ACUTE PANCREATITIS without the forward

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ACUTE PANCREATITIS

This section of the booklet deals with the particular problem you have with your

pancreas - " acute pancreatitis " . So what is it, what causes it, and how can it

be

treated?

WHAT IS ACUTE PANCREATITIS?

This is an inflammation of the pancreas. It develops very suddenly and, in the

majority of

patients (about 75%) improves steadily with good hospital treatment over the

course of a

week or so. A blood sample usually shows the presence of a large amount of

amylase (a

pancreatic enzyme) in the blood. There are many causes of acute pancreatitis,

and it can

affect people of any age. Once a patient has recovered from an attack of acute

pancreatitis, they are perfectly well and there is usually no permanent damage

to the

pancreas.

It is very important to find out the cause of acute pancreatitis, which must

then be dealt

with otherwise further attacks may follow. If more than one attack of acute

pancreatitis occurs, it is called recurrent acute pancreatitis.

CAUSES OF ACUTE PANCREATITIS

SOME CAUSES OF ACUTE PANCREATITIS

Common causes

Gallstones

Sensitivity to alcohol

Unusual causes

Mumps

Hyperlipidaemia (too much fat in the blood)

Narrowing of the pancreatic duct

Pancreas divisum

Annular pancreas

Surgery

Trauma

Idiopathic

Hereditary

GALLSTONES

This is by far the commonest cause of acute pancreatitis in Europe. Because

gallstones affect women more commonly than men, acute pancreatitis usually

affects

women and even teenagers. Not all patients with known gallstones however develop

acute pancreatitis - the figure is about 1 in 15 only. Gallstones cause acute

pancreatitis because they pass into the bile duct and temporarily block the

opening into

the duodenum at the point where it is joined by the pancreatic duct (see the

diagram on

page 6).

In severe cases, patients may benefit from an emergency ERCP and cutting the

lower

end of the bile duct. This cutting is called 'endoscopic sphincterotomy' and

makes the

opening of the lower bile duct bigger which allows any stones to pass through

into the

duodenum without causing blockage.

Once symptoms have improved the best way to prevent further attacks of acute

pancreatitis is to have the gallbladder removed ('cholecystectomy'). This

operation is

now done best by keyhole surgery - so called laparoscopic cholecystectomy or

'lap

chole' - under a general anaesthetic.

Elderly patients may not be suitable for a general anaesthetic. An alternative

to a 'lapchole'

is to cut the lower end of the bile duct during ERCP. This cutting (endoscopic

sphincterotomy) causes complications in about 10% of cases and very occasionally

a

patient dies as a result. This risk is worth taking in an elderly patient who

cannot have

a 'lap-chole'. On the other hand the risk of endoscopic sphincterotomy

complications

may be too high in younger patients when a 'lap-chole' is safer.

ALCOHOL

This is the second commonest cause of acute pancreatitis in Europe overall

although in

some parts of Europe, it is a commoner cause than gallstones.

It is not known how alcohol causes acute pancreatitis. Some people have a

pancreas

which is sensitive to the effects of alcohol. These people develop attacks of

acute

pancreatitis a few hours or 1-2 days after they have been drinking alcohol.

Often the

sensitivity only develops after they have been drinking for several years. Such

people

may only be drinking a moderate amount of alcohol (not 'heavy' drinkers).

Other people who are much heavier drinkers may never develop acute pancreatitis

but

instead develop liver cirrhosis. Some patients who drink alcohol in moderate

amounts

never develop either acute pancreatitis or liver cirrhosis.

If alcohol is the cause of your acute pancreatitis, it is essential that you

stop all

future alcohol drinking. Non-alcoholic drinks mimicking wine or beer are now

reasonable substitutes. Low-alcoholic (LA) drinks should also be avoided

however.

MUMPS

Children may occasionally develop acute pancreatitis if they develop mumps which

is a

viral infection. Sometimes children will have a high amylase level in the blood

due to

the virus affecting the salivary glands in the neck. This might cause some

confusion at

first to the doctors looking after the child. A simple ultrasound scan of the

pancreas

helps to make the diagnosis or rule-out the possibility of pancreatitis.

Mumpsassociated

pancreatitis usually resolves and does not recur.

HYPERLIPIDAEMIA

This refers to an excessive level of lipids (particles of fat) in the blood.

Lipids are

essential to life and need to be transported by the blood from one tissue to

another.

