What Is Cystic Fibrosis (CF)?
In cystic fibrosis (CF) give the mucous abnormally thick and sticky mucus. Mucous membranes are in the lungs, the pancreas and the liver. The tough mucus causes there clogging. The organs go thereby getting worse work.
With CF you will be born. The tough mucus is caused by an error in the DNA (the genetic material). By a broken Gene makes the body a specific protein. As a result, the mucus thick. The disease is hereditary. The way of inheritance is autosomal recessive.
CF is unfortunately incurable. The treatment includes a diet, training to keep fit, physical therapy and medications to the mucus thinner and medicine for infections. People with CF don't live as long: the average life expectancy is 35 to 40 years.
For the medical and psychosocial guidance in CF there are CF special centres. These centres work in medical specialists, such as a pediatrician and a lung doctor, together. In addition, at home the family doctor, home care or social services a great help.
What Are The Symptoms Of Cystic Fibrosis (CF Disease)?
Symptoms of cystic fibrosis (CF disease) are:
- persistent cough and phlegm;
- common respiratory infections, such as pneumonia;
- breathlessness;
- smelly, greasy stools;
- falling off;
- abdominal cramps and bloating;
- sweat with a lot of salt in it;
- not feel like eating (especially during a respiratory infection);
- growth retardation.
In a number of babies with CF is blocked intestine (meconium ileus). They can not sell their first stool. Usually, it is then necessary surgery.
Some people with CF get diabetes mellitus. That is because the pancreas is not working properly.
In people with CF updates the lung eventually becoming less well. This is due to the continued lung inflammation. Sometimes you need extra oxygen, or a lung transplant.
Cystic Fibrosis Is Hereditary?
Cystic fibrosis (CF) is a hereditary disease. The disorder is inherited recessive. This means that both parents are carriers of the cystic fibrosis gene have been. As a carrier, you have no symptoms, but can still pass the illness.
If you both are carriers of the CF gene, what chance does your child than on CF?
- your child has a 25% to have CF;
- your child has a 50% chance to be carriers;
- your child has a 25% chance to be a carrier and also to have CF.
Whether you are a carrier know you almost certainly after carrier testing. This happens when CF carrier testing in your family. The research is the clinical genetics department of a university hospital.
If you are not a carrier of the research? Then there is a very small chance that you are a carrier. You then have a cystic fibrosis gene that is not yet known, or may be not yet been investigated.
How Does The Doctor Determines That Your Child Has Cystic Fibrosis?
The doctor suggests cystic fibrosis to the following ways:
- Sweat Test. Children with cystic fibrosis (CF) have strikingly lot of salt in their sweat. A sweat test, the physician measures the salinity. The doctor can test babies from the age of six weeks.
- DNA research . Doubts the doctor on the results of the sweat test, it will do a DNA test. It should examine the blood and see if the child has the genetic characteristics of cystic fibrosis (CF).
- Examination of the mucous membrane of nose or rectum. In this test, the doctor inserts a thin tube in the nose or in the rectum. The study takes a few minutes. It does not hurt.
What Is The Treatment For Cystic Fibrosis?
The treatment for cystic fibrosis (CF) is comprised of three parts:
- Respiratory infections prevention and treatment.
This is done with medications and physical therapy.
The physiotherapist learns to pick up your child in a special way breathing. This keeps the lungs of your child clean longer.
In addition, your child must breathe mucus-thinning medications. Spraying is called.
Respiratory infections treats the physician with a high dose of antibiotics. In older children and adults, it is not always possible to treat the infections with antibiotics pills. The treatment then takes place in the hospital. Sometimes home treatment systems for portable infusion pumps.
- Endurance, such as fitness, running and cycling. You or your child is training with a physiotherapist. Why is exercise important?
- Training improves the resistance.
- Training ensures good condition of organs and muscles.
- Training helps prevent respiratory infections.
- Training makes it easier to cough up phlegm.
- A diet.
In people with cystic fibrosis fat digestion is not good. This is because the pancreas is not working properly and by many antibiotics. The diet is enriched energy (sometimes up to more than 150% of the normal power supply). Also, you or your child swallow at all meals digestive enzymes and vitamins.
If these treatments do not help, a lung transplant may be an option. Not everyone qualifies for such a transplant. And there is a waiting list due to lack of donor lungs.
Fertility And Pregnancy In Cystic Fibrosis
Women with cystic fibrosis (CF) often have fertility problems because of their disease. They take much longer to get pregnant. Half of women with cystic fibrosis and have children eventually become pregnant.
Almost all men with CF are infertile. Tough mucus namely also damage the gonads.
If pregnancy does not 'automatically' fails, consider fertility treatment. You can discuss this with your doctor. One factor is that children of parents with CF has a chance to be self carrier or also to get the disease. Also, the child is at risk at an early age to lose his parent to this disease.