Visitors, Birthday, Hospital and other stories.

Hi everyone,

We had another busy couple of weeks, so a quick message, with some pictures. We actually didn't take many nice ones, so you'll just have to wait for the next update to see some proper pictures :-).
Lorraine, a friend of Louise and Noah's fairy godmother visited us, and I took some time off, so we had some time to enjoy a holiday like feeling. Louise celebrated her birthday two weeks ago (yes we haven't been updating very much), which we celebrated with a very nice diner, and some cakes.

We also had to go to the hospital, for a 24 hour observation. All went well, although no news or anything about his belly.  The outcome of the observation was that we can reduce the amount of support the breathing machine gives him. Although he will stay on it for the same amount of hours, it is a big step ahead. The support is reduced by about 25 %, and so far he seems to be doing good on his new settings.  Noah wasn't too grumpy in the hospital, but we think he prefers being home a lot more!

As I mentioned the doctors didn't investigate his digestion problems very seriously. We are very angry about this, and have send a letter to the hospital concerning this. Just to show you what we mean, a few pictures (not very nice ones unfortunately). 
As you can see his belly resembles a balloon sometimes, and taking the air out is not a nice thing to do, as it is very invasive.

Noah's belly can shrink up to 10cm when he has no air in it.

 Unfortunately Noah also caught a virus in the hospital (or shortly afterwards) so he had a pretty high fever last weekend. He had a 39.0 celsius (102 fahrenheit) temperature on sunday, but some stronger paracetamol and a good night sleep dropped the temperature, and he was a lot better by wednesday.

So we took it easy for the last few days,but manage to get out for a few nice walks. Since this is the Netherlands, the weather is sometimes a bit wet. That does not stop us though! Out in the rain!

We hope to post a nice update soon, as we are taking steps to resolve Noah's digestive problems.

Thijs

Call to all CHAOS parents

Hi everyone,

As we are slowly adjusting to life with a child with CHAOS, we are wondering how other parents go through this. We are also curious about your experiences, tips and most importantly how your child is doing. We know of one other child with CHAOS, Kalmin, in the U.S.A. and really enjoy talking to his family through skype.

Thus we would like to get in touch with you! So, please send us a mail, and hopefully we can get in contact and help each other out. We have opened a special mail address for this:

CHAOS supportgroup at gmail.com (all one word before the @ sign)

With your permission I would also like to gather some information about the doctors that have treated your child (names, specialisation and email address of the treating doctor), so any doctor with a new CHAOS child can get information from his or her colleague. This is also important for us, as Noah will need throat reconstruction surgery in the future, and this is still a very experimental procedure. So any extra information is welcome. We will of course supply you with this information as well.

Thank you very much and we hope to hear from you,

Noah, Thijs and Louise

What's the story morning glory?

Well, it feels like not much has really happened since we last updated you all but then looking at our photos we thought we could squeeze in an update.

We're still waiting to see what's going to happen with Noah's tummy. Pretty much it's been lots of pooh and lots and lots of washing!! I hope we will get some advise on the options available to Noah in order to fix his unstable tummy. I'm half afraid that no one will think about it until we're under their noses. Unfortunately we will just have to wait and see and hope that the difference of opinions has been resolved and there is a clear direction for Noah.

Until then we're working on Noahs' development with regards to his core muscles and his mouth. Happily we can report that Noah is doing great in both areas.
He's getting a lot more physically active. He doesn't crawl or do the big things but he's mastering all the in-between steps. So it's all going in the right direction. Thijs does a lot of work with him, using his own belly as a nice soft training gym! You can see in the background that Noah has a baby size yoga/pilates ball. I think that's so cute as I'm using the adult version to retrain my tummy muscles! :)






With regards to his mouth development, he now has two canine or incisor tooth, (hoektand), with the other two pushing through. (He's also grown to 86cm, he is the amazing stretchy baby!!)
The speed at which his teeth keep growing through is having an impact on Noah eating as well as all the problems with his tummy. He is showing a lot of positive signs of being ready for food. He is now occasionally bringing toys to his mouth, he sticks his tongue out and he can make lots of different noises now. He can "click" his tongue, make kissing noises and his favourite is the gnashing of teeth - it's now a familiar sound at 2am!
Here you can see his tongue sticking out, he's looking very cute in his pyjamas watching his favourite character, Makka Pakka, on the telly.


