What is Cystic Fibrosis – CF?

Cystic fibrosis is a disease, which affects one in 2500 newborns in most European populations. This practically means that four out of 10,000 children born annually will have cystic fibrosis. Cystic fibrosis (CF) is an inherited disease that affects boys and girls. Children are born with cystic fibrosis and the disease is not acquired throughout life. Cystic fibrosis is not a contagious disease and cannot be passed from one child to another. Since a child already has cystic fibrosis at birth, this disease is referred to as congenital or hereditary. Cystic fibrosis affects children who have two CF genes, one inherited from each parent. People who have only one copy of the CF gene are “carriers of cystic fibrosis” and are completely healthy. A child with cystic fibrosis is born only if both parents carry the “CF gene”. Symptoms and severity of the disease vary significantly in different patients with cystic fibrosis. Cystic fibrosis attacks several organs, but causes the most problems in: lungs, intestines, liver and pancreas. Some sufferers have more problems with the lungs, and others more with the digestive organs; every patient is unique in this regard. Cystic fibrosis does not affect a child’s intelligence. For now, there is still no cure for cystic fibrosis.
Mucus (sputum) that is produced in the lungs of a healthy person is much rarer compared to the sputum of cystic fibrosis patients, which is thick and sticky. That thick mucus sticks to the lungs and clogs the airways. If the mucus stays in the lungs, it clogs the small airways and creates a suitable environment for the growth of microorganisms (bacterial infections). For this reason, it is very important to clear the airways with physical therapy.

WHAT HAPPENS IN THE PANCREAS The pancreas plays a very important role in the process of digesting food. It produces ingredients called enzymes, which help break down the food we eat. When food is broken down, the intestines can absorb it. In people with cystic fibrosis, the pancreas is clogged with thick juices, so the digestive enzymes can’t get the food in properly. Undigested food cannot be absorbed by the intestines, so the nutrients are excreted in the stool. The pancreas therefore helps to break down food, which is necessary for the growth and general health of the body. In cystic fibrosis, the pancreas cannot perform its role properly. If children with cystic fibrosis are not treated properly, their stools are abundant and have a specific smell of undigested food. The stool contains droplets of oil, floats in water and is difficult to wash off the diaper. The color of the stool is usually lighter than normal stool. A child with cystic fibrosis may have diarrhea or loose stools, abdominal bloating and abdominal pain. It is a consequence of a large amount of undigested food in the intestines. If constipation occurs, bowel obstruction may also occur.

WHEN SHOULD YOU SUSPECT CYSTIC FIBROSIS?
The first signs of cystic fibrosis can appear at any time, but are most often noticed at the age of two years. You may suspect cystic fibrosis if you notice any of these symptoms:
• frequent cough, production of thick sputum
• frequent lung infections that resemble pneumonia
• lack of progress or weight loss, despite a normal or even increased appetite
• stomach problems
• intestinal obstruction in the newborn
Another important characteristic of children with cystic fibrosis is that their sweat is very salty. Every person’s sweat is salty, but the sweat of a child with cystic fibrosis is very salty. This is most often noticed by parents when they kiss a child or see salt crystals on the child’s skin. The test used to confirm whether a child has cystic fibrosis is a sweat test and it detects excess salt in the sweat.

HOW IS CYSTIC FIBROSIS INHERITED FROM PARENTS?
Each person inherits his appearance from his parents and grandparents, i.e. ancestors. Eye or hair color, height and many other physical characteristics are characteristic of each individual. Sometimes, in addition to other traits, we can also inherit a disease, as is the case with cystic fibrosis. A child will have cystic fibrosis if he inherits two copies of the “diseased” gene – one from each parent. This form of inheritance is called “autosomal recessive”. The parents of a child with cystic fibrosis are normal, healthy people, although they each carry one copy of the normal gene and one copy of the CF gene, where they can pass the CF gene on to their offspring.
• Both parents must have one copy of the CF gene for any of their children to have cystic fibrosis
• A child will only have cystic fibrosis if they inherit two CF genes, one from each parent
• A child will not have cystic fibrosis if they inherit a CF gene from one parent and a normal gene from the other. In that case, the child is a “carrier” of cystic fibrosis, as are his parents.

In European populations, approximately 1 in 25 people carry the CF gene. As a result, the incidence of cystic fibrosis is, on average, 1 in 2500 newborns. The exact frequency varies between different populations in Europe. Today, there is the possibility of molecular testing that can detect the most common changes in the CF gene that lead to cystic fibrosis. In this way, the majority of CF gene carriers can be identified. The test can be performed on a small sample of blood, or a swab of the buccal mucosa (the sample is taken with a brush from the inside of the cheek). A genetic test can also be performed with the aim of establishing a prenatal diagnosis (on a sample of amniotic fluid or chorionic villi). The correct interpretation of the results depends on whether the man who was tested is the actual biological father of the baby who is about to be born. In our country, neonatal screening is done – blood is taken from the baby’s heel in the first 6 weeks after birth, and thus the presence of Cf is detected gene. These analyzes are done at the Institute for the Treatment of Children and Youth in Novi Sad.

