Nephrotic Syndrome

Last year, around November, shortly after my daughter’s 3^rd birthday she was diagnosed with Nephrotic Syndrome or Nephrosis.

It was not a forthright diagnosis but rather a succession of doctors’ visits later, many prescriptions (and lots of pharmacy payments) and several days out of school due to her being unwell but with no distinct symptom that is usually associated with common illnesses that we finally got an answer.

We’ve never heard of Nephrotic Syndrome, have never known anyone who ever had it. Kidney issues could not have been hereditary so we couldn’t quite understand this unheard –of kidney condition.

What is Nephrotic Syndrome?

Nephrotic syndrome occurs when the filters in the kidney leak an excessive amount of protein. The level of protein in the blood then falls and this allows fluid to leak across very small blood vessels into the tissues. Swelling around the eyes, abdomen and legs is then noted. Protein in the body is normally made by the liver and the liver then responds by producing extra protein. Nephrotic syndrome is a combination of proteinuria (excessive protein in the urine), low blood protein and oedema (swelling in the tissues). 

What does Nephrotic Syndrome look like?

It’s not obvious in the beginning that she had Nephrotic Syndrome or that it was her kidneys. She only complained of tummy aches, she vomited quite often and did not feel well every other day. We thought she had food allergies so we took her off cow’s milk and bought soya milk instead and tried to figure out which foods aggravated her bouts of tummy issues. We couldn’t distinguish the culprit in her diet so we tried to look elsewhere. She went to the GP many times, always because we thought she had a tummy bug. The same medicines were prescribed but sooner rather than later, the symptoms were back. We then also noticed that she had puffy eyes, we then thought that it has to be allergies. Back to the GP and more medicines. It was NOT ALLERGIES. Eventually, we got tired of the GP’s and we could see our child was not happy. She looked bloated, puffy eyed and very grumpy from persistent and severe abdominal pain, we then headed straight to the paediatrician. This time, the paediatrician dismissed tummy bugs and allergies like the GP’s kept diagnosing. He ordered blood tests and urine tests.

How is it diagnosed?

Tests and procedures used to diagnose nephrotic syndrome include:

Urine Test: A urinalysis can reveal abnormalities in your urine, such as large amounts of protein, if you have nephrotic syndrome.

Blood Test: If you have nephrotic syndrome, a blood test may show low levels of the protein albumin (hypoalbuminemia) specifically and, often, decreased levels of blood protein overall. Loss of albumin is often associated with an increase in blood cholesterol and blood triglycerides.

And lastly it can be confirmed by means of a kidney biopsy; luckily we didn’t reach this stage of testing.

Now getting samples in order to do these tests are not very easy when the patient is a 3 year old. Blood tests seemed to be the easiest as the blood can be drawn and not waited on or requested from the patient, it does require force to hold her down and atleast two nurses to assist to get blood from her.

Reasonably, one would assume that a pee sample should be simple enough to get. She just needs to pee in a cup and then we’re done. Right? NOOOOOOOOoooooooo!!! Getting a pee sample was a nightmare, a conflict that lasted hours…days even.  To get a 3 year to understand why we need to get her to pee in a cup is the hardest thing ever. She simply refused and chose to hold in her pee until the discomfort level was unbearable to witness. We knew there was a simpler way, we tried to explain to her that she needed to be pee in a cup or just to pee at all or face the consequences of the awkward and distressing CATHETER. Nonetheless, the catheterisation procedure had to be done, twice. It’s not easy for a parent to watch the catheterising of your 3 year old little girl so I’ll just leave it at that.   

Three days in hospital, under observation and constant testing, the blood and urine test confirmed, she suffers from Nephrotic Syndrome.

What causes Nephrotic Syndrome?

Most cases of nephrotic syndrome are caused by changes in the immune system. Certain types of white cells produce a substance that acts on the filters in the kidney to produce a change in the filters. This allows protein to leak into the urine. Other forms of nephrotic syndrome are caused by swelling and increased cell numbers in the filters. In some instances this may result in permanent damage to the filters. 

So, it was not hereditary, it was her immune system. A few weeks/months before the diagnoses, she was ill. The usual illnesses kids who are at a daycare centre experience. She also had grommets inserted in her ears and adenoids removed at a private clinic, in a day ward but we can't say if it was then. We simply can’t pinpoint when her immune system broke down or what exactly happened, it’s not easy for the doctors to say it was this or it was because of that either. All we know is that it was caused by her immune system breaking down and eventually attacking her kidneys.

Life with Nephrotic Syndrome…

Is like any other life except our little girl and the whole family needed an immediate change in diet. As the she was losing excessive protein in the urine a good diet is necessary. A normal or high protein diet is encouraged. Mild reduction of fluid intake will assist in preventing excess swelling of the tissues. Salt reduction should be encouraged by not adding salt to the food. Most children can continue with normal activities. 

Medications

The majority of children with nephrotic syndrome respond to steroid therapy. Prednisolone is the initial drug used with "minimal change" nephrotic syndrome. The urine usually becomes clear of protein within 2-3 weeks. 

Side effects of steroids
MASSIVE APPETITE. We never worry about getting her to eat. She eats so much. We have to worry about what she eats though. Cooking without salt was quite an adjustment but it was a good exercise for the entire family as we all need to improve our food intake, not just for our daughter’s sake but for our own good health.

Is it forever?

No, but it can and did reoccur since her first diagnosis. It can return after an onset of a cold or any other immune weakening illnesses. We have to keep testing her urine to make sure the protein levels are not excessive. The swelling should not be what we look out for as swelling is the sign of severe nephrotic syndrome so urine testing every few weeks is a must.

If the nephrotic syndrome does not respond to the prednisolone a renal biopsy is usually performed. A small percentage of children with other forms of nephrotic syndrome do not respond to drug therapy and they may develop kidney failure. In these patients medications that act on the kidney to increase urine output will decrease the amount of oedema. If high blood pressure is present then it is important that this be well controlled and smoking should be strongly discouraged.

So, Nephrotic syndrome in childhood is a condition that most children outgrow. Relapses are common and are more likely to occur with the common cold. Prednisolone is very effective in controlling the urine protein leak in the majority of cases. Every effort should be made so that the child continues to lead a normal school life.

I will *soon post some food products which have low sodium levels. Unfortunately, nearly ALL foods contain salt so it was not easy to find but I’ll make a list of grocery items and recipes for a nephrotic syndrome sufferer’s diet.

Nephrotic Syndrome facts and medical  info taken from Renal Resource Centre.

* Food products posted, see Nephrotic Syndrome Diet