A guide to the treatmentand management of

Childhood Nephrotic Syndrome

CONTENTS

Introduction

What is Nephrotic Syndrome?

What is the cause of Nephrotic Syndrome?

What is the treatment?

Side-effects of prednisolone

What to do at home?

The importance of a diary

Keeping a diary

Clinic visits

Does the steroid treatment always work?

What is a kidney biopsy?

Side effects of Cyclophosphamide

Is there a special diet?

Immunisations

Will my child continue to suffer fromNephrotic Syndrome?

Sport and exercise

Holidays

When to call the doctor

The Parents Group

Useful telephone numbers

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INTRODUCTION

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You have recently learned that your child hasNephrotic Syndrome. This causes problems whichrequire special attention. Hopefully as your childgrows older the nephrotic syndrome will becomeless troublesome.

This booklet has been prepared to help parentsunderstand the treatment and management ofchildhood Nephrotic Syndrome.

?WHAT IS

NEPHROTIC SYNDROME?

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Nephrotic Syndrome occurs when the kidneysleak large amounts of protein (mostly albumin)into the urine. Protein lost in the urine results ina lower level of proteins in the blood. It is theseproteins in the blood which help to hold waterwithin the blood vessels. Therefore, when theprotein level is very low, water passes intothe tissues and this is recognised as swelling(oedema).

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WHAT IS THE CAUSE OFNEPHROTIC SYNDROME?

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The cause is still unknown but research isbeginning to provide more information aboutthe actual changes in the kidney.

Nephrotic Syndrome affects boys more thangirls. The usual age at which the problem beginsis 2 to 5 years.

It is a rare condition which affects approximately1 in 50,000 children per year. The conditiontends to be more common in families with ahistory of allergies.

WHAT IS THE TREATMENT?

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Prednisolone (steroid) will be thedrug prescribed for your childwhen he/she is first diagnosed.

Most children respond to this drugwith disappearance of the protein in their urineand loss of swelling within 1-2 weeks (we callthis REMISSION).

Diuretics (water tablets) may also be given tocontrol the oedema (swelling) before thePrednisolone has worked.

Penicillin V may be prescribed if your child hasa lot of swelling as excess fluid in the tissuesprovides a good environment for bacteria togrow.

AS A PRECAUTION

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Children who are receiving steroid treatmentshould carry a steroid warning card withthem at all times. Such steroid warning cardsare available from the hospital pharmacy.

Children who have recently been on steroids mayrequire increased doses if involved in an accidentor requiring an operation. This is the reasonfor carrying the steroid warning card.You may wish to consider purchasing aMedic Alert pendant or bracelet (thisprovides emergency identificationfor medical problems).

WHAT ARE THE SIDE-EFFECTSOF TREATMENT?

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When Prednisolone is prescribed for short periodsof time there are usually no serious or permanentside effects.

However, we have listed below the side effectswhich are most common

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, bed wetting or mood changes.

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

While taking steroid medication the skinbecomes more sensitized to the effects of the sun and extra care should be taken to apply sun screen whenever sun exposure is expected.

WHAT DO I HAVE TO DOAT HOME?

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While your child is having treat-ment with steroids you will betaught to:

l Test your child’s first morningurine for protein with the urinetesting sticks on a DAILY basis,

unless told otherwise by your doctor /renal nurse.

l Keep a diary of all urine results so thatyour doctor can review your child’s progress inclinic. The dose of Prednisolone and anyother medications and othercomments should alsobe recorded.

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WHY IS KEEPING A DIARYIMPORTANT?

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The urine test results will show if your child isresponding to the prescribed treatment. If yourchild has a large amount (2+) of protein in theurine for three days in a row this could meanthere is a RELAPSE of the conditions and youshould contact your doctor / renal nurse. If yourchild has NO protein in the urine for three ormore days he / she is in REMISSION and shouldbe well. Records that are complete and accurategive the doctor a good idea of your child’sgeneral health and progress and this recordshould be brought to each clinic visit.

Here is an example of how to record your child’sprogress.

DATE:JULY 08

123456789

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URINE TEST

N (negative)0.3g (trace)N (negative)N (negative)N (negative)N (negative)

1.0g (++)3.0g (+++)3.0g (+++)>5g (++++)

PREDNISOLONEDOSE

10mg-

10mg-

10mg-

10mg-

10mg

OTHERTREATMENT

----------

COMMENTS

ColdWellWellWellWellWell

Pale & not feeling wellUnwellUnwell

PHONE DOCTOR

How you record the urine test will depend uponthe testing sticks used.

