Another relapse...another challenge!

I apologise for the lack of blogs recently...but we've been having a busy old time with Sam and his condition.

I realise that I haven't brought you fully up to date yet (that will follow).....but here's what's been going on recently...

Sam had been coping really well and been healthy and relapse free for a while.

He'd been prescribed a low-dose Ciclosporin (an anti-rejection drug ?....but seems to work well with NS cases like Sam's)
After many regular blood tests and changes in the dose to get the correct levels for his body, we rested on a 0.45ml dose twice a day.

Although not without it's side effects and complications, Ciclosporin was more favourable then steroid as a long-term solution to hopefully reducing or stopping Sam relapses.

Looking back now...this drug (and dose) worked really well for Sam and although he had to attend hospital regularly for blood tests and check-ups...we had no admittances due to relapse, infection or illness for 2 whole years!
We were all so relieved and really, really pleased for him!
Life returned to relative normality with Sam becoming a healthy, happy child and resuming a normal diet etc. Also, his behaviour and mood-swings improved due to him having a longer period off the steroid treatment. He was doing really well at primary school now (academically and socially!) and was settled into his routines and making friends.

So, after 2 clear years and Sam being as healthy as he was, it was decided to start a wean off the ciclosporin.

It is common for everday illnesses (ie colds etc) to trigger a relapse in a sensitive case, so, to give him the best chance of remaining relapse free, we started the wean after the winter months in Feb 2012.
It was to be a very slow wean to allow Sam the best chance of success.
So, on 1st Feb we reduced the dose by 0.05ml and were to remain on this dose for a further 2 months before the next drop.

After 3 weeks, Sam relapsed.
His protein check shot straight up to 4+ (this is a scale ranging from negative or trace, 1+ upto 4+ and beyond to show how much protein is leaked into the urine - tested with a dipstick)
As you can imagine...we were devastated!
So, for the first time in over 2 years, Sam was back on high-dose steroid.

Well.....that was a shock to the system I can tell you! I wouldn't have believed how much we'd forget and how much it changed Sam! After only a couple of days back on the Prednisolone (and penicillin - to combat any infection) Sam was a totally different child! His whole behaviour changed causing issues at school with his massive mood-swings. His struggling to concentrate and inability (yes! I'm positive it is inability!) to listen! He would be off in his own world for hours and nothing you said would get through to him at all! So, in times of major tantrum...you can imagine the scene! The only way to get through to him...was to stay calm, hold him and wait for his focus to come to you. Once he was focussed...it was easy! He'd listen, understand and collapse in an exhausted heap of tears! Then we'd be left with a very fragile, sensitive Sam...that needed lots of time, attention and comfort! It was heart-breaking to see him like this (again) and extremely wearing on the whole family! His siblings having to re-adjust just as much as Me and Gra.We'd all have to learn (or re-learn) a whole new way of treating Sam. Avoiding situations, reacting to different moods and then dealing with the aftermath!!

Medically, Sam did well and was back in remission after 11 days on the steroid. This is the point that he stopped leaking protein.
Daily urine tests with his dipsticks show the level reducing over time and upon the 3rd day of a negative or trace reading - this is called remission.
At this point reduced the dose of steroid slightly and he took it only on alternate days. A kind of quick blast of high dose medication to stop the relapse...and then a quick wean. This continued for 4 weeks before stopping the steroid completely.

During this relapse, we were given options for the way ahead!
1 - see it as a "blip" and continue with weaning the ciclosporin (the only risk being a further relapse on completing his steroid dose)
2 - increase the ciclosporin back to the original level. (this would mean Sam undergoing a 2nd biopsy to check the kidney's health)
3 - stopping it all and looking into something completely different.

We decided together that we would go with option 1.
Given that Sam did so well for so long on ciclosporin before, we didn't like the idea of option 3. And if we could avoid the biopsy (for now at least) we would take the risk of another relapse.

So, 7th April 2012, Sam was back to only taking the lowered dose of ciclosporin. We continued to check his urine daily....just to be sure and he had check-ups at the hospital.
We were so pleased that he seemed to be doing really well and holding off another relapse (even on the lower dose of ciclosporin!!)

