Coming of Age
A Need for
Change in
the Way
We Care for
People with
Sickle Cell
Disease
Recognizing the issues
facing individuals with sickle
cell disease (SCD) that are
discussed in this feature,
as well as other challenges,
the American Society
of Hematology (ASH)
is partnering with other
organizations in the SCD
community to launch the
Sickle Cell Disease Coalition
(scdcoalition.org) and a call
for action to improve the
state of SCD care.
On September 6, ASH
will release the “State of
Sickle Cell Disease: 2016
Report,” which evaluates the
disease in four priority areas
— access to care, training
and professional education,
research and clinical trials,
and global health – and
identifies opportunities for
researchers, clinicians, policymakers, and the private
sector to focus collective
efforts to change the way
we care for people with SCD
and improve the state of
SCD around the world.
Look for more about
this initiative in our October
issue.
56
ASH Clinical News
Boston, Massachusetts, patient care at her
institution typically transfers from the
pediatric to the adult system at age 22.
“In an ideal world, the transfer of care
would happen when a patient is ready
to move to the adult system,” Dr. Sobota
said. Unfortunately, at some institutions
there are many times when abrupt transfers happen – for example, if a patient
becomes pregnant or begins to exhibit
other “adult” behaviors, she said.
For some patients, this transfer comes
at a critical time in their illness. This can
be particularly challenging for patients
with SCD, Dr. Sobota noted. One study
found that SCD patients had the highest
rates of emergency department visits and
hospitalizations between the ages of 18
and 30 years.1
The neurocognitive issues patients
with SCD face can also make it difficult for them to manage this complex
disease. A study published in 2010 in
JAMA found that, compared with healthy
adults, adult patients with SCD had
poorer cognitive function performance,
working memory, and processing speed.2
“The disease really begins to worsen
during young adulthood, at a time when
we are expecting them to take on more
responsibility and transition from one
system to another,” Dr. Sobota said.
Defining an exact age when a patient
is ready to make the transition from
pediatric to adult care is impractical,
agreed Sumit Gupta, MD, PhD, assistant
professor in the department of pediatrics at The Hospital for Sick Children in
Toronto, Canada.
“Just as some toddlers learn to walk
and talk faster than other toddlers,
some adolescents learn to manage their
independence and health care better and
sooner than others,” he said. “To pretend
one 18 year old is as independent as another is silly. Our system is not really well
suited for gearing transition to stages of
readiness as opposed to chronologic age.”
Smoothing Abrupt Transitions
Many institutions likely do not have a
model in place to ease this transition for
patients, according to Dr. Bering.
“Unfortunately, [the transition] may
simply [involve] patients being told to go
see a certain person and hand over their
medical records,” she said.
For young adults who have survived
a childhood cancer, there are often two
options for future care, according to Dr.
Gupta. In some places there are specialized survivorship clinics geared toward
adolescents and young adults who survived these cancers and are now moving
into adulthood; in others, however, transitioning patients are transferred back to
the care of their primary-care physician.
Because adult childhood cancer
survivors are often at increased risk
for illness or premature death, they
require surveillance and monitoring
that primary-care physicians may not be
familiar with. In a survey of 2,000 general
internists’ attitudes and knowledge
about the care of these patients, more
than half reported caring for at least
one survivor, but 72 percent reported
having never received a treatment
summary of the patient’s disease. On
average, the physicians reported being
only “somewhat comfortable” caring
for survivors of common childhood
cancers, including Hodgkin lymphoma,
acute lymphocytic leukemia, and
osteosarcoma.3
“Just as some
toddlers learn
to walk and talk
faster than other
toddlers, some
adolescents learn
to manage their
independence
and health
care better and
sooner than
others.”
—SUMIT GUPTA, MD, PhD
“To expect providers to develop specialized knowledge based on only one to
three individuals is not feasible,” Dr. Gupta
said. “I think it is the system’s responsibility
to figure out how to support primary-care
providers and offer easy access to knowledge that will allow them to confidently
take care of these young adults.”
For patients who are still undergoing
maintenance therapy, Dr. Gupta said that
he is lucky enough to work with a nurse
practitioner from the lymphoma program at the adult center across the street
who is willing to come meet patients in
the pediatric clinic a few months before
they transfer to adult care.
“You can imagine that this is incredibly comforting for patients to know that
there is that overlap during the transition,” Dr. Gupta said.
Care of patients with chronic hematologic conditions typically is transferred
to an adult hematologist, and Dr. Sobota
said that there has been some success
with the idea of co-located pediatric and
adult care. At Boston Medical Center,
she holds her pediatric clinic in the adult
clinic once a month. That allows her
pediatric patients who are getting ready
to transition to visit and become familiar
with the adult clinic.
Fortunately, Dr. Sobota said, she
works at an institution that provides both
pediatric and adult care, but she knows
that this system is not available to all patients. To improve the transition process
for patients moving from one system to
another, more widespread change in incentives and payment structure will have
to occur. Physicians are not reimbursed
for making phone calls to past providers
or for taking the time to do transition
planning for their patients.
“As things move more toward the
accountable care organization model,
where providers and care teams are
reimbursed for quality of care and keeping patients out of the emergency room,
hopefully there will be incentives for
physicians to successfully transition these
types of patients,” Dr. Sobota said.
Until then, communication between
providers in different systems will be
important, Dr. Advani added, recalling
one case in which she reintroduced a
treatment to a patient in the adult setting
that had previously been used in the
pediatric setting. “It was reassuring to the
patient to know that I had talked with
the pediatric provider and that we had
developed a plan of care together,” Dr.
Advani said.
Dr. Sobota pointed out another
important challenge as these transition models continue to be developed:
evaluating their success. One small study
examining the transition of patients with
SCD showed that the majority of deaths
occurred after the transfer from a pediatric to an adult provider, with a mean
time to death after transfer of 1.8 years.4
“That study has been used to show
that appropriate transition to adult care
is necessary to avoid early mortality,” Dr.
Sobota said. “But is it fair to use early
mortality to show that transition is not
good? I don’t just want to prevent my
patients from dying – I want them alive,
thriving, and not in the hospital.”
Physician Preparedness
One barrier to successful care of patients
with hematologic conditions diagnosed
during childhood is a lack of adult providers who have familiarity with these
conditions which, until more recently,
often claimed the lives of these patients
before they reached adult care.
“Nationally, there is an incredible
lack of adult providers with an interest or expertise in conditions like SCD,”
said Dr. Sobota. “A lot of people who go
into adult hematology/oncology do it to
become an oncologist, and not all hematologists are interested in seeing a patient
with SCD. That has to change.”
Dr. Bering agreed, “When I was in
training, many of the people with these
September 2016