Pickwickian syndrome

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Pickwickian
Syndrome
Ms. Feba Geevarghese
Vice Principal & Ph.D. Scholar
College of Nursing, St. Stephen’s
Hospital, Delhi

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GENERAL OBJECTIVE
 At the end of the teaching, students will
acquire understanding about Pickwickian
Syndrome, its pathophysiology, clinical
manifestations, medical management and
demonstrate skills in nursing management of
the patient

z SPECIFIC OBJECTIVES
At the end of the class, the students will
be able to:
 define Pickwickian Syndrome
 identify the history of Pickwickian
Syndrome
 explain the epidemiology of
Pickwickian Syndrome
 outline the anatomy and physiology of
the lungs
 classify Pickwickian Syndrome
 outline the pathophysiology of
 enumerate the clinical
manifestations of Pickwickian
Syndrome
 describe the diagnostic
evaluation of Pickwickian
Syndrome
 discuss the treatment of
 explain the nursing management
of Pickwickian Syndrome
 describe the prognosis of

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Definition
Pickwickian Syndrome also known as Obesity
Hypoventilation Syndrome is defined as the combination
of obesity (body mass index above
30 kg/m2), hypoxemia(falling oxygen levels in blood)
during sleep, and hypercapnia (increased blood carbon
dioxide levels) during the day, resulting
from hypoventilation (excessively slow or shallow
breathing).

z History of Pickwickian syndrome
The discovery of obesity hypoventilation syndrome is
generally attributed to the authors of a 1956 report of a
professional poker player who, after gaining weight, became
somnolent and fatigued and prone to fall asleep during the
day, as well as developing edema of the legs
suggesting heart failure. The authors coined the condition
"Pickwickian syndrome" after the character Joe
from Dickens' The Posthumous Papers of the Pickwick
Club (1837), who was markedly obese and tended to fall
asleep uncontrollably during the day.

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Epidemiology of Pickwickian Syndrome
 The exact prevalence of obesity hypoventilation syndrome is
unknown, and it is thought that many people with symptoms of OHS
have not been diagnosed. About a third of all people with morbid
obesity (a body mass index exceeding 40 kg/m2) have
elevated carbon dioxide levels in the blood.
 When examining groups of people with obstructive sleep apnea,
researchers have found that 10–20% of them meet the criteria for
OHS as well. The risk of OHS is much higher in those with more
severe obesity, i.e. a body mass index (BMI) of 40 kg/m2 or higher.

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Epidemiology of Pickwickian Syndrome
 It is twice as common in men compared to women. The average
age at diagnosis is 52. American Black people are more likely to
be obese than American whites, and are therefore more likely to
develop OHS, but obese Asians are more likely than people of
other ethnicities to have OHS at a lower BMI as a result of
physical characteristics. It is anticipated that rates of OHS will
rise as the prevalence of obesity rises. This may also explain
why OHS is more commonly reported in the United States,
where obesity is more common than in other countries.

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Anatomy and Physiology of the Lungs

z Anatomy and Physiology of the Lungs
 The lungs are the primary organs of the respiratory
system in humans. The function of the respiratory system is to
extract oxygen from the atmosphere and transfer it into
the bloodstream, and to release carbon dioxide from the
bloodstream into the atmosphere, in a process of gas
exchange. Humans have two lungs, a right lung and a left lung.
They are situated within the thoracic cavity of the chest. The
right lung is bigger than the left, which shares space in the chest
with the heart.

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 The lower respiratory tract is part of the respiratory system, and
consists of the trachea and the structures below this including the
lungs. The trachea receives air from the pharynx and travels down to
a place where it splits (the carina) into a right and left bronchus.
These supply air to the right and left lungs, splitting progressively
into the secondary and tertiary bronchi for the lobes of the lungs, and
into smaller and smaller bronchioles until they become
the respiratory bronchioles. These in turn supply air through alveolar
ducts into the alveoli, where the exchange of gases take place.
Oxygen breathed in, diffuses through the walls of the alveoli into the
enveloping capillaries and into the circulation, and carbon dioxide
diffuses from the blood into the lungs to be breathed out.

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 The lungs are located in the chest on either side of the heart in the rib cage.
They are conical in shape with a narrow rounded apex at the top, and a broad
concave base that rests on the convex surface of the diaphragm. The lungs
are surrounded by the pulmonary pleurae. The pleurae are two serous
membranes; the outer parietal pleura lines the inner wall of the rib cage and
the inner visceral pleura directly lines the surface of the lungs. Between the
pleurae is a potential space called the pleural cavity containing a thin layer of
lubricating pleural fluid. The main or primary bronchi enter the lungs at the
hilum and initially branch into secondary bronchi also known as lobar bronchi
that supply air to each lobe of the lung. The lobar bronchi branch into tertiary
bronchi also known as segmental bronchi and these supply air to the further
divisions of the lobes known as bronchopulmonary segments.

