Pediatric Sickle Cell Program
For many years we at SUNY have advocated a comprehensive approach to health
education and treatment of children with sickle cell disease. Each year,
15-30 infants are newly diagnosed at our institution and introduced to
comprehensive care. By offering "well-child" care in addition to sickle
cell-specific counseling and education, we (contact
members of our staff) have succeeded in substantially improving the
health and survival of our children. Continued provision of comprehensive
sickle cell care in an era of managed care and "down-sizing" is a major
challenge to all urban health care providers.
SUNY-Brooklyn has participated in the Cooperative Study
of Sickle Cell Disease, a project designed to document the "natural history"
of sickle cell disease sponsored by the Heart,
Lung and Blood Institute
of the National Instates of Health, since
1978. A review of some of the goals and accomplishments
of the CSSCD has been distributed to parents and participants, and numerous
important publications have resulted from the
efforts of all involved. Recently, CSSCD investigators have reported that
manifestations of sickle cell disease may be used to predict severe complications
later in childhood. It is hoped that awareness of these factors
will allow doctors to determine which children may most appropriately be
considered for, or excluded from, a number of recently available, but potentially
dangerous, treatments. In the past, SUNY researchers have contributed
to a number of publications related to sickle cell disease; Dr.'s Margaret
Robinson and Janet Watson were the first to describe the increased susceptibility
of individuals with sickle cell disease to pneumococcal meningitis. Click
for a list of
publications by current faculty.
We were one of 14 institutions to participate in The
Stroke Prevention Trial (STOP) sponsored by the Medical College of
Ga. at Augusta. The study was stopped early, on September 2, 1997, when
it became clear that regular
blood transfusion does
stroke in children with sickle cell anemia
found to be at high risk due to an abnormal transcranial doppler (TCD)
study. Details are provided in a news
release and Clinical
Alert from the Heart, Lung and Blood Institute of the NIH. It is important
to understand that at present TCD must be performed at a site qualified
to do the Doppler studies as they were done for STOP, and that physicians
familiar with STOP results be involved in treatment discussions and recommendations.
All of the STOP centers, including our own, are able to assist and advise
and are listed in the links above. There is optimism that a second STOP
study is imminent, which will determine whether or not transfusion therapy
might safely be stopped in some children who achieve normal TCD studies
while receiving transfusions. The NIH has posted some guidelines
for transfusion and a brief review of risks which may be of interest.
For over 20 years, the
Center has been screening
all newborns in New York State for sickle cell disease; newborn screening
results are available to licensed physicians in NY State by calling 1-800-535-3079.
Information about various abnormal hemoglobins encountered can be accessed
at the Globin Gene Server Homepage.
See also the Human
Genome Project for information about sickle cell disease, updates as
to genome mapping progress, and ethical considerations related to genetic
Another important resource supported by the NY State Department
of Health is the NY State Sickle Cell Advisory Committee,
which works in many ways to address concerns of the sickle cell community.
It is a standing committee of GENES, the Genetic Network for New York State,
Puerto Rico, and the Virgin Islands, a federally designated region that
is funded by the federal government to coordinate genetic services and
provide education about genetic disorders to medical professionals and
the lay population. GENES is one of ten regions represented by CORN (the
Council of Regional Networks for Genetics Services).
An excellent reference as to care of children with sickle
cell disease is available to review or download (Sickle
Cell Disease (Clinical Guidelines) NIH, MCHB); additional Guidelines
related to other issues, including pain management, are also available.
An updated Black Book, Management and Therapy of Sickle Cell Disease,
was published (in orange and purple),
but additional printings are on hold. A modified, maneuverable text version
is available here, and a PDF or text download
is offered by the NIH/NHLBI.
Finally, there is an online summary of the medical needs of infants with
sickle cell disease, Consumer
Guide 6: Sickle Cell Disease, that can be read by parents. For additional
information about the Black Book or other sickle cell information call
the sickle cell branch of the NHLBI by Comprehensive
Sickle Cell Centers phone at 301-435-0055, send email
or try the developing Sickle Cell Centers web site.
New York state is in the process of requiring that all
Medicaid recipients enroll with a managed care organization (MCO) to continue
to receive benefits; if families don't pick a provider within 60 days of
being requested to do so, an MCO will be assigned by the State.
