Biliary Atresia2012.pdf

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Biliary Atresiaa guide

Medical Information Series

fighting childhoodliver disease


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4

This leaflet has been written for:

Parents/carers of a child with biliary atresia

Young people diagnosed with biliary atresia (as a baby)

Others who may find this leaflet helpful are:

Relatives and friends of families

Healthcare and allied professionals, school, college, university and

nursery teams

This leaflet aims to:

Explain biliary atresia Explain the symptoms and diagnosis

Discuss treatment and possible complications

You may find it helpful to read the following CLDF leaflets:

Conjugated jaundice in babies

Guide to the liver

Glossary of terms

Routine investigations for liver disease

Childrens Liver Disease Foundation (CLDF) also has leaflets in its support series

which are available to download from our website childliverdisease.org. Leaflets

can be mailed to UK patients free of charge, our contact details are on this

leaflet. You may find it helpful to have a copy of CLDFs making the most of an

appointment leaflet which will help you prepare for appointments and meetings

following discharge.

If you have been given this leaflet at a UK hospital you should have received a

CLDF Introduction Pack, supplied, free of charge, to UK hospitals. We are happy

to send you an Introduction Pack as well as a CLDF Pack if you live in the UK.

Our contact details are on this leaflet.

We also have the following information packs available, free of charge:

Nurseries, schools and colleges

GP Practices

Friends and Families

All are available on request to UK families and young people.

Overseas families should contact CLDF to discuss their literature needs.

Welcome


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What causes biliary atresia?

The cause of biliary atresia is not known.

Research is being carried out but it has not

yet provided any answers. Biliary atresia,

however, is not a hereditary condition.

Parents frequently feel guilty that they may

have in some way have contributed to their

child having this problem. These feelings

are groundless as at present there is no

known way of preventing or anticipating the

occurrence of biliary atresia.

Introduction

One important function of the liver is to make a substance

called bile. Bile is a liquid which passes into the gut through small

tubes called bile ducts. Bile is necessary for the proper digestion

of fat within the diet.

Showing the normal liver, gall bladder, bile ducts

and upper guts.

Showing the same structures in a child with

biliary atresia. The darkened areas represent the

damaged bile ducts.

Diagram 2Diagram 1

What is biliary atresia?

Biliary atresia is a condition in which

inflammation develops within the bile ducts

around the time of birth. The inflammation

can occur in any of the bile ducts both

inside and outside the liver. This leads to

bile duct damage and reduces the flow of

bile which subsequently causes scarring

(fibrosis) of the liver. The bile ducts outside

the liver are always irreversibly damaged

preventing any bile flow into the gut.

The gall bladder is also affected.

01Biliary Atresia a guide


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What are the signs ofbiliary atresia?

In the first weeks of life babies with biliary

atresia often seem well, apart from being

jaundiced (yellowing of the skin and whites

of the eyes).

Jaundice in babies is very common and

mostly it clears within the first two weeks of

life. Childrens Liver Disease Foundation has

a booklet explaining baby jaundice which

is available on request. If the cause of the

jaundice is liver disease then frequently the

jaundice does not fade.

Other important signs are:

Yellow coloured urine

Normally the urine of a newborn baby is

colourless. If a babys urine is persistently

yellow this can be an indication of liver

disease.

Pale stools

Normally the stools of a baby are greenor yellow. If the stools of a baby are grey,

white, fawn or pale then this can be an

indication of liver disease.

Bleeding

Prolonged bleeding from the umbilicus

or bleeding elsewhere (e.g. a nosebleed)

may be an indication of liver disease

.How is biliary atresiadiagnosed?

The signs that have been described

are non-specific and can indicate other

disorders. Therefore, there will need to be a

number of investigations in order to make a

diagnosis. The tests need to be carried out

in hospital and include blood tests, scans

and liver biopsy. Your baby will be admitted

to hospital for a period of about a week for

the tests.

Once other causes of liver disease have

been excluded and investigations indicate

biliary atresia, your baby will be scheduled

for surgery. The doctors and nurses will

explain to you the type of surgery planned.

