Cystic fibrosis

April 2017
Cystic fibrosis remains the most severe of all genetic diseases. The following protocol explains to health professionals in a pragmatic way how to care and deal with diagnosed patients.


Diagnosis

It concerns:
  • The newborn or the patient diagnosed after his family history,
  • Any patient showing the symptoms of the disease.


It is established on:
  • Sweat tests using the Gibson and Cook method,
  • A test for CGTR genes mutation,
  • Other tests among which the functional study of CFTR protein.

Therapeutic care

Treatments

  • Treatment of the respiratory system (bronchi/lungs)
    • Respiratory physical therapy;
    • Administration of aerosols and treatments to be inhaled (aerosoltherapy);
    • Dealing with infections (antibiotherapy).
  • Digestive and nutritional treatment
    • Strict feeding of the infant: breastmilk, supplement in sodium, proteins
    • Daily diet in the child and the adult
      • Rich in sodium, vitamins A, E, D, K, B-carotene, trace elements
      • Administration of pancreatic extracts
      • Reinforced hydration

Therapeutic education

A therapeutic management is recommended to prevent complications related to the disease:
  • Strict rules of hygiene to limit risks of infection,
  • Breathing exercises to repeat,
  • Specific nutrition, food, salt, water, calcium and glucids
  • Up to date vaccines, having a “cystic fibrosis care and information card”, taking his medicine,
  • Healthy lifestyle and environment
    • No contact with tobacco, or allergens;
    • Incite to do sports or any physical activity;
    • Help facilitate professional or school integration by a special welcoming;
    • Ask for the assistance of a social worker or psychologist.

Treatment of the evolution of the disease

An observation and a specific treatment for the inherent symptoms of the evolution of cystic fibrosis is recommended.
  • Breathing system
    • Aggravation of the general physical state and the respiratory system,
    • Allergic bronchopulmonary aspergillosis,
    • Pneumothorax and hemoptysy,
    • Chronic breathing difficulties.


Any aggravation symptoms requires an immediate consultation.
  • Digestive system
    • Stomach pains, oesophagitis, colopathy;
    • Complications of the liver and the pancreas.
  • Other symptoms
    • Diabetes,
    • Dehydration,
    • Chronic sinusitis (ENT complications),
    • Slowed down growth, bone and articular pains,
    • Urogenital complications, impaired renal function,
    • Depression.

Sources

Cystic Fibrosis Trust (CFT) the national support group for Cystic Fibrosis
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