HAVING A CHILD WITH CYSTIC FIBROSIS… AND ACCEPTING THAT FACT
Having a child with cystic fibrosis is no one’s fault. It is difficult and painful, but no one is to blame. Neither you nor your child or family should feel any guilt or shame about the fact that the child has CF. It is quite common to feel anger and frustration, and it is not easy to overcome these feelings and learn to live with the fact that a child has cystic fibrosis. Everyone carries some of the abnormal genes, but the parents of a child with cystic fibrosis had the misfortune of both carrying the CF gene. When the diagnosis of cystic fibrosis is made, it is very important to accept the fact that your child has cystic fibrosis. There is nothing you can do to change that fact. The sooner you and your child learn to live with cystic fibrosis, the sooner you will learn to cope with the new situation and the less time you will lose before effective treatment can be started.
Because cystic fibrosis was discovered relatively recently, most people, including some doctors, know quite a bit about the disease. When cystic fibrosis was discovered in 1930, very little was known about this disease and only a few drugs were available for its treatment. Today, much more is known about CF, a large number of drugs are used, especially enzymes and antibiotics, which help patients live longer and improve their quality of life. In some cases, parents may be aware for some time that something is wrong with their child, but they are unable to determine the real cause. Before cystic fibrosis is diagnosed, the child may be examined by several doctors and a large number of analyzes may be performed. In that case, it is quite natural that you are worried and distrustful of doctors, nurses and other medical personnel. You should never forget that diagnosing CF is not always easy. When parents are first told that their child has cystic fibrosis, they usually know very little about the disease itself. When the doctor explains that it is a chronic disease that cannot be cured, the parents may be in shock. Sometimes it takes a long time to accept the fact that your child has CF. At the first moment, you can simply not believe the doctor and think the following: Is it really true? How is it possible that my child has this disease? Is it possible that he/she may have another disease that was misdiagnosed as CF, and that is curable? This is often what parents think at the time of diagnosis, or even later. To doubt is human, but not one important thing
should be lost sight of :
If your child has some or all of the symptoms of cystic fibrosis, a positive sweat test, and if the doctor has convinced you that it is cystic fibrosis, it is important to face those facts.
It’s normal to have a certain amount of doubt, but that shouldn’t stop you from facing reality. Today, it is possible, in most cases, to confirm the diagnosis through a genetic test. You can spend a lot of time, money and energy looking for a different diagnosis. This will of course delay the start of treatment, and it is much better to spend time helping your child to start the treatment program as soon as possible. Once the diagnosis is made, it is better to start the treatment as soon as possible.

