Sickle Cell Program provides comprehensive
care
Working in partnership with primary
care providers
Advances in preventative care and new medications have reduced
the life-threatening complications of sickle cell disease, and
many individuals can now live into their mid-40s and beyond. However,
sickle cell disease is still severe, chronic and sometimes fatal.
In October, the Childrens Hospital Boston division of Hematology
recruited Matthew Heeney, MD, to direct the hospitals
Sickle Cell Program. The addition will allow the program to see
more patients not only under urgent circumstances, but also for
routine care, a goal that Dr. Heeney says is crucial to meeting
the needs of this underserved patient population.
Sickle cell disease can be deceptive in that patients can
be relatively healthy for years before there are serious complications,
says Dr. Heeney. For that reason, many primary care practitioners
dont refer patients to our program soon enough. My goal
is to encourage pediatricians and other care providers to refer
patients for consultation and expert patient education at least
once a year, regardless of their condition.
Dr. Heeney says he was drawn to the Childrens programwhich
currently serves about 185 patients from infancy through young
adulthoodbecause it offers cutting-edge screening, therapy
and treatment in a field that is constantly changing. The program
offers genetic counseling to families and helps them to manage
the complications of sickle cell disease, which include pain,
stroke risk, acute chest syndrome and life-threatening infection.
The program hopes to partner with primary care providers to manage
the disease, not to take over the community physicians role.
Ellis
Neufeld, MD, director of Clinical Hematology and the Boston
Center for Genetic Blood Diseases, is a national advocate of the
medical home concept, and says the physician-specialist
partnership is particularly important because most doctors see
very few sickle cell patients. When you have only one or
two sickle cell patients, its difficult to have a full view
of what this disease can be like and what patients need to know,
says Dr. Neufeld. Early referral for comprehensive assessment
and expert education is the best way to prevent long-term complications.
Using advanced assessment tools, Childrens hematologists
can gauge each sickle cell patients risk of stroke and pursue
the appropriate level of treatment. Treatment options range from
health maintenance through pain medication, blood transfusions
and penicillin, to the use of new drugs such as hydroxyurea (which
may help reduce the frequency of pain crises and acute chest syndrome,
as well as the need for frequent blood transfusions). Bone marrow
transplantation is an option for the most advanced cases.