Myelodysplastic Syndromes: Have You Seen Your Patient Beyond His Hemoglobin?

Background: Palliative care (PC) is a patient-centered care model that aims to relief suffering by establishing a plan of care that integrates physical, psychosocial, cultural, familial and spiritual issues during the course of disease's evolution. Thus, PC applies not only to patients who face a diagnosis beyond the possibility of cure, but to all those who experience significant symptoms throughout the course of the disease. Myelodysplastic syndromes (MDS) are a heterogeneous group of myeloid neoplasms characterized by cytopenias and an elevated risk of developing acute leukemia. As MDS display a wide genetic heterogeneity, patients have a variable clinical presentation, ranging from asymptomatic patients to individuals with severe cytopenias and high-risk disease. MDS are more prevalent in the elderly population, which usually experience several morbidities; thus, MDS frequently lead to notable symptoms and deterioration of quality-of-life, making most of them eligible to PC in addition to standard hematologic care. In spite of that, previous studies demonstrated that patients with hematologic malignancies appear to have restricted access to PC services and receive more aggressive therapies at the end of life.

Aims: To evaluate eligibility criteria for PC in a cohort of MDS patients and correlate with clinical and laboratory data.

Methods: Clinical and demographic data of MDS patients were collected through interviews using a standardized questionnaire: time from diagnosis, number of morbidities, need for seeking the emergency during the last 12mo, delirium events, wounds, dysphagia, recurrent falls, adverse events to medication, quality of communication with the medical team, fears regarding the disease and its complications, religious support, age, gender, monthly household income and level of schooling. Specific PC scores were also applied: Edmonton Symptom Assessment Scale (ESAS) and Palliative Performance Scale (PPS). Clinical and laboratory data were collected: hemoglobin (Hb), platelet and neutrophil counts, Revised International Prognostic Scoring System (IPSS-R) and transfusion burden. Statistical univariate and multivariate analysis were performed. P value <.05 was considered statistically significant. This research was approved by the Institutional and National Review Board; written informed consent was obtained from all subjects.

Results:Thirty-six patients were evaluated: median age 68y (21-90), sex 16F/20M. According to ESAS, tiredness and anxiety were the most relevant symptoms in MDS patients [median (min-max)]: pain 0 (0-10), tiredness 4.5 (0-10), drowsiness 1.5 (0-10), nausea 0 (0-7), lack of appetite 0 (0-10), shortness of breath 0 (0-10), depression 0 (0-10), anxiety 3.5 (0-10), best wellbeing 2.5 (0-8). Younger patients (<60y, n=10) had a worse ESAS for best wellbeing (5 (2-8)) when compared to older individuals (≥60y, n=26): (2 (0-7)), p=.007, and tended to have worse ESAS scores for tiredness: 8.5 (0-10) vs 3.5 (0-10), p=.56. Importantly, ESAS for tiredness was not correlated to Hb levels, the number of red blood cell transfusions nor with IPSS-R (all p>.05). ESAS for drowsiness was significantly higher in patients with two (5 (0-10)) and ≥three morbidities (3 (0-8) vs those with only one morbidity (0 (0-10)): p=.01 and p=.03, respectively. ESAS for best wellbeing was better in individuals with higher household income 0 (0-0) vs patients with lower financial resources 3 (0-7), p=.04). PPS median was 90% (60-100%) and negatively correlated with transfusion burden (r=0.407, p=.01) and with the need for seeking the emergency in the past 12mo (r=-0.332, p=.04). Finally, despite facing a potential life-threatening disease, 94.4% of the patients reported that their doctors had never talked to them about aspects related to end-of-life care. Conversely, 75% of them reported fears and doubts regarding this phase.

Conclusions: In our casuistic of MDS patients, tiredness was the most important symptom observed. Surprisingly, it was not correlated with Hb levels and transfusion burden, suggesting that Hb levels alone should not be used to justify symptoms. The number of morbidities and lower household income also impacted ESAS scores. Finally, a great part of the patients revealed miscommunication with their hematologists regarding end-of-life planning. Our data indicate that MDS patients might benefit from a PC multidisciplinary team approach.