Factor for felons: how can we provide haemophilia care to the incarcerated?

OBJECTIVES: In 2011, 6.98-million offenders were documented in the adult correctional system, with state operating costs designated 12% towards medical care ($11.97 day per inmate) for the general population. Common co-existing health problems identified are: arthritis (13%), hypertension (11%), asthma (10%) and heart problems (6%). Less than 5% of inmates have health issues related to cancer, diabetes, liver or renal problems and communicable diseases. The leading cause of death is suicide (33.2%), followed by heart disease (26.1%). Despite these statistics quality is lacking. Given these statistics, one would expect that a small proportion of patients from Hemophilia Treatment Center (HTC) will spend some time within the justice system. Currently there are no data addressing haemophilia care needs while incarcerated. METHODS: This article will review the current health care issues in the adult correctional system. Additionally, six case reports of incarcerated haemophiliacs will be highlighted exploring the successes and challenges with maintaining haemophilia care addressing the priority of meeting the haemophilia care needs verses the penal system regulations. SUMMARY: It can be expected that at some point, the HTC will experience a patient incarcerated for some period of time. The HTC will continue to advocate for their patient within this system, despite the many challenges faced. CONCLUSIONS: Despite the challenges outlined, ongoing communication and education with the correctional system, education of the medical personnel and prison personnel remains the priority as we advocate for our patients. Continued strategies in these areas are paramount.

The challenges of the homeless haemophilia patient.

The current economic hardships within the United States can increase the risk of persons becoming homeless. In 2001, it was estimated that between 0.1% and 2.1% of the population were homeless every night and that 2.3 - 3.5 million persons could become homeless every year [1]. Many issues can increase the risk of homelessness including: home foreclosure, declining work force due to declining wages, low-wage opportunities and less secure jobs, decline in public assistance, lack of affordable housing with limited housing assistance programs, poverty, lack of affordable health care, domestic violence, mental illness, and addiction disorders. Many on the streets may suffer from mental illness, developmental disabilities, and or chronic physical illness [6]. Given these issues, the Hemophilia Treatment Center (HTC) can expect to experience the issue of homelessness within their own population of persons with hemophilia. Currently, there are no studies that address the issue of the person with hemophilia who may become homeless. This presents unique challenges that this population may encounter to survive in addition to managing bleeding issues related to the diagnosis of hemophilia. This article will review the issues related to homelessness in the general population. Two case studies of persons with hemophilia who became homeless will be discussed outlining the strategies utilized to assist the patient during this crisis.

A national study of pain in the bleeding disorders community: a description of haemophilia pain.

UNLABELLED: The National Pain Study was a prospective, computer-based, descriptive survey of the pain experience of persons with a bleeding disorder conducted in the United States over a 28 month period from 2007 to 2009. The aim of this study was to (i) determine the language used by patients to describe and differentiate acute and persistent pain, (ii) describe pharmacological and non-pharmacological strategies utilized to control pain, (iii) assess the perceived effectiveness of current pain management on quality of life and, (iv) to determine who provides pain management to this population. One thousand, one hundred and four surveys were received. Only the responses of the 764 respondents who reported having hemophilia A or B were evaluated for this paper. Thirty nine percent of participants reported their pain was not well treated. The average acute pain score associated with a bleed reported was 5.97/10 while the average persistent pain score reported was 4.22/10. The most frequently reported word descriptors for acute pain were: throbbing, aching, sharp, tender and miserable. The most frequently reported word descriptors for persistent pain were aching, nagging, tiring, sharp, and tender. The most frequently reported pain strategies for acute and persistent pain included factor, rest, ice, elevation, and compression. Alcohol and illicit drugs were reportedly used to manage both acute pain as well as persistent pain. Primarily, short-acting opioids and acetaminophen were reported to treat both acute and persistent pain. Hematologists and primary care providers provide the majority of pain management for persons with hemophilia (PWH). Quality of life (QOL) scores were lowest in the domains of pain, energy/fatigue and physical problems indicating disruption of QOL. This substantiates under-recognition and under-treatment of pain in the hemophilia population when combined with the 39% of respondents who felt their pain was not well treated and literature in the general pain population of wide spread under-treatment of pain. RECOMMENDATIONS: The NPS is an initial step in recognizing the prevalence and description of pain in PWH. HTC providers should educate themselves in pain management techniques to better serve this population. Further research is necessary to develop specific pain management guidelines for the bleeding disorders population that include multimodal holistic treatment plans.

