WHAT IS CHRONIC PANCREATITIS AND WHAT CAUSES IT

February 28, 2004

WHAT IS CHRONIC PANCREATITIS AND WHAT CAUSES IT?

This refers to an inflammation of the pancreas which is continuous. The

inflammation is usually of a low-grade

so that there is no fever but often there is some pain. Because of the

continuous inflammation, scar tissue

develops within the pancreas. At first this may result in loss of part of the

enzyme-making part of the pancreas.

After a variable period of time (which could be after some weeks or months but

is usually after many years), the

insulin-making part of the pancreas may become destroyed. For reasons that are

not understood, many patients

with chronic pancreatitis develop calcium deposits in the pancreas tissue and

may form calcium stones in the

pancreatic ducts. Blockage of the ducts by scar tissue or stones will stop

enzymes being delivered to the gut and

impair digestion. The pancreatic duct may enlarge if it is blocked.

The cause of chronic pancreatitis is usually due to alcohol drinking but there

are other causes such as narrowing of

the pancreatic duct and pancreas divisum (see below). In many cases, the cause

remains unknown. If alcohol is

thought to be the cause, it is essential that all alcohol drinking is stopped.

The main symptoms of chronic pancreatitis are:

.. poor digestion

.. sugar diabetes

.. pain

.. weight loss

There are many causes for abdominal pain so that it is essential to establish a

diagnosis of chronic pancreatitis by

investigation. Most patients can be treated by medical treatment only, but a few

will require surgery.

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 intestinal 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 sometimes this may eventually lead to chronic pancreatitis.

The treatment involves enlarging the narrowed pancreatic duct opening and

sometimes removing a part of the

pancreas.

OTHER CAUSES OF CHRONIC PANCREATITIS

Sometimes the cause is not known for certain. Doctors use the loosely applied

term idiopathic to mean " the

cause is specific to an individual person. "

Another factor which may predispose to chronic pancreatitis in later life is a

condition called annular

pancreas. 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 become 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 an attack

of pancreatitis. This may be

difficult to recognise but once it is, surgery is required.

DOES CHRONIC PANCREATITIS RUN IN FAMILIES?

In general, the answer is NO. There is, however, a very rare form of chronic

pancreatitis, which can run in

families. The features of this are no different from other types of chronic

pancreatitis except it does tend to

afflict young members of a family. Treatment of this condition is identical to

the treatment of other types of

chronic 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 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.

MEDICAL TREATMENT OF CHRONIC PANCREATITIS

.. Stop all alcohol drinking if this is the cause.

If a patient works in an alcohol related industry such as a brewery or bar or

pub, a change of employment is

recommended. A change of lifestyle is often very helpful. Attendance at a drug

addiction unit also can be very

helpful.

.. Ideally smokers need to stop smoking altogether (see page * " Does Chronic

Pancreatitis Cause Cancer? "

.. Pancreatic enzyme supplements (see page *). These help digestion and may

reduce the pain.

.. If sugar diabetes is present, then insulin treatment will be required (see

page *).

.. Mild pain-relieving tablets are acceptable.

It is sometimes suggested that the nerves of the pancreas which are responsible

for taking the sensation of pain to

the brain should be destroyed. This usually involves an injection into the back

to destroy the nerves around the

pancreas. Most pancreatic surgeons do not recommend this as any effect is

short-lived and can make any further

surgery very difficult.

SURGICAL TREATMENT OF CHRONIC PANCREATITIS

This is necessary if there are surgical complications of chronic pancreatitis or

if the pain becomes severe.

Once it is necessary to be taking strong pain killers on a regular basis, then

surgery will be required. If a patient is

already receiving regular pethidine or morphine, attendance at a drug addiction

unit may also be necessary

following surgery.

The chances of achieving a good result following surgery for pain are at least

80% in the first instance. There is,

however, no guarantee of success and some patients may develop a recurrence of

pain sometime after surgery.

This may necessitate further surgery. For these reasons, it is essential that

the patient and relatives and friends

remain committed to addressing all the social problems as well as the medical

problems involved. Patience and

optimism are required by all.

The underlying disease process will largely dictate the choice of operation.

Some operations can be relatively

simple - for example removal of a single pancreatic stone, enlarging a narrowing

of the pancreatic duct or

performing an internal drainage operation for a dilated pancreatic duct. In

principle, however, a resection of part

of the pancreas (partial pancreatectomy) will be required if there has been

severe pain.

