September 8th is the Day of the Patient with Cystic Fibrosis. What kind of disease is this? The director tells charitable foundation“Oxygen” by Maya Sonina.
When is cystic fibrosis diagnosed?
Now, according to the newborn screening program, blood tests are taken from infants in the maternity hospital to identify a number of hereditary diseases, including cystic fibrosis. True, those who were born before 2006 and who were not affected by this program risk not receiving the correct diagnosis on time. There are still even adults who were previously treated for anything, but the correct diagnosis was made to them very late, already when the disease had irrevocably progressed.
Since laws in our country are constantly changing, and there is a tendency to save public money on the social and medical budget, it is very difficult to predict how lucky the parents of sick children and the patients themselves will be in the future. Nevertheless, medical science is developing, even in our country, and there is hope for those medical enthusiasts who, despite going through a lot of obstacles, still promote progressive methods of treating this disease in Russia. Therefore, let's hope that today's babies with CF are luckier than those who are 15-18 years old today.
Now the drug Kalydeco, or Ivacaftor, has been registered in the United States, which affects the very cause of cystic fibrosis, the production of exocrine glands of too viscous mucus that accumulates in all internal organs. So far, of the many, many mutations of cystic fibrosis, a drug has been developed for only one rare mutation, but trials are also underway for drugs that help with other mutations. The patient must take such a drug for life, and today, unfortunately, the annual course of Kalydeco is approximately 300 thousand dollars per year. The amount is prohibitive for benefactors, but let's hope that Kalydeco will eventually cease to be a rarity and will be available on the international market. He has amazing treatment results that return a person to a full life.
In our country there are few specialists who know cystic fibrosis, and they are worth their weight in gold. Moscow treatment centers are trying to spread their knowledge and experience throughout the country. They themselves are in close contact with European centers for the treatment of cystic fibrosis. Until now, the treatment protocols for this disease adopted in our country have not been brought into line with modern European standards. Therefore, doctors have to somehow independently find a way out of the situation in especially severe cases.
We have many neglected and untreated patients. The reason for this is the lack of beds, the lack of specialists and the state’s underfunding of the treatment needs of patients. The main simplified treatment regimen consists of antibiotics, mucolytics, hepatoprotectors and a set of special physiotherapeutic exercises to rid the lungs of stagnant infected sputum. This scheme is used to treat both here and abroad. Another thing: often our patients receive instead effective medicines their domestic or eastern substitutes, or often do not receive anything from the state.
Is rehabilitation possible?
For a patient with cystic fibrosis, the first main condition in his life is discipline and diligence. Every day for life you need to take medications by the hour, you need to actively follow physical exercise so that phlegm does not stagnate in the lungs. The second main condition: to be able to fight with the state, which is reluctant to help the patient, for your life and your rights to it. Be able to prove, be able to return to the office from which you were kicked out, be able to insist on proper treatment and provision of medications. Not everyone can do this, and often parents, not accustomed to defending rights, give up or rely on charity alone. It shouldn't be this way.
Charity cannot replace the state and plug all the holes. Positive result is possible only when a full-fledged team has been created: patient, doctor, relative. Neither the relative of the patient nor the patient himself can take a passive position of waiting, otherwise he will not survive. Each region must have its own active patient organization and simply mutual assistance, so that people do not hide in their own corners, and study their capabilities and their rights, and share such difficult experiences.
There are patients with cystic fibrosis abroad who have reached retirement age and living full life. For us, these are mainly children and young guys who are trying to combine the usual healthy life With daily treatment. No matter what threatens them, they enter universities, fall in love and get married, work and even play sports while they are on their feet, before the disease knocks them down.
Several portraits
Anya Kolosova is 32 years old. She considers herself a long-liver.
Anya is a doctor by profession. She defended her PhD and plans to live and work further.
Anya is sick with cystic fibrosis and is on a waiting list for a lung transplant, hoping to live until the day when life takes a sharp turn, because she will begin to breathe freely.
Anya works for the Pomogi.org charity foundation and is a volunteer and board member of the Oxygen charity foundation. Anya helps people like her.
This good example for all those who complain of fatigue or depression. Anya saw death and said goodbye to those like her, those who were less fortunate, who could not be saved due to lack of beds and due to lack of medicine. Anya has a philosophical attitude towards death, and she has nothing to fear, because everything bad in her life has already happened: she was born with cystic fibrosis in Russia.