Certain individuals have unusually high lipid levels in the blood and this can

cause

acute pancreatitis. Patients who drink a large amount of alcohol can also

develop

hyperlipidaemia. Not all lipids are the same and it is only a certain pattern of

lipids

which is associated with acute pancreatitis. For example, individuals who have a

high

blood cholesterol level are predisposed to heart disease and a high blood

pressure but

are not usually predisposed to developing acute pancreatitis.

Hyperlipidaemia is not a common cause of acute pancreatitis - it accounts for no

more

than 5% of cases. The diagnosis is made by measuring blood lipid levels at the

time of

an attack of acute pancreatitis. At other times, the blood lipid levels may be

normal.

Treatment involves adopting a low fat diet. Occasionally special drugs need to

be

taken which can lower the level of abnormal lipids in the blood.

NARROWING OF THE PANCREATIC DUCT

There are many different reasons why the pancreatic duct becomes narrowed. For

this reason, it is important not only to show that the pancreatic duct is narrow

but also

the cause for this. Surgery is often required to deal with pancreatic duct

narrowing.

PANCREAS DIVISUM

The pancreas develops as two separate buds from the intenstinal tube during

embryological development of the foetus in the womb. These buds each have a

separate pancreatic duct. The two buds eventually combine together before birth

to

form a solid single organ. When this occurs, the separate pancreatic ducts also

combine. In about 5-10% of healthy individuals, the pancreatic tissue combines

but the

two pancreatic ducts remain divided and they empty separately into the duodenum.

This situation is called pancreas divisum because the pancreatic ducts remain

divided.

Pancreas divisum is not harmful in the vast majority of cases. Very occasionally

one

of the ducts becomes narrowed and this can result in recurrent attacks of acute

pancreatitis. Sometimes this may eventually lead to chronic (or continuous)

pancreatitis.

The treatment involves enlarging the narrowed pancreatic duct opening and

sometimes

removing a part of the pancreas.

ANNULAR PANCREAS

This is an extremely rare cause of acute pancreatitis which often affects small

children but which can affect adults. The problem arises during embryological

development of the two pancreatic buds as described above (see pancreas

divisum).

In simple terms, the head of the pancreas becomes partly or totally wrapped

around

the duodenum. This can cause an obstruction to the flow of food in very young

babies. Alternatively the flow of pancreatic juice along the pancreatic duct may

be

hindered leading to acute pancreatitis. This may be difficult to recognise but

once it is,

surgery is required.

TRAUMA

Any major blunt trauma to the abdomen may cause acute pancreatitis.

SURGERY

This is also another rare cause of acute pancreatitis. Surgery performed to

organs

which lie near the pancreas such as the stomach or kidneys can cause acute

pancreatitis. For reasons we do not understand, surgery to organs well away from

the pancreas (such as the prostate gland, heart and brain) can also cause acute

pancreatitis.

IDIOPATHIC

This is a loosely applied term used by doctors to mean " the cause is specific to

an

individual person " - in other words the cause is not known for certain. Many

patients

initially diagnosed as 'idiopathic' turn out to have a known cause - such as

tiny

gallstones missed by routine investigations. There are lots of other possible

reasons

for an initial diagnosis of idiopathic pancreatitis such as certain types of

drugs, for

which there is no convincing evidence. Patients with idiopathic acute

pancreatitis

pose a problem because if the specific cause is not known, then no specific

treatment

can be given. In this case, it is important that the search for a cause should

be

thorough. Ultimately, the doctor has to guess as to the likely causes and advise

the

patient appropriately.

PREGNANCY

A small number of women develop acute pancreatitis during pregnancy. The cause

is

not the pregnancy itself but another underlying reason. Investigation nearly

always

shows that the cause is due to gallstones because in some women pregnancy

speeds-up the development of gallstones.

DOES ACUTE PANCREATITIS RUN IN FAMILIES?

In general, the answer is NO. Because acute pancreatitis is common and there are

many causes of this, simply by chance two or more members of a family may suffer

from acute pancreatitis. For example, it wold not be surprising if a mother and

her

adult daughter both developed gallstones and that both developed an attack of

acute

pancreatitis.

There are two main groups of patients in whom pancreatitis can be inherited.

