Pulmonary ConditionsPulmonary Conditions

Medical and Psychosocial Medical and Psychosocial Aspects of DisabilityAspects of Disability

RCS 6080RCS 6080

Description & DefinitionsDescription & Definitions

Chronic Obstructive Pulmonary Chronic Obstructive Pulmonary Disease (COPD) is characterized by Disease (COPD) is characterized by decreased expiratory airflowdecreased expiratory airflow

Reduction in expiratory airflow has 2 Reduction in expiratory airflow has 2 causes:causes:• Decreased expiratory air flow pressure Decreased expiratory air flow pressure

(decrease in driving pressure)(decrease in driving pressure)• Increased resistance to expiratory air Increased resistance to expiratory air

flow (resulting from narrowing of flow (resulting from narrowing of airways)airways)

Descriptions/DefinitionsDescriptions/Definitions

Emphysema & chronic bronchitis are Emphysema & chronic bronchitis are often considered together under the often considered together under the term COPD because most people with term COPD because most people with one of these conditions has the other. one of these conditions has the other. Thus most people with COPD with have Thus most people with COPD with have both airway & alveolar disease.both airway & alveolar disease.

COPD affects as many as 30,000,000 COPD affects as many as 30,000,000 AmericansAmericans

COPD is the 5th leading cause of deathCOPD is the 5th leading cause of death

Etiology, Pathophysiology Etiology, Pathophysiology & Clinical Features& Clinical Features

Several factors are involved in the Several factors are involved in the pathogenesis of COPD, but smoking is pathogenesis of COPD, but smoking is the most importantthe most important• Other factors include occupational Other factors include occupational

exposure to dust, fumes & air pollution.exposure to dust, fumes & air pollution. Aside from these factors, the Aside from these factors, the

development and progression of development and progression of COPD is largely related to genetic COPD is largely related to genetic disposition.disposition.

EmphysemaEmphysema

Emphysema is an Emphysema is an enlargement of air enlargement of air spaces caused by spaces caused by destruction of alveolar destruction of alveolar walls. Air spaces walls. Air spaces greater than one cm greater than one cm are bullae. This photo are bullae. This photo shows apical bullous shows apical bullous disease with relatively disease with relatively little involvement of little involvement of the rest of the lung. the rest of the lung.

EmphysemaEmphysema

Emphysema can be a result Emphysema can be a result of obstruction caused by of obstruction caused by chronic bronchitis. It chronic bronchitis. It occurs when there is back occurs when there is back pressure on the alveoli. pressure on the alveoli. This increased pressure This increased pressure tends over time to make tends over time to make their walls break down, and their walls break down, and instead of having lots of instead of having lots of tiny air sacs functioning tiny air sacs functioning well, you end up with large well, you end up with large cavities consisting of cavities consisting of alveoli that have coalesced, alveoli that have coalesced, and which do not and which do not adequately perform the adequately perform the task of gas transfer. task of gas transfer.

Chronic BronchitisChronic Bronchitis The lungs essentially comprise lots The lungs essentially comprise lots

of tubes and tubules (called bronchi of tubes and tubules (called bronchi and bronchioles) of gradually and bronchioles) of gradually diminishing size, which end in little diminishing size, which end in little collections of air sacs called alveoli. collections of air sacs called alveoli. It is across the walls of the alveoli It is across the walls of the alveoli that the gases are exchanged, that the gases are exchanged, oxygen being taken into the oxygen being taken into the bloodstream and carbon dioxide bloodstream and carbon dioxide passing into the alveoli to be passing into the alveoli to be exhaled. Chronic bronchitis is an exhaled. Chronic bronchitis is an inflammation or irritation of the inflammation or irritation of the airways in the lungs which is airways in the lungs which is associated with:associated with:

Scarring or fibrosis of the walls of the Scarring or fibrosis of the walls of the bronchioles making them less pliablebronchioles making them less pliable

Thickening of their lining causing Thickening of their lining causing narrowing of the airwaynarrowing of the airway

Production of excessive quantities of Production of excessive quantities of thick mucus which further plugs the thick mucus which further plugs the tubules and compromises breathingtubules and compromises breathing

Categories of COPDCategories of COPD

Type A - “Pink Puffer”Type A - “Pink Puffer”• Considered to have predominantly Considered to have predominantly

emphysemaemphysema Type B - “Blue Bloater”Type B - “Blue Bloater”

• Considered to have predominantly Considered to have predominantly chronic bronchitis.chronic bronchitis.

