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