The saga of the biopsy......

The saga of the biopsy.....

Well, where to start?

Ok...so since last posting, Sam quickly went back into remission after writing.
He continued a routine with his medication and was taking his full dose of ciclosporin bi-daily and a reducing level of steroid (prednisolone) which ran over the next 4 weeks before stopping.
This was all great news and meant that all was back to normal in family and school life for a while :)

We saw the consultant with regards to booking Sam in for a 2nd biopsy to check and make sure that things were all going well and the kidneys were not being damaged in any way by the medication that Sam had been taking.
Generally, with ciclosporin, Sam would take this for 2 years before weaning off. As the wean didn't work well for him this time, the level of ciclosporin has been retained and therefore the second biopsy is required.

As this is only a precautionary biopsy, it was not given priority and was booked in for September.
I was actually quite relieved to hear this, as it meant that the family could enjoy a lovely long Summer together without the stresses of the operation.

September came and the date for the biopsy and pre-op booked in.
The day of the pre-op - and Sam was unwell!
He'd had a cough for a couple of weeks and didn't seem to be clearing it but had been quite well up until now. Today, he started with a mild temperature and by the time he was giving blood, was quite distraught....most unlike him normally!

The following day and we were asked to go back in, as the pre-op test results were showing abnormal readings and Sam needed to be re-tested.
His temperature had now gone....but his cough seemed worse and was keeping him up at night!
That morning we saw Dr Rodd (Sam's consultant at Winchester) to take the new bloods. This was fantastic as he knew Sam's history already. He took the bloods, explaining why the levels may have been abnormal, checked him over and instantly prescribed an antibiotic to get rid of Sam's cough. There was still hope that the biopsy would go ahead.

Unfortunately the cough didn't fully clear up and required a further prescription and the test results.....still abnormal!
More tests and more blood taken a few days later and this time there was a chance that they were testing for something more serious that may be causing the abnormal results.

This was bad news for us.... I understood that there was only a slim chance....but to find out that what you thought you'd been dealing with for years....could now turn out to be something completely different? Was a little stressful to say the least.
After a few worrying days, we received a call from the nephrologists saying that the biopsy would be able to go ahead in another month and that the test results may have been jeopardised by Sam being unwell over the time they had been taken.
A little confusing to be told that he may have a more serious condition....or he had a cold? I did find that a little hard to understand....but we went with it and Sam in the meantime was recovering well.

So now we're almost upto date....

It's now early October and the new biopsy date and pre-op a week prior had arrived. It was now set for 8th November.

Mid October and Sam's behaviour started to slip!
We started to see him quickly slide into chaos and tantrum at home...lots of sibling bickering and Sam's school had to speak to me on a couple of occasions for him being "naughty" and losing concentration and not listening.

Immediately this rang alarm bells to me!
After all this time...at last...I saw a pattern!
The last time his behaviour slipped like this was a couple of weeks before his last relapse.
As much as I didn't want to blame his condition (especially if it was just a case of him having a naughty phase!!) I couldn't ignore the similarity to the previous episodes.
I'd remembered the last time, looking back and thinking "that's why he's been acting like this!"...and then feeling immensely guilty for keep telling him off!
Clearly whatever is going on inside his body, takes so much of his concentration, that the other things slip.

Well, as expected a week later Sam was off school with a mild temperature and immense tummy pain - again!
On the 4th day of pain, we dipped Sam's urine...and we were right...4+!!
After 4 days of 4+ dipsticks we rang the hospital, went straight in and were prescribed his usual steroid and antibiotic combination.
Strangely, the following day, Sam's dip showed only 2+, so I made the decision to hold off starting the steroid for a couple of days.
Sam remained at 2+ for a few days and even dropped to a Trace and a couple of 1+ dips....which excited us....but this quickly declined back to 3+ and again 4+ over the last couple of days.

So, steroid and antibiotic re-started, ciclosporin still going at full dose and....the cough!...it's back!
Today, Sam is looking very puffy (over the bridge of his nose is initially the worst area) and a little lethargic (for him, anyway!)

