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EMPHYSEMA
CHRONIC BRONCHITIS
ASTHAMATIC BRONCHITIS
BRONCHIECTASIS
CYSTIC FIBROSIS
NOTE :-
EMPHYSEMA & CHRONIC BRONCHITIS comes under
chronic obstructive pulmonary disease hence they are shown under
a common heading COPD.
PLEASE CONSIDER
DEFINITION
CHRONIC BRONCHITIS :-
EMPHYSEMA :-
It is defined as chronic respiratory disease where there is
over-inflation of the air sacs (alveoli) in the lungs, causing a decrease in
lung function, and often, breathlessness.
ASTHMATIC BRONCHITIS :-
It is defined as acute episode of airway obstruction is
characterized by airway hyperactivity to various stimuli that results in
recurrent wheezing brought about by edema and bronchospasm.
BRONCHIECTASIS:-
It is defined as a condition that is characterised by the
permanent dilatation of the bronchi associated with destruction of elastic
and muscular components of their walls, usually due to acute or chronic
infection.
CYSTIC FIBROSIS :-
It is defined as a hereditary disorder of exocrine glands,
with high sodium chloride content in sweat and pancreatic insufficiency,
resulting in mal-absorption. There is hypertrophy and hyperplasia of
mucus-secreting glands, resulting in excessive mucus production in the
lining of bronchi, which predisposes the patient to chronic
bronchopulmonary infection.
Anatomy & Physiology of the
Respiratory System
The nose, pharynx, larynx, trachea and bronchi all work like a system of
pipes through which the air is funnelled down into our lungs. There, in
very small air sacs called alveoli, oxygen is brought into the bloodstream
and carbon dioxide is pushed from the blood out into the air.
The Upper Airway and Trachea
During breathing, air enters into body through nose or
mouth. From there, it travels down your throat through the larynx
(or voice box) and into the trachea (or windpipe) before entering
into the lungs. All these structures act to funnel fresh air down from
the outside into the body.
The upper airway is important because it must always stay
open to be able to breathe. It also helps to moisten and warm the
air before it reaches the lungs.
The Lungs
Structure
The lungs are paired, cone-shaped organs which take up most of
the space in our thorax, along with the heart.
Their role is to take oxygen into the body, which we need for our
cells to live and function properly, and to help us get rid of carbon
dioxide, which is a waste product.
We have two lungs, a left lung and a right lung. These are divided
up into 'lobes', or big sections of tissue separated by 'fissures' or
dividers. The right lung has three lobes but the left lung has only
two, because the heart takes up some of the space in the left side
of our chest.
The lungs can also be divided up into even smaller portions, called
'bronchopulmonary segments'. These are pyramidal-shaped areas
which are also separated from each other by membranes. There
are about 10 of them in each lung. Each segment receives its own
blood supply and air supply.
Physiology of Breathing
When a person inhales, air travels through the following pathways
into the lungs:
Air is carried from the trachea (the windpipe) into the lung through
flexible airways called bronchi.
Like the branches of a tree, bronchi divide successively into over a
million smaller airways called bronchioles.
The bronchioles lead to grape-like clusters of microscopic sacs
called alveoli.
In each adult lung there are millions of these tiny alveoli. The thin
membrane of the alveoli allows oxygen and carbon dioxide to pass
to and from capillaries.
During deep inhalation, the elastic alveoli unfold and unwind
to allow this passage to occur
Capillaries, the smallest of our blood vessels, carry blood
throughout the body. Red blood cells carry oxygen throughout the
body, and return carbon dioxide to the lungs; white blood cells are
the critical infection fighters in our body.
Mechanics of Breathing
To take a breath in, the external intercostal muscles
contract, moving the ribcage up and out.
The diaphragm moves down at the same time, creating
negative pressure within the thorax.
The lungs are held to the thoracic wall by the pleural
membranes, and so expand outwards as well.
This creates negative pressure within the lungs, and so air
rushes in through the upper and lower airways.
Expiration is mainly due to the natural elasticity of the
lungs, which tend to collapse if they are not held against
the thoracic wall.
This is the mechanism behind lung collapse if there is air
in the pleural space (pneumothorax).
EPIDEMIOLOGY
Chronic Obstructive Pulmonary Disease (COPD) is one of the
leading causes of morbidity and mortality in the industrialized and the
developing countries.
