Publications

IMPORTANCE

Patients with advanced-stage cancer are receiving increasingly aggressive medical care near death, despite growing concerns that this reflects poor-quality care.

OBJECTIVE

To assess the association of aggressive end-of-life care with bereaved family members' perceptions of the quality of end-of-life care and patients' goal attainment.

DESIGN, SETTING, AND PARTICIPANTS

Interviews with 1146 family members of Medicare patients with advanced-stage lung or colorectal cancer in the Cancer Care Outcomes Research and Surveillance study (a multiregional, prospective, observational study) who died by the end of 2011 (median, 144.5 days after death; interquartile range, 85.0-551.0 days).

EXPOSURES

Claims-based quality measures of aggressive end-of-life care (ie, intensive care unit [ICU] admission or repeated hospitalizations or emergency department visits during the last month of life; chemotherapy ≤2 weeks of death; no hospice or ≤3 days of hospice services; and deaths occurring in the hospital).

MAIN OUTCOMES AND MEASURES

Family member-reported quality rating of "excellent" for end-of-life care. Secondary outcomes included patients' goal attainment (ie, end-of-life care congruent with patients' wishes and location of death occurred in preferred place).

RESULTS

Of 1146 patients with cancer (median age, 76.0 years [interquartile range, 65.0-87.0 years]; 55.8% male), bereaved family members reported excellent end-of-life care for 51.3%. Family members reported excellent end-of-life care more often for patients who received hospice care for longer than 3 days (58.8% [352/599]) than those who did not receive hospice care or received 3 or fewer days (43.1% [236/547]) (adjusted difference, 16.5 percentage points [95% CI, 10.7 to 22.4 percentage points]). In contrast, family members of patients admitted to an ICU within 30 days of death reported excellent end-of-life care less often (45.0% [68/151]) than those who were not admitted to an ICU within 30 days of death (52.3% [520/995]) (adjusted difference, -9.4 percentage points [95% CI, -18.2 to -0.6 percentage points]). Similarly, family members of patients who died in the hospital reported excellent end-of-life care less often (42.2% [194/460]) than those who did not die in the hospital (57.4% [394/686]) (adjusted difference, -17.0 percentage points [95% CI, -22.9 to -11.1 percentage points]). Family members of patients who did not receive hospice care or received 3 or fewer days were less likely to report that patients died in their preferred location (40.0% [152/380]) than those who received hospice care for longer than 3 days (72.8% [287/394]) (adjusted difference, -34.4 percentage points [95% CI, -41.7 to -27.0 percentage points]).

CONCLUSIONS AND RELEVANCE

Among family members of older patients with fee-for service Medicare who died of lung or colorectal cancer, earlier hospice enrollment, avoidance of ICU admissions within 30 days of death, and death occurring outside the hospital were associated with perceptions of better end-of-life care. These findings are supportive of advance care planning consistent with the preferences of patients.

A brother and sister developed a previously undescribed constellation of autoimmune manifestations within their first year of life, with uncontrollable bullous pemphigoid, colitis, and proteinuria. The boy had hemophilia due to a factor VIII autoantibody and nephrotic syndrome. Both children required allogeneic hematopoietic cell transplantation (HCT), which resolved their autoimmunity. The early onset, severity, and distinctive findings suggested a single gene disorder underlying the phenotype. Whole-exome sequencing performed on five family members revealed the affected siblings to be compound heterozygous for two unique missense mutations in the 70-kD T cell receptor ζ-chain associated protein (ZAP-70). Healthy relatives were heterozygous mutation carriers. Although pre-HCT patient T cells were not available, mutation effects were determined using transfected cell lines and peripheral blood from carriers and controls. Mutation R192W in the C-SH2 domain exhibited reduced binding to phosphorylated ζ-chain, whereas mutation R360P in the N lobe of the catalytic domain disrupted an autoinhibitory mechanism, producing a weakly hyperactive ZAP-70 protein. Although human ZAP-70 deficiency can have dysregulated T cells, and autoreactive mouse thymocytes with weak Zap-70 signaling can escape tolerance, our patients' combination of hypomorphic and activating mutations suggested a new disease mechanism and produced previously undescribed human ZAP-70-associated autoimmune disease.

OBJECTIVES

To evaluate the association of frailty, a measure of diminished physiological reserve, with both major and minor surgical complications among patients undergoing urological surgery.

MATERIALS AND METHODS

Using data from the American College of Surgeons National Surgical Quality Improvement Program (NSQIP) from 2007 to 2013, we identified all urological cases that appeared > 1000 times in the dataset among patients aged ≥40 years. Frailty was measured using the NSQIP frailty index (FI), a validated measure that includes 11 impairments, such as decreased functional status and impaired sensorium. We created multivariable logistic regression models using the NSQIP FI to assess major and minor complications after surgery.

RESULTS

We identified 95 108 urological cases representing 21 urological procedures. The average frequency of complications per individual was 11.7%, with the most common complications being hospital readmission (6.2%), blood transfusion (4.6%) and urinary tract infection (3.1%). Major and minor complications increased with increasing NSQIP FI. Frailty remained strongly associated with complications after adjustment for year, age, race, smoking status and method of anaesthesia (adjusted odds ratio 1.74 [95% confidence interval 1.64, 1.85] for an NSQIP FI ≥0.18). Increasing NSQIP FI was associated with increasing frequency of complications within age groups (by decade) up to age 81 years and across most procedures.

CONCLUSION

Frailty strongly correlates with risk of postoperative complications among patients undergoing urological surgery. This finding is true within most age groups and across most urological procedures.