Familial Hypercholesterolemia Management Guidelines Analysis

This assignment involves thoroughly examining the National Lipid Association's clinical guidance on screening, diagnosis, and management of pediatric and adult patients with Familial Hypercholesterolemia. You are required to analyze and summarize key points from the provided guidelines by Goldberg et al. (2011), focusing on the recommendations for managing this inherited disorder effectively.

Managing Care of
the Adult Patient

Table of Contents
TASK 1............................................................................................................................................1
1) What is the structure and function of the following:..............................................................1
2) Structures in the nose help to moisten, filter and warm the air.............................................1
3) Three divisions of the pharynx..............................................................................................2
4) Function of the epiglottis.......................................................................................................2
TASK 2............................................................................................................................................2
1) Structure and function of the following:.................................................................................2
2) Pulmonary aspiration more likely to occur in right bronchus than in left bronchus..............3
3) Prevents alveoli from collapsing among inhalations.............................................................3
TASK 3............................................................................................................................................3
1) Process of Breathing...............................................................................................................3
2) Respiration..............................................................................................................................3
3) Muscular action cause inhalation to occur..............................................................................3
4) Intercostal muscle and Diaphragm during exhalation............................................................4
5) Chemoreceptors in aorta influence respiration and carotid arteries.......................................4
6) Respirations are controlled by involuntary muscle movement..............................................4
TASK 4............................................................................................................................................4
1) Process of gas exchange.........................................................................................................4
TASK 5............................................................................................................................................4
1) Assessment and its significance of the following:.................................................................4
TASK 6............................................................................................................................................6
1) Symptoms of acute bronchitis................................................................................................6
2) Management of Acute bronchitis............................................................................................6
3) Prevent complications from occurring with acute bronchitis................................................6
Task 7...............................................................................................................................................6
Task 8...............................................................................................................................................7
Task 9...............................................................................................................................................8
Task 10...........................................................................................................................................10
.........................................................................................................................................................1

TASK 1
1) What is the structure and function of the following:
Nasal Cavity: Structure has three divisions:
Vestibule: Area posterior to the nostrils,and have multiple layers of same cells that make our
skin.
Respiratory region: Begins at the end of vestibular area,and includes the entire nasal cavity.
Olfactory region: Top part of nasal cavity,lined with olfactory cells and olfactory mucosa.
Functions: It has two primary purpose in process of breathing:
Role as a passage for inhaled Air: Air enters through the nostrils and passes through the
nasal cavity into the Pharynx and Larynx.
Role of mucus membrane in purifying the Air: Purifying the inhaled air by trapping dust ,
bacteria, small particles.
Pharynx Structure: The structure is not universe the same in all species but in human
beings the pharynx is part of digestive system and respiratory system.
Function: Works as a connecting tube between nasal cavity and larynx and also the oral cavity
and esophagus (Barr and et. al., 2013). It is also an integral part of respiratory and digestive
system.
Also have functions in such parts: Respiratory system, Digestive system, Speech organs
Larynx
Structure:
Cartilages: Posterior view of the larynx is cartialges and intrinsic muscles. There are nine
cartilages in which three are unpaired and three are paired which supports the mammalian larynx
unpaired cartilagess
Trachea
It is a windpipe and also a hollow tube which is related to the larynx to bronchi of lungs.
This is an internal part of human's body and its main function is to giving an air flow and also
lungs for the respiration.
2) Structures in the nose help to moisten, filter and warm the air
For the warm the air, moisten and the filter, nasal cavity organ is helpful and its structure
are:
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 It is lined with capillaries.
 It is lined with the cilia. It filter out the debris inside an air.
3) Three divisions of the pharynx
The threes divisions of the pharynx are Nasopharynx, Oropharynx and the
Laryngopharynx. These three are discussed below as above:
Nasopharynx- It is a throat's upper part and it is behind a nose. It is a main part of the
pharynx. Its is 2 to 3 cm wide and its length is 3 to 4 cm.
Oropharynx- Its main function is Swallowing and voluntary is the first phrase of
Oropharynx. In this, tongue propulsion a food bolus posteriorly in the oropharynx.
Laryngopharynx- Air and food both are passes through this function. It is found among
the esophagus and also the hybrid bone.
