may decide to purchase our own specialist buggy as an alternative to the wheelchair as they are generally better on rough terrain.

Bubbles for big William

When William was born we read that children rarely live past 2 with his condition. We also read that the number of people born with lissencephaly was so low we didn't think there would be anyone else living with the condition for miles and miles. 

I remember distinctly one of our first meetings with Williams neurologist discussing the prevalence of lissencephaly and the moment she told us she had another patient living locally with the same condition and that he was in fact 16. It was a significant moment for us, it made us feel a little less alone, it gave us hope for more than 2 years and it gave us confidence knowing that this was a road our neurologist was already on with another child. 

Over time we learnt extra little pieces of information about this other family living with lissencephaly. Their child was a son also called William. From then on he became known to us as big William and our William became little William to their family. We learnt we shared the same occupational therapist, our VI teacher had worked with him in the past and the nursing team who comes in to provide respite also looked after him. Another friend of ours told me one day she had met him on the children's ward and reported what a lovely family they were. We were so keen to meet the family but unfortunately big William was having a rough time with his health and it was just never the right time.

Both the Williams were known to be great sleepers, they could both fall asleep anywhere. They seemed to share so many characteristics. Both of the boys are well known on the local children's ward and at the hospice and we'd recently been discussing with the nurse how we couldn't keep calling our William little William as he was getting so big, and maybe he'd have to become known as long William!

This week we got the devastating news that big William had passed away last week after a short illness. We are sad we never got to meet him but we feel like we know him in our own special way. We have cried many tears this week for his family, for all the people that loved him and for ourselves because we know one day it will be us walking that horrible path. 

Today is his funeral so this afternoon William and I have been in the garden blowing bubbles to big William. We want to say thank you for the hope you gave us for little Williams future, for walking the path before him and helping us to know where to go. We hope you're now free from the ties of this horrible condition and we hope one day little William can meet you and you can run together. 

On a cheerier note...

Things aren't always rubbish....

Difficult days

I wanted to write about what makes some days with William really hard.

It's been a difficult month. After several months of exceptionally good health we've had a string of little things going wrong. His seizures have been still amazingly controlled but he suddenly started suffering with severe constipation which is common in children with neuro problems and children that don't move. He then caught conjunctivitis. After previously having had a serious eye infection we are understandably nervous about his eye so we were watching it like a hawk. The constipation continued, we started a new med, but the only way he would go the toilet was after a suppository. He then started being more sick and his gastrostomy got infected so we got him checked out and he had tonsillitis as well as a gastrostomy infection. He was started on antibiotics which got rid of the tonsillitis but gave him terrible diarrhoea and bad nappy thrush. They then discovered the antibiotics he was on weren't the best ones suited to the infection in his gastrostomy so after a week of the first course we are now on a different second course for a week. The new antibiotic is making him sick, after every dose, but the diarrhoea hasn't set in so we are back to constipation.

So none of these things are really particular to child like William but the difference is the effect on me.

We don't get out a lot in general. We go to some special needs children groups, we pop to the post office, but there's not a lot else to do. Not much point going to the park, there's not much point going anywhere, William can't see, he's not usually too fond of being in his chair and in general would rather roll one the floor at home. On top of that he's heavy and awkward to move. He also has a cough/gag that scares people, a lot, so taking him out in public gets a bit uncomfortable. When he's ill we don't really go out at all. Not necessarily because he's too ill, but because I don't want to spread infection to the other vulnerable children we see and because if managing out the house was a challenge, managing him with sickness and diarrhoea out the house is really difficult. Most places have no where to change him, I would have to lie him on the floor in the disabled toilet.

Add on top of all this the anxiety problems I've been battling of late, and the motivation to do anything is small.

I don't know what it's like having a healthy child, but in my head if I imagine the last month with a healthy two year old, we would have at least managed some trips to the park to get out. We could have done some painting, crafts, playing of many varieties. They'd have kept me busy and if I'd have needed a break I could have left them with nanna and grandpa. Maybe that's too idyllic.

