A 17-year-old boy is hospitalized with an acute exacerbation of cystic fibrosis. His respiratory function had remained stable for many years but has begun to deteriorate, with 3 hospital admissions within the past 6 months. The patient's FEV1/FVC ratio has decreased compared with a baseline reading taken 9 months ago and is 30% of predicted value. He is placed on supplemental oxygen, antimicrobial therapy, and inhaled bronchodilators. The hospitalist physician notices that there are no advance directives for this patient's care. Which of the following is the best approach to take?

Question

A 17-year-old boy is hospitalized with an acute exacerbation of cystic fibrosis.  His respiratory function had remained stable for many years but has begun to deteriorate, with 3 hospital admissions within the past 6 months.  The patient's FEV1/FVC ratio has decreased compared with a baseline reading taken 9 months ago and is 30% of predicted value.  He is placed on supplemental oxygen, antimicrobial therapy, and inhaled bronchodilators.  The hospitalist physician notices that there are no advance directives for this patient's care.  Which of the following is the best approach to take?

Quizplus · Accepted Answer

This patient with cystic fibrosis (CF) has experienced an increased frequency of pulmonary exacerbations and a decrease in FEV1. These findings correlate with decreased survival and therefore warrant timely discussion about advance care planning. It is important that adolescents with a chronic and life-limiting illness, such as CF, be provided the opportunity to participate in these discussions. Studies have found that a family-centered approach involving the adolescent is effective and helpful. Discussions should include information regarding prognosis and care options, including detailed information about lung transplantation and the patient's and family's preferences for end-of-life care. Gaining an understanding of the patient's priorities and values ensures that the patient's wishes are respected should a life-threatening complication occur during hospitalization. (Choices A and E) A primary care clinician who knows the patient well is in the ideal position to initiate a discussion about advance care planning. However, given this patient's deteriorating clinical status, it is inappropriate to defer the conversation until after discharge (in case he develops a significant respiratory decompensation during this hospitalization) or until the patient turns age 18 (the age of legal consent in most jurisdictions). (Choice B) It is inappropriate to defer the conversation based on the assumption that the patient would lose motivation to participate in treatment. Studies have found that adolescent patients and their families are able to tolerate a wide range of emotions during the advance care planning conversation and find the experience to be beneficial. (Choice D) Adolescents can understand medical implications and are likely to find it demeaning to be left out of conversations about their end-of-life wishes. Excluding this patient from the discussion and relying solely on the parents' directives would be inappropriate. Educational objective: Discussions regarding advance care planning for adolescents with chronic, life-limiting illnesses (eg, cystic fibrosis) should take a family-centered approach and directly involve the adolescent patient. Discussion should include information regarding prognosis, care options, and the patient's and family's preferences for end-of-life care. References: A longitudinal, randomized, controlled trial of advance care planning for teens with cancer: anxiety, depression, quality of life, advance directives, spirituality. (http://www.ncbi.nlm.nih.gov/pubmed/24411819) Acceptability of family-centered advanced care planning for adolescents with HIV. (http://www.ncbi.nlm.nih.gov/pubmed/27940700)

A 17-Year-Old Boy Is Hospitalized with an Acute Exacerbation of Cystic