The diagnosis and type of biliary atresia will

only be confirmed at the time of operation.

Biliary atresia splenic

malformation syndrome (BASM)

In 1020% of infants with biliary atresia,

additional abnormalities may be found.

These may include:

A spleen which is in multiple pieces

(polysplenia) or absent (asplenia). The

spleen is an organ which sits under

the left lower ribs next to the stomach

and helps to maintain immunity against

infection and removes blood cells,

especially platelets at the end of their life.

It is normally a solid single organ.

An abnormal arrangement of intestine,

known as intestinal malrotation.

Some abnormalities of the veins in the

abdomen.

Heart defects.

The presence of some or all of these

features make up a condition known

as biliary atresia splenic malformation

syndrome. This subgroup of children with

biliary atresia may be less likely to clear

their jaundice after a Kasai operation. They

also need additional tests such as a heart

scan to check for problems. They are

usually advised to have immunisations and

take daily antibiotics to reduce their risk of

infection from poor splenic function.

02 Biliary Atresia a guide


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How can biliary atresiabe treated?

The initial treatment for biliary atresia is

a surgical operation called the Kasai

procedure after the Japanese surgeon

who pioneered the operation. The aim of

the Kasai procedure is to allow bile to

drain from the liver into the gut.

Babies born with prolonged jaundice and

suspected of having a liver disease such

as biliary atresia will often need special milk

formula. The hospital dietitian will advise.

There are a number of special milks for

babies suffering from liver disease. Thesemilks have fats which are more easily

digested by babies with liver disease. Extra

calories may also be added to the feeds

in the form of powders to help your baby

maintain an adequate weight gain. Most

babies with biliary atresia are unable to gain

weight satisfactorily on breast milk alone. In

these cases the dietitian will advise how to

combine breast and bottle-feeding.

The doctor may prescribe extra vitamins as

babies with prolonged jaundice also have

problems absorbing certain vitamins.

What happens before theoperation?

When any operation is needed some

preparation is necessary. Blood tests will

be done so that your babys blood group

can be identified in case there is a need to

give blood to your baby before, during or

after surgery. Your baby may need special

medicines to prepare the gut for surgery.

Your baby may also need some feeds to

be replaced by special non-milk feeds and/or some fluids to be given into the vein by

means of a drip. The nurses in the ward will

tell you what you should give and when.

If you wish, you will normally be allowed to

go down to the operating theatre with your

baby and stay until he/she is asleep.

What happens duringthe operation?

The aim of the operation is to make a

drainage channel to allow bile to drain

from the liver. The exact way in which this

is done will depend on the type of biliary

atresia your baby has. During the operation

the surgeon will examine the liver, look

for abnormalities and may take a liver

biopsy, i.e. remove a tiny piece for further

laboratory tests. The operation usually takes

a whole morning or afternoon.

There are 2 types of operation:

Type A

The surgeon does not find an open major

bile duct outside the liver. See diagram 3.

This is the most common finding.


Diagram 3


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All the abnormal bile ducts outside the liver

are removed completely. At the highest

point there are usually enough microscopic

bile ducts which, if drained, will allow some

bile flow to be re-established. The surgeonthen joins a loop of intestine to the under

surface of the liver.

Type B

The surgeon finds that there is a blockage

in the bile duct outside the liver. See

diagram 5.

The blocked bile duct is removed. This

leaves a single open major bile duct that

drains the liver. This is then joined to a

loop of intestine. Usually there is a better

restoration of bile flow from the operation

and jaundice tends to resolve quicker.

What happens immediatelyafter the Kasai operation?

As soon as your baby returns from the

operating theatre and the nurses have

settled him/her you will be able to sit by

the cot, hold your babys hand and begin to

help with care, e.g. changing nappies.

There will be a tube running up your babys

nose and down into the stomach, this is

called a naso-gastric tube. This usually

drains into a clear bag or small pot and

keeps the stomach empty which helps to

prevent vomiting.

There will also be an intravenous infusion or

drip into a vein, usually in the back of the

babys hand. This is used to give:

The surgeon will carry out the following

operation, called the Kasai procedure

(Kasai portoenterostomy).