What funds care for such patients?
Thank God, those suffering from cystic fibrosis are not spared by charity. There are multidisciplinary foundations dedicated to helping CF patients, such as “Pomogi.org”, “Creation”, “Give Hope”, “Devotion”. There are foundations that specialize in helping such patients: “Islands” in St. Petersburg, and in Moscow - “In the Name of Life” and “Oxygen”. The guys have a place to turn for help, but there are still so many who don’t know about the funds or what the state is obliged to give them.
It is necessary to establish contact with the Ministry of Health, we need legal education for patients and their relatives, we need to adopt European standards of treatment, and, in the end, we need to not invest fabulous money on the international inflated image of the country, but spend this money on helping the disadvantaged in this country. Only such a power can be strong.
Cystic fibrosis (mucoviscidosis; synonym: cystofibrosis, pancreatic fibrosis) is a hereditary disease characterized by systemic damage exocrine glands (exocrine glands) and manifested by severe disorders of the functions of the respiratory system, gastrointestinal tract and a number of other organs and systems.
The disease was first described in 1938 by American pathologist Dorothy Anderson. She also proved its hereditary nature.
In 1946, American pediatrician Sydney Farber proposed the term “cystic fibrosis” (from the Latin mucus - mucus, viscus - viscous).
The disease is associated with a mutation in the CFTR gene (cystic fibrosis transmembrane conductance regulator, or the Russian designation CFTR - cystic fibrosis transmembrane regulator), which is responsible for the transport of sodium and chlorine ions across the cell membrane.
The CFTR gene was identified in 1989. To date, more than 1,500 of its mutations have been identified.
Every 20th inhabitant of the Earth (every 30th inhabitant of Europe) is a carrier of a defective gene. Cystic fibrosis occurs in a person if both of his parents are carriers of such a gene. In this case, the probability of their birth healthy child is only 25%.
Cystic fibrosis is most common among Caucasians, but cases have been reported in all races. Among the Caucasian population, one case of the disease is recorded per 2000-3000 newborns, and among the black African and Japanese populations its frequency is one case per 100 thousand.
It is equally common in both boys and girls.
In Russia, 1,509 patients with cystic fibrosis are officially registered. In our country, according to experts, for every identified patient there are 10 undiagnosed cases.
The disease usually appears in early childhood. Symptoms: salty taste sweat, the appearance of mucus plugs in the bronchi and bronchioles, frequent bronchitis and pneumonia, poor bowel function.
Different mutations provide different clinical picture diseases.
As a result of a defect in the CFTR gene in all organs that secrete mucus (bronchopulmonary system, pancreas, liver, sweat glands, salivary glands, intestinal glands, gonads), thick and viscous contents accumulate, the release of which is difficult.
In cystic fibrosis, the pancreas may be affected, which leads to the fact that the required amount of enzymes does not enter the digestive system and food is not digested. Almost all patients suffer from underweight. The pancreatic ducts become blocked, resulting in the formation of cysts. Diabetes mellitus often develops against the background of cystic fibrosis.
When the bronchopulmonary system is damaged, patients develop very viscous sputum, into which pathogenic microorganisms easily enter, which lead to gradual destruction lung tissue. The most common pathogens in the disease are Staphylococcus aureus and Pseudomonas aeruginosa. Mucus plugs appear in the bronchi and bronchioles. Inflammatory process in the bronchi in combination with their obstruction purulent plugs often leads to irreversible expansion of the pulmonary lumen and the formation of bronchiolectasis.
Due to thickening of bile and blockage bile ducts The disease also affects the liver. Cirrhosis often develops.
Defeat sweat glands manifests itself immediately after birth, with sweat a large amount of salt is removed from the body.
The most common is the mixed (pulmonary intestinal) form of cystic fibrosis (75–80%). About 15-20% of patients suffer from the pulmonary form and about 5% from the intestinal form.
Most patients are diagnosed before the age of two; in 4% of patients, cystic fibrosis is diagnosed in adulthood.
Modern possibilities of prenatal (antenatal) examination make it possible to identify mutations leading to the disease.
Therapy for cystic fibrosis is complex and is aimed at thinning and removing viscous sputum from the bronchi, fighting infection in the lungs, replacing missing pancreatic enzymes, correcting multivitamin deficiency, and diluting bile.