This

happens because they have a gene which predisposes to pancreatitis. Each person

has exactly the same number of genes as every other person. There are 100,000

genes in every cell of the body but only 10,000 genes are selected for use in

any

particular cell. Genes are always in pairs, so that one set comes from the

mother and

one set comes from the father. There are tiny variations in each gene. These

tiny

variations are essential to make every person an individual. Occasionally a tiny

variation in a gene can give rise to a disease condition. Patients and their

families

require the care of a specialist surgeon, paediatrician or gastroenterologist

and

counselling from a geneticist.

Hereditary Pancreatitis

In this type there is a tiny variation in the cationic trypsinogen gene (which

makes

trypsin and is used to digest protein) results in an active trypsin in the

pancreas. This

activation occurs before it has had a chance to be secreted into the duodenum.

The

gene is officially called the PRSS1 gene and the two commonest alterations (or

gene

mutations) are called R122H and N29I. Affected individuals tend to develop

pancreatitis

as children, adolescents or young adults. There may be other members of the

family

with sugar diabetes. Not all members of the family will be affected in the same

way.

On average only half the individuals will carry the altered gene. This is called

a

dominant mutation. This means that half the children of an affected parent will

have the

gene passed on to them. Even then, some members of the family (about 20%) with

the

altered gene (or mutation) will not be affected at all. The presence of the gene

can be

tested for by a single blood test. Genetic counselling is required before any

tests can

be performed. Some families with Hereditary Pancreatitis have a normal set of

PRSSI

genes. This means that another gene is affected and scientists are trying to

find out

which one this is.

Idiopathic Pancreatitis

Some patients have an alteration in the gene that causes cystic fibrosis (the

CFTR

gene). One in 20 of the normal population has a CFTR gene mutation but only a

tiny

handful has idiopathic pancreatitis. Individuals with cystic fibrosis disease

have both of

the CFTR genes altered. We do not understand why some people with only one CFTR

gene mutation develop pancreatitis. Scientists are trying to find out why this

happens.

In these rare forms of pancreatitis, the symptoms begin as acute pancreatitis

and

progress to chronic pancreatitis. This also applies to a number of other causes

of

acute pancreatitis but gallstones never cause chronic pancreatitis.

SEVERE PANCREATITIS AND ITS COMPLICATIONS

We do not know why some patients develop severe pancreatitis. Once this occurs,

then the chances of complications and death are high. Because we still do not

understand all the factors responsible for causing the various complications,

treatment

is sometimes not successful and patients can unfortunately die. For these

reasons, it

is important that patients with severe pancreatitis are looked after by

specialists if this

is at all possible.

Severe pancreatitis places a stress on all the main organs of the body: the

heart,

lungs, kidneys, other gut organs, the brain and the peripheral vasculature (the

blood

vessels that nourish all the organs). Patients who are elderly are less capable

of

coping with these stresses. Equally, for reasons we do not understand, some

young

people also cannot cope with the stresses, and death will occur despite every

effort

on the part of those caring for them.

TREATMENT OF SEVERE PANCREATITIS

At first, patients will be transferred to a High Dependency Unit (HDU) for

intensive

monitoring and given intravenous fluids and oxygen by mask. If the cause is due

to

gallstones, an ERCP and endoscopic sphincterotomy may be performed (see pages 12

and 17). Most patients recover from acute pancreatitis but some require to be

transferred to an Intensive Therapy Unit (ITU). This is necessary for assisted

ventilation of the lungs and to treat kidney failure by dialysis.

One or more CT or MRI scans may be necessary to assess the state of the

pancreas.

There may be severe death of the tissue (necrosis) of the pancreas or tissues

surrounding the pancreas. If the necrosis is extensive or if there is evidence

of

infection of the necrosis, then surgery will be needed. The timing of the

surgery and

the extent of surgery are extremely difficult decisions to make even for

experienced

pancreatic surgeons. Once it is decided to operate for severe necrosis, the

likelihood

of success is anywhere between 50% and 80%, but this depends very much on

individual cases.

The procedure used for removing extensive pancreatic dead tissue (necrosis) is

called

a 'necrosectomy'. The procedures that are used include the following:

Open Necrosectomy: This requires a large operation to remove dead pancreatic

tissue. For several weeks (or longer) tubes are left behind to wash out and

drain

small pieces of dead tissue. More than one operation may be necessary.

Minimally Invasive Necrosectomy: It may be possible to remove dead pancreatic

tissue using " keyhole " surgery. The advantage is that the success rate may be

better

in very ill and elderly patients compared to open necrosectomy.

