How would you approach a patient with cystic fibrosis (CF) who is considering advanced care planning and completion of a Physician Orders for Life-Sustaining Treatment (POLST) form?

Approaching Advanced Care Planning in Cystic Fibrosis

Question 1: Approach and Considerations

You should initiate advance care planning discussions now with Celeste, as her recent hospitalizations with respiratory failure and bronchiectasis indicate disease progression that warrants proactive end-of-life planning. 1

Key Communication Strategies

• Begin by acknowledging her concerns directly: "Your recent hospitalizations were scary, and I understand you're worried about what comes next. Let's talk about how we can honor what matters most to you—staying independent and spending time with friends—as we plan for your care." 1

• Provide honest prognostic information: With two hospitalizations in 6 months for respiratory failure and bronchiectasis, Celeste has advanced CF with significant risk of further decompensation. The European Cystic Fibrosis Society emphasizes that end-of-life discussions should be conducted "openly, honestly, with sensitivity and compassion." 2

• Use professional interpreters if language barriers exist, as the European Society of Cardiology emphasizes that family members should not interpret these complex discussions, even if present, because they may have conflicting preferences. 3

Critical Considerations

• Assess her understanding of prognosis: Does she understand that repeated respiratory failures indicate progressive disease? 1

• Explore her values systematically: You've already identified that independence and time with friends define her quality of life—this becomes the anchor for all decisions. 1

• Address transplant candidacy explicitly: Her uncertainty about transplant eligibility needs clarification, as this significantly impacts care planning. 1, 4

• Identify a surrogate decision-maker: In 70.3% of patients near end-of-life, decision-making capacity is lost, making this designation critical. 1

• Document everything thoroughly: All discussions and decisions must be recorded and communicated to all providers involved in her care to prevent fragmented decision-making during crises. 1

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Question 2: Completing POLST Sections A and B

Section A: Cardiopulmonary Resuscitation

You should mark "Attempt Resuscitation/CPR" in Section A, as Celeste explicitly stated she wants CPR attempted. 1, 3

• However, you must educate her about realistic CPR outcomes: The American College of Chest Physicians reports that patients with multiple comorbidities and recent critical illness requiring intubation have survival rates less than 5%, often less than 1%. 3

• Document the time-limited trial explicitly: Her preference for "full escalation for 1 week" needs to be clearly noted in the POLST form's additional orders section, as standard POLST forms don't capture time-limited trials well. 1

Section B: Medical Interventions

You should select "Full Treatment" in Section B, with explicit documentation that this is time-limited to 1 week or until meaningful recovery to baseline is deemed unlikely. 1

• "Full Treatment" includes: Transfer to hospital, intubation/mechanical ventilation, and intensive care as indicated. 1, 3

• Critical caveat: The POLST must specify that after 1 week, or if meaningful recovery to her current baseline is not expected, goals should be reassessed and potentially transitioned to "Selective Treatment" or "Comfort-Focused Treatment." 1

• This requires a follow-up mechanism: Designate who will reassess at the 1-week mark and what "meaningful recovery to baseline" means functionally (e.g., ability to be independent, spend time with friends). 1

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Question 3: Completing Section C (Artificial Nutrition)

You should mark "Provide feeding through new or existing surgically-placed tubes" in Section C, as Celeste explicitly stated willingness to try artificial nutrition. 1

Important Clarifications Needed

• Define "try": Does she want a time-limited trial? If so, what duration and what outcomes would constitute success or failure? 1

• Specify the context: Is this for short-term support during acute illness with expected recovery, or would she want long-term tube feeding if she cannot return to baseline? 1

• Document her quality-of-life threshold: Since she values independence and time with friends, would she want artificial nutrition if it meant prolonged hospitalization or inability to engage socially? 1

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Question 4: When Artificial Nutrition Becomes Burden vs. Benefit

Artificial nutrition transitions from benefit to burden when it prolongs dying without improving quality of life, prevents the patient from achieving their stated goals, or causes physical complications that worsen suffering. 1

Specific Situations Where Burden Exceeds Benefit

• Progressive multiorgan failure: When CF has progressed to end-stage with hepatic dysfunction, renal insufficiency, and irreversible respiratory failure, artificial nutrition cannot reverse the underlying disease trajectory. 2, 4

• Loss of meaningful interaction: For Celeste, who values spending time with friends and independence, artificial nutrition that requires hospitalization or prevents social engagement contradicts her stated goals. 1

• Complications of tube feeding: Aspiration pneumonia, tube dislodgement requiring repeated procedures, abdominal pain, diarrhea, or metabolic derangements that worsen overall comfort. 1

• When transplant is no longer an option: If Celeste becomes too ill for transplant candidacy, artificial nutrition that merely prolongs the dying process without possibility of meaningful recovery becomes burdensome. 1, 4

• Inability to return to baseline function: If artificial nutrition is required but she cannot regain the ability to be independent or engage with friends—her stated quality-of-life markers—it becomes a burden. 1

The American Heart Association Framework

The AHA emphasizes that for patients with advanced disease, "palliative care should be integrated across stages of illness, starting early and intensifying in end-stage disease," with attention to "relief of suffering" as the primary goal. 1

Practical Reassessment Points

• Set specific timeframes: Reassess artificial nutrition at 2 weeks, 1 month, and 3 months to determine if it's achieving the goal of meaningful recovery. 1

• Use functional markers: Can she interact with friends? Is she independent in any activities of daily living? If not, the burden likely exceeds benefit. 1

• Involve palliative care: The AHA recommends specialist palliative care consultation for patients with "uncontrolled symptoms, major medical decisions, or multimorbidity" to help navigate these complex decisions. 1