Huntington's disease and the law

Huntington's disease (HD) is a relentlessly progressive and fatal neurological condition that is inherited. It has serious and disabling physical and mental components. As such, it impacts upon those who have HD, those with the potential to inherit it, and those who care for those with HD in a wide variety of ways. These can have many legal ramifications including in relation to evolving impairments of capacity which can have an outcome in terms of involuntary status as mental health patients, testamentary capacity and the need for guardianship and administration. It can have effects upon fitness for parenting, obligations for spousal maintenance, and the quantum of compensation from a tortious incident to which a person is entitled. It has repercussions for criminal liability and culpability. This article reviews case law from a number of countries in relation to such matters, noting the broader radiation to others of the effects of HD, and reflecting on the need for legal and medical professionals to be aware of the legal consequences of HD for them to be able to discharge their responsibilities holistically, sensitively and informedly.     

Breath alcohol analysis in one subject with gastroesophageal reflux disease.

A large number of people suffer from the heartburn symptoms associated with gastroesophageal reflux disease (GERD). Relatively little has been published on its potential for biasing a breath alcohol measurement. The present case describes an individual (white male, aged 23) who experimentally consumed 1.0 g/kg of an alcohol beverage and subsequently provided breath and blood samples for analysis. Breath expirograms were also collected following several different preexhalation breathing maneuvers. Shortly after the end of drinking the mean of replicate breath alcohol results exceeded that of the corresponding venous blood alcohol. A later paired comparison (during the postabsorptive phase) showed the blood alcohol to exceed the breath. None of the expirograms provided evidence that "mouth alcohol" due to gastroesophageal reflux had biased any test results. People with GERD can provide biased-free end-expiratory breath alcohol results where sound forensic practice is followed, which includes: 15-min. preexhalation observation, duplicate testing, instrumental detection systems, and trained alert operators who ask appropriate questions and watch for associated signs.     

Reliability of breath-alcohol analysis in individuals with gastroesophageal reflux disease.

Gastroesophageal reflux disease (GERD) is widespread in the population among all age groups and in both sexes. The reliability of breath alcohol analysis in subjects suffering from GERD is unknown. We investigated the relationship between breath-alcohol concentration (BrAC) and blood-alcohol concentration (BAC) in 5 male and 5 female subjects all suffering from severe gastroesophageal reflux disease and scheduled for antireflux surgery. Each subject served in two experiments in random order about 1-2 weeks apart. Both times they drank the same dose of ethanol (approximately 0.3 g/kg) as either beer, white wine, or vodka mixed with orange juice before venous blood and end-expired breath samples were obtained at 5-10 min intervals for 4 h. An attempt was made to provoke gastroesophageal reflux in one of the drinking experiments by applying an abdominal compression belt. Blood-ethanol concentration was determined by headspace gas chromatography and breath-ethanol was measured with an electrochemical instrument (Alcolmeter SD-400) or a quantitative infrared analyzer (Data-Master). During the absorption of alcohol, which occurred during the first 90 min after the start of drinking, BrAC (mg/210 L) tended to be the same or higher than venous BAC (mg/dL). In the post-peak phase, the BAC always exceeded BrAC. Four of the 10 subjects definitely experienced gastric reflux during the study although this did not result in widely deviant BrAC readings compared with BAC when sampling occurred at 5-min intervals. We conclude that the risk of alcohol erupting from the stomach into the mouth owing to gastric reflux and falsely increasing the result of an evidential breath-alcohol test is highly improbable.     

Medicare program; end-stage renal disease quality incentive program. Final rule

This final rule will implement a quality incentive program (QIP) for Medicare outpatient end-stage renal disease (ESRD) dialysis providers and facilities with payment consequences beginning January 1, 2012, in accordance with section 1881(h) of the Act (added on July 15, 2008 by section 153(c) of the Medicare Improvements for Patients and Providers Act (MIPPA)). Under the ESRD QIP, ESRD payments made to dialysis providers and facilities under section 1881(b)(14) of the Social Security Act will be reduced by up to two percent if the providers/facilities fail to meet or exceed a total performance score with respect to performance standards established with respect to certain specified measures.     