Failure to survive a severe attack of acute pancreatitis despite all the

treatment on ITU

and surgery is due to the inability of the different organs to cope with the

stress of

acute pancreatitis. In young people, even though the heart and lungs respond to

drugs

and ventilation and the kidney function is replaced by dialysis, the peripheral

vasculature becomes unresponsive to drugs. The blood pressure then falls and it

becomes impossible to keep the patient alive.

On the whole, most patients tend to survive an attack of severe acute

pancreatitis

although this may take several months of treatment in hospital. Despite an

overall

improvement at a later stage, localised complications may develop, which are

principally a pseudocyst or an abscess (see below).

Pseudocyst: (Pronounced 'Sue-doe-cyst') This is a cystic swelling which lies in

the

pancreas or next to the pancreas and which contains high concentrations of

pancreatic enzymes. Often pseudocysts disappear without any specific treatment.

If

a pseudocyst remains or enlarges, it may cause nausea, vomiting, pain and weight

loss, in which case, treatment is necessary. There are different ways to treat

large

pseudocysts but usually the most appropriate method is by surgery. Sometimes it

is

possible to insert a tube into the pseudocyst under local anaesthetic in the

X-ray

department and drain the fluid away without surgery. Another treatment is by

endoscopy.

Pancreatic Abscess: A collection of pus may develop in or near the pancreas

during or after an attack of severe pancreatitis. The treatment is usually

successful and may require surgery or just drainage with a tube inserted

under local anaesthetic in the X-ray department.

WHAT CAN I EAT?

For a few weeks after an attack of acute pancreatitis you should eat at

regular intervals. It is usually better to take four or five snacks a day than a

full meal. If you have gallstones, and for some reason your gall bladder has

not been removed, avoid fatty foods such as butter, eggs, fried foods,

sausages and bacon. Following removal of your gall bladder you are free to

eat anything you wish. You will have a very healthy appetite and you may

put on more weight than you would otherwise, unless care is taken to avoid

excess calories.

LIVING WITHOUT A PANCREAS

There are some patients who have had either their pancreas removed or

who still have pancreatic tissue but which is not functioning at all. Both

types of patient are perfectly able to lead a normal life provided they take

regular enzyme supplements and insulin injections.

PANCREATIC ENZYME SUPPLEMENTS

There are many preparations available. These preparations differ

considerably in their effectiveness of action. The better preparations

consist of capsules containing scores of small granules. The enzyme

preparations can also be divided into two types depending upon their

strength of action: regular and high dose. The capsules need to be taken

during each meal and with any snack. Requirements vary enormously from

patient to patient: typically 20-30 high-dose capsules per day are required

but this can be lower or much higher. The requirements vary greatly from

patient to patient partly because of the different level of secretion by any

functioning pancreas and partly because there are still some enzymes

secreted by the salivary glands, tongue, stomach and small intestines but

which also varies greatly from person-to-person.

In a few cases of children and adults with cystic fibrosis, a serious problem

with the large bowel (colon) has been reported. This condition is called

fibrosing colonopathy and causes narrowing of the bowel. It seems to be

related to the use of a particular acid-resistant coating of the enzyme

preparations (called methacrylic copolymer). The problem does not arise

with preparations without this covering. The latter preparations are

therefore recommended. The ingredients are always listed on the pack

leaflet or label. Once patients are accustomed to taking enzyme

supplements, they are usually allowed to adjust the number they take

themselves to suit their own individual needs

INSULIN

There are many types of insulin available including human insulin obtained

by genetic engineering. Precise dosing and frequency of injections is an

individual matter. Being under the care of a diabetic specialist is obviously

important in the first instance.

GASTRIC ACID SUPPRESSING TABLETS

Medication of this sort is often prescribed to be taken once or twice a day.

Pancreatic juice normally counters the acid of the stomach. In the absence

of the pancreas, there may be excess acid which can cause dyspepsia.

There is also some evidence that taking this type of medication helps the

action of pancreatic enzyme supplements which means that fewer

capsules are required each day.

LIVING WITHOUT A SPLEEN

Pancreatic surgery sometimes necessitates removal of the spleen. This is

much more of a problem in children than in adults. Without the spleen

there is a small but real risk of developing a serious infection caused by

certain bacteria especially pneumococcus. All children and adults without a

spleen therefore require regular pneumococcal vaccination. All patients

should also receive vaccination for meningococcus groups A and B, and

children less than 4 years old require Haemophilus influenzae type b

vaccination. Children may also need to take a daily antibiotic. Since the risk

is much less in adults, daily antibiotics are not prescribed usually.