4) Function of the epiglottis
The shape of Epiglottis is leaf and it is situated behind a tongue and also at the voice box
and the tongue. Its major function is to seal off a windpipe at the time of eating the food so from
this accidentally the food is not inhaled (Haugen and et. al., 2016). It is very necessary for the
digestion and it helps in preventing the liquid items and food from going to trachea.
TASK 2
1) Structure and function of the following:
Bronchi- It is known as the bronchus. It is the extension wind pipe that helps in shuffle
the air from lungs. It is pass away from the lungs. It is close to the lungs tissue and after this it is
regarded as the bronchioles. Its major function is to site of carbon dioxide and also the oxygen in
exchange of respiratory system.
Bronchioles- It passes by the mouth or the nose to alveoli of lungs under which the
branches do not include the glands in submucosa.
Alveoli- These are the small sacs in the lungs that helps the carbon dioxide and also the
oxygen for moving among blood stream and the lungs. In this, an exchange of the both the gases
carbon dioxide and oxygen is takes place.
Lung- It is a pair of the air- filled and the spongy which is situated in the chest. In its
structure consists an air- tubes and bronchial tree branching off from bronchi in the tiny air tubes.
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Diaphragm- It is a primary and important muscle of body which is ides in inspiration or
can say inhalation process (Zebrack and Isaacson, 2012). Its shape is some. Its function is to
separate a abdominal cavity from thoracic cavity.
2) Pulmonary aspiration more likely to occur in right bronchus than in left bronchus
A Pulmonary aspiration is occur in right bronchus because it is small angulated from
trachea as comparison to the left bronchus.
3) Prevents alveoli from collapsing among inhalations
An intrapleural pressure and also surfactant both are very helpful in preventing alveoli or
lungs from the collapsing. The surfactant is developed through the 2nd types Alveolar cells. On
the other hand, intrapleural pressure helps in giving a relaxation at the time of expiration that
allows a lungs for the regain and also recoil.
TASK 3
1) Process of Breathing
When a person is inhale then in this case the contracts of diaphragm and also the moves
downward. In the process of breathing the inhalation and exhalation both are included. The
breathing is started with the inhalation. It is beginning with a diaphragm of contraction which
helps in pulling down the lungs. Contraction maximise lungs volume also minimize the pressure
of air in lungs. Relaxation of intercostals and diaphragm less lungs volume which enhance an
pressure of air under lungs from the external air. After this, an air of lungs is out. Then full cycle
is repeated regularly.
2) Respiration
It is not similar as breathing. It is also known as the ventilation. It is chemical procedure
under which an energy is released with the help of any food items like sugar, glucose etc. IT
works as a oxygen.
3) Muscular action cause inhalation to occur
Muscles of respiration are the muscles which gives their contribution in the exhalation
and also inhalation through an addition in contraction and expansion of thoracic cavities. The
main muscle which is responsible for breathing is diaphragm.
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4) Intercostal muscle and Diaphragm during exhalation
This happens because of the lungs elastic properties along with the intercostal muscles.
This reduces the rib cage and also minimize the volume of throat (Goldberg and et. al., 2011).
During inhaling, contract of intercostal muscles, increasing ribcage. On the other hand,
diaphragm contracts, pulls down for enhancing chest volume.
5) Chemoreceptors in aorta influence respiration and carotid arteries
These include the aortic body, which helps in detecting the changes in blood oxygen and
carbon dioxide, but not pH, and the carotid body which detects all three. They do not desensitize,
and have less of an impact on the respiratory rate compared to the central chemoreceptors.
6) Respirations are controlled by involuntary muscle movement
The function Of the respiratory system is to supply the oxygen to the tissues and removing the
carbon dioxide. This is achieved with the inspiration and expiration of Air.
The patients oxyzen saturation may be measured using pulse meter. This will provide an accurate
reading of oxygenetion in the blood cells. While using pulse oximeter require that patient have
less arterial blood gases performed.
TASK 4
1) Process of gas exchange
It is a delivery of an oxygen from lungs to bloodstream. Process of gas exchange
transfers gas through blood of humans in exchange of gas. It helps in removing of carbon dioxide
from bloodstream to lungs (Provan and et. al., 2010). During inhale air by nose it is go through
trachea by the bronchioles and bronchus. It is go through alveolus in which an exchange of gas is
taken place. An alveolus is a sac filled through air and it is a unit of lung. From this the gas is
exchange here. From this, various types of gases are come out through method of diffusion.