When Williams ill there is nothing to keep us busy. I could paint with him, I do paint with him sometimes, but can you imagine what it's like to do that with someone who shows no enjoyment, no recognition what they're doing. We can play with lots of things but it gets very boring very quickly. William barely interacts with anything, he smiles away but most the time he would be smiling if he was just left on his own to play. I might do an activity with him but at the end of it you can't help thinking, what's the point.

So we got to yesterday, I was fed up from not being out, I needed to do something for my brain. William had been much better and was booked to go for two nights at the hospice. We were going to go shopping in Cambridge for the afternoon. Nothing major, but I was really looking forward to it. It was something! Yesterday arrived and he slept nearly all morning, never a good sign. Then he starts vomiting more and coughing more and he does his poorly face. So we cancelled his respite. They would have taken him, they would have managed fine I'm sure but I know from experience that leaving him when he's poorly does not make for an enjoyable break for me.

You see with William, any cold could become a chest infection and a chest infection could become a bad chest infection and it could kill him. When he's poorly his seizures often worsen. At any point he could have a seizure that would kill him. One of my biggest fears is him dying and me and steve not being there. I know it's unlikely that he would deteriorate so rapidly that we wouldn't have to get to him but I feel like when he's ill, as his mum, I know exactly what things to look out for. I know his indicators that show he needs treating sooner rather than later. I know how to manage his feeds, his positioning to try and minimise the damage done by be illness. This isn't stuff I can teach to someone, this is stuff I know from being with him all the time through every illness, it's my instinct. When he's poorly I feel he needs to be with me.

So yesterday happens and all I was hoping for was a little shopping trip out and I feel like that one little bit of something for me is taken away. And this has happened so many times before. There can be so many wonderful respite services in place but often using them is hard. We use them because I make myself, I know it's good for us, but with a child who can't communicate what he wants and relies on you to figure it out, leaving him with anyone who knows him less than me is hard.

We've been thinking a lot about having another child. It would be hard when Williams poorly to have someone else to think about but I think it would do us good. Someone to make us want to make the effort to go out and do things because with them there would be a point to it. But it's really really scary. What if they have the same condition as William, what if they have something more complex, what if they need more hospital treatment. I seem to have stumbled across a lot of news stories and blogs recently about healthy children dying unexpectedly. What if this happens. Will I ever stop worrying about something happening to that child. I often describe living with William as living on the edge. We cope with it, we often cope well with it but I feel like we couldn't cope with anything else. So I worry a lot about Steve dying, about me dying, about a grandparent dying, about something happening to us that would make us unable to look after william, about Steve losing his job, and about something happening to other children we will hopefully have.

I believe everyone in life has their struggles, some are harder than others, often we feel like ours are bad, maybe the worst. I don't think ours are the worst, but they are hard. There's not a day goes by where I don't think about what it will be like when William dies, will I cope, how will I ever get over the pain of missing him. While death is a certainty that could happen at anytime, I doubt many of you with healthy children face the reality of daily worrying when your child will go. I'm sure it crosses your mind, but I carry that all the time and it never goes away. Add on to this the sadness of knowing william will never say my name, never look at a toy and play with it, never walk, never sit up, never enjoy food, never put his arms round me and give me a big hug, never do any of those things that you might take for granted, and it makes for a whole lot of sadness some days.

I'm sorry this is a sad miserable post but I needed to say it.

A re-vamp!

You might notice I've given the blog a bit of a facelift! I was getting a bit bored so have changed a few things around but it's still basically the same!

