Cystic fibrosis
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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.
It is established on:
Any aggravation symptoms requires an immediate consultation.
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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 FibrosisPhotographic copyright: © Eraxion | Dreamstime.com