From this, gas go by high concentration in the alveolus.
TASK 5
1) Assessment and its significance of the following:
 Rate: Respiratory rate is on of the sensitive mark of a patient condition and is one of the
main elemets of various tools of medical assessment. Rate of respiration system vary
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from gender and age. Respiratory rate of 12-18 breaths per minute in a healthy adult is
consider as normal.
 Rhythm: Respiratory rhythm is a regular cycle of inspiration and expiration which is
controlled by neuronal transmitted between the respiratory centres in muscles and brain
in the chest (Jacobi and et. al., 2012). Normal breathing pattern of an individual get
affected by variety of situations and conditions.
 Depth: A group of symptoms accompany fulminant pulmonary edema.
 Work of breathing: Energy expand by an individual to inhale and exhale breathing gas is
known as work of breathing. Basically this is expressed as work per unit volume. Further
it can be calculated in terms pulmonary pressure or in terms of oxygen consumption. At a
normal state of work of breathing constitute about 5 % of total body oxygen
consumption.
 Posture: Position in which a person hold his whole body is known as posture. Posture of
an individual largely affect on the expiratory activity. This is also known as a particular
attitude or approach of an individual.
 Skin colour: Colour of human skin range in variety from the lightest to the darkest. Skin
pigmentation of individual is known as result from of his or her genetics affect by the
biological parents of individual (Minet and et. al., 2010). Skin colour of an individual get
affected by various substances through which pigment melanin is known as the important
one. This is produced by cells in skin called melanocytes and is known as the main
determinant of darker skin of humans.
 Symmetry of chest movement: basically this is a method of chest expansion. Under this,
patient have seated on one side. After that doctor stand behind the patient and grab the
lower hemithorax to bring his thumps to the midline.
 Deformities of chest: Basically there are two basic types of congenital chest wall
deformities known as pectus excavatum and pectus carinatum. Some individulas born
with combination of both of these.
 Pain: Distressing feeling which is cause by damage a stimuli is known as pain. Due to a
complex phenomenon it is very difficult to describe this term. Pain is a unpleasant
emotional experience which associated with actual tissue damage of an individual. Pain
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motivate the individual to recover from damaging situations and to avoid the same in
future.
 Mental status: This is known as the real state of mind of an individual. For improve
mental status of an individual mental examination is done during the medical assessment
(Sidbury and et. al., 2014). Basically this is known as structured way of observe and
describe psychological functioning of an individual at a particular situation and time.
 Cough: Cough is a very common reflex action that clears the throat of mucus. It is a
sudden. Protecting reflex which help an individual to clear the breathing passages from
foreign, particles and fluids.
TASK 6
1) Symptoms of acute bronchitis
There are some symptoms of acute bronchitis are fatigue, cough, shortness of breath,
chest discomfort, muscus production, fever, wheezing, chills and slight fever.
2) Management of Acute bronchitis
IN the acute bronchitis, treatment with the beta2- agonist bronchodilators can be in teh
patients which are related to the wheezing with deseaswe related to underlying lung and
wheezing with the cough. It is very common diagonis, and depend on the clinical findings.
3) Prevent complications from occurring with acute bronchitis
The acute bronchities is caused from viral infection and also from teh bacterial infection.
Its prevention is to stop smoking. It is helpful for lungs. The nurse helsp in preventing this by
providing the good medical teratment. Nurse gives a medicine of coungh to patient. To prevent
this disease, nurse gives the antibiotics to patient. From this, person overcomes from this slowly
slowly.
Task 7
COPD means -chronic obstructive pulmonary disease and includes two respiratory disorders
emphysema and chronic bronchitis. These two disorders cause -by smokingand commonly occur
together. viruses causes inflammation of the bronchi while chroniccauses damage to the small
airways and respiratory system which leads to
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serious inflammation where there is a loss in the surface area reducing gaseous exchange. The
main cause of COPD is cig grate smoking but polluted air can also be a contributing factor.
Chronic is defined asmore serious which persists for more timeand obstruction is caused by—
smoking and polluted air.
Pulmonary is a term meaning –functioning with or carried on by the lungs.
