What is cystic fibrosis? 

Cystic Fibrosis (CF) is an inherited disorder.  A baby can only be born with the disorder if both parents carry the abnormal gene. It is not caused by any previous illness suffered by one of the parents or by anything that happened during the mother’s pregnancy.  It is non-contagious.

CF affects a number of organs in the body (especially the lungs and pancreas) by clogging them with thick, sticky mucus.  In the lungs this can cause shortness of breath, a chronic cough and repeated chest infections.  If the blockages affect the pancreas this will reduce or stop enzymes from being released to digest food, causing problems with poor weight gain and malnutrition due to malabsorption.

At present there is no cure for CF, but the affected gene has been identified and researchers are working to find ways to repair or replace it, and medications to treat CF complications.

Symptoms: Lung, Digestive System and Other Organs

The type and severity of CF symptoms varies from person to person. In some people not identified with CF through newborn screening, there may be health problems that indicate the presence of CF, such as:

  • salty-tasting skin
  • slow weight gain, failure to thrive, even with good appetite
  • wheezing, coughing, pneumonia
  • abnormal bowel movements.

These symptoms are not unique to CF, but when many of these symptoms are present a doctor may want to do a sweat test for CF, to obtain a conclusive diagnosis.

Lungs

Almost everyone with cystic fibrosis will sooner or later develop lung disease. However, when it occurs and how bad it is differs from person to person.

The thickened mucus builds up in the bronchioles and reduces airflow, making the patient short of breath and wheeze with exercise. This mucus can build up to such an extent that it plugs the smaller airways of the lungs and can then harbour bacteria which cause infection.

Effective Airway Clearance Techniques are required to help remove this mucus reducing the chances of infection. 

Digestive System

Cystic Fibrosis mainly affects the digestive system by mucus blocking the pancreas, stopping the digestive enzymes produced from making their way to the gut to digest food.

This complication can cause malnutrition and malabsorption even though the patient has good appetite.

Symptoms of malabsorption include poor weight gain, frequent foul-smelling stools, stomach ache, and excessive gas.

Enzyme supplements, supplementary feeding, and vitamin replacement all assist with compensating for decreased activity of the pancreas.

In older CF patients, the pancreas may become less active and reduce or stop the production of insulin, resulting in the CF-related diabetes (CFRD). This rarely occurs in children. CFRD is treated in much the same way as Type 1 diabetes, but varies between individuals.

Treatments:

  • Physiotherapy
  • Medications
  • Exercise
  • Nutrition

Source: Cystic Fibrosis New Zealand

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