Question

Pediatrics: Cystic Fibrosis ( please fill all lines)

1) Alterations in health (diagnosis)

2) Pathophysiology Related to Client Problem

3) Health Promotion and Disease Prevention

4) Risk Factors

5) Expected Findings

6) Laboratory Tests

7) Diagnostic Procedures

8) Safety Considerations

9) Nursing Care

10) Therapeutic Procedures

11) Medications

12) Client Education

13) Interprofessional Care

14) Complications

ACTIVE LEARNING TEMPLATE System Disorder STUDENT NAME DISORDER/DISEASE PROCESS REVIEW MODULE CHAPTER Alterations in Health (Diagnosis) Pathophysiology Related to Client Problem Health Promotion and Disease Prevention ASSESSMENT SAFETY CONSIDERATIONS Risk Factors Expected Findings Laboratory Tests Diagnostic Procedures PATIENT-CENTERED CARE Complications Nursing Care Medications Client Education Therapeutic Procedures Interprofessional Care

Answer 1

Cystic fibrosis

Cystic fibrosis is the most common fatal autosomal recessive disease, the multisystem genetic disease is usually diagnosed in infancy or early childhood but may be diagnosed later in life, respiratory complications are the major manifestation of cystic fibrosis.

1. Answer

Diagnosis

Alteration in health.

1. Ineffective airway clearance related to inability to remove mucus from the respiratory tract.

2. Risk for infection related to accumulation of mucopurulent discharge .

3.ineffective breathing pattern related to congestion into the respiratory system.

2. Answer

Pathophysiology of cystic fibrosis.

It is caused by mutation or dysfunction of the protein cystic fibrosis transmembrane conductance regulator, which transport chloride ion across epithelial cell membranes,

Gene mutation affect transport of these ion leads to cystic fibrosis,

Characterised by thick mucous ,viscous secretion in lungs pancreas,liver, intestine , as well as decrease in salt content of sweat gland ,

Hallmark pathology of cystic fibrosis is bronchial mucus plugging,inflammation and eventual bronchiectasis begins in the upper lobes ,

Progressive loss of lung function.

3. Answer

Health promotion and disease prevention is done by

1. Hand hygiene

Wash hand before and after food, after coughing, before and after touching client, after touching patient's environment.

2. Droplet precautions

Use mask while dealing with client , provide mask to the client when he deals with others.

3. Standard precaution.

Teach client, coworker , family members regarding hand hygiene and personal hygiene while coming in contact with client.

4.Isolation.

Patient should be kept in a single room to avoid cross contamination from patient to others

5. Patient and family members should be taught to clean utensils, cloths, and all the article regularly to avoid contamination.

4.Answer.

Risk factors of cystic fibrosis.

1. People who are heterozygous for cystic fibrosis.

2. Heterozygous genes such as one normal and defective gene.

3. Do not have disease but can pass to their children.

4. If both parents are carrier then there are chances with cystic fibrosis one in four children.

5 Answer.

Expected findings for cystic fibrosis.

1.Chron sinopulmonary disease as manifested by chronic cough and sputum production,

2. Persistent infection consistent with typical cystic fibrosis.

3.chronic sinusitis with nasal polyps.

4. Gastrointestinal abnormalities failure to thrive or chronic malnutrition.

5.male urogenital problem and obstructive azoospermia.

6. Lab test

Lab test is done to determine whether the individual is genetic carrier or this can be managed it's conditions in the future.

Some states uses IRT(immunoreactive trypsinogen) blood test alone while other uses IRT( immunoreactive trypsinogen) along with gene for screening.

7.Answer

Dignostic procedures.

1. Sweat test - it is performed to check the amount of chloride ion in sweat.

2. Genetic test - it is done to detect chromosomal abnormality.

8. Answer.

safety considerations in cystic fibrosis.

1.use hand hygiene.

2. Take proper nutrition.

3. Administration of medicine on time.

4. Response to the therapy.

5.interaction with all the members.

9. Answer.

Nursing care.

1. Help patient in managingpulmonary symptoms and prevent complications of cystic fibrosis.

2.for removal of pulmonary secretions chest physiotherapy ,vibration, percussion and postural drainage is used.

3.breathing exercises which are implemented and are taught to the patient and family when the patient is young.

4.patient is reminded to reduce risk factors associated with respiratory infections which is exposure to crowds or to the people with known infection.

5.adequate fluid and dietary intake to promote removal of secretions and to ensure an adequate nutritional status.

6.patient is to be taught regarding modification in daily activities as cystic fibrosis is a lifelong disorder.

10. Answer

Therapeutic procedures.

1. Airway clearance.

2. Postural drainage.

3. Nebulization.

4. Chest physiotherapy.

5. Oxygen therapy .

11. Answer

Medications.

1.Antibioti based on sputum culture, such as cornerstone to clear airway.

2. Nebulization is given with dornase alpha to destroy large amount of DNA accumulates with cystic fibrosis mucus.

3. Nebulization antibiotics include tobramycin ,colistin, gentamicin ceftaxidime.

For infection antibiotics which are given macrolide Azithromycin,

12. Answer

Client education.

Educate client regarding the method of transmission of this disease, as it is a genetic mutation disorders that affect respiratory tract ,gastoin gastrointestinal tract, etc,

Frequent mucopurulent discharge generated and it should be dealt carefully.

Separate items of daily living should be kept as it is a communicable disease.

Maintain asepsis whenever needed.

Use mask while talking to others.

Maintain fluid and electrolyte.

Regular physiotherapy.

Nutrition intake should be proper.

Exercise should be performed to get rid of breathing difficulty.

13. Answer

Interpersonal care.

It is a complex disorder all health care provider, nursing personnel , family of client and patient itself should be involved in care of client during cystic fibrosis, all kind of support should be given to the client according to need.

14.Answer

complications

Bowel disease.

Chronic cough.

Blockage in intestine.

Biliary tract disease.

Blood in sputum.

Nasal polyps

Hyperglycemia.

Disease of liver.

Malnourishment.

And various complications may occur.