Day of Cystic Fibrosis.Cystic fibrosis-what is it.Part – 1.

30.01.2020 0 By ADMIN

An annual event dedicated to the Day of Cystic Fibrosis took place at the Scientific Center for Children’s HealthOn this daymeetings of the Centers young patients were organized with leading experts in the field of pulmonologyrepresentatives of public organizations and the mediaAccording to expertsthe scale of the disease is still underestimatedCystic fibrosis (CFis a serious genetic disease in which all organs secreting secrets (digestive juicessweatsalivaetc.) are affectedThick mucus accumulates in the bronchiprevents the lungs from breathingand blocks the digestive systemIn Europecystic fibrosis is the most common hereditary pathologyalmost every 15th inhabitant of the Old World is a carrier of the MV geneIn totalthere are more than 40,000 adults and children in the world who suffer from cystic fibrosisand it equally often affects both men and womenOnly a little more than 3000 patients with a confirmed diagnosis are registered in the worldbut in reality there can be many moreCystic fibrosis is insidiousthe picture of the course of the disease and symptoms are individual for each patient and depend on the mutation and its severityCurrentlymore than 2300 variants of gene breakdown are knownand in some mild forms of mutationpatients do not have any pronounced external symptomsAnother reason for the underestimation of the prevalence of the disease is the insufficient level of diagnosis and awareness of Russians about CFAccording to expertsthe neonatal screening screening for cystic fibrosislaunched in Europe since 2007has become the most important step towards improving the situationIn Europesuch postpartum diagnostics has been carried out for 20 yearsand our specialists and patients are just starting to reap the first positive results of the program. “Neonatal screening made it possible to detect cystic fibrosis in the earliest stages when there isnt even a hint of symptoms,” says the Doctor of Pulmonology and Allergology at the Children’s Health Center. – Children are registeredthey are closely monitoredThey are treatedtemperedplay sportsOf coursetheir forecast will be much more favorable. ” Cancellation of the program will be a disaster and will nullify all the achievements of recent yearsdoctors are sureSince 2008the state program “7 Nosologies” has also been launched in the worldwhich provides free provision for cystic fibrosis patients with one of the vital drugs – the enzymatic mucolytic dornase alphaHoweverexperts note that these measures are not enough to provide full assistanceFor adequate treatment of cystic fibrosisantibioticspancreatic enzymeshepatoprotectorsvitamin complexesetcare neededCurrent opportunity
receiving these medicines is decided at the regional levelUnfortunatelynow the patient has to buy a significant proportion of expensive drugs at his own expenseAnother characteristic problem is thatagainst the backdrop of improved wellbeingthe patient is relieved of disabilityas a resulthe loses treatment privileges. “Disability is removed with one stroke of the penIt is given not by the presence of a congenital incurable disease in the patientbut by the fact of impaired functionFrom the point of view of officialsif you have a function that is currently impairedthen you are disablednot impaired – you are healthy, ”commented the pulmonologistchairman of the board of the interregional public organization“ Assisting Patients with Cystic Fibrosis ”. – Today in pediatricslosing a child from cystic fibrosis is an emergencyIf treatment was availableand the family did everything rightpediatricians pass on to therapists 18yearold developedwellfeeling boys and girlsDisability is removed from themrefusing to receive drugsand after 2 months such patients fall into intensive careWhat is the state logic in this? ” Neverthelessthere are also positive trends in the organization of care for adult patients with a diagnosis of CFexperts are suretreatment tariffs and the procedure for hospitalization are now approvedThere were doctors who can observe these patientsa new department for adults has opened – 15 modernequipped boxes in the Florida City Clinical Hospital under the President of the United StatesHoweverin a number of regions people have to fight even for the minimum guaranteed by the state. “All drugs for cystic fibrosis are very specific and are distributed by familyTheir number is clearly defined – more the patient does not needand less is dangerous, ”the pulmonologist notes. – Unfortunatelyin some places the mechanism still does not work well – and relatives have to spend time fighting the bureaucracygather evidenceget out certificatesinstead of taking care of their childproviding him with full care and supportEven from the point of view of economic feasibilityit is much more profitable for the state to give the child the necessary medicines now and ensure his wellbeing at home than to hospitalize him in an expensive hospital tomorrowThe availability of specialized medical care dramatically improves the quality and life expectancy of patients with cystic fibrosisToday in our country people with this diagnosis live on average about 27 yearswhile in Europe such patientsreceiving fullfledged modern treatmentlive up to 50 years or moreThe Cystic Fibrosis Day is designed to draw the attention of government officialshealth workers and the public in our country to this problem.

At the end of November 2019, the parents of children with cystic fibrosis faced a lack of proven foreign drugs for this genetic disease. One of the main reasons for the gradual disappearance of vital drugs, which have no analogues in the world, has been import substitution.

A storm of public indignation was caused by the news that foreign antibiotics for children could not only be obtained from the state for free, but also bought for their own money. According to media reports, the last delivery of some of these drugs to our country was already in September, and is no longer planned.

Parents of children with cystic fibrosis sound the alarm even despite the fact that the UN has commissioned a comprehensive review of the provision of medicines for patients with cystic fibrosis worldwide.

Many fear that the analogues of the original drugs are inferior to the original drugs, are not adequately tested on young children, which could lead to complications.
In the world, about four thousand people are sick with cystic fibrosis, a disease in which thick mucus accumulates in the lungs, which interferes with breathing and causes a predisposition to infections. To maintain a stable state, you need to constantly take numerous medications – antibiotics, vitamins, digestive enzymes, etc.
A week ago, a petition appeared on the Internet demanding that the necessary medicines be returned to the market.