What is cystic fibrosis?


Know the basics

What is cystic fibrosis?

Cystic fibrosis (CF) is a lifelong illness affecting cells that make sweat and mucus. Mucus is a slippery, somewhat sticky fluid that lubricates and protects musous membranes. CF mucus is abnormally thick and sticky, it clogs the lungs and causes frequent lung infection.

CF also affects the pancreas, which has trouble making special chemicals called enzymes to digest food. Without enzymes, nutrients can’t be absorbed from food.

How common is cystic fibrosis?

CF is common genetic disease in Northern European. People with CF have a shorter life span, but with modern treatments, more people with CF live to middle age or older.

Know the symptoms

What are the symptoms of cystic fibrosis?

Cystic fibrosis signs and symptoms vary, depending on the severity of the disease.

There may be some signs or symptoms not listed above. If you have any concerns about a symptom, please consult your doctor. People with cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children.

Other symptoms may include:

Respiratory signs and symptoms

eople who have CF have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick sputum (spit) or mucus that’s sometimes bloody.

People who have CF also tend to have bouts of sinusitis, pneumonia and lung infection doesn’t respond to standard antibiotics. As CF gets worse, you may have more serious problems, such as pneumothorax or bronchiectasis

Digestive signs and symptoms

In CF, mucus can block tubes, or ducts, in your pancreas (an organ in your abdomen). These blockages prevent enzymes from reaching your intestines.

As a result, your intestines can’t fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockages also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort.

As CF gets worse, other problems may occur, such as:

  • Pancreatitis. This is a condition in which the pancreas become inflamed, which causes pain.
  • Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum.
  • Liver disease due to inflamed or blocked bile ducts.
  • Diabetes.
  • Gallstones.

When should I see my doctor?

If you or your child has symptoms of cystic fibrosis — or if someone in your family has cystic fibrosis — talk with your doctor about testing for the disease. Seek immediate medical care if you or your child has difficulty breathing. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Know the causes

What causes cystic fibrosis?

A defect in the CFTR gene causes cystic fibrosis (CF). This gene makes a protein that controls the movement of salt and water in and out of your body’s cells. In people who have CF, the gene makes a protein that doesn’t work well. This causes thick, sticky mucus and very salty sweat.

Children need to inherit one copy of the gene from each parent in order to have the disease. If children inherit only one copy, they won’t develop cystic fibrosis, but will be carriers and possibly pass the gene to their own children.

Know the risk factors

What increases my risk for cystic fibrosis?

Risk factors for CF include:

  • Family history. Because cystic fibrosis is an inherited disorder, it runs in families.
  • Race. Although cystic fibrosis occurs in all races, it is most common in white people of Northern European ancestry

Understand the diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is cystic fibrosis diagnosed?

Doctors diagnose cystic fibrosis (CF) based on the results from various tests include:

  • Sweat testA sweat-producing chemical is applied to a small area of skin. The collected sweat is then tested to see if it’s saltier than norma
  • Genetic testing. DNA samples from blood can be checked for specific defects on the gene responsible for cystic fibrosis.
  • Test on the bowel movements (stools) and blood to check effects on the pancreas.
  • Chest X-rays or do breathing tests to check the lungs.
  • CF can also be diagnosed with prenatal (before birth) testing.

How is cystic fibrosis treated?

There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications.

  • Medicines help thin the mucus and prevent lungs from clogging.
  • Antibotics are also often given for infection.
  • Enzyme supplements supply missing pancreatic enzymes. A special high-protein, low-fat diet may also improve nutrition.
  • Raspiratory therapy can help remove mucus from lungs by tapping on the chest ( chest percussion), lying with the head lower than the feet.
  • Nasal polyp removal.Your doctor may recommend surgery to remove nasal polyps that obstruct breathing.
  • If you have severe breathing problems, life-threatening lung complications or increasing resistance to antibiotics used to treat lung infections, lung transplantation may be an option.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage cystic fibrosis?

Epididymitis usually involves considerable pain. To ease your discomfort:

  • Follow your doctor’s instructions and give medicines and treatments as directed. Take your child to the doctor at least three or four times yearly.
  • Avoid contact with people with respiratory infections
  • Get your child a yearly flu shot.
  • Have your child drink plenty of fluids
  • Have your child avoid fumes and smoke. These things irritate lungs and make CF worse.
  • Exercise regularly.
  • Use enzyme supplements. A special high-protein, low-fat diet may also improve nutrition.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

msBahasa Malaysia

Review Date: May 30, 2016 | Last Modified: January 4, 2017

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