The Weston Family Blog: January 2011

Monday 24 January 2011

A Hectic Week.

Goodness! It's only Monday and the Weston household is exhausted! I'd forgotten how demanding toddlers can be; Romilly is very clingy and wingey at the moment, especially at tea-time when I am tying to cook. Romilly always wants food and wants it NOW! She's also still waking in the night for milk which is REALLY frustrating. If we stand firm and try to settle her without, she'll scream for hours and we just can't do it and go out to work the next day so I'm probably defying every baby manual ever written and just going with the flow. I'm guessing she'll give up wanting milk at 3 am at some point (I'm just hoping it's sooner rather than later)!

On a positive note, Marcus' sleep has dramatically improved. After 4 years of waking numerous times in the night he's even managing to sleep right through the night WITHOUT his sleeping drug, which is a huge step. Marcus slept the first 2 years of his life away, I just don't think he had the energy to stay awake for long. However, when we weaned him off his 20hour jejunal tube feeds, he would only take milk when he was relaxed and sleepy and this lead in turn to nocturnal feeding habits which persisted for about four years.

Marcus has been very enthusiastic about his eating this week. He's sat with us at every meal and I'm certain that slightly larger morsels of food are passing his lips. I am especially impressed by his love of carrot cake. He loves to lick the icing off (then I finish the cake off).

We finally took Joely to get her ears pierced yesterday. Our eldest daughter is such a good girl and such a help to me and this is a small reward for her. She often gets Marcus dressed for me in the morning and she is always willing to fetch and carry when required. We almost always have to place Marcus at the heart of any decisions we make for the family: holidays, days out and other activities. Joely always seems to accept these with understanding and without question. Growing up with a disabled sibling certainly has made Joely a very mature and sensitive young girl and I am so proud of her. Romilly seems to be following her big sister's lead and she has begun to 'help' me change Marcus' nappies and likes to wash her big brother's face and smother him with kisses. However, when Marcus reciprocates with a big heart-warming bear-hug, she moves like lightning to avoid his clutches.

Life is always hectic but never boring in the Weston household. At the moment, the children are all tucked up in bed; my favourite time of day when Gary and I can finally enjoy a hot cup of tea and chat about our day without interuption : )

Monday 17 January 2011

One bite leads to another ...

Hello all,

Marcus recovered well from his operation on Friday, thank you for all the good wishes and messages. It rained in Richmond for most of the weekend and with Gary working, we had a pretty quiet one.

On Sunday, I took the kids to Wackadayz (indoor soft play area) to burn off some energy. We met some friends there and everyone had a lovely time. We ordered some lunch and Marcus made it very clear that he did not want to be excluded from the meal, "Box" he asked me very clearly. "Box????" I hear you ask. This means Happy Meal or one of those cardboard sandwich boxes which come filled with goodies. Marcus generally loves the colourful box more than the food but at least I can get away with buying a box and feeding Romilly with its contents!!

So, I bought Marcus a children's lunch box and guess what? He opened his sandwiches with great enthusiasm and chewed small pieces of bread before spitting them out. He then moved on to the apple and took a small bite of that too.

Video link - (you'll need to copy and paste this into the status as I can't work out how to do it properly)

http://www.youtube.com/watch?v=POZbFh_sIT

I have also been persevering with the powder but I'm struggling to get Marcus to put the food on his tongue - I have to be very firm with him. He prefers to purse his lips and wipe the powder onto his lips. On Sunday morning he gagged and vomited after I put some banana Nesquik powder on his tongue. This is such a frustrating journey. How can it be so hard to eat??????

Friday 14 January 2011

A Minor Operation!

It was an early start for the Weston household today and thankfully all the children decided to sleep all night, for a change! We had to take Marcus to the Freeman Hospital in Newcastle to have his remaining grommit removed from his right ear because he's been suffering from repeated infections. The alarm sounded at 6.10am, a full hour earlier than normal for me and it was a rare treat, only having to dress one child as Granddad stayed last night to help by looking after the girls today.

