Nephrotic Syndrome Questions and answers

Bethan OK

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What is NEPHROTIC SYNDROME

What is the cause of NEPHROTIC SYNDROME What is the treatment?

Does the steroid treatment always work?

Are there any side effects from Cyclophosphomide?

What are the side effects of treatment?What is a kidney biopsy?

Will my child continue to suffer from "Nephrotic Syndrome"?

What do I have to do at home?

Will my Childs kidneys fail as He/She gets older?

Clinic Visits

Will my child receive immunisations while taking Prednisolone?

Healthier eating plan.....

When should I get in touch with the Doctor?

As a precaution..... Can my child continue to participate in exercise and sporting activities?

What about holidays?

THIS IS A BRIEF INTRODUCTION TO "NEPHROTIC SYNDROME" AND WHAT IS USUALLY THE CASE IN THE UNITED KINGDOM. TREATMENT AND MANAGEMENT CAN VARY FROM COUNTRY TO COUNTRY AND EVEN FROM TOWN TO TOWN.
WHEN YOU HAVE SPECIFIC QUESTIONS, PLEASE GET IN TOUCH WITH US. WE WILL
EITHER HAVE OR ATTEMPT TO GET THE ANSWER.

"What is NEPHROTIC SYNDROME"
Nephrotic syndrome occurs when the kidneys leak large amounts of protein (mostly albumin) into the urine. Protein lost in the urine results in a lower level of proteins in the blood. It is these proteins in the blood which help to hold water within the blood vessels. Therefore, when the protein level is very low, water passes into the tissues and this is recognised as swelling (oedema).

What is the cause of "NEPHROTIC SYNDROME"
The cause is still unknown but research is beginning to provide more information about the actual changes in the kidney. Nephrotic syndrome affects boys more than girls. The usual age at which the problem begins is 2 to 5 years. It is a rare condition which affects approximately 1 in 50,000 children per year. The condition tends to be more common in families with a history of allergies.

What is the treatment?
Prednisalone (steroid) will be the drug prescribed for your child when he/she is first diagnosed. Most children respond to this drug with disappearance of the protein in their urine and loss of swelling within 1-2 weeks (we call this REMISSION. Occasionally water tablets (diuretics) are also given to control the oedema before the Prednisalone has worked.

As a precaution....
Children who are receiving steroid treatment should carry a steroid warning card with them at all times. Such steroid warning cards are available from the hospital pharmacy. Children who have recently been on steroids may require increased doses if involved in an accident or require an operation. This is the reason for carrying the steroid warning card. You may wish to consider purchasing a Medic Alert pendant or bracelet. This provides emergency identification for medical problems.

What are the side-effects of treatment?
When Prednisalone is prescribed for short periods of time there are usually no serious or permanent side effects. However, we have listed below the side effects which may occur.
a) Decreased resistance to infections such as coughs and colds
b) Increase in appetite which may lead to a rapid increase in weight
c) Flushed, swollen cheeks and stretch marks on the skin
d) Rise in blood pressure which will be monitored at the clinic or at home
e) Behavioural problems e.g. temper tantrums or mood changes and/or depression
f) Steroids in very high doses can cause an increase in blood sugar and fat Lipid levels. Sugar in the urine does not mean your child has diabetes.

What do I have to do at home?
While your child is having treatment with steroids you may be asked to:-
a) Test your child's first morning urine for protein with the urine testing sticks on a DAILY basis, unless told otherwise by your Doctor.
b) Keep a diary of all urine results so that your doctor can review your child’s progress in clinic. The dose of Prednisalone and other comments should also be recorded. The urine test results will show if your child is responding to the prescribed treatment. If your child has a large amount of protein in the urine for three days in a row this may mean there is a relapse (often referred to as 3 plus for 3 days)and you should contact your doctor. If your child has no protein then they are in remission and should be well.

Clinic visits
After your child has been diagnosed as having "Nephrotic Syndrome" he/she will be followed at regular intervals in an out-patient clinic. Even if your child is well it is important that the consultant or his deputy sees your child at regular intervals in order to keep an on his/her progress. At each clinic visit your child will have his/her height, weight and blood pressure recorded as well as analysis of the urine. Physical examination will usually be carried out and only if it is necessary will blood tests be performed. If your child is very worried about blood tests then we can try using a special cream (for example, EMLA cream which makes needle injections less painful.
It is a good idea for you to write down any questions you may wish to ask the doctor before coming to the clinic and you may also have an opportunity to talk to a dietician or social worker.

