Thursday, February 15, 2007

Update on Jon, this is what they think he has

I got a call from Jonathan last night and he has been diagnosed pretty much by the head GI doc with Chronic Pancreatitis. He still is going to have another test to verify, but the symptoms and blood enzyme levels seem to indicate this is an accurate diagnosis. Here is some information on the disorder:

Chronic pancreatitis

What is the pancreas?
The pancreas is a soft, elongated gland situated at the back of the upper abdominal cavity behind the stomach.
It is divided into the head (through which the common bile duct runs as it enters the duodenum) and the body (which extends across the spine and the tail), which is close to the left kidney and to the spleen. Because the pancreas lies at the back of the abdominal cavity, diseases of the pancreas may be difficult to diagnose.

What does the pancreas do?

The pancreas has two main functions: it produces a series of enzymes which help in the digestion of food. Enzymes produced in the pancreas are important in the digestion of proteins, carbohydrates and, particularly, fats. Bicarbonate is also produced in large amounts to neutralise the acid produced by the stomach. it produces a series of hormones which are important in maintaining a normal level of sugar in the blood. The best known of these hormones is insulin. Insulin deficiency of this hormone results in the development of diabetes. Another hormone (glucagon) helps to raise blood sugar, and several other hormones control intestinal function.

What is pancreatitis?
Any inflammation of the pancreas is called pancreatitis.
Acute pancreatitis results in severe inflammation of the gland and patients may be seriously unwell. Chronic pancreatitis develops either as the result of repeated attacks of acute pancreatitis or as the result of other injuries to the pancreas (see below). It is thought that the damage to the pancreas occurs as the result of digestive enzymes leaking into the pancreas and starting to digest it. This sets up inflammation, and when the inflammation settles, the scarring process distorts the pancreas making further attacks of inflammation likely. Thus a vicious cycle develops.
As a result of prolonged damage to the pancreas, the pancreas fails to produce enough digestive enzymes to permit adequate digestion of food. This leads to weight loss and the frequent passage of pale greasy stools which contain excess amounts of fat. Further, the destruction of the cells which produce insulin may lead to the development of diabetes.

What causes chronic pancreatitis?
The most common cause of chronic pancreatitis is long-term excessive alcohol consumption. (Jon does not drink) There is a direct relationship between the amount of alcohol consumed and the risk of developing chronic pancreatitis.
Other causes include:
high levels of calcium in the blood
abnormalities in anatomy which are usually present at birth
cystic fibrosis (already ruled out in Jon's case)
high blood fats (hypertriglyceridaemia)
in rare cases, some drugs can cause pancreatitis
in a number of cases no specific cause can be identified, a condition known as idiopathic pancreatitis.

What are the symptoms of chronic pancreatitis?
The symptoms are very variable.
Pain occurs in most patients at some stage of the disease. This may vary in intensity from mild to severe. It may last for hours or sometimes days at a time and may require strong painkillers to control it.
It often radiates through to the back and can sometimes be relieved by crouching forward. It is commonly brought on by food consumption and so patients may be afraid to eat. It is also commonly severe through the night.
The pain varies in nature, being gnawing, stabbing, aching or burning, but it tends to be constant and not to come and go in waves. It may sometimes burn itself out but can remain an ongoing problem.
The mechanism of the pain is unclear. It seems to be related to pancreatic activity since it is frequently caused by food, especially fatty or rich foods.
Some patients will have obstruction to the small ducts in the pancreas by small stones, and this is thought to cause back pressure and destruction of the pancreas. There is no relationship between the severity of the pain and the severity of the pancreatic inflammation.

The pain is often difficult to diagnose and can be mistaken for pain caused by virtually any other condition arising from the abdomen or lower chest.

It can be difficult to distinguish pain caused by pancreatitis from pain caused by a peptic ulcer, irritable bowel syndrome, angina pectoris, gallstones.

Diabetes is also a common symptom which affects over half of all patients with long-standing chronic pancreatitis.

Long-standing chronic inflammation results in scarring of the pancreas which destroys the specialised areas of the pancreas which produce insulin.

Deficiency of insulin results in diabetes. Diabetes causes thirst, frequent urination and weight loss. It may be possible in the early stages of chronic pancreatitis to treat the diabetes with tablets, but in the late stage of chronic pancreatitis, insulin injections are usually needed.

Diarrhoea occurs in just under half of patients. Normally, all the fat in food is broken down by enzymes from the pancreas and small intestine, and the fat is then absorbed in the small bowel. With a reduced level of digestive enzymes the fat is not absorbed. When the fat reaches the large intestine, it is partially broken down by the bacteria in the colon. This produces substances which irritate the colon and result in diarrhoea. The undigested fat also traps water in the faeces, resulting in pale, bulky, greasy stools which are difficult to flush away. They may make the water in the toilet look oily, smell offensive and may be associated with bad wind.

Weight loss occurs in virtually all patients with chronic pancreatitis. It is due to failure to absorb calories from food, and diabetes may also contribute to this. In addition, patients may be afraid to eat because eating brings on the pain. Depression is also common in chronic pancreatitis and this can also reduce appetite and lead to weight loss.