We arrived at the admissions desk just after 8am and were directed to ward 11, to our usual spot! We've been to ward 11 on more than one occasion. Within a short space of time, we'd had a chat with the anaesthetist, the surgeon and the sister; Marcus was weighed and measured and on with the robe. Miss Inglis, Marcus' teacher from St Mary's, had kindly lent Marcus a little bag of toys and books based on a hospital theme so after a little play and a fiddle with the TV, it was soon time to take Marcus to theatre.

The walk to theatre always seems to take an age and it doesn't ever really get much easier. We had another wait in the waiting room outside the theatre and another two rounds of the same questions: Name? Age? Date of birth? Do you understand why Marcus is here? Any allergies?

Marcus Weston?

It was his turn, only one of us could go in with him while the anaesthetic was administered - I decided it would be me. Marcus gave Gary a kiss and a hug and I took him through. Marcus was so brave while a canula was inserted into the back of his hand and after a little cry he was soon asleep. I didn't linger long enough to look at his lifeless body, the anaesthetist promised to look after my little man and I hot-footed it back to the waiting room where Gary was sitting with another little boy and his mother. I swallowed my tears and we headed for the hospital restaurant to wait.

About forty minutes later, we were called back to theatre and as we headed through the door, I could hear my little man wailing. He has a fine pair of lungs on him when he's cross - luckily, it doesn't happen so often these days. Marcus was very upset for about forty-five minutes while he recovered from the anaesthetic and thankfully, the doctors gave him some anti-sickness drugs so we didn't have a repeat performance of last time. The grommit had been removed and his ears received a good clean.

Shortly, the nurse finally arrived from ward 11 to take him back on the trolley, he perked up and seemed to enjoy the ride back. Back on the ward, he improved dramatically once we'd switched the Patient-line TV on for him and he was soon back to his normal cheery self.

After a lunch of mushy peas and Paediasure, armed with a course of antibiotic ear-drops, we were set free around one-thirty. We made it home in time to go to St Mary's weekly celebration assembly; after which, Marcus went to see Miss Inglis to tell her all about his exciting day.

Wednesday 12 January 2011

One small step forward ...

http://www.facebook.com/video/video.php?v=10150161791363835¬if_t=video_processed

Oh! My! God!

Marcus got off to a roaring start!!

When I finished this morning's post, I went straight to the Co-op in search of powdered food-stuffs and I bought cinnamon, garlic salt, Nesqik milkshake powders, icing-sugar and ground almonds. We had a busy evening planned but I couldn't wait to get started. Have a look at the video to see what happened.

http://www.youtube.com/watch?v=lbuSe0XKP8M

For along time now, I've been certain that Marcus WANTED to eat but wasn't ABLE. We've spent a long time encouraging Marcus to eat puree because that's how you wean babies. However, small amounts of fine powder though seem safer, they offer strong tastes without needing too many oral skills to swallow them. It's so simple!!

I am one VERY happy and optimistic mummy tonight!

We're BACK!!

Hello Everyone!!

After a very busy couple of years expanding our family, I've decided to start up my Blog again. The Westons have been VERY busy over the last couple of years and the blogging has had to take a back seat. The reason I have been inspired to pick it back up again is because I have been inspired by one of my CFC parents/friends from 'across the pond' to make a radical attempt to help Marcus eat solid food.