Functional DisabilitiesFunctional Disabilities

Earliest manifestations of COPD may be Earliest manifestations of COPD may be relatively mild, but as time goes on, relatively mild, but as time goes on, dyspnea becomes the most limiting factordyspnea becomes the most limiting factor• Years may pass before the degree of dyspnea Years may pass before the degree of dyspnea

is severe enough to limit routine ADLs such as is severe enough to limit routine ADLs such as walking.walking.

• As time progresses, activities such as As time progresses, activities such as dressing, bathing, speech and even eating dressing, bathing, speech and even eating cannot be accomplished without severe cannot be accomplished without severe shortness of breathshortness of breath

Functional DisabilitiesFunctional Disabilities

Until the disease is extremely Until the disease is extremely advanced, sedentary activities advanced, sedentary activities may be accomplished without may be accomplished without much difficulty.much difficulty.

Driving may be possible, but Driving may be possible, but walking - even limited distances - walking - even limited distances - may not be feasible, particularly is may not be feasible, particularly is there is an incline or stairs.there is an incline or stairs.

Functional DisabilitiesFunctional Disabilities

Assessment of a given person’s Assessment of a given person’s functional capabilities may be difficult functional capabilities may be difficult to determine based solely on pulmonary to determine based solely on pulmonary function studies and blood gases.function studies and blood gases.

Depression, fear, & anxiety are potent Depression, fear, & anxiety are potent factors that may further exacerbate the factors that may further exacerbate the person’s physical limitations.person’s physical limitations.

Preparation of sedentary occupation is Preparation of sedentary occupation is useful even when COPD is mild because useful even when COPD is mild because the rate of progression is variable.the rate of progression is variable.

Treatment of COPDTreatment of COPD

Many people with COPD might be Many people with COPD might be able to have some reversibility – able to have some reversibility – through proper medical through proper medical management.management.

Adequate fluid intake and use of Adequate fluid intake and use of expectorants are needed to clear expectorants are needed to clear the respiratory tract of secretions.the respiratory tract of secretions.

Oxygen therapyOxygen therapy

Treatment of COPDTreatment of COPD

Chest physical therapy and pulmonary Chest physical therapy and pulmonary rehabilitation programs are useful to:rehabilitation programs are useful to:• Learn how to expel mucus from Learn how to expel mucus from

respiratory tractrespiratory tract• Learn breathing exercises and relaxation Learn breathing exercises and relaxation

techniques (useful in ADLs)techniques (useful in ADLs)• Do exercise reconditioning that can help Do exercise reconditioning that can help

increase endurance & improve work increase endurance & improve work capacitycapacity

Psychological ImplicationsPsychological Implications

Counseling often helps the person Counseling often helps the person deal with the anxiety/stress deal with the anxiety/stress associated with diseases that can associated with diseases that can cause shortness of breath & cause shortness of breath & limitations of activity.limitations of activity.• Learning to deal effectively with Learning to deal effectively with

problems & make satisfactory lifestyle problems & make satisfactory lifestyle can reduce feelings of desperation.can reduce feelings of desperation.

Vocational implicationsVocational implications

People may have to change employment People may have to change employment goalsgoals

The American Thoracic Society:The American Thoracic Society:• Mild impairment – usually not correlated with Mild impairment – usually not correlated with

reduced ability to perform most jobs.reduced ability to perform most jobs.• Moderate impairment – correlates with a Moderate impairment – correlates with a

decreased ability to meet the demands of many decreased ability to meet the demands of many jobsjobs

• Severe impairment – pulmonary function is so Severe impairment – pulmonary function is so impaired that a person cannot meet the impaired that a person cannot meet the demands of most occupations.demands of most occupations.