Nevertheless if any infection develops, then appropriate antibiotics (such as

penicillin or erythromycin) must be taken over-and-above any other types of

antibiotic that are required.

Removal of the spleen sometimes causes the number of platelets in the

blood to increase. This increases the risk of developing unwanted blood

clots. Regular blood tests are therefore needed. If the number of platelets

in the blood rises excessively, it is common practice to prescribe low-dose

aspirin which reduces the risk of undesirable clotting.

CAN I DRINK ALCOHOL?

Alcohol is not recommended for patients who have recurrent acute pancreatitis

and should be avoided in patients for whom the cause of their acute

pancreatitis is alcohol.

If alcohol is not the cause of your acute pancreatitis you may drink alcohol if

you wish. Indeed certain drinks such as English beer or stout are a good

source of calories, iron and vitamins.

The recommended intake for healthy adults should be no more than 14

units per week for women and no more than 21 units per week for men. A

unit of alcohol is equivalent to a half-pint of regular beer or lager, a small

glass of table wine, or a single measure of spirit.

DOCTORS DEALING WITH PANCREATIC DISEASE THAT YOU MAY MEET

All surgeons are called 'Mr' and other medically qualified doctors are called

'Dr'. Either may be called 'Professor' if they work for a University. Senior

doctors are called 'consultants' and the junior doctors are called house

officers, senior house officer, registrar and senior registrar. In University

departments, they are also called 'lecturer' (registrar or senior registrar)

and 'senior lecturer' or 'reader' (consultants).

General physician:

A consultant medical doctor who works in a hospital and who is broadly

specialised including 'gut' problems.

General surgeon:

A consultant surgeon who works in a hospital and who is broadly

specialised including 'gut' problems.

Gastroenterologist:

A physician who is highly specialised in 'gut' problems and is also usually

an 'endoscopist'.

Endocrinologist:

A physician who is highly specialised in glandular problems including sugar

diabetes.

Specialist surgeon:

A general surgeon who is highly specialised - a so called PB-specialist is a

pancreato-biliary surgeon.

Endoscopist:

This may be a gastroenterologist or a surgeon who is able to undertake

endoscopy (examination of the stomach or bowel using a flexible

telescope). A few endoscopists can also perform ERCP, a specialist form of

endoscopy that examines the bile ducts and pancreatic ducts.

Radiologist:

A consultant who only specialises in taking X-rays and scans of various

sorts at the request of other specialists. A few radiologists are also expert

endoscopists.

Paediatrician:

A consultant who specialises in the care of children and who may be called

to investigate a pancreatic problem in young children or teenagers.

Dietician:

This is a specialist who is not a doctor but is an expert in advising on

various types of diet.

USEFUL ORGANISATIONS

Pancreatitis Supporters Network

This is a support group which has members throughout the UK. The Network

provides

information and support to patients with pancreatitis and their relatives. This

is a

registered charity. For further information, write to

Mr Jim Armour

Chairman, Pancreatitis Supporters Network

PO Box 8938

Birmingham

B13 9FW

Tel. 0

Pancreatic Society of Great Britain and Ireland

This is a professional organisation of specialist doctors involved in the care

of patients with pancreatic disease. The Society is allied to the European

Pancreatic Club and the International Association of Pancreatology. For

further information, write to: Mr R Charnley, Secretary, Pancreatic Society of

Great Britain and Ireland, Department of Surgery, Freeman Hospital,

Newcastle-upon-Tyne, NE7 7DN.

Pancreas Research Fund

Specifically supports clinical and basic research of diseases of the

pancreas. Write to: Pancreas Research Fund, Department of Surgery, Royal

Liverpool University Hospital, Daulby Street, Liverpool, L69 3QA.

Digestive Disorders Foundation

3 St 's Place

London

NW1 4LB

Tel : 0

EUROPAC

European Register for Familial Pancreas Cancer and Hereditary Pancreatitis.

The principal register in Europe providing advice and research in inherited

pancreatic disorders. Write to: EUROPAC Co-ordinator, Department of

Clinical Services, Alder Hey Children's Hospital, Eaton Road, Liverpool, L12

2AP.

europac@...

http:/www.liv.ac.uk/surgery/europac.html.