Marcus making terrific progress in lots of ways; he's walking now and making great leaps in his communication skills. Eating solid food is still a great challenge that Marcus needs to overcome and there seem to be very few specialists in this field. The Speech and Language therapists at school are working hard and Marcus is keen to sample a wide variety of flavours but he has not progressed much beyond licking very tiny amounts. His sole source of nutrition continues to be Paediasure Plus with Fibre though he will now drink strawberry, vanilla and banana flavours! Frustratingly for me, Marcus will only drink his milk from certain beakers and he will only drink lying down which attracts some disapproving looks now, in public places. (I guess people who don't know Marcus see a six year old boy,lying on the ground, drinking milk from a bottle - I see a miracle : )

Anyway, my fellow cfc mummy from the USA, Becky, has a son called Jack who is about to undergo an eating programme called 'Food-Chaining'. I was very jealous because I am not aware of any programme like this in the UK but I have done some research and the premise of this process seems very logical and straight forward so I have put a plan in place and I am going to use this blog to record and share his progress. If you have any suggestions to offer; then please, please write a comment. This is a very lonely problem to have with your child because it's so uncommon.

Check out the link if you want to read about the programme.
http://cheriandlaura.blogspot.com/

Week 1

Learning Objective:
Introducing fine powders

cinnamon powder
mustard powder ( Marcus loves strong tastes)
icing sugar
hot chocolate powder
Nesquik milkshake powder
...and any other powders I can find in the supermarket

My plan is to present Marcus with very tiny amounts with some finger food that he could lick and dip into the powder. I'm going to also try to find a plate with dividers and sections. I'm also going to get some liquorice to dip in the various powders. Sounds so simple. Wish us luck!!

NB Marcus is having an operation on his ears on Friday and Granddad's coming to help us out - thanks dad!!!

I'll be back as soon as there's something to report.

Sam xxx

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Marcus' Story

Isn’t it funny, the most complicated of puzzles always seems so obvious…once you’ve discovered the solution? This was definitely the case with Marcus.
On the 17th October 2004, my friends and their children had come round to our house to play. I had finished work just three days before and was already feeling quite strong twinges but I still had almost four weeks to go before the baby was due. I was huge! That afternoon, my friends joked that I seemed preoccupied; and I was … I was wondering whether the baby was coming! My bump was so big, there was no way it would stay in there for another 4 weeks; there wasn’t any room left!

Three days later, I was in bed cuddling my husband and my newborn baby boy. We were ecstatic. He was huge: 7lb 15oz and three weeks early. The midwife commented that he appeared more mature and we noticed his face was pretty puffy and bruised. He also looked as though he hadn’t quite grown into his skin. We put this down to the fact he was early and was stuck in my pelvis for three days. That first night, I noticed he didn’t feed well, but thought little of it. The next day, Marcus was checked over by the Paediatrician who noted his droopy eyelid before allowing us to take our new bundle home.

After just three days at home, we had to take Marcus back to the hospital. He had quite a nasty eye infection and his droopy eyelid was really swollen. We were pretty concerned about his eyes; they looked really sore, very swollen and a bit wonky. Over the following two weeks, his eyes improved but his feeding was becoming problematic. I was breast feeding but knew he was not feeding for long enough or often enough. He was also vomiting pretty regularly. However, I wasn’t overly concerned until he was 2 weeks old and had still not regained his birth-weight.

At three weeks old, Marcus contracted RSV virus and refused to feed altogether and lost another 8oz - he was now four weeks old and only 6lb 15 oz, a whole pound lighter than when he was born and he looked terrible. We were desperately worried about Marcus, he’d been prescribed reflux medicine to control the vomiting. It didn’t make any difference and I began bottle feeding so I could be sure about how much milk he was receiving. Life was becoming increasingly difficult by this stage. We had to take towels with us if we went out because he was vomiting so much. I was also spending almost all day feeding Marcus, drip by drip. He would arch his back and scream every time the milk touched his tongue. It was as if he knew it would cause him pain later down the line. He started to scream when you brought the bottle near him. Despite spending every waking minute trying to feed him, he still wasn’t taking enough to grow. Marcus also slept through at night, I’d have set the alarm clock to wake me up so I could force feed Marcus - it was such a dreadful time. I’d sit in bed sobbing, why won’t my baby eat?