Age:-
COPD develops slowly over years, so most people are
at least 40 years old when symptoms begin.
Genetics:-
A rare genetic disorder known as alpha-1-
antitrypsin deficiency is the source of a few cases of COPD.
Researchers suspect that other genetic factors may also
make certain smokers more susceptible to the disease.
Cigarette smoking:-
The primary cause of COPD is exposure to tobacco
smoke Clinically significant COPD develops in 15% of
cigarette smokers.
Age of initiation of smoking, total pack-years, and current
smoking status predict COPD mortality. People who smoke
have a greater annual decline in FEV1. Overall, tobacco
smoking accounts for as much as 90% of the risk.
Second hand smoke, or environmental tobacco smoke,
increases the risk of respiratory infections, augments asthma
symptoms, and causes a measurable reduction in pulmonary
function.
Air pollution:-
Although the role of air pollution in the etiology of COPD is
unclear, the effect is small when compared to cigarette
smoking.
The use of solid fuels for cooking and heating may result in
high levels of indoor air pollution and the development of
COPD.
Airway hyper responsiveness:-
Airway hyper responsiveness (i.e., Dutch hypothesis)
stipulates that patients who have nonspecific airway hyper
reactivity and who smoke are at increased risk of developing
COPD with an accelerated decline in lung function.
Nonspecific airway hyper reactivity is inversely related to
FEV1 and may predict a decline in lung function.
The possible role of airway hyper responsiveness as a risk
factor for the development of COPD in people who smoke is
unclear. Moreover, bronchial hyper reactivity may result from
airway inflammation observed with the development of
smoking-related chronic bronchitis.
Alpha1-antitrypsin deficiency:-
AAT deficiency is the only known genetic risk factor for
developing COPD and accounts for less than 1% of all cases
in the United States. AAT is a protease inhibitor produced by
the liver that acts predominantly by inhibiting neutrophil
elastase in the lungs.
CLASSIFICATION
Emphysema can be classified into primary and secondary.
However, it is more commonly classified by location.
Emphysema can be subdivided into panacinary and centroacinary
(or panacinar and centriacinar, or centrilobular and panlobular).
Panacinary (or panlobular) emphysema is related to the destruction
of alveoli, because of an inflammation or deficiency of alpha 1-
antitrypsin. It is found more in young adults who do not have
chronic bronchitis.
Centroacinary (or centrilobular) emphysema is due to destruction of
terminal bronchiole mucosis, due to chronic bronchitis. This is found
mostly in elderly people with a long history of smoking or extreme
cases of passive smoking.
Other types include distal acinar and irregular.
A special type is congenital lobar emphysema (CLE).
Congenital lobar emphysema
CLE is results in overexpansion of a pulmonary lobe and resultant
compression of the remaining lobes of the ipsilateral lung, and
possibly also the contra lateral lung. There is bronchial narrowing
because of weakened or absent bronchial cartilage.
CLE is potentially reversible, yet possibly life-threatening, causing
respiratory distress in the neonate.
PATHOLOGY
Airway Pathology in COPD Pathway
The pathological hallmarks of COPD are destruction of the lung
parenchyma, which characterizes emphysema, inflammation of the
peripheral airways, which characterizes bronchiolitis, and inflammation
of the central airways which characterizes chronic bronchitis.
Functional consequence of these abnormalities is expiratory airflow
limitation.
As flow is the result of a driving pressure (elastic recoil of the lung) and
of an opposing resistance (airway obstruction), it is best to refer to the
changes in flow seen in smokers as airflow limitation, rather than airflow
obstruction, since both loss of elastic recoil and increase in airway
resistance play an important role in the observed decrease in flow .
Emphysema will contribute to the airflow limitation by reducing the
elastic recoil of the lung through parenchyma destruction, as well as by
reducing the elastic load applied to the airways through destruction of
alveolar attachments.
On the other hand, bronchiolitis will contribute to the airflow limitation by
narrowing and obliterating the lumen and by actively constricting the
airways. The role of symptoms of chronic bronchitis in the development
of chronic airflow limitation is still controversial. In fact, chronic sputum
production has traditionally been considered to be irrelevant to the
development of chronic airflow limitation.
However, a recent study has shown that chronic sputum production was
significantly associated with both an increased decline in FEV1 and an
increased risk of subsequent hospitalization because of COPD,
suggesting a causal role of mucus hyper secretion in the development of
chronic airflow limitation in smokers.
