| Cystic fibrosis (CF) is a common
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| | her hands several times a day. Devices
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| hereditary disease that affects the
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| | that recreate this percussive therapy
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| entire body, causing progressive
| |
| | include the ThAIRapy Vest and the
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| disability and early death. Breathing
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| | intrapulmonary percussive ventilator
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| difficulties are the most common symptom
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| | (IPV). These are portable and adapted for
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| and result from frequent lung infections,
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| | home use.[34] Aerobic exercise is of
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| which are treated, though not always
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| | great benefit to people with cystic
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| cured, by antibiotics and other
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| | fibrosis. Not only does exercise increase
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| medications. CF is a multi-system
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| | sputum clearance, but it improves
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| disorder of exocrine glands causing the
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| | cardiovascular and overall health.
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| formation of a thick mucus substance that
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| | Aerosolized medications which help loosen
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| affects the lungs, intestines, pancreas,
| |
| | secretions include dornase alfa and
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| and liver. The standard test for
| |
| | hypertonic saline (saltwater).
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| diagnosis is a sweat test which evaluates
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| | Dornase is a recombinant human
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| the level of chloride excreted by the
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| | deoxyribonuclease which breaks down DNA
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| body. A multitude of symptoms, including
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| | in the sputum, thus decreasing its
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| sinus infections, poor growth, diarrhea
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| | viscosity. N-Acetylcysteine may also
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| and infertility, result from the effects
| |
| | decrease sputum viscosity, but research
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| of CF on other parts of the body.
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| | and experience have shown its benefits to
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| CF is one of the most common fatal
| |
| | be minimal. Albuterol and ipratropium
|
| inherited diseases. It is most prevalent
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| | bromide are inhaled to increase the size
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| among Caucasians and Ashkenazi Jews; one
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| | of the small airways by relaxing the
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| in 25 people of European descent carries
| |
| | surrounding muscles.
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| one gene for CF, making it the most
| |
| | As lung disease worsens, breathing
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| common genetic disease among them.
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| | support from machines may become
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| Individuals with cystic fibrosis can be
| |
| | necessary. Individuals with CF may need
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| diagnosed prior to birth by genetic
| |
| | to wear special masks at night that help
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| testing or in early childhood by a sweat
| |
| | push air into their lungs. These
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| test. There is no cure for CF, and the
| |
| | machines, known as bilevel positive
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| current median age of survival for
| |
| | airway pressure (BiPAP) ventilators, help
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| patients living in the U.S. is 36.8
| |
| | prevent low blood oxygen levels during
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| years. Ultimately, lung transplantation
| |
| | sleep. BiPAP may also be used during
|
| is often necessary as CF worsens.
| |
| | physical therapy to improve sputum
|
| CF is caused by a mutation in a gene
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| | clearance.[37] During severe illness,
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| called the cystic fibrosis transmembrane
| |
| | people with CF may need to have a tube
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| conductance regulator (CFTR). This gene
| |
| | placed in their throats and their
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| helps create sweat, digestive juices, and
| |
| | breathing supported by a ventilator.
|
| mucus. Although most people without CF
| |
| | Treatment of other aspects of CF Newborns
|
| have two working copies of the CFTR gene,
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| | with meconium ileus typically require
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| only one is needed to prevent cystic
| |
| | surgery while adults with distal
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| fibrosis. CF develops when neither gene
| |
| | intestinal obstruction syndrome typically
|
| works normally. Therefore, CF is
| |
| | do not. Treatment of pancreatic
|
| considered an autosomal recessive
| |
| | insufficiency by replacement of missing
|
| disease. The name cystic fibrosis refers
| |
| | digestive enzymes allows the gut to
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| to the characteristic scarring (fibrosis)
| |
| | properly absorb nutrients and vitamins
|
| and cyst formation within the pancreas,
| |
| | that would otherwise be lost in the
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| first recognized in the 1930s.
| |
| | feces. Even so, most individuals with CF
|
| The most consistent aspect of therapy in
| |
| | take additional amounts of vitamins A, D,
|
| cystic fibrosis is limiting and treating
| |
| | E, and K and eat high calorie meals. The
|
| the lung damage caused by thick mucus and
| |
| | diabetes common in CF is typically
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| infection with the goal of maintaining
| |
| | treated with insulin injections.
|
| quality of life. Intravenous, inhaled,
| |
| | Development of osteoporosis can be
|
| and oral antibiotics are used to treat
| |
| | prevented by increased intake of vitamin
|
| chronic and acute infections.
