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Mrs Chan - Fractured Hip

Read the workplace simulated scenarios on the following pages and answer the questions related to the information provided in each case study scenario.

Mrs Chan has been bought in by ambulance following a fall at home, where she fractured her hip. She believes she fell around 10pm last night, and was unable to get up to seek help. Her daughter found her still on the floor this morning at 6.00am and called the ambulance.

Mrs Chan’s past medical history includes:

  • Urinary incontinence
  • Osteoporosis
  • Compression fractures to her spine due osteoporosis
  • Removal of a Stage II melanoma in 2006

Regular medications:

  • Fosamax
  • Vitamin D
  • Calcium
  • Fentanyl patch

PRN endone

When Mrs Chan is admitted to hospital, she is found to have mild hypothermia.

  • Explain what hypothermia is and at least three factors that may have caused this in her case.
  • Define homeostasis and name the three components that make up any biological homeostatic control mechanism
  • Describe at least four different changes that would occur in Mrs’s Chan’s body to regain temperature homeostasis.

Further Information:

Mrs Chan had a Stage II melanoma removed in 2006 and the tissue was sent to pathology for testing.  The pathology report came back stating the tumour showed signs of dysplasia.  

  • The four major types of cellular adaptation are hypertrophy, hyperplasia, metaplasia and dysplasia.  Briefly describe each of these types of cellular adaptation
  • Define atrophy
  • What does the term ‘surgical insult’ mean?

Part of the ENs role is to assist in referring clients care to the appropriate people within a multi-disciplinary team.

  • List six (6) different professions that may be part of a multi-disciplinary team.
  • Which part of the team has the role of interpreting test results in order to formulate a diagnosis?
  • Why does the EN need to know the purpose of blood collection?
  • Can an EN disclose pathology results to their client? Give a reason for your answer.

Mr Marconi is a 76-year old man who has hypertension, hyperlipidaemia and congestive heart failure. He is brought in to the Emergency department at 06:00.  On arrival, Mr Marconi states that he has chest pain when breathing in, he is coughing up yellow phlegm and he feels exhausted.  His left arm has a shallow wound that Mr Marconi said he got while weeding his garden two days earlier.

His vital signs include:

  • Respiratory Rate: 28 bpm (shallow with pain on inspiration)
  • SaPO2: 91% (2L O2)
  • Blood Pressure: 105/70 mmHg
  • Pulse Rate: 105 bpm
  • Temperature: 38.9 degrees C
  • BGL: 4.5mmol
  • Weight: 78 kg
  • Height: 174 cm

His current medications include Atenolol, Ramipril and Lipitor.

He has no known allergies.

Describe the process of coronary artery atheroma formation - include the following in your answer:

  • Describe the steps of atheroma formation:
  • Name three factors that trigger endothelial inflammation:
  • How do high and low density lipoproteins contribute to atheroma formation?
  • What is the role of macrophages in relation to atheroma formation?
  • The patient is taking Atenolol and Ramipril. Describe in simple terms (that a patient could understand) how these two medications work in treating ischaemic heart disease.

You are asked to clean the wound on the patient’s arm.

  • Explain the structure and the function of the skin and the problems associated with a break in the skin such as this wound.
  • You notice that the patient’s skin appears thin and like ‘tissue paper’. Explain what may have caused this.

The patient wears reading glasses.  Explain degenerative changes that occur in the eye as we age and how this may have resulted Mr Marconi’s need to wear glasses.

Sarah Thompson is a 65 year old woman who presents to her GP clinic for an annual check up.  She was diagnosed with Type II Diabetes Mellitus (T2DM) in 2009:

Weight: 95kg

Height: 164cm

BMI 36

Mrs Thompson also has hyperlipidaemia and hypertension, and she is taking Metoprolol and Rosuvastatin daily.

Discuss Type 2 diabetes – include the following in your answer:

  • What body systems and/or organs can be affected by T2DM?
  • Name five risk factors for developing T2DM:
  • Describe appropriate treatment for a patient such as Mrs Thompson who has T2DM
  • Which part of the cell makes ATP (adenosine triphosphate) needed for cellular energy
  • What is required to ensure a cell remains healthy?

Define hyperlipidaemia and explain the associated risks and causes of this condition. In your answer identify the roles of high density lipoprotein (HDL), low density lipoprotein (LDL) and triglycerides.

