Cystic fibrosis is a genetic disorder that affects the lungs, pancreas, and sweat glands. It causes thick, sticky mucus to build up in these areas. This leads to frequent lung infections, issues digesting and absorbing food, and salty sweat. While there is no cure, treatments aim to clear mucus from the lungs, prevent infections, and ensure adequate nutrition. Nursing care focuses on airway clearance techniques, administering enzymes to aid digestion, monitoring for complications, and supporting patients and their families.
THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.
Cystic fibrosis (also known as CF or mucoviscidosis) is a recessive multi-system genetic disease characterized by abnormal transport of chloride and sodium across epithelium, leading to thick, viscous secretions in the lungs, pancreas, liver, intestine.[Yankas JR, et al. (2004). "Cystic fibrosis adult care consensus conference report". Chest 125: 1-39.]
THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.
Cystic fibrosis (also known as CF or mucoviscidosis) is a recessive multi-system genetic disease characterized by abnormal transport of chloride and sodium across epithelium, leading to thick, viscous secretions in the lungs, pancreas, liver, intestine.[Yankas JR, et al. (2004). "Cystic fibrosis adult care consensus conference report". Chest 125: 1-39.]
1Case Study Cystic FibrosisCystic Fibrosis Case StudyEttaBenton28
1
Case Study Cystic Fibrosis
Cystic Fibrosis: Case Study
The patient is a 7-year-old female. Due to the fact that the patient is a minor, her mother walked into the ER with her. The mother explained that her symptoms consist of persistent coughing throughout the day and it becomes worse at night, the coughing often results in spitting out phlegm. Along with wheezing, stuffy nose, loss of appetite, pain in the abdomen, and the taste of salty skin. The patient has been up coughing and complaining about the pain for 5 hours. Since the patient’s mother had thought it was a regular cold, she had been giving her children’s Tylenol for the past 3 days, 5 mL every 4 hours. Once the patient was taken in, a physical assessment was performed. The questions that were asked to the patients mother were about family history of CF, history of bowel obstruction as an infant, stool, and eating habits (nurses labs 2018). Because of the fact that the patient’s mom mentioned her skin having a salty taste, a sweat test has been recommended to check for cystic fibrosis. The sweat test measures the amount of chloride in sweat while the genetic test detects chromosomal mutations (Very Well Health 2019). Patient has now been diagnosed with cystic fibrous and will be admitted to the hospital for further instructions.
Pathophysiology
The pathophysiology of cystic fibrosis is based on the defects in the cystic fibrosis gene, which codes for protein transmembrane conductance regulator (CFTR) that functions as a chloride channel and is regulated by cyclic adenosine monophosphate (cAMP) (Nurselabs 2018). Cystic fibrosis is often caught at birth but there are cases where people begin to develop it later in life. If it is detected as birth, many persons with cystic fibrosis acquire a lung infection which incites an inflammatory response, the infection becomes established with a distinctive bacterial flora (Nurselabs 2018). If there is a case of a person developing cystic fibrosis as they get older, there are many symptoms that come along with it and it does affect many parts of your body. It can affect your GI tract, pancreas and your sweat glands which causes the skin to taste salt like. Symptoms may include pain in the abdomen, having a chronic cough that may include blood or phlegm, any gastrointestinal problems such as diarrhea, fat in the stool, heartburn, severe constipation, or bulky stools. When it affects your respiratory system, it can cause pulmonary hypertension, shortness of breath, sinusitis, wheezing, acute bronchitis, and/or pneumonia. There might also be a delay in puberty, growth, and in development. Other commons side effects include deformity of nails, different infections, male infertility, nasal polyps or weight loss (Mayo Clinic 2020).
History
The symptoms presented by the patient include coughing up phlegm, wheezing, stuffy nose, pain in the abdomen, salty skin and loss of appetite. In the past the patient has h ...
This powerpoint gives a brief introduction to Cystic Fibrosis. I created it for research, educational, and promotional purposes. I hope that someone who watches it can learn something about cf that they didn't know before.
NOTE: You'll find it easier to maneuver through the powerpoint if you flip each slide individually, otherwise the slides are too fast. Use the two buttons closest to the center triangle to do so.
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QA Paediatric dentistry department, Hospital Melaka 2020Azreen Aj
QA study - To improve the 6th monthly recall rate post-comprehensive dental treatment under general anaesthesia in paediatric dentistry department, Hospital Melaka
Leading the Way in Nephrology: Dr. David Greene's Work with Stem Cells for Ki...Dr. David Greene Arizona
As we watch Dr. Greene's continued efforts and research in Arizona, it's clear that stem cell therapy holds a promising key to unlocking new doors in the treatment of kidney disease. With each study and trial, we step closer to a world where kidney disease is no longer a life sentence but a treatable condition, thanks to pioneers like Dr. David Greene.