Diagram 4

The surgeon will carry out the following

operation called a Hepaticojejunostomy.

Diagram 5


Diagram 6


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Fluids

These are given in order to maintain

the correct sugar, salt and water levels

in your babys body. Daily blood tests

will be done to check these levels so

they can be adjusted as needed. All

fluid needed by your baby will be giventhrough the drip until feeding is restarted.

Pain relieving drugs

These are often given continuously

through the drip. A special pump may

control the rate of this. Sometimes

a baby will receive pain relief via an

epidural catheter a fine tube inserted

into the back at the time of operation

and similar to that used in some women

in labour. Pain relief will be discussed

with you.

Antibiotics

These will be given immediately after

the operation for at least 48 hours. If

your baby does not develop a high

temperature and once oral feeds are

started, these will often be given orallyrather than through the drip.

A dressing will cover the wound on your

babys tummy for the first few days.

The dressing will be changed as necessary.

The stitch(es) do not need to be removed

as they dissolve after the wound

has healed.

By the following day it may be possiblefor you to hold your baby, the nurse will

help you. If your baby is upset a dummy

often helps.

The doctor will listen to the tummy with a

stethoscope for the sounds of the bowel

starting to work normally again after the

operation. Once this happens, usually

about the third day, the babys feeds willbe restarted gradually.

If your baby was being breast fed before

diagnosis at least a partial return to this

method may be possible. It is important

that the milk has been maintained by

expressing the milk while your baby is

unable to feed. The ward staff will help and

advise you with this.

When will my baby beable to leave hospitaland what happens next?

Providing that there are no complications

following surgery, your baby will be

home within two weeks of the operation.

Before returning home the wound must

be healed and your baby must have a

normal temperature and have started to

put on weight. You will be given or sent an

outpatient appointment.

Before your baby leaves hospital you will

be taught all about the special medicines

and feeds. You will be given an initial supply

and you can arrange more through yourGP. It is quite common for the hospital

staff to advise your health visitor, GP and

local hospital about your childs condition

and any special aspects of care which are

needed. They can often offer you useful

support when you are at home.

When you get home you need to try and treat

your baby as normally as possible. Obviouslyyou will be worried at times and this is

quite normal. Babies with biliary atresia get

common illnesses, just like any normal baby.

Symptoms for which you should seek advice

from your GP or hospital are:

Your baby is obviously unwell

Raised temperature for more than

24 hours More jaundice accompanied by

paler stools and darker urine



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Unless there is some problem other than

biliary atresia it is very important that your

baby is immunised against Diphtheria,

Whooping Cough, Tetanus, Polio, Hib

and MMR in the normal way. It may be

advised that your baby has Prevanar a

new anti-pneumococcal vaccine, especiallyin the BASM patient. It does not matter if

the immunisations are a little late starting

because of the operation and hospital stay.

You will be advised that your baby should

receive vaccination against hepatitis A

and B.

If you have any queries regarding your

child, please do not hesitate to contact your

Specialist Nurse at the liver unit treating

your child. If you would just like a chat and

some reassurance the staff are always at

the end of a phone, and like to hear how

you are getting on.

Childrens Liver Disease Foundation

provides family support and will be pleased

to hear from you. The contact details for

CLDF are in this leaflet and the numbersfor the hospital will be given to you before

you leave.

What medicines may mybaby need and why arethey given?

The medicines needed will depend uponyour childs condition and progress but

some commonly used are:

Antibiotics

After the operation a course of

antibiotics is initially given by drip and

completed by mouth. They are used to

reduce the risk of infection spreading

from the gut into the liver (cholangitis).

Further courses will be prescribed as

needed.

Vitamins

Additional vitamins are necessary

because poor bile flow can reduce

absorption of vitamins. Some may need

to be given by injection.

Phenobarbitone

This may be given to increase the

flow of bile. As it may make your baby

sleepy at first it is usually given in one

dose a day, in the evening.

Questran

This comes in the form of a powder

in individual sachets. Each sachet is

mixed with water and given in liquid

form. This may be given to improve liverfunction and remove substances which

may otherwise cause the skin to itch.