PATHOGENESIS
Chronic obstructive pulmonary disease
(COPD) is characterized and defined by limitation of expiratory
airflow.
This can result from several types of anatomical lesions, including
loss of lung elastic recoil and fibrosis and narrowing of small
airways.
Inflammation, oedema, and secretions also contribute variably to
airflow limitation. Smoking can cause COPD through several
mechanisms.
First, smoke is a powerful inducer of an inflammatory response.
Inflammatory mediators, including oxidants and proteases, are
believed to play a major role in causing lung damage.
Smoke can also alter lung repair responses in several ways.
Inhibition of repair may lead to tissue destruction that
characterizes emphysema, whereas abnormal repair can lead to
the peribronchiolar fibrosis that causes airflow limitation in small
airways.
Genetic factors likely play a major role and probably account for
much of the heterogeneity susceptibility to smoke and other
factors. Many factors may play a role, but to date, only alpha-1
protease inhibitor deficiency has been unambiguously identified.
Exposures other than cigarette smoke can contribute to the
development of COPD. Inflammation of the lower respiratory tract
that results from asthma or other chronic disorders may also
contribute to the development of fixed airway obstruction.
COPD is not only a disease of the lungs but is also a systemic
inflammatory disorder. Muscular weakness, increased risk for
atherosclerotic vascular disease, depression, osteoporosis, and
abnormalities in fluids and electrolyte balance may all be
consequences of COPD.
Barrel-chest :-
One telling sign is the change in the shape of the chest, known as
barrel chest.
When the lungs become enlarged, the diaphragm is displaced
downward and able to contract efficiently.
Pursed-lip breathing :-
Because airflow out of the lungs becomes limited,
exhalation takes longer. Because the alveoli lose their elasticity,
one tries to shorten the time needed for exhalation by forcefully
exhaling.
Productive cough :-
A productive cough is caused by inflammation and
excessive amounts of mucus in the airways. Coughing becomes
less effective because of
obstructed airflow.
Cyanosis :-
People who have a poor supply of oxygen usually have a
bluish tinge to their skin, lips, nail beds. Called cyanosis.
Symptoms
Shortness of Breath (Dyspnea)
Chronic Cough:-
Chronic cough typically begins as morning cough
and slowly progresses to an all-day cough.
The cough usually produces small amounts of
sputum (less than 60ml/day) and is clear or whitish but may be
discolored.
Sputum production decreases when one quits smoking.
Wheezing :-
It is a high pitched sound of air passing through narrowed
airways. A person with copd may wheeze during on acute
exacerbation or chronically.
Sometimes the wheezing is heard only at night or with exertion.
Hemoptysis :-
COPD is one of the more common causes of hemoptysis
(coughing up blood).
Usually, there are only very small amounts of blood streaking the
sputum. Hemoptysis may be a sign of lung cancer in a patient with
COPD, so any blood appearing in the sputum should be brought to
a doctor's attention.
Weight Loss :-
Patient with COPD work hard and burn a lot of calories just
breathing.
Lower Extremity Edema :-
In severe cases of COPD. Pulmonary artery pressures increase
and the right ventricle of the heart contracts less efficiently.
When the heart is unable to pump enough blood to meet the needs
of the kidneys and liver, edema (swelling) in the feet, ankles, and
lower legs results.
Physical
Depending on the type of COPD, physical examination may vary.
Stages of COPD
1. Cor Pulmonale
Cor Pulmonale is caused by an increase in blood pressure in the
pulmonary artery, the vessel that carries blood from the heart to the
lungs. This leads to enlargement and subsequent failure of the right side
of the heart.
Normally, the blood flows from the heart to pass through the lungs,
where blood cells pick up oxygen and deliver it to the body.
People who have COPD are at greater risk for pneumothorax because
the structure of their lungs is weak and vulnerable to the spontaneous
development of these types of holes
5. Secondary Polycythemia
When too many red blood cells are produced, the blood becomes thick,
hindering its passage through the smaller blood vessels.
6. Respiratory Failure
Pulmonary function tests are the primary diagnostic tools for COPD.
Lung biopsy is rarely used to diagnose emphysema.
There are four components to pulmonary function testing:
Spirometry,
Post bronchodilator spirometry
Lung volumes and
Diffusion capacity.
• Spirometry: -
The most reliable way to determine reversible airway
obstruction is with spirometry.