Miss Walker is a 46-year-old female who presented to the emergency department with shortness of breath which has got progressively worse over the last two days. She has no other significant past medical history.

Mr Marconi - Congestive Heart Failure

RR:   26

SpO2:  93%.

The doctor orders a chest X-ray which shows consolidation of fluid on his right lower lung. The doctor diagnoses right lower lobe pneumonia.

Relate the diagnosis of pneumonia to two stages of respiration - external respiration and gas transport.

  • Describe what occurs during normalexternal respiration.
  • Describe what occurs during normalrespiratory gas transport. Discuss how O2 and CO2 are transported in the blood
  • Differentiate between pulmonary ventilation, internal respiration and cellular respiration.
  • Briefly explain what respiratory acidosis and respiratory alkalosis are:

Describe the steps involved in homeostasis of respiratory rate - include the following in your answer 

  • Name two (2) respiratory gases detected in the blood?
  • Where is the respiratory control centre located?  What is the stimulus that causes the respiratory control centre to increase respiratory rate?
  • Why would the patient have an increased respiratory rate if they are suffering from pneumonia?You need to explain the physiology behind why having pneumonia causes increased respiratory rate
  • How does administration of supplementary oxygen help to maintain homeostasis of respiration?
  • What impact would the administration of supplementary O2 have on the patient’s respiratory rate? What impact would the administration of supplementary O2 have on the patient’s SpO2?

Later in your shift, you find the patient crying. She tells you that she has been crying a lot lately and feeling sad since her husband asked her for a divorce. Explain psychological health as it relates to the patient and who you could refer them to.

Mrs Nguyen is a 75 year old woman who is bought into the GP clinic by her daughter Rosemary. Mrs Rosemary says that her mother has complaining of painful urination and her mental state has become confused over the past 24 hours.

The RN asks you to perform a urinalysis test.

  • Explain how you would prepare for a urinalysis test?
  • You assess the results of a urine dipstick test – list two (2) signs would you expect if a UTI was present?
  • What symptoms could the patient be experiencing if she has a UTI?
  • A urine test is then ordered for culture and sensitivity. Describe what each of these tests are evaluating and how the results relate to medication prescription.
  • Explain how the patient’s age could impact on the urinary system?

Mrs Nguyen also has had some dental problems, and lets you know that she is getting dentures in the next month or so.

  • List five (5) oral health diseases
  • What is the term used to describe people with teeth and without teeth

During assessment, Rosemary shows you Mrs Nguyen’s medication webster pack. She is concerned that there are three days of medications missing and she thinks that her mother may have forgotten that she had taken her pills and took another dose.

The medication list on the webster pack lists the drugs as:

Serepax: 10mg  at night

Metoprolol: 50 mg twice per day

Aspirin: 100mg in the morning.

Metforman: 500mg BD

  • List three (3) body systems that may be affected by the accidental ingestion of these medications.

Ruth and Michael Jackson have been trying to have a baby for the past five years.  In November 2016, Ruth gives birth to a son, Peter.  48 hours after birth, Peter undergoes the standard neonatal screening test, which helps to identify those infants at risk of rare, but serious medical conditions such as phenylketonuria, hypothyroidism and cystic fibrosis.  

Peter is found to have cystic fibrosis (CF), an autosomal recessive condition that affects mostly the lungs, but also the pancreas, liver, kidneys and small intestine.

  • Discuss difference between having the disease of CF compared to being a carrier of CF from a genetic perspective.

Further Information:

At three months, Ruth and Michael take Peter to the paediatrician for a follow up.  The paediatrician notes FTT (failure to thrive) on Peter’s medical record.  She explains to the Jackson’s that this means Peter has poor weight gain, and that he will need a high calorie diet and enzyme supplementation for the rest of this life.

  • Explain how CF causes failure to thrive and how this may impact on Peter’s biological maturation
  • Why does Peter need a high calorie diet and pancreatic enzyme supplementation?
  • How does CF contribute to malabsorption of nutrients in a teenager?

Further Information:

Ruth is worried about Peter’s future and how long he is likely to live.

  • What is the life-expectancy of a patient with CF, compared to a non-CF patient?  How has current treatment affected this, compared to a patient diagnosed with CF twenty years ago?

You are talking to Ruth during a routine visit to the GP. She says that her husband Michael has taken up scuba diving. Ruth doesn’t understand much about scuba diving and is concerned that her husband may be injured, leaving her to care for Peter.