R3 Stem Cells and Kidney Repair A New Horizon in Nephrology.pptxR3 Stem Cell
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CRISPR-Cas9, a revolutionary gene-editing tool, holds immense potential to reshape medicine, agriculture, and our understanding of life. But like any powerful tool, it comes with ethical considerations.
Unveiling CRISPR: This naturally occurring bacterial defense system (crRNA & Cas9 protein) fights viruses. Scientists repurposed it for precise gene editing (correction, deletion, insertion) by targeting specific DNA sequences.
The Promise: CRISPR offers exciting possibilities:
Gene Therapy: Correcting genetic diseases like cystic fibrosis.
Agriculture: Engineering crops resistant to pests and harsh environments.
Research: Studying gene function to unlock new knowledge.
The Peril: Ethical concerns demand attention:
Off-target Effects: Unintended DNA edits can have unforeseen consequences.
Eugenics: Misusing CRISPR for designer babies raises social and ethical questions.
Equity: High costs could limit access to this potentially life-saving technology.
The Path Forward: Responsible development is crucial:
International Collaboration: Clear guidelines are needed for research and human trials.
Public Education: Open discussions ensure informed decisions about CRISPR.
Prioritize Safety and Ethics: Safety and ethical principles must be paramount.
CRISPR offers a powerful tool for a better future, but responsible development and addressing ethical concerns are essential. By prioritizing safety, fostering open dialogue, and ensuring equitable access, we can harness CRISPR's power for the benefit of all. (2998 characters)
Struggling with intense fears that disrupt your life? At Renew Life Hypnosis, we offer specialized hypnosis to overcome fear. Phobias are exaggerated fears, often stemming from past traumas or learned behaviors. Hypnotherapy addresses these deep-seated fears by accessing the subconscious mind, helping you change your reactions to phobic triggers. Our expert therapists guide you into a state of deep relaxation, allowing you to transform your responses and reduce anxiety. Experience increased confidence and freedom from phobias with our personalized approach. Ready to live a fear-free life? Visit us at Renew Life Hypnosis..
Medical Technology Tackles New Health Care Demand - Research Report - March 2...pchutichetpong
M Capital Group (“MCG”) predicts that with, against, despite, and even without the global pandemic, the medical technology (MedTech) industry shows signs of continuous healthy growth, driven by smaller, faster, and cheaper devices, growing demand for home-based applications, technological innovation, strategic acquisitions, investments, and SPAC listings. MCG predicts that this should reflects itself in annual growth of over 6%, well beyond 2028.
According to Chris Mouchabhani, Managing Partner at M Capital Group, “Despite all economic scenarios that one may consider, beyond overall economic shocks, medical technology should remain one of the most promising and robust sectors over the short to medium term and well beyond 2028.”
There is a movement towards home-based care for the elderly, next generation scanning and MRI devices, wearable technology, artificial intelligence incorporation, and online connectivity. Experts also see a focus on predictive, preventive, personalized, participatory, and precision medicine, with rising levels of integration of home care and technological innovation.
The average cost of treatment has been rising across the board, creating additional financial burdens to governments, healthcare providers and insurance companies. According to MCG, cost-per-inpatient-stay in the United States alone rose on average annually by over 13% between 2014 to 2021, leading MedTech to focus research efforts on optimized medical equipment at lower price points, whilst emphasizing portability and ease of use. Namely, 46% of the 1,008 medical technology companies in the 2021 MedTech Innovator (“MTI”) database are focusing on prevention, wellness, detection, or diagnosis, signaling a clear push for preventive care to also tackle costs.
In addition, there has also been a lasting impact on consumer and medical demand for home care, supported by the pandemic. Lockdowns, closure of care facilities, and healthcare systems subjected to capacity pressure, accelerated demand away from traditional inpatient care. Now, outpatient care solutions are driving industry production, with nearly 70% of recent diagnostics start-up companies producing products in areas such as ambulatory clinics, at-home care, and self-administered diagnostics.
Global launch of the Healthy Ageing and Prevention Index 2nd wave – alongside...ILC- UK
The Healthy Ageing and Prevention Index is an online tool created by ILC that ranks countries on six metrics including, life span, health span, work span, income, environmental performance, and happiness. The Index helps us understand how well countries have adapted to longevity and inform decision makers on what must be done to maximise the economic benefits that comes with living well for longer.
Alongside the 77th World Health Assembly in Geneva on 28 May 2024, we launched the second version of our Index, allowing us to track progress and give new insights into what needs to be done to keep populations healthier for longer.
