Leyla's Blog

It's Been A While...

kemal.altug@ntlworld.com (Leylasdad) — Mon, 22 Nov 2010 20:44:00 +0000

Since I last posted. Tons of stuff happening - mostly not good stuff. So it may take a few posts before I get up to speed again.

Tragically, in the interim of my last post, one of Leyla's Mito friends, Samuel, lost his brave battle against this cruel and relentless disease. Our heart goes out to Samuel's family, and especially to Missy. We continue to think of you, and pray for the strength to carry on.

For Leyla, there's been a ton of procedures since we last spoke, and some tests, including an Magnetic Resonance Spectroscopy - but nothing much that has yielded any positive news.

Also, her Mitochondrial Disorder has now been re-classed as probable secondary - to an unknown Primary. To the layman that means she still has it, but something else to boot - something else that is suppressing mitochondrial function.

Notwithstanding any of this, however important that snippet of information may or may not be, her gut motility issues are big. I mean seriously big - and this is where our focus still is - namely, pain management, which just doesn't work.

I will post more later - I need to fully get back into the frame of mind for blogging.

Everything changes - but stays the same

kemal.altug@ntlworld.com (Leylasdad) — Sat, 08 May 2010 00:42:00 +0000

A bad few days in terms of pain - looks like we're no further forward than we were months ago. Still Midazolam and Chloral Hydrate is required, still fed up.

Just when I think we've figured Leyla out, she proves us wrong. Just when we think the meds may be working, she proves them wrong. Just when we think we're turning a corner, the road just keeps going straight.

Everything changes - but stays the same.

Marathon Result for Team Chase!

kemal.altug@ntlworld.com (Leylasdad) — Thu, 29 Apr 2010 18:44:00 +0000

Many congrats to James and Diane who completed the London Marathon, running for Chase Children's Hospice.

So far they have managed to raise a combined total of over £6,200. Now factor in that the place we all work has pledged to match that pound-for-pound. That's a whopping £12,400 straight to Chase Hospice. And they're still collecting too.

So, many thanks to James and Diane.

Brilliant stuff

Here's picture of a triumphant James who finished in an incredible time of 3 hours 12 minutes (haven't got a pic of Diane yet, but will post this soon!)

Marathon Weekend

kemal.altug@ntlworld.com (Leylasdad) — Fri, 23 Apr 2010 06:34:00 +0000

Yes, it's this weekend! And if you haven't yet sponsored James or Diane, both of whom are running for Chase Hospice, click on the links on the right, or the links below this message, and jump straight onto their Virgin Giving Sponsorship page. The money they raise goes straight to Chase Hospice, and if your a UK taxpayer, Gift Aid will be eligible too!.

They've done brilliantly with over £5,000 raised so far (inc Gift Aid!), but the more they can raise, the more Chase Hospice will be able to continue their work in these financially difficult times.

Oh, one more thing, the place where we all work (can't name it!) is going to double both Diane's and James's efforts - so a further fantastic incentive if ever there was one!

Good luck James and Diane, and God Speed!

Click here for James's sponsorship page

Click here for Diane's sponsorship page

Startle Reflex

kemal.altug@ntlworld.com (Leylasdad) — Tue, 20 Apr 2010 07:10:00 +0000

is back and I am curious as to why. Even soft sounds will result in a dramatic and exaggerated startle. But the feature is strange because literally 2 seconds later the same noise will cause a startle again, and again etc..

I know it will pass- it has done in the past - but still its a feature that is puzzling.

I wonder...

kemal.altug@ntlworld.com (Leylasdad) — Fri, 16 Apr 2010 10:48:00 +0000

I wonder if Leyla's jejunostomy has become displaced, and that's why we're seeing so much gastric issues. For example, feed is coming back into her stomach after being put through her Jejunal tube. That's not especially right, and it's been consistent for many days (nay weeks) now. There doesn't seem to be any inter current illness, and you can almost time when her stomach fills and becomes uncomfortable shortly after starting feeds.

I know that jejunostomy tubes can coil up and migrate back through the pyloric sphincter into the stomach, and I suspect something like this may have happened. I can't be sure.

However, getting into GOS to get this checked is a bit like getting into Fort Knox wearing a balaclava - so it's something that won't be checked over quickly. Hey, but that's another story....