A person with cystic fibrosis constantly needs medications throughout his life, often in large doses. They need mucolytics - substances that destroy mucus and help its separation. In order to grow, gain weight and develop according to age, the patient must receive enzyme preparations. Otherwise, the food simply will not be digested. Also important has food. Easily digestible foods are prepared for sick children. Food should not contain coarse fiber.
Antibiotics are often necessary to control respiratory infections and are prescribed to relieve or prevent exacerbations. In case of liver damage, hepatoprotectors are needed - drugs that dilute bile and improve the function of liver cells. Many medications require inhalers to administer.
Lung and liver transplantation is another option for cystic fibrosis patients after resources drug therapy exhausted.
Kinesitherapy is vital for patients with cystic fibrosis - breathing exercises and special exercises aimed at removing phlegm. Classes should be daily and lifelong.
It is not advisable for patients with cystic fibrosis to stay in preschool institutions. Attending school in good and satisfactory condition is possible, but an additional day off per week and free attendance at school on days of treatment and examination at the clinic (at the pulmonology center), and exemption from exams are required. The question of the possibility of vaccinations for children with cystic fibrosis is decided individually.
Children with cystic fibrosis are not removed from the dispensary register, but upon reaching the age of 15 are transferred under the supervision of a therapist to a clinic for adults.
Among patients with cystic fibrosis, the mortality rate is very high: 50-60% of children die before reaching adulthood. In the USA and European countries The average lifespan of these patients increases every year. On this moment this is 35-40 years of life.
Recent studies conducted by Australian scientists have shown that women with cystic fibrosis die earlier than men.
According to experts, a patient born in 2000 modern medicine can guarantee 50 years life. American doctors are already monitoring elderly patients, some of whom are over 60 years old.
In Russia, cystic fibrosis has become a national priority program, and the first lung transplants have been performed for patients with this disease. Since 2007, mass screening has been introduced in all constituent entities of the Russian Federation within the framework of the National Health Program (a complex of mass diagnostic measures) newborns for cystic fibrosis. A major role in improving care for patients with cystic fibrosis was played by the organization in the country of specialized Russian and regional centers for diagnosis (including prenatal), treatment and rehabilitation of patients with cystic fibrosis, as well as the current government orders and orders of the Ministry of Health and Social Development on a number of benefits for disabled children with cystic fibrosis.
On September 8, Russia celebrates International Cystic Fibrosis Day - genetic disease, in which, due to a mutation of a certain gene, stagnant mucus accumulates in the organs, and vital organs, in particular the lungs, suffer. This is the most common pathology among hereditary diseases. The establishment of this memorial day is another way to draw attention to the problems of diagnosis and treatment of this serious disease. About the problems of patients diagnosed with cystic fibrosis in Russia - our conversation with Maya Sonina, director of the Oxygen charity foundation, which helps patients with cystic fibrosis.
Reference:
The word "cystic fibrosis" comes from the Latin words mucus - "mucus" and viscidus - "viscous". This means that the allocated different organs the secretions have too high density and viscosity, which is why the bronchopulmonary system, intestinal glands, liver, pancreas, sweat and salivary glands, etc. are affected. The lungs are especially affected, where chronic inflammatory processes. Their ventilation and blood supply are disrupted, causing painful cough and shortness of breath. The main cause of death in patients with cystic fibrosis is hypoxia and suffocation.
– Maya, as far as we know, in our country the situation of patients with cystic fibrosis is very different from that which exists abroad. What are the main problems of our fellow citizens suffering from this disease?
– This is a catastrophic lack of beds for patients over 18 years of age, and a constant shortage of expensive medicines. With children, the situation is still better, there are more specialists and beds, and the state is paying attention to children Special attention. Donors also love to help children. And adults, as they say, are in flight.
When sick children turn 18, they immediately become participants in a marathon for survival. Firstly, it is difficult for them to obtain disability, and this raises the threat that they will not have benefits for medications and treatment. Imagine, there are still only 4 beds available for adult patients with cystic fibrosis in the capital. There are certain regions, such centers as Yaroslavl, Samara, from where patients do not tend to go to Moscow or St. Petersburg, since everything is more or less stable there, everything is at hand, and good treatment and drug provision. The rest, unfortunately, are forced to wait in line. Therefore, skip treatment and routine diagnostics. And this is irreversible, it brings us closer to death. That's why average duration The lives of patients with cystic fibrosis in our country are significantly shorter than abroad.