Neoplastic disease in a medicolegal autopsy material. A retrospective study in northern Sweden

Only a small fraction of sudden unexpected deaths are caused by neoplastic disease and thus subject ot medicolegal autopsy. The medicolegal autopsy forms an opportunity to study not only medically diagnosed and treated neoplasms, but also the natural evolution of untreated disease. In a series of 7,020 consecutive medicolegal autopsies in northern Sweden, we found 171 cases with malignant and/or intracranial neoplasms. In 41 cases, sudden death was caused by previously unknown tumors. The most common mechanisms of death in this group were disseminated cancer, intracranial tumors, pulmonary thromboembolism, hemoptysis, and aspiration of blood, and the most common locations were the bronchi and the lung. In some of these cases, the mechanism was sometimes dramatic, raising a question of violent death or intoxication. In 30 cases, sudden unexpected death was caused by previously known tumors, and also in this group disseminated cancer was the most common cause of death, and the most common locations were the bronchi and the lung. In 22 cases, tumors were found suicidal cases; in 14 of these, the tumor was considered to be a major causative factor to the suicide, while in eight cases the tumor was considered to be an incidental finding. The expected number of cancers in the 1,060 suicides investigated in this series was 27, according to the official cancer prevalence data. Thus, a possible over-representation of suicides among persons with cancer seems doubtful and needs further exploration.     

Chronic beryllium disease prevention program; worker safety and health program. Final rule

The Department of Energy (DOE) is today publishing a final rule to implement the statutory mandate of section 3173 of the Bob Stump National Defense Authorization Act (NDAA) for Fiscal Year 2003 to establish worker safety and health regulations to govern contractor activities at DOE sites. This program codifies and enhances the worker protection program in operation when the NDAA was enacted.     

Chronic beryllium disease prevention program. Office of Environment, Safety and Health, Department of Energy. Final rule

The Department of Energy (DOE) is today publishing a final rule to establish a chronic beryllium disease prevention program (CBDPP) to reduce the number of workers currently exposed to beryllium in the course of their work at DOE facilities managed by DOE or its contractors, minimize the levels of, and potential for, exposure to beryllium, and establish medical surveillance requirements to ensure early detection of the disease. This program improves and codifies provisions of a temporary CBDPP established by DOE directive in 1997.     

Does advancing age modify the presentation of disease?

A group of patients who had died of either cardiac tamponade due to ruptured myocardial infarct, perforated peptic ulcer, or diverticulitis with perforation were studied to determine the effect that age has on the presentation of these three potentially fatal diseases. With advanced age, a decrease in the number of patients who had symptoms typical for the disease that was ultimately the cause of death was found. We also noted a number of patients who did not experience any symptoms, and some patients who experienced symptoms that were not typical of their disease. In many instances, the disparity between disease severity and symptoms had resulted in the disease not being diagnosed either because the patient did not seek medical advice or because the doctor was unable to make the appropriate diagnosis from the patient's symptoms.     

Medicare programs: changes to the end-stage renal disease prospective payment system transition budget-neutrality adjustment. Interim final rule with comment period

This interim final rule with comment will revise the end-stage renal disease (ESRD) transition budget-neutrality adjustment finalized in the CY 2011 ESRD Prospective Payment System (PPS) final rule for renal dialysis services provided on April 1, 2011 through December 31, 2011. We are revising the transition budget-neutrality adjustment to reflect the actual election decision to receive payment under the ESRD PPS for renal dialysis services furnished on or after January 1, 2011 made by ESRD facilities, rather than projected elections using the same methodology as described in the ESRD PPS proposed and final rules. This results in a zero percent adjustment for renal dialysis services furnished April 1, 2011 through December 31, 2011.