I've been wondering for some time about the point of the blog. I read quite a lot of other people's blogs, mostly blogs written by families in similar situations to ours. A lot of those blogs are really lovely and well written and I couldn't see the need for another blog on a similar vein. But then I realised I only read those blogs because I'm interested in what they have to say because we're in similar situations. Many of the people that know us won't know other people in similar situations and therefore probably don't read any other similar blogs. So the long and short of it is that I'm going to try and update the blog more often to try and offer an insight into life with a disabled child to those that wouldn't read any other similar blog. I'm also going to try and use it to give information on William specifically to friends who are keen to understand more about him as an individual. 

Recently we've been trying to understand more about how best to communicate with William. We've started having input from the charity Sense who work with deafblind people. It's really made us think differently about how we communicate with him. Before we had William we wouldn't have had a clue how to start trying to communicate effectively with someone like William. Sometimes I take a step back and look at William and try to see him how other people might see him. I see this floppy boy, eyes rolling in his head, not focussing on anything, often making no sounds. Maybe when you've seen severely disabled children this is all you see. Let me tell you there's so much more there and when you start understanding the reasons behind their actions it starts to make more sense and you start to see past their seemingly weird behaviour. Have you ever seen a disabled child being pushed down the street and they've got a look on their face that says 'nobody's home'? They might have their eyes open but they look completely out of it, like there's nothing going on inside. Well did you know that for children like William with brain disorders, it takes so much more time and energy to process what is going on around them. When William is out and about there are so many new sights, smells, noises, people, etc that it can become overwhelming for his brain to try and make sense of it all. It's tiring for him and so often he just switches off. There's too much for him to make sense of so he closes down. 

William has a visual impairment called cortical visual impairment which I'll talk more about another time. We spend time, using light up toys and his ipad, getting William to practice using his eyes. When we do this we try to remove all other stimuli. We turn the lights off and we don't talk to him. This helps him to use all his brain power to focus on trying to interpret what his eyes are seeing. 

Next time you see a disabled child, try and see past the obvious and through to the little person in there and spend time getting to know them. They have personalities too, likes and dislikes, it just takes more effort to figure them out!

Many of our friends are wonderful at getting to know William. They ask us questions about what he likes and dislikes and ask us about his different responses. But there are still some people who to be honest seem a bit scared of him! I know this comes from a lack of understanding and they simply don't know what to do. I don't blame them, I would have been the same before I had William! So here a few pointers in communicating with William. I'll add to this over time and it'll hopefully be helpful. Feel free to ask any questions about specific things you are interested in.

1) When you talk to William it's good to touch him, because he probably can't see you. Perhaps stoke his arm, hold his hand, stroke his cheek. Some children with disabilities don't like being touched, especially if they have sensory processing disorders  William doesn't mind being touched at all but it might be a good idea to ask the parents/carers if their child will mind. 

2) Don't feel the need to chitter chatter, speak clearly, focussing on the key words. You might say: Hello William, it's (your name). You could repeat your name a few times to help him to remember who it is. You might choose to always greet him in the same way to help him too. 

3) If you are speaking to William in a noisy, busy environment you can get close to his ear to help him to hear you better.

Birthday Blog!

Well we've reached the grand old age of 2! Hurray! I thought I should write a little blog entry about the significance of this birthday.

When we first received William's diagnosis of Lissencephaly at about 22 weeks into the pregnancy one of the first things we did...google it! One of the first things we read was this little gem from wikipedia...

"Lissencephaly, which literally means smooth brain, is a rare brain formation disorder caused by defective neuronal migration during the 12th to 24th weeks ofgestation resulting in a lack of development of brain folds (gyri) and grooves (sulci).^[1] It is a form of cephalic disorder. Terms such as 'agyria' (no gyri) or 'pachygyria' (broad gyri) are used to describe the appearance of the surface of the brain. Children with lissencephaly are severely neurologically impaired^[2] and often die within several months of birth."

However, when we read it the last bit said, children rarely live past the age of two. We soon learnt it wasn't that straight forward. Children like William rarely die from their condition, but from complications associated with it. In William's case the biggest threats to his life are chest infections and seizures. Either of this could become major problems at any point.