AsthmaAsthma

Asthma is considered an Asthma is considered an inflammatory disease of the airwaysinflammatory disease of the airways• Reversible airway obstructionReversible airway obstruction• Bronchial hyper-reactivityBronchial hyper-reactivity

Frequency, duration, & severity of Frequency, duration, & severity of asthma attacks varies from person asthma attacks varies from person to person.to person.

Asthma attacks are characterized by Asthma attacks are characterized by shortness of breath & wheezing.shortness of breath & wheezing.

AsthmaAsthma

Mainly a bronchial Mainly a bronchial disease, asthma is disease, asthma is characterized by features characterized by features in the following 3 images: in the following 3 images:

Mural inflammation Mural inflammation (eosinophils, mast cells, (eosinophils, mast cells, lymphocytes) lymphocytes)

Wall thickening by Wall thickening by edema, hyperemia, edema, hyperemia, fibrosis fibrosis

Smooth muscle Smooth muscle thickening (arrow) thickening (arrow)

Mucous plugs Mucous plugs Epithelial sloughEpithelial slough

Pathology of AsthmaPathology of Asthma

Normal LungsNormal Lungs AsthmaAsthma

Source: “What You and Your Family Can Do About Asthma” by the Global Initiative For Asthma Source: “What You and Your Family Can Do About Asthma” by the Global Initiative For Asthma Created and funded by NIH/NHLBICreated and funded by NIH/NHLBI

Asthma Prevalence* by AgeAsthma Prevalence* by AgeUnited States: 1980United States: 1980––19961996

0

1

2

3

4

5

6

7

8

Year

Pre

vale

nce (

%)

Source: National Health Interview Survey* 12-month prevalence

Under 18Under 18TotalTotal

18+18+

Asthma Prevalence* by SexAsthma Prevalence* by SexUnited States: 1982United States: 1982––19961996

0

1

2

3

4

5

6

7

8

Year

Pre

va

len

ce

(%

)

Source: National Health Interview Survey* 12-month prevalence

MaleMale

FemaleFemale

TotalTotal

Asthma Prevalence* by RaceAsthma Prevalence* by RaceUnited States: 1982United States: 1982––19961996

0

1

2

3

4

5

6

7

8

Year

Pre

va

len

ce

(%

)

Source: National Health Interview Survey* 12-month prevalence

BlackBlack

WhiteWhite

Asthma Prevalence* by RaceAsthma Prevalence* by RaceAges 5-34, United States: 1980Ages 5-34, United States: 1980––19961996

0123456789

Year

Pre

vale

nce (

%)

Source: National Health Interview Survey* 12-month prevalence

Black, 5-34Black, 5-34

White, 5-34White, 5-34

Age-Adjusted* Asthma Mortality Age-Adjusted* Asthma Mortality RatesRates by Sex, United States: 1979by Sex, United States: 1979––19981998

0

5

10

15

20

25

30

Year

Ra

te p

er

mil

lio

n

MaleMale

TotalTotal

FemaleFemale

Source: Underlying Cause of Death dataset by the National Center for Health Statistics

* Age-adjusted to 2000 U.S. population

Age-Adjusted* Asthma Mortality Age-Adjusted* Asthma Mortality RatesRates

by Race, United States: 1979by Race, United States: 1979––19981998

Source: Underlying Cause of Death dataset by the National Center for Health Statistics

* Age-adjusted to 2000 U.S. population

0

10

20

30

40

50

60

1979

1981

1983

1985

1987

1989

1991

1993

1995

1997 Year

Ra

te p

er

mill

ion BlackBlack

OtherOther

WhiteWhite

Asthma Mortality Rates by RaceAsthma Mortality Rates by RaceAges 5-34, United States: 1979Ages 5-34, United States: 1979––1998 1998

