Haematologica 2000;85:supplement to no. 10 - Supplements ...

Haematologica 2000; 85(suppl. to n.10)
p. 78-80
Clinical and Organizational Aspects
Related to Immunotolerance Protocols
Immune tolerance: a nursing perspective
REGINA B. BUTLER
The Children’s Hospital of Philadelphia, USA
Since immune tolerance (IT) therapy was
first introduced 25 years ago, 1 several versions
of the original protocol have been
developed to eliminate factor VIII and factor IX
inhibitors. 2 The protocols vary in dose and frequency
of factor administration, in the use of
additional drugs, and in the use of supplemental
clotting concentrates. However, all immune
tolerance protocols entail frequent intravenous
infusions and require strong commitment on
the part of patients and families. Nursing participation
in IT can enhance the program and
contribute to a successful outcome.
Nurses provide essential elements of clinical
care to patients with hemophilia who develop
inhibitors. Often, nursing assessment of a
patient’s lack of response to treatment leads to
the initial diagnosis of inhibitors. Once an
inhibitor is identified, nurses work closely with
physicians and the family to develop a plan of
care for the patient. They help in the selection
of appropriate candidates for immune tolerance
induction, conducting patient/family education,
assessing venous access, assisting in the
management of acute bleeding episodes during
induction, and monitoring patient response to
therapy. Nursing interventions are critical in
helping families meet the challenges required
for a successful outcome. 3
While data have been collected about the success
of various prescribed regimens for immune
tolerance, little information is available regarding
nursing perspectives or about facilitating
family participation in such a protocol. To identify
the challenges and determine successful
nursing strategies, input was requested from
hemophilia nurses with experience in IT.
Methods
An informal questionnaire was developed and
sent to 115 nurses at Hemophilia Treatment
Centers (HTCs) in the United States. The 14-
item questionnaire requested information
Correspondence: Regina B. Butler, RN, M.D., Division of Hematology,
The Children’s Hospital of Philadelphia, 34th and Civic Center Blvd.,
Philadelphia, PA 19104 USA. Phone: international +001-215-590-
2198 Fax: international +001-215-590-3992 E-mail:
Butler@email.chop.edu
about nurses’ experience working with patients
on IT programs, including the number of
patients begun on IT, and the number who successfully
completed, discontinued or failed
therapy. Participants were asked to rank issues
related to IT in order of importance. Five openended
questions elicited information about
patient adherence, financial considerations,
venous access and central venous lines,
patient/family education, and implications of
the patient’s age at the time of induction. Finally,
nurses were asked to discuss psychosocial,
educational, and nursing considerations in
patient selection and preparation of families,
barriers to care, and strategies to promote optimal
participation and successful outcomes.
Results
Thirty-one nurses from 22 states and the District
of Columbia completed and returned the
survey. These nurses represent experience with
176 patients who have begun IT induction.
All survey participants noted patient/family
selection and preparation of the family as
important components of successful immune
tolerance induction. Consideration must be given
to the readiness of the family to undertake a
demanding medical intervention. Both parents,
if available, must agree to and support the plan,
while recognizing the importance of adhering
to the protocol; once started, IT requires total
commitment. In single parent homes, it is helpful
to seek another family member or friend who
can support the parent in this endeavor. Family
members need to understand the expected
duration of the induction, including expectations
about continuing maintenance when the
inhibitor is no longer detected.
The age of the child is an additional consideration
in patient selection, according to 88% of
nurses surveyed. Age has an impact on venous
access and types of central venous lines that
may be recommended. Whatever the access, the
child must be able to co-operate with infusions;
babies often require at least two adults to assist
in treatment, even with central lines. Older boys
may help with treatment, or self-infuse, but
often tire of the daily infusions and can resist
Haematologica vol. 85(supplement to n. 10):October 2000