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 The right lung has both more lobes and segments than the left. It is divided into
three lobes, an upper, middle, and a lower, by two fissures, one oblique and one
horizontal. The upper, horizontal fissure, separates the upper from the middle lobe.
The lower, oblique fissure, separates the lower from the middle and upper lobes,
and is closely aligned with the oblique fissure in the left lung.
 The left lung is divided into two lobes, an upper and a lower, by the oblique fissure.
 All of the lower respiratory tract including the trachea, bronchi, and bronchioles is
lined with respiratory epithelium. This is a ciliated epithelium interspersed
with goblet cells which produce mucus, and club cells with actions similar
to macrophages. Incomplete rings of cartilage in the trachea and smaller plates of
cartilage in the bronchi, keep these airways open.

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 Both lungs have a central recession called the hilum at the root
of the lung, where the blood vessels and airways pass into the
lungs.
 Blood supply-The lungs have a dual blood supply provided by
a bronchial and a pulmonary circulation. The bronchial
circulation supplies oxygenated blood to the airways of the
lungs, through the bronchial arteries that leave the aorta. There
are usually three arteries, two to the left lung and one to the
right, and they branch alongside the bronchi and bronchioles.
The pulmonary circulation carries deoxygenated blood from the
heart to the lungs and returns the oxygenated blood to the heart
to supply the rest of the body.

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 Nerve supply- The lungs are supplied by nerves of the autonomic
nervous system. Input from the parasympathetic nervous system
occurs via the vagus nerve. When stimulated by acetylcholine,
this causes constriction of the smooth muscle lining the bronchus
and bronchioles, and increases the secretions from glands. The
lungs also have a sympathetic tone from norepinephrine acting on
the beta 2 receptors in the respiratory tract, which
causes bronchodilation.
 The action of breathing takes place because of nerve signals sent
by the respiratory centres in the brainstem, along the phrenic
nerve to the diaphragm.

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 Gas Exchange
(a). External Respiration- The major function of the lungs is gas
exchange between the lungs and the blood. The lungs are not
capable of expanding to breathe on their own, and will only do so
when there is an increase in the volume of the thoracic cavity. This is
achieved by the muscles of respiration, through the contraction of
the diaphragm, and the intercostal muscles which pull the rib
cage upwards. During breathing out the muscles relax pulling the
ribcage down returning the lungs to their resting position.

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(b). Internal respiration- It is the process by which gases in the air that has
already been drawn into the lungs by external respiration are exchanged with
gases in the blood (that has returned to the lungs after passing through tissues
around the body) so that carbon dioxide (CO2) is removed from the blood and
replaced with oxygen (O2). When the concentration of oxygen in the airways of
the lungs (including the alveolar sacs) is high relative to the concentration of
oxygen in the blood(passing through the capillaries that cover the alveoli), the
concentration of oxygen in the lungs decreases while the concentration of oxygen
in the blood increases.

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 When the concentration of carbon dioxide in the blood (passing through
the capillaries that cover the alveoli) is high relative to the concentration of carbon
dioxide in the airways of the lungs (including the alveolar sacs), then
concentration of carbon dioxide in the blood decreases while the concentration of
carbon dioxide in the lungs increases. The exchange of gases between the
alveolus and the blood occurs by diffusion of the gases through the very fine
structures of the boundary (or 'wall') of the capillary and the alveolar-membrane
separating the capillary from the alveolus.

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Classification of Pickwickian Syndrome
 Two subtypes are recognized, depending on the nature of disordered breathing
detected on further investigations.
 The first is OHS in the context of obstructive sleep apnea; this is confirmed by
the occurrence of 5 or more episodes of apnea, hypopnea or respiratory-related
arousals per hour (high apnea-hypopnea index) during sleep.
 The second is OHS primarily due to "sleep hypoventilation syndrome"; this
requires a rise of CO2 levels by 10 mmHg (1.3 kPa) after sleep compared to
awake measurements and overnight drops in oxygen levels without
simultaneous apnea or hypopnea.
 Overall, 90% of all people with OHS fall into the first category, and 10% in the
second.

z • Firstly, work of breathing is increased as adipose tissue restricts the
normal movement of the chest muscles and makes the chest wall
less compliant, the diaphragm moves less effectively, respiratory
muscles are fatigued more easily, and airflow in and out of the lung is
impaired by excessive tissue in the head and neck area. Hence,
people with obesity need to expend more energy to breathe effectively.
• These factors together lead to sleep-disordered breathing
and inadequate removal of carbon dioxide from the
circulation and hence hypercapnia; and
eventually acidosis
• Under normal circumstances, central
chemoreceptors in the brain stem detect the acidity,
and respond by increasing the respiratory rate; in
OHS, this "ventilatory response" is blunted.