The speakers included:
Professor Orazio Schillaci, Minister of Health, Italy
Dr Hans Groth, Chairman of the Board, World Demographic & Ageing Forum
Professor Ilona Kickbusch, Founder and Chair, Global Health Centre, Geneva Graduate Institute and co-chair, World Health Summit Council
Dr Natasha Azzopardi Muscat, Director, Country Health Policies and Systems Division, World Health Organisation EURO
Dr Marta Lomazzi, Executive Manager, World Federation of Public Health Associations
Dr Shyam Bishen, Head, Centre for Health and Healthcare and Member of the Executive Committee, World Economic Forum
Dr Karin Tegmark Wisell, Director General, Public Health Agency of Sweden
2. DEFINITION:
Cystic Fibrosis (CF) is a disorder of the endocrine
glands that affects primarily the lungs, gastrointestinal
(GI) tract and sweat glands.
CF is a genetic disorder and was thought to be just
a childhood disease, because most affected children
did not survive past puberty. However, with new
treatments, Patients with CF are lining longer and more
productive lives. Some CF patients now marry, have
careers and live well into their thirties.
CF is the most common fatal genetic disease of
children of European-American heritage.
3. PATHOPHYSIOLOGY:
CF, affects primarily the lungs, GI tract and
sweat glands. The disease varies in severity;
some patients have no GI involvement.
Abnormal sodium and chloride transport across
cell membranes, causing thick, tenacious
secretions, is responsible for many of the
characteristic symptoms.
Thick, sticky respiratory secretions that are
difficult to remove cause airway obstruction,
resulting in frequent respiratory infections.
4. CONT…..
Similar abnormalities in the pancreas cause
blocked ducts and retained digestive enzymes.
These retained enzymes digest and destroy the
exocrine pancreas.
The absence of digestive enzymes in the
intestines causes:
5. CONT….
a) Malabsorption of essential nutrients
b) Frequent foul smelling Fatty stools
c) Excess flatus
Patients with CF secrete sweat that is high in
sodium and chloride because these
electrolytes are not reabsorbed as they pass
through the sweat ducts.
6. ETIOLOGY:
Hereditary
Both parents must be carriers of the
defective gene for CF to be present in a
child.
Pancreatic insufficiency
7. CLINICAL MANIFESTATION:
Presentation usually occurs younger than age 6
months but may occur at any age. Signs and
symptoms and severity of the disease vary and
change over time as the disease progresses.
RESPIRATORY MANIFSTATION:
1. Recurrent pulmonary infections-H. influenza, S.
aureus, P. aeruginosa.
2. Cough, dry to productive. Chronic clearing of throat
may indicate increased mucus production.
8. CONT….
3. Wheezing, crackles on auscultation are indicative
of respiratory exacerbation.
4. Dyspnea
5. Barrel-shaped chest (increased anteroposterior
chest diameter).
6. Cyanosis
7. Clubbing of fingers and toes
8. Nasal polyps and pansinusitis
9. CONT…..
GI MANIFESTATION:
1. Meconium ileus found in neonates.
2. Failure to thrive and failure to gain weight in the
presence of a good appetite
3. Abdominal distention
4. Vomiting, dehydration and electrolyte imbalance
5. Maldigestion, steatorrhea (fatty stools, loss of fat
soluble vitamins).
6. Rectal polapse
11. CONT…
OTHER MANIFESTATIONS:
1. Thin extremities, sallow skin, wasted buttocks.
2. Hyperglycemia, glucosuria, polyuria, weight loss.
3. Salty taste when parents kiss the skin.
4. Sterility in males
5. Hypoproteinemia and anemia6. Bleeding diathesis
7. Hyponatremia and heat prostration
8.Kyphosis
12. COMPLICATIONS:
Patients with CF are at high risk for a variety of
complications including:
-bronchiectasis
-pneumothorax
-corpulmonale
-respiratory failure
-bowel obstruction can occur as a result of thick mucus
binding with poorly digested fecal matter.
-Diabetes from pancreatic islet cell involvement may
be present late in the disease.
13. DIAGNOSTIC TESTS:
Quantitative sweat chloride test; pilocarpine
iontophoresis, performed at a CF foundation
accredited center by skilled personnel.
Measures sodium and chloride content in sweat.
a) Chloride level greater than 60mEq/L is
diagnostic.
b) Chloride of 4o to 60mEq/L is borderline and
should be repeated, followed by genotype for
the most frequent CFTR mutations.
c) Sodium level greater than 60mEq/L is
diagnostic.
14. CONT…..
Measurement of trypsin concentration in
duodenal secretions; absence of normal
concentration virtually diagnostic.
Analysis of digestive enzymes (trypsin and
chymotrypsin ) in stool- reduced, used for initial
screening for CF.
Chest x-ray may be normal initially; later shows
areas of infection, overinflation, bronchial
thickening and plugging, atelectasis, fibrosis and
emphysema.
15. CONT…..
Sinus radiograph or computed tomography shows
mucus plugging.