Does the steroid treatment always work?
There are a very small group of children who do not respond to steroid treatment and continue to have lots of protein in their urine It is this group of children who usually require a kidney biopsy to investigate more thoroughly what is going on in the kidney. Also some children with Nephrotic syndrome RELAPSE more often and begin to have side effects from Prednisalone.
It is in these children that we may decide to use a drug called CYCLOPHOSPHOMIDE which may help the child to have a long REMISSION. Other treatments that may be considered include Levamisole or Cyclosporin.

What is a kidney biopsy?
This is a procedure which is carried out with the child sedated (given a medicine to make your child sleepy). Occasionally in young children a general anaesthetic is required. The child lies flat on his/her stomach and we use an ultrasound scan to find out exactly where the kidneys are and where to insert the needle. The procedure is more uncomfortable than painful and only a very small sample of one kidney is removed using a special needle. The sample is then examined under the microscope and it may take several days before the answer is available.
After the biopsy the child may rest for a while but then can be up and about quite quickly. Your child may be able to go home the same day.

Are the any side-effects from CYCLOPHOSPHOMIDE?
Treatment with Cyclophosphomide is usually 8-12 weeks and this will be discussed with you by the children's kidney specialist before commencing the drug. There are usually no serious or permanent side effects from Cyclophosphomide. However we have listed the side effects which may occur.
1. Thinning of the hair - this is usually only slight and the hair will regrow after the course of treatment.
2. Irritation of the bladder - sometimes Cyclophosphamide can irritate the bladder and give rise to blood in the urine. By giving the drug first thing in the morning with plenty of fluids this should not occur.
3. Cyclophosphamide can affect the number of white cells in the blood, which help to fight infection. Your child may therefore be more susceptible to catching infections. A weekly blood test should be carried out to check that the cell count is not too low and the dose can be adjusted as necessary.
4. Future fertility. The short course of Cyclophosphomide now used should not have any effect on your child’s fertility however this point should be discussed with the consultant.

Healthier eating plan
It is recommended that your child follows a "healthy eating plan" No added salt - avoid adding salt to food at the table and try to reduce the intake of processed foods e.g. crisps, tinned and packet foods
Ensure an adequate protein intake - it is not usually necessary to take extra protein in the diet, a good healthy appetite will usually ensure an adequate intake, however it may be necessary in some cases. Ask your dietician.
Eat more fibre rich foods-try using wholemeal bread, whole-wheat breakfast cereals, fruit and vegetables
Reduce sugar-try to avoid adding sugar to drinks and food, replace fizzy drinks with sugar free varieties. Try to cut down on sweets and chocolate.
Replace saturated fats with mono or polyunsaturated fats.

Will my child recieve immunisations while taking PREDNISALONE?
Not usually. Steroids can reduce the effectiveness of immunisations and relapses can sometimes be caused by the vaccination.
Children who are having steroid drugs or Cyclophosphamide can become very unwell if exposed to CHICKENPOX or MEASLES. Let the hospital know if your child is in very close contact with another child who may have chickenpox or measles. Speak to your child's school teacher about the possibility. If your child develops diarrhoea and/or vomiting while taking steroids contact your doctor.

Will my child always have "NEPHROTIC SYNDROME" ?
Most children will have at least one recurrence of protein in their urine (we call this a relapse) and they will need further courses of steroid treatment.
If they have many relapses they will need to take their steroids for a longer period of time.
As the child becomes older the relapses usually become less frequent. It isn't possible to predict accurately when the relapses will cease but a relapse is very uncommon if the child has been free of protein in the urine for five years.

Will my child's kidneys fail as he/she gets older?
Children with the usual types of Nephrotic syndrome which responds to steroids usually have NO risk of the kidneys failing with time.

Can my child continue to take part in sports/games?
Yes. Exercise is important for all the family. The school should allow the child to participate in all sports within the child's capabilities. If you are unsure about certain sports ask your doctor.

What about holidays?
Unless your child is having complications a normal family holiday should be possible. It is worth making sure though that medical insurance will cover "pre-existing conditions" Also it is worthwhile telling your Doctor of your plans.

WHEN SHOULD I GET IN TOUCH WITH THE DOCTOR?
1. If your child is in close contact with chicken pox or measles contact your doctor within 24 hours as he/she may need a booster injection to increase their resistance to these infections.
2. If your child shows protein ++ or greater in the urine for 2 consecutive days.
3. If your child is "puffy", unwell or has a fever.
4. If your child has diarrhoea or vomiting.
5. For any small worry it is best to phone, no matter what time it is.

Model Model Kidney image courtesy of www.3dscience.com