• It is a simple test procedure that measures the amount of air
entering and leaving the lungs.
• With the patient sitting comfortably in front of the spirometry
machine. The machine measures airflow that passes through the
inhalation port attached to the machine. The inhalation device is
usually a disposable cardboard tube or a reusable tube that can be
sterilized after use.
PREVENTION
Quitting smoking
Avoiding respiratory irritants and infections
Avoiding allergens
Maintaining good nutrition
Drinking lots of fluids
Avoiding excessively low or high temperatures
Very high altitudes
Maintaining proper weight,
Exercising to increase muscle tone.
Avoid smoking tobacco or exposure to second hand tobacco smoke.
Smoking is the leading cause of COPD. Although you cannot undo the
damage that smoking has already caused, you can prevent further lung
damage by quitting. Avoiding conditions that may irritate the lungs can
reduce breathing problems in people with COPD. These conditions
include indoor and outdoor air pollution; smog; cold, dry air; hot, humid
air; or high altitudes. Avoiding respiratory illnesses, such as the flu
(influenza) and pneumonia, can decrease the risk of your COPD
worsening. Talk with your doctor about getting vaccinations
against them. Use appropriate protective gear (e.g. face mask) in the
workplace to avoid inhaling hazardous substance. Get plenty of physical
activity for good lung health. If you already have COPD, avoid colds and
flu’s, which can worsen the disease. Get annual flu and pneumococcal
vaccinations to avoid such infections. Quitting smoking is the most
important thing you can do to prevent or slow damage to your lungs.
CLINICAL MANAGEMENT
Non-drug treatment
• Advice on how to respond promptly to symptoms of an
exacerbation, including starting oral corticosteroid therapy,
starting antibiotic therapy if their sputum is purulent and adjusting
their bronchodilator therapy to control their symptoms.
• Advice on when and how to contact a health care professional if
symptoms do not improve.
• Smoking cessation: an up to date smoking history, including pack
years smoked (number of cigarettes smoked per day, divided by
20, multiplied by the number of years smoked), should be
documented for everyone with COPD. An assessment of their
"readiness to change" should also be made.2
• Nutrition: BMI should be calculated. If the BMI is abnormal (high
or low), or changing over time, the patient should be referred for
dietetic advice. If the BMI is low, patients should also be given
nutritional supplements to increase their total calorific intake, and
be encouraged to take exercise to augment the effects of
nutritional supplementation.
Drug therapy
Bronchodilator therapy:
• Long-acting bronchodilators are not suitable for the relief of
acute bronchospasm but may have additional benefits over
combinations of short-acting drugs. However they may also
have additional side effects:
• Long acting beta2 agonists:
○ The use of long term beta2 agonists in the absence of
inhaled steroids appears to carry an increased incidence of
death or near death complications in some groups.3
○ Recent research has also suggested that patients taking
long acting beta2 agents also appear to have more
difficulties during an exacerbation due to down regulation
of the receptors.
○ Therefore the role of long acting beta2 agonists in the
management of COPD is currently being re-evaluated.
• Tiotropium (a long-acting anticholinergic bronchodilator):4
○ Is effective in controlling symptoms and improve exercise
capacity in patients who continue to experience problems
despite the use of short-acting drugs.
○ Tiotropium reduces COPD exacerbations and hospital
admissions and improves health-related quality-of-life in
patients with moderate and severe disease.
○ Tiotropium possibly slows the decline in FEV1.
○ Additional long-term studies are required to evaluate its
effect on mortality and change in FEV1, to confirm its role
compared to, or in combination with, long-acting beta2-
agonists, and to assess its effectiveness in mild and very
severe COPD.
• Mucolytic drug therapy: should be considered in patients with a
chronic cough productive of sputum and continued if there is
symptomatic improvement (e.g. reduction in frequency of
cough and sputum production).
• Theophylline: should only be used after a trial of short-acting
bronchodilators and long-acting bronchodilators, or in patients
who are unable to use inhaled therapy.
• Phosphodiesterase type 4 inhibitors: there is insufficient long-
term data on which to base any evidence statements or
recommendations.
• Inhaled corticosteroids:
○ None of the inhaled corticosteroids currently available are
licensed for use alone in the treatment of COPD.
○ Oral corticosteroid reversibility tests do not predict
response to inhaled corticosteroid therapy.
