Care of the Chronically Ill Patient with a Bleeding Disorder

Authors

  • Detrice Green Barry PhD, MSN, RN,

    Corresponding author
    1. Wright State University–Miami Valley, College of Nursing and Health, Dayton, OH, USA
    • 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: detrice.barry@wright.edu

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  • Sandra Hibner MSN, RN

    1. The Children's Medical Center of Dayton, Dayton, OH, USA
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Abstract

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.

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 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 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.

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)

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.

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% (Lanzkowsky, 2010). A common myth about people with hemophilia is that they bleed faster. The fact is that they do not bleed faster; they bleed longer due to the delay in clot formation (Santagostino, Gringeri, & Mannucci, 2002). The availability of the clotting protein defines how quickly bleeding ceases following injury to blood vessels (Table 1).

Table 1. Relationship of factor levels to severity of clinical manifestations of hemophilia A and B
TypePercentage of Factor VIII/IXType of Hemorrhage
  1. Adapted from Lanzkowsky, 2010.

Severe<1Spontaneous, joint bleeding, deep soft tissue bleeding
Moderate1–5Bleeding following mild-to-moderate trauma some joint bleeding; rarely spontaneous
Mild6–25Severe hemorrhage only if following moderate-to-severe trauma or surgery
High-risk carrier femaleVariableGynecologic and obstetric hemorrhage common, symptoms depend on factor level

Ninety percent of people with severe hemophilia (<1% of factor activity) experience repeated episodes of bleeding into their muscles and joints, often beginning in early childhood; most commonly affected joints are the elbows, knees, and ankles (Manco-Johnson et al., 2007). The final consequence of repeated bleeding into joints is the development of synovial hypertrophy known as arthropathy, which impairs mobility and causes acute and chronic pain (Santagostino & Mancuso, 2008) and morbidity (Manco-Johnson). “Because of its frequency and chronicity, arthropathy is the complication of hemophilia associated with the greatest morbidity. Therefore, one of the primary goals of hemophilia treatment is the prevention of arthropathy” (Manco-Johnson et al., p. 4). There is a growing body of evidence that shows that bleeding episodes must be treated as soon as possible to prevent severe disability in joints or even death (Simpson & Valentino, 2012).

Diagnosis

Diagnosis of hemophilia is made by a series of clotting tests performed on the plasma. The partial thromboplastin time is a screening test that is abnormal in both hemophilia A and B. Hematologists obtain specific clotting factor assays to differentiate between hemophilia A and B and to establish the severity of the disorder. In the United States, most babies are diagnosed by age 2 with 69% diagnosed in the first month of life (Centers for Disease Control & Prevention, 2012). Approximately 15% of babies born with hemophilia are diagnosed between 1 and 6 months, and about 12% are diagnosed between 7 months and the second year of life (Centers for Disease Control & Prevention, 2012).

In approximately two thirds of the newly diagnosed cases of hemophilia, there is a family history of bleeding (Kulkarni & Soucie, 2011). In the remaining one third of cases, there is no family history of a bleeding disorder and these individuals represent new mutations in either the factor VIII or factor IX gene (Kulkarni & Soucie, 2011). Often hematologists diagnose hemophilia after an unusual bleeding event occurs, for example, prolonged bleeding in a newborn from heel sticks, bleeding after circumcision, unexplained bruising following a vaginal delivery, bleeding in the muscle from immunizations, or mouth bleeds when teething (NHF, n.d.; Riske, Butler, & Munn, 2011).

Treatment

Currently, treatment for hemophilia is intravenous replacement of the missing clotting factor (VIII for hemophilia A and IX for hemophilia B); dosage is based on the height and weight of the patient, site of bleeding, and severity of bleeding (NHF, 2002). Other treatments include demopressin acetate, which can be given intravenously or through nasal spray. There are oral coagulants that may be prescribed for mouth bleeds or dental procedures (NHF, 2002). Cryoprecipitate is not widely used in the United States, although is still used in developing countries (Evatt, Austin, Leon, Ruiz Saez, & de Bosch, 2000).

Hoots and Nugent (2006) demonstrated that patients who treated with prophylaxis on a regular basis, for example, Monday, Wednesday, and Friday, have less joint damage than those who treated on demand or episodically. “Episodic” or “on demand” refers to treating after a bleed occurs. Treatment of a bleeding episode will differ depending on the affected site. For example, the amount of clotting factor required for successful management of a joint bleed is often different from that of a muscle bleed.