– So it turns out that in Russia a patient with cystic fibrosis has little chance of becoming an adult? How deadly is this disease? Can the patient adequate treatment live to old age?
– Uninitiated people, and even the state, judging by its approach, usually get the impression that cystic fibrosis affects children, and that such patients do not live to be of legal age. But in fact, cystic fibrosis is not fatal; a person with this disease requires lifelong treatment, but he can and should live, as, for example, people with asthma or diabetes live. Some popular media literally “drive it into the brain” that patients with cystic fibrosis are suicide bombers, and it turns out that there is no point in helping them, because they will die anyway. However, children who reach their 18th birthday try to lead active lives and have the same interests as their healthy peers. If they are properly supported, fully comply with doctors’ instructions, provide adequate medications, carry out therapy at a good level, then they can live a long time, even have healthy children, work, study, start families, and, in principle, live to old age. Abroad, it is no longer uncommon for patients with this diagnosis to be of retirement age. In Russia there is no.
These patients can live a full life, and the first thing to do is to abandon the imposed stereotype. So that both ordinary people and, most importantly, officials understand that these are the patients who need to be helped and met halfway.
– What are the statistics of the disease today? Can we talk about trends?
“People with this disease have always existed. We can say with confidence that diagnostics are now well established, much better than, say, in the 90s. There is an increase in the number of infants diagnosed. Moscow pediatricians now have a significantly greater workload due to the fact that the admission of sick children from all over the country to hospitals has now increased. It's unclear to me what will happen next. Unfortunately, the treatment of this disease in our country is progressing mainly only through the efforts of enthusiastic doctors. Nowadays, intrauterine diagnostics are well developed, and some mothers, unfortunately, decide to terminate their pregnancy after learning that the child will have such a diagnosis. Often, such a prenatal examination is done in families where there have already been cases of the birth of children with this disease.
– What is the probability of having subsequent children with a similar diagnosis in the family?
– Everything is individual. There are large families where four children were born in a row, and all are sick. And in some families only one child is sick. It's impossible to predict. But according to statistics, if a mother and father are carriers of the mutation, then there is a 25% chance that they will have a sick child.
– Do we have data on the mortality of children diagnosed with cystic fibrosis in Russia?
– The mortality rate of children has now become significantly lower compared to the indicators of the 90s. Pediatrics is more or less supported in our state. Pediatricians have more opportunities than those who treat patients who have transitioned to the adult sector. Therefore, overall, child mortality has decreased. Children in serious condition are now extremely rare, unlike what happened 10 years ago. These are normal, ordinary children: they run, walk, play. The only thing is that they need constant support with medications and a regimen.
– So, the situation with adult patients is depressing?
-Very depressing. We have to see how patients, due to the fact that they cannot receive the required treatment, inevitably worsen their condition, and they simply die before our eyes. And we can't do anything about it. In Moscow there are too few places for hospitalization of patients with cystic fibrosis. And in the regions, doctors most often do not understand how to treat and from which side to approach these patients. We don't even have the necessary therapeutic standards that take into account the specifics of this disease. In the regions, everyone is treated the same way: symptomatically, according to standards common to all diseases, and the characteristics of the disease and its course are not taken into account. So we keep hearing about the deaths of young people who could have lived if they had had access to modern technologies treatment.
– Who is your main hope for help? More on the state or private philanthropists?
– It turns out that the main hope is in philanthropists, but now the state, at least in the person of the Ministry of Health, seems to have turned its face to our patients. The Ministry of Health has become more democratic, its officials are ready for dialogue. Appeared hotline Ministry of Health, and this simplifies many serious problems problems that arise during hospitalization and treatment of our patients in regional hospitals, in providing them with medications. Still, we place certain hopes on the state. And, God forbid, the current Ministry of Health will last, then we will have time to do a lot together.
It is also impossible to do without private charity, because the Ministry of Health in our country cannot solve all problems, primarily financial ones. But at the same time, no aggregate charity will meet the needs of all patients, not only those with cystic fibrosis, but also other serious diseases, if they are not heard by the state.
– Do significant sums of money go into maintaining the life of a patient with cystic fibrosis?
– Yes, they are simply too much for individual philanthropists. And patients and their relatives really hope that philanthropists will save them. Unfortunately, there are too many patients and not everyone can be helped. Therefore, you often have to make such difficult choices, just like in war.