At this stage, Marcus was admitted to hospital where they discovered his heart problems - which are quite mild fortunately. The doctors also changed his milk, added some more reflux drugs and started naso-gastric feeding. We went home for another couple of weeks but Marcus still didn’t gain weight. The vomiting worsened as he couldn’t cope with the increased volumes we were forcing on him with the NG feeds. Going out was impossible with the vomiting and every time someone asked about his NG tube, I cried. I didn’t know why he had it, why he wasn’t growing or what was wrong with my baby.

Around this time, our GP commented that Marcus’ ears were rather low-set. I was furious, what did his ears have to do with anything? I was more concerned about his feeding, that’s why I went to see him. He said, “You can’t make a baby eat if they’re not hungry.” Great! Really helpful….NOT! I got straight on-line and Googled “Low-set ears.” I was gob-smacked by what came up on screen. Noonan Syndrome. This was the first time I’d really considered that there may be something serious and long-term wrong with Marcus. I read the symptoms: low set ears, wide-spaced eyes, loose skin, pigeon chest, heart-defects, and developmental delay. Marcus was lying across my knee. I looked at him and then at the screen…and back to him. I knew in my stomach, this was it, The GP was right.

Eventually, Marcus was readmitted and remained in hospital for another 7 weeks while the doctors tried bolus feeds, continuous feeds, experimented with milk and reflux drugs. He had all sorts of scans and x-rays to try and determine his problems but to no avail. I asked the doctors there about Noonan Syndrome - they didn’t think so, his feeding problems were too severe, they didn’t fit.
At 15 weeks old, we said goodbye to the brilliant staff at Northallerton and Marcus was admitted to the Royal Victoria Infirmary in Newcastle, 50 miles away from home. He had just regained his birth weight and the doctors there decided to perform biopsies of his gut and insert a central line so they could get some nutrition into him. His gut biopsies were normal, as was the barium study, MRI scan, chromosome studies and abdominal scans. Marcus was fed via parenteral nutrition and NG tube for the following few weeks.

Meanwhile, Joely was almost 2 and her life had been turned upside down with Marcus’ arrival. We were all missing each other but there was no easy way to manage the situation. When Marcus was transferred to Newcastle, family life became easier in some ways as we were all able to stay in Crawford House, accommodation provided for the families of poorly children by The Sick Children’s Trust. We ended up living there for 3 months.

At first, Marcus seemed to make little progress and the doctors were no nearer to determining why he couldn’t feed. We were visited by the genetics team who felt there might be a genetic reason for his problems. Again, I asked about Noonan syndrome, and they said they didn’t think his problems fitted that pattern.
After 4 weeks at the RVI, Dr Bunn struck gold. She thought it might be worth feeding Marcus into the top of the bowel, into his jejunum. It worked, Marcus gained weight, the vomiting improved and he slowly came off the parenteral nutrition. We decided it would be best for Marcus if he had a surgical jejunostomy inserted so we could take him home and feed him that way. We were so excited, we had a solution.

Our excitement was very short lived. At 5 months old, he went to have the surgery to insert the feeding tube. It all appeared to go well but later that evening; it became clear that Marcus was very unwell. His heart was racing, blood pressure was high and his breathing was much more rapid than usual. He was writhing in pain. The surgeon sat with us all night, then Marcus’ nappy filled with blood; rich, dark and red. He had a couple of blood transfusions before being taken back to theatre in the morning.