The nurse working in the HTC has a critical role in providing the medical care and coordination of services for patients with hemophilia. The nurse works with both the individual and family to determine the cause of the bleed; such as whether the bleed is related to an injury or is a spontaneous event. The physician uses clinical judgment to evaluate the bleeding episode and make recommendations for dosing and treatment, such as how much and how often the clotting factor should be given. Additional comfort measures include pain control, limitation of activity, and sometimes physical therapy. The goals of physical therapy are to restore the full range of motion of an affected joint or to rebuild muscle tissue that has been compromised from the effects of bleeding episodes.

When a bleeding episode occurs, the clotting factor should be given as soon as possible. Additional treatment may include providing protection, rest, ice, compression, and elevation (PRICE) (NHF, n.d.; Riske et al., 2011). For example, if a person is experiencing a bleed in a knee, that knee must be protected to prevent further injury. Limitation of mobility provides rest to the joint. Application of ice to the affected site will reduce swelling. Compression with an elastic wrap or bandage will provide comfort and elevation on a pillow above the heart will reduce swelling and provide additional comfort.

When a person with hemophilia is experiencing a bleed, control of pain is crucial and must be monitored continuously. An increase in pain may indicate that bleeding is not under control and healthcare providers may need to alter treatment. Analgesics may be given or narcotics prescribed for pain control. Acute pain during a bleed, although it may be severe, can generally be successfully controlled (Goldfarb, 2009). Many people with hemophilia and other bleeding disorders also experience chronic pain (Goldfarb, 2009). Chronic pain is defined as long-term discomfort, lasting months or longer that usually results from damage to the body, such as arthritic joints; thus, chronic pain is managed differently and may be more challenging than the treatment of acute pain (Goldfarb, 2009). A review by Simpson and Valentino (2012) suggests that issues of arthropathy prevention are challenging due to a nonstandardized dosing regimen. Nonpharmacologic therapies such as physiotherapy or hydrotherapy may be prescribed, with a growing body of evidence of its effectiveness (Simpson & Valentino, 2012), although these treatments may not be covered by insurance.

Studies demonstrate that treatment with prophylactic therapy can prevent joint damage in young boys with hemophilia A and prevent joint and other hemorrhages (Manco-Johnson et al., 2007). Prophylaxis also results in less frequent bleeds, improved joint function, and overall greater patient well-being (Hoots & Nugent, 2006). The benefits of the use of prophylaxis must be weighed against the exorbitant cost of the clotting factor and the economic burden on the family (Guh, Grosse, McAlister, Kessler, & Soucie, 2012a,b).

More than two decades ago, the safety of the blood supply was compromised and of considerable concern for people with hemophilia since factor was manufactured from human plasma. The source plasma was derived from thousands of donors, some of whom were unknowingly infected with viruses such as hepatitis and human immunodeficiency virus (HIV). Consequently, more than 10,000 hemophilic men, women, and children contracted HIV (Evatt, 2006b). Today medical technology and biopharmacology, such as recombinant processing of factor products, are free of all animal and human proteins, and may provide a higher level of confidence against any future microbiological contamination (Charlebois et al., 2001).

There are processes and systems in place to monitor the nation's blood supply. The Centers for Disease Control and Prevention Surveillance System is used to monitor the safety of the blood supply in the United States (Centers for Disease Control & Prevention, 2011). In addition, the NHF, Hemophilia Federation of America (HFA), the Committee of Ten Thousand and others, in conjunction with the bleeding disorders community, continue to work collaboratively with the Plasma Protein Therapeutics Association to remain vigilant, ensuring the safety of the blood supply, not just for people with a bleeding disorder but for everyone.

Hemophilia treatment center

Today, there are approximately 140 federally funded, comprehensive HTCs located throughout the United States. Since their inception in 1973, these designated centers of excellence provide family-centered care of the person and family with attention to not only the physical and medical needs but also the psychological, emotional, educational, financial, and vocational factors (NHF, 2006a). In 1983, staff from the CDC and HTCs partnered and focused on improving health outcomes through risk reduction (Baker et al., 2005). In the early years of collaboration, the focus was on risk reduction for the transmission of HIV and hepatitis by individuals infected from contaminated clotting factor concentrates. Since the mid-1990s, the emphasis has changed to assessing and reducing the complications of hemophilia such as joint disease and inhibitors. Evatt (2006a) suggests that the comprehensive care model has significantly improved patient outcomes in HTCs. This model has been used successfully in other chronic conditions such as cystic fibrosis (Grosse et al., 2009).