○ Inhaled corticosteroids should be prescribed for patients
with an FEV1 50% or less of predicted, who are having 2 or
more exacerbations requiring treatment with antibiotics or
oral corticosteroids in a 12 month period.
○ The aim of treatment is to reduce exacerbation rates and
slow the decline in health status and not necessarily to
improve lung function.
• Oral corticosteroids:
○ Maintenance use of oral corticosteroid therapy in COPD is
not normally recommended. If oral corticosteroids cannot
be withdrawn following an exacerbation, the dose of oral
corticosteroids should be kept as low as possible.
○ Patients treated with long term oral corticosteroid therapy
should be monitored for the development of osteoporosis.
• Combination therapy:
○ If patients remain symptomatic on monotherapy, effective
combinations include:
Beta 2-agonist and anticholinergic
Beta 2-agonist and theophylline
Anticholinergic and theophylline
Long-acting beta 2-agonist and inhaled corticosteroid
• Combination treatment should be discontinued if there is no
benefit after 4 weeks.
• Delivery systems:
○ In most cases bronchodilator therapy is best administered
using a hand held inhaler device (including a spacer device
if appropriate).
○ There is no evidence to suggest superiority of nebulised
therapy over the use of an MDI with a spacer device.
• Non-invasive ventilation:
○ Adequately treated patients with chronic hypercapnia
ventilator failure who have required assisted ventilation
(whether invasive or non-invasive) during an exacerbation
or who are hypercapnia or acidosis on oxygen therapy
should be referred to a specialist centre for consideration
of long-term NIV.
• Treatments not recommended include anti-oxidant therapy with
alpha-tocopherol and beta-carotene supplements, anti-tussive
therapy and prophylactic antibiotic therapy.
Palliative care
• Opioids should be used when appropriate to palliate
breathlessness in patients with end-stage COPD which is
unresponsive to other medical therapy.
• Benzodiazepines, tricycle antidepressants, major tranquillisers and
oxygen should also be used when appropriate for breathlessness
in patients with end stage COPD unresponsive to other medical
therapy.
• Patients with end stage COPD and their family and carers should
have access to the full range of services offered by multidisciplinary
palliative care teams, including admission to hospices.
ASTHMA
AETIOLOGY
The aetiology of asthma is complex, and multiple environmental
and genetic determinants are implicated.
➢ Living in farms, large families, childhood infections, including
parasites, exposure to pets in early life.
➢ Obesity may also increase the risk of asthma.
➢ Smokers may be at high risk.
PATHOPHYSIOLOGY
Airway hyper reactivity is integral to diagnosis of asthma.
Others factors are the behaviour of airway smooth muscles,
degree of airway narrowing and influence of neurogenic
mechanism.
TYPES OF ASTHMA
CLINICAL FEATURES
INSVESTIGATIONS
➢ Asthma is diagnosed by the characteristic pattern of symptoms.
➢ A peak flow meter can record variations in the severity of asthma
over time.
➢ Spirometry, a measurement of lung function, can provide an
assessment of the severity, reversibility, and variability of airflow
limitation, and help confirm the diagnosis of asthma.
➢ An elevated sputum or peripheral blood eosinopil count may be
observed.
➢ In radiological examination of acute asthma hyperinflation and
lobar collapse may be seen.
➢ Induced sputum and exhaled breath allow the non invasive
assessment of airway inflammation.
➢ In emergency department doctors use capnography which
measures the amount of exhaled carbon dioxide along with pulse
oxymetry.
➢ More recently, exhaled nitric oxide has been studied as breath test
indicative of airway inflammation in asthma.
MEDICAL TREATMENT
BRONCHIECTASIS
PATHOGENSIS
TYPES OF BROCHIECTASIS
CAUSES
DIAGNOSIS
➢ The diagnosis of bronchiectasis is based on the review of clinical
history and characteristic patterns in high-resolution CT scan
findings.
➢ Such patterns include "tree-in-bud" abnormalities and cysts with
definable borders. In one small study, CT findings of bronchiectasis
and multiple small nodules were reported to have a sensitivity of
80%, specificity of 87%, and accuracy of 80% for the detection of
bronchiectasis.
➢ Bronchiectasis may also be diagnosed without CT scan
confirmation if clinical history clearly demonstrates frequent,
respiratory infections, as well confirmation of an underlying
problem via blood work and sputum culture samples.
