Cystic FibrosisCystic Fibrosis(Mucoviscidosis)(Mucoviscidosis)

IntroductionIntroduction CF is an inherited disease of your mucus CF is an inherited disease of your mucus

and sweat glandsand sweat glands It affects mostly the lungs, pancreas, liver, It affects mostly the lungs, pancreas, liver,

intestines, sinuses and sex organsintestines, sinuses and sex organs An abnormal gene causes mucus to An abnormal gene causes mucus to

become extra thick and stickybecome extra thick and sticky This gene makes a protein that controls This gene makes a protein that controls

the movement of salt and water not work the movement of salt and water not work properly (retaining salt=thick secretions)properly (retaining salt=thick secretions)

This leads to mucus plugsThis leads to mucus plugs

Introduction ContinuedIntroduction Continued

Mucus plugs lead to Mucus plugs lead to collapsed lungs collapsed lungs (atlectasis)(atlectasis)

Increased mucus in Increased mucus in the lungs also allows the lungs also allows for more bacterial for more bacterial growth which leads to growth which leads to frequent pneumoniafrequent pneumonia

Constant infections Constant infections lead to inflammation lead to inflammation in the lungin the lung

Introduction ContinuedIntroduction Continued

Cystic fibrosis is the most common cause of Cystic fibrosis is the most common cause of chronic lung disease in children and young chronic lung disease in children and young

adults, and the most common fatal hereditary adults, and the most common fatal hereditary disorder affecting Caucasians in the US. disorder affecting Caucasians in the US.

CF is a multi-system disorder of exocrine glands CF is a multi-system disorder of exocrine glands causing the formation of a thick mucus causing the formation of a thick mucus

substance that affects the lungs, intestines, substance that affects the lungs, intestines, pancreas, and liver. The standard test for pancreas, and liver. The standard test for

diagnosis is a sweat test which evaluates the diagnosis is a sweat test which evaluates the level of chloride excreted by the body. level of chloride excreted by the body.

DiagnosisDiagnosis

The sweat chloride test is performed to determine the amount of The sweat chloride test is performed to determine the amount of chloride that is excreted in sweat from the body during a certain 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 period of time. The test may be performed on infants to determine if cystic fibrosis is present. Children with cystic fibrosis have cystic fibrosis is present. Children with cystic fibrosis have

increased sodium and chloride concentrations in their sweat.increased sodium and chloride concentrations in their sweat.

Normal SweatNormal Sweat

18 meq/L18 meq/L

Positive TestPositive Test

60 meq/l60 meq/l

Diagnosis ContinuedDiagnosis Continued Detailed medical history is obtained (CF is Detailed medical history is obtained (CF is

Hereditary)Hereditary) Chest X-RAY to show scarring from frequent Chest X-RAY to show scarring from frequent

inflammationinflammation Sinus X-RAY Sinus X-RAY Pulmonary Function Test (CF is a COPD); used Pulmonary Function Test (CF is a COPD); used

only with individuals old enough to comply > 8years only with individuals old enough to comply > 8years old usuallyold usually

Sputum Cultures to determine certain bacteria Sputum Cultures to determine certain bacteria growthgrowth

Blood tests to find abnormal CF geneBlood tests to find abnormal CF gene

Often the first sign of CF begins after birth, the Often the first sign of CF begins after birth, the mother kisses the baby and they taste salty.mother kisses the baby and they taste salty.

Poor feeding occurs from blocked bile ducts (bile Poor feeding occurs from blocked bile ducts (bile released from pancreas helps digest food)released from pancreas helps digest food)

Diagnosis ContinuedDiagnosis Continued

SymptomsSymptoms

SymptomsSymptoms Increased WOB from plugged airways and Increased WOB from plugged airways and

air trappingair trapping Tenacious SecretionsTenacious Secretions Frequent productive coughFrequent productive cough Frequent bouts of bronchitis and Frequent bouts of bronchitis and

pneumoniapneumonia Dehydration and malnutrition despite huge Dehydration and malnutrition despite huge

appetite; failure to thriveappetite; failure to thrive Infertility (mostly in men)Infertility (mostly in men) Ongoing Diarrhea and stomach painOngoing Diarrhea and stomach pain

CF leads to…CF leads to… SinusitisSinusitis: the sinuses have mucus build : the sinuses have mucus build

up leading to head ache ear and up leading to head ache ear and equilibrium problemsequilibrium problems

Bronchiectasis:Bronchiectasis: damaged lungs become damaged lungs become overly stretched and retain secretions and overly stretched and retain secretions and gasgas

Pancreatitis:Pancreatitis: Leads to inability to digest Leads to inability to digest food, leading to bowl obstruction and food, leading to bowl obstruction and sepsissepsis

Liver Disease, Diabetes, Gallstones and Liver Disease, Diabetes, Gallstones and low bone density from lack of Vitamin Dlow bone density from lack of Vitamin D