The specially trained staff at the HTC provides individualized treatment and management to patients and families. The core HTC staff includes a hematologist, a physician who specializes in hemophilia, a nurse coordinator, psychosocial professional, and a physical therapist (Grosse et al., 2009) (Figure 1). Other members of the healthcare team may include a genetic counselor, orthopedist, and dentist (Grosse et al., 2009). Some HTCs may have additional members of the hemophilia team such as a child life therapist, research nurse, or data manager (Riske et al., 2011).

Complications

One of the major complications of hemophilia is frequent bleeding into the joints that can lead to degenerative arthritis, permanent joint damage, and chronic pain with decreased mobility and loss of muscle tone, all of which has been associated with decreased quality of life (Arranz et al., 2004). People with hemophilia are at risk for complications related to treatment, bleeding episodes, joint disease, heart disease, and renal disease (Arranz et al., 2004). In the past, clotting factor products that were manufactured from donated human plasma lead to complications of related blood infections such as hepatitis C and HIV (Evatt, 2006b).

Another major complication of hemophilia is inhibitor development occurring after exposure to clotting factor. In approximately 30% of patients with hemophilia, the immune system makes antibodies to the clotting factor, which is perceived by the immune cells as a foreign protein (Chalmers et al., 2007). The rate of inhibitor development is lower in patients with >50 exposure days to the clotting factor (Kempton, Soucie, & Abshire, 2006). Inhibitors can, however, develop throughout life (Kempton et al., 2006). The current treatment for inhibitors is immune tolerance therapy, in which a recurrent dose of clotting factor is infused on a regular basis such as daily for up to 12–24 months. This routine is continued until the patient establishes a “tolerance” and ceases making more antibodies or immune tolerance therapy is no longer feasible. This treatment has proven relatively successful; however, in about 30% of patients on immune tolerance therapy, the inhibitor is not eradicated. Such patients are at greater risk for significant complications of hemophilia such as crippling joint damage and a decreased quality of life (Chalmers et al., 2007).

Detection of a bleeding episode

Bleeding under the skin causes hematoma formation with bruising or discoloration. The initial onset of a bleed into the muscle or joint may be reported as a “tingling or funny feeling” in the affected area, progressively followed by heat or warmth to touch, swelling, tightness of the skin, the patient guarding or being unable to move the affected area, and complaint of ache or pain (Centers for Disease Control & Prevention, 2012). Recurrent bleeding into the joints may cause joint damage and limited mobility (U.S. Department of Health & Human Services, 2013). Pain during a bleeding episode can be excruciating and may require narcotics for pain control (Santagostino et al., 2002).

With hemophilia, bleeding can occur anywhere in the body. Injuries to the head, face, eye, chest, neck, throat, tongue, spinal cord, hip, stomach or intestine, urinary tract, and genitalia can lead to permanent deformities or even death if not treated urgently and aggressively (Centers for Disease Control & Prevention, 2012). Common sites of muscle bleeds include: triceps, deltoid, gluteus, hamstring, calf/soleus, biceps, forearm, groin, and thigh.

Case Study

The following case study will examine the various roles of the rehabilitation nurse as the nurse collaborates with the HTC to assist the patient and family toward recovery with positive health outcomes. The rehabilitation nurses' role for care of a patient with hemophilia with the HTC team will be discussed with regard to coordinator of care, patient advocate, and promoter of health.

History

M.T. is a 15-year-old, 150-pound, 6-foot tall, African American male diagnosed with severe hemophilia A at birth. Home management regime includes self-infusion with clotting factor three times a week. He was admitted to the hospital after sustaining injuries from a motor vehicle accident. He was a passenger in the car and was not wearing a seat belt. M.T.'s best friend, J.R. was drinking while driving and is now in critical condition. M.T. has a broken right tibia, broken right femur, three broken fingers on the right hand, cracked ribs, and edema with severe bruising and multiple hematoma formations to his face. M.T. received 50 units/kg or 100% correction of Factor VIII upon admission to the emergency department. After three days in the hospital, M.T. was referred for rehabilitation to an extended care facility.