COMPLICATIONS
• Recurrent haemoptysis
• Pneumonia
• Pleurisy and empyema
• Respiratory failure
• Right ventricular failure
• Emphysema and systemic amyloidosis are rare
TREATMENT
➢ Treatment of bronchiectasis is aimed at controlling infections and
bronchial secretions, relieving airway obstruction, and preventing
complications.
➢ This includes the prolonged usage of antibiotics to prevent
detrimental infections, as well as eliminating accumulated fluid with
postural drainage and chest physiotherapy.
➢ Surgery may also be used to treat localized bronchiectasis,
removing obstructions that could cause progression of the disease.
➢ Inhaled steroid therapy that is consistently adhered to can reduce
sputum production and decrease airway constriction over a period
of time, and help prevent progression of bronchiectasis.
➢ One commonly used therapy is beclometasone dipropionate,
which is also used in asthma treatment.
➢ Use of inhalers such as albuterol (salbutamol), fluticasone
(Flovent/Flixotide) and ipratropium (Atrovent) may help reduce
likelihood of infection by clearing the airways and decreasing
inflammation.
PREVENTION
➢ In order to prevent future development of bronchiectasis, an x-ray
of the chest should be taken after any severe attack of measles,
whooping cough or other acute respiratory infection in childhood.
CYSTIC FIBROSIS
➢ Cystic fibrosis (also known as CF, mucovoidosis, or
mucoviscidosis) is a genetic disorder known to be an inherited
disease of the secretory glands, including the glands that make
mucus and sweat.
➢ The hallmarks of cystic fibrosis are salty tasting skin, normal
appetite but poor growth and poor weight gain, excess mucus
production, and coughing/shortness of breath. Males can be
infertile due to the condition Congenital absence of the vas
deferens. Often, symptoms of CF appear in infancy and childhood.
➢ Meconium ileus is a typical finding in newborn babies with CF.
➢ Although technically a rare disease, cystic fibrosis is ranked as one
of the most widespread life-shortening genetic diseases.
CAUSES
➢ Cystic Fibrosis has an autosomal recessive pattern of inheritance.
➢ CF is caused by a mutation in the gene cystic fibrosis
transmembrane conductance regulator (CFTR). The product of this
gene is a chloride ion channel important in creating sweat,
digestive juices and mucus.
➢ Although most people without CF have two working copies
(alleles) of the CFTR gene, only one is needed to prevent cystic
fibrosis. CF develops when neither allele can produce a functional
CFTR protein.
➢ Therefore, CF is considered an autosomal recessive disease.
PATHOPHYSIOLOGY
➢ The protein created by this gene is anchored to the outer
membrane of cells in the sweat glands, lungs, pancreas, and other
affected organs.
➢ The protein spans this membrane and acts as a channel
connecting the inner part of the cell (cytoplasm) to the surrounding
fluid.
➢ This channel is primarily responsible for controlling the movement
of chloride from inside to outside of the cell; however, in the sweat
ducts it facilitates the movement of chloride from the sweat into the
cytoplasm.
➢ When the CFTR protein does not work, chloride is trapped inside
the cells in the airway and outside in the skin.
➢ Because chloride is negatively charged, positively charged ions
cross into the cell because they are affected by the electrical
attraction of the chloride ions.
➢ Sodium is the most common ion in the extracellular space and the
combination of sodium and chloride creates the salt, which is lost
in high amounts in the sweat of individuals with CF. This lost salt
forms the basis for the sweat test.
➢ One theory suggests that the lack of chloride exodus through the
CFTR protein leads to the accumulation of more viscous, nutrient-
rich mucus in the lungs that allows bacteria to hide from the body's
immune system.
➢ Another theory proposes that the CFTR protein failure leads to a
paradoxical increase in sodium and chloride uptake, which, by
leading to increased water reabsorption, creates dehydrated and
thick mucus.
➢ Yet another theory focuses on abnormal chloride movement out of
the cell, which also leads to dehydration of mucus, pancreatic
secretions, biliary secretions, etc.
➢ These theories all support the observation that the majority of the
damage in CF is due to blockage of the narrow passages of
affected organs with thickened secretions.
➢ These blockages lead to remodelling and infection in the lung,
damage by accumulated digestive enzymes in the pancreas,
blockage of the intestines by thick faeces, etc
SYMPTOMS
DIAGNOSIS
CF can be diagnosed at birth, but most often is diagnosed during
the early childhood years in young children (by the age of 3 years)
who have had a history of respiratory infections, excessive fat in
their stools, and who have poor weight gain.