Thijs was sitting opposite Noah enjoying his "witlof met ham en kaas", or chickory wrapped in ham with a cheese sauce. Noah was fascinated and so Thijs just gave him some mashed potato with some cheese sauce. As usual the camera was handy so we took this little film. What pleased us most was that he leaned in for the food, also stuck his tongue out to taste the food and as you can see, he is getting interested in cutlery - but he doesn't put it even near his mouth yet. Oh well, every little step is a step forward.




We decided to take Noah out for dinner to a family friendly Greek restaurant in the neighbourhood. We weren't able to do this earlier as he was on his oxygen. A great improvement with the new bigger canule is that he is off his oxygen again (oxygen and the candle light that is standard in all dutch restaurants don't really go together). He was immediately interested in the free ouzo that they offer you before dinner. "Hands off kid, this one's your fathers"!! :)


A bit of tzasiki went down well but the out and out favourite was Mummy's chocolate mousse. He took it off the spoon and managed to get a bit in his mouth, but most of it ended up everywhere else......


We covered the table as a precaution, but maybe we should have just covered Noah up.
So ... ouzo and chocolate cake ... yup, he's our kid alright. No doubt about it!

We had a big scare on Sunday, not with Noah this time but rather it was the electricity supply. Our electricity shut off and we had no power whatsoever. Not knowing if this was a street problem or just our house frightened me to death as Noah was sleeping on his breathing machine and it hit me how vulnerable we were.
Luckily it turned out to only be our house and the engineer came out and fixed the problem before the breathing machine internal power unit run out, which was twice lucky as it turned out that our reserve batteries were also very low on power!!
HUGE reminder that we have to be responsible and think of all sorts of disaster and recovery scenarios. I should know this, as in another life this was part of my work. The power outage was caused by the fact that we are supposedly pulling so much power that our master circuit breaker overloaded and cut out. It's not an easy problem to permanently resolve, but for now a new fuse will fix it. I really don't want to see our energy bill in January. Gulp!!
Needless to say all of the reserve batteries are fully charged now!


The useless battery-pack on the bed next to the charger.
Noah's breathing machine hooked up to the almost empty battery pack. It cut out after 10 mins but luckily there is an internal backup of 3 hours in the breathing machine itself.


I was so stressed out I almost didn't go but I'm so glad we did. A friend gave us two tickets to see Bjorn Again, an ABBA tribute band. They were playing in a local venue and we'd been looking forward to going to the concert because we knew it would be fun. It was fantastic. These aren't great photos but they do sum up the atmosphere.

Here is "Bjorn" doing an amazing guitar solo in front of a glitter ball - does it get any more disco than this??!!
"Agnetha" & "Bjorn"
The whole group were amazing and got the crowd really into the show. A great tribute band and a great gift. A very nice end to the week.

Mental Relaxation

Hi everyone,

After a very stressfull period, people always tell you to relax. This is of course easier said (by them) than done (by us).
Something we're having difficulty adjusting too is other people organizing our life for us, which seems necessary to them but is very intrusive to us - so their well meaning advice does not always help.
That does not mean we don't listen at all to this advice, so Louise has upped her work out schedule to three times a week, and I am trying to reserve some time for my hobby, cooking.
After all that exercising Louise is hungry, and so I am ready with some, hopefully, very nice food which we then sit together and enjoy.

Louise refuses to be photographed during her excercising!!! However, we have got some pictures of some of our dinners for you. First this tomato creation, with a picture of what it is supposed to look like behind it.


And a nice fig and almond cake, thanks to zen can cook.


Served with a glas Pedro Ximinez Sherry. It was open, so we had to drink it.... At least that is my excuse and I am sticking to it.


And our wedding anniversary diner, which we starting eating before remembering to take a picture. It just smelled too good. We went with an Italian theme and Louise seemed to like it a lot!
We both plan to do some more things, (joining a choir for Louise, Aikido for me) but finding time is difficult. Visiting or seeing friends is always de-stressing as well, but we don't always have the energy.
It is possible us to hire a bit more care for Noah, but having a stranger in the house does not make it easier to relax. It's a really tough choice, sleep or privacy! You'd think we'd be used to it by now, but I don't think we ever completely will. Also, even the nurses need a bit of supervision (maybe they don't need it, but we like to check anyway. "Control-freaks-are-us"!). Plus the nurses need to be updated on the latest developments which costs us energy trying to remember everything.