M.T. lives with his father, who is a pilot for an airline, his mother, who is an interior designer, his 12-year-old sister, and paternal grandmother. He and his friend J.R. are sophomores in high school, participate in the marching band, and play basketball with the local youth organization. M.T. and his family are actively involved and attend a nondenominational Christian church.

Coordinators of care

The initial communication between the HTC nurse coordinator and discharge nurse on the unit could be the referral to the rehabilitative nursing center. If the HTC staff has not been consulted during M.T.'s hospitalization, the rehabilitation nurse would then initiate communication. Collaboration with the staff at the HTC will ensure appropriate treatment, care, and evaluation occurs. Contact with the HTC staff will demonstrate to the patient and family that a coordinated effort is being made to ensure the best possible outcomes. The HTC staff most likely has been involved in the care of the M.T. and his family since diagnosis, which was at birth. Hopefully, a trusting relationship has been built, and involving the HTC staff will provide a level of comfort to the family during the rehabilitation and recovery process.

Note that the nature of hemophilia and bleeding tendencies will be different for each person. As a coordinator of care, the rehabilitation nurse must manage and evaluate the plan of care for effectiveness with the patient, family, and the hemophilia team. The goals of rehabilitation must be met while keeping the requirements of hemophilia care in the forefront. For example, M.T. will require additional factor replacement while participating in physical therapy to reduce the possibility of bleeding. Bleeding episodes would deter healing and prevent progress as bleeding would require an interruption in physical therapy and consequently, the goals of rehabilitation would be delayed.

The plan of care must include provisions for obtaining the factor replacement, how and when to administer, and appropriate follow-up. Follow-up could include coordination of visits to all specialists involved and lab draws. Periodic lab testing, such as factor VIII levels, could be ordered to evaluate the effectiveness of factor replacement therapy. The hematologist will want to maintain M.T.'s factor VIII level at an appropriate level to decrease the chance of bleeding. Coordination among the company supplying the factor replacement, the HTC, and the rehabilitation nurse would ensure necessary and timely supply and delivery of medication.

Decisions regarding venous access must also be made. In this case, M.T. had a peripherally inserted central catheter (PICC) line placed while hospitalized as his plan of care included ongoing frequent infusions. Care of the PICC must follow standards set in place by the institution. Special attention to the prevention of venous thromboembolism is necessary as M.T. is at high risk due to limited mobility and injuries.

Timely revisions and collaboration with teachers and counselors are necessary to ensure students like M.T. stay on target in school. An individualized healthcare plan (IHP) may need to be initiated if one is not already established. This is an individualized plan developed for people with special medical needs to keep them on target for success in school. The IHP is established to protect the student as part of section 504 of the Americans with Disabilities Act (U.S. Department of Justice, 2009). Collaboration between the rehabilitation nurse and HTC social worker and school nurse should occur. The social worker from the HTC will likely have experience in coordinating with school nurses, counselors, and teachers. Input from the parents and the patient is pivotal for successful school progress during rehabilitation.

In this case, the rehabilitation nurse may need to provide ongoing education and support to prepare parents as the person with a bleeding disorder transitions from adolescent to young adulthood. Transition programs for the teen and parents may have been started by the HTC staff. Parents are intricately involved in healthcare decision making and day-to-day management of the person with a bleeding disorder such as insurance, home care (ordering medication and supplies), self-management (infusion and assessment of bleed).

The rehabilitation nurse should provide information to the patient and parents about Health Insurance Portability and Accountability Act and information sharing. Use of social media (Facebook, Twitter, YouTube, etc.) among M.T.'s age group is highly likely. For example, the agency policy regarding the use of social media should be reinforced. If M.T. chooses to use social media, he and the visitors should be reminded of the agency policy specifically about taking pictures of other patients and recording of videos and posting on social media outlets.

In consideration of discharge planning, the rehabilitation nurse would coordinate the physical therapy schedule with the patient and his family. Additional issues to consider include how to carry books while using crutches and possible need to use the elevator. Obtaining a handicap parking pass and of absence from marching band would be addressed with the IHP. Physical therapy should be scheduled to occur on the days that M.T. does his infusion of factor (Monday, Wednesday, and Friday). If any bleeding occurs, the HTC should be contacted and the infusion schedule would need to be adjusted.

