Therapy of cystic fibrosis
Every person affected by cystic fibrosis is recommended to consult a cystic fibrosis outpatient clinic or a human geneticist (specialist for hereditary diseases). These can help to improve the quality of life or, if you wish to have children, calculate the probabilities of a sick child. Provided the parents are fertile and capable of reproduction.
Otherwise, the treatment is symptomatic, since the cause, the defective gene, cannot be eliminated. In cystic fibrosis, it is important to ensure a sufficient intake of common salt (sodium chloride, NaCl).Of course, a mucolysis is aimed at. Mucolysis is the dissolving of mucus, especially in the lungs, to make breathing easier.
Drugs but also inhalation can relieve the symptoms. If the lung function becomes increasingly poor, oxygen can be given. Intensive physiotherapy (physiotherapy), such as tapping massage and respiratory gymnastics, is used to treat the lung changes caused by cystic fibrosis.
The disease often ends with a necessary lung transplantation. The waiting lists, however, are long. The oral administration of pancreatic enzymes and fat-soluble vitamins is also part of the therapy.
The task of the pancreas must therefore be supported or rather replaced. Fat-soluble vitamins are A, D, E and K. They must be given directly into the blood, as they cannot be absorbed from food due to a lack of digestive enzymes. The diet should also contain many calories, as only a fraction of them can be absorbed from food.
To avoid additional risk factors for complications such as flu or pneumonia, the child should be vaccinated. The following vaccinations are recommended: Of course, these measures require a consultation with a doctor with whom the risks should be discussed. Great hope of cystic fibrosis therapy is nowadays put into genetic research.
Attempts are being made to introduce the missing genetic information into the human genome. The search is on for vectors that can master this task. Vectors can be, for example, bacterial or viral DNA, which are able to integrate the healthy frequency into our genetic material.
The therapeutic approach for unborn patients is also currently being tested. In mice, it has already been possible to introduce the healthy gene containing the correct gene sequence into the mouse embryos through amniocentesis (amniotic fluid inoculation). Thus, the healthy CFTR gene was produced in these mice.
Amniocentesis is a puncture and removal of fetal cells from the amniotic fluid through the abdominal wall of the mother. In Germany, however, this form of intrauterine (= in the uterus) “therapy” is prohibited.
- Measles
- Pneumococci
- Flu