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• Obese people tend to have raised levels of the
hormone leptin, which is secreted by adipose tissue and
under normal circumstances increases ventilation. In
OHS, this effect is reduced.
• Furthermore, episodes of night time acidosis (e.g. due to
sleep apnea) lead to compensation by the kidneys with
retention of the alkali bicarbonate. This normalizes the
acidity of the blood.
• However, bicarbonate stays around in the
bloodstream for longer, and further episodes of
hypercapnia lead to relatively mild acidosis and
reduced ventilatory response in a vicious circle.

z • Low oxygen levels lead to hypoxic pulmonary
vasoconstriction, the tightening of small blood vessels in the
lung to create an optimal distribution of blood through the
lung
• Persistently low oxygen levels causing chronic
vasoconstriction leads to increased pressure on
the pulmonary artery (pulmonary hypertension), which in turn
puts strain on the right ventricle, the part of the heart that
pumps blood to the lungs
• The right ventricle undergoes remodeling, becomes
distended and is less able to remove blood from the veins.
When this is the case, raised hydrostatic pressure leads to
accumulation of fluid in the skin (edema), and in more
severe cases the liver and the abdominal cavity.

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The chronically low
oxygen levels in the blood
also lead to increased
release
of erythropoietin and the
activation
of erythropoeisis, the
production of red blood
cells.
This results
in polycythemia,
abnormally increased
numbers of circulating red
blood cells and an
elevated hematocrit.

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Clinical Manifestations of
Most people with obesity hypoventilation syndrome have
concurrent obstructive sleep apnea. The symptoms are:
 snoring
 brief episodes of apnea (cessation of breathing) during the night
 interrupted sleep
 excessive daytime sleepiness
 sleepiness may be worsened by elevated blood levels of carbon
dioxide, which causes drowsiness ("CO2 narcosis")

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Clinical Manifestations of
 Other symptoms present in both conditions are:
 depression
 uncontrolled hypertension (high blood pressure) that is difficult
to control with medication. headaches due to high carbon dioxide
levels which tend to be worsening in the morning
 ventilation-perfusion mismatching
 low oxygen level and resultant physiologic constriction of the
pulmonary arteries puts excessive strain on the right side of the
heart leading to right sided heart failure, known as corpulmonale
edema (swelling),
 ascites

z Clinical Manifestations of
 decreased exercise tolerance
 exertional chest pain may occur
 presence of a raised jugular venous pressure
 a palpable parasternal heave
 a heart murmur due to blood leaking through the
tricuspid valve
 hepatomegaly (an enlarged liver)
 leg edema

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Diagnostic Evaluation
Formal criteria for diagnosis of OHS are:
 Body mass index over 30 kg/m2 (a measure of obesity, obtained by
taking one's weight in kilograms and dividing it by one's height in
meters squared)
 Arterial carbon dioxide level over 45 mmHg as determined
by arterial blood gas measurement
 No alternative explanation for hypoventilation, such as use
of narcotics, severe obstructive or interstitial lung disease, severe
chest wall disorders such as kyphoscoliosis,
severe hypothyroidism (underactive thyroid), neuromuscular
disease or congenital central hypoventilation syndrome

z Diagnostic Evaluation
 Polysomnography (Sleep Study)- This usually requires brief admission to a
hospital with a specialized sleep medicine department where a number of
different measurements are conducted while the subject is asleep; this
includes electroencephalography , electrocardiography, pulse oximetry and
often other modalities.
 Blood tests are also recommended for the identification
of hypothyroidism and polycythemia.
 To distinguish between OHS and various other lung diseases that can cause
similar symptoms, medical imaging of the lungs (such as a chest X-
ray or CT/CAT scan), spirometry, electrocardiography
and echocardiography may be performed. Echo- and electrocardiography
may also show strain on the right side of the heart caused by OHS, and
spirometry may show a restrictive pattern related to obesity.

z Medical Management
 In people with stable OHS, the most important treatment
is weight loss—by diet, through exercise, with medication, or
sometimes weight loss surgery (bariatric surgery). This has
been shown to improve the symptoms of OHS and resolution
of the high carbon dioxide levels.

 Night time positive airway pressure (PAP) treatment is tried;
this involves the use of a machine to assist with breathing.

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Medical Management
 Admission to an intensive care
unit with intubation and mechanical ventilation is necessary
in acute emergency condition

 In some occasions, the oxygen levels are persistently too low
(oxygen saturations below 90%). In that case, the hypoventilation
itself may be improved by switching from CPAP treatment to an
alternate device that delivers "bi-level" positive pressure (BIPAP):
higher pressure during inspiration (breathing in) and a lower
pressure during expiration (breathing out).

