Cystic Fibrosis
Your Personalized Treatment Plan
Do not limit your treatment options only to the nearest hospital.
We offer the most effective, quick and affordable treatment for cystic fibrosis,
thanks to the world’s largest hospital network.
Years
0
Countries
0
Hospitals
0
Doctors
0
Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.
Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with CF, a defective gene causes the secretions to become sticky and thick. Instead of acting as lubricants, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.
Symptoms usually start in early childhood and vary from child to child, but the condition gets slowly worse over time, with the lungs and digestive system becoming increasingly damaged.
Treatments are available to help reduce the problems caused by the condition and make it easier to live with, but sadly life expectancy is shortened.
Complete Recovery
No Hidden Fees
No Waiting Lists
Leading Surgeons
Private treatment in our hospital network allows you skip waiting lists while at the same time reducing the costs treatment considerably, up to 50% cheaper, in countries that are just a short flight from home. The countries listed below make excellent choices with modern hospitals and well-trained English speaking surgeons.
Our medical consultants will advise you on the best choice for treating your cystic fibrosis in one of our leading hospitals. We will evaluate your current condition, expectations and other related factors, and offer you the most suitable option, respecting your privacy and the individual needs of your body and lifestyle. Your health is unique and so should be your solution.
- Berlin
- Zurich
- Vienna
- Paris
- Madrid
- Milan
- Warsaw
- Istanbul
Treatment of Cystic Fibrosis
There is no cure for cystic fibrosis, but treatment can ease symptoms, reduce complications and improve quality of life. Close monitoring and early, aggressive intervention is recommended to slow the progression of CF, which can lead to a longer life.
Managing cystic fibrosis is complex, so consider getting treatment at a center with a multispecialty team of doctors and medical professionals trained in CF to evaluate and treat your condition.
People with cystic fibrosis may need to take different medicines to treat and prevent lung problems.
Physical activity and the use of airway clearance techniques may also be recommended to help clear mucus from the lungs.
Medications
Options include:
- Medications that target gene mutations, including a new medication that combines three drugs to treat the most common genetic mutation causing CF and is considered a major achievement in treatment
- Antibiotics to treat and prevent lung infections
- Anti-inflammatory medications to lessen swelling in the airways in your lungs
- Mucus-thinning drugs, such as hypertonic saline, to help you cough up the mucus, which can improve lung function
- Inhaled medications called bronchodilators that can help keep your airways open by relaxing the muscles around your bronchial tubes
- Oral pancreatic enzymes to help your digestive tract absorb nutrients
- Stool softeners to prevent constipation or bowel obstruction
- Acid-reducing medications to help pancreatic enzymes work better
- Specific drugs for diabetes or liver disease, when appropriate
Medications that target genes
For those with cystic fibrosis who have certain gene mutations, doctors may recommend cystic fibrosis transmembrane conductance regulator (CFTR) modulators. These newer medications help improve the function of the faulty CFTR protein. They may improve lung function and weight, and reduce the amount of salt in sweat.
Doctors may conduct liver function tests and eye exams before prescribing these medications. While taking these drugs, testing on a regular basis is needed to check for side effects such as liver function abnormalities and cataracts. Ask your doctor and pharmacist for information on possible side effects and what to watch for. Keep regular follow-up appointments so your doctor can monitor you while taking these medications. Talk to your doctor about any side effects that you experience.
Airway clearance techniques
Airway clearance techniques — also called chest physical therapy (CPT) — can relieve mucus obstruction and help to reduce infection and inflammation in the airways. These techniques loosen the thick mucus in the lungs, making it easier to cough up.
Airway clearing techniques are usually done several times a day. Different types of CPT can be used to loosen and remove mucus, and a combination of techniques may be recommended.
- A common technique is clapping with cupped hands on the front and back of the chest.
- Certain breathing and coughing techniques also may be used to help loosen the mucus.
- Mechanical devices can help loosen lung mucus. Devices include a tube that you blow into and a machine that pulses air into the lungs (vibrating vest). Vigorous exercise also may be used to clear mucus.
Your doctor will instruct you on the type and frequency of chest physical therapy that’s best for you.
Pulmonary rehabilitation
Your doctor may recommend a long-term program that may improve your lung function and overall well-being. Pulmonary rehabilitation is usually done on an outpatient basis and may include:
- Physical exercise that may improve your condition
- Breathing techniques that may help loosen mucus and improve breathing
- Nutritional counseling
- Counseling and support
- Education about your condition
Surgical and other procedures
Options for certain conditions caused by cystic fibrosis include:
- Nasal and sinus surgery. Your doctor may recommend surgery to remove nasal polyps that obstruct breathing. Sinus surgery may be done to treat recurrent or chronic sinusitis.
