Cystic Fibrosis (cystic Fibrosis), Cystic Fibrosis

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Cystic Fibrosis (cystic Fibrosis), Cystic Fibrosis
Cystic Fibrosis (cystic Fibrosis), Cystic Fibrosis
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Cystic fibrosis

Cystic fibrosis is an inherited metabolic disease that causes symptoms in early childhood and progresses chronically. Cystic fibrosis (cystic fibrosis, CF) is also known as cystic fibrosis. The cause of the disease is a genetic defect, which means that a particularly thick mucus is formed in many organs of the body. The viscous mucus clogs the organs' ducts, which leads to pathological changes.

This is where the name "cystic fibrosis" originated: This refers to a connective tissue-like transformation of the pancreas, i.e. fibrosis, while conspicuous cysts arise due to the backflow of the secretion.

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  • more on the subject
  • Advice, downloads & tools
  • Newborn screening for early detection
  • What are the symptoms?
  • How is the diagnosis made?
  • How is cystic fibrosis treated?
  • Whom can I ask?
  • How are the costs going to be covered?

The organ changes affect not only the pancreas, but a number of organs, especially the lungs, but also the intestines, the liver and the reproductive organs.

Newborn screening for early detection

The examination for cystic fibrosis is part of the newborn screening program in Austria, a program for the early detection of congenital hormonal disorders and metabolic diseases. This test will detect cystic fibrosis in approximately one in approximately 3,500 babies.

What are the symptoms?

Symptoms of cystic fibrosis include:

  • chronic cough,
  • frequent respiratory infections,
  • chronic abdominal pain and indigestion,
  • Pancreatic insufficiency,
  • lack of weight gain as well
  • unusually salty sweat caused by a disruption in the formation of salt in the sweat glands.

How is the diagnosis made?

An early diagnosis significantly improves the prognosis, as close-knit medical care is possible due to the ever better therapeutic options.

Although newborn screening is very reliable, the disease can sometimes still be present even if the test is negative. If there is a suspicion of cystic fibrosis in a child based on the symptoms, a so-called sweat test is first carried out, in which the increased salt content in the sweat is detected. The diagnosis is then confirmed by the detection of the genetic defect in a laboratory test, for which a simple blood sample is required.

How is cystic fibrosis treated?

Cystic fibrosis is incurable, the therapy is aimed solely at relieving the symptoms (symptomatic treatment). A common complication in patients with cystic fibrosis is pneumonia, which requires special antibiotic treatment. Physiotherapy and breathing exercises as well as inhalation or intake of expectorant drugs prevent pneumonia.

Ailments in the digestive tract are treated by taking enzymes from the pancreas, giving vitamins and eating a particularly high-calorie diet. Regular exercise also prevents complications. Ideally, cystic fibrosis is cared for and treated in a special outpatient department by a multidisciplinary team.

forecast

The disease is usually associated with a lower life expectancy. In the last few decades, however, the life expectancy and quality of life of those affected have risen sharply due to early diagnosis and improved therapeutic options.

Whom can I ask?

To clarify signs of cystic fibrosis or a congenital metabolic disorder in a newborn or child, it is best to contact a specialized outpatient department or metabolism center in a children's hospital. If cystic fibrosis is suspected during the newborn screening, the parents or legal guardians receive a written invitation for an examination with a list of special outpatient departments.

It is rare that mild forms of the disease are only discovered in adulthood. Adults are then sent by their doctors to a children's hospital for a sweat test for clarification. Further care then takes place in our own adult centers for cystic fibrosis.

A list of special outpatient clinics for cystic fibrosis can be found on the website of

Cystic Fibrosis Help Austria.

How are the costs going to be covered?

The e-card is your personal key to the benefits of the statutory health insurance. All necessary and appropriate diagnostic and therapeutic measures are taken over by your responsible social insurance agency. A deductible or contribution to costs may apply for certain services. You can obtain detailed information from your social security agency. Further information can also be found at:

  • Right to treatment
  • Visit to the doctor: costs and deductibles
  • What does the hospital stay cost?
  • Prescription fee: This is how drug costs are covered
  • Rehabilitation & cure
  • Medical aids & aids
  • Health Professions AZ

and via the online guide to reimbursement of social insurance costs.

Additional Information:

  • Cystic Fibrosis (Orphanet)
  • Cystic Fibrosis Help Austria

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