Randomized controlled trial of individualized treatment summary and survivorship care plans for hematopoietic cell transplantation survivors.

Survivorship Care Plans (SCPs) may facilitate long-term care for cancer survivors, but their effectiveness has not been established in hematopoietic cell transplantation recipients. We evaluated the impact of individualized SCPs on patient-reported outcomes among transplant survivors. Adult (≥18 years at transplant) survivors who were 1-5 years post transplantation, proficient in English, and without relapse or secondary cancers were eligible for this multicenter randomized trial. SCPs were developed based on risk-factors and treatment exposures using patient data routinely submitted by transplant centers to the Center for International Blood and Marrow Transplant Research and published guidelines for long-term follow up of transplant survivors. Phone surveys assessing patient-reported outcomes were conducted at baseline and at 6 months. The primary end point was confidence in survivorship information, and secondary end points included cancer and treatment distress, knowledge of transplant exposures, health care utilization, and health-related quality of life. Of 495 patients enrolled, 458 completed a baseline survey and were randomized (care plan=231, standard care=227); 200 (87%) and 199 (88%) completed the 6-month assessments, respectively. Patients' characteristics were similar in the two arms. Participants on the care plan arm reported significantly lower distress scores at 6 months and an increase in the Mental Component Summary quality of life score assessed by the Short Form 12 (SF-12) instrument. No effect was observed on the end point of confidence in survivorship information or other secondary outcomes. Provision of individualized SCPs generated using registry data was associated with reduced distress and improved mental domain of quality of life among 1-5 year hematopoietic cell transplantation survivors. Trial registered at clinicaltrials.gov 02200133.

Cochlear Implantation in a Patient with Kabuki Syndrome.

Criteria for cochlear implants are expanding and now include children with disabilities in addition to hearing loss, such as those with Kabuki syndrome (KS). This case report describes language outcomes and changes in the quality of life of a female child with KS after cochlear implantation. The subject had a profound progressive sensorineural hearing loss, cognitive impairments, and other disabilities and communicated using vocalized sounds and the Dutch Sign Language. After cochlear implantation at an age of nine years and three months, the patient displayed no progress in speech production and minimal progress in receptive language development, but she had an increased awareness of the world and an increase in the quality of life.

Relationship between corticosteroid dose and declines in physical function among allogeneic hematopoietic stem cell transplantation patients.

PURPOSE: The aim of this study was to investigate the relationship between corticosteroid dose and degree of physical function decrease in allogeneic hematopoietic stem cell transplantation (allo-HSCT) patients during the early stage of recovery. We further investigated the confounding factors affecting loss of physical function. METHODS: The study included 113 patients who underwent allo-HSCT between July 2007 and April 2012 at Hyogo College of Medicine Hospital in Japan. Physical function was assessed using tests for hand-grip strength, knee-extensor strength, and the 6-min walk test (6MWT). Fatigue was measured using the Piper Fatigue Scale. Total corticosteroid dose, frequency of physical therapy, body weight, and nutritional status were also collected from medical records. RESULTS: Total corticosteroid dose was correlated with decrease of hand-grip and knee-extensors strength (P < 0.01) but was not correlated with 6MWT performance. Results of multivariate analysis confirmed that low physical function was associated not only with high corticosteroid dose but also with low frequency of physical therapy, increase in fatigue, and body weight loss (P < 0.05). Also, hemoglobin levels were associated with 6MWT (P < 0.05). CONCLUSIONS: This study showed the relationship between corticosteroid dose and declines in physical function and also showed other clinical factors affecting loss of physical function among allo-HSCT patients. Our results indicate that the effectiveness of rehabilitation may be influenced by corticosteroid treatment.

Malignant and benign thyroid nodules after total body irradiation preceding hematopoietic cell transplantation during childhood.

BACKGROUND: The risk of radiation-induced benign and malignant thyroid nodules is well known. OBJECTIVE: The aim of this study was to determine the occurrence of thyroid nodules and carcinomas after fractionated total body irradiation (TBI) preceding hematopoietic stem cell transplantation (HSCT) for malignant hematological disease during childhood. METHODS: We conducted a retrospective university hospital-based observational study. The participants were 76 patients receiving fractionated TBI between 1989 and 2009 as part of the conditioning regimen for HSCT to treat malignant hematological disease, with a median age of 8.2 (5.7-11.4) years, for whom the last ultrasound examination was performed at a median age of 14.2 (11.2-17) years. The main outcome measure was cumulative incidence of thyroid nodules detected by ultrasound scans followed by biopsy if necessary. RESULTS: Thyroid nodules were examined in 21 (28%) patients, six (29%) of whom were diagnosed with thyroid carcinoma at the age of 2.2-18.6 years after TBI. The cumulative incidence of nodule occurrence increased with increasing time from diagnosis. The 10-year cumulative incidence of benign and malignant thyroid nodules was 16% (95% confidence interval (CI) 4-27%) and 8% (95% CI 0-16%) respectively. Seventeen (22%) patients had hypothyroidism (compensated n=12, in five patients it was transient). No significant independent risk factors were identified in the multivariable competing risk model as a function of nodule occurrence. CONCLUSION: Short-term and life-long monitoring, with screening for nodules of the thyroid gland using ultrasound scans, is recommended for survivors subjected to TBI for HSCT during childhood.