 Oxygen therapy
 Tracheostomy may be necessary combined
with mechanical ventilation with an assisted
breathing device

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Medical Management
 Medroxyprogesterone acetate, a progestin, has been shown to
improve the ventilatory response, but this has been poorly studied
and is associated with an increased risk of thrombosis
 The drug acetazolamide can reduce bicarbonate levels, and
thereby augment to normal ventilatory response, but this has been
researched insufficiently to recommend wide application

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NURSING
MANAGEMENT USING
NURSING PROCESS
APPROACH

z NURSING PROCESS
Assessment
 vital signs
 current weight
 snoring
 brief episodes of apnea during the night
 excessive daytime sleepiness
 ventilation-perfusion mismatching
 decreased exercise tolerance

z NURSING PROCESS
Nursing Diagnoses
 Impaired gas exchange and airway clearance related to the disease
process
 Impaired gas exchange related to ventilation–perfusion inequality
 Ineffective breathing pattern related to shortness of breath
 Activity intolerance due to fatigue, ineffective breathing patterns, and
hypoxemia
 Deﬁcient knowledge of self-care strategies and weight reduction
program
 Ineffective coping related to reduced socialization, anxiety, depression,
lower activity level, and the inability to work

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NURSING PROCESS
Planning and Goals
The major goals for the patient include:
 improved gas exchange
 airway clearance
 improved breathing pattern
 improved activity tolerance
 maximal self-management
 improved coping ability
 adherence to the therapeutic program and home care
 absence of complication

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NURSING PROCESS
Nursing Interventions
 Improving Breathing Pattern
 The patient should assume a comfortable position to promote rest
and breathing (eg, semi-Fowler’s) and should change positions
frequently to enhance ventilation/perfusion in the lungs.
 Use of positive airway pressure devices at night during sleep
 Oxygen supplementation
 Pulse oximetry and ABG monitoring
 Promote compliance to treatment
 Dietary modification and weight reduction
 Avoidance of smoking

z NURSING PROCESS
 Improving Activity Tolerance
 Avoid overexertion from activities
 Maintain balance between activity and rest
 Oxygen therapy
 Avoid extremes of temperature

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NURSING PROCESS
 Enhancing Individual Coping Strategies
 Promote interventions for improved physical functioning, psychological
and emotional stability, and social support
 Educate regarding the use of respiratory devices at home
 Teach patient regarding monitoring of symptoms, medication and
treatment compliance
 Allow patient to verbalize concerns
 Encourage socialization

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NURSING PROCESS
 Monitoring and Managing Potential Complications
 Assess for various complications, such as life-threatening
respiratory insufﬁciency and failure
 Monitor for cognitive changes (personality and behavioral
changes, memory impairment), increasing dyspnea,
tachypnea, and tachycardia, which may indicate increasing
hypoxemia and impending respiratory failure

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NURSING PROCESS
Evaluation
Expected Patient Outcomes
1. Demonstrates improved gas exchange
a. Shows no signs of restlessness, confusion, or agitation
b. Has stable pulse oximetry or arterial blood gas values
2. Demonstrates improved activity tolerance
a. Performs self-care activities within tolerance range
b. Paces self to avoid fatigue and dyspnea
c. Uses controlled breathing while performing activities
d. Uses devices to assist with activity tolerance and decrease energy
expenditure

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NURSING PROCESS
3. Demonstrates knowledge of self-care strategies
a. Participates in determining the therapeutic program and
weight reduction
b. Understands the rationale for activities and medications
c. Follows the medication plan
d. Uses oxygen therapy as prescribed
e. Maintains acceptable activity level

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NURSING PROCESS
4. Uses effective coping mechanisms
a. Uses self-care strategies to lessen stress associated with
disease
b. Verbalizes feelings and concerns
5. Avoids or reduces complications
a. Has no evidence of respiratory failure or insufﬁciency
b. b. Maintains adequate pulse oximetry and arterial blood gas
values

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Prognosis of Pickwickian Syndrome
 Obesity hypoventilation syndrome is associated with a
reduced quality of life, and people with the condition incur
increased healthcare costs, largely due to hospital admissions
including observation and treatment on intensive care units.
 OHS often occurs together with several other disabling medical
conditions, such as asthma (in 18–24%) and type 2 diabetes (in
30–32%). Its main complication of heart failure affects 21–32% of
patients.

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Prognosis of Pickwickian Syndrome
 Those with abnormalities severe enough to warrant treatment
have an increased risk of death reported to be 23% over 18
months and 46% over 50 months. This risk is reduced to less
than 10% in those receiving treatment with PAP.
 Treatment also reduces the need for hospital admissions and
reduces healthcare costs

Pickwickian syndrome

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