CLINIC VISITS

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After your child has been diagnosed as havingNephrotic Syndrome he/she will be followed atregular intervals in the out-patient clinic. Evenif your child is well it is important that theconsultant or his deputy sees your child atregular intervals in order to keep an eye onhis/her overall progress.

At each clinicvisit your child will have his /

her height, weight and blood pressurerecorded as well as analysis of the urine.

Physical examination will usually be carried outand only if it is necessary will blood tests beperformed. If your child is very worried aboutblood tests then we can try using a specialcream, or spray which makes this procedureless painful.

It is a good idea for you to write down anyquestions you may wish to ask the doctorbefore coming to the clinic and youmay also have an opportunity totalk with the renal nurse,dietitian or socialworker.

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DOES STEROID TREATMENTALWAYS WORK?

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There is a very small group of children whodo not respond to steroid treatment andcontinue to have lots of protein in their urine.It is this group of children who may requirea kidney biopsy to investigate morethoroughly what is going on in the kidney.Also some children with Nephrotic SyndromeRELAPSE more often and beginto have side effects fromPrednisolone.

It is for these children that wemay decide to use a medicationcalled CYLCOPHOSPHAMIDE whichmay help the child to have along REMISSION (in other wordsurine free of protein and offPrednisolone). Other medications thatmay be considered include LEVAMISOLE,CYCLOSPORIN, TACROLIMUS.

The doctor will always discuss the need forfurther investigations and treatment before abiopsy or other medications are suggested.

WHAT IS A KIDNEY BIOPSY?

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This is a procedure which is carried out with thechild sedated (medicine will be given through a

cannula to make your child sleepy).Occasionally in young children a generalanaesthetic is required. The child lies flat

on his/her stomach and we use anultrasound scan to find out exactly where the

kidneys are and where to insert the needle.The procedure is more uncomfortable thanpainful and only a very small sample of one

kidney is removed using a special needle.The sample is then examined under themicroscope and it may take several daysbefore the answer is available.

After the biopsy the child mayrest for a while but then can be upand about quite quickly. Yourchild may be able to go homeafter the biopsy the same day.

The biopsy should have no long-term effect onthe kidney. We generally restrict heavy exercisefor one week after the hospital admission butyour child can safely return to school.

ARE THERE ANYSIDE EFFECTS FROM

CYCLOPHSOPHAMIDE?

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The course of treatment withCyclophosphamide is usually 8-12 weeks

and this will be discussed with youby the children’s kidneyspecialist before commencingthe drug.

There are usually no serious orpermanent side effects from

Cyclophosphamide when given atthe low doses used to treat Nephrotic

Syndrome. However we have listed the sideeffects which may occur.

1. Nausea and vomiting – anti sickness drugs canbe given to reduce this

2. Thinning of the hair – this is usually only slightand the hair will re grow after the course oftreatment.

3. Strange taste – your child may experience astrange taste in their mouth while receivingcyclophosphamide. This is only temporary.

4. Cyclophosphamide can affect the numberof white cells in the blood which helpto fight infection. Your child maytherefore be more susceptible tocatching infections. A weekly bloodtest will be done to check the whitecells are not low and the dose ofCyclophosphamide will bealtered if necessary.

5. Future fertility – the short course ofCyclophosphamide that we now use should nothave any long term effects on your child’sfertility. However, this point will be discussedfully with you by the specialist.

There are other less common side effects, thesewill be discussed with your doctor and mayinclude:

1. A slight increased risk of developing someforms of cancer, this is more likely withindividuals taking higher or longer term doses

2. Irritation of the bladder wall, which may leadto blood in the urine.

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IS THERE A SPECIAL DIET?

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When in remission A ‘Healthy Eating’ diet isgenerally recommended for your child. It is agood idea for all members of the family to alsofollow a healthy eating plan.

At other times when your child may be in aNephrotic state diet and fluid restrictions orguidelines may be in place – and should befollowed.

GUIDELINES TO FOLLOW

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l no added salt – avoid adding saltto food at the table and try toreduce the intake of processedfoods e.g. crisps, tinned andpacket foods.

l ensure an adequateprotein intake – it is notusually necessary to take extraprotein in the diet, a good healthyappetite will usually ensure an adequate intake.

l eat more fibre rich foods – try using whole-meal bread, whole wheat breakfast cereals, fruit

and vegetables

l reduce sugar – try to avoidadding sugar to drinks and

food, replace fizzydrinks with sugar free

varieties. Try to cut downon sweets and chocolate.

l replace saturated fats with mono orpolyunsaturated – use margarines and oils

labelled ‘high in polyunsaturates, low incholesterol’.

