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Cystic FibrosisPresented by Ofori-Addo, Eugene
The Disease – Cystic Fibrosis Cystic fibrosis is known to be an inherited
autosomal (pattern of inherited genetic disease) chronic disorder mostly seen in children that affects the lungs and the digestive system.
It is also known to be most common cause
of chronic lung disease mostly seen in children
and younger adults.
Also known to be the most fatal hereditary disorder that affects numerous Caucasians in the United States (Medline Plus, 2013, Cystic Fibrosis Foundation)
Statistics As the most common Chronic
lung disease in children and some adults, this disorder affects about- 30, 000 children and young adults Where about 1200 children are diagnosed
every year About 70% of these patients are diagnosed
with the disease at age 2 And about bout 40% of the population
being 18 years of age and older. Life span of patients with Cystic Fibrosis is
about 37 years (Cystic Fibrosis Foundation, 2014)
* Respiratory failure is mostly the major consequence of Cystic Fibrosis and usually what causes death amongst CF Patients
Population
Children age 2- 70%Young Adults - 40%
Causes of Cystic FibrosisThis is an inherited diseases complication so its is genetically transmitted
From parents through Genes 2 copies of genes are needed to
signify inheritance of the disease
Pathophysiology Cystic Fibrosis becomes
present when there is a Defective gene i.e. related to
protein that is involved in chloride ion transport
Patients with the diseases usually develop unusual thick sticky mucus plugging that clogs the lungs and causes obstruction which leads to life-threatening lung infections and also
obstruction in the pancreas. Cystic Fibrosis Explained
https://www.youtube.com/watch?v=LItSsVJPQEY
Symptoms Cystic fibrosis have several
symptoms which include - A persistent cough that
produces thick spit (sputum) and mucus
Wheezing Shortness of breath Meconium ileus which
appears at birth Salty-tasting skin which is
usually greasy feeling, bulky and has a bad smell
Frequent lung infections
Diagnostic Testing Genetic Study (analysis)
When a new born child has signs and symptoms confirmed, diagnosis of CF can usually be done by using Blood Test
Since it is an inherited diseases, family members should be checked for possible diagnosis
The Sweat test This test is a very
accurate way of diagnosing CF.Test measures Sodium
and or chloride amounts present in patient’s sweat. (test not reliable in newborns)
Other common diagnosis
When a patient presents Cystic Fibrosis, they are usually seen to have these further complications-Inability for effective airway clearanceInability to digest food and absorb nutrients which
causes imbalanced nutrition
Cystic Fibrosis – X-RAY Young man with a history
of cystic fibrosis has hyperinflation and predominantly upper lobe bronchiectasis.
Bronchiectasis is a condition in which damage to the airways causes them to widen and become flabby and scarred.
A chest x ray can show areas of abnormal lung and thickened, irregular airway walls.
Therapy and Management
Planning and intervention usually include- Provision of respiratory
therapy treatmentsChest physiotherapy to
clear secretions Administering
medications Meeting nutritional needs
Providing fat soluble vitamins like A, D, E, K to prevent deficiency
Providing psychosocial support
Treatments These help and aim at
relieving symptoms and complications of CF Aerosolized antibiotics Mucus-thinning drugs that
thin out secretions and makes it easier to cough up
Use of bronchodilators As the purpose of
relaxing smooth muscles in the airways
Bronchial airway drainagePostural drainage
Drugs
Drugs Contd..
Treatment contd.. Enzymes taken orally for
better nutrition Should contain high calorie
diet since CF patients are usually malnutritioned due to pancreatic enzymes needed to digest are not available
Lung transplant at its worstSignificant when there is severe breathing problems that has lead to life-threatening infections of the lungs
Pain relievers such as Ibuprofen- may help slow
down lung deterioration
Questions?
What do you call a nun with a lung infection and general mucus build-up?
Sister Fibrosis.
www.cysticfibrosisfoundation.com