  • List three (3) common emergencies experienced by divers.

Mrs Chan - Fractured Hip

When Mrs Chan is admitted to hospital, she is found to have mild hypothermia.

  • Explain what hypothermia is and at least three factors that may have caused this in her case.

Hypothermia refers to a condition where the body loses more heat than it produces result in a state of abnormally low temperatures.

In her case, the causes would have been:

  1. Lying on the floor for a long period increases the rate of loss of heat
  2. A removal of melanoma can alter the thermoregulatory mechanisms of the skin hence its inability to respond to decreased temperatures
  3. The use of fentanyl, an opioid, has an effect on the central thermoregulatory centres in the brain and is associated with hypothermia, (Yoo, Lee& Suk, 2015).
  • Define homeostasis and name the three components that make up any biological homeostatic control mechanism.

It refers to the continuous attempt of the body to maintain a constant internal environment

Components of a homeostatic control mechanism include:

  • Sensor
  • Control centre
  • Effector
  • Describe at least four different changes that would occur in Mrs’s Chan’s body to regain temperature homeostasis.

Shivering which increases muscle contraction to generate heat. It is a centrally mediated mechanism in the thermoregulatory centre (Werner, 2018).

The erector Pilli muscle will contract causing the hair to stand erect maintaining the air along the surface which is warm lowering the rate of diffusion

Vasoconstriction to shunt blood away from the body surface reducing the rate of loss

Increased metabolism in the liver to generate more heat through exothermic reactions. Heat resulting from theses reactions is distributed to other parts

Decreased rate of sweating to decrease the rate of heat loss through the skin.

Further Information:

Mrs Chan had a Stage II melanoma removed in 2006 and the tissue was sent to pathology for testing.  The pathology report came back stating the tumour showed signs of dysplasia.  

  • The four major types of cellular adaptation are hypertrophy, hyperplasia, metaplasia and dysplasia.  Briefly describe each of these types of cellular adaptation

Hyperplasia refers to an increase in the number of cells in a tissue. For example, the increase in cells in breast tissue in puberty due to hormonal influence.

Hypertrophy refers to an increase in the size of the cells in a tissue or organ which results in a general increase in its size. For example, hypertrophy of the cardiac muscle of the left ventricle in stenosis of the aorta.

Metaplasia refers to an abnormality in cellular differentiation. An irreversible change where an adult cell type is replaced by another adult cell type. For example, a change from columnar epithelium to stratified squamous epithelium in the respiratory epithelium of cigarette smokers

Dysplasia refers to an abnormality in cellular development. There is an abnormal cell proliferation which is usually non-neoplastic for instance an increase in the number of epithelial layers. There are also some cytology changes in the cells that differ from the normal cells. For instance, loss in the basal cell polarity.

  • Define atrophy

it refers to the shrinkage of a cell due to the loss of cellular substance resulting in the decrease in size of a tissue or the organ. May result from factors such as decreased work load, diminished blood supply or loss of innervation.

  • What does the term ‘surgical insult’ mean?

Mr Marconi - Congestive Heart Failure

It refers to the physiological response of the body following stress inflicted on the body due to a surgical procedure. It is usually a multisystem approach producing a particular outcome. They all produce an additive effect.

Part of the ENs role is to assist in referring clients care to the appropriate people within a multi-disciplinary team.

  • List six (6) different professions that may be part of a multi-disciplinary team.

General practitioner

Practice nurses and Community health nurse

Psychiatrists

Physiotherapists and occupational therapists

Dieticians

Social workers

Health educators

  • Which part of the team has the role of interpreting test results in order to formulate a diagnosis?

The general practitioner is usually the first contact with the patient. They take history, examine and order for tests to confirm a diagnosis.

In the case of a mental illness, a psychiatrist will make the diagnosis and interpret the tests.

A nursing diagnosis is done and the care plan formulated.

  • Why does the EN need to know the purpose of blood collection?

In order to collect it in the right specimen bottle for a particular intended test. For example, blood for hemogram would be differently collected than that for a coagulation screen.

In order to send it to the right laboratory for testing and confirmation of diagnoses.

  • Can an EN disclose pathology results to their client? Give a reason for your answer.

No.

Pathology results can only be well interpreted be the doctor who requested the test. The doctor is only one in the best position to disclose them since after taking a history and making the examination, they can make a meaningful diagnosis.