| |
| | D and calcium and can be treated by
|
| Mechanical devices and inhalational
| |
| | bisphosphonates. Poor growth may be
|
| medications are used to alter and clear
| |
| | avoided by insertion of a feeding tube
|
| the thickened mucus. Other aspects of CF
| |
| | for increasing calories through
|
| therapy involve treatment of diabetes
| |
| | supplemental feeds or by administration
|
| with insulin, pancreatic disease with
| |
| | of injected growth hormone.
|
| enzyme replacement, and infertility with
| |
| | Sinus infections are treated by prolonged
|
| advanced reproductive techniques. In
| |
| | courses of antibiotics. The development
|
| addition, therapies such as
| |
| | of nasal polyps or other chronic changes
|
| transplantation and gene therapy aim to
| |
| | within the nasal passages may severely
|
| cure some of the effects of cystic
| |
| | limit airflow through the nose. Sinus
|
| fibrosis.
| |
| | surgery is often used to alleviate nasal
|
| Antibiotics to treat lung disease
| |
| | obstruction and to limit further
|
| Antibiotics are given whenever pneumonia
| |
| | infections. Nasal steroids such as
|
| is suspected or there has been a decline
| |
| | fluticasone are used to decrease nasal
|
| in lung function. Antibiotics are often
| |
| | inflammation. Female infertility may be
|
| chosen based on information about prior
| |
| | overcome by assisted reproduction
|
| infections. Many bacteria common in
| |
| | technology, particularly embryo transfer
|
| cystic fibrosis are resistant to multiple
| |
| | techniques. Male infertility may be
|
| antibiotics and require weeks of
| |
| | overcome with intracytoplasmic sperm
|
| treatment with intravenous antibiotics
| |
| | injection. Third party reproduction is
|
| such as vancomycin, tobramycin,
| |
| | also a possibility for women with CF.
|
| meropenem, ciprofloxacin, and
| |
| | Transplantation and gene therapy Lung
|
| piperacillin. This prolonged therapy
| |
| | transplantation often becomes necessary
|
| often necessitates hospitalization and
| |
| | for individuals with cystic fibrosis as
|
| insertion of a more permanent IV such as
| |
| | lung function and exercise tolerance
|
| a PICC line or Port-a-Cath. Inhaled
| |
| | declines. Although single lung
|
| therapy with antibiotics such as
| |
| | transplantation is possible in other
|
| tobramycin and colistin is often given
| |
| | diseases, individuals with CF must have
|
| for months at a time in order to improve
| |
| | both lungs replaced because the remaining
|
| lung function by impeding the growth of
| |
| | lung would contain bacteria that could
|
| colonized bacteria. Oral antibiotics such
| |
| | infect the transplanted lung. A
|
| as ciprofloxacin or azithromycin are
| |
| | pancreatic or liver transplant may be
|
| sometimes given to help prevent infection
| |
| | performed at the same time in order to
|
| or to control ongoing infection. Some
| |
| | alleviate liver disease and/or diabetes.
|
| individuals spend years between
| |
| | Lung transplantation is considered when
|
| hospitalizations for antibiotics, while
| |
| | lung function approaches a point where it
|
| others require several antibiotic
| |
| | threatens survival or requires assistance
|
| treatments each year.
| |
| | from mechanical devices.
|
| Several common antibiotics such as
| |
| | Gene therapy holds promise as a potential
|
| tobramycin and vancomycin can cause
| |
| | avenue to cure cystic fibrosis.
|
| hearing loss or kidney problems with
| |
| | Gene therapy attempts to place a normal
|
| long-term use. In order to prevent these
| |
| | copy of the CFTR gene into affected
|
| side effects, the amount of antibiotics
| |
| | cells. Studies have shown that to prevent
|
| in the blood are routinely measured and
| |
| | the lung manifestations of cystic
|
| adjusted accordingly.
| |
| | fibrosis, only 5-10% of the normal amount
|
| Other methods to treat lung disease
| |
| | of CFTR gene expression is needed.[45]
|
| Several mechanical techniques are used to
| |
| | Many approaches have been theorized and
|
| dislodge sputum and encourage its
| |
| | several clinical trials have been
|
| expectoration. In the hospital setting,
| |
| | initiated but, as of 2006, many hurdles
|
| physical therapy is utilized; a therapist
| |
| | still exist before gene therapy can be
|
| pounds an individual's chest with his or
| |
| | successful.
|