[Medico-social characteristics of disabled persons with diseases of blood system and blood-forming organs].

396 disabled persons from three regions of Ukraine (Vinitsa, Zhitomirsk and Khmelnitsk regions), with compromised blood system and blood-forming organs have been observed. Differences on sex, age, pathology, severity of disability in these regions, course of disabilities were determined. The possible rehabilitation courses depending on pathology, nosological form and clinical prognosis were considered in the article.

Health-related quality of life assessment in hematologic disease.

Evaluating health-related quality of life (HRQL) for children who have benign hematologic disorders can: (1) aid in the evaluation of different therapies and emerging new technologies; (2) serve as an invaluable source of anticipatory guidance, for current and future patients; and (3) serve an important evaluative function of clinical services or programs to identify potential areas in which additional services, supports, or interventions are needed. The past 20 years have been marked by the dramatic growth in the field of HRQL research, although much of the progress in conceptualization, instrument development, and refinement has occurred in adults. Pediatric health services researchers and their clinical colleagues have had to grapple with the methodologic challenges in evaluating HRQL in children. This article includes a discussion of instrumentation, respondent selection, and study design consideration in the evaluation of HRQL in children who have benign hematologic disorders.

Recovery after stem-cell transplantation for hematologic diseases.

PURPOSE: Although the number of autologous and allogeneic stem-cell transplantations (SCT) is increasing, relatively little information about recovery after transplantation is available. Quantitative information appropriate for patient counseling is difficult to discern from the literature. We sought to suggest reasonable expectations for recovery and symptoms after SCT for hematologic malignancies and other disorders using the following measures: (1) objective measures of health status, such as frequency of clinic visits, need for rehospitalization, medication usage, work status, and overall and event-free survival; (2) qualitative assessment of quality of life, such as returning to a normal life, resumption of normal activities, satisfaction with appearance, and whether recovery has occurred; and (3) quantification of specific bothersome symptoms. PATIENTS AND METHODS: Autologous and allogeneic SCT recipients at a tertiary-care transplant center participated in the prospective, longitudinal questionnaire study. RESULTS: Three hundred twenty patients were studied. Questionnaire response rates at 6, 12, and 24 months range from 85% to 88% among survivors. Although autologous patients had better event-free and overall survival, fewer symptoms, and more complete recovery at 6 months, these advantages had largely equalized by 12 months. Specific bothersome symptoms were reported by less than 24% of patients after transplantation, except for fatigue and financial and sexual difficulties, which were more prevalent. CONCLUSION: These findings may help counsel patients considering transplantation and educate them about reasonable expectations for recovery. Overall, the low level of bothersome symptoms and continued recovery through the first year after transplantation are encouraging.

[Necessary diagnostic and therapeutic measures in the rehabilitation of "cured" patients with Hodgkin's disease]. / Notwendige diagnostische und therapeutische Massnahmen bei der Rehabilitation "geheilter" Patienten mit Morbus Hodgkin.

On account of the fundamental changes in treatment modalities, an ameliorated prognostic outlook, as well as a not inconsiderable variety of reversible and irreversible treatment-generated disorders, current concepts for rehabilitation of patients with M. Hodgkin require thorough reconsideration. It is not the early detection of recidives, but identification and, whenever possible, therapy of the treatment aftermath that should be the primary function of tumour aftercare. Splenectomy, radiotherapy, and chemotherapy may entail long-term immune-system or cardiac disorders, scelettal impairments, functional disorders of the lung, liver, kidney, or the endocrine system, vascular diseases, dental problems, leukeamia, or second carcinoma. As primary therapy is increasingly being geared toward taking into account this possible aftermath of treatment, aftercare and rehabilitation of patients with M. Hodgkin, too, should be adjusted to an increased awareness of unwanted long-term side-effects.