THEN...after 4 more weeks.....he caught a cold!!
By day 2 of his cold, his distick showed 4+ again! And again we were devastated!
The Doctors hoped that again it was a "blip" and that once Sam got over his cold, his protein loss would return to normal.
So, we were advised to hold off with the steroid treatment and were then giving daily updates to the hospital on his dipstick reading and his health.
Over the next week Sam was quite happy. He was getting over his cold well and seemed healthy enough (apart from remaining on his 4+ readings). By the next week (21st May), we noticed little changes. He was becoming more tired and frustrated with things.
A check-up on monday didn't show anything significant. He seemed well enough...but still had a cough left over from his cold and was just tired. He didn't look to be retaining much fluid at this point, so we continued to ride it out and stay off the steroids, though Sam did start a course of penicillin to fight away the cough.
Tuesday brought a disturbed night, with Sam's cough the worst it had been and he was awake and coughing for quite a few hours. We kept him off school to rest on Wednesday and he seemed fine all day and slept well that night.
We were still phoning the hospital each day with updates...but as nothing much had changed there were no further developments.
On Thursday (back to school), we noticed that Sam was starting to look a little puffy over the bridge of his nose. (On past days this had subsided over the day...but it seemed more prominent now). By lunch-time he'd gone downhill and seemed quite unwell at school and was sent home. Back home and resting...Sam perked up a little, so instead of visiting, we phoned the hospital.

Now, we were advised to start the steroid treatment! Same as before in terms of dose and period.

So, now we're almost upto date!
We gave the first dose of steroid on Friday.
Sam has been getting more moody and has been complaining of tummy pains all weekend...and getting worse! On inspecting him last night he seemed to be quite puffy facially and had a much larger belly than normal. Worried by his crying and writhing (not normal for Sam!)..I phoned the hospital and made a late night visit to get him checked out! And although all his bits were ok, ie - no infections or worrying problems....he had gained 1.7 kg in weight since being weighed at check-up on Monday! That's 6 days! Most definitely all fluid...and all around his tummy area, legs and face! Definitely glad that I got him looked at! :)

So.....today...we're relaxing together....watching dvd's......and waiting for the hospital to phone with news of the next bit of the challenge! Most probably moving onto option 2 and another biopsy ?? but...better not to think of all that until we get there I suspect!

My cuddle awaits...so until next time
Take Care :)
Sonja xx

What is Nephrotic Syndrome?

This week we are feeling the full force of a 6year old on steroids!! It's hard work, so has inspired me to write this next blog for you all :)

So, what is Nephrotic Syndrome and what is happening inside the body?

Well, simply, it is a condition whereby the kidneys do not function efficiently.

The kidneys are bean-shaped organs, each about the size of a fist. They are located near the middle of the back, just below the rib cage, one on each side of the spine. A "normal" kidney would allow blood to flow through it to be "cleaned". The kidneys then sift out the waste products and extra water which then becomes urine which is then released via the bladder.
The kidneys in a person with NS are likened to a sieve, so this means that the filters become "leaky" and so allowing large amounts of protein to leak from the blood into the urine. Having a lowered amount of protein in the blood then causes water to leak from the blood into other parts of the body and can cause puffiness under the skin, fluid in the tummy area and fluid around the lungs.
If this "leaking" continues, then the blood becomes thicker and can cause other complications such as blood clots, infection and dehydration and can if untreated cause damage to the kidneys themselves.

There are 3 main types of NS
1. Minimal Change Disease or MCD - where there is little else found along with the leaking of protein.
2. FSGS or focal segmental glomerulosclerosis - where there is noticable damage to the kidneys.
3. Congenital nephrosis - where the kidney problem is inherited.

It is assumed at first diagnosis that you have Minimal Change Disease (MCD) unless further complications arise and further testing is done.

Sam (as with most children with NS) was started on a course of steroid treatment (prednisolone). Most children will get better throughout the course and at some point (usually between 2 and 4 weeks) the body will stop losing protein and return to normal. This can be tested with a blood test and/or using dipsticks to test the urine. The dipsticks read pluses of protein ie negative, trace, 1+, 2+ etc. Once the reading has been negative or trace for 3 consectutive days, this is called "remission" and at this point a programme for the steroid wean would be put in place.

As steroids are a powerful drug, they must not be stopped immediately and require a reduction weaning programme. At first, this would be a long, slow wean in the hope that this will correct the initial problem and no further instances of NS will occur.

As with many drugs, steroids also have poweful side-effects!! These become more of a problem the longer they are used so reducing doses are usually preferred. Lowering the effective dose, stopping as soon as is safe, alternating the day that doses are given and using other drugs to prevent re-using steroids and monitoring the child regularly.

Short-term side effects usually occur within a few days of taking prednisolone and can include: tiredness, weight gain, increased appetite, higher blood pressure, higher blood sugar and definitely behaviour changes which can include hyperactivity, aggressiveness, difficult to handle and rapid changes in mood (for no apparent reason!)
Medium-term effects (within a few weeks/months): risk of infections due to lowered (suppressed) immune system, change in appearance (rounder face, thinning skin, stretch marks and hairiness), stomach problems and muscle weakness.
Longer-term (within a few months/years): growth problems, bone problems, delayed puberty and cataracts can develop.