0

5

10

15

20

Year

Ra

te p

er

mill

ion

Source: Underlying Cause of Death dataset by the National Center for Health Statistics* Unreliable (< 20 deaths) 1979–1995

BlackBlack

Other*Other* WhiteWhite

Costs of AsthmaCosts of AsthmaUnited States, 1980United States, 1980––19981998Projection for the Year 2000Projection for the Year 2000

0

5

10

15Estimatedcosts in billions of dollars

1980* 1985* 1990** 1994** 1998** 2000

Year

Source: * Weiss, et al. 1992** Weiss, et al. 2001

Risk Factors Risk Factors for Development of for Development of AsthmaAsthma

Genetic characteristicsGenetic characteristics Environmental exposuresEnvironmental exposures Contributing factorsContributing factors

Risk Factors for Development of Risk Factors for Development of Asthma:Asthma:Genetic CharacteristicsGenetic Characteristics

Atopy Atopy The body’s predisposition to develop an The body’s predisposition to develop an

antibody called immunoglobulin E (IgE) in antibody called immunoglobulin E (IgE) in response to exposure to environmental response to exposure to environmental allergensallergens

Can be measured in the bloodCan be measured in the blood

Clearing the AirClearing the AirCategories for Associations of Various Categories for Associations of Various ElementsElements

Sufficient evidence of a causal Sufficient evidence of a causal relationshiprelationship

Sufficient evidence of an associationSufficient evidence of an association Limited or suggested evidence of an Limited or suggested evidence of an

associationassociation Inadequate or insufficient evidence to Inadequate or insufficient evidence to

determine whether an association existsdetermine whether an association exists Limited or suggestive evidence of no Limited or suggestive evidence of no

associationassociation

Clearing the AirClearing the AirIndoor Air Exposures and Asthma Indoor Air Exposures and Asthma DevelopmentDevelopment

Biological AgentsBiological Agents Sufficient evidence of a Sufficient evidence of a

causal relationshipcausal relationship• House dust miteHouse dust mite

Sufficient evidence of an Sufficient evidence of an associationassociation• None foundNone found

Limited or suggestive Limited or suggestive evidence of an associationevidence of an association• Cockroach (in Cockroach (in

preschool-preschool-aged aged children)children)• Respiratory Respiratory syncytial syncytial virus (RSV)virus (RSV)

Chemical AgentsChemical Agents Sufficient evidence of a Sufficient evidence of a

causal relationshipcausal relationship

• None foundNone found Sufficient evidence of an Sufficient evidence of an

associationassociation

• Environmental Environmental tobacco tobacco smoke (in preschool-smoke (in preschool-aged children)aged children)

Limited or suggestive Limited or suggestive evidence of an associationevidence of an association

• None foundNone found

Clearing the AirClearing the AirIndoor Air Exposures and Asthma Indoor Air Exposures and Asthma ExacerbationExacerbation

Biological AgentsBiological Agents Sufficient evidence of a causal relationshipSufficient evidence of a causal relationship

• CatCat• CockroachCockroach• House dust miteHouse dust mite

Sufficient evidence of an associationSufficient evidence of an association• DogDog• Fungi/MoldsFungi/Molds• RhinovirusRhinovirus

Limited or Suggestive Evidence of an Limited or Suggestive Evidence of an Association Association • Domestic birdsDomestic birds• Chlamydia and Mycoplasma Chlamydia and Mycoplasma pneumoniaepneumoniae• RSVRSV

Chemical AgentsChemical Agents Sufficient evidence of a causal Sufficient evidence of a causal

relationshiprelationship• Environmental tobacco Environmental tobacco

smoke (in preschool-smoke (in preschool-aged children)aged children)

Sufficient evidence of an Sufficient evidence of an associationassociation

• NONO22, NO, NOxx (high (high levels)levels)

Limited or suggestive evidence Limited or suggestive evidence of an associationof an association