This advice should be treated as a HEALTHIER wayof eating rather than as a diet.

Further dietary information and advice can beoffered to you from the Paediatric Renal Dietitianwhose number is in the back of this booklet.

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CAN MY CHILD RECEIVEIMMUNISATIONS WHILE TAKING PREDNISOLONE?

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Not usually. Steroids can reduce theeffectiveness of immunisations and relapsescan sometimes be caused by the vaccination.

Children who are having steroid medications orCyclophosphamide can become very unwell ifexposed to CHICKENPOX or MEASLES. Let thehospital know if your child is in very closecontact with another child who may havechickenpox or measles.

Speak to your child’s school teacher aboutthe need to be advised in the event ofinfectious conditions in the their class.

If your child developsdiarrhoea and/or vomitingwhile taking steroidscontact your doctor.

WILL MY CHILD CONTINUETO SUFFER FROM

NEPHROTIC SYNDROME?

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Most children will have at least one relapse, withsignificant protein in their urine (2 – 3+ for 3 daysor more) and requiring a further course of steroidtreatment.

If they have many relapses they will need to taketheir steroids for a longer period of time.

As the child becomes older the relapses usuallybecome less frequent. It isn’t possible to predictaccurately when the relapses will cease but arelapse is less likely if the child has been free ofprotein in their urine for five years.

Will my child’s kidneys fail as he/she getsolder?

Children with the usual types of NephroticSyndrome, which respond to steroids,have no increased risk of their kidneysfailing with time.

Are other children in the family likelyto be affected?

There are a few rare types ofNephrotic Syndrome whichdo run in families, but it isextremely unlikely thatyour child will sufferfrom these.

CAN MY CHILD CONTINUE TOPARTICIPATE IN EXERCISE

AND SPORTING ACTIVITIES?

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YES. Exercise is important for all the family.

The school should encourage the child toparticipate in all sports within the child’scapabilities.

If you are unsure about certain sports ask yourdoctor / renal nurse

WHAT ABOUT HOLIDAYS?

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Unless your child is having complications, anormal family holiday should be possible.

A copy of your latest clinic letter shouldaccompany you and also an adequate supply ofprednisolone in case of relapse.

Remember the need for extra sun protectionwhen taking specific medications

Always ensure adequate hydration andmovement if undertaking a long haul flight

Tell your doctor / renal nurse about your plans.

WHEN SHOULD I GET INTOUCH WITH THE DOCTOR/

RENAL NURSE?

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1. If you child is in close contact with chickenpoxor measles contact the hospital unit within 24hours as he/she may need a special injection toboost his/her resistance to these infections.

2. If your child has protein (++ or greater) in theurine for three days.

3. If your child is puffy, unwell or has a fever.

4. If your child has diarrhoea and vomiting.

5. For any small worry it is best to phone, nomatter what time, as it may be important. Thenumbers you can contact if you have any worriesare at the back of the booklet.

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In some areas the Renal or Community Nurseor Health Visitor may visit you at home. Ifappropriate the renal nurse or community nursemay visit your child’s nursery or school.

You will see the Renal Nurse at clinic visits andwhen admitted to the hospital

Discussion with the renal social worker may behelpful. It may be possible to receive practicalassistance and more importantly support indealing with your family’s concerns.

THE PARENT’S GROUP

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www.nephrotic-natter.org and www.nephrotic-natter.co.uk - An on-line resource for families andpatients, providing an opportunity to shareexperiences, problems, and questions withothers who are similarly affected.

Some hospitals have parent’s groups and sometry to have an occasional parents’ evening duringthe year where parents can get together anddiscuss any worries. These are usually informalevents and give an opportunity for everyone toshare their experiences and common problems.

Even if there is no formal parents’ group, yourdoctor, nurse, or paediatric social worker shouldbe able to help you contact another parent who has a child with Nephrotic Syndrome.

This booklet will not answer all your questions orgive all the information you wouldlike, so please ask questions as weare always willing to help.

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HERE ARE SOME USEFULTELEPHONE NUMBERS:

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NOTES

Compiled by the members of the

Design: Room at the Top Design Nottingham • Illustrations: Jan Smith

©ARW 1988 • REPRINT 1997 • REVISED 2008