It is important that parents of children with sickle cell disease or other
chronic medical conditions be certain that managed care providers they
are considering can provided the kind of sickle cell care they expect and
require. The Childrens Hospital at Oakland as posted some recommendations
regarding choosing a provider. As we become progressively challenged
by bureaucratic demands, it may be helpful to healthcare providers to have
access to ICD-9-CM International (Disease) Codes;
sickle cell anemia is 282.6. Thanks to the anonymous provider.
Information related to new treatments for sickle cell disease is also available
Pain and acute
chest syndrome remain the most common complication of sickle cell disease.
Hydroxyurea was effective in reducing both complications in adults with
moderate to severe sickle cell anemia. Questions
and answers about the Multicenter
Trial have been posted by the NIH/NHLBI. We at SUNY currently
offer hydroxyurea therapy to selected children with severe disease manifestations,
and although we are concerned about long-term risks of treatment, recent
studies suggest safety and efficacy in children. A trial of hydroxyurea
in infants with sickle cell disease has been proposed to determine
whether early implementation of therapy might prevent damage to brain,
spleen, lungs, kidney and other organs which are early targets of sickle
cells. The NIH/NHLBI has also suggested guidelines
for use of hydroxyurea in children.
Bone marrow transplantation can cure
sickle cell disease (NEJM
article), but is associated with substantial risk and is still experimental.
Children who have suffered severe complications and who have a compatible
sibling donor might consider transplant. An exciting new alternative source
of stem cells for potential transplantation is umbilical cord (placental)
blood. Cord blood transplantation may be associated with lower risks of
complications, and potentially will increase the possibility of finding
a donor for an unrelated stem cell transplant; the NIH
has recently supported several programs to routinely store cord blood.
Mothers of children with sickle cell disease who are pregnant may want
to discuss with their physician the possibility of storing cord (placental)
blood at the time of delivery; the Childrens
Hospital of Oakland is providing this service free to eligible, expectant
mothers anywhere in the country who already have a child with sickle cell
disease or other transplant-treatable condition.
While we do not advocate homeopathic alternatives to standard care,
healthy life choices are important for individuals with sickle cell disease,
and a wealth of information is available via Healthy.Net
Home Page .
Joan Fleitas, Assistant Professor of Nursing at Fairfield University, has
developed a page that will amuse and inform children, adults, parents and
providers interested in sickle cell disease and other chronic conditions
and Blackboards. Ivor and Deborah Pannell are friends of the SUNY program
who are founders of S.C.A.R.E. (Sickle Cell Advocates for Research and
Empowerment) and its web page at defiers.com.
The page is an extremely comprehensive resource for those interested in
sickle cell disease and recently has been expanded to include access to
a sickle cell chat room and message
board. Mr. Platt at Emory has expanded their sickle
cell web page, and we are soliciting ideas as to how to best
utilize sickle cell.org.
The availability of beneficial but potentially dangerous therapies for
sickle cell disease raises complicated ethical issues. An excellent series
of review articles on several bioethical topics is provided on-line by
the Canadian Medical
Association Journal (link updated 9/98), and additional
information and resources are available from the
Medical College of Wisconsin.
This page is referenced in PedInfo
an enlarging database for pediatricians, geneticists and others. Pediatric
hematologists should be aware of the web site by the American
Society of Pediatric Hematology/Oncology, the American
Society of Hematology, and Bloodline
for meeting abstracts and relevant links. And you know about the London
New York Times.
Finally, years ago Brooklyn baseball fans were devastated by the loss of
the Dodgers. Though they
now have the Cyclones, a long-suffering
Indians fan (didn't it take the NY Rangers 49 years to win their most
recent World Championship?) suggests Brooklynites might want to adopt the
Tribe, particularly given their recent success. Or, perhaps, if you like
to suffer, you may adopt the new Browns!
My Dad's old paper, the Plain Dealer
is also finally online, for all displaced Clevelanders.
This page was posted 3/17/96 and last updated
9/27/2001. Please send comments and greetings to Scott Miller MD at firstname.lastname@example.org.
Links provided were chosen as likely to be of interest to visitors to this
page, but their listing does not necessarily imply endorsement of their
content by Dr. Miller, SUNY, or any other entity or individual.
Copyright 1996, 1997, 1998 by Scott T. Miller