On to the stuff that keeps us stressed.... and some stuff we're doing about it.

On our insistence all Noah's regular carers had a big overview meeting, to get everyone on the same plan and informed. This was a good step, as it cleared up a few things, and helped the caretakers (physio, nurses, etc.) communicate directly to one another. This means that we don't have to remember everything and pass it on between them.
A few more things came up in the meeting, one of them being a small worry from the nurses that Noah is growing very well. This may sound a bit weird, but they worry about not being able to carry him upstairs. Thus the subject of moving is back on our plate.... So much about de-stressing! We are looking into improving the stairs as they are quite slippery, but we don't know if that is enough.

After the visit to the KNO doctor (ear-nose-throat), and the decision that he needed a bigger canule we needed to wait about two weeks before it actually happened. That meant putting up with Noah looking like this quite often:

Not our favourite look, plus he was back on oxygen support and longer periods on his breathing machine. Since Friday he seems much improved now with his bigger size canule. He no longer needs oxygen (we're back to how good it was a month and a half ago), and he seems to sleep less. We hope to start weaning him off his machine as well, but lets first see how he does for a week.
About the sleeping: he now sleep on his tummy sometimes, although we don't know how he gets there, as he doesn't turn over when we are watching.
Anyway the results are quite fun to see, with the added bonus that he sleeps quite peacefully on his tummy and doesn't wake up during the night so much.



Another ongoing concern is his tummy, and how much air he traps within his bowels. His bowels are getting stretched out of shape with all of the air being trapped inside. I asked my uncle Jan for independent advice, since he is a pediatrician. He had some good ideas, and he talked to the Sophia Rotterdam hospital for more information. He then explained it all to us, so we have a bit better understanding of what is going on. In short the doctors think that Noah stomach has rotated along a vertical axis, and is not in a completely stable position.
This is not a well known disorder so they are unsure what exactly caused it, and how it influences his digestion. They think it can be anchored, in a position that causes hopefully no further problems. The problem is operating in this very sensitive area. Noah is just developing his muscles, and his breathing is obviously still not equal to a normal child.
We are still weaning Noah off his corset, but it is still very helpful when he is tired or full of wind, so he's not completely free yet. The corset helps him to have core muscle stability and something to push against to get rid of the wind. He must be completely independent of his corset before any operation, as it will be impossible to wear with stitches from the operation.

Last but not least, Noah is still not eating. One of the main reasons we suspect is that his canine teeth are now showing up. The first molars are all four through, but not completely. He seems to be a bit more interested in bringing stuff towards his mouth but he isn't chewing on his toys yet.
We are also still waiting for advice on his new food, so he is still on his milk. Since all the blood tests on vitamins and minerals came back okay, we are not too worried, but it would be nice to get this sorted.

We will have to go to the Sophia soon for a 24 hour observation period, in which we will get a private room, so all the doctors can visit and examine Noah. I will take care of Noah for that day, and sleep with him. We hope that a lot of our questions will be answered, but it will probably take some time for the doctors to process the results of all the investigations.

So a lot of work, and as usual a lot of question still open.

One last thing we both like to do is read, and we stumbled across these little quotes for you:

“The best thing about the future is that it comes only one day at a time.” Abraham Lincoln

“We have normality. I repeat, we have normality. Anything you still can't cope with is therefore your own problem.” Douglas Adams

“He felt that his whole life was some kind of dream and he sometimes wondered whose it was and whether they were enjoying it.” Douglas Adams

We hope to update you soon with some pictures of Noah, so see you soon!

Thijs

Anyone interested in a bed?

Dag allemaal, Hi everyone

Due to the remodelling of Noah's bedroom, we have a double bed leftover. Anyone who wants it is free to pick it up at our house in the next two weeks. It is an ikea bed-frame, with decent, manually adjustable slats and a latex mattress.

Vanwege de herverbouwing van Noah's slaapkamer hebben we een twee persoons bed over. Als iemand dit bed wil hebben kan je het binnen twee weken ophalen bij ons. Het is een ikea bed ombouw, met handmatig verstelbare latten bodems en een latex matras.

Laat maar horen, let us know

Thijs

Holiday disasters and miracles do happen!

