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

Immune Tolerance and the Treatment of Hemophilacs with an Inhibitor 79
following the prescribed protocol. All children
will require age-appropriate explanations about
the protocol and the importance of maintaining
the treatments.
Compliance and venous access were identified
by 90% of the nurses as the most critical issues
to consider when preparing families for immune
tolerance. Patient compliance is crucial to the
effectiveness of any therapeutic plan. Without
patient participation in the medical plan, the
goals of treatment cannot be achieved. 4 However,
rates of non-adherence to medication
schedules for patients with chronic illness are
reported to be about 50%. 5 Compliance is influenced
by several factors, including the patient’s
motivation, the complexity of the medical regimen
and the ease in which the new activity can
be incorporated into the patient’s lifestyle. 6 As IT
protocols require frequent intravenous infusions
for children, compliance can be difficult. As disruptions
in the protocol are likely to yield poor
results, clinicians are challenged to assist families
with maintaining the desired schedule and
dose of factor replacement.
Adherence to the demands of an IT protocol
can be enhanced through educational and
behavioral approaches. This begins with the
development of a trusting partnership with the
family, promoting skill acquisition and providing
information that enables them to make an
informed choice about treatment. 7
Financial and insurance barriers were reported
as significant by only 7% of the participants.
Most comments indicated that, with education
of insurance companies and primary care
providers, coverage for IT is usually obtained.
However, nurses emphasized the importance of
assisting families with insurance issues and
adjusting treatment and visit schedules to minimize
disruption of parental employment.
Discussion
Patient education, noted to be essential by all
nurses, begins before IT is initiated and continues
throughout the program. Families need a
basic understanding of inhibitors and how their
continued presence can complicate treatment
of bleeding episodes. The need for faithful
adherence to the IT protocol and the potential
implications of disrupted doses should be clear
to patients and families. Details about the protocol
to be used should be clearly explained and
written down for further reference. Administration
of the newly prescribed factor concentrate
must be reviewed with the family; often storage
requirements and rate of infusion differ from
those of the patient’s current product. Families
need to recognize the extent of the commitment
needed for a successful outcome; the regular
infusions, explicit details about laboratory monitoring,
follow up visits, and expectations about
recovery studies. The potential duration of the
protocol, the guidelines for dose adjustments,
and parameters for determining success or failure
of the protocol should be clarified at the
outset.
Management of acute bleeding episodes during
IT is an important topic to discuss before
starting IT; the treatment plan requires revision
and review throughout the program. Families
may need help in understanding the need for
two inventories of factor concentrate; one for
IT, another for use in treatment of acute bleeding.
Venous access is a critical issue for children on
IT and is a patient teaching challenge. If treatment
is to be given via peripheral access, parents
or caregivers will need training and support
in venipuncture. Most young children, however,
will require a central venous access device. Considerable
education of patients and families
about different types of lines with risks and benefits
of each is needed. Line placement raises
issues about hemostasis for the procedure. Families
and hospital staff will require education
about the procedure and the factor replacement
plan, particularly if products less familiar to the
staff are considered. Caregivers will need intensive
education about the care and access of the
line. All caregivers will require education and
reinforcement about sterile technique and standard
precautions. Simple, written instructions
with illustrations may be helpful and less experienced
families may benefit from home nursing
visits until the parents and the child are more
comfortable with the procedure.
While patient education is integral to IT and
helps support adherence to the protocol, information
alone is not sufficient. Usually, families
need additional support to promote optimal
participation in IT. Strategies include eliciting
patient/family feedback about the protocol,
adjusting the treatment schedule to consider the
family lifestyle, providing clear, written instructions,
development of cues as reminders of
scheduled treatments or interventions, and
negotiating a reward system for children participating
in IT. A good relationship with the family
promotes adherence to medical regimens such
as IT. This can be enhanced by regular phone
calls to the family to provide encouragement and
identify potential problems early.
The use of nursing clinic visits during IT can
contribute to the success of the program and
serves several purposes. Nursing visits may allow
for more flexible scheduling, minimizing disruption
to the family and promoting school and
work attendance. These visits allow for a review
Haematologica vol. 85(supplement to n. 10):October 2000