Static Motility?

kemal.altug@ntlworld.com (Leylasdad) — Fri, 09 Apr 2010 23:22:00 +0000

Had to withdraw Clonazepam after adverse side effects of hyper-salivation. However, following this Leyla's gut motility has almost stopped for last few days, and feeding her via her Jejunostomy will result in feed coming back up into her stomach - so having to deal with that (though I'm not sure how best to).

Suggestions welcomed.

Enough Already!

kemal.altug@ntlworld.com (Leylasdad) — Tue, 30 Mar 2010 19:51:00 +0000

Azathioprine, an immuno-suppressant was prescribed for Leyla early December last year at GOS Hospital. It was thought (perhaps) that if Leyla's gastro issues were an auto-immune response, then suppression may help.

Well, today we have suspended this med for Leyla, as to be honest , the only effect this med has had is make Leyla even more ill, with inter-current illnesses one after another.

With a highly compromised immune system, she's just picking up infections left and right, (man, she just needs to glance in the direction of someone with a bug and she'd get it), and what is more, her symptoms would appear deeper and longer lasting, taking longer to recover (only to be hit by something else straight after)..

Let me point out that her WBC (White Blood Cell) counts have up till now been generally OK, but nonetheless, despite this, there is some strong correlation, chronologically, of the med being introduced, and within a few weeks, picking up infections.

But what about the gastric issues? The Colitis? Has the Azathioprine helped? Well no - none that we can detect.

So - with a powerful medication that (so far) has yielded no perceivable benefit - yet it seems, stacks of disadvantages, I would be tempted to say "Enough already!", but in truth I'm more inclined to say (in a broad British accent) "F**k this", and so, today Sony and I decided Azathioprine will be suspended until we speak to Gastro at Great Ormond Street.

In the interim, we consulted our local Dr, and he agreed, suspend the med - so that's what we've done.

(sorry - don't mean to swear on my Blog, but hey, it feels good to sometimes)

Respiratory Infection?

kemal.altug@ntlworld.com (Leylasdad) — Fri, 26 Mar 2010 08:05:00 +0000

Drats. Back at East Surrey Hospital last night as Leyla's breathing sounded very laboured, and more worryingly, she turned blue. Chest X-Ray was thankfully clear, however she is clearly having some upper respiratory airway difficulties, especially with phlegm she cannot expel herself - so lots of mechanical suctioning has been required.

Atrovent was prescribed, an inhaler of the anticholinergic family containing Ipratropium Bromide. This relaxes the muscular tubes of the lungs and hence ease breathing difficulties.

So, back home late last night, and I think the Atrovent had helped, though to be sure we placed her on our Oxygen Sats monitor, and monitored her O2 and BPM which showed she was tachycardic (BPM average of 175 peaking on occassion to 195), so close monitoring throughout the night was necessary.

This is an intercurrent illness, one of several of late - and we are convinced that her Azathioprine (an immuno-suppressant) drug prescribed for her eosinophilic colitis may be the smoking gun. You see, since Azathioprine, which essentially zaps your immune system like a 20lb sledgehammer was introduced last December, Leyla has been picking up infections like they're going out of fashion.

Having seen no direct benefit of Azathioprine, but it seems, the main side effect of a compromised immune system it sets out to acheive, we wonder weather this med should be withdrawn, or at least temporarily paused, so a re-test of her white blood cell count could be made, and a call to Great Ormond Street to seek clarity on its continuation.

Once more unto the breach my friends, once more...

Leyla Back Home from Hospice

kemal.altug@ntlworld.com (Leylasdad) — Tue, 23 Mar 2010 09:18:00 +0000

She had a wonderful time there, and seems to be responding well to both the Buscopan and the altered feeding regime of all feeds during the day with zero feed during the night. Let's hope this good spell continues.

Chase Hospice is a fantastic place - with truly excellent staff - brilliant people, and nothing one perceives a Hospice to be. It's friendly, and doesn't look clinical at all. Excellent place.

Today we have to travel up to Evalina Children's' Hospital in London for an appointment with Leyla's neuro-movement disorder specialist. Naturally I have a few questions to ask, namely is her Trihexyphenydil at the right dose, and is a trial of L-Dopa a reasonable and safe option. Her Dystonia, although slightly better with the anticholinergic, is not significantly better to be of great noticeable significance. There is scope to increase this, and that's one area of discussion.