Analysis of stool for steatorrhea.
Sputum or throat cultures to rule out infection.
Pulmonary function studies (after age 4)
a) Decreased vital capacity and flow rates.
b) Increased residual volume or increased total
lung capacity.
16. CONT….
Present diagnostic tests- prenatal genetic
screening for families affected with CF.
Neonatal screening: immunoreactive
trypsinogen ; if elevated, DNA assay for
single and multiple CFTR mutations.
17. MEDICAL TREATMENT:
No cure for CF, Treatment is aimed at
relieving symptoms.
Hydration, flutter mucous clearance device
and chest (CPT) physiotherapy up to 4
times/day.
Regular exercise.
Hot shower.
Nebulize mist treatment using saline or
mucolytic medications before CPT.
18. CONT….
High doses of ibuprofen.
Breathing exercise, incentive spirometry and
effective coughing techniques.
Lung transplant is potentially promising treatment.
Antibiotics / prophylatic antibiotic therapy.
Pancreatic enzyme replacement
An increase in calories requirements necessitates a
high calorie, nutrient-dense diet.
19. ASSESSMENT
1. Check for family history of CF ,failure to thrive
;check for clients history and physical condition.
Carefully listen for subtle information that may
suggest CF.
2. Assess respiratory status; respiratory rate, presence
of tachypnea, wheeze, cough, character of sputum,
and oxygen saturation saturation level.
- Increased work of breathing.
- Quality of breath sounds by auscultation.
- Ability to participate in activities of daily living ,
exercise tolerance, quality of sleep.
20. - Assess for oxygen desaturation with sleep.
3. Assess nutritional status and characteristics
of stool.
21. NURSING DIAGNOSIS:
Ineffective airway clearance related to thick pulmonary
secretion.
Risk for infection related to thick , tenacious secretions.
Imbalanced nutrition: less than body requirements
related to decreased appetite .
Disturbed body image related to chronic disease
process.
Interrupted family processes related to child with
a chronic disease.
22. NURSING INTERVENTIONS
Promoting Airway clearance:
1. Use intermittent nebulizer therapy three to four times
per day when client is symptomatic.
- Use pretreatment postural drainage.
- Administer bronchodilators and other medications,
diluted in normal saline, in aerosal form to penetrate
respiratory tract.
2. Perform CPT 3-4 times after nebulization if infection
is present.
- Perform prior to meals or 1hour after eating to prevent
vomiting or discomfort
23. - Place the client in a position that gives the greatest
access to affected lobes of lung and facilitate gravity
drainage of mucus from the lung area.
- Client with gastro- esophageal reflux should not use
head down position.
3. Help client to relax to cough more easily.
4. Suction the cough if necessary and monitor oxygen
saturation levels during procedures.
5. Monitor for haemoptysis and provide treatment for it
.
6. Maintain cautious oxygen therapy due to chronic
CO2 retention.
24. Preventing infection:
1. Provide frequent mouth care to reduce chance of
infection because mucus is present.
2. Administer antibiotics as prescribed if client is
symptomatic.
3. Monitor closely for deteriorating respiratory status.
4. Provide good skin care and position changes to
prevent skin breakdown.
Promoting adequate nutrition:
1. Encourage diet composed of high calories and
protein foods and moderate to high fat because
25. absorption of food is incomplete.
2. Administer fat soluble vitamin in water miscible
solution three to four times as prescribed.
3. Administer pancreatic enzymes with each
meals and snacks .dose is based on client’s
weight, weight gain, growth , food intake and
character of bowel movements.
4. Increase salt intake in hot weather, fever, or
excessive exercise to prevent sodium depletion.
5. Check weights at least weekly to assess
nutritional interventions.
26. Enhancing self esteem and Body image
1. Explain each procedure, medication and treatment
to client as appropriate to age.
2. Allow client to show frustration , fears and feelings by
talking , complaining or crying.
3. Provide diversional activities according to client’s
interest.
4. Help the client to indentify the strengths and
limitations and to feel good about self.
5. Encourage regular exercise and activity to foster a
sense of accomplishment and independence and to
improve pulmonary function.
27. Family education and Health maintenance:
1. Teach parents or family members to have through
understanding of the dietary regimen and special
need for calorie , fat and vitamins.
2. Help the family members to schedule care for client
within framework of family life.
3. Provide emotional support to client and family
members.
4. Stress the importance of regular medical care.
5. Stress the importance of follow up and regular
health care .
28. Discuss with family members about limitation
and expectation for client.
7. Investigate homecare facilities for families
and especially respite services for
caregivers.
29. EXPECTED OUTCOMES
Tolerates CPT four times per day for 30
minutes with stable oxygen saturation.
No signs of respiratory infection.
Eats well with no vomiting ; weight stable.
Family caretakers take part in care of their
client.