Implementing touch-screen technology to enhance recognition of distress.

OBJECTIVE: The University of California, San Diego, Moores Cancer Center implemented a systematic approach for patients to communicate with their health-care team in real-time regarding psychosocial problem-related distress using touch-screen technology. The purpose of this report is to describe our experience in implementing touch-screen problem-related distress screening as the standard of care for all outpatients in a health-care setting. Although early identification of distress has recently gained wide attention, the practical issues of implementing psychosocial screening with and without the use of technology have not been fully addressed or investigated. METHODS: 'The How Can We Help You and Your Family?' screening instrument was used to identify and address patient problem-related distress for clinical services, program development, research and education. Using a HIPPA-compliant approach, the touch-screen technology also helped to identify patients interested in clinical trials and additional support services. RESULTS: We found that the biggest barrier to implementing this technology was the attitude of the front desk staff (i.e. schedulers, clerks, administrative staff) who felt that the touch-screen would be burdensome. Our experience suggested that it was essential to actively involve these personnel from the beginning of the planning process. As specifically acknowledged in the recent 2007 Institute of Medicine report (Cancer Care for the Whole Patient: Meeting Psychosocial Health Needs. The National Academies Press: Washington, DC, 2007), use of this computerized version of the screening instrument was able to bridge the gap between the detection of problem-related distress and referrals for assessment or treatment. CONCLUSION: We found that it is feasible to implement a computerized problem-related distress screening program in a comprehensive cancer center.

The presence of germ line mosaicism in cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is typically an autosomal dominant condition. The possibility of alternative causes, such as an autosomal recessive form or germ line mosaicism, have been suggested in some families with CCD, but not proven. We present a family consisting of a mother having three sons affected with CCD. One of the affected boys is a half brother to the other two affected children. The diagnosis of CCD was confirmed by DNA analysis of the RUNX2 gene in all three of the boys in blood; however, initial DNA testing in the mother's blood did not detect the presence of a RUNX2 mutation in the mother. Further testing through heteroduplex analysis applying high-resolution melting analysis followed by subcloning detected low-level mosaicism in DNA isolated from maternal blood and buccal swab, confirming low-level mosaicism in somatic cells. We present the first case of confirmed germ line mosaicism in CCD.

Interstitial deletion of chromosome 2q32-34 associated with multiple congenital anomalies and a urea cycle defect (CPS I deficiency).

A de novo deletion of the long arm of chromosome 2 at 2q31-33 was observed in the fetal amniocyte G-banded karyotype performed because of possible multiple malformations identified by ultrasound at 23 weeks gestation. Two days after the uneventful term delivery of a 2.45 kg male, the neonate experienced cardiopulmonary decompensation and biochemical changes compatible with carbamoyl phosphate synthetase I (CPS I) deficiency (elevated ammonia with a peak of 948 micromol/L, deficiency of citrulline, and no increase in orotic acid). The child died on day 3 of life. Physical anomalies confirmed at autopsy included double superior vena cava, ectopic adrenal tissue, and metatarsus adductus. The autopsy also revealed histologic evidence consistent with CPS deficiency, most notably microvesicular steatosis of the liver and Alzheimer's Type II changes with hypertrophic astrocytes in the basal ganglia. A postnatal lymphocyte karyotype confirmed the chromosome 2q31-33 deletion. Enzyme analysis on postmortem liver tissue confirmed the diagnosis of CPS deficiency. CPS I is reported to be mapped to 2q35 by NCBI (http://www.ncbi.nlm.nih.gov/mapview/) and 2q34 by ENSEMBL (http://www.ensembl.org/). The UCSC Human Genome Browser July 2003 assembly also places the gene at 2q34 (http://genome.UCSC.edu/). Fluorescence in situ hybridization (FISH) analysis with a BAC clone (RP11-349G4) of CPS I demonstrated that one copy of the gene was deleted in this infant. Using additional probes corresponding to the bands in the region of deletion, we identified the deleted region as 2q32-2q34. Our observations support the CPS I map position (ENSEMBL, UCSC) at 2q34. Additionally, potential conditions associated with deletions narrowly defined by standard cytogenetic techniques merit consideration in prenatal counseling. As demonstrated here, deletions may not only result in malformations and mental retardation but also increase the likelihood of revealing mutated genes located in the undeleted region of the homologous chromosome.

Using pictographs to enhance recall of spoken medical instructions II.

