FEATURE

Care of the Chronically Ill Patient with a Bleeding Disorder

Detrice Green Barry1, PhD, MSN, RN & Sandra Hibner2, MSN, RN 1 Wright State University–Miami Valley, College of Nursing and Health, Dayton, OH, USA 2 The Children’s Medical Center of Dayton, Dayton, OH, USA

Keywords

Abstract

Hemophilia; bleeding disorders; rehabilitation. Correspondence Detrice G. Barry, Assistant Professor, Wright State University – Miami Valley College of Nursing and Health, 3640 Colonel Glen Highway, Dayton, OH 45435-0001. E-mail: [email protected] Accepted August 07, 2013.

Purpose: This article provides an overview of hemophilia, a chronic bleeding disorder. Design: The epidemiology, diagnosis, treatment, complications, and management of hemophilia are described. Collaborative care using the Hemophilia Treatment Center (HTC) Comprehensive Care Model is presented in a case study. Conclusions/Clinical Relevance: Rehabilitation nurses may encounter patients across the lifespan with bleeding disorders and with basic hemophilia knowledge; coordination with the HTC can provide high-quality care for positive outcomes.

doi: 10.1002/rnj.129

Introduction Chronic conditions such as heart disease, cancer, hypertension, and stroke account for 80% of mortality rates in the United States (Freudenberg & Olden, 2011). Estimated projections suggest that 157 billion U.S. residents will have at least one or more chronic conditions by 2020 and with an aging population, is a significant contributor to the increased incidence of those with chronic conditions (The Lancet, 2009). Consistent with this trend, the prevalence of genetic, x-linked, chronic conditions such as hemophilia will increase as the life expectancy of those with hemophilia increases. Hemophilia is a sex-linked genetic bleeding disorder that results in the decreased plasma activity of either clotting factor VIII or clotting factor IX. Hemophilia A, or classic hemophilia, refers to factor VIII deficiency and hemophilia B, or Christmas disease, refers to factor IX deficiency (National Hemophilia Foundation [NHF], 2006a). Before the availability of advanced clotting factor replacement therapy, the average life expectancy of boys © 2013 Association of Rehabilitation Nurses Rehabilitation Nursing 2015, 40, 197–206

with severe hemophilia was 16 years (NHC, 2013). It was rare for people with severe hemophilia to reach the age of 30; as a result of modern therapy and treatment, the life expectancy of those with severe hemophilia is approaching normal (Nordic Hemophilia Council, 2013). As rehabilitation nurses prepare to care for patients with common chronic conditions such as obesity, diabetes, asthma, heart disease, cancer, hypertension, stroke, and fractures, etc., rehabilitation nurses should also prepare for the impact on care of rare, chronic conditions such as hemophilia. Although rehabilitation nurses cannot expect to be knowledgeable with every disease and disorder, attention should be given to patients with hemophilia due to the chronic nature of the disorder and the potential impact of care on the quality of life. It is crucial for the rehabilitation nurse to work in collaboration with the staff of the Hemophilia Treatment Centers (HTCs). Staff at these centers of excellence has successfully cared for those with hemophilia since 1975 (Baker, Crudder, Riske, Bias, & Forsberg, 2005). The

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Care of the Patient with a Bleeding Disorder

D. G. Barry and S. Hibner

Nurse Coordinator Medical Director

Clinic Nurse Research Nurse

Social Worker

Hematologist

Orthopedist

Physical Therapist

Patient and Family

Genetic Counselor

Child Life Therapist

Psychologist Data Manager

Infectious Disease Specialist

hemophilia team uses the comprehensive care model (Figure 1) and follows the guidelines provided by the Medical and Scientific Advisory Council (MASAC) of NHF (NHF, 2006b). Components of the comprehensive care model envelope multidisciplinary, comprehensive care that is prevention-focused, and based on education and coordination of services available inside and outside the HTC in specific regions (Baker et al., 2005). According to Soucie et al. (2000), people treated through the HTCs experience a 40% decrease in both morbidity and mortality. Healthy People 2020 have six new (not included in Healthy People 2010) science-based national health objectives, which include people with bleeding and clotting disorders (U.S. Department of Health & Human Services, 2013). Attention to impact on quality of life and early preventive care for this rare patient population is imperative. Thus, rehabilitation nurses equipped with basic knowledge of hemophilia care, management, and treatment, and the HTC comprehensive model of care is essential.

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Figure 1 Hemophilia treatment center comprehensive care model. Circles represent members of the HTC team. Overlap of the circles represents the connection of each of the team members. The patient and family in the center denote family-centered care. Adapted from Grosse et al. (2009) and Riske et al. (2011)

Epidemiology Hemophilia occurs in one in 5,000 live male births; of these, 80% represent hemophilia A and 20% represent hemophilia B (Lanzkowsky, 2010). The NHF estimates the incidence of hemophilia in the United States to be 20,000 and the worldwide incidence to be greater than 400,000 (Soucie, Evatt, & Jackson, 1998). Hemophilia is a lifelong disorder and at present, there is no cure. This disorder is not specific to a particular economic class, racial, or ethnic group. (Soucie et al., 1998). The abnormal gene responsible for hemophilia is carried on the X chromosome. Consequently, as females have two X chromosomes, the presence of the defective gene on one X generally does not cause hemophilia since the other X compensates. A female with one hemophilia gene is known as a hemophilia carrier. The presence of an affected X chromosome in males results in the deficiency or absence of factor VIII or IX (Lanzkowsky, 2010). Normal range of factor VIII and factor IX present in the plasma, the liquid portion of the blood, is 50%–150%

© 2013 Association of Rehabilitation Nurses Rehabilitation Nursing 2015, 40, 197–206

D. G. Barry and S. Hibner

Care of the Patient with a Bleeding Disorder

Table 1 Relationship of factor levels to severity of clinical manifestations of hemophilia A and B

Type

Percentage of Factor VIII/IX

Type of Hemorrhage

Severe

Care of the chronically ill patient with a bleeding disorder.

This article provides an overview of hemophilia, a chronic bleeding disorder...
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