Liver transplantation in the haemophilia patient.

Summary. Hepatitis C is a chronic condition that many persons with haemophilia contracted in the 1980s due to the infusion of factor concentrates that did not have viral inactivation processes in place. Many patients with haemophilia are now living longer lives, well into their eighties due to the improvement of their care. The effects of the hepatitis C virus on the liver over time are now being realized as this population ages. Although the new treatments for hepatitis C have a prolonged response, as demonstrated by a persistent negative viral load, many haemophilia patients have either not responded to the therapy or had significant side effects to treatment, which prevented continued therapy. Of these infected haemophiliacs with liver disease, many have demonstrated a slow progressive decline resulting in liver failure, cirrhosis or liver cancer. Liver transplant then becomes their only option. This article will review liver transplantation in the haemophilia patient highlighting three case studies demonstrating the effectiveness of specific short-term factor infusions and other haemostatic support to minimize bleeding during the surgical period. These cases confirm the opportunity for successful liver transplantation for haemophilia patients utilizing specific factor recommendations with minimal bleeding risk.

Liver transplantation in the haemophilia patient.

Hepatitis C is a chronic condition that many persons with haemophilia contracted in the 1980s due to the infusion of factor concentrates which did not have viral inactivation processes in place. Many patients with haemophilia are now living longer lives, well into 80 years of age, due to the improvement of their care. The effects of the HCV on the liver over time are now being realized as this population ages. Although the new treatments for hepatitis C have a prolonged response, as demonstrated by a persistent negative viral load, many haemophilia patients have either not responded to the therapy or had significant side-effects to the treatment, preventing continued therapy. Of these infected haemophiliacs with liver disease, many have demonstrated a slow progressive decline resulting in liver failure, cirrhosis or liver cancer. Liver transplant then becomes their only option. This article will review liver transplantation in the haemophilia patient highlighting three case studies demonstrating the effectiveness of specific short-term factor infusions and other haemostatic support to minimize bleeding during the surgical period. These cases confirm the opportunity for successful liver transplantation for haemophilia patients utilizing specific factor recommendations with minimal bleeding risk.

Assessment of acute and persistent pain management in patients with haemophilia.

A descriptive survey was conducted in Region V-E of the United States to bridge the gap in available information on pain issues in the bleeding disorders population. The aim of this study was to a) determine language used by patients to describe and differentiate acute and persistent pain, b) describe pharmacological and non-pharmacological strategies utilized to control pain, c) determine the providers of pain management to this population and d) evaluate quality of life incorporating the SF-36 QOL tool. A total of 202 surveys were returned. For the purposes of this paper, it was decided to analyse only haemophilia data (n = 114). Average persistent daily pain levels were 5/10 (P < 0.001). The three most common word descriptors for both acute and persistent pain were the same - achy, throbbing and tender; the most utilized pain medications were NSAIDs and acetaminophen. Factor replacement was used for what respondents described as acute pain management 79% of the time and for persistent pain management 38% of the time. Participants described acute and persistent pain with the same pain descriptors leading to the conclusion that patients have difficulty distinguishing between acute and persistent pain. This lack of differentiation was further displayed by the use of factor replacement to treat persistent pain associated with arthritic discomfort (38%) which would be viewed as inappropriate, as well as lack of factor replacement use by 21% of respondents who identified pain as from an acute bleed. Opportunities exist to improve pain management through patient and provider-directed educational programs.

Dialysis in the haemophilia patient: a practical approach to care.

The major focus of care for patients with haemophilia is to ensure health with minimal joint dysfunction. As this population ages, additional coexisting conditions can develop including rare instances of nephrotic syndrome in haemophilia B inhibitor patients undergoing immune tolerance, hypertension, diabetes, and coronary artery disease, all of which can adversely affect the renal system over time. In haemophilia patients, co-infected with HIV and hepatitis C, these conditions can also increase the risk of renal problems resulting in the need for dialysis. This article provides a practical approach for the haemophilia patient who requires dialysis and outlines the decision making process to ensure a positive outcome. The goal of care is to optimize dialysis treatment without increasing the bleeding risk.