• Environmental Environmental tobacco smoke (school-tobacco smoke (school-aged, older aged, older children children and adults)and adults)

• FormaldehydeFormaldehyde• FragrancesFragrances

Medications to Treat AsthmaMedications to Treat Asthma

Medications come in a variety of forms.Medications come in a variety of forms. Two major categories of medications are:Two major categories of medications are:

• Long-term controlLong-term control• Quick reliefQuick relief

Medications to Treat Asthma:Medications to Treat Asthma:Long-Term ControlLong-Term Control

Taken daily, over a long period of Taken daily, over a long period of timetime

Used to reduce inflammation, Used to reduce inflammation, relax airway muscles, and improve relax airway muscles, and improve symptoms and pulmonary functionsymptoms and pulmonary function• Inhaled corticosteroidsInhaled corticosteroids

• Long-acting betaLong-acting beta22-agonists-agonists

• Leukotriene modifiersLeukotriene modifiers

Medications to Treat Asthma:Medications to Treat Asthma:Quick-ReliefQuick-Relief

Used in acute Used in acute asthma asthma episodesepisodes

Generally they Generally they are short-acting are short-acting betabeta22-agonists-agonists

Medications to Treat Asthma:Medications to Treat Asthma:How to Use a Spray InhalerHow to Use a Spray Inhaler

Health-care Health-care provider should provider should evaluate inhaler evaluate inhaler technique at technique at each visit.each visit.

Source: “What You and Your Family Can Do About Asthma” by the Global Initiative For Asthma Source: “What You and Your Family Can Do About Asthma” by the Global Initiative For Asthma Created and funded by NIH/NHLBICreated and funded by NIH/NHLBI

Medications to Treat Asthma:Medications to Treat Asthma:Inhalers and SpacersInhalers and Spacers

Inhalers

Spacers

Spacers can Spacers can help help patients patients who have who have difficulty difficulty with with technique technique and can and can reduce reduce potential potential side effects.side effects.

Medications to Treat Asthma:Medications to Treat Asthma:NebulizersNebulizers

Uses compressed Uses compressed air machine to air machine to deliver medicine deliver medicine as a mistas a mist

Good for small Good for small children or for children or for severe asthma severe asthma episodesepisodes

Managing Asthma:Managing Asthma:Asthma Management GoalsAsthma Management Goals

Control symptomsControl symptoms Prevent exacerbationPrevent exacerbation Maintain lung function as close to Maintain lung function as close to

normal as possiblenormal as possible Avoid adverse effects from Avoid adverse effects from

medicationsmedications Prevent irreversible airway obstructionPrevent irreversible airway obstruction Prevent asthma mortalityPrevent asthma mortality

Managing Asthma:Managing Asthma:Asthma Management PlanAsthma Management Plan

Develop with a physicianDevelop with a physician Tailor to meet individual needsTailor to meet individual needs Educate patients and families on Educate patients and families on all all aspects of the planaspects of the plan

Recognizing symptomsRecognizing symptoms Medication benefits and side Medication benefits and side

effectseffects Proper use of inhalers and peak Proper use of inhalers and peak

expiratory flow (PEF) expiratory flow (PEF) metersmeters

Managing Asthma:Managing Asthma:Indications of a Severe AttackIndications of a Severe Attack

Breathless at restBreathless at rest Hunched forwardHunched forward Talking in words rather than Talking in words rather than

sentencessentences AgitatedAgitated Peak flow rate is less than 60% of Peak flow rate is less than 60% of

normalnormal

ResourcesResources

National Asthma Education and Prevention National Asthma Education and Prevention ProgramProgram• http://www.nhlbi.nih.gov/about/naepp/index.htmhttp://www.nhlbi.nih.gov/about/naepp/index.htm

Asthma and Allergy Foundation of AmericaAsthma and Allergy Foundation of America• http://www.aafa.orghttp://www.aafa.org

American Lung Association American Lung Association • http://www.lungusa.org http://www.lungusa.org