Clearly, she has been responding to benzodiazpines such as Midazolam and Clonazepam due their muscle relaxant effects (GABA regulation), however, I wonder if a trial of low-dose L-Dopa might be more effective and less sedative. Though to be honest I wouldn't want Leyla to start a new med, its a difficult one to call because if it helps (and it's a big if), and helps significantly, I cannot allow the worry of adding another med deprive Leyla of what may (just may) be of significant benefit.

But you don't know until you try. It'll be empirical. Of course, the Doctor might say no to a trial - and that will be that - but I want to discuss this with him today, and ultimately I will be guided by his opinion.

Leyla in the Night Garden...

kemal.altug@ntlworld.com (Leylasdad) — Sat, 20 Mar 2010 15:19:00 +0000

With Iggle Piggle and Upsy Daisy...
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Leyla at Chase - Just Chilling

kemal.altug@ntlworld.com (Leylasdad) — Sat, 20 Mar 2010 15:02:00 +0000

Just had to post this - Leyla chilling out with her headphones...

She's had a good nights sleep and relaxed and settled today - always a great thing to see.
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Hospice

kemal.altug@ntlworld.com (Leylasdad) — Fri, 19 Mar 2010 12:42:00 +0000

Haven't posted for some time - things have been hectic. Leyla is at Chase Hospice, and has been for a few days. Aside from respite for us, they are trialling her on higher doses of Buscopan, and possibly an increase of her Clonazepam dose.

Previous to the hospice, Leyla was back in our local hospital for several days with unexplained temp spikes, and continued gastric pain.

Whose House Is This?

kemal.altug@ntlworld.com (Leylasdad) — Wed, 10 Mar 2010 18:45:00 +0000

Back home since yesterday afternoon. Leyla has been started on Clonazepam, a long lasting benzodiazepam in the belief (hope) that its muscle relaxant qualities will help the symptoms gut dysmotility, and we believe, of dystonia

Ultimately, this is just symptom management, and we need to find and treat the underlying cause in a more effective way - but for now, the plan is 500 micrograms of Clonazepam at 8pm each night. The last few nights however, Leyla still woke up circa 2 am screaming - so I don't think the dose is yet right - but I do know there is scope to increase this, and will liaise with the Hospice again to make any adjustments.

Her norovirus is now gone, and the C Diff is to be treated at home with Metronidazole (75mg three times a day) contuniung for the next 5 days.

It's weird being back home. We've spent the last month or so at either Great Ormond Street, a few days at Chase Hospice, and a big chunk at East Surrey. I didn't recognise home - luckily my home address is in our Sat Nav otherwise I would have spent ages looking for it.

Thanks again to everyone for their kind wishes and prayers - we always appreciate these.

Still in Hospital...

kemal.altug@ntlworld.com (Leylasdad) — Sat, 06 Mar 2010 21:35:00 +0000

Firstly, thanks to everyone who've sent us messages of support - we appreciate this very much, as it lifts our spirits and hopes.

There was some discussion of Leyla being discharged on Friday - but after a review by the paediatric consultants, this was quickly quashed - leyla still showing signs of her inter current illness. So we are still here.

Earlier today the labs confirmed that Leyla now has Clostridium Difficile (C Diff).

She has been started on Metronidazole (Flagyl), an antibiotic that will hopefully tackle this tricky and nasty bug

In terms of fever, this is now under good control, so that's always good news. For feed tolerance, we're not quite there yet, so still on half neocate, half dioralyte. But off the IV fluids.

I think the norovirus has generally been defeated, but with her compromised immune system, and now C Diff, its taking longer to get back to baseline.

The Docs will re-assess her on Monday

For symptom management, ie the long standing pain issues (those following Leyla's story will know about), a trial of clonazapam (0.25mg) has also been initiated on recommendation by her palliative care Dr.

The intention here is the muscle relaxant qualities of clonazepam could be better suited than her current benzo (Midazolam)

Clonazepam is less 'steeper' in its effect, yet of longer duration than the former benzo.

The Docs and Nursing staff here have been great, and Sony and I are splitting shifts to be here with Leyla.

Will update again soon.

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Urgent Prayers Needed

kemal.altug@ntlworld.com (Leylasdad) — Tue, 02 Mar 2010 15:16:00 +0000

Leyla is very poorly in hospital suffering from either rotavirus or norwalk virus (as yet undetermined). She is on IV fluids.

