Question

which finding requires immediately intervention when planning care for an adolescent with cystic fibrosis?     a.poor weight gain.                                                                 b.chest pain with dypsnea.                                                     c.delayed puberty.                                                                    d.large,foul smelling, bulky stools

Answer 1

b. The finding that require immediate intervention when planning the care for an adolescent with cystic fibrosis is Chest pain with dyspnea.

Cystic fibrosis is an inherited disease characterized by an abnormality in the body's salt, water and mucous-making cells. It is a chronic, progressive and usually fatal condition. Adolescent with cystic fibrosis have an abnormality in the function of a cell protein in the body called Cystic Fibrosis Transmembrane Regulator. This cell protein controls the water flow and certain salt in and out of the body's cell. Due to the movement of salt and water in and out of cells is altered, the mucous becomes thickened. Because of this thickened mucous can affect many organs in the body system.

Thick mucous that accumulates in the lungs may leads to frequent respiratory infections, breathing difficulties and lung diseases. These leads to;

• Chest pain
• Dyspnea
• Frequent episodes of wheezing
• Frequent episodes of pneumonia
• Persistent cough
• Pneumothorax (Presence of air or gas in the pleural cavity causing the lung to collapse)
• Hemoptysis (coughing up blood)

TREATMENT

There is no cure for cystic fibrosis, but medications and therapies can easy the symptoms, reduce the complications and help to improve the quality of life.

The management of problems that cause respiratory system like

• Chest physiotherapy helps to loosen and clear lung secretions
• Breathing exercise helps to loosen the mucous, stimulate the coughing and improve all physical condition.
• Medications: helps to open the airway, loosen the mucous,and prevent infections. The doctor may prescribe the medications like;

1. Antibiotic - That helps to treat lung infections and help the lungs to work better.
2. Anti-inflammatory medicines - These include Ibuprofen and Corticosteroids
3. Bronchodilators - These are in the form form of inhaler helps to relax and open the airway
4. Mucus thinners - Helps to reduce the thickness of the mucus in the airway and make easy the respiration.
5. CFTR modulators - It helps the lungs to work better and help in weight gain.

• Psychological support is require to help the adolescent and family to deal with issues such as independence, health, quality of life etc.
• The newer therapies include lung transplantation for patients with end-stage lung diseases.
• Gene therapy is now available for cystic fibrosis.