– What measures, in your opinion, should be taken to improve the situation?
– Standards for the treatment of cystic fibrosis must be adopted at the global level and in accordance with global practice. And in the future, full support must be established necessary treatment. Unfortunately, our treatment is not the same as in Europe: there are not enough resources. And also, of course, finances. Finances are being reduced, but on the contrary they should increase, and not from time to time, and not only to help children! After all, in the end, these children will also soon become adults. They received treatment from philanthropists and from the state until they were 18 years old; when they grew up, they can no longer count on such help. They became not so interesting, not so attractive. It shouldn't be this way. Everyone wants to live, not only children, but also adults.
Title Cystic fibrosis patients live longer, but many problems remain
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According to the Cystic Fibrosis Foundation, the average life expectancy of patients with cystic fibrosis (cystic fibrosis of the pancreas) has increased significantly and now averages 35 years. Currently, adults account for up to 40% of patients with cystic fibrosis. In recent years, life expectancy has increased by about 50%.
The main factors that improve life expectancy and its quality are a more aggressive nutrition strategy, improved effectiveness of antibiotics and mucolytics, as well as the development of Centers specialized assistance.
The average person with cystic fibrosis can experience joy today family life, attends college and pursues a career.
However, there are still problems that need to be solved: 1) bacteria that are difficult to treat. Approximately 80% of patients are colonized with the bmicroorganism Pseudomonas by age 18. Over time, this bacterium becomes increasingly resistant to antibiotics.
2) An important problem is lung diseases. Young people have large reserves, but a quarter of patients vital capacity lungs are more than 40 percent less than expected.
Cystic fibrosis not only destroys the lungs, but also clogs the pancreatic ducts with mucus plugs. As a result, pancreatic enzymes do not enter the digestive tract, and food cannot be absorbed. Problems with the pancreas continue to shorten the lives of patients. 20 to 25% of patients with cystic fibrosis develop diabetes. Average age“debut” of diabetes from 18 to 24 years.
Bone fragility in this disease is noted in utero. About 67% experience bone thinning and osteoporosis. This is due to the fact that pancreatic insufficiency disrupts the processes of fat digestion and absorption. fat-soluble vitamins A, E, K and especially D, which is critical for bone strength. Regular exercise helps strengthen bones, but patients with cystic fibrosis should not exercise because respiratory failure can not.
Family matters
Men with cystic fibrosis have problems having children. More than 95% of men with cystic fibrosis are sterile. In cystic fibrosis, a congenital absence of vas deferens can often be observed - abnormal development of the duct, which ensures the passage of sperm from the testicles.
Fertility in women is also reduced. They can have children, but severe forms Cystic fibrosis often causes problems with conception due to nutritional disorders. Plus, impaired lung function can cause problems with pregnancy. If there are problems with conception, adoptive children or the use of donor sperm may be a solution. For example, intracytoplasmic sperm injection (ICSI) can be used, in which sperm is injected directly into the cytoplasm of mature eggs. However, ICSI costs $10,000 per injection with no guarantee of success.
Psychological picture: the decision to start a family is hindered by the feeling of approaching death. Even if they are able to have children, they will not always have time to “put them on their feet.”
Lung transplantation remains the treatment for despair. This treatment is recommended if lung function drops to 30%. However, only 60% of patients live more than 5 years after transplantation. According to many scientists, lung transplantation does not so much prolong life as improve its quality.
Cystic fibrosis ( cystic fibrosis) – hereditary disease, common among the white race. In European countries, approximately four in ten thousand are at real risk of developing cystic fibrosis.
It is not transmitted by airborne droplets or any other way, it cannot be contracted like influenza or hepatitis, it is a congenital disease. Cystic fibrosis in children can occur if both parents are carriers of the altered gene; if only one such mutant gene is inherited, the child will also be a carrier, but he will not face the disease.
If back in 1969, patients with such a diagnosis lived only up to fourteen years, now cystic fibrosis can no longer be called a death sentence.
It’s easy to find out about this disease in detail; just type “Cystic Fibrosis Presentation” in the search program.
The presenters of Mayak radio, back in October of last year, tried to explain in the most accessible, although not at all correct, form what cystic fibrosis is. “Mayak”, represented by its management and presenters, later apologized; the program was closed under pressure from the indignant public. And it’s true, can any disease be a reason for jokes, especially one like cystic fibrosis, the treatment of which still does not make it possible to live more than 40 years?