COPD and asthma have similar symptoms but they are very different diseases. In COPD the
damage to the airways by way of swollen them whereas in asthma it is caused because of
smoking or dust (Topolovec-Vranic and et. al., 2010). It is important to remember that some
people with COPD also have asthma. In order to confirm the diagnosis of COPD a
spirometry test is carried out. This test measures the level of infection in chest and with a result
of it can be decided that it is acute or chronic. value combined with four typical symptoms of
COPD
1.shortness of breath2chest tightness3wheezing4excess mucus in lungs confirms the diagnosis of
COPD. Symptoms usually begin at the age of 40 after smoking for 10years or more. There is
usually a gradual increases over the years with a serious problem related to the cough, and
increased drastically.
Chest infections become more frequent and there is often an increase of
symptoms which is known as an –chronic bronchitis. As the disease becomes more severe it
becomes swollen the pipes and more inflamed
the lungs due to the reason of chronic. This in turn decreases the amount of -
water entering the blood stream, which can lead to –increase of blood pressure.
Task 8
 Difference between acute bronchitis and chronic bronchitis:
Acute bronchitis: Acute bronchitis comes on quickly and takes at least two to three weeks
for patients to recover. The another thing about Acute bronchitis is it follows three to four days
after an upper respiratory tract infection.
Chronic bronchitis: A case of bronchitis is considered as chronic when it persists most
days of the month, three months out of the year.
 Diagnostic characteristics of chronic bronchitis
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The chronic bronchitis should be diagnosed through the cough, excessive mucus
production must have occurred for 3 months or more or having for at least 2 consecutive years
and not be due to any other disease or condition.
 common cause of chronic bronchitis
The most common cause of chronic bronchitis is cigarette smoking. Breathing in air
pollution,fumes,or dust over a long period of time is also causing chronic bronchitis.
 affect of this disease on the airways and cause obstruction
Chronic obstructive pulmonary disease is the name for a group of lung conditions that
cause breathing difficulties. It includes: emphysema- damage to the air sacs in the lungs. Chronic
bronchitis- long term inflammation of the airways.
 assessments made by nurse about the cough of a patient in chronic bronchitis
In early stage of the bronchitis the colour of the cough is white but when then bronchitis
becomes chronic the colour of the cough is becomes yellow.
 management for patients with chronic bronchitis
The persons suffering from chronic bronchitis are required proper treatment. Inhaled
ipratropium bromide and sympathomimetic agents are the current mainstays of management. oral
steroid therapy should be reserved for use in patients with demonstrated improvement in airflow.
Task 9
 main reasons for oxygen therapy
Oxygen therapy refers to supplemental oxygen given to people who, largely due to
breathing disorders, aren't able to get enough naturally.
Symptoms of low oxygen include rapid breathing, coughing or wheezing and changes in the
colour of our skin. Many people with COPD require long term therapy.
 oxygen devices and their uses and the advantages and disadvantages of each
1.Non-rebreather mask: The non Re breathing mask, 'Trauma mask', provides a high
concentration of oxygen and the reservoir bag allows for adequate oxygen.
Advantages:
1. Highest possible Fio2 without intubation
2. suitable for spontaneously breathing patients with severe hypoxia
Disadvantages:
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1. Expensive
2. Require tight seal, uncomfortable
2. simple mask: It is disposable mask made of plastics used for the purpose
providing oxygen to the patients whom experiencing non breathing.
Advantages:
1. Less expensive
2. low flow device
Disadvantages:
1. Doesn't deliver high conc.O2, interferes with eating and talking
3. Nasal cannula: The nasal cannula is a device used to deliver supplemental oxygen
or airflow to a patient or person in need of respiratory help.
Advantages:
1.less expensive
2. comfortable,well tolerated
Disadvantages:
1. Does not deliver high Fio2
2. Irritation and nasal obstruction
 principles of oxygen therapy
1. Variable performance: Variable performances devices, such as nasal cannule and masks
with or without a re breathing bag because the patients breathing affects the concentration
of the inspired mixture.
Various factors influence the inspired oxygen concentration when using variable performance
devices including an irregular breathing pattern coupled with the use of oxygen flow less than
the patients inspiratory flow rate.
