Mucoviscidosis (Cystic Fibrosis): Symptoms, causes and treatment

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Mucoviscidosis (Cystic Fibrosis)
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Mucoviscidosis (Cystic Fibrosis)

• Mucoviscidosis, which is also referred to as
  cystic fibrosis (CF) is a congenital disease
  wherein the lungs, intestines, and the pancreas
  become clogged with thick mucus due to damage to
  the cells that produce saliva, sweat, mucus, and
  digestive juices. Normally, these secretions are
  thin and slippery and act as a lubricant. The
  thick and sticky secretions block the ducts and
  passageways in the lungs and pancreas. If mucus
  is accumulated in the lungs, it can cause
  breathing problems, repeated lung infections, and
  lung damage. The accumulation of mucus in the
  pancreas can lead to digestive problems,
  preventing the digestive enzymes produced in the
  pancreas to reach the small intestine due to
  blockage in the pancreatic duct. 

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Causes of Cystic Fibrosis

• This is a genetic disorder caused by a defective
  gene called cystic fibrosis transmembrane
  conductance regulator (CFTR). The protein
  produced by CFTR gene is responsible for
  regulating the normal movement of salt and water
  in and out of the cells. This defective gene
  causes thick, sticky mucus secretions in the
  digestive and respiratory tracts. It could also
  affect the reproductive system, thereby
  increasing the risk of infertility. There is an
  increased amount of salt in sweat. If both the
  parents carry the defective gene, then there are
  25 chances that their child will be affected by
  this condition, 50 chances that the child will
  carry the defective gene, and 25 chances that
  the child will neither be a carrier nor be
  affected by this condition. Family history is the
  biggest risk factor for cystic fibrosis. 

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Symptoms

• The severity of the symptoms can vary from
  person to person. Some may either suffer from
  digestive problems or respiratory problems while
  others may suffer from both. Signs and symptoms
  may vary depending upon the age. In newborns,
  there may be a blockage in the intestine, causing
  bulky and greasy stools (steatorrhea). Newborns
  also suffer from frequent respiratory infections
  due to the accumulation of mucus in the
  lungs. In children and young adults, symptoms
  include salty taste to the skin, diarrhoea,
  foul-smelling and greasy stools, thick sputum,
  delayed growth, frequent coughing or wheezing,
  chest and sinus infections with recurring
  bronchitis or pneumonia, dehydration, rectal
  prolapse, and enlargement of fingertips or toes.
  Some other medical conditions that are related to
  cystic fibrosis include nasal polyps, liver
  cirrhosis, and intussusceptions.

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Treatment

• The treatment is aimed at preventing infections,
  removing thick mucus from the lungs, relieving
  respiratory and digestive problems, and
  maintaining adequate nutrition. There are
  different treatment options for respiratory and
  digestive problems. Lung problems due to cystic
  fibrosis can be treated with drug therapy.
  Respiratory infections are common in people with
  cystic fibrosis. Antibiotics (oral, inhaled or
  intravenous) are administered in order to treat
  infections. Anti-inflammatory medications like
  ibuprofen might also be prescribed to decrease
  the inflammation in the lungs.

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Treatment
Continue

• While the use of bronchodilators like albuterol
  is suggested to relax the muscles around the
  airways, mucus-thinning drugs like hypertonic
  saline and acetylcysteine saline help reduce the
  stickiness of mucus in the airways.
• Oxygen therapy is administered to increase the
  levels of oxygen in the blood. If the patient
  shows increasing resistance to antibiotics, and
  severe pulmonary complications, then lung
  replacement is recommended. Cystic fibrosis can
  be managed by following drug therapy and
  lifestyle modification. Following a balanced diet
  and exercise regimen will certainly prove
  beneficial.

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