Mr Marconi is a 76-year old man who has hypertension, hyperlipidaemia and congestive heart failure. He is brought in to the Emergency department at 06:00.  On arrival, Mr Marconi states that he has chest pain when breathing in, he is coughing up yellow phlegm and he feels exhausted.  His left arm has a shallow wound that Mr Marconi said he got while weeding his garden two days earlier.

His vital signs include:

  • Respiratory Rate: 28 bpm (shallow with pain on inspiration)
  • SaPO2: 91% (2L O2)
  • Blood Pressure: 105/70 mmHg
  • Pulse Rate: 105 bpm
  • Temperature: 38.9 degrees C
  • BGL: 4.5mmol
  • Weight: 78 kg
  • Height: 174 cm

His current medications include Atenolol, Ramipril and Lipitor.

He has no known allergies.

Describe the process of coronary artery atheroma formation - include the following in your answer:

  • Describe the steps of atheroma formation:

Chronic endothelial injury- the exposure of the endothelium to agents such as toxins viruses, agents causing inflammation. Hypertension also causes endothelial damage.

repeated exposure to a harmful stimulus results in the endothelial wall of the coronary vessels becoming dysfunctional. This results in increased permeability of the endothelium causing emigration of the leukocytes and monocytes.

The macrophages and smooth muscle cells migrate into the intimal layer of the blood vessels. smooth muscles underdo proliferation while the macrophages undergo activation. Due to the dysfunction of the vessel the lipids get into the intima layer and are taken up by the macrophages resulting in foam cell formation. Smooth muscle cell proliferation and deposition of the ECM results in the thickening of the intima with formation of an atheroma formation

  • Name three factors that trigger endothelial inflammation:

Sarah Thompson - Type II Diabetes Mellitus

Infection

Toxins

Endothelial wall dysfunction. Expression of receptors for inflammatory cells

  • How do high and low density lipoproteins contribute to atheroma formation?

They are oxidised by the free radicals released by the macrophages, making them bind to the scavenger receptor of the macrophage where they are internalised leading to formation of foam cells. Oxidised lipoproteins promote the production and release of cytokines and chemokines which promote increased recruitment of leukocyte. These facilitates atheroma formation and intimal thickening.

  • What is the role of macrophages in relation to atheroma formation?

They take up oxidised LDL forming the foam cells.

Dying macrophages form the necrotic core

Release radicals that cause the oxidation of LDL

Promote the release of pro inflammatory and anti-inflammatory cytokines, (Ramji, & Davies, 2015).

  • The patient is taking Atenolol and Ramipril. Describe in simple terms (that a patient could understand) how these two medications work in treating ischaemic heart disease.

They allow the blood vessels to remain wide facilitating the blood to reach the cardiac muscles thus preventing the sharp pain the results from the increased oxygen requirement or decreased nourishment of the muscles.

You are asked to clean the wound on the patient’s arm.

  • Explain the structure and the function of the skin and the problems associated with a break in the skin such as this wound.

The skin is made up of the epidermis which consists of stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum and stratum basale. The dermis is the second layers composed of receptors sensitive to touch and heat, sebaceous glands, hair follicles and blood vessels. The subcutaneous layer also the hypodermis made up of fat. (Archer,2012) states that the skin plays important roles such as heat conservation, acts as a barrier to infectious microorganisms, excretion of wastes and promotes sensation. A break into the skin causes risk of deeper infection which may lead to septicaemia. There is also blood loss as a result of damage to the blood vessels. Healing may be poor if the wound is huge.

  • You notice that the patient’s skin appears thin and like ‘tissue paper’. Explain what may have caused this.

This might have been caused by the application of medicines while dressing the wound and also application of tight dressing while nursing the wound leading to the skin appearing thin.

The patient wears reading glasses.  Explain degenerative changes that occur in the eye as we age and how this may have resulted Mr Marconi’s need to wear glasses.

The vitreous humor of the eye with age becomes less dense and fibres holding it detach from the retina this may cause changes in vision and constant floaters. According to (Spitzer and Januschowski,2015) the humor liquefies causing reduction in its volume due to increased age since there is change in molecular organization of hyaluronic acid. Also, with age the number of multiplying cells is decreased while cell death is occurring in the cornea and retina leading to vision loss. Presbyopia refers to the changes that occur with age as to inability to focus on near objects. This necessitated Mr Marconi to wear glasses as they correct the refractive error.