TREATMENT OF CYSTIC FIBROSIS
Cystic fibrosis is a chronic condition, which the child will have for the rest of his life. It must be treated in the right way, in order for your child to survive and live as close to a normal lifestyle as possible.
Treatment of CF requires the following therapies:
PHYSICAL THERAPY OF RESPIRATORY ORGANS -physical therapy should be carried out regularly throughout life
Physical therapy will be prescribed by your doctor and consists of a series of procedures and/or exercises. The purpose of this therapy is to clear the airways, which are full of thick mucus, and it should be started simultaneously with the diagnosis of CF. In general, it is important to carry out physical therapy:
• in the morning, as soon as the child wakes up, before breakfast
• after school, before going to sleep
The type of physical therapy will change depending on what the doctor or physiotherapist advises. The duration of physical therapy will depend on the condition of your child’s lungs. It is very important that you precisely follow and apply the instructions of your doctor or physiotherapist. If you have not mastered the technique of physical therapy, you should not hesitate to ask the doctor or physiotherapist to show it to you again. It is very important that physical therapy becomes part of the daily routine for your child and that you try to make it fun for him by turning it into a kind of game. Children very often perceive physical therapy as a nuisance and try to avoid it in various ways. You must have a firm position on this issue from the very beginning, and you should not allow the child to dictate the conditions and manipulate you. This can happen at the very beginning of treatment, when your child is still sensitive and weak. You may feel sorry for the child and you will want to fulfill his wishes. Physical therapy is difficult, because it requires a high degree of discipline from both you and your child. Don’t forget that it is in the interest of your child, so don’t give up without a good reason, because it can become a habit.
Coughing in CF is very good and important because it helps to clear the lungs
You should encourage your child to cough, since he was a child, he “threw up mucus”. At school or in the presence of unknown people, your child may feel uncomfortable and will try to stifle a cough. This will cause the mucus to stay longer in the lungs, thus creating a greater chance of infection. Never let your child feel embarrassed because they cough during physical therapy or at any time of the day. A cough in a healthy child can be a sign of the beginning of a cold, but in your child it is crucial for clearing the lungs.
SPORT sports and physical exercises are very important
You can start only with physical therapy, but if your child is in the mood and able to play sports, sports can help him to cough and thus expel phlegm. If the child exercises regularly, he becomes stronger and begins to breathe better. The child should be encouraged to exercise, but not insisted on. Never prevent your child from doing physical exercises because they are very useful. Which type of sport is best for your child? Of course, that depends on your child and the local opportunities for playing sports. Any sport is good as long as the child enjoys it. Active sports in which the child moves a lot, uses his hands and breathes deeply are especially recommended. Good examples are: football, volleyball, swimming and running.
ANTIBIOTICS-antibiotics are very important medicines that help your child survive and feel well
Antibiotics kill the bacteria that get into your child’s lungs and have helped greatly to prolong the lives of children with cystic fibrosis around the world. Sometimes your child will need to take antibiotics often or for quite a long time. Antibiotics are usually swallowed, but if it is necessary to use a stronger antibiotic, then it will have to be given intravenously. In that case, the child will have to receive antibiotics in the hospital. Inhaled antibiotics are starting to be used in some countries. Sometimes parents are worried, and fear that strong antibiotics may be harmful to the child. However, you should not be afraid: many studies have shown that the effect of antibiotics in children with CF is almost always very beneficial. The bacteria that infect the lungs of CF patients always try to “outsmart” antibiotics, so in the fight against them, they constantly have to
develop and apply new antibiotics. For this very reason, “usual” antibiotics are not the best choice, but those that sound “unknown”.
VACCINATION Vaccinations are very important in order to prevent infections
Vaccinations will help protect your child from many dangerous diseases. You must vaccinate your child against smallpox (measles) because they are particularly dangerous for the lungs of a child with cystic fibrosis. Vaccination must also be regular against seasonal flu, because any additional infection caused by viruses or bacteria further worsens the condition of people with cystic fibrosis, as well as prolonged use of antibiotics. Also , there are specialized vaccines against the biggest “enemy” of the lungs of people with Cf. These are vaccines against streptococcal pneumonia, and they can only be given to a child on the recommendation of a pulmonologist and immunologist.
FOOD AND ENZYMES Replenishment of pancreatic enzymes is very important because it helps your child digest the food they eat
Pancreatic enzymes will help your child digest food, gain weight and grow normally. The doctor will instruct you how the child should take enzymes, but there are some general rules:
• enzymes should be taken before, or on the advice of a doctor, during meals
• enzymes should not be taken after a meal
• enzymes should not be chewed; if necessary, open the capsule and give the child to swallow the granules. You should encourage the child to learn how to swallow the whole enzyme capsule from an early age. It should be started when children turn 4 or 5 years old. Learning to swallow a capsule can be started as a game by first encouraging the child to push through the boiled bean.
rice, lentils or peas, with a sip of your favorite drink. Then you should gradually move on to something the size of a cooked bean. Do not make a big deal out of swallowing, because then your child will get the impression that it is a very difficult undertaking. When a child swallows an enzyme capsule for the first time, he should be rewarded. If your child swallows normally, he will not choke when he swallows the capsule. Do not forget that if you are uncertain or afraid about it, your child will also be afraid and uncertain. The child should not take food, except for juice, water and fruit, and you have not given him the enzyme before. If you forget to take the enzyme with a meal or snack, the food will not be digested and the nutrients will not be absorbed. Always encourage your child to take enzymes regularly before meals or snacks. just to take enzymes. Inform the teacher and other authorities in the school, so that they occasionally remind the child not to forget to take the enzyme tablet.

TREATMENT OF CYSTIC FIBROSIS IS NOT EASY
But all parents learn how to relate to a pre-teen with CF over time
Although it is difficult when you first start treatment, it becomes routine as you learn and practice more. Treatment for CF is lifelong. Sometimes co-parents are suspicious about treatment and wonder if they should look for treatment elsewhere? Several centers in the world are working on finding a cure for CF, but a cure has not been found to date. There is a possibility that in the future gene therapy or new treatment methods will be helpful. Some parents do not believe in the diagnosis of CF in their child, and give up the prescribed therapy. There is no miraculous alternative, stopping treatment. It is something a person is born with and cannot change. That’s it
found in “genes”. You must remember that if your child feels well at the moment, it is the result of the treatment and care that is being carried out. Physical therapy and enzymes should never be interrupted, even if the child looks healthy. If your child looks weak or sick, especially if the condition worsens, you will have to visit the teledoctor more often. If the treatment is correct, your child will need to be examined to see if it is a new lung infection, or if there is another cause. Additional treatment may include admission to the hospital due to intravenous administration of antibiotics. If the child is unable to perform physical therapy or does not take enzymes, it is necessary to immediately take him to the hospital.

