Question

Case scenario topic 1

A 6 year old boy with cystic fibrosis who has recurrent chest infections, and newly diagnosed with type 1 diabetes.

Adam (6 year old) boy was dignosed with cystic fibrosis soon after birth. He has recently recovered from chest infection and refusing to attend prep, due to increased fatigue. He has just recovered from his 3rd chest infection in 9 months and was hospitalized for 2 weeks. he often refuses treatments such as his daily nebuliser and physio routine.Whilst in hospital he was diagnosed with Cystic fibrosis related diabetes and the family is now learning to manage his insulin regime.

Part A Written case analysis essay

Length: 1500 words

In this essay you will analyse the case study chosen giving a brief description of the chronic condition and the presenting health issues for the person.

• Identify both the chronic disease and the presenting condition. • Succinctly describe the pathophysiology, symptoms, anatomy and physiology associated with the chronic condition and the presenting issue. • Provide a brief outline of the relevant diagnostic and ongoing tests (e.g. blood tests, vital signs, x-rays, physiotherapy), associated with the patient’s condition. • Outline two (2) potential problems (complications) associated with the chronic disease. • Briefly outline the developmental, cultural and health literacy considerations for the person in your case study. Developmental theorists have been identified in the modules on the StudyDesk, you are encouraged to use your recommended texts and library resources. • Describe three (3) priorities of nursing management for the patient’s chronic and presenting condition. These may include nursing management, and/ or pharmacological /non-pharmacological management and/ or self-management.

Part B: A patient information resource offers you, as a nurse, a method of providing information to patients and their families on various chronic conditions. The information resource that you produce for this assessment can be used as part of a professional portfolio or used within your nursing practice in a health facility to educate clients. • Describe the condition, symptoms, anatomy/ physiology behind the condition (i.e. causes) • Outline the potential tests, treatments and medications that the patient may experience in the course of managing the condition • Briefly describe three (3) management strategies e.g. options for relief of symptoms, lifestyle changes, prevention of relapse/escalation/complications. • Include a link to an additional resource on the condition that the ‘lay person’ can read or watch (i.e.: a website, YouTube video). Note these must be from reputable sources but with a patient, not medical practitioner, focus

Answer 1

#. Chronic disease - Cystic fibrosis

#. Parts of body affected by cystic fibrosis :-

Sweat glands

Respiratory system

Digestive system

Reproductive system

#. Etiology, Onset, & Pathogenesis

autosomal recessive disorder--having only one copy of gene makes them a carrier

mutation of CFTR gene on chromosome 7

when 2 carriers have a child:

25% chance child will have CF

50% chance child will be carrier

25% not a carrier & don't have CF

#. Pathophysiology

mutation in CFTR gene---dysfunction/absence of protein

result: abnormal cAMP-regulated chloride transport across epithelial cells on mucosal surfaces

affects: muscle production, secretory granules, intracellular organelles

areas most affected: respiratory tract, GI tract, pancreas, sweat glands

#. Signs & Symptoms

respiratory-- cough, lung infection, pneumonia, bronchitis, SOB/wheezing

genitourinary-- undescended testicles, amenorrhea, delay in puberty

gastrointestinal: meconium ileus, salty skin at birth, poor growth, inability to gain weigh, frequency of stools

#. Clinical Features & Impairments

fatigue

failure to meet growth milestones

clubbed finger

#. Complications

gallstones

male infertility

liver failure

respiratory failure

takes longer to recover from illness

#. Comorbidities

diabetes

osteoporosis

bronchiectasis

severe gastric reflux

periportal fibrosis

#. Diagnosis

genetic testing

stool analysis-- check for fat content & trypsin (pancreatic enzyme)

abnormal electrolyte balance in sweat