What is the essence of the disease
How does cystic fibrosis occur? Reason - gene mutation. The human body contains a membrane protein, the main task of which is to transport chlorine ions through cell membranes in the liver, in the gastrointestinal tract, in the lungs. The changed gene is not able to correctly encode the membrane protein, its work is disrupted and, as a result, the mucus covering the inner surface of the organs becomes more viscous. Approximately one in forty people can be a carrier of such an altered gene, i.e. The phenomenon is quite common; one in 2,500 babies born is diagnosed with cystic fibrosis. Internet forums on this topic are clear confirmation.
What is the danger
Cystic fibrosis in adults and children is undoubtedly a life-threatening disease. Only early diagnosis gives a chance to avoid fatal outcome, because almost the entire body is under threat. A genetic defect leads to the fact that the endocrine glands cannot function normally, and the organs undergo secondary changes, this is the danger posed by cystic fibrosis. Wikipedia talks about severe violations functions of the respiratory and digestive organs and gastrointestinal tract. Bacteria multiply intensively in viscous mucus, hence constant health problems. The blood supply and ventilation of the lungs are impaired, resulting in frequent pulmonary infections and asthma attacks.
The pancreas does not secrete enough enzymes, food is poorly broken down and poorly absorbed by the body. The functions of the liver and gallbladder are impaired, and stagnation of bile is dangerous for the development of cirrhosis and the formation of stones. There is a danger of developing diabetes.
Symptoms
Being hereditary, cystic fibrosis in children makes itself felt already in early age and it only progresses over time. Moreover, it should be noted that the occurrence of the disease does not depend either on the age of the parents, or on the environment, or on bad habits. Stress and medications during pregnancy have no effect on whether children will develop cystic fibrosis; symptoms will only appear if both parents pass on the altered gene.
Changes in the pancreas, in respiratory organs, in the digestive organs can begin already during the period intrauterine development, therefore, cystic fibrosis in newborns can be determined by a number of signs.
This includes jaundice, which develops from the very first days of life, due to the fact that the outflow of bile is obstructed, and difficulty breathing, and lack of appetite. Intestinal obstruction– one of the main indicators that suggests cystic fibrosis in newborns. Symptoms: the child suffers from vomiting and bloating. Restless at first, he becomes lethargic after a few days, his skin becomes dry and pale due to general intoxication of the body.
Cystic fibrosis of the lungs, or rather the bronchopulmonary form, affects the respiratory system. Symptoms – persistent cough, painful, with attacks. In the future - frequent bronchitis, prolonged pneumonia with a very high temperature.
Cystic fibrosis, intestinal form or cystic fibrosis of the pancreas is a dysfunction of all endocrine glands, when the enzymes necessary for digesting food are not produced, nutrients almost do not break down and are not absorbed, i.e. the body is actually starving. Symptoms: bloating and abdominal pain, lethargy, lack of vitamins, and a little later - malnutrition, lack of weight.
The most common form is the pulmonary intestinal form; in 80 cases this type of cystic fibrosis is diagnosed. The symptoms in this case are mixed, but the symptom common to all forms is that the child’s skin tastes salty
Can it be cured?
If at least some of the above symptoms are present and there is a suspicion of cystic fibrosis, a sweat test is simply necessary. The level of chlorides is determined, usually elevated in patients. Only genetic analysis can definitively confirm the diagnosis.
Modern medicine is not yet able to completely cure cystic fibrosis. Help lies in minimizing the symptoms of the disease with drugs to dilute mucus and bile, giving the body the necessary enzymes that it cannot produce itself, vitamins and microelements, and protecting as much as possible from infections. These are pancreatic enzymes, hepatoprotectors, bronchodilators and antibiotics - vital for the diagnosis of cystic fibrosis. Articles about new opportunities to combat this disease give hope that research into the causes of its occurrence at the molecular level will help create an effective and safe medicine.
International symposiums and conferences that are held every year, as well as the combined efforts of doctors and scientists, undoubtedly bear fruit in solving the problems of cystic fibrosis. News recent years in this area, it can be noted that the disease has ceased to be a death sentence; patients have the opportunity to live not just longer, but to live fully, work and study in the same way as healthy people.