CF leads to Respiratory failureCF leads to Respiratory failure The mucus plugs the airways causing The mucus plugs the airways causing

collapse and increased WOBcollapse and increased WOB Increased PaCO2, decreased PaO2 and Increased PaCO2, decreased PaO2 and

eventual death if not treated.eventual death if not treated. Infections lead to inflamed and damaged Infections lead to inflamed and damaged

lung lininglung lining Blocked pancreas leads to vitamin Blocked pancreas leads to vitamin

deficienciesdeficiencies There is no cure for CF only treatments; There is no cure for CF only treatments;

average life span is 30 yearsaverage life span is 30 years

Treatments for CFTreatments for CF

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

Treatment for CFTreatment for CF

Chest Physical Therapy:Chest Physical Therapy:

Using the “Vest” or manual Using the “Vest” or manual precursor. Helps loosen precursor. Helps loosen

secretions with percusionsecretions with percusion

Treatment ContinuedTreatment Continued PEPPEP is a technique that uses a hand held device is a technique that uses a hand held device

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

PEP DevicePEP Device

Treatment ContinuedTreatment Continued Vibratory Positive Expiratory Pressure (Flutter®, Vibratory Positive Expiratory Pressure (Flutter®,

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

about 20 minutesabout 20 minutes..

Treatment Continued…Treatment Continued…Intrapulmonary Percussive VentilationIntrapulmonary Percussive Ventilation.. The IPV is a pneumatic (air driven) device thatThe IPV is a pneumatic (air driven) device thatdelivers both continuous airway pressure and minidelivers both continuous airway pressure and minibursts of air. At the same time the IPV delivers abursts of air. At the same time the IPV delivers adense aerosol. dense aerosol. The combination allows air to The combination allows air to enter behind mucus blockage, enter behind mucus blockage, and vibration to dislodge mucus and vibration to dislodge mucus from the airway walls so it can from the airway walls so it can be more easily coughed up..be more easily coughed up..

Treatment ContinuedTreatment Continued Active Cycle of BreathingActive Cycle of Breathing

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

TreatmentsTreatments

Autogenic DrainageAutogenic DrainageAutogenic Drainage is a breathing technique Autogenic Drainage is a breathing technique which involves 3 phases of breathing levels: which involves 3 phases of breathing levels:

The first phase is the unsticking phase which is The first phase is the unsticking phase which is inhalation and exhalation of small amounts of inhalation and exhalation of small amounts of air. air.

Phase two is the collection phase where medium Phase two is the collection phase where medium sized breaths are inhaled and exhaled. sized breaths are inhaled and exhaled.

Phase three is the evacuation phase where Phase three is the evacuation phase where large amounts of air are inhaled and exhaled. large amounts of air are inhaled and exhaled.

Treatments Treatments

Hand Held Nebulizers are used in Hand Held Nebulizers are used in conjunction with PEP, IPV, CPT and conjunction with PEP, IPV, CPT and breathing techniquesbreathing techniques

The nebulizer will nebulize medications The nebulizer will nebulize medications that bronchodilate and helpthat bronchodilate and help

Break up mucus, as well asBreak up mucus, as well as antibioticsantibiotics

Medications UsedMedications Used AntibioticsAntibiotics: Tobramycin and azithromycin to fight : Tobramycin and azithromycin to fight

bacterial infection. Given by aerosol in nebulizer bacterial infection. Given by aerosol in nebulizer or by IVor by IV

Anti-Inflammatory Drugs:Anti-Inflammatory Drugs: Steroids given inhaled Steroids given inhaled or by IV; also Ibuprofen is givenor by IV; also Ibuprofen is given

Bronchodilators: Bronchodilators: Albuterol/Xopenex given to Albuterol/Xopenex given to relax smooth musclerelax smooth muscle

Mucolytics:Mucolytics: Given with bronchodilators to break Given with bronchodilators to break up thick secreations. Main one is Dornase Alfa up thick secreations. Main one is Dornase Alfa (Pulmozyne) made specifically for CF patients(Pulmozyne) made specifically for CF patients

More TreatmentsMore Treatments

Oxygen Therapy at low concentrations.Oxygen Therapy at low concentrations. Lung Transplantation; depends on severity Lung Transplantation; depends on severity

of illness and health of participateof illness and health of participate Nutritional therapy; oral pancreatic Nutritional therapy; oral pancreatic

enzymes to digest fats and proteins and enzymes to digest fats and proteins and absorb vitamins. absorb vitamins.

Vitamin supplements of A, D, E and KVitamin supplements of A, D, E and K Feeding tube at night (G-Tube)Feeding tube at night (G-Tube) Enemas and stomach meds to control acidEnemas and stomach meds to control acid

ConclusionConclusion CF is a deadly hereditary disease that is CF is a deadly hereditary disease that is

treatable but not curabletreatable but not curable CF causes abnormally thick mucus which CF causes abnormally thick mucus which

blocks bile ducts and plugs up the lung blocks bile ducts and plugs up the lung and sinusand sinus

May lead to respiratory failure, malnutrition May lead to respiratory failure, malnutrition and frequent pnuemoniasand frequent pnuemonias

Treatment includes methods to remove Treatment includes methods to remove and thin mucus and medications to treat and thin mucus and medications to treat digestive problems, and infectionsdigestive problems, and infections