However, from the moment we read that we had the age of two stuck in our minds. And now here we are! We feel so happy that William has done so well and reached this wonderful age with relatively few major complications

Many of you will know we're throwing a bit of an extravaganza of a birthday party at the weekend for William. The theme is rainbows. I partly picked it because rainbows are so good to look at but I also picked it because in the Bible God sends a rainbow as a symbol of his promise. 

When Steve and I got married there was a particular Bible verse that kept popping up: "For I know the plans I have for you,” declares the Lord, “plans to prosper you and not to harm you, plans to give you hope and a future." (Jeremiah 29v11). Throughout our pregnancy with William and the difficult times we have faced since it's been so hard to keep hope in that promise. We know our future won't always have William in it and it seems so hard to see how we will get through that. I think I chose rainbows because God has brought us this far and now as we face an uncertain future we need to remember God's promises, that he gives us hope and has a future for all three of us.

Two years ago we were in hospital having just had William and I felt so overwhelmed, uncertain and scared about what was ahead of us. We had a tiny little baby who had crashed into our world, turned it upside down forever and who was very scary to look after! All the things we had thought we'd do one day as a family had to be re-thought. I always used to say I'd take my children to disney in florida one day, that was one little thing that was probably not going to be possible. At the time I felt resentful, hard done by, and miserable. 

Over the last two years we've gradually got to know William, his care needs have become second nature to us and we've been able to see his little personality grow and focus on him as a person. We are so privileged to call him our son, he has taught us so much about what matters in life. We have met many many amazing people through him. There have been some very hard times, some very happy times, a lot of very sad times but we wouldn't change it. He is one of the happiest people I know, he puts up with a lot of horrible stuff and yet he still smiles. 

So Happy Birthday to our beautiful boy William, every day with you is a blessing and we pray for lots lots more!

Wednesday 10th October 2012

There's a few things I've been musing over lately that I wanted to blog about, but firstly a quick update...

Since I last wrote William has started being sick again. It started with a cold a few weeks ago. The specialist nurse at Addenbrookes has advised this is not unusual with a bug to be sick but if it doesn't die down we can have an x-ray to see whether his nissens has failed or not. The sickness has died down and is no where near pre-op levels but there is still some sick so we'll have to watch this space. Initially we were really upset that the nissens might have failed but now we're just really hoping that the sickness dies off again.

We had a lovely holiday at the start of Sept at Center Parcs. We were really pleased to see other families with disabled children there and in general the place was really well set up for William. We'll be definitely going back and we were very excited to find somewhere close by where we can easily go for a good holiday. For those not on facebook here's our holiday photos: https://www.facebook.com/media/set/?set=a.10100515670026928.2667190.199702150&type=1&l=ca6ea05465. We also had fun making our own music video while we were there! http://www.youtube.com/watch?v=g_deTKZZWhw&feature=g-upl.

In general William has stayed well apart from one cold which is brilliant. It'll be interesting to see how he gets on over the winter months but he's started off well. In the last 6 months he spent one week and then one night in hospital. If he makes it through the winter with that few stays I'll be mighty impressed!

Things are slowly moving along with the house adaptations and the first set of plans have come back and been reviewed and sent back. I'm really hoping the work will begin within the next 6 months. We're still waiting to hear back from the geneticist on the latest gene test carried out. At the moment we're not feeling hopeful that they'll ever identify the faulty gene, which basically means if we go on to have more children we'll have no idea whether they're at higher risk of having the same condition and we wont be able to find out if they do until around our 20 week scan, in the same way as we did with William.