Discharge planning would include education of family about when to contact the HTC if any bleeding is suspected or not responding to regularly scheduled infusion time. Also, the importance of keeping follow-up appointments with the orthopedic, HTC, and primary care doctors must be emphasized. Social support needs should be assessed and included in the home discharge plan. For example, family and friends may need to run errands and assist with transportation.

Advocate

The financial burden for the cost of care for a family with a child with hemophilia is astronomical. A child with severe hemophilia can reach the insurance lifetime maximum by age 5. New programs and resources may be available to assist the family. Although some programs are need based, keep in mind that hemophilia affects all ethnic groups and socioeconomic levels. In this family, both parents are employed and have private insurance; however, there may still be in a need for financial assistance. For example, some of the ancillary supplies such as needles, alcohol prep pads, syringes, sharps containers, and bandages needed for management and treatment, mileage for transportation, and parking fees may not be covered.

For the pediatric and adolescent patient and their families, rehabilitation nurses fulfill a valuable role as a patient and family advocate. The first year of life of a child diagnosed with a bleeding disorder can be overwhelming and challenging for the family throughout life. Parents may have guilt or shame about not having the perfect child. The rehabilitation nurse can serve as an advocate to collaborate with the HTC social worker the family for need for counseling. In addition, there are local and national community resources such as the local hemophilia chapter, NHF, or HFA that provide parent support groups, blogs, and web-based support efforts to connect families.

The financial burden resulting from the immense cost of care can place added strain on the family—particularly, in today's struggling economy. Advocating for the family at the institutional level by providing information for the financial specialist to work through issues with insurance regarding home care, and necessary clotting factor medication, ancillary products and durable medical equipment is another important role for the rehabilitation nurse. If there is a major bleed, mobility could be impaired and the patient will require the use of assistive devices. In this case, M.T. will initially need an electric wheelchair, then the use of crutches or a walker to assist with mobility.

It is particularly noteworthy that the patient and family receive timely education and have an understanding of the proper use of crutches and assistive devices. For example, the crutches need to be padded (with a towel or foam padding) on top and the handle. Improper use of crutches can cause a bleed under the arm or on the hands in a person with hemophilia.

The rehabilitation nurse could advocate for the patient upon returning to school from an inpatient stay at the hospital. The rehabilitation nurse could provide resources for the family using the American with Disabilities Act and, specifically, the 504 Plan. As an advocate, the rehabilitation nurse would work with the family to know, understand, and direct the family to proper resources to develop an IHP for school. Thus, the rehabilitation nurse would coordinate with school officials, school nurse, guidance counselor, and teachers. In this case, M.T. has an IHP in place. The role of the rehabilitation nurse would serve as advocate and coordinate with teachers and school counselors for completion of assignments.

To prevent depression, isolation, and self-pity, it is essential for M.T. to remain in contact with his friends while he is recovering. Visitation times, assistance with homework, and talking on the phone will be necessary. Use of computer and social media will be limited due to broken fingers that are casted. It is beneficial to encourage parents and M.T. to openly discuss options and limitations such as planning to attend the game and watch the band perform as he is unable to actively participate.

Cultural and healthcare practices and beliefs should be assessed. Social support through visitation from clergy and church members should be encouraged. Use of prayer, Bible, Holy Oil, and or prayer cloth may be used. When providing care and cleaning the room, if there is a question about an object (prayer cloth), the healthcare provider should ask the patient or family instead of discarding it.

Promoter of health

As the promoter of health, the rehabilitation nurse could use primary prevention strategies to educate the patient with hemophilia and family members about principles of a healthy lifestyle. In this case, the rehabilitation nurse may want to address the use of safety belts. Although M.T.'s blood alcohol level was negative, his best friend was legally drunk and remains in critical condition. Discussion of teenage drinking, use of drugs among friends, and the effects of peer pressure are but a few possible topics to consider.

