A. Lambing

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

Summary.  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.

Better adherence to prescribed treatment regimen is related to less chronic pain among adolescents and young adults with moderate or severe haemophilia

Little data exist, especially for adolescent and young adult (AYA) persons with haemophilia (PWH), about the relationship between adherence to prescribed treatment regimen and chronic pain. We examined this relationship among PWH (moderate or severe) aged 13–25 via cross‐sectional survey. Adherence was assessed using the Validated Hemophilia Regimen Treatment Adherence Scale (VERITAS)‐Pro and VERITAS‐PRN for prophylactic and on‐demand participants respectively. VERITAS scores range from 24 (most adherent) to 120 (least adherent). Chronic pain was measured using the FPS‐R and was dichotomized as high for FPS‐R scores ≥4 and low for <4. Logistic regression models were constructed to assess factors associated with having high (vs. low) chronic pain. Of 80 AYA respondents (79 men), most had severe disease (91%), infused prophylactically (86%) and had haemophilia A (91%). Fifty‐one per cent were aged 13–17 and most were white (76%), non‐Hispanic (88%) and never married (93%). Chronic pain was reported as high for 35% of respondents. Mean VERITAS‐Pro scores for those with high and low chronic pain were 53.6 ± 12.3 vs. 47.4 ± 12.9, P = 0.05. VERITAS‐PRN scores were similar across chronic pain status. Logistic regression revealed that for each 10‐point reduction (i.e. increase in adherence) in the combined VERITAS (Pro and PRN) and VERITAS‐Pro scores there was a 35% (OR = 0.65; 95%CI = 0.44, 0.96; P = 0.03) and 39% (OR = 0.61; 95%CI = 0.39, 0.96; P = 0.03) reduction in odds of having high chronic pain respectively. Among AYA PWHs, better adherence was associated with significantly lower odds of having high chronic pain. Moreover, non‐whites were >4 times as likely as whites to report high chronic pain.

Dialysis in the haemophilia patient: A practical approach to care

Summary.  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.

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 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.

Use of acupuncture in the management of chronic haemophilia pain

Summary.  Persons with haemophilia experience persistent pain resulting in chronic arthritic symptoms. The older person with haemophilia who did not benefit from primary prophylaxis are particularly at risk for persistent pain in multiple target joints as a result of repeated joint bleeding with delayed treatment received. The National Pain Study, Ref. [11] identified over 700 persons with haemophilia who rated daily persistent pain as 4.22/10 (SD ± 2.05) using a visual analogue scale. The study suggests that persons are continually seeking additional resources to relieve pain. This study seeks to identify: (i) effects of acupuncture use for persons with haemophilia who experience persistent joint pain as a result of repeated haemarthrosis and (ii) document no reported increased bleeding risk as a result of acupuncture use. End points include the subjects reported perception of reduction in pain via the 10‐point visual analogue pain scale and bleeding. Nine subjects participated in the study, some who received a factor replacement to 15% correction and others who did not receive factor prior to any acupuncture sessions totaling 14 acupuncture treatments. No one of the subjects experienced bleeding or bruising. Six of the nine subjects reported an improvement in pain scores by at least 50%. Seven of the nine quality of life (QOL) domains within the QOL SF‐36 questionnaire improved, suggesting a perception by subjects of improvement. This study suggests that acupuncture therapy can be a safe additional modality for pain management therapies in persons with haemophilia, although larger randomized studies are needed for further validation.

Enhancing Communication for End-of-Life Care: An Electronic Advance Directive Process

An advance directive, a description of ones future preferences for medical treatment, must be easily available to care providers to ensure that ones treatment preferences are honored. The transition of our health care system to an electronic medical record complicated the availability of a patients written advance directive. This paper describes the development of an electronic advance directive to facilitate access to a patients treatment wishes at any site in our health care system. The successes and challenges encountered in the development process are discussed.