Here's a link to some of my other recent photos for those not on facebook: https://www.facebook.com/media/set/?set=a.10100174233932598.2595430.199702150&type=1&l=8249cc6671 and https://www.facebook.com/media/set/?set=a.10100538570638918.2673349.199702150&type=1&l=7d31a6f682


I've been thinking a lot recently about taking things for granted in life. Ever since having William, one of my biggest bug bears is people thinking they are entitled to things in life, to good health, to healthy children, to material stuff. Having William and meeting many of the other families we meet in similar situations has made us realise how insanely wonderful it is that most babies are born relatively totally healthy. It seems a miracle that most babies are fine while only small numbers have complex problems considering all the hundreds of things that can go wrong. I adore William with all my heart but there's still a big part of me that would love to have the experience of having a healthy child, one that will hopefully learn to walk and talk and call me mum and say funny things, and play with his/her big brother. At the moment we have several things in the way of this happening, we aren't in a position at the moment to choose to have another baby. I have friends and friends of friends who have faced multiple miscarriages, read of families having multiple profoundly disabled children and having more than one of their children die. If you're one of those people who has never had to face any of that or who has a healthy child, don't ever take it for granted, consider it a miracle every day that you have them. William is a wonderful gift to us who I will always be thankful for...which sort of leads me on to my next two points.

This evening we visited the fair with William in town. We had a nice time walking around and even took William on a ride. What made the evening enjoyable was as we walked around William had a slight smile on his face the whole time. We're so used to walking round with mr grumpy face/mr nobody's home that it was lovely to take him out when he actually looked like he was enjoying himself. It made the whole experience much more enjoyable. And then I thought if it's so nice taking a vaguely happy William to the fair what must it be like to take an excited, able to go on the rides, regular child to the fair?!

Secondly, earlier today I was looking at William as he lay on our bed, again in a vaguely happy mood, and I thought isnt it amazing how much I love him despite the fact that he rarely does anything to make me love him. He sometimes offers us brilliant smiles and quirky laughs but a lot of the time he doesn't really do a lot, and in fact some days he does absolutely nothing. And yet I love him with all my heart. And then it made me think of God and how He loves us unconditionally, not because of anything we've done to earn that love, but because we are His children who He made. And for the first time today I saw that link and understood a bit how God's love for us works.

Generally other children dont play too much with William, hes pretty boring to be honest to them. A couple of weeks ago, however at play group, a little girl who has a bit of a soft spot for William spent a good half hour sitting with him showering him with kisses. She was so excited to be giving William kisses and it was really lovely to watch because it was a rare occasion where another child played with him. Such a small thing but I love it when it happens.

I think my ramblings seems a bit disjointed but I think I just wanted to get across that the things we take joy in over William are the things other people probably don't think about. The things that other families do all the time are rare special moments for us. Never take any good thing in your life for granted, never think you're entitled to it. If I can sit holding my fitting son who is crying at the intense spasms pulling his limbs in every which way and still feel thankful for him and his beautiful smile then I'm sure there's a lot most of us have to be very thankful for.

Wednesday 8th August

It's now been nearly two and a half months since William's surgery and still no sick! It really has made life a lot easier, a lot more flexible, a lot less washing and a lot less sick smelling.
We've recently started William on a new medicine to help dry up his secretions which has helped a little, he is dribbling less but still choking now and then on secretions, but he manages to clear it so it's no big problem. I mentioned last time that William had been sent home with a Farell Valve, well I got fed up of it and didnt think it was doing anything so agreed with some of our team to try William without it and he's been fine which is good and saves a lot of fuss.

A couple of weeks ago we went to Activ8 with William again. We had a great week and William was really good, especially as he can't be sick now! He went swimming twice and even managed to fall asleep in the pool. Here's a link to some of my photos from the week:

https://www.facebook.com/media/set/?set=a.10100483579147308.2655508.199702150&type=3&l=8dc32d171a. The children who come on the holiday really love to hang out with William. They ask lots of questions about him but aren't phased by anything, they just accept him as he is. The childen loved singing and playing with him and it was really lovely to see. Some of our friends joined the team this year and bought their two lovely girls. Their youngest, Charlotte, is just six weeks older than William and has always seemed to have a soft spot for him. While we were away she would often go over to him and stroke him or do 'round and round the garden' on his hand. One time she was pushing him in his buggy and William started whinging so she just stopped, walked round to him and started stroking his cheek, it was so lovely. Another time we were singing with the children and doing actions so I had William laid on the floor and was moving his arms to do the actions. Charlotte came over and just took over from me, moving Williams hands around and clapping them together. It was such a lovely moment and I absolutely love seeing other children engage with him. Often I find the yougest children are the most natural at interacting with him, it's a shame we lose some of that as we get older.

