feature
Interview
The Road to the New Sickle
Cell Disease Guidelines:
Interview with Co-Chair
George R. Buchanan, MD
On September 9, 2014, the National Heart, Lung, and
Blood Institute (NHLBI) released an evidence-based expert
panel report on the management of sickle cell disease
(SCD) — the first-ever comprehensive guidelines providing
guidance for all clinicians who care for patients with SCD,
from primary-care physicians to specialists.
To get the behind-the-scenes look at how the guidelines
went from plan to conception to reality, ASH Clinical
News spoke with George R. Buchanan, MD, professor of
pediatrics and internal medicine at University of Texas
Southwestern Medical Center in Dallas, and co-chair of the
Expert Panel.
Based on its overall
merits and potential to
improve care — while still
recognizing the limitations
of currently available
evidence and need for more
research — ASH decided
to endorse the new NHLBI
report. Accordingly, the
Society is working to ensure
that clinicians understand
how to implement these
new recommendations.
Read more about the
Society’s efforts in ASH
Directions, including
new pocket guides and
a stakeholders meeting
planned to take place at
ASH Headquarters early
next year, on page 13.
ASHClinicalNews.org
ASH Clinical News: Let’s
first talk about the need for
this type of guideline — was
there anything like this
before now?
NHLBI had previously published
some recommendations over
the years, often referred to as
“The Black Book” or “The Red
Book,” which contained expertwritten chapters regarding
various aspects of SCD and
disease management, but these
were not evidence-based. There
had also been comprehensive
systematic literature reviews of
hydroxyurea — one of the two
disease-modifying treatments
for SCD — published in 2008,
but, to my knowledge, no
systematic, comprehensive,
evidence-based reviews
regarding other treatments and
management issues regarding
SCD have existed prior to the
document we developed.
Who is the target audience
for this report?
These new guidelines are mainly
designed for the frontline
primary-care physicians,
general practitioners, family
physicians, pediatricians, general
internists, and other specialists
or subspecialists who deal with
sickle cell patients — rather
than hematologists who have
historically been more involved
with managing SCD.
Hematologists who frequently
encounter SCD may not really
“need” these guidelines, at least not
as much as the other physicians
who are less involved with the
disease. However, hematologists
and oncologists who may be less
directly involved with SCD may
also be called upon to help with
managing SCD, so, hopefully, all
ASH members will derive some
benefit from the publication of
these guidelines. And, there is
no question that hematologists
are very keen on having these
comprehensive evidence-based
recommendations compiled in one
document.
How long did it take to
develop the report?
This process began in 2009,
when Susan Shurin, MD, then
the deputy director of NHLBI,
asked me to co-chair this report.
Our first face-to-face meeting
occurred in early 2009, with the
initial thinking that the report
would be complete in a couple of
years.
There were a couple of major
reasons behind the delay. First,
there was initially some turnover
in the membership of the
committee, and then a change
in the methodology group who
assisted the writing panel with
literature review and grading of
the evidence.
Second, this set of guidelines
was fundamentally different
from any others that NHLBI
has developed. Typically,
NHLBI guidelines deal with the
management of one aspect of a
disease, but our task here was to
develop the recommendations for
an entire disease — meaning every
organ, every part of the patient’s
body, and managing all of the
unique complications from SCD.
During the process, we also
stopped temporarily to allow
for public comments and input
from ASH and other professional
societies, which offered many
helpful recommendations.
We did make a fair number of
modifications as a result of those
comments.
How did the expert panel
begin the process of
making recommendations
and grading supporting
evidence?
In the early meetings, we
developed our major strategy
and the overarching questions we
needed to answer in five major
topic areas:
• Ongoing health maintenance
(i.e., necessary monitoring and
laboratory testing)
• Managing acute complications
• Managing chronic
complications
• Hydroxyurea therapy
• Blood transfusion
“ ur task was
O
to develop
recommendations for an
entire disease
— meaning
managing all
of the unique
complications
from SCD.”
After identifying those areas and
specific questions we wished to
answer, we got involved with
the methodologists who actually
performed the systematic review
of the literature. Around 12,000
publications were reviewed,
dating back from 1970 to April
2014, to make sure we didn’t
miss any pertinent information.
Then, the promising data from
each paper or manuscript
were assessed according to the
GRADE system and entered into
“evidence tables.” The GRADE
system had to be modified
because it only allowed for two
Continued on page 44
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