Questran can affect the absorption

of some drugs, especially vitamins.

Therefore, with babies it is best to

give some at each feed time except

when vitamins are given. As your baby

becomes older and manages larger

volumes the daily dose of Questran can

be given all at once or split into two. For

a toddler or older child needing Questran

it is best given in one or two doses away

from the main meal times and vitamins.

Always leave at least two hours between

giving Questran and vitamins.

Spironolactone

This may need to be given to helpyour baby get rid of excess fluid which

otherwise collects in the abdomen and

is called ascites. It is not needed in

all cases.

URSO (Ursodeoxycholic acid)

This drug may be given at the

onset of feeding after the Kasai. It is

thought to help promote the flow of bile.



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Ranitidine

It is common to find stomach irritation

after any type of abdominal operation

and this drug may be given to help

alleviate this problem.

If your baby/child needs medicine to reduce

a temperature, remember aspirin and drugs

containing aspirin must not be given. Use

paracetamol (panadol/calpol). Ibuprofen

should not be given.

How is it possible to tellif the operation has beensuccessful?

At the time of the operation the surgeon

will not be able to tell if bile drainage will

occur satisfactorily. The first stools after

the operation are usually very dark, then

they will become paler. If bile drainage

does occur the stools gradually change to

a yellow/green/brown colour and later the

urine becomes paler.

The colour of the stools will be monitored

whilst your baby is in hospital. It is not

unusual for there to be some variation in

the colour of the stools. It is impossible to

say how long it may take to observe these

changes, as every baby is different.

If bile drainage is established the jaundice

gradually fades and blood tests showthat the chemical, bilirubin which causes

jaundice, drops towards a normal level.

Since the operation may not drain bile from

all sections of the liver it is not unusual for

liver function tests to remain abnormal even

if the jaundice completely clears.

Approximately two-thirds of babies clear

their jaundice after a Kasai operation. There

may be a higher chance of success if their

baby is less than eight weeks old at the

time of the operation. However, even some

of the children who lose their jaundice may

develop some of the complications due to

liver damage. So all children continue to be

seen for regular check-ups.

More than 90% of patients with biliary

atresia can be expected to survive these

days. Of those that clear their jaundice

some will achieve completely normal liver

function and are unlikely to experience

further problems. Children who clear

their jaundice but who have persistently

abnormal liver function tests may develop

treatable problems such as portalhypertension or deteriorating liver function

eventually requiring liver transplantation.

Most children who undergo successful

Kasai portoenterostomy will be well with

their own liver ten years later. Results in the

UK are equally encouraging although the

operation has only been performed here

since 1973.

What happens if the biliaryatresia is not treated ortreatment is unsuccessful?

In these cases bile produced in the liver

builds up causing damage to the cells

of the liver eventually resulting in severe

scarring (cirrhosis). If this continues liverfunction deteriorates, complications occur

and general health suffers. In the past sadly

these children often died in infancy, but

liver transplantation has altered this outlook

and is now an accepted form of treatment.

It is obviously a major undertaking and is

only considered when other treatments are

ineffective.



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Liver transplantation became available

for children in the 1980s, the results

are now encouraging and are steadily

improving. There is an overall 90% one-year

survival rate for liver transplantation. Liver

transplantation is now possible in children

of any age.

If liver transplantation is indicated for your

child this will be discussed with you by the

team caring for you and your child. Diet

and medication will be intensified in order

to ensure that your baby or child is as well

as possible leading up to transplant, and

progress will be monitored. The aim of liver

transplantation is to restore your child to

good health.

What other problems

might occur?

Even after a successful Kasai operation

(when jaundice has completely cleared) a

number of problems may occur of which

you should be aware:

Cholangitis

This is an infection if the bile ducts in the

liver resulting in inflammation.

This may cause a fever or worsening

or recurrent jaundice or both. It needs

urgent treatment with intravenous

antibiotics which can only be given

at your local hospital or the liver unittreating your child. If the treatment is

given locally, please ask the doctors to

contact the liver unit treating your child.