American Academy of Allergy, Asthma, and American Academy of Allergy, Asthma, and ImmunologyImmunology• http://www.aaaai.orghttp://www.aaaai.org

Resources Resources

Allergy and Asthma Network, Mothers of Allergy and Asthma Network, Mothers of Asthmatics. Inc.Asthmatics. Inc.• http://www.aanma.org/http://www.aanma.org/

American College of Allergy, Asthma, and American College of Allergy, Asthma, and ImmunologyImmunology• http://allergy.mcg.eduhttp://allergy.mcg.edu

American College of Chest PhysiciansAmerican College of Chest Physicians• http://www.chestnet.orghttp://www.chestnet.org

American Thoracic SocietyAmerican Thoracic Society• http://www.thoracic.orghttp://www.thoracic.org

Cystic FibrosisCystic Fibrosis

CF is a hereditary disease that causes some CF is a hereditary disease that causes some glands to produce abnormal secretions that glands to produce abnormal secretions that results in tissue and organ damage. Lungs results in tissue and organ damage. Lungs and digestive tract appear to be affected and digestive tract appear to be affected the most.the most.

Most common inherited disease leading to a Most common inherited disease leading to a shortened life among white people in the US shortened life among white people in the US • 1:3,300 white infants1:3,300 white infants• 1:15,300 black infants1:15,300 black infants• Rare in AsiansRare in Asians• Found equally in boys and girlsFound equally in boys and girls

Cystic Fibrosis Cystic Fibrosis

Currently, there is no cure for CF, but Currently, there is no cure for CF, but there are many promising new there are many promising new treatments in use and even more on treatments in use and even more on the horizon. the horizon.

The median life expectancy for a The median life expectancy for a person with CF is now 32person with CF is now 32• thirty years ago, a CF patient was not thirty years ago, a CF patient was not

expected to reach adulthood. Many people expected to reach adulthood. Many people even live into their fifties and sixties.even live into their fifties and sixties.

Cystic FibrosisCystic Fibrosis

Cystic fibrosis results when a Cystic fibrosis results when a person inherits two defective person inherits two defective copies of a particular gene. copies of a particular gene. This gene controls the This gene controls the production of a protein that production of a protein that regulates the transport of regulates the transport of chloride and sodium across cell chloride and sodium across cell membranes. Worldwide, about membranes. Worldwide, about 3 of 100 white people carry one 3 of 100 white people carry one defective copy of the gene. defective copy of the gene. About 3 of 10,000 white people About 3 of 10,000 white people inherit two defective copies of inherit two defective copies of the gene; thus, they develop the gene; thus, they develop cystic fibrosis. In these people, cystic fibrosis. In these people, chloride and sodium transport chloride and sodium transport is disrupted and dehydration is disrupted and dehydration and increased stickiness of and increased stickiness of secretions occur.secretions occur.

The key to Cystic Fibrosis is clogging.

The affected areas of the body are the airways, liver, pancreas, intestine, and reproductive tract.

CF - SymptomsCF - Symptoms

The lungs are normal at birth, but breathing The lungs are normal at birth, but breathing problems can develop at any time afterward. Thick problems can develop at any time afterward. Thick secretions eventually block the small airways, secretions eventually block the small airways, which leads to inflammation and thickening of their which leads to inflammation and thickening of their walls. As larger airways fill with secretions, areas of walls. As larger airways fill with secretions, areas of the lung collapse and contract (a condition called the lung collapse and contract (a condition called atelectasis)atelectasis) and the lymph nodes enlarge. All these and the lymph nodes enlarge. All these changes make breathing increasingly difficult and changes make breathing increasingly difficult and reduce the lungs' ability to transfer oxygen to the reduce the lungs' ability to transfer oxygen to the blood. Respiratory tract infections occur because of blood. Respiratory tract infections occur because of bacterial growth in the bronchial secretions and bacterial growth in the bronchial secretions and walls of the airways.walls of the airways.

