WHAT IS CYSTIC FIBROSIS?

Cystic Fibrosis is a genetic disease of the body's mucus glands.  It affects the respiratory and digestive systems.  A bad gene causes the body to produce abnormally thick, sticky mucus that clogs the lungs.  On the average, people with Cystic Fibrosis live until the age of 30 years.

WHO DISCOVERED CYSTIC FIBROSIS?

Dr Dorothy Anderson, a pathologist of Columbia University in New York, identified Cystic Fibrosis in 1938.  She noted the symptoms of Cystic Fibrosis and the changes of the organs that were affected.  She named the disease, calling it "Cystic Fibrosis of the Pancreas" from cysts and scar tissue that grew in the pancreas.  The gene responsible for causing Cystic Fibrosis was discovered in 1989.

 

LUNGS WITH MUCUS

WHAT ARE THE SIGNS AND SYMPTOMS OF CYSTIC FIBROSIS?

People with Cystic Fibrosis have a variety of symptoms.  They sweat, losing excessive amounts of salt that cause abnormal heart rhythms.

The mucus in Cystic Fibrosis patients is very thick in the intestines and lungs.  This causes poor growth, respiratory infections, breathing difficulties and eventually permanent lung damage.  Lung disease is the usual cause of death in most patients.

 
Cystic Fibrosis can also cause :

sinusitis
nasal polyps
clubbing (rounding of the fingers and toes)
enlargement of the right side of the heart and abdominal pain
liver disease
diabetes
inflammation of the pancreas

 
HOW IS CYSTIC FIBROSIS DIAGNOSED?

The most common test for Cystic Fibrosis is called the sweat test.  It measures the amount of salt (sodium chloride) in the sweat.  Sweat is usually taken from the forearm in a simple procedure and analysed.  A high level indicates Cystic Fibrosis.

A chemical test for a mutated gene can be used for people who have normal sweat chloride levels.

Some other tests that can assist in the diagnosis of Cystic Fibrosis are chest e-rays. lung function tests, sputum (phlegm) cultures and stool examinations.

 
HOW IS CYSTIC FIBROSIS TREATED?

Patients need daily chest therapy.  Chest infections need to be treted quickly with antibiotics.  They should also be vaccinated against flu and pneumococcus to help prevent chest infections.

Patients need to take capsules with each meal or snack.  This supplies the missing pancreatic enzymes and helps with digestion.

These are other treatments, according to each person's condition :

  • daily inhaled anti-asthma therapy
  • corticosteroid tablets
  • vitamin supplements
  • inhalation of Pulmozyme to make the sputum less sticky
  • oxygen to help with breathing
Postural drainage is a technique for 
loosening mucus in the airway so 
that it may be coughed out

 

GENE THERAPY - THE FUTURE OF CYSTIC FIBROSIS TREATMENT

There is no current cure for Cystic Fibrosis.  There is a lot of research going on by scientists to try and find a cure for Cystic Fibrosis lung disease through gene therapy.

Dr. Jonathan Orens at a medical conference in Orlando, in May this year, said that lung transplantation can improve survival and quality of life for many Cystic Fibrosis patients.

 
SIXTY FIVE ROSES 
by The Wolverines

When I was just a small child, my mum and daddy came to me,
They sat me down and told me of the flowers my sister received.
Sixty-five roses with yellow and red,
Made her so tired she had to stay in bed.
I just couldn't believe the flowers my sister received,
Made it so hard for her to breathe.
Why did she have sixty-five roses?
Must be her birthday today.
She must have been good to get sixty-five roses,
What can't she come out play?

Sixty-five roses, with yellow and red,
Made her so tired she had to stay in bed.
I looked all around, but I couldn't find,
The sixty-five roses were all in my mind.
I've thrown up and I see that,
The only one thinking of roses was me.
And the reason that the sixty-five roses came,
Was because I was too young to understand the name.

Sixty-five roses, cystic fibrosis,
Made her so tired, she had to stay in bed.
Sixty-five roses, cystic fibrosis,
I wish she had roses instead.
Sixty-five roses, cystic fibrosis,
I wish that she could come out to play.
Life, one suppooses, is no bed of roses,
I wish she had roses instead.

BIBLIOGRAPHY

School Science - Cystic Fibrosis

Dr Koop.Com

Medscape - requires membership

BUPA - Cystic Fibrosis Factsheets

OTHER LINKS

Cystic Fibrosis Australia

Australian Goverment Health Insite

Wikipedia Encyclopaedia - Cystic Fibrosis

Sixty Five Roses - Cystic Fibrosis Research