It would be beneficial for the nurse to emphasize the importance of daily exercise and encourage M.T. to discuss how to continue exercising while he is unable to participate in marching band. Information for the patient and family may include but are not limited to: eating a balanced diet from a variety of foods, more whole grains, fresh fruits, and vegetables (choosemyplate.gov). During the recovery, information on daily exercise should include both aerobic and strength training recommendations to promote healthy muscles and bones. Certain exercises such as swimming, walking, jogging, yoga are recommended; however, high-contact sports are discouraged (NHF, 2005).

As the rehabilitation nurse coordinates with the HTC staff, it is important to keep the patient and family informed and included in the decision-making process. The patient's physical, spiritual, and social well-being should be considered. The scheduling of visits, follow-up appointments, and potential isolation may get burdensome for the family. Resources from local, regional, and national organizations are available (see Table 2). Encourage the family to accept help from friends, family, and church members. Attendance of local hemophilia chapter events provides an opportunity for education, in addition to connecting with others in the hemophilia community. Working consistently to evaluate the needs of the patient and family, coordinating with the HTC and local chapter, will ensure the best possible outcome for M.T.

Table 2. Hemophilia resources
Association of Pediatric and Hematology and Oncology Nurses http://www.aphon.org/
National Hemophilia Foundation http://www.hemophilia.org
World Federation of Hemophilia http://www.wfh.org/index.asp?lang=EN
Hemophilia Federation of America http://hemophiliafed.org/
National Institutes of Health http://ghr.nlm.nih.gov/condition/hemophilia
Centers for Disease Control and Prevention http://www.cdc.gov/NCBDDD/hemophilia/index.html
Hemophilia Treatment Center Directory http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=51&contentid=193
SolutionSight, Inc. (www.solutionsight.com) is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's Commission on Accreditation. Free continuing education is available on a variety of topics related to care of people with hemophilia.
Consumer Choice Directories
Product Manufacturers of Blood Clotting Factor http://hemophiliafed.org/choicedirectories/Flip-Manufacturer/
Care Providers http://hemophiliafed.org/choicedirectories/Flip-Care/
Pharmacy Providers http://hemophiliafed.org/choicedirectories/Flip-Pharmacy/

Discharge planning should include discussion with family about a home assessment and modifications for safety such as stairs, use of crutches on stairs, ability to perform self-care, and activities of daily living. Considerations for modes of transportation must be discussed in addition to the impact on the family. Additional time will be needed in getting ready for school, and some assistance may be needed for time management.

Key Practice Points

  • Hemophilia is a chronic condition with no cure.
  • Include the patient and family in all aspects of treatment and decision making.
  • The patient and family have life experiences in management of hemophilia, listen to them.
  • Maintain early and constant collaboration with staff at the hemophilia treatment center.

Implications for rehabilitation nursing practice

Rehabilitation nurses need to know that bleeding can occur anywhere in the body in a person with hemophilia. Believe the patient and family when concerns are raised. Parents are very knowledgeable and are familiar with usual behaviors for their children. Family-centered care and including the family and patient in all aspects of treatment decision making are important for building trust, adherence to prescribed treatment, and positive outcomes. Changes in behavior and unusual patterns of activity may indicate a need for assessment of bleeding or pain. Below are some essentials in caring for a person with hemophilia for rehabilitation nursing practice:

  • knowledge of basics of hemophilia care and treatment
  • early collaboration with HTC for care coordination
  • timely administration of clotting factor
  • continuous assessment of pain and use of comfort measures (PRICE)
  • respect of cultural and healthcare practices and beliefs
  • home safety considerations
  • emphasis on keeping appointments with HTC and other referrals
  • utilization of resources (See Table 2)
  • avoid administering aspirin or aspirin-containing products (consult with hematologist)
  • avoid intramuscular injections, unnecessary X rays and lab work, arterial punctures, and nasal cautery.

Conclusion

As people with the chronic condition of hemophilia enter the healthcare system with the need for care beyond discharge from an inpatient setting, the rehabilitation nurse is positioned to provide a high level of quality care in collaboration with the HTC team. It is crucial to provide an individualized, family-centered approach to care by including input from the patient and family. Rehabilitation nurses may encounter diverse patients with hemophilia across the life span and can serve as coordinators of care, advocates, and promoters of health. Resources are available for providing evidence-based interventions for a positive rehabilitation experience and health outcomes for the patient.

Acknowledgment

The authors acknowledge Dr. Keith Hoots for his advice in the preparation of this manuscript.

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