Cystic Fibrosis part-2 Return to page-1

Gut symptoms
The pancreas normally makes digestive juices which contain chemicals (enzymes). The digestive juices normally flow out from the pancreatic duct into the duodenum and digest food.

         

In people with cystic fibrosis, thickened secretions block the normal flow of the digestive juices from the pancreas. This can result in food not being digested or absorbed properly, in particular, fatty foods and fat-soluble vitamins (vitamins A, D, E and K). This can cause:

• Malnutrition leading to poor growth and poor weight gain (even if you have a good appetite and eat a lot, as the problem is with digesting and absorbing the food).
• Large, smelly, greasy, fatty faeces (stools or motions) occur in about a third of cases.
• Bloated abdomen.

In about 3 in 20 cases the pancreas functions well and there are no or minimal gut symptoms, and mainly just lung symptoms.

Symptoms sometimes occur at birth
About 1 in 10 children with cystic fibrosis are diagnosed shortly after birth. This is due to a condition called meconium ileus where in some cases the gut becomes blocked with meconium - a thick, dark, sticky substance which is made by the baby's gut before being born. Urgent surgery may be needed to relieve the blockage.

Other symptoms and complications
Other organs may be affected which may cause various other problems in some cases. Also, the pancreas and airways may become severely affected. Therefore, other problems which may also occur in some cases include:

• Repeated sinus infections.
• Polyps forming in the nose.
• Infertility (especially in males as the tubes which carry the sperm can become blocked).
• Damage to the liver which may lead to cirrhosis occurs in about 1 in 12 cases (if the small ducts in the liver become blocked or damaged).
• Diabetes. (Special cells in the pancreas make insulin. If the pancreas becomes severely damaged over time then insulin levels go down and diabetes may develop.) This is rare in children but is more common in adults who have had cystic fibrosis for years.
• Pancreatitis (inflammation of the pancreas).
• Rectal prolapse.
• Constipation.
• Osteoporosis (thinning of the bones) may develop due to poor absorption of food, and in particular, poor absorption of vitamin D which is needed to maintain healthy bones.
• The sweat tastes very salty.

General
Ongoing poor nutrition combined with persistent lung symptoms and repeated chest infections often causes 'failure to thrive' in young children, delayed maturation and sexual development in teenagers, and general poor health at any age.

Mild cases
Recently, some cases of cystic fibrosis have been diagnosed in adults who have relatively mild symptoms. This may be due to some mutations of the cystic fibrosis gene not being as 'faulty' as others. The handling of sodium and chloride may only be mildly affected in these cases.

How is cystic fibrosis diagnosed?

Sweat test
A doctor may arrange a sweat test if he or she suspects cystic fibrosis from the symptoms. This test measures the amount of salt (sodium and chloride) in skin sweat. People with cystic fibrosis have an abnormally high salt level in sweat.

Genetic test
A genetic test can confirm the diagnosis. Some cells are scrapped from the inside of the cheek. These can be tested to detect the cystic fibrosis gene.

Screening test
Some countries screen all newborn babies for cystic fibrosis. A small 'heel prick' blood test is taken about the sixth day after birth. This can detect a chemical called immunoreactive trypsin which is high in babies with cystic fibrosis. If it is high then a sweat test and genetic test can be done to confirm the diagnosis.

There are very good arguments to screen all babies as the earlier the diagnosis is made, the sooner treatment can begin which improves the outlook (prognosis). However, only a few places in the UK routinely screen newborn babies for cystic fibrosis. This may change in the future and screening may be introduced to include all areas in the UK.

What is the treatment for cystic fibrosis?

There are many aspects to the treatment of people with cystic fibrosis. Treatment involves the input, advice, and expertise of various professionals such as child health doctors, specialist nurses, physiotherapists, dieticians, etc. It is usual to have regular checks and tests to monitor the condition and to keep a check on children's growth, development and well-being.

The following list is a brief overview of the commonly used treatments, but is not a full or exhaustive account of all the treatments used. An individual treatment plan is needed for each case to take into account individual circumstances.

All patients need to  on  celiac diet.

All patients should use a electronic zapper, or Tens unit everyday.

Treatments for lung problems

Physiotherapy and exercise
Regular chest physiotherapy is very important. This helps to clear the airways of the thick mucus. A physiotherapist usually shows parents how to do this for their children. It involves a special way to firmly pat the chest whilst the child lies head-down to encourage mucus and sputum to be coughed out. Twice daily chest physiotherapy is common practice. This may need to be increased during times of chest infections. It is also important to encourage children to exercise and to be as active and fit as possible. So, sports and games are encouraged.

Antibiotics and antifungals
Courses of antibiotics are a mainstay of treatment specially doxycycline. Many children with cystic fibrosis take regular long-term antibiotics. The dose is increased and/or other types of antibiotics are given when a chest infection develops. Various bacteria can cause infections and the antibiotics chosen depend on which bacteria are found in samples of sputum. Antibiotics given intravenously (into a vein) are often required for severe infections that are not controlled with antibiotic tablets.

A bacterium called pseudomonas aeruginosa commonly persists in the thick mucus in the airways. To keep this from flaring up into repeated infections, an antibiotic given by nebuliser (inhaled antibiotic) is a common treatment.

Please continue to next page

Further help and information

  Return back to first page of Cystic Fibrosis

   Internet help Available  see our services & contact information