Chilling with Pheno

William also attended the dentist for the first time the other week. He has to attend the dentist at the hospital as they are used to dealing with similar children. It was just a check up but William managed to sleep through the whole appointment, despite the dentist having a look at all his teeth!

I find these days it's much harder to know what to write on the blog, hence posts being less frequent. When William was younger things changed quickly, we had a lot to get our heads round very quickly and it was pretty overwhelming but now things are much calmer. However I wanted to write about a book I just read. It was lent to me by Steve's mum and is the story of a family who had three children. The first was diagnosed with a brain tumour at only a few months old and given 2-6 months to live. She lived to be 12. Their second child had severe development delay and had the mental age of a 9 month old. Their third daughter was born 2 and a half months early but went on to be ok despite initial fears she might also be disabled in some way.

The book has a short chapter written by the dad at the beginning that I really related to so I've tweaked it a bit to be true for me. The book is called Hurting Hope, by Charles and Joanne Hewlett.

Sometimes being a mother isnt what it was meant to be.

I thought motherhood would mean helping my son take his first precious steps. Instead, it's taking him to clinic to be assessed for a specialist pushchair.

I thought it was mum's job to take those adorable, if embarassing, photos to show at his 21st birthday. Instead it's taking photos so that after he dies I won't forget what he looked like.

I pictured myself standing over my child's bed as he slept, thanking God for his precious life. Instead, I look out at him from a mind that is numb from questioning God: 'Why?'

I imagined going to school interviews to hear from the teacher how my child was progressing. Instead, I am discussing EEG results with the neurologist to see if there was any change in his fits.

I dreamt of the joy of watching my children grow into healthy adults. Instead, I worry about him getting bigger and how we'll manage to move him around.

I thought I would be arranging family holidays in the sun. Instead, I'm worried about travelling out of the county in case he suddenly falls ill.

I imagined being the coolest mum in the world when my children's friends came over to play. Insetad, I am explaining to other children why William doesn't play with them.

I thought motherhood meant being there for my son when he got older, helping him to make wise decisions in life. Instead, it's wondering where I'll find a private place to change his nappy when he's 10.

I thought motherhood was being at my son's wedding seeing him marry the woman of his dreams. Instead, it's beginning to put together in my mind an order of service for his funeral.

Sometimes, being a mother isn't what it was meant to be.

While this is so true, as I typed it out I realised how depressing it sounded. It made me realise how far I've come with my attitude towards William. When we first had him everything seemed hard and scary and life seemed to have much less to look forward to. I think over time two big things have changed. Firstly we've got to know William. We've got to know more of his personality, seen him develop in teeny tiny ways and fallen in love with his smile. Our love for him has grown so much that he feels every bit worth all the difficultness. Secondly, we've just got used to it. There are many many things we can't do that we wished we could but we just look for the things we can do. William will never learn to ride a bike but we're looking at ordering a special frame for his buggy so we can tow that behind a bike. We'll probably never be brave/stupid enough to take him on an airplane but we can holiday at Centre Parcs and still have fun. We might find it difficult to get out the house at times but it's those days where it's just me and him where he seems more comfortable and gives me the most smiles.

Of course there will always be sadnesses when we think of all the things he won't do and we wonder what he'd be like but William is William, he is who he is and he's so amazing. He has taught us so much and introduced us to so many wonderful people so what's the point in dwelling on what will never be. Take joy in the things you do have, don't miss out on them by dwelling on what you don't.