Miss Walker - Shortness of Breath

Sarah Thompson is a 65 year old woman who presents to her GP clinic for an annual check up.  She was diagnosed with Type II Diabetes Mellitus (T2DM) in 2009:

Weight: 95kg

Height: 164cm

BMI 36

Mrs Thompson also has hyperlipidaemia and hypertension, and she is taking Metoprolol and Rosuvastatin daily.

Discuss Type 2 diabetes – include the following in your answer:

  • What body systems and/or organs can be affected by T2DM?

Cardiovascular system

The CNS

The kidneys

The eye

  • Name five risk factors for developing T2DM:

Obesity 120 percent greater than desired.

Family history of type 2 diabetes.

Age above 45 years

History of gestational diabetes

Hypertension

  • Describe appropriate treatment for a patient such as Mrs Thompson who has T2DM

Oral antidiabetic drugs metformin

  • Which part of the cell makes ATP (adenosine triphosphate) needed for cellular energy

Mitochondria.

  • What is required to ensure a cell remains healthy?

Proper nutrition to ensure availability of nutrients to the cell. A balanced nutrition provides proper nutrients to the cells such as proteins, fats and carbohydrates.

Define hyperlipidaemia and explain the associated risks and causes of this condition. In your answer identify the roles of high density lipoprotein (HDL), low density lipoprotein (LDL) and triglycerides.

Hyperlipidaemia refers to the state in which the levels of lipids is abnormally high. The high density lipoprotein is the cholesterol protective to the human body by decreasing the amount of low density lipoprotein while low density lipoprotein is harmful as it causes thickening of the arteries a condition known as atherosceloris.Triglycerides refer to stored fat in the body cells.Hyperlipidemia is associated with genetics, obesity  and metabolic conditions such as diabetes.( Nestruck& Davignon,2012) states that hyperlipidaemia is associated with the risk of myocardial infarction and ischemic heart attack due to narrowing of the heart arteries

Miss Walker is a 46-year-old female who presented to the emergency department with shortness of breath which has got progressively worse over the last two days. She has no other significant past medical history.

RR:   26

SpO2:  93%.

The doctor orders a chest X-ray which shows consolidation of fluid on his right lower lung. The doctor diagnoses right lower lobe pneumonia.

Relate the diagnosis of pneumonia to two stages of respiration - external respiration and gas transport.

  • Describe what occurs during normalexternal respiration.

There is air entry through the nose which is filtered by small hairs found in the nose and warmed by the blood in the capillaries while also humidification occurs. Any noxious particles are trapped by the cilia and trapped preventing further entry. The air then flows into the trachea then bronchi then bronchioles and finally into the alveoli. The oxygen is exchanged into the blood cells while carbon (IV) oxide diffuses into the alveoli.

  • Describe what occurs during normalrespiratory gas transport. Discuss how O2 and CO2 are transported in the blood.

When the deoxygenated blood is brought to the lung by the pulmonary artery it enters into the capillaries lining the alveoli whose oxygen saturation is higher than that in the blood diffuses into the blood while carbon (IV) oxide diffuses into the alveoli. Oxygen is transported in the blood by binding to the haemoglobin in the red blood cells to form oxyhemoglobin while majority of carbon (IV)oxide binds to haemoglobin as a bicarbonate ion.

  • Differentiate between pulmonary ventilation, internal respiration and cellular respiration.

Mrs Nguyen - Painful Urination

Pulmonary ventilation refers to process of movement of air in and out of the lungs (McLafferty, Johnstone, Hendry& Farley,2013). Internal respiration refers to the exchange of blood gases between blood and cells while cellular respiration refers to the breakdown of nutrients into adenosine triphosphate for cellular energy.

  • Briefly explain what respiratory acidosis and respiratory alkalosis are:

Respiratory acidosis is a decrease in blood pH due to retained carbon (IV)oxide while respiratory alkalosis is an increase in blood pH due to decreased arterial carbon (IV)oxide as result of hyperventilation.

Describe the steps involved in homeostasis of respiratory rate - include the following in your answer 

  • Name two (2) respiratory gases detected in the blood?

Oxygen

Carbon (IV)oxide

  • Where is the respiratory control centre located?  What is the stimulus that causes the respiratory control centre to increase respiratory rate?