Small update

Noah has grown to 84cm / 33 inches and weights 12.5 kilo / 2stone (28lbs)


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Hi everyone,

We're back! It's that old saying, "man! I need a holiday to get over THAT holiday!"

Let's just say we had a few lovely moments, so the holiday wasn't completely wasted but it wasn't the rest that we were so looking forward to. Luckily we now have our budget so we will just have to go on holiday and try again! No one can call us Drostens quitters! :)

Noah has grown an awful lot, plus he'd had a lot of examinations over a relatively short period of time, so we didn't worry too much that he was a bit tired. It turned out that he had caught a cold, so before we left and the first few days of holiday Noah was tired easily and not his happy little self. The second day we were there a nurse had blocked up his feeding tube so he had to have a new one inserted, that did not make him any happier. He was even less happy when his plaster was also messed up and pappa & mamma had to replace it with a new one. These nose tubes are very uncomfortable, we can't wait till he doesn't need it any more.

Noah had a lovely big bed, which he loved. We would put him in bed and tuck him in securely but he would still manage to turn himself around somehow. Due to Noah's cold, his canule became quite blocked with slime so we arranged a change. The canule change went flawlessly and it was great practice for the nurse. However later that evening Noah had turned around so much in bed he'd trapped his breathing machine hose which in turn pulled the canule out of his throat.
The nurses reacted amazingly quickly but it was very frightening because Noah had already turned blue (because he can't breath) and 'went away'. Thijs was amazing. He just took over and popped the old canule back in, by which time I'd managed to get his balloon connected to his oxygen and Thijs pumped the oxygen back into Noah and brought him back. It seemed to happen and be over in seconds but it affected us all for a long time. Thank goodness for professional nurses because this is pretty much our worst nightmare, what can happen if you don't get there in time.
In typical 'bengel' or 'rascal' style, not content with doing it once, he did it twice. This time the nurses were ready for him so he didn't get a chance to 'go away'. Noah does like to check peoples reaction speed and we're happy to report that a GretaBonteKoe nurse goes from 0 to 60 in 0.5 seconds. ;)

Like I said, we did have some lovely times too. Our first evening we went out for dinner with Oma, Opa and Tanty Anneke to celebrate Tanty Anneke's humpety humpth birthday, (I am of course sworn to secrecy as you can never divulge a lady's age!).
Lex told some of his 'big fish' stories, and we all had a good time.




We went packed to the gills prepared for anything the Achterhoek could throw at us! The last time we were at the GBK, Noah came back looking like a herd of mosquitos had eaten him for breakfast, lunch and dinner for a week, which they had! So this time we weren't taking any chances.
We had a klamboe / mosquito net,
Noah had a Klamboe / mosquito net,
so we expected a great night sleep followed by a long lie in bed in the morning. However we were not prepared for ...
Attila the Haan!


Yes, they had a cockerel. No, I am not joking! I wish I was. After appealing to the managers good nature, they agreed to keep the cockerel in the hutch until a reasonable time in the morning. Phew!

We took the bakfiets with us again, so Noah enjoyed a great ride around Aalten and Thijs blamed the wind for reducing his cycling speed!


Thijs needed a few glasses of wine to recover, luckily the GretaBonteKoe fridge is well stocked as I couldn't let him drink on his own. :)


Noah's daily activities involved cow watching, making music to entertain the other guests ...


and chilling out on the big bed with mamma or pappa watching Makka Pakka from 'In the night garden'. Don't worry if you haven't heard of the programme, it will be taking over the world soon, at least if Noah has his way!!

Today we were at the Sophia hospital in Rotterdam for Noah's follow up appointment with the ear, nose and throat (KNO) doctor. Noah has grown so much that he needs a biger canule again. We're just waiting until one of Noah's regular nurses is back from holiday to set up the appointment. The doctor will also set up an appointment to take a look at his throat and lungs. It takes some coordination as a surgeon wishes to piggy back on the operation to see if he can operate to descend one of Noah's testicles. This piggy backing is in order to spare Noah from two separate operations under general anesthetic, which has extra risks for Noah because of his lungs. This will give us a better idea on how his lungs and airway is developing, which is very important for his future operation.

