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