“The Nurse Coordinator’s Perspective on Managing Hemophilia Patients With Inhibitors”

 
 
 
  TABLE OF CONTENTS
The Nurse Coordinator’s Perspective on Managing Hemophilia Patients With Inhibitors
Jim Munn, RN, MS

Introduction

More than 30 years have passed since the National Hemophilia Foundation launched its campaign to establish the creation of a network of hemophilia diagnostic and treatment centers across the country.1 Today, more than 140 federally funded hemophilia treatment centers and programs exist in the United States, and multidisciplinary team care with an emphasis on early diagnosis and intervention to prevent complications has evolved into the norm for the treatment and management of hemophilia.1,2

The comprehensive team care model has become one of the most successful public health initiatives in developed countries. Studies have shown that this model results in significantly improved outcomes for patients and reduced healthcare utilization.3 Within this model, a core treatment team is assembled, consisting of a nurse coordinator, a medical director (usually a hematologist), physiotherapist, and social worker.3 Each member is an integral part of the team, offering specialized care and expertise to hemophilia patients and their families.


 

The Nurse Coordinator’s Role in Hemophilia Team Care
  

In Standards and Criteria for the Care of Persons With Congenital Bleeding Disorders, the National Hemophilia Foundation outlines the following responsibilities of the hemophilia nurse coordinator: 2

•   Assessment of patient’s general health and bleeding-related issues
•   Patient and family education
•   Coordination and evaluation of home therapy and home infusion programs
•   Development and coordination of patient plan of care
•   Interaction with all team members to ensure plan carried out safely
•   Participation in quality assurance activities
•   Facilitation of data collection and documentation
•   Participation in research studies
•   Liaison between hemophilia treatment center and community, including primary care providers

The nurse coordinator serves as the link that connects the patient to other members of the care team and extended medical community, including the patient’s primary care provider. Nurse coordinators enroll patients in clinical trials about genetic mutations; liaise with outside hospital, inpatient units, and emergency rooms; and function as the first line of triage for patients and their families.



“The nurse coordinator serves as the link that connects the patient to other members of the care team and extended community, including the patient’s primary care provider.”


Listen to Nurse Coordinator Munn discuss...
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...how he selects the ideal candidates to enroll in clinical trials.

Jim Munn, RN, MS

1:19


Table 1a

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Predicting and Preventing Inhibitor Development
  

The development of inhibitors is the most serious complication associated with hemophilia. Antibody response to factor VIII can occur in up to 30% of patients with hemophilia A, and in up to 5% of patients with hemophilia B.4 Orthopedic complications and the effect on quality of life are more severe in patients with inhibitors, and bleeds are more difficult to control.4

Patients with high-titer inhibitors are at increased risk for potentially life-threatening bleeds, which in turn necessitate complex and costly treatment.5 Identifying and understanding those risk factors that are modifiable is essential for the potential prevention of inhibitor development in patients with hemophilia.5

Known patient-related risk factors for inhibitor development include ethnicity, family history of inhibitors, and factor VIII gene mutation type (Tables 1a and 1b).5

Treatment-related risk factors (Tables 2a and 2b) include early age at onset of treatment and intensity of treatment, perhaps due to the tissue damage and inflammation that is associated with the major bleeds or surgeries that lead to intensive treatment.5

Conversely, some studies have noted a protective effect with prophylactic treatment. In the CANAL study, a retrospective multicenter cohort study performed by Gouw and colleagues, a 60% lower risk of inhibitor development was observed with regular prophylaxis.5 Gouw and colleagues also compared the incidence of inhibitor development when recombinant factor VIII products were used as compared with plasma-derived factor VIII products. The authors found no evident difference in inhibitor incidence between plasma-derived and recombinant factor VIII products (Tables 3a and 3b).6

There was a slightly lower risk of inhibitor development in patients who received plasma-derived factor VIII concentrate, but this risk was not statistically significant. Switching between products also did not appear to affect inhibitor risk.6


Table 1b

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Table 2a

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Table 2b

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Listen to Nurse Coordinator Munn discuss...
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...his clinical experience with predicting which of his patients are at risk for inhibitor development and the steps he took to circumvent inhibitor development.

Jim Munn, RN, MS

2:02



Table 3a

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Table 3b

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Preparing Patients for Immune Tolerance Induction
  

When patients develop inhibitors, immune tolerance induction (ITI) is the only proven method of eradication. Carcao et al report a 60% to 80% success rate with ITI. ITI therapy regimens involve regular infusions of replacement factor, with the goal of inducing antigen-specific tolerance, which allows patients to reinstitute factor replacement therapy.4

Patients who may be candidates for ITI need effective education and counseling so they can make informed decisions, and in order for optimal outcomes to be achieved. Key points for the nurse coordinator to relay to patients include the costs, time commitment, and lifestyle changes associated with ITI, as well as the potential for adverse effects. Adverse effects generally are related to the factor concentrate used, the type and quantity of bypassing agent employed, the administration of any immunosuppressive agent, and the use of central venous access devices.4


Listen to Nurse Coordinator Munn discuss...
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...his clinical experiences with preparing patients for inhibitor eradication with ITI and bypassing therapy.

