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DEFINITION
The FQ is the most frequent genetic and hereditary illness in the white race. Fond of the glands secretoras of the body, hurting to organs like the lungs, pancreas, liver and to the digestive and reproductive devices. The persons who have FQ, produce a very viscous snot, which stops up the lungs and the digestive system, being difficult the respiration and the correct assimilation of the food.
INCIDENCE
Approximately one of the 2.500 children is born with FQ. It is anticipated that one of the 25 persons is a bearer of the defective gene that the FQ causes. The bearers are completely healthy, they do not endure the illness, but yes they can transmit it. If both parents are bearers of a gene defective FQ, a possibility exists between four in every pregnancy of that the son of padezca FQ (if he inherits the defective gene of every spouse).
DIAGNOSIS
The FQ diagnosis is confirmed generally with a test of the perspiration (iontoforesis): the FQ patients segregate excessive salt quantities in the perspiration. Also genetic analyses can be realized with blood samples.
TREATMENT
For the time being there is no priest for the FQ. Nevertheless, the advances in the treatments are helping the patients to control the symptoms, and the life expectancy has turned out to be increased in the last years, so that the FQ patients come at the adult age, often enjoying a working and almost normal life.
The future is put in the gene therapy, by means of which it will be possible to replace the causative defective gene of the FQ with the normal one that works correctly.
- Treatment of the affectation of the respiratory system: The excessive stickiness of the mucuses provokes the obstruction of the bronchi, giving place to a respiratory insufficiency associated with infectious processes of repetition that damage the pulmonary textile. The treatment to be continued is a combination of antibiotics (for oral route, nebulizados or intravenous), respiratory physical therapy and continued physical exercise, with the target to support the lungs as clean as possible. In cases of severe affectation, there will be necessary the achievement of a pulmonary transplant.
- Treatment of the affectation of the digestive tract: The complaint of the pancreas is characterized by a blockade of the flow of the enzymes digestive (tripsina, amilasa and lipasa) to the duodenum, producing a bad absorption of the proteins, carbohydrates and fat, causing digestive disorders, pain and abdominal distension. There takes place the state of loss of weight, malnutrition and alterations in the growth of the patients of FQ. The most widespread treatment is the administration of enzymes pancreatic, food supplements and complex vitamínicos. Other complications that can appear are a hepatic, intestinal problems and diabetes.
- Treatment of the affectation of the glands sudoríparas: The FQ patients lose excessive salt quantities in the perspiration, therefore in warm time precautions must take, as well as in periods of intense physical activity or feverish processes. It is necessary therefore to drink important liquid quantities and sometimes to administer salt supplements.
The FQ must be treated by a multidisciplinary sanitary team formed by pediatricians, neumólogos, gastroenterologists, cardiologists, microbiologists, dietitians, physiotherapists, psychologists, social workers, nurses and auxiliary personnel. Every professional has a very important role in the treatment of the FQ.
At present the FQ tends to talk each other in Units of Reference, which allow a particular alertness and pursuit of the patients.
The parents and the family are a fundamental support so that the patient grows in an ambience of normality and responsibility with regard to his treatment. Also they have a fundamental role the associations of patients' parents.
TO SEE ALSO
Spanish federation against the Fibrosis Quística WHAT DOCTOR CAN TREAT ME?
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