Designation of Regional Care Centers for adult cystic fibrosis patients- 1A designation of Regional Treatment Centres for adult patients with cystic fibrosis in hospitals / pneumophysiological clinics Iași, Cluj, Timișoara, Marius Nasta Institute-Bucharest and assigning patients to one centre in each territorial profile- 1B responsibility of at least two persons from each of the institutions mentioned above for the care of adult cystic fibrosis patients; training of appropriate persons in the management of cystic fibrosis patients.
Ambulatory monitoring and treatment in Pneumology clinics in designated referral centers for adult cystic fibrosis patients- 2A provision of a referral program for adult cystic fibrosis patients in the pulmonology department of designated reference centers- 2b employment of specialised staff for the care of adult patients with cystic fibrosis (doctor, nurse, physiotherapist, dietician, psychologist, social worker);
Ambulatory monitoring and treatment in Pneumology clinics
Inclusion in the job description of specific entitlements if appropriate staff already exists in the hospital staff structure – 2C periodic Monitoring (quarterly) by Pulmonology Clinics of regional centres; every patient with cystic fibrosis will be able to use clinical trials, lung radiography, spirometry, bacteriological sputum examination, biochemical and haematological tests).
Setting up a home care system for adult cystic fibrosis patients – could become functional by raising funds from potential donors (NGOs))4. Care for patients with cystic fibrosis requiring hospitalisation in reference centres for the treatment of adult cystic fibrosis patients- 4A purchase of medicines necessary for the treatment of patients with cystic fibrosis (see Annex 1)- 4b purchase of medical materials and medical devices necessary for patients with cystic fibrosis hospitalized for exacerbations (see Annex 1))- 4C arrangement of optimal spaces for the treatment of patients with cystic fibrosis in the hospital (adherence to infection control rules, given the multi-drug resistant nature of microbes found in patients with cystic fibrosis)
Screening of adult patients with suspected cystic fibrosis; 5A study of patients with severe bronchiectasis and cystic fibrosis symptoms using a sweat test in collaboration with paediatric wards where a sweat test is performed5.B study of patients with symptoms indicative of cystic fibrosis by genetic testing (by covering the cost of 25 genetic tests per year for patients with high suspicion of cystic fibrosis).
Cystic fibrosis symptoms using a sweat test
Defining a multidisciplinary system of care with other specialties (Pediatrics, gastroenterology, nutrition disorders) for adult patients with respiratory and extrapulmonary disorders6.A joint analysis (pulmonologist and pediatrician) of cystic fibrosis cases to be taken over by the adult network6.B designation of Gastroenterology, nutrition and diabetes departments in cities with Regional Adult Cystic Fibrosis Treatment Centers to serve adult cystic fibrosis patients 6.C establish continuous cooperation with cystic fibrosis patient associations7. Determination of the incidence of adult cystic fibrosis.
Report all cases of cystic fibrosis identified in the country to the Marius Nasta Institute of Pneumology (through existing reporting sheets)7.B entry of the data received into the Electronic Cystic Fibrosis case Register 8. Increase access to specific drugs for adult cystic fibrosis patients- Inclusion in the list of cystic fibrosis compensated medicines of other medicinal products necessary for specific treatment: colistin, gentamicin, ciprofloxacin, moxifloxacin, azithromycinreferences.
Physical indicators: a number of adult cystic fibrosis patients monitored in reference centresabout the number of annual visits of cystic fibrosis patients in reference centresabout the number of days of hospitalization for adult patients with cystic fibrosismany patients with cystic fibrosis diagnosed in adulthoodCystic fibrosis affects about 1 in 2,500 to 3,500 Caucasian newborns. It is less common in black (1 in 17,000 newborns) and yellow (1 in 31,000 newborns), with people in northern Europe most affected.What is Cystic Fibrosis?
The condition is diagnosed soon after birth
Cystic fibrosis or cystic fibrosis is a genetic disease characterized by damage to the lungs, liver, pancreas, intestines and many other organs. This is due to dysfunction of the protein that regulates the water content in the body’s secretions. In cystic fibrosis, the secretions are water-poor, become sticky and stop in the glandular elimination pathways.
The condition is diagnosed soon after birth due to a lack of elimination of tar, but most often due to the child’s malnutrition despite an adequate diet. People with cystic fibrosis have high mortality due to damage to the lungs, pancreas and liver, but survival has increased recently due to advances in medicine, which is now around 50 years old.What do we need to know about cystic fibrosis?
Cystic fibrosis affects several organs and systems:Lung damage: – in cystic fibrosis there is a viscous discharge of the lungs which accumulates in the airway conducive to the appearance of infections and inflammation, which over time lead to changes in the lung tissue through the formation of cysts and fibrosis.
For this reason, the exchange of oxygen and carbon dioxide is difficult.
Pancreatic damage: due to the increased viscosity of the pancreatic secretion, the enzymes it produces are not eliminated in the intestine and are activated inside the pancreas, causing the pancreas to self-destruct, become inflamed and eventually destroy the organ.
At the same time, the damage also affects the cells responsible for insulin secretion in diabetic patients.Liver damage: because of the difficulty in removing bile, the liver will be damaged, the appearance of cirrhosis will cause inflammation and fibrosis.Intestinal damage: due to poor elimination of pancreatic enzymes and bile, food is not completely digested and nutrients are not absorbed in the intestine. In addition, due to the composition of the under-digested feeding bowl, the passage is altered and can lead to blockage of the intestines.Impaired fertility: men are often infertile due to lack of sperm or low sperm production.
Females are generally fertile, but may have low fertility due to thickening of cervical mucus or malnutrition.Risk factors for cystic fibrosis:The risk factors for cystic fibrosis are purely genetic.Cystic fibrosis is caused by mutations in the CFTR gene located at locus 7q31.2. This gene encodes a protein in the membrane of secretory cells (which produce mucus, sweat, saliva, tears and digestive enzymes) and plays a role in the transport of water into the cell. A mutation in the CFTR gene causes an abnormal protein to form, resulting in a decrease in the concentration of water in the secretions.More than 1,000 mutations in this gene are responsible for cystic fibrosis.