- Oxygen therapy. If your blood oxygen level declines, your doctor may recommend that you breathe pure oxygen to prevent high blood pressure in the lungs (pulmonary hypertension).
- Noninvasive ventilation. Typically used while sleeping, noninvasive ventilation uses a nose or mouth mask to provide positive pressure in the airway and lungs when you breathe in. It’s often used in combination with oxygen therapy. Noninvasive ventilation can increase air exchange in the lungs and decrease the work of breathing. The treatment may also help with airway clearance.
- Feeding tube. Cystic fibrosis interferes with digestion, so you can’t absorb nutrients from food very well. Your doctor may suggest using a feeding tube to deliver extra nutrition. This tube may be a temporary tube inserted into your nose and guided to your stomach, or the tube may be surgically implanted in the abdomen. The tube can be used to give extra calories during the day or night and does not prevent eating by mouth.
- Bowel surgery. If a blockage develops in your bowel, you may need surgery to remove it. Intussusception, where a segment of intestine has telescoped inside an adjacent section of intestine, also may require surgical repair.
-
Lung transplant. If you have severe breathing problems, life-threatening lung complications or increasing resistance to antibiotics for lung infections, lung transplantation may be an option. Because bacteria line the airways in diseases that cause permanent widening of the large airways (bronchiectasis), such as cystic fibrosis, both lungs need to be replaced.
Cystic fibrosis does not recur in transplanted lungs. However, other complications associated with CF — such as sinus infections, diabetes, pancreas conditions and osteoporosis — can still occur after a lung transplant.
- Liver transplant. For severe cystic fibrosis-related liver disease, such as cirrhosis, liver transplant may be an option. In some people, a liver transplant may be combined with lung or pancreas transplants.
Complications of Cystic Fibrosis
Complications
Types
Causes
Symptoms
Diagnosis
Complications
The build-up of sticky mucus in the lungs can cause breathing problems and increases the risk of lung infections. Over time, the lungs may stop working properly.
Mucus also clogs the pancreas (the organ that helps with digestion), which stops enzymes reaching food in the gut and helping with digestion.
This means most people with cystic fibrosis don’t absorb nutrients from food properly and need to eat more calories to avoid malnutrition.
People with cystic fibrosis also have a higher risk of developing other conditions.
These include:
- weak and brittle bones (osteoporosis) – medicines called bisphosphonates can sometimes help
- diabetes – insulin and a special diet may be needed to control blood sugar levels
- nasal polyps and sinus infections – steroids, antihistamines, antibiotics or sinus flushes can help
- liver problems
- fertility problems – it’s possible for women with cystic fibrosis to have children, but men won’t be able to father a child without help from fertility specialists
Types
Cystic fibrosis is caused by mutations, or errors, in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which result in either no CFTR protein being made or a malformed CFTR protein that can’t perform its key function in the cell.
Over the years, scientists have used several different ways of grouping these mutations into different classes. The most recent classification system groups mutations by the problems that they cause in the production of the CFTR protein:
- Protein production mutations (Class 1)
- Protein processing mutations (Class 2)
- Gating mutations (Class 3)
- Conduction mutations (Class 4)
- Insufficient protein mutations (Class 5)
Causes
Cystic fibrosis is a genetic condition. It’s caused by a faulty gene that affects the movement of salt and water in and out of cells.
This, along with recurrent infections, can result in a build-up of thick, sticky mucus in the body’s tubes and passageways – particularly the lungs and digestive system.
A person with cystic fibrosis is born with the condition. It’s not possible to “catch” cystic fibrosis from someone else who has it.
Symptoms
Symptoms of cystic fibrosis include:
- recurring chest infections
- wheezing, coughing, shortness of breath and damage to the airways (bronchiectasis)
- difficulty putting on weight and growing
- yellowing of the skin and the whites of the eyes (jaundice)
- diarrhoea, constipation, or large, smelly poo
- a bowel obstruction in newborn babies (meconium ileus) – surgery may be needed
People with the condition can also develop a number of related conditions, including diabetes, thin, weakened bones (osteoporosis), infertility in males, and liver problems.
Diagnosis
The newborn blood spot test (heel prick test) is usually carried out shortly after a child is born.
If the screening test suggests a child may have cystic fibrosis, they’ll need these additional tests to confirm they have the condition:
- a sweat test – to measure the amount of salt in sweat, which will be abnormally high in someone with cystic fibrosis
- a genetic test – where a sample of blood or saliva is checked for the faulty gene that causes cystic fibrosis
These tests can also be used to diagnose cystic fibrosis in older children and adults who didn’t have the newborn test.
The genetic test can also be used to see whether someone is a “carrier” of cystic fibrosis in cases where the condition runs in the family.
This test can be important for someone who thinks they may have the faulty gene and wishes to have children.
