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
Wednesday 28 March 2012
Thursday 15 March 2012
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
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
Friday 9 March 2012
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
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
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