The dangers of iron overload: Bring in the Iron Police

Purpose: To increase awareness of nurse practitioners (NPs) about the dangers of iron overload and to review common hematologic conditions where transfusions are essential, iron mechanism and transport within the body, effects of iron overload on the body, and treatment options available. Finally, the process for development of a protocol for monitoring such patients will be introduced. Data sources: Review of existing literature, myelodysplastic syndrome guidelines, sickle cell guidelines, thalassemia guidelines. Conclusions: Transfusions of packed red blood cells save lives. There are many hematologic conditions for which packed red blood cell transfusions are required as a result of bone marrow suppression. However, extended exposure to red blood cell transfusions places the patient at an additional risk of iron overload. Iron overload is a real concern for patients who become transfusion dependent, with increased risks of liver cirrhosis and cardiac arrhythmias. Implications for practice: NPs in all areas of practice can increase awareness of the dangers of transfusional iron overload, and become familiar with the treatment options available. Additionally, NPs can institute a monitoring program based on the protocol suggested here within their own practices to prevent poor outcomes for patients with transfusion‐related iron overload.Purpose: To increase awareness of nurse practitioners (NPs) about the dangers of iron overload and to review common hematologic conditions where transfusions are essential, iron mechanism and transport within the body, effects of iron overload on the body, and treatment options available. Finally, the process for development of a protocol for monitoring such patients will be introduced. Data sources: Review of existing literature, myelodysplastic syndrome guidelines, sickle cell guidelines, thalassemia guidelines. Conclusions: Transfusions of packed red blood cells save lives. There are many hematologic conditions for which packed red blood cell transfusions are required as a result of bone marrow suppression. However, extended exposure to red blood cell transfusions places the patient at an additional risk of iron overload. Iron overload is a real concern for patients who become transfusion dependent, with increased risks of liver cirrhosis and cardiac arrhythmias. Implications for practice: NPs in all areas of practice can increase awareness of the dangers of transfusional iron overload, and become familiar with the treatment options available. Additionally, NPs can institute a monitoring program based on the protocol suggested here within their own practices to prevent poor outcomes for patients with transfusion-related iron overload.

The dilemma of inherited dysfibrinogenemia during pregnancy.

Inherited dysfibrinogenemia is a rare disorder caused by mutations in the fibrinogen gene, described in approximately 400 families to date. We present the case of a 20-year-old woman at 7 weeks of pregnancy with a history of two first-trimester spontaneous abortions and a family history of thrombotic events. Her testing revealed evidence of dysfibrinogenemia, necessitating multidisciplinary management planning including Hematology, OB-GYN, Maternal-Fetal Medicine, Blood Bank Services and Anesthesia. Antenatal care included a combination of intravenous fibrinogen infusions to maintain fibrinogen levels above 100 mg/dl and anticoagulation with low molecular weight heparin. She had an uneventful full-term delivery and continued fibrinogen infusions and thromboprophylaxis for 6 weeks postpartum. The combination of fibrinogen infusions and anticoagulation maintained the balance between bleeding and clotting in our patient during pregnancy. We recommend a multidisciplinary team approach for the management of dysfibrinogenemia during pregnancy to provide successful pregnancy outcomes.

Infection risk and stability of a continuous 8-h 250 mL rFVIII infusion.

This study seeks to identify the delivery method of continuous infusion using a 250 cc IV bag via pump, change every 8 h. Additionally, the study will examine the infection risk with the use of 8 h infusions. Ten hemophilia A patients were identified for the study. Each patient received a bolus factorVIII (FVIII) infusion with a pre FVIII level and 1 h post FVIII level to determine recovery levels for optimal dosing. On the day of 8‐h continuous infusion, the pt received a bolus VIII (Kogenate FS ™) for correction to 100% followed by individually calculated continuous infusion (Kogenate FS ™) FVIII. FVIII levels were drawn from the IV bag and peripherally from the patient in the opposite arm at time points: pre infusion, 1, 2, 3, 4, 5, 6 and 8 h. Additionally, blood cultures were drawn from the IV bag and from the IV tubing at time points pre infusion, 4 and 8 h. Fourteen subjects agreed to participate in the study; 4 failed to follow up, hence 10 subjects were included in the analysis of data; 7 severe, 2 moderate, and 1 mild hemophilia A. Age range was 26–62 years. Ethnic breakdown included 5 African American, 4 Caucasian, 1 Hispanic. With all infusions, the range of FVIII was 65–135% (blood) and 62–200% (bag). After the start of infusion, there were no significant differences noted between the hourly FVIII levels in the subjects and the IV values (P‐value range 0.36–0.9). Additionally, given three time points with six cultures per patient, totaling 60 points of cultures drawn for the study, all cultures from the IV bag and patient were negative. The effective delivery method and safety of an 8‐h continuous infusion of FVIII (Kogenate FS ™) has been confirmed. This method can be helpful given that many hospitals may not carry the required mini‐pumps, allowing a standard safe delivery of FVIII (Kogenate FS ™) continuous infusion by available means.

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

In 2011, 6.98‐million offenders were documented in the adult correctional system, with state operating costs designated 12% towards medical care (