2. Fixed performance:
These devices allow controlled oxygen dosage. They create a constant proportion of air /oxygen
mixture in excess of patient inspiratory flow rate and are independent of patient factors or fit to
the face. With gas flow constantly in excess of patient demand and with enhanced co2
washout,re breathing is virtually eliminated.
 dangers of oxygen therapy
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 Skin irritation and Nasal Dryness: Because oxygen therapy has a drying effect on the
nasal passages, it is not uncommon for skin irritation,skin breakdown and nasal dryness.
 Fire Hazard: Although oxygen is not a flammbale gas, it does support combustion
meaning that things will burn more readily in its presence.
 Oxygen Toxicity: People who are in contact with owygen long period are at a risk of
oxygen toxicity.
 Suppression of breathing: in some patients, oxygen therapy supress the drive to breathe.
This should be managed through by adjusting oxygen flow rate.
Task 10 Tachypnoea: It is a abnormally rapid breathing. In adult humans at rest, respiratory rate
should be in between 12 to 20 breaths per minute normally but in case of Tachypnoea it
should be greater than the 20 breaths per minutes. Bradypnoea: It is the situation of abnormally slow breathing. The respiratory rate at
which bradypnoea is diagnosed depends on the age of the patient. Hyperventilation: It occurs when the rate or tidal volume of breathing eliminates more
carbon dioxide than the body can produce (Nunnally and et. al., 2011). This lowers the
level of carbon dioxide dissolved in the blood leading to hypocapnia. Hypoventilation:It occurs when the ventilation is inadequate to perform needed gas
exchange. By definition it should be understand that it increases the level of carbon
dioxide and respiratory acidosis. Hypoxia: It is the situation in which the body or any region of the body is deprived of
adequate oxyzen suppkly at the tissue level. It may be classified as either genralized,
affecting the whole body, local any region of the body. Apnoea: It is the serious condition where the muscles in the throat relax during sleep
causing the sufferer to temporarily stop breathing. Orthopnoea: Orthopnoea is the situation of the shortness of breath that occurs when
lying flat, causing the person to have sleep propped up in bed or sitting on chair. Dyspnoea: It is the situation of the shortness of breath or breathless, is subjective
sensation of breathing discomfort. Cyanosis: It is the defines as the bluish or purplish discolouration of the skin or mucus
membranes due to the tissues near skin surface having low oxygen saturation.
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 Hypoxaemia:It is the situation of an abnormal low level of oxygen in the blood. In other
words we can say that it is an oxygen deficiency in arterial blood.
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REFERENCES
Books and journals
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delirium in adult patients in the intensive care unit. Critical care medicine. 41(1).
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Goldberg, A.C and et. al., 2011. Familial hypercholesterolemia: screening, diagnosis and
management of pediatric and adult patients: clinical guidance from the National Lipid
Association Expert Panel on Familial Hypercholesterolemia. Journal of clinical
lipidology. 5(3). pp.S1-S8.
Haugen, B.R and et. al., 2016. 2015 American Thyroid Association management guidelines for
adult patients with thyroid nodules and differentiated thyroid cancer: the American
Thyroid Association guidelines task force on thyroid nodules and differentiated thyroid
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Jacobi and et. al., 2012. Guidelines for the use of an insulin infusion for the management of
hyperglycemia in critically ill patients. Critical care medicine. 40(12). pp.3251-3276.
Minet, L and et. al., 2010. Mediating the effect of self-care management intervention in type 2
diabetes: a meta-analysis of 47 randomised controlled trials. Patient education and
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Nunnally, M.E and et. al., 2011. Targeted temperature management in critical care: a report and
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pp.1113-1125.
Provan, D and et. al., 2010. International consensus report on the investigation and management
of primary immune thrombocytopenia. Blood. 115(2). pp.168-186.
Sidbury, R and et. al., 2014. Guidelines of care for the management of atopic dermatitis: section
3. Management and treatment with phototherapy and systemic agents. Journal of the
American Academy of Dermatology. 71(2). pp.327-349.
Topolovec-Vranic, J and et. al., 2010. Patient satisfaction and documentation of pain
assessments and management after implementing the adult nonverbal pain scale.
American Journal of Critical Care. 19(4). pp.345-354.
Zebrack, B. and Isaacson, S., 2012. Psychosocial care of adolescent and young adult patients
with cancer and survivors. Journal of Clinical Oncology. 30(11). pp.1221-1226.
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dictionary.thefreedictionary.com/respiratory+depth>./ [Accessed on 23rd October 2017].