HOSPITAL TREATMENT
One of the most common questions parents ask is whether their child can get a more serious disease than cystic fibrosis during their stay in the hospital. Admission to the hospital usually means that it is necessary to use special antibiotics to fight a lung infection. The risk of your child not going to the hospital is far greater than the risk of getting a serious illness during the hospital stay.
Treatment of cystic fibrosis is very expensive when you consider the cost of antibiotics and hospital stay. In some European countries, treatment costs are covered by health insurance; in countries where this is not the case, you should contact the local state/health institutions. Of course, the request has much more weight if it is submitted by an association to help patients than by individual parents.
It is up to you to decide to whom and when to say yes to your children’s CF. Later, your child will decide for himself. Some people like to talk to others about their own problems and that attitude should be respected. However, you should keep in mind that sometimes it is easier to talk to someone about your problems, especially when your children have a chronic illness. Some families also find comfort in faith and prayer.
It is important to inform the siblings of a child with cystic fibrosis
Explain to them the importance of daily physical therapy, taking vitamins and going to the hospital. Help them understand the situation by answering their questions in the best possible way. Don’t forget that they also need your love and attention. Confide in your closest relatives and friends and explain to them what CF is. Tozaista helps. When your child looks healthy, they should understand that this is the result of proper treatment, without which the child would soon be sick again.
You have to be strong to help a child fight CF

The child may find it difficult to tolerate treatment, and you may be tempted to take a break from treatment. Remember that it is in your child’s best interest not to stop or reduce physical therapy. Also, remember that except for CF, your child is normal in every other way. A child with CF is expected to go through all the physical and psychotic experiences, like other healthy children. Your child will get a cold, a sore throat, get injured in play or sports, just like other children, except that he has CF. As the child grows up, you should encourage him to become independent and take responsibility for his own treatment. This should happen gradually, and the burden of treatment should never fall solely on your child. The child will not be able to carry out the treatment completely independently, especially while he is still small. At the age of eighteen or nineteen, he will be more independent, but it is unrealistic to expect a child or adolescent to behave responsibly like an adult. The gradual transfer of responsibility for treatment should be very carefully monitored. It is better for your child to encourage him to accept responsibility than to force him to do so and impose it on him as an obligation. This will allow you to work together and make sure that your child is doing the treatment in the right way. Neither you, nor your child, nor anyone in the family should be ashamed of having cystic fibrosis. If the child feels ashamed, he will try to He hid the fact that he had CF from his friends. There is a possibility that he will stop taking enzymes or refrain from coughing in public Your child should feel loved like any other child, he fully deserves it.

WHAT IS CYSTIC FIBROSIS – CF?

Cystic fibrosis is a disease that affects one in 2500 newborns in most European populations. This practically means that out of 10,000 children born annually, four will be with cystic fibrosis.
Cystic fibrosis (CF) is a hereditary disease that affects both boys and girls
little girls. Children are born with cystic fibrosis and the disease is not acquired during life.
Cystic fibrosis is not a contagious disease and cannot be passed from one child to another. Since the child already has cystic fibrosis at birth, this disease is designated as congenital or hereditary.
Cystic fibrosis affects children who have two CF genes, one inherited from each parent. People who have only one copy of the CF gene are “cystic fibrosis carriers” and are completely healthy.

A child with cystic fibrosis is born only if both parents carry the CF gene.
Symptoms and severity of the disease vary significantly in different patients with cystic fibrosis. Cystic fibrosis attacks several organs, but causes the most problems in: lungs, intestines, liver and pancreas. Some sufferers have more problems with the lungs, and others more with the digestive organs; every patient is unique in this regard.,Cystic fibrosis does not affect the child’s intelligence. For now, there is still no cure for cystic fibrosis.

WHAT HAPPENS IN THE LUNGS

Mucus (sputum) that is produced in the lungs of a healthy person is much rarer compared to the sputum of cystic fibrosis patients, which is thick and sticky. That thick mucus sticks to the lungs and clogs the airways.
If the mucus stays in the lungs, it clogs the small airways and creates a suitable environment for the growth of microorganisms (bacterial infections). For this reason, it is very important to clear the airways with physical therapy.