Today has turned into an extraordinary day, so mark your calenders! Today Noah ate his first food, his first ever since leaving the hospital! I learned today exactly what makes Dutch kids grow so strong. It's the attitude of "go away with your baby food, bring on the 'uitsmijter'!" or ham and eggs with melted cheese on top to us non-Dutch! I couldn't believe my eyes. Our little boy had a bit of dipping egg yolk on a bread crust and he just ate it. Bit it off, chewed and swallowed. All with a huge grin, like this was a normal every day occurence. I had tears in my eyes but still had the time to pass Thijs the camera to film this monumentous event. So this explains why the film is in black and white! ;)



Lots of love,
The Drostens xxx

Hospital Update

Hi Readers,

We had a lot of hospital visits last week, trying to find out what was troubling Noah. On top of the discomfort of his tummy, Noah has been pretty upset with all of the procedures and blood work that needed to be taken. In turn we've been working hard and we were just so tired, so sorry for the lack of updates. There are no final results but we have an initial diagnosis of a rotated stomach in combination with his colon being very distended.

They suspect a motoric problem more than additional deformity, but they are not yet certain. Normally the intestines contract in a lovely wave movement, pushing the food along rhythmically. Noah may not have this rhythm perfected as yet.
As you know, Noah still takes air in through his mouth and nose. This cannot go to his lungs and so can only go through his digestive system. Because all of his tummy muscles are so distended, it's very difficult for Noah to get rid of gas, and we try to assist him by leaving his feeding tube open, so that the gas can easily escape.

This can be a hit and miss affair, sometimes it works sometimes it doesn't. Sometimes Noah is vomiting with too much wind and other times he's ok. Sometimes his food flows in, other times it can take up to 2 and a half hours for one feed. We thankfully have some understanding of these symptoms when we see his stomach is rotated, you can imagine a twisted hose pipe to visualise what happens when you try to put milk down the pipe.

The open feeding tube only helps with gas that's in his stomach. When it passes further into his system we need to use another method. Anyone who's ever suffered trapped wind will understand how painful it can be. Just like a baby with colic, we give him tummy massages for the cramps and if all else fails we insert a rectum canule to release the trapped wind. What the x-ray photo showed is that Noah's colon is very very distended with air. The least intrusive measure is to regularly insert a rectum canule to relieve the air and see if that helps to bring the colon back down to a normal size. More test need to be done in a few weeks, including a biopsy of the colon before we know more.

We're relieved that the doctors found a reason for all of Noah's problems, and even better if it's treatable. It's just really horrible watching your kid in pain, so we really hope that this will get sorted out sooner rather than later because it's taken so very long to get this far.

Further to this medical investigation into the mechanics of Noah's digestive system, we've also been busy with blood work to see how his system is processing his food. A dietitian is looking see if Noah has any allergies or whether he's deficient of any vitamins. Noah continues to grow in length and weight, so we don't foresee any problems but due to Noah's age he would normally move onto a different formula food. The formula 3 food takes into account that a child of 12 months has regular meals, but of course Noah doesn't yet eat. We are in consultation with the dietitian to see what's the best next step for Noah with regards to which food he should have. It's quite exciting really because this is yet another milestone in Noah's development.

With regards to Noah's development, we have to say - and not just because we're the proud parents!! - but he's doing amazingly well. He sits unassisted with really good balance. He can reach forward and come back again, which shows how well his stomach muscles are improving. We've found him frequently lying on his stomach in the morning, so he's also mastering the turn.
His arms are getting stronger as he's able to lift himself up into a sort of push-up position. Thijs has been doubling as a soft cushion for Noah, working to support him when he's trying to crawl. Noah's tummy is just too sensitive to lie on the floor, both love doing this and there's lot's of smiles all around.
We've been busy building up how long Noah can be free of his corset and this is also going very well. This means that his diaphragm can support his breathing a bit better, and he is using his core muscles a lot more to support him when he's sitting.
Noah's fingers are also getting more dexterous and we're working on his releasing of objects and fine motor control. This will come more into focus when he starts using more sign language gestures. We've also been researching a lot of sign language methods. We're very curious about the Makaton system, which is sign language assisted by symbols. This would mean that not everyone has to learn sign language in order for Noah to be able to communicate with them. A huge plus point!
This takes a lot of energy as you can imagine, from both parents and child! It's great though because Noah gets less frustrated and that can only be a good thing.

We are all ready for our holiday at the GretaBonteKoe - long live summer holidays!

xx Thijs, Louise & Noah.