So, in some cases this will be the only instance of NS occuring. Some children will have other infrequent occurances (relapses) maybe a couple a year and others will go on to become frequent relapsers and/or develop other problems.

As Sam falls into the latter category...I'll write more on this later.

Hope this hasn't been too technical (or boring) and I'll write a bit more on Sam's own case next time.

I've added a few links to the bottom for you to look at if you'd like to know where I got some of my info from or would like to go into it in more detail? Most of the info is from what we've been told over the years, so things may be slightly different if a diagnosis was to be made today?

Thanks for reading
S x


   

niddk

BBC health

In The Beginning......

In The Beginning....

In 2005 Sam was born a very happy and healthy baby.
My pregnancy and birth had been completely normal with him and for the first 2 years of his life, everything went according to plan. Sam was learning everything at the right times and was growing well.
At around 2yrs 5mths I noticed that Sam was not quite himself and seemed a little under the weather. There was absolutely nothing that I could put my finger on....and everything that seemed unusual, I could explain away due to his age and what I knew of growing up.
Some of the things I noticed happened regularly...some were more infrequent, so for quite some time, we carried on as normal.
Some days he would be overly grumpy, sometimes he would sleep more during the day. He had mild stomach pains on and off, his appetite dropped and his face looked "different". At the time I could explain all of these things away, due to him starting to grow into a toddler.
After some time (over a couple of months!), I noticed that his appetite was much reduced, yet he seemed to be putting on weight and becoming more chunky. This seemed strange to me....1. because my other 2 children had had similar appetites...but were tiny! and 2. if he was eating so little...how could he get so chunky?
At this stage things seemed to move quite quickly! Sam was now eating very little, had very little energy, had quite bad balance (kept tripping over nothing) and yet was getting still more podgy. At this point we also noticed that he was not using his potty very much at all. 
I booked him an appointment the next afternoon at the doctors surgery..just to voice my concerns. I still thought I was going mad and that he would just be given a course of something to get him right again.
As soon as he was seen and I'd gone through all the symptoms (even telling the doctor that I thought I was going mad....but knew there was something not quite right!?), she instantly referred us up to the hospital. She had not told us what she supsected, but even though Sam's condition is very rare, (it only affects approx 1 in 100,000), she had spotted it straight away!
As you can imagine...I didn't sleep much that night...and was now really worried!
The next morning Sam woke screaming and very floppy and I couldn't do anything to console him at all!
We brought our hospital apointment forward.

On 11th Feb 2008 we spent many hours in Sophie's Place at Winchester Hospital having blood tests, doctors checks, blood pressure, urine tests, temperture checks...anything and everything that they could look at on Sam....and many, many forms and questions to complete!
By the end of the day...Sam was diagnosed with Nephrotic Syndrome and was admitted into hospital.....

Please remember....
These are my experiences of Sam's condition and not a conclusive list of all the symptoms that may be present. Likewise, as I said, all the symptoms presenting individually are perfectly normal in a child of that age.


For my next post, I will dig out my (official) notes on "what is Nephrotic Syndrome"...just so you have details of what is happening inside the body.

Thanks for reading :)
Sonja xx

Welcome to our new blog

Well....I have no idea where to start....except to say "Thank You" for coming and visiting my blog and welcome!

Those of you who know me, will know that I can talk ...and talk....so, I will try my best to make my posts as informative and useful as possible.

I decided to write this blog to record the events of everyday life with my son Sam, who has Nephrotic Syndrome.

NS is a rare condition affecting the kidneys and little is known about the causes and treatment at this time. Every presented case of NS is different and has to be treated individually.

Sam was diagnosed at around 2 years old, and is now 6. He has been relatively well recently, but this latest relapse has prompted me to share our experiences.

Over time, I will take you back to the beginning and give you some background information about the last 4 years. What the condition is, what it means to Sam, how he copes with everything and also try to share what is has meant to the family. Once up-to date we will use the blog to keep you informed on any new developments.

I hope that this blog will help people who know Sam (and us) to understand about NS and what he deals with.
I would also love to help people who are coming across NS in their lives for the first time.
I know that when Sam was diagnosed, I really wanted some help dealing with all the emotions, but I could find very little about it on any UK sites (lots of USA ones...but methods over there are not always the same).
If nothing else, it will help others whose loved ones are starting on the journey, that it is maybe not going to always be as hard as they expect.

So, I hope that you will keep on reading...and will find it useful?

S x