She has already lost over a kilo of weight, bringing her well below the percentiles for her age. We've all been hit by this virus, and not sure where we picked it up - but we are worried about her, especially as she is on azathioprine, an immuno suppressant drug that already weakens her already compromised immune system

Prayers please for little Leyla

Manometry Result

kemal.altug@ntlworld.com (Leylasdad) — Sun, 28 Feb 2010 21:12:00 +0000

The result was clear. This was disappointing as we hoped we had nailed the problem

The colonoscopy was not done, as the Dr wants to perform another manometry (this time a colonic manometry as opposed to an anorectal) at the same time. These tests won't happen before Easter, which is frustrating

Things been pretty awful today, so we've been admitted back to East Surrey Hospital for Leyla to be started on Clonazapam.

The idea being a muscle relaxant with a longer lasting duration may be beneficial.

So that's where we are

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Frustration

kemal.altug@ntlworld.com (Leylasdad) — Mon, 22 Feb 2010 21:48:00 +0000

Leyla has been mega cranky, mega restless again today. A mixture of of distress and pain. When I say mega, I mean mega. Think in megatons not kilos.

It's so difficult to understand what's going on, but these last few days have been different. No meds to calm her down work for more than 30 minutes tops. And that leaves us, and Leyla, with no options, no plan B.

I still think it's gastro related.

Still haven't heard back from the Gastro team about making the list for an ano-rectal manometry or repeat colonoscopy - so there's some chasing up to do tomorrow.

Again.

Stop Dreaming Of The Quiet Life...

kemal.altug@ntlworld.com (Leylasdad) — Sat, 20 Feb 2010 17:29:00 +0000

"Stop dreaming of the quiet life
It's the one you'll never know,
And quit running for that runaway bus
coz those rosey days are few.."

So said The Jam in 1982's 'A Town Called Malice', and never a truer word spoken (or sang).

Leyla has been super, nay, mega cranky all last night and all day today. Monday we are going to Chase Hospice for Leyla to stay for a few days - this is so tiring - but it has to be done. Energy levels are down to almost zero. And I mean ours...

We didn't hear from the Gastro team at GOS when the manometry and repeat colonoscopy will be done, so we'll have to wait until the working week begins - but if I'm honest, I think if we did get a call from the GOS Gastro team before close of play Friday, the shock would've killed me - know what I mean?

Back Home..

kemal.altug@ntlworld.com (Leylasdad) — Sat, 20 Feb 2010 00:30:00 +0000

So here's the plan. Carbamezepine is to be tapered off, and a manometry and repeat colonoscopy is to be scheduled as an urgent case. We haven't a date yet, but hope and pray it will be soon because this just cannot go on. It's just a constants round of buccal Midazolam and chloral hydrate.

I think in the past year and a half she's been sedated over 300 times or more. Most of you reading this blog with typical kids would baulk at the idea of sedating your child once, let alone hundreds of times - but that's the reality we live in.

One of the top gastro consultants saw us and agreed the ano-rectal manometry and colonoscopy would be the next step, so that was pleasing to hear as I've advocated hard for this, especially the manometry, for quite some time. I felt victorious when he said it, yet exhausted.

Now it's a waiting game for when the appointment will come up - and hoping that the tests show something that can be treated.

Perfect Timing & The Carbamezepine Theory.

kemal.altug@ntlworld.com (Leylasdad) — Tue, 16 Feb 2010 22:58:00 +0000

Had a bedside visit from the metabolic team this evening, and Leyla was in a good mood, content and otherwise pain free. Now that's a great thing to behold, and most sorely welcome.

As if on cue - halfway during the visit Leyla griped and moaned for a second or two, and then had a prolonged spell of dystonia, first locking her legs fully outstretched for a couple of minutes, before drawing them up in a frog-leg posture and holding them rock solid for several minutes more. When I say rock-solid I mean rock solid.

In the mean time her entire body skin went mottled, and she sweated heavily, particularly in the palms of her hands and soles of her feet, whilst crying, almost screaming - clearly in acute pain.

I know that she's had these episodes several hundred times before, so I'm not glad that she had another one, just that timing was right, know what i mean?

Whilst they've seen Leyla scream in pain before, it's always been during an episode - I think this is the first time they've seen how quickly Leyla can turn from being settled to deeply distressed in a matter of seconds. It may help them make the diagnosis process easier - who knows.

We also spoke about when these episodes started, and what they aligned with. Nothing really, except Sony and I think possibly (just possibly) there may be a relationship between Carbamezepine and her colitis.