Then we waited…and waited… After about four hours, the surgeon came and explained that Marcus had malrotation and because he’d inserted the J-tube using key hole methods, the gut had knotted in two places and caused a volvulus. He felt the surgery had gone well; he’d re-situated the j-tube on the other side of the abdomen and performed a Ladds procedure to correct the Malrotation. He said we’d be able to see him soon…

4 hours later, they still wouldn’t let us see him and no-one could tell us anything. Gary and I were going out of our minds. Eventually, it all got too much and I threatened to go and find Marcus myself. The Sister took us to PICU and sat with us in an office, the surgeon, who told us the surgery had gone well, entered. I knew it was bad. I was terrified. Why had he brought us into his office? Had Marcus died? I waited for him to speak; I didn’t trust my own voice. His words are a blur to me now but he explained that Marcus had taken a turn for the worse while in recovery. He had sepsis and he was on a ventilator. “Can we see him?” I heard myself ask. I held Gary’s hand very tightly as he led me into a cubicle. There was a baby on the bed, intubated, sedated, swollen and very bruised. He didn’t look at all like he did this morning. I had to look closely at his face just to make sure they hadn’t made a mistake. “Is he going to die?” I asked suddenly.

The doctor replied in a soft Scottish accent, “I suppose the honest answer is yes, he could … but I don’t think he will.” That was enough for me; I focused on the surgeon’s faith or belief in Marcus. I didn’t think he would die either.

My Dad drove up to Newcastle that night and the three of us sat in vigil around Marcus’ bed all night. We stared at the monitors; praying, willing the numbers to reverse - willing his temperature and heart rate down. Marcus little body lay still on the bed for most of the evening, only moving when the sedation wore off. We sat watching the rise and fall of his little chest, inflated with every compression of the concertina within the ventilator chamber. His tiny body was covered in wires, tubes, canulas, blood and adhesive plaster. By morning, the worst was over, his temperature was down, his heart rate had stabilised. Our prayers had been answered.

After a week, Marcus made his way back to ward 7 and we were able to start using the jejunostomy to feed him. We did have a few teething problems but by the time Marcus was six months old, we were all back at home in Richmond.
This marked a new phase in our lives. It was very frightening at first, having a small baby fed permanently, twenty hours a day via a pump. He was attached to a drip stand at home and we had a little ruck-sack to put the pump in when we went out. Gradually we got used to it and all the people coming to our house to attend to Marcus’ needs - we now fondly refer to them all as ‘his entourage’. They’ve become friends, the faces change sometimes but their presence is ever felt. They come in the shape of the community nurse, health visitor, dietician, physiotherapist, speech therapist …

Marcus still didn’t grow too well, and the next year was a struggle. We tried many different types of milks and regimes. Eventually, we found that Paediasure worked and his weight improved dramatically but his vomiting gradually got worse again. Sometimes he’d vomit green bile up to 10 times a day, writhing and screaming in agony. We went back and forth to see the doctors but they couldn’t find a reason why he was so sick.

Meanwhile, we were trying desperately to get him to feed orally. I managed to get him to drink water and eventually, after one particularly sicky weekend, I stopped his feeds and waited. I offered him water which he took readily and then I tried offering some milk - he took it!

He has been taking his milk orally for a year now. At first he lost a lot of weight but he seems to have found his own line at the bottom of the centile chart now. I believe Marcus cannot cope with huge volumes of feed, he like little and often. He vomits very rarely now, only when he’s drank too much or is poorly. He doesn’t eat solids yet but we’re working very hard on that at the moment.
Marcus was diagnosed with CFC last November, just after his 2nd birthday. It came as a shock at first but within a few days we realised how lucky we were to know for definite, what was wrong. Knowing is far, far better than not knowing. And all the pieces of the puzzle fit together and seem so obvious now. Marcus has always itched and sweated. He has sparse curly hair, low muscle tone, ptosis, sensory processing difficulties, hearing loss, short sighted, feeding difficulties, heart defects ... The list goes on an on ...

Today, Marcus is doing really well. He will be 11 in October and his speech is improving, he can read, he's eating solid food, and he's brilliant with technology. He’s a complete joy to us and we appreciate each and every milestone all the more because we know how much effort and determination it’s taken him to reach it.

Thanks to the doctors who figured out the puzzle, we now know why and how Marcus struggled so much at the beginning and why he has so many complex problems, and it all seems so obvious now!