Nearly 8 percent of people with CF are diagnosed at 18 years of
age or older because they have experienced only mild symptoms
of CF.
CF's major symptoms is respiratory infection, a CF diagnosis
sometimes may be confused with other respiratory conditions such
as asthma, pneumonia, or chronic bronchitis.
Genetic Testing
Couples planning a family may decide to have themselves
tested if one or both have a family history of CF
Sweat Test
The sweat test is an accurate, safe, and painless way to
diagnose CF. In the sweat test, a small electric current is used to
carry the chemical pilocarpine into the skin of the forearm. This
stimulates sweat glands in the area to produce sweat. Over a
period of 30 to 60 minutes, sweat is collected on filter paper (or
gauze) and tested for chloride. A chloride reading of more than 60
mEq/L points to CF.
COMPLICATIONS
• Haemoptysis
• Spontaneous pneumothorax
• Osteoporosis
• Liver diseases
• Diabetes mellitus
• Deformities like kyphosis and lordosis
• Cor Pulmonale
MANAGEMENT
The cornerstones of management are proactive treatment of
airway infection, and encouragement of good nutrition and an
active lifestyle.
➢ Targets for therapy are the lungs, gastrointestinal tract (including
insulin treatment and pancreatic enzyme supplements), the
reproductive organs (including Assisted Reproductive Technology
(ART)) and psychological support.
➢ In addition, therapies such as transplantation and gene therapy
aim to cure some of the effects of cystic fibrosis.
➢ Gene therapy aims to introduce normal CFTR to airway.
Theoretically this process should be simple as the airway is easily
accessible and there is only a single gene defect to correct.
➢ However there are some problems associated with these methods
involving efficiency (liposome’s insufficient protein) and delivery
(virus provokes an immune response).
➢ The most consistent aspect of therapy in cystic fibrosis is limiting
and treating the lung damage caused by thick mucus and infection
with the goal of maintaining quality of life.
➢ Intravenous, inhaled, and oral antibiotics are used to treat chronic
and acute infections.
➢ Mechanical devices and inhalation medications are used to alter
and clear the thickened mucus
➢ Antibiotics to treat lung disease
• Many CF patients are on one or more antibiotics at all times,
even when they are considered healthy, to suppress the
infection as much as possible.
• Many bacteria common in cystic fibrosis are resistant to
multiple antibiotics and require weeks of treatment with
intravenous antibiotics such as vancomycin, tobramycin,
meropenem, ciprofloxacin, and piperacillin.
• Inhaled therapy with antibiotics such as tobramycin and
colistin is often given for months at a time in order to improve
lung function by impeding the growth of colonized bacteria.
• Inhaled therapy with the antibiotic aztreonam is also being
developed and clinical trials have shown great promise.
• Oral antibiotics such as ciprofloxacin or azithromycin are
given to help prevent infection or to control ongoing infection.
• Some individuals spend years between hospitalizations for
antibiotics, whereas others require several antibiotic
treatments each year.
➢ Transplantation and gene therapy
• Lung transplantation often becomes necessary for individuals
with cystic fibrosis as lung function and exercise tolerance
declines.
• Although single lung transplantation is possible in other
diseases, individuals with CF must have both lungs replaced
because the remaining lung would contain bacteria that could
infect the transplanted lung.
• A pancreatic or liver transplant may be performed at the same
time in order to alleviate liver disease and/or diabetes.
• Gene therapy holds promise as a potential avenue to cure
cystic fibrosis.
• Gene therapy attempts to place a normal copy of the CFTR
gene into affected cells. Studies have shown that to prevent the
lung manifestations of cystic fibrosis, only 5–10% the normal
amount of CFTR gene expression is needed.
• Ideally, transferring the normal CFTR gene into the affected
epithelium cells would result in the production of functional
CFTR in all target cells, without adverse reactions or an
inflammation response.
