Cystic Fibrosis: Symptoms, Causes, Diagnosis & Treatment

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Cystic Fibrosis: Symptoms, Causes, Diagnosis & Treatment

Cystic fibrosis (CF) is an inherited/genetic disorder that affects mostly the lungs along with pancreas, liver, kidneys, and intestine. CF causes changes in the electrolyte transport system causing cells to absorb too much sodium and water.

It affects the cells that produce mucus, sweat and digestive juices which are normally thin and slippery. But in people with cystic fibrosis, a defective gene causes the secretions to become sticky and thick. These secretions then plug up tubes, ducts, and passageways, especially in the lungs and pancreas.

One usually experience weight gain, poor growth, salty sweat, continuous cough and wheezing as the first signs of cystic fibrosis. There is no permanent cure for Cystic fibrosis. Treatment only eases the symptoms, prevents and treats complications, and slows the progress of the disease.

The average life expectancy is between 42 and 50 years and is responsible for death in 80% of people with cystic fibrosis. CF is the most common among people of Northern European ancestry and affects about one out of every 3,000 newborns. It is estimated that one in 25 people is a carrier. Cystic fibrosis is least common in Africans and Asians.

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How Does Cystic Fibrosis Affect Your Body?

Cystic fibrosis affects various organ and organ systems in children and young adults, including the respiratory system, digestive system, and reproductive system.

The abnormal electrolyte transport system causes the cells in the respiratory system, especially the lungs, to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move. This increases the risk for frequent respiratory infections. Repeated respiratory infections lead to progressive damage and eventually death of the cells in the lungs. The high rate of infection in the lower respiratory tract develops a chronic cough, blood in the sputum, and often even collapses the lungs.

What Are The Causes of Cystic Fibrosis?

Cystic fibrosis is inherited and is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. People with a single working copy are carriers and otherwise, mostly normal but those affected with CF, have one copy of the gene from each parent.

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What Are The Risk Factors of Cystic Fibrosis?

  • Family history – Cystic fibrosis is an inherited disorder.
  • Race – It most common in people of Northern European ancestry.

What Are The Symptoms of Cystic Fibrosis?

  • People with CF have a higher than normal level of salt in their sweat
  • Adults diagnosed with CF are more likely to have pancreatitis, infertility and recurring pneumonia

Respiratory signs and symptoms:

  • A persistent cough with thick mucus
  • Wheezing
  • Exercise intolerance
  • Repeated lung infections
  • Inflamed nasal passages or a stuffy nose

Digestive signs and symptoms:

  • Foul-smelling, greasy stools
  • Poor weight gain and growth
  • Intestinal blockage, particularly in newborns
  • Severe constipation
  • Rectal prolapse (when this occurs in children, it may be a sign of cystic fibrosis)
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How is Cystic Fibrosis Diagnosed?

  • Newborns are screened for cystic fibrosis. A genetic or blood test is performed to check for signs of the disease. The genetic test detects whether the baby has a defective CFTR gene. A blood test determines whether the baby’s pancreas and liver are working correctly.
  • An Immunoreactive Trypsinogen (IRT) Test is a standard newborn screening test that checks for abnormal levels of the protein called IRT in the blood. High level of IRT may indicate cystic fibrosis.
  • Sputum Test confirms the presence of a lung infection and also shows the types of germs present. It also determines which antibiotics work best to treat them.
  • A Chest X-Ray is useful in revealing swelling in the lungs due to blockages in the respiratory passageways.
  • A CT Scan allows viewing internal structures, such as the liver and pancreas, making it easier to assess the extent of organ damage caused by cystic fibrosis.
  • A Pulmonary Function Tests (PFTs) helps to measure how much air can be inhaled or exhaled and how well the lungs transport oxygen to the rest of the body. Any abnormalities in these functions may indicate Cystic fibrosis.
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How To Prevent And Control Cystic Fibrosis?

The couple should get themselves tested before having a baby if both of them are carriers of Cystic fibrosis. This can help determine the risk of having a child with CF.

Treatment of Cystic Fibrosis – Allopathic Treatment

  • Nasal polyp removal – To remove nasal polyps that obstruct breathing.
  • Oxygen therapy – To prevent high blood pressure in the lungs (pulmonary hypertension).
  • Endoscopy and lavage – To remove mucus by suctioning it from obstructed airways through an endoscope.
  • Bowel surgery – If a blockage develops in the bowel, you may require surgical repair.
  • Lung transplant – Lung transplantation may be an effective option to treat severe breathing problems, lung complications, or increasing resistance to antibiotics used to treat lung infections.

Medications prescribed for cystic fibrosis are as follows:

  • Antibiotics – To get rid of a lung infection and to prevent another infection from occurring in the future.
  • Mucus-thinning medications – To make the mucus thinner and less sticky, which significantly improves lung function.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)Ibuprofen and indomethacin, may help reduce any pain and fever associated with cystic fibrosis.
  • Bronchodilators – This helps in relaxing the muscles around the tubes that carry air to the lungs, which helps increased airflow.
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Treatment of Cystic Fibrosis – Homeopathic Treatment

  • Antimonium tartaricum – It is used for those who have a wet, rattling cough that is accompanied by extreme fatigue and breathing problems. These symptoms usually worsen when the person is lying down.
  • Carbo vegetabilis – It is for shortness of breath with anxiety, chills, and bluish skin discoloration.

Cystic Fibrosis – Lifestyle Tips

  • Take antacids
  • Take supplemental high-calorie nutrition
  • Special fat-soluble vitamins
  • Involve extra fiber in the diet to prevent intestinal blockage
  • Stay hydrated
  • Keep immunizations up to date
  • Exercise regularly
  • Quit smoking
  • Encourage hand-washing

What Are Recommended Exercise For Person With Cystic Fibrosis?

The following exercises are recommended for a person with Cystic Fibrosis:

  • Walking
  • Cycling
  • Running
  • Swimming

Cystic Fibrosis And Pregnancy- Things to Know

  • Women with CF who become pregnant should anticipate more medical care and complications during pregnancy, than non-cystic fibrosis women.
  • Underlying cystic fibrosis-related diabetes mellitus, will likely become visible and require intensified treatment. In this case, weight gain may be difficult.

Common Complications Related to Cystic Fibrosis

One can face the following common complications due to Cystic Fibrosis:

  • Respiratory system complications
  • Bronchiectasis
  • Chronic infections
  • Growths in the nose (nasal polyps)
  • Coughing up blood (hemoptysis)
  • Pneumothorax
  • Acute exacerbations
  • Digestive system complications
  • Diabetes
  • Blocked bile duct
  • Intestinal obstruction
  • Reproductive system complications
  • Thinning of the bones (osteoporosis)

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