Respiratory control centre is located in the medulla oblangata in the brain stem. The respiratory rate is increased while the control centre senses a decrease in blood pH meaning increased level of carbon (IV)oxide.

  • Why would the patient have an increased respiratory rate if they are suffering from pneumonia?You need to explain the physiology behind why having pneumonia causes increased respiratory rate

Pneumonia affects the alveoli by causing infection; immunological cells attempts to fight off the infection so the cell debris and pus accumulate in the alveoli compromising the gaseous exchange this leads to an increased level of carbon (IV)oxide. The patient will have an increased respiratory rate in attempt to excrete the excess levels of carbon (IV)oxide.

  • How does administration of supplementary oxygen help to maintain homeostasis of respiration?

Supplementary oxygen ensures compensation of reduced oxygen in circulation in the body.

  • What impact would the administration of supplementary O2 have on the patient’s respiratory rate? What impact would the administration of supplementary O2 have on the patient’s SpO2?

Supplementary oxygen causes a decrease in patient’s respiratory rate while the oxygen saturation increases when a patient is administered with supplementary oxygen.

Later in your shift, you find the patient crying. She tells you that she has been crying a lot lately and feeling sad since her husband asked her for a divorce. Explain psychological health as it relates to the patient and who you could refer them to.

Psychological health refers to the wellness to function mentally and adapt to various situations spiritually, socially, emotionally and physically. I could refer them to a counsellor and a psychologist.

Mrs Nguyen is a 75 year old woman who is bought into the GP clinic by her daughter Rosemary. Mrs Rosemary says that her mother has complaining of painful urination and her mental state has become confused over the past 24 hours.

The RN asks you to perform a urinalysis test.

  • Explain how you would prepare for a urinalysis test?

Collection of all the needed instruments such as the urine dipstick and the specimen bottle. Advise Mrs Nguyen to collect only the midstream urine and provide her space for privacy.

  • You assess the results of a urine dipstick test – list two (2) signs would you expect if a UTI was present?

Presence of pus cells in the urine

Presence of leukocytes in the urine dipstick test

  • What symptoms could the patient be experiencing if she has a UTI?

Pain in the flank

Ruth and Michael Jackson - Cystic Fibrosis

Vomiting

Headache

Fever

  • A urine test is then ordered for culture and sensitivity. Describe what each of these tests are evaluating and how the results relate to medication prescription.

Urine culture and sensitivity is used to evaluate what specific microorganism is causing the urinary tract infection. The results of this test will ensure the specific medication prescribed is sensitive to the causative microorganism

  • Explain how the patient’s age could impact on the urinary system?

According to (Mody, L. and Juthani-Mehta,2014) hormonal changes such as decreased estrogen contributes to urinary tract infection. Also, with age the bladder and ureter muscles tend to be weak causing stasis of urine hence increased chances of urinary tract infections.

Mrs Nguyen also has had some dental problems, and lets you know that she is getting dentures in the next month or so.

  • List five (5) oral health diseases

Dental caries

Periodontal gum disease

Gingivitis

Oral herpes

Cleft lip and palate

  • What is the term used to describe people with teeth and without teeth

Edentulism

During assessment, Rosemary shows you Mrs Nguyen’s medication webster pack. She is concerned that there are three days of medications missing and she thinks that her mother may have forgotten that she had taken her pills and took another dose.

The medication list on the webster pack lists the drugs as:

Serepax: 10mg  at night

Metoprolol: 50 mg twice per day

Aspirin: 100mg in the morning.

Metforman: 500mg BD

List three (3) body systems that may be affected by the accidental ingestion of these medications.

Neurological system can be affected by accidental ingestion of serepax

Gastrointestinal system can be affected by accidental ingestion of metoprolol

Genitourinary system can be affected by accidental ingestion of aspirin

Ruth and Michael Jackson have been trying to have a baby for the past five years.  In November 2016, Ruth gives birth to a son, Peter.  48 hours after birth, Peter undergoes the standard neonatal screening test, which helps to identify those infants at risk of rare, but serious medical conditions such as phenylketonuria, hypothyroidism and cystic fibrosis.  

Peter is found to have cystic fibrosis (CF), an autosomal recessive condition that affects mostly the lungs, but also the pancreas, liver, kidneys and small intestine.

  • Discuss difference between having the disease of CF compared to being a carrier of CF from a genetic perspective.