If cholangitis is left untreated it can

cause further damage to the liver. That

is why prompt investigation of any

high temperature (over 37C) or a non-

specific illness lasting for more than

24 hours is important.

Cholangitis is most likely in the first year

or two after the Kasai procedure.

Ascites

This is pronounced Ay-sigh-tees

and is an abnormal collection of fluid

in the abdomen. CLDF produce a leaflet

on ascites and it can be obtained

on request.

A small amount of ascites can be present

for up to six weeks after the operation

but should then disappear. If it recurs at

any time this is usually a sign that the

disease has affected the ability of the

liver to make a protein called albumin.

One of the blood tests frequently needed

after the operation and each time blood

is taken in outpatients, is to measure thealbumin level. When the albumin level

is too low, water from the childs blood

leaks out of the blood vessels and

collects in the abdomen. In severe

cases it may also collect in other parts

of the body.

The first sign of this problem is often

when clothes and nappies do not fit so

well over the tummy. This usually occurs

gradually but may be started or made

worse by infection.



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Biliary Atresia a guide

The doctor may measure your childs

girth at each hospital visit and/or may

ask you to do this at home. If your

childs tummy becomes noticeably

bigger while at home you should see

the GP to exclude other causes such

as wind or constipation for which he/shecan advise you. Treatment for ascites

may also be started by your GP or local

Paediatrician in consultation with the

liver unit and does not necessarily mean

admission to hospital.

Treatment is initially by medicine and/

or altering the diet to maintain calorie

intake but reduce fluid and salt intake. In

more serious cases it may be necessary

to admit your child to hospital and give

albumin intravenously through a drip.

You should never attempt to alter your

childs diet and, in particular, their fluid

intake without medical advice.

Portal Hypertension

Portal Hypertension means high blood

pressure in the portal vein, the main veincarrying blood from the gut to the liver

and is different from high blood pressure.

It may occur due to scarring in the liver

which causes back pressure in the portal

vein. This may cause veins like varicose

veins to develop in the lining of the

gullet, stomach or gut.

These veins become fragile and bleed.It is rare for this to occur before the

child is 2 years old. Bleeding can cause

black stools, marked pallor or vomiting

of blood. If bleeding does occur, then

contact your local hospital immediately.

Treatment is available.

Childrens Liver Disease Foundation has

a separate leaflet on portal hypertension.

Never give your child aspirin or

ibuprofen or any medicines that

contain aspirin as this may trigger

the onset of bleeding.

Itching (Pruritus)

Some children with reduced bile flow

experience itching of the skin. This can

develop at any time and can make your

child quite irritable and miserable. If you

think your child may be affected consult

your GP or liver unit. Additional medicine

may be required.

A leaflet on itching (pruritus) is available

from the Childrens Liver Disease

Foundation.

What will happen in thefuture?

Your baby/childs growth and development

will be followed up by periodic visits to the

hospital. The frequency will vary with each

child. Blood tests will be done to measure

the bilirubin level and the liver function.Periodically an ultrasound will be performed

to assess the size of the liver and other

structures in the abdomen (e.g. the spleen).

09


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Is there a charity fightingto stamp out childhoodliver disease?

Yes. Started by families in 1980, Childrens

Liver Disease Foundation (CLDF) leads the

way in fighting all childhood liver disease.

CLDF funds vital research, develops

information and awareness programmes

and supports families, young people and

adults diagnosed in childhood who are

living day in, day out with a liver condition

or transplant. And its work has made a big

difference and continues to help save lives.

CLDF has so much to offer you:

information, the opportunity to meet other

families, events and regular updates. To find

out more, call, email or write today:

Childrens Liver Disease Foundation,

36 Great Charles Street, Birmingham,

B3 3JY

0121 212 3839Main site: childliverdisease.org

Young peoples site: cldf-focus.org

[email protected]

What are the roles of CLDFsFamily and Young Peoplesteams?

CLDFs Family and Young Peoples teamsare here for you, whether you want to talk

about issues affecting you, meet and share

with others or just belong to a group which

cares, knows what its like and is fighting to

make a difference. You are not alone.

Our parents say . . .

. . . We dont know how we would have

coped without CLDFs care and support.

