Cystic Fibrosis (Mucoviscidosis)

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Cystic Fibrosis(Mucoviscidosis)
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Introduction

• CF is an inherited disease of your mucus and
  sweat glands
• It affects mostly the lungs, pancreas, liver,
  intestines, sinuses and sex organs
• An abnormal gene causes mucus to become extra
  thick and sticky
• This gene makes a protein that controls the
  movement of salt and water not work properly
  (retaining saltthick secretions)
• This leads to mucus plugs

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Introduction Continued

• Mucus plugs lead to collapsed lungs (atlectasis)
• Increased mucus in the lungs also allows for more
  bacterial growth which leads to frequent
  pneumonia
• Constant infections lead to inflammation in the
  lung

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Introduction Continued

• Cystic fibrosis is the most common cause of
  chronic lung disease in children and young
  adults, and the most common fatal hereditary
  disorder affecting Caucasians in the US.
• CF is a multi-system disorder of exocrine glands
  causing the formation of a thick mucus substance
  that affects the lungs, intestines, pancreas, and
  liver. The standard test for diagnosis is a sweat
  test which evaluates the level of chloride
  excreted by the body.

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Diagnosis

• The sweat chloride test is performed to determine
  the amount of chloride that is excreted in sweat
  from the body during a certain period of time.
  The test may be performed on infants to determine
  if cystic fibrosis is present. Children with
  cystic fibrosis have increased sodium and
  chloride concentrations in their sweat.
• Normal Sweat
• 18 meq/L
• Positive Test
• 60 meq/l

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Diagnosis Continued

• Detailed medical history is obtained (CF is
  Hereditary)
• Chest X-RAY to show scarring from frequent
  inflammation
• Sinus X-RAY
• Pulmonary Function Test (CF is a COPD) used only
  with individuals old enough to comply gt 8years
  old usually
• Sputum Cultures to determine certain bacteria
  growth
• Blood tests to find abnormal CF gene

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• Often the first sign of CF begins after birth,
  the mother kisses the baby and they taste salty.
• Poor feeding occurs from blocked bile ducts (bile
  released from pancreas helps digest food)

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Diagnosis Continued
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Symptoms
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Symptoms

• Increased WOB from plugged airways and air
  trapping
• Tenacious Secretions
• Frequent productive cough
• Frequent bouts of bronchitis and pneumonia
• Dehydration and malnutrition despite huge
  appetite failure to thrive
• Infertility (mostly in men)
• Ongoing Diarrhea and stomach pain

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CF leads to

• Sinusitis the sinuses have mucus build up
  leading to head ache ear and equilibrium problems
• Bronchiectasis damaged lungs become overly
  stretched and retain secretions and gas
• Pancreatitis Leads to inability to digest food,
  leading to bowl obstruction and sepsis
• Liver Disease, Diabetes, Gallstones and low bone
  density from lack of Vitamin D

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CF leads to Respiratory failure

• The mucus plugs the airways causing collapse and
  increased WOB
• Increased PaCO2, decreased PaO2 and eventual
  death if not treated.
• Infections lead to inflamed and damaged lung
  lining
• Blocked pancreas leads to vitamin deficiencies
• There is no cure for CF only treatments average
  life span is 30 years

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Treatments for CF

• Chest physiotherapy (CPT) is the traditional
  means of airway clearance in CF. It uses postural
  drainage in various positions, percussion,
  vibration, deep breathing, and coughing to loosen
  and move secretions out of the lungs. The
  treatment time including an aerosol before is
  about 45 minutes. Done so by using manual
  percussion with hand, pneumatic precursor with
  device or by Vest.

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Treatment for CF

• Chest Physical Therapy
• Using the Vest or manual precursor. Helps
  loosen secretions with percusion

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Treatment Continued

• PEP is a technique that uses a hand held device
  which can be used with a nebulizer attached. It
  has a restricted orifice. When exhaled into, this
  creates pressure in the lungs. This pressure
  allows air to enter behind areas of mucus
  obstruction and keeps the airways open during
  exhalation. As you exhale, mucus moves towards
  the larger airways, so it can be more easily
  coughed up with the huff technique. PEP can be
  taught to children as young as 5 years, and can
  be passively given to infants via a mask. The
  treatment time is about 20 minutes.

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PEP Device
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Treatment Continued

• Vibratory Positive Expiratory Pressure (Flutter,
  Acapella) Vibratory positive expiratory
  pressure is a hand held device. Exhaling into
  this device results in oscillations of pressure
  and airflow which vibrate the airway walls
  (loosening mucus), helps hold the airway open
  (which allows air to get behind secretions and
  keeps the airways open during exhalation). It
  speeds up airflow helping mucus move up to the
  larger airways where it can be more easily
  coughed up. Vibratory PEP can be taught to
  children as young as 2 years old by mask, and to
  ages 5 and up via mouthpiece. Treatment time is
  about 20 minutes.

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Treatment Continued

• Intrapulmonary Percussive Ventilation.
• The IPV is a pneumatic (air driven) device that
• delivers both continuous airway pressure and mini
• bursts of air. At the same time the IPV delivers
  a
• dense aerosol.
• The combination allows air to
• enter behind mucus blockage,
• and vibration to dislodge mucus
• from the airway walls so it can
• be more easily coughed up..

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Treatment Continued

• Active Cycle of BreathingActive cycle of
  breathing is a series of breathing techniques,
  consisting of thoracic expansion exercises (deep
  breathing), breathing control (using the
  diaphragm), and the forced expiration technique
  (huff). These breathing cycles are performed in
  various positions of drainage similar to CPT
  positions but without the percussion. This can be
  taught at about the age of 8 years. Treatment
  time, including an aerosol before, is about 45
  minutes.

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Treatments

• Autogenic DrainageAutogenic Drainage is a
  breathing technique which involves 3 phases of
  breathing levels
• The first phase is the unsticking phase which is
  inhalation and exhalation of small amounts of
  air.
• Phase two is the collection phase where medium
  sized breaths are inhaled and exhaled.
• Phase three is the evacuation phase where large
  amounts of air are inhaled and exhaled.

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Treatments

• Hand Held Nebulizers are used in conjunction with
  PEP, IPV, CPT and breathing techniques
• The nebulizer will nebulize medications that
  bronchodilate and help
• Break up mucus, as well as
• antibiotics

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Medications Used

• Antibiotics Tobramycin and azithromycin to fight
  bacterial infection. Given by aerosol in
  nebulizer or by IV
• Anti-Inflammatory Drugs Steroids given inhaled
  or by IV also Ibuprofen is given
• Bronchodilators Albuterol/Xopenex given to relax
  smooth muscle
• Mucolytics Given with bronchodilators to break
  up thick secreations. Main one is Dornase Alfa
  (Pulmozyne) made specifically for CF patients

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More Treatments

• Oxygen Therapy at low concentrations.
• Lung Transplantation depends on severity of
  illness and health of participate
• Nutritional therapy oral pancreatic enzymes to
  digest fats and proteins and absorb vitamins.
• Vitamin supplements of A, D, E and K
• Feeding tube at night (G-Tube)
• Enemas and stomach meds to control acid

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Conclusion

• CF is a deadly hereditary disease that is
  treatable but not curable
• CF causes abnormally thick mucus which blocks
  bile ducts and plugs up the lung and sinus
• May lead to respiratory failure, malnutrition and
  frequent pnuemonias
• Treatment includes methods to remove and thin
  mucus and medications to treat digestive
  problems, and infections