In a person who has cystic fibrosis the genes are autosomal dominant meaning that the first allele is dominant with cystic fibrosis trait and hence there are signs of cystic fibrosis. The carrier of cystic fibrosis, the genes are autosomal recessive meaning the signs are not evident but their offspring may have cystic fibrosis.

Further Information:

At three months, Ruth and Michael take Peter to the paediatrician for a follow up.  The paediatrician notes FTT (failure to thrive) on Peter’s medical record.  She explains to the Jackson’s that this means Peter has poor weight gain, and that he will need a high calorie diet and enzyme supplementation for the rest of this life.

  • Explain how CF causes failure to thrive and how this may impact on Peter’s biological maturation

Hypothermia and Cellular Changes

Cystic fibrosis causes a defect in the makeup of the endocrine cells and tissues by production of excess mucus hence no production of hormones influencing the growth and maturation. According to (

Giglio, Candusso, D’orazio, Mastella, Faraguna,2012) the earliest sign of an infant with cystic fibrosis is failure to thrive due to the sticky meconium that blocks the intestines leading to poor weight gain. This will impact on Peter’s maturation since the absorption of required nutrients for growth will be slow.

  • Why does Peter need a high calorie diet and pancreatic enzyme supplementation?

High calorie diet is important since there is slow absorption of nutrients so there is need to ensure whatever is absorbed provides maximum energy. Peter needs pancreatic enzyme supplements as the pancreas is affected by cystic fibrosis and produces mucus instead of the essential pancreatic enzymes

  • How does CF contribute to malabsorption of nutrients in a teenager?

According to (Barni, Forte., Forgiarini, Abrahão, Dalcin,2017) states that declining lung function causes decreased nutrient absorption. Constant lung infections lead to increased calorie needs while at the same time decreased appetite

Further Information:

Ruth is worried about Peter’s future and how long he is likely to live.

  • What is the life-expectancy of a patient with CF, compared to a non-CF patient?  How has current treatment affected this, compared to a patient diagnosed with CF twenty years ago?

The life expectancy of a patient with cystic fibrosis is 37 years while that of a non-cystic fibrosis is 65 years. According to (Cohen-Cymberknoh, Shoseyov & Kerem,2011) strategies such as treatment targeting lung infections and nutritional adequacy have greatly improved prognosis compared to twenty years ago.

You are talking to Ruth during a routine visit to the GP. She says that her husband Michael has taken up scuba diving. Ruth doesn’t understand much about scuba diving and is concerned that her husband may be injured, leaving her to care for Peter.

  • List three (3) common emergencies experienced by divers.

Hypothermia due to extreme cold in the water

Arterial gas embolism which causes death easily.

Barotrauma which refers to injury caused by pressure differences in air and body volume such as sinuses

Insert Reference List on following page:

References

Archer, C. B. (2015). Functions of the skin. Rook's textbook of dermatology, 129-140.

Barni, G. C., Forte, G. C., Forgiarini, L. F., Abrahão, C. L. D. O., & Dalcin, P. D. T. R. (2017). Factors associated with malnutrition in adolescent and adult patients with cystic fibrosis. Jornal Brasileiro de Pneumologia, 43(5), 337-343.

Cohen-Cymberknoh, M., Shoseyov, D., & Kerem, E. (2014). Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. American journal of respiratory and critical care medicine, 183(11), 1463-1471.

Giglio, L., Candusso, M., D'orazio, C., Mastella, G., & Faraguna, D. (2015). Failure to thrive: the earliest feature of cystic fibrosis in infants diagnosed by neonatal screening. Acta Paediatrica, 86(11), 1162-1165.

McLafferty, E., Johnstone, C., Hendry, C., & Farley, A. (2013). Respiratory system part 1: pulmonary ventilation. Nursing Standard, 27(22).

Mody, L., & Juthani-Mehta, M. (2014). Urinary tract infections in older women: a clinical review. Jama, 311(8), 844-854.

Nestruck, A. C., & Davignon, J. (2014). Risks for hyperlipidemia. Cardiology clinics, 4(1), 45-56.

Ramji, D. P., & Davies, T. S. (2015). Cytokines in atherosclerosis: Key players in all stages of disease and promising therapeutic targets. Cytokine & growth factor reviews, 26(6), 673-685.

Spitzer, M. S., & Januschowski, K. (2015). Aging and age-related changes of the vitreous body. Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft, 112(7), 552-554.

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