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Cystic Fibrosis
 
 
Cystic Fibrosis

Cystic fibrosis (CF) is a hereditary disease affecting certain glands in the mucus membranes, causing those glands to produce thick, sticky mucus. This condition mainly affects organs such as the lungs and digestive tract.

Causes of Cystic Fibrosis

Cystic fibrosis (CF) occurs when the gene responsible for manufacturing an ion channel becomes mutated, causing the gene to work abnormally. This channel creates mucus, digestive juices and sweat. When this gene mutates it tells the body to produce thick, sticky mucus which promotes sinus conditions.

Symptoms of Cystic Fibrosis

Cystic Fibrosis can cause multiple problems in your body, ranging from treatable to incurable.

Some of the conditions cystic fibrosis can cause:

  • People who have cystic fibrosis are more susceptible to colds and flu. When mucus cannot fulfill its function effectively, the body becomes very susceptible to infections. A weakened immune system as a result of numerous infections makes colds and flu's common and recurrent in individuals that suffer with cystic fibrosis.

  • Thick mucus production as well as a weak immune system can cause recurrent lung infections. A lung disease may result from these continuous infections and blockages in the airways, which cause inflammation.
    Symptoms of Lung disease include:
    • Breathing problems (not enough air to lungs)
    • Fungal infections
    • Large production of mucus
    • Coughing blood
    • Pneumonia
    • Bronchitis
    • Excessive coughing
    • High blood pressure
    • Heart failure

  • As there is no cure, Cystic fibrosis (CF) patients may die at a young age (many before they reach 40 years of age). New treatments and lung transplants however have increased the life expectancy of Cystic fibrosis (CF) sufferers.

  • The small emissions of pancreatic enzymes can cause growth deficiencies, a fat-soluble vitamin deficiency, fertility problems and diarrhea. Damage to the pancreas can cause diabetes (metabolic disorder categorized by constant thirst and excessive urine production), arthritis (inflamed joints) and osteoporosis (brittle bones).

  • People with Cystic fibrosis (CF) also experience Clubbing (swelling, broadening and increased nail curve) of the fingers and toes due to a low amount of oxygen in their tissue.

  • Cystic Fibrosis can cause sinus infections. Sinusitis can be identified by the following symptoms:
    • Facial pain
    • Fever
    • Runny nose
    • Headaches
    • Blocked nose
    • Congestion
    • Inflamed mucus membranes
    • Chronic sinusitis

  • One in every four people who have Cystic fibrosis (CF) develop nasal polyps. A nasal polyp is a non-cancerous growth that forms from chronically inflamed mucus membranes. Nasal polyps can cause:
    • Blocked nasal passages and sinuses
    • Increased breathing problems
    • Decreased sense of smell
    • Headaches
    • Pain in the sinus regions
    • Runny nose
    • Snoring
    • Lack of sleep (Apnea)
    • Altered facial structure because Cystic fibrosis (CF) promotes the growth and enlargement of nasal polyps.

  • Infertility - This affects both men and women. Men with CF may suffer from a congenital (inherited) deficiency of the Vas deferens (tube connecting the testes to penis). In women the cervical mucus is chronically thick and may sometimes cause amenorrhea (absence of menstrual period in women of reproductive age).

  • CF is more common in infants and children. Children may suffer from:
    • Poor growth because their bodies cannot absorb enough nutrients and their metabolism does not allow them to gain weight.

Diagnosis of Cystic Fibrosis

Cystic fibrosis (CF) is an inherited condition placing diagnosis at a young age. Children suspected of cystic fibrosis usually have two or more sweat tests done. A clear, odorless solution is applied to the skin causing the glands to produce sweat rapidly. An absorbent material is then placed on the skin, to collect the sweat that will be used to measure the amount of sodium and chloride in the perspiration. A high level of sodium in the sweat will confirm that the child has cystic fibrosis.

Other diagnostic methods includes newborn testing (blood test), which is not common, and gene testing (blood test) that can be used for people over 18 years. Candidates suffering with Cystic fibrosis (CF) after the age of 18 suffer a milder form of the disease.

Treatment of Cystic Fibrosis

Since there is no cure for cystic fibrosis, an effort should be maintained to minimize symptoms and keep this condition under control. There are two main ways one can treat the symptoms:

  • Home treatments
    • The use of specific antibiotics that treat Pseudomonas species and staphylococci bacteria, which are more prominent in patients who have cystic fibrosis.
    • The use of a nettipot. These rinsing methods decrease bacterial growth and help the thick mucus to drain.

  • Surgical treatment
    • A Polypectomy for the removal of small nasal polyps.
    • Sinusotomy to treat sinusitis in one or more of the different sinus cavities.
    • Powered FESS (Functional Endoscopic Sinus Surgery) using a microdebrider (a powered instrument) to enlarge sinus openings and remove infected tissue.
    • Septoplasty is a corrective surgery done to straighten a deviated septum, which benefits the patient by improving breathing, treating a runny nose and post nasal drip.

As with all surgeries there are major concerns and risks.

  • Anesthetic risks
    • Infections
    • Headaches
    • Numbness
    • Bruising
    • Inflammation
  • Pulmonary concerns (pulmonary [affecting the lungs] disease may make the anesthetic unacceptable to the body).
  • Procedural concerns:
    • Surgery if possible should be performed within an hour to avoid respiratory complications and bleeding.
    • Nasal polyps are likely to recur.

Cystic Fibrosis sufferers have a life long struggle with illnesses of all sorts, from colds or flu to sinuses, bone irregularities and lung infections. When treating the related symptoms of Cystic fibrosis (CF) it is important that you take into consideration all the potential benefits as well as the potential downfalls of the treatments. With proper care you can lead a healthier and longer life.

 

 
 
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