Question

Sam is a 20 years old and has been admitted to the hospital for a large bowel obstruction. Sam was diagnosed with Cystic Fibrosis at the age of 2. Cystic Fibrosis is a genetic disorder that affects not only the respiratory system but also the gastrointestinal system. Sam’s parents are very worried about him at this time. During surgery he had 4 liters blood loss and was admitted to the ICU where you work. He was intubated at time of admission.

Assessment: Vital Signs: B/P 90/45, HR 116, T 98.9, RR 16 Weight 150 pounds, Height: 6 foot Neuro: Lethargic but opens eyes to voice Respiratory: Lungs diminished in bases bilaterally Vent: SIMV TV 650, FiO2 70%, RR 16, PEEP 5 ABG: pH 7.30, PaCO2 46, Bicarb 22, PaO2 70 PaO2/FiO2 ratio 150 Cardiac: Heart tones normal GI: Bowel sounds absent GU: Foley in place with 40ml/hr, Urine yellow and clear Lab Values prior to surgery: NA 137 meq/L, K 3.9 meq/L, CL 100 meq/L, Mg 2 meq/L, WBC 12,000 mm3, H/H 15 g/dl and 42%, Lactic Acid 20 mg/dl, CXR: Ground glass appearance in lower lobes

What is the genetic component of Cystic Fibrosis and how is it diagnosed?

How can genetic testing be used to make predictions?

Explain the pathophysiology of Cystic Fibrosis. How does it affect the respiratory system?

How does it affect the GI system?

Sam is in the early stages of ARDS. What manifestations do you see that would indicate this finding?

What is the Pathophysiology of the three stages of ARDS? What are your top three nursing priorities with rationale at this time?

How might the lab values change after 4 units of PRBC are given? Use ADPIE to write a plan for Sam for the next 12 hours.

Answer 1

1. Cystic fibrosis is an autosomal recessive inherited disorder. This occurs when both the copies of the gene for the cystic fibrosis transmembrane conductance regulator CFTR protein.

Those with a single copy will be carriers and mostly healthy.

This is diagnosed by Sweat test & Generic testing

Sweat test is used to measure chloride concentration in sweat which will be elevated.

Genetic testing: Also called DNA testing to identify any gene mutations in that particular gene

2. Prediction Including screening a new born for raised concentration of Immnunoreactive trypsinogen.

Preimplantation generic diagnosis will help to predict.

so we can give proactive treatenvoy airway infection with good nutrition and healthy lifestyle.

3. The abnormal CFTR gene produces abnormal CFTR protein wh8ch causes abnormal transport of water & salt across the cells lining the respiratory tract leading to the less water around the respiratory tract lining which leads to thicker sputum production and clog airways leading to bronchiectasis. The vicious cycle of airway obstruction with inflammation along with infection leads to reduced lung function causing respiratory distress and death.

Clogged mucus secretions in the digestive tract lead to malnutrition and vitamin deficiencies arc.

4. Sam is in early stage of ARDS because his blood gas indicated he is in respiratory acidosis. His ratio of Pa O2 / Fi O2 is indicating ARDS being 150. RR is 16 which indicates too