We've mentioned this several times before - and here is our rational, our logic:-.

Nearly all of her meds have at some point changed, or been withdrawn or new ones added since the start of the pain episodes. But one has been constant and present before the screaming and still remains - that is her anti-epilepsy drug - Carbamezepine. Other meds that were present at the onset of screaming includ meds that all have been withdrawn - except Carbamezepine. Can you see what I'm getting at.

Now - possibly millions of people take this med (also known as Tegrotol) - but Leyla is different, she has a metabolic disease, and even then , you have to consider that no two kids with even the same rare metabolic condition, even with the exact same diagnosis, are the same.

Sony and I have often wondered weather this drug could be implicated merely by this simple deduction., and to their credit, the Dr's tonight took on board this theory and will discuss a plan to safely and gradually withdraw this med after further consultations amongst themselves.

It may mean a different anti-convulsant takes it's place, and there's a real risk this is a red herring, a blind alley, a failed clutch at a loose straw., but it's a lead. There's also a risk that is't gradual withdrawal will lead to seizures coming back with a vengeance, but we just don't know.

I read about a 57 year old man who had Carbamezepine induced colitis, so it's not entirely without merit - bleeding rare - as us Brits would say, but this theory still has legs. A remote theory, but still.

Off to sleep now, another day on the ward beckons.

Blood tests

kemal.altug@ntlworld.com (Leylasdad) — Tue, 16 Feb 2010 16:40:00 +0000

Day two at Great Ormond Street.

Today, Leyla had several blood draws to re-check Copper, Creatine Kinase, Plasma Amino Acid profiles, and other routine nutritional markers. She was (and curiously always has been) remarkably tolerant of needles, unlike me.

The Metabolic Team will have a meeting with the Gastro team to decide next steps (this may have happened already - I don't know yet).

I do know that the metabolic team agree with me in that the status quo can't remain. In her motility issues, something has to change.

I don't know what options, if any, they'll consider - but we'll find out soon enough.

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Back home since Sat...But...

kemal.altug@ntlworld.com (Leylasdad) — Sun, 14 Feb 2010 21:49:00 +0000

East Surrey hospital contacted Great Ormond Street, and arranged an admission for this Monday - providing that the spare bed isn't taken up in the interim by another more urgent case.

So tomorrow, unless the bed reserved for Leyla is taken up - that's were we are heading to.

East Surrey was fantastic in their care for Leyla and us. Nothing was too much, and the Doctors and Nurses were brilliant. Leyla was put on fluids for 36 hours, and as we suspected, resting her gut gave her respite from pain.

In a complex case such as Leyla's, they did say GOS is the best place for her current medical needs, hence the re-referral - so we're grateful for that.

The slightly puzzling thing was that the Gastro team didn't necessarily think the referral was urgent enough - that decision is most likely linked to a bed shortage too - however, the metabolic team came to the rescue, and it's a metabolic bed we've been offered. But its a foot in the door, and that's what matters.

The East Surrey consultant told us that its just a matter of waiting until Monday to transfer to Great Ormond Street, but as nothing in terms of medication will change between now and then, and as the immediate plan was to follow the same symptom management plan we follow at home, we had a choice of doing exactly what we do at the hospital, or do exactly what we do at home until Monday comes.

So tomorrow off the GOS again.

Back in Hospital..

kemal.altug@ntlworld.com (Leylasdad) — Fri, 12 Feb 2010 12:33:00 +0000

Things not good. Not good at all. Pain episodes are frequent, and may get a referral back to Great Ormond Street.

Sulfasalazine

kemal.altug@ntlworld.com (Leylasdad) — Tue, 09 Feb 2010 14:12:00 +0000

Another new med for Leyla, this time Sulfasalazine, a drug primarily used to treat ulcerative colitis and arthritis - though we are trialling this for eosinophilic colitis, but the theory's the same...

Sulfasalazine itself is a poorly absorbed drug, that mainly works in the gut - therefore it is thought ideal as it works as an internal topical treatment of the intestines, reducing inflammation.. That's the idea anyway, and if it does work for Leyla, it could mean ending the steroid treatment which to be honest, hasn't yielded much in her favour, and neither has Azathioprine - but we have to wait and see.

Besides, you cant stay on high dose steroids forever, so we are praying this med works.

There are side effects to this drug, and every three months or so she should be checked for serum levels of sulfapyridine.