And...the 2nd biopsy date.....cancelled again!.....and the saga continues........


Thanks to all those who support me and the family through these times :)
It's really hard (emotionally more than anything really...), am feeling rather subdued and fragile today......so lots of love to everyone who thinks about Sam and sends their love and good wishes whenever things get difficult :)

I love you all - and you know who you are :)

Thanks for reading as usual :)

Sonja xx 

Swings and Roundabouts?

Swings and Roundabouts?

Since my last post we've had a few developments to let you know about :)

Sam has remained at 4+ on his dipsticks up until Wednesday and has still been having bouts of terrible pain in his tummy! These moments are really hard to watch...knowing that you can't do anything for him at all! Holding his tummy seems to help a little...but I suspect only mentally for him.
On reporting all this to the hospital, we were advised to increase Sam's ciclosporin dose back up to our good level of 0.45ml twice daily.
This does mean that a biopsy will be required....but this, at the moment, can wait until Sam is comfortable and back in remission.

On Thursday, we had a small breakthrough...at last! Day 7 of the steroid treatment and we are certain that the dipstick reading was a clear 3+. Unfortunately, Sam didn't seem to be feeling any better at this stage, but we hoped that this would be the start of things improving. We visited the hospital in the afternoon...just so that things could be checked before the long weekend.
Again, although he was still retaining lots of fluid (now weighing in at 20kg!) and feeling very uncomfortable, there were no signs of anything more worrying.
The tummy pain could only be explained as pressure from the fluid and anytime any food was added, this caused extra pressure and the pain. Hence, Sam had not really eaten much at all, all week. Each meal was slow going with Sam eating a couple of bites at a time, before having to stop with pain for longer periods!

Saturday morning now....and what seemed a major breakthrough!
Sam woke up and seemed much happier in himself. Very lively compared to any other day for the last 2 or 3 weeks! His eyes were bright and he was up and playing with his toys and joining in with the family more. This was such a relief and so lovely to see at last!
His reading was showing 2+ today. This was a major improvement and proved to me that we weren't imagining the reduction of earlier in the week!
We had a slow start to the day, but Sam seemed so much improved that we decided to venture out (to our local Jubilee Celebrations) for the afternoon.
We had such a good time....enhanced by the fact that Sam was with us and not couped up at home still! This was his first time out and about in the last 8 days!

During the afternoon Sam ate the equivalent of 2 meals! Another major breakthrough! I was shocked and thrilled!! Not only did he manage more than 2 mouthfuls...there was absolutely no pain!
He lasted 2 good hours out enjoying himself...then had to rest. We brought him back home (with Gra) and returned to the celebrations.
A few hours later Sam rejoined us...but clearly had overdone it all as he instantly had no energy to do anything out there. He lasted another hour outside...but spent most of that time in my arms!  

The evening saw Sam eating another full meal with no pain. Again...such good news!

Then, a set back!

Sam awoke at around 2am in extreme pain! He was very disturbed and crying out and nothing we did seemed to help him!
The worst part was him shouting out that he needed to go to hospital!
This was so heartbreaking. After having such a fantastic day...this really was not the ending I'd imagined!
We sat up for a good 3 hours with him writhing and crying out in pain, before he finally relaxed into sleep again!

So, today...I'm good for absolutely nothing!! Exhausted!!

And Sam......

Is bouncing around like a little sprite again! (humph!....but still fab to see!)

His dipstick read trace/1+ this morning......probably trace...but we err on the side of caution for the moment.
He seems completely fine again.
Eating quite well....and we've had 2 wees from him this morning! (we've been averaging 2 wees all day for the last 2 weeks!!)

So, we're really hoping that last night was just a blip...and that now that the dipsticks are reading low levels of protein lost, that the drain out of fluid is beginning and remission is just around the corner!

Keep your fingers crossed for him :)

Sonja xx

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