Operations have become more 'conservative' in recent years. This means that only

the affected pancreas tissue

is removed and that other nearby organs such as the duodenum, stomach and spleen

are left untouched. This is

so-called " designer-pancreatic surgery " .

This type of surgery is particularly demanding and requires a specialist

pancreatic surgeon to undertake the

procedure. Although it is nearly always intended to preserve the spleen, this

may prove excessively difficult at

operation when it becomes necessary for reasons of immediate safety to the

patient to remove the spleen

(splenectomy).

In addition to relieving pain, the principal aims of designer-pancreatic surgery

are to improve drainage of the

pancreas, reduce the risk of developing sugar diabetes (diabetes mellitus), and

maintain important normal

anatomy.

Common operations include the following:

Beger's operation: The head of the pancreas is removed preserving the duodenum.

The base of the removed

pancreatic tissue (which contains the bile duct and pancreatic duct) is drained

into the small bowel by a special

procedure (Roux-en-Y).

Peustow Procedure: In this operation no tissue is removed but the dilated main

pancreatic duct is drained into

the small bowel by a Roux-en-Y procedure. This operation is used if the pancreas

is not badly affected apart from

obstruction to the pancreatic duct.

Frey's procedure: This is almost identical to the Peustow operation but some

tissue is removed from the head

of the pancreas although less than in Beger's operation.

Other operations include the following:

Kausch-Whipple's Operation: Some surgeons offer this operation instead of a

Beger's operation or if there is

concern about the presence of a small cancer. Part of the stomach is removed

along with the pylorus, the

duodenum and the head of the pancreas.

Pylorus-Preserving Kausch-Whipple's Operation: In this procedure the stomach and

pylorus are preserved

whilst removing the duodenum and head of the pancreas.

Bilateral Thoracoscopic Sympathectomy (BITS): In some cases in which surgery has

failed to control pain

even though all the pancreas has been removed it may be helpful to undergo the

BITS procedure. This involves

cutting the pain nerves from the pancreas as they travel through the chest. The

operation is done using fine

instruments and telescopes. This is so called 'keyhole' surgery. The operation

lasts only 30 minutes and may be

performed as an outpatient.

Left Pancreatectomy: In this operation the left part of the pancreas is removed.

This operation is performed if

the head and neck of the pancreas are completely normal. The spleen is normally

preserved, but may need to be

removed sometimes.

Conservative Total Pancreatectomy: In this operation 95% of the pancreas is

removed. A small amount of

pancreas tissue is preserved near the duodenum to maintain its blood supply. The

spleen is also preserved if

possible. This operation is performed if the whole of the pancreas is badly

affected. Designer total

pancreatectomy may be necessary especially if:

.. there has been previous pancreatic surgery

or

.. the patient already has sugar diabetes

Other conditions that may complicate chronic pancreatitis are pseudocysts and

ascites:

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. It

is often better in chronic

pancreatitis however to remove the cyst rather than simply drain it.

Ascites: (Pronounced 'ass-eye-teas'). This refers to a discharge of pancreatic

juice into the general cavity of the

abdomen. Ascites may occur if the pancreatic duct or a pseudocyst ruptures and

leaks at a certain point.

Treatment may involve surgery and/or the use of a special drug called octreotide

which suppresses the secretion

of pancreatic juice.

DOES CHRONIC PANCREATITIS LEAD TO CANCER?

Unfortunately it does appear that some patients with chronic pancreatitis are

more likely to develop pancreatic

cancer, as they become older.

The risk is greatest amongst smokers and those with Hereditary Pancreatitis.

These risk factors are

'independent' and therefore all patients are recommended not to smoke. The risk

appears to increase with age

and with the duration of symptoms. The risk is lowest in those under 30 years

old and highest in those over 70

years of age.

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.

Modern preparations consist of capsules containing scores of small

acid-resistant granules. The enzyme

preparations can also be divided into two types depending upon their strength of

action: regular and high strength.

The capsules need to be taken during each meal and with any snack. Requirements

vary enormously 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 intestine but which also vary

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 (called methacrylic acid

copolymer) in some enzyme preparations.

The problem does not arise with preparations without this coating. 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

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WHAT IS CHRONIC PANCREATITIS AND WHAT CAUSES IT

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