Jaundice (when patients develop yellow eyes and skin) occurs in about a third of patients with chronic pancreatitis. It is usually due to damage to the common bile duct which drains bile from the liver to the duodenum.

The common bile duct normally passes though the head of the pancreas. In long-standing chronic pancreatitis, the scarring in the head of the pancreas narrows the common bile duct.

Some degree of narrowing may occur in up to half the patients with chronic pancreatitis but when the narrowing is severe, it prevents the bile draining from the liver into the duodenum. It then spills back into the blood and the patient's eyes and skin become yellow. In addition, the stools become paler (since bile makes the stools brown) and the urine becomes dark (because it contains more bile than normal).

Vomiting after meals is a less common symptom but can occur as a result of severe pain. It may also be due to duodenal ulceration, which is often connected with chronic pancreatitis. In rare cases, the duodenum may be narrowed as a result of scarring secondary to chronic pancreatitis.

Vitamin and mineral deficiency. Prolonged passage of stools containing fat can result in low levels of calcium and magnesium in the blood. In addition, some vitamins may not be absorbed properly. This includes vitamins D and A.

Is chronic pancreatitis dangerous?
The major problem with chronic pancreatitis is pain control. This may require the use of morphine-like drugs (pethidine, morphine (eg MST continus) and diamorphine). There is always the risk of addiction to these drugs, particularly if their use is not controlled.

Chronic pancreatitis is associated with a reduction in life expectancy. Only half of the patients with a diagnosis of chronic pancreatitis will survive for longer than seven years following diagnosis. There is also an increased rate of cancer of the pancreas in patients with chronic pancreatitis and this accounts for a fifth of the deaths. Other causes of death include complications of diabetes and complications of alcoholism.

How is chronic pancreatitis treated?

There is no cure for chronic pancreatitis. Once the pancreas is damaged, then it is not able to return to normal function and there is always the potential for further attacks. Treatment is, therefore, directed towards preventing attacks, controlling the pain and treating the complications.

Preventing symptoms worsening
Patients with chronic pancreatitis should avoid alcohol altogether. If the pancreatitis is due to excess alcohol consumption, then this is essential. If it is due to other causes, then it seems sensible to avoid a substance which is capable of damaging the pancreas.

If an underlying cause has been identified then this should be treated. Disorders of calcium metabolism and of fat metabolism will be treated appropriately. Your doctor may recommend removal of the gall bladder if pancreatitis is thought to be caused by gall stones.

Preventing attacks

The long-standing principle has been to try and rest the pancreas. This involves giving pancreatic supplements such as Creon (which contain pancreatic enzymes in high concentration) together with drugs which reduce acid secretion by the stomach. Patients should also follow a low-fat diet.

These measures reduce the presence of fat in the duodenum, reduce acid in the duodenum and reduce the need for pancreatic enzyme secretion. These measures are very successful in about a third of patients, moderately successful in a third and unhelpful in a third.

Some eminent specialists have supported the use of antioxidants in the treatment of chronic pancreatitis. These antioxidants include selenium and vitamin C. You should take specialist advice (via your GP) before taking them.

Control of pain
This is a very important aspect of the treatment of chronic pancreatitis. Pancreatic pain varies in severity from mild (controllable with simple analgesics such as paracetamol (eg Panadol)) to severe (requiring morphine-like drugs for control).

In addition to the preventive measures listed above, the basic principle is to use the drug lowest down the analgesic ladder which controls the pain. Since the pain is often worse at night and since both body and mind are at their lowest ebb in the early hours of the morning, the lowest rung of the analgesic ladder may be pethidine or morphine (eg MST continus tablets). Since the pain is chronic and severe, there is a fine line between adequate analgesia and addiction.

Pain management often needs specialist help either from the specialist gastroenterologist or from the local pain clinic. Your GP will help with appropriate referral, although in most cases the diagnosis of chronic pancreatitis will have been made by a hospital specialist, who will probably supervise your ongoing care.

Other medications may also help. Antidepressants may reduce the requirement for painkillers and may enable a patient to descend the 'analgesic ladder'. Other measures include the injection of local anaesthetic or other substance into the nerve supply from the pancreas.

Treatment of the complications
Malabsorption is treated by administering pancreatic supplements in capsule or powder form.

Diabetes is treated either by tablets or, more commonly, insulin.

Jaundice is treated by ERCP and stent insertion across the stricture in the common bile duct where it passes though the head of the pancreas.

Surgery for chronic pancreatitis
In rare cases, it may be necessary to consider surgery as a treatment of chronic pancreatitis. The indication for surgery is usually severe pain unresponsive to standard measures or a high level of morphine or similar drug usage in a young person.

The surgery may involve measures to improve drainage of the pancreatic duct, partial or complete removal of the pancreas. The major problem with removal of all or part of the pancreas is that it could lead to the development of diabetes in those patients who don't already have it.

1 comment:

Liz said...

Oh Vicki, I am SO sorry to hear that about Jon, but at least you now know what it is he'll be fighting.

Please send good wishes from Kirk & I.