The first study in this series [Houts PS, Bachrach R, Witmer JT, Tringali CA, Bucher JA, Localio RA. Patient Educ. Couns. 1998;35:83-8] found that recall of spoken medical instructions averaged 14% but that, when pictographs (drawings representing the instructions) accompanied the spoken instructions and were present during recall, 85% of medical instructions were remembered correctly. Those findings suggested that spoken instructions plus pictographs may be a way to give people with low literacy skills access to medical information that is normally available only in written form. However, there were three important limitations to that study: (1) the subjects were literate and perhaps literate people remember pictograph meanings better than people with low literacy skills; (2) only short term recall was tested and, for medical information to be useful clinically, it must be remembered for significant periods of time and (3) a maximum of 50 instructions were shown in pictographs, whereas managing complex illnesses may require remembering several hundred instructions. This study addresses those limitations by investigating 4-week recall of 236 medical instructions accompanied by pictographs by people with low literacy skills. Subjects were 21 adult clients of an inner city job training program who had less than fifth grade reading skills. Results showed 85% mean correct recall of pictograph meanings immediately after training (range from 63 to 99%) and 71% after 4 weeks (range from 33 to 94%). These results indicate that people with low literacy skills can, with the help of pictographs, recall large amounts of medical information for significant periods of time. The impact of pictographs on symptom management and patient quality of life remains to be studied.

Problem-solving cancer care education for patients and caregivers.

PURPOSE: A program evaluation was conducted to explore the potential effects of a 90-minute problem-solving education session for persons with advanced cancer and their families. DESCRIPTION OF PROGRAM: Patients with advanced cancer and their families, who were visiting a tertiary-care outpatient setting, were invited to attend a 90-minute individualized educational session that taught basic problem-solving principles using a cognitive-behavioral framework. Pre-education and posteducation data were collected about the confidence of participants in providing care, their feelings about being informed about resources, and their perceptions of their problem-solving ability. RESULTS: At baseline, most participants reported low confidence about their ability to provide cancer care and felt uninformed about community resources, but they viewed themselves as moderate-to-good problem solvers. Forty-two educational sessions were delivered to 49 caregivers and 40 patients. Two months later, participants reported feeling more informed about community resources and achieved higher posteducation scores for problem-solving ability. More caregivers than patients reported that reading The Home Care Guide for Cancer made a great deal of difference in their approach to home care. CLINICAL IMPLICATIONS: Most educational sessions for families affected by cancer focus on delivering information, not on building skills. These findings suggest that a one-on-one educational session that teaches problem-solving skills can be successfully delivered in a busy clinic setting. Family caregivers are especially likely to benefit from this program.

NCCN Practice Guidelines for Cancer Pain.

The overall approach to pain management encompassed in these guidelines is comprehensive. It is based on objective pain assessments, utilizes both pharmacologic and nonpharmacologic interventions, and requires continual reevaluation of the patient. The NCCN Cancer Pain Practice Guidelines Panel believes that cancer pain can be well controlled in the vast majority of patients if the algorithms presented are systematically applied, carefully monitored, and tailored to the needs of the individual patient.

Creutzfeldt-Jakob disease diagnosable by EEG and cerebrospinal fluid analysis without brain biopsy: a case report.

This case illustrates a classic example of CJD in its clinical presentation and course and the EEG. It also shows dramatically the utility of a newly developed protein assay in the diagnosis of this disease. This assay has the potential of eliminating the need for brain biopsy in most cases, thus providing a safer diagnostic method for both staff and patients. In addition, the case points out that anatomical structural studies such as CT and MRI do not replace the utility of EEG in the comprehensive evaluation of rapid onset dementia, but rather complement the usefulness of EEG.

Psychological approaches to the management of pain in patients with advanced cancer.

Although extremely valuable, cognitive-behavioral interventions are always integrated with and should never be a substitute for appropriate and comprehensive medical management. Cancer-related pain and associated distress significantly challenge the physical and mental well-being of patients with advanced cancer and their families. As patient demands require greater vigilance in the management of noxious symptoms, families have a parallel need for emotional support and the acquisition of coping skills. Under ideal circumstances, coping skills are taught to the patient and family when psychic and physical energy are relatively high. Consequently, these skills should be incorporated into the early phases of care, prior to advanced diseases or the final stages of the illness. Cognition, behaviors, and feelings are negatively affected by chronic illness, resulting in an overall diminution of psychic energy, coping, and adaptation. Patients frequently manifest symptoms and maladaptive behaviors that require specialized interventions to restore a sense of focus and control. Cognitive-behavioral interventions are uniquely suited to address the most common psychological and emotional problems of patients with advanced cancer and their families. As an adjunct to medical care, cognitive-behavioral interventions promote optimal functioning through the encouragement of active participation in the control of symptoms and acquisition of specific skills that increase self-efficacy and hope.

Cognitive-behavioral interventions in management of cancer pain: principles and applications.

Cognitive-behavioral interventions are effective for symptoms control and reduction of suffering in cancer pain patients. This article outlines the theoretical and technical principles of these nonmedical interventions and illustrates their integrated application with two case reports.