Jim Munn, RN, MS

1:42


Preparing Patients for Surgery
  

Traditionally, all but the most essential surgeries have been avoided in hemophilia patients with inhibitors because of concerns that adequate hemostasis could not be achieved or maintained in such patients. As a result, many patients were denied procedures that could greatly improve their quality of life, such as orthopedic procedures.7

Recently, however, a movement has been underway to offer more surgeries to hemophilia patients with inhibitors provided that adequate preoperative and postoperative measures are employed to ensure patient safety. These include preoperative screening for the presence of inhibitors, notification of all members of the hemophilia treatment team of any impending surgeries, and assurance of adequate laboratory and blood bank support. Factors to consider in surgical hemophilia patients with inhibitors are listed in Table 4.7

Table 4. Surgery in inhibitor patients: factors to consider

  • Age of patient
  • Inhibitor titre and anamnestic response
  • Relevant co-morbidities
  • Indication for surgery
  • Urgency of the surgical procedure
  • Type of surgical procedure
  • Experience of surgeon
  • Availability of multidisciplinary team of experts experienced in perioperative management of haemophilia (haemophilia treatment centre)
  • Risk factors for thrombosis (age, type of procedure, thrombophilic genotypes)
  • Patient and family preferences
  • Sufficiency of supply of replacement and bypassing products
  • Cost of replacement and bypassing products to national blood system, patient, or third-part insurer

Posted with permission from Teitel J, et al. Haemophilia. 2009;15:227-239. Copyright © by Wiley-Blackwell.

Elective surgical procedures should always be performed in coordination with the hemophilia treatment team. The nurse coordinator can then ensure the availability of adequate laboratory support for reliable monitoring of clotting factor level. It is recommended that elective surgery be scheduled early in the week and early in the day in order to allow for optimal laboratory and blood bank support as well as the availability of sufficient quantities of clotting factor concentrates and bypassing agents.8

Preoperative prophylaxis in inhibitor patients involves high-dose factor VIII replacement by intermittent bolus injection or continuous infusion: a bolus infusion of FEIBA of 50-75 IU/kg -1 or immediate bolus infusion of factor VIIa.7

Postoperative management involves clinical monitoring, FEIBA or recombinant factor VIIa infusions, and coordination of factor delivery to the patient’s home or long-term care facility. The patient’s home environment and therapeutic requirements must be assessed prior to discharge and caregivers must be educated on postoperative rehabilitation. Nurse coordinators should also ensure reimbursement for long-term care and clotting factor usage, the availability of adequate amounts and appropriate vial sizes of clotting factor, and patient follow-up post-discharge.



Presurgical Management of Hemophilia Patients With Inhibitors: Key Points
  

  • Know the type of surgery
  • Emergent or planned?
    • Orthopedic or nonorthopedic?
  • Screen for presence of inhibitors
    • If present, high or low responder?
  • Historical response to factor concentrates
  • Communicate appropriate treatment plan to treatment team

Listen to Nurse Coordinator Munn discuss...
Jim Munn's image
...his recommendations for preparing patients with inhibitors for surgery, and monitoring them postoperatively.

Jim Munn, RN, MS

2:34

Expert Commentary



REFERENCES
  
     
  1. National Hemophilia Foundation. Available at: www.hemophilia.org. Accessed August 3, 2010.
  2. National Hemophilia Foundation. Standards and Criteria for the Care of Persons With Congenital Bleeding Disorders (revised April 2002). Available at: www.hemophilia.org/NHFWeb/Resource/StaticPages/menu0/menu5/menu57/masac132.pdf. Accessed April 20, 2010.
  3. Evatt BL. The natural evolution of haemophilia care: developing and sustaining comprehensive care globally. Haemophilia. 2006;12(suppl 3):13-21.
  4. Carcao M, Lambert T. Prophylaxis in haemophilia with inhibitors: update from international experience. Haemophilia. 2010;16(suppl 2):16-23.
  5. Gouw SC, van der Bom JG, Marijke van den Berg H, for the CANAL Study group. Treatment-related risk factors of inhibitor development in previously untreated patients with hemophilia A: the CANAL cohort study. Blood. 2007;109:4648-4654.
  6. Gouw SC, van der Bom JG, Auerswald G, Ettinghausen CE, Tedgård U, Marijke van den Berg H, for the CANAL Study group. Recombinant versus plasma-derived factor VIII products and the development of inhibitors in previously untreated patients with severe hemophilia A: the CANAL cohort study. Blood. 2007;109:4693-4697.
  7. Teitel JM, Carcao M, Lillicrap D, et al. Orthopaedic surgery in haemophilia patients with inhibitors: a practical guide to haemostatic, surgical and rehabilitative care. Haemophilia. 2009;15:227-239.
  8. World Federation of Hemophilia. Guidelines for the Management of Hemophilia. Available at: www.ehc.eu/fileadmin/dokumente/Gudelines_Mng_Hemophilia.pdf. Accessed April 24, 2010.