CF – Symptoms (cont)CF – Symptoms (cont)

The blockage of pancreatic ducts and The blockage of pancreatic ducts and intestinal glands leads to digestive problems, intestinal glands leads to digestive problems, including poor absorption of fats, proteins, and including poor absorption of fats, proteins, and vitamins. This, in turn, can lead to nutritional vitamins. This, in turn, can lead to nutritional deficiencies, and slower than expected growth. deficiencies, and slower than expected growth. Some people may have episodes of intestinal Some people may have episodes of intestinal obstruction when abnormal stool contents obstruction when abnormal stool contents block the bowel.block the bowel.

About 15 to 20% of newborns who have cystic About 15 to 20% of newborns who have cystic fibrosis have meconium ileus, a serious fibrosis have meconium ileus, a serious obstruction of the small intestine obstruction of the small intestine

CF - SymptomsCF - Symptoms

About half the children with cystic fibrosis are About half the children with cystic fibrosis are first taken to the doctor because of frequent first taken to the doctor because of frequent coughing, wheezing, and respiratory tract coughing, wheezing, and respiratory tract infections. Coughing, the most noticeable infections. Coughing, the most noticeable symptom, is often accompanied by gagging, symptom, is often accompanied by gagging, vomiting, and disturbed sleep. As the disease vomiting, and disturbed sleep. As the disease progresses, the chest becomes barrel-shaped, progresses, the chest becomes barrel-shaped, and insufficient oxygen may make the fingers and insufficient oxygen may make the fingers clubbedclubbed and the nail beds bluish. Polyps may and the nail beds bluish. Polyps may form in the nose. The sinuses may fill with thick form in the nose. The sinuses may fill with thick secretions, leading to chronic or recurrent sinus secretions, leading to chronic or recurrent sinus infections.infections.

CF - SymptomsCF - Symptoms

When a child or adult with cystic fibrosis When a child or adult with cystic fibrosis sweats excessively in hot weather or because sweats excessively in hot weather or because of a fever, dehydration may result because of of a fever, dehydration may result because of the increased loss of salt and water. A parent the increased loss of salt and water. A parent may notice the formation of salt crystals or may notice the formation of salt crystals or even a salty taste on the child's skin.even a salty taste on the child's skin.

Adolescents often have slowed growth, Adolescents often have slowed growth, delayed puberty, and declining physical delayed puberty, and declining physical endurance. As the disease progresses, lung endurance. As the disease progresses, lung infection becomes a major problem. infection becomes a major problem. Recurrent bronchitis and pneumonia Recurrent bronchitis and pneumonia gradually destroy the lungs.gradually destroy the lungs.

CF - ComplicationsCF - Complications

About 15% of adults with cystic About 15% of adults with cystic fibrosis develop insulin-dependent fibrosis develop insulin-dependent diabetes because the scarred diabetes because the scarred pancreas can no longer produce pancreas can no longer produce enough insulin. The blockage of bile enough insulin. The blockage of bile ducts by thick secretions can lead to ducts by thick secretions can lead to inflammation of the liver and inflammation of the liver and eventually to scarring of the liver eventually to scarring of the liver (cirrhosis) in about 5% of adults with (cirrhosis) in about 5% of adults with cystic fibrosiscystic fibrosis

CF – Complications (cont)CF – Complications (cont)

People with cystic fibrosis often have impaired People with cystic fibrosis often have impaired reproductive function. Almost all men have a low reproductive function. Almost all men have a low sperm count (which makes them sterile) because one sperm count (which makes them sterile) because one of the ducts of the testis (the vas deferens) has of the ducts of the testis (the vas deferens) has developed abnormally and blocks the passage of developed abnormally and blocks the passage of sperm. In women, cervical secretions are too thick, sperm. In women, cervical secretions are too thick, causing decreased fertility. Otherwise, sexual function causing decreased fertility. Otherwise, sexual function is not affected. Women with cystic fibrosis have a is not affected. Women with cystic fibrosis have a higher likelihood of complications during pregnancy higher likelihood of complications during pregnancy (such as developing a lung infection or diabetes), but (such as developing a lung infection or diabetes), but many women with cystic fibrosis have given birth.many women with cystic fibrosis have given birth.