They have been just fantastic from the

outset tremendous people, who are

compassionate and so positive. They really

care about families and children struggling

with liver disease.

When Emily was very ill we felt we were on

the sidelines, knowing we couldnt influence

the outcome and not in control. Getting

involved in fundraising is something you can

control and achieve a positive result. I really

took comfort from that.

Our young people say . . .

Knowing CLDF is there is what I need.

I can call whenever I want. Whatever I think

and feel is listened to. Even when I called to

tell them it was my birthday!

I feel really well. Its great that CLDF has

given us the chance to meet other young

people outside of the hospital and have afun time. I want them to do more things

like this.

Families Team

[email protected]

0121 212 6008

Young Peoples Team

[email protected] 212 6009

Childrens Liver Disease Foundation: 2002

Updated: November 2005; May 2006; February,

September 2008; July 2010; April 2012

Biliary Atresia a guide 10


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Donation, Regular Gift & Gift Aid Declaration Form

To make a one-off gift or set up a direct debit gift online,go to childliverdisease.org

Id like to make a gift to CLDF

How much? 10 20 Other ...............

I enclose a cheque made payable to Childrens Liver Disease Foundation I wish to pay by card MASTERCARD/VISA/ DEBIT CARD (delete as appropriate)

Card No. Expiry Date ...... /...... /......Name on Card ............................................. Security Number: ................ (back of card)

Id like to make a regular gift by direct debit to CLDF

How much? 5 10 20 25 50 other ...........

How often? monthly quarterly half-yearly annually

My bank details:

Bank name: ...................................................... Branch name: ..............................................

My bank address: ..................................................................................................................

.................................................................................................. Postcode: ............................

My bank sort code: My bank account number: .........................................Please pay to Childrens Liver Disease Foundation, account no. 00181442, sort code: 12-05-65

Starting on ...... /...... /...... until further notice. My signature: ................................................

Are you a UK taxpayer? Yes / No If yes, please give your gift under Gift Aid.

This means that CLDF can claim the basic rate of tax you have already paid on theamount you are donating. It will not cost you anything.

In order to qualify you must have paid enough UK income or capital gains tax to cover allyour charitable donations. Other taxes such as Council Tax or VAT do not apply. We willconfirm all gift aid details in your acknowledgement letter.

Yes, please treat this and any future donations as given under gift aid. Date: ................

About you:

First name: ............................ Surname: ..........................Title: Mr / Mrs / Ms / Miss / ..........

My address is: ........................................................................................................................

....................................................................................... Postcode: .......................................

Home telephone: ........................................ Mobile: ............................................................

Home email: ............................................... Work email: ......................................................

To claim gift aid we are required to have your full name and address including postcode.

Please return your completed form to CLDF, address below. Thank you.

Childrens Liver Disease Foundation, 36 Great Charles Street, Birmingham B3 3JY


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fighting childhoodliver disease

Scan with yoursmartphone tovisit CLDFswebsite

Scan with yoursmartphoneand visit CLDFsYoung People'swebsite

Join in the conversation

follow CLDF on Twitter and become a fan on Facebook

Childrens Liver Disease Foundation is the UKs leading organisation

dedicated to fighting all liver diseases of childhood.

It provides free of charge:

A huge selection of literature and online animations on the working of the liver available in

print and online

Information packs for a wide range of audiences, including young people, parents/carers,

GP practices, schools and nurseries, friends and relatives

Families and young peoples teams providing services in person, online, facebook, text

and phone

Developing services for adults diagnosed with a liver disease in childhood

Website childliverdisease.org

Young peoples website cldf-focus.org

National event programme for families and young people to meet, share and have fun

Secure online message board childliverdisease.org/forum

Around 75% of CLDFs annual income is derived from voluntary donations. Please help us

to continue to support young people, families and adults diagnosed in childhood by making

a donation. You can do this online or by completing the donation form in this leaflet. Even

better, a regular direct debit gift will enable us to plan our work more fully.

Thank you.

Childrens Liver Disease Foundation

36 Great Charles Street

Birmingham

B3 3JY

0121 212 3839 [email protected]

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