PT ASSESSMENT
PATIENT PROFILE:
NAME:
AGE/SEX:
ADDRESS:
SUBJECTIVE ASSESSMENT:
OCCUPATION:
CHIEF COMPLIANT:
PRESENT HISTORY:
PAST HISTORY:
PAST MEDICAL HISTORY:
PRESENT MEDICAL HISTORY:
FAMILYHISTORY:
PRESONAL HISTORY:
OCCUPATIONAL HISTORY:
SOCIO-ECONOMICAL HISTORY:
OBJECTIVE ASSESSMENT:
ON OBSERVATION:
BUILT
POSITION OF PATIENT
POSTURE
BREATHING PATTERN
CLUBBING
CYANOSIS
CHEST WALL DEFORMITIES
OEDEMA
TROPICAL CHANGES
RESPIRATORY MUSCLES
DYSPNOEA
SPUTUM
HAEMOPTYSIS
CHEST PAIN
SKELETAL MOBILITY
ON EXAMINATION
VITAL SIGNS
TEMPERATURE
RESPIRATORY RATE
PULSE RATE BLOOD PRESSURE
ON PALPATION
PRESENCE OF NODULES
TENDERNESS
WARMTH
SWELLING
CHECK THE INSPIRATORY EFFORT
COUGH REFLEX
FREMITUS
ON AUSCULTATION
COARSE CREPITATIONS
WHEEZES
CHEST MEASUREMENTS
LEVEL OF FOURTH COSTAL
CARTILAGE
XIPHISTERNUM
NINTH COSTAL CARTILAGE
PHYSIOTHERAPY MANAGEMENT :-
Chronic obstructive pulmonary disease (COPD) is characterised by
intractable dyspnoea, reduced functional capacity and episodes of acute
exacerbation.
Physiotherapy plays a key role in multidisciplinary interventions. The
evidence in relation to airway clearance, pulmonary rehabilitation,
Inspiratory muscle training and non-invasive ventilation is now robust
whilst further evidence is required for other interventions in order to
clarify where application, skills and training should be focused.
The challenge is to translate sound clinical evidence-based practice into
novel models of service with resultant improvements in care for patients
with COPD.
POSITIONING :-
THE PURPOSE OF POSITIONING
B) Relaxed sitting posture (side view). Note: Forward head position, increased
sternocleidomastoid activity, increased low cervical lordosis and thoracic kyphosis, abducted
and protracted scapulae, anterior position of humerus in glenoid fossa, internal rotation of
humerus, lax abdominal muscles.
POSTURAL DRINAGE:-
BREATHING EXERCISES
These exercises are fundamental interventions for
prevention or comprehensive management of COPD.
These exercises are diaphragmatic.
Breathing exercises are designed to restrain the muscles of
respiration and improve or redistribute ventilation, lessen the work of
breathing, and improve the gas exchange and oxygenation.
Active range of motion exercises, to the shoulders and trunk also
help expand the chest, facilitate deep breathing, and often stimulate the
cough reflex.
Diaphragmatic Breathing
Pursed-lip Breathing
Breathing while bending forward
Precautions
• Never allow a patient to force expiration. Expiration should be
relaxed and passive. Forced expiration only increases turbulence
in the airways, which can lead to bronchospasm and increased
airway restriction.
• Do not allow a patient to take a very prolonged expiration. This
causes the patient to gasp with the next inspiration. The patient’s
breathing pattern then becomes irregular and inefficient.
• Do not allow the patient to initiate inspiration with the accessory
muscles and the upper chest. Advise the patient that the upper
chest should be relatively quiet during breathing.
• Allow the patient to practice deep breathing for only three or four
inspirations and expirations at a time to avoid hyperventilation.
HUMIDIFICATIONS :-
Respiratory gas humidification is a method
of artificial warming and humidifying of respiratory gas for the patient
during mechanical ventilation.
The upper chest and shoulder remain relax, lower chest and
abdomen should be active.
This level is sustained for 20-45% and repeated 3-4 times a week
Training at this intensity, within is well above the anaerobic
threshold, increase maximal exercise performance, causes
physiologic adaptations in peripheral muscle and improves cardiac
function in healthy subjects.
Training specificity
Refers to the observation that benefit is gained only in those
activities involving the muscle groups that are specifically trained.
BIBILOGRAPHY
DAVIDSON-GENERAL MEDICINE
HARRISON’S-INTERNAL MEDICINE
HARSHA MOHAN-PATHOLOGY
JULLIAN-
JENNIFER PRIOR-
TIDY’S PHYSIOTHERAPY
CASH TEXTBOOK OF CARDIOVASCULAR
DISEASE FOR PHYSIOTHERAPY
INTERNET