Other complications may include arthritis, kidney Other complications may include arthritis, kidney stones, and inflammation of the blood vessels stones, and inflammation of the blood vessels (vasculitis).(vasculitis).

CF - TreatmentCF - Treatment

Advances in antibiotic therapy, nutritional support, and Advances in antibiotic therapy, nutritional support, and chest physiotherapy have markedly increased survival chest physiotherapy have markedly increased survival in people with CF in people with CF

Heart-lung transplantation has been applied to people Heart-lung transplantation has been applied to people with CF with CF

5-year survival rates has reached more than 50% in 5-year survival rates has reached more than 50% in some centers some centers

People with CF require daily chest physiotherapy to People with CF require daily chest physiotherapy to loosen secretions and prevent stagnation and loosen secretions and prevent stagnation and secondary infections secondary infections

Antibiotics are essential in treating infection, often Antibiotics are essential in treating infection, often given intravenously given intravenously

Nutritional support can also be given intravenously in Nutritional support can also be given intravenously in people who are malnourished people who are malnourished

CF – Voc & Psych CF – Voc & Psych ImplicationsImplications

People with CF have excellent educational People with CF have excellent educational success and are typically productive individuals success and are typically productive individuals

The counselor will have to work with employers to The counselor will have to work with employers to provide the support mechanisms that will allow provide the support mechanisms that will allow the person to remain in the workplace the person to remain in the workplace

This may include the provision of time for chest This may include the provision of time for chest physiotherapy or antibiotic treatment during the physiotherapy or antibiotic treatment during the workday workday

The work environment must be reviewed to The work environment must be reviewed to ensure the absence of irritants that might ensure the absence of irritants that might exacerbate the disease exacerbate the disease

CF – Voc & Psych CF – Voc & Psych ImplicationsImplications

Supplemental oxygen may be necessary to allow Supplemental oxygen may be necessary to allow the person the continue to be productive and the person the continue to be productive and ambulatory ambulatory

Psychological outcome in people with CF appears Psychological outcome in people with CF appears to depend on factors such as altered physical to depend on factors such as altered physical appearance, loneliness, and family strife that the appearance, loneliness, and family strife that the person may attribute to his or her illness person may attribute to his or her illness

The counselor can also work with the person's The counselor can also work with the person's family to improve support at home that will allow family to improve support at home that will allow the person to increase social and vocational the person to increase social and vocational activities activities

ReferencesReferences

American Thoracic Society (www.thoracic.org) American Thoracic Society (www.thoracic.org) American Lung Association (www.lungusa.org) American Lung Association (www.lungusa.org) American Lung Association of Florida (www.lungfla.org) American Lung Association of Florida (www.lungfla.org) American Association of Cardiovascular and Pulmonary American Association of Cardiovascular and Pulmonary Rehabilitation (www.aacvpr.org) Rehabilitation (www.aacvpr.org) Asthma Information Center Asthma Information Center (http://cooke.gsf.de/asthmainfocenter/home.cfm) (http://cooke.gsf.de/asthmainfocenter/home.cfm) Asthma and Allergy Foundation of America Asthma and Allergy Foundation of America (www.aafa.org) (www.aafa.org) Allergy and Asthma Network (www.aanma.org) Allergy and Asthma Network (www.aanma.org) COPD-Support, Inc. (www.copd-support.com) COPD-Support, Inc. (www.copd-support.com) Cystic Fibrosis Foundation (www.cff.org) Cystic Fibrosis Foundation (www.cff.org) Cystic Fibrosis Research, Inc. (www.cfri.org/home.htm) Cystic Fibrosis Research, Inc. (www.cfri.org/home.htm) Cystic Fibrosis Resources (www.cysticfibrosis.com) Cystic Fibrosis Resources (www.cysticfibrosis.com)