The transition and transfer of patients with hematologic conditions from pediatric to adult care often comes at a crucial time.
The years spent transitioning from adolescence to adulthood are flush with emotional, financial, and social challenges, but for young adults who are also dealing with a hematologic malignancy or chronic condition, these transitional years have many added layers of complexity.
As adolescent patients move into adulthood, they are often moving from a state of dependence to independence – like moving from high school to college or from their parents’ house to their own apartment – but that also means suddenly being responsible for their own medical appointments, medications, and health-care insurance.
“For patients with a chronic illness, transitioning care from a pediatric to adult setting can be a really difficult time,” said Harriet Bering, MD, site medical director of Harvard Vanguard Medical Associates, an affiliate of Atrius Health, in Cambridge, Massachusetts. “Many patients are very attached to their pediatric providers. Getting to know a new team after having a long illness can be difficult – especially during this critical time when people are going through the maturing process with all the different emotional issues related to it.”
A variety of hematologic conditions can be diagnosed during childhood or adolescence (between the ages of 10-19 years, according to the World Health Organization’s definition). These conditions can range from childhood leukemias or iron deficiencies to even rarer conditions like hemophilia or sickle cell disease (SCD), which require long-term, complex, and specialized care.
ASH Clinical News spoke with several physicians about transitioning young adult patients to the adult system and what can be done to make this transition smooth and successful for the patient, their caregivers, and their health-care team.
Is Age Just a Number?
In most states, the legal age of adulthood is 18 years, though the same may not always be true when it comes to health care.
“There is really no clear age of transition, and the age seems to differ according to where the patient is being treated,” said Anjali Advani, MD, a staff physician in the Leukemia Program, Department of Hematologic Oncology and Blood Disorders and director of inpatient leukemia at Cleveland Clinic in Cleveland, Ohio. “Here at Cleveland Clinic, we tend to see many patients that are 18 and older, but I know of other places with pediatric programs where they will sometimes see patients into their 30s.”
According to Amy E. Sobota, MD, MPH, a pediatric hematologist/oncologist at Boston Medical Center in 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
“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.”
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 chronic hematologic conditions did not live to adulthood, but now they do. Educating adult providers about these childhood conditions is essential to successful transfer of care.”
In an effort to help health-care professionals ease patients’ transition from pediatric to adult care, the American Society of Hematology (ASH) recently released a toolkit that contains general resources for supporting quality care for all hematologic conditions, as well as for patients with hemophilia and SCD.
The toolkit was developed to provide guidance and tools for both primary-care internal medicine and subspecialty practices that treat patients transitioning from pediatric to adult care. The ASH toolkit includes transition readiness assessments and clinical summary forms for three segments: (1) patients with any hematologic condition, with an addendum that includes links to additional condition-specific guidelines and resources; (2) patients with hemophilia; and (3) patients with SCD. (See the SIDEBAR for more information about the toolkit and where to access it.)
“Adult providers must understand this transition and be flexible with younger patients,” Dr. Gupta said. “Adult practitioners cannot expect to be able to treat an 18 or 19 year old like they would a 35 or 50 year old. For these patients, the transition continues to happen even once they are in the adult care setting.”
Physicians are not the only ones that need assistance in preparing for this transition. Patients must also learn about the health-care system they are entering and be encouraged to act as their own advocates.
“Knowledge is important. These patients can’t advocate for themselves if they don’t know what they are advocating for,” Dr. Gupta said.
To help assess their readiness for the transition, Dr. Gupta said he starts asking patients more questions about their condition when they are a couple of years away from the 18-year-old transfer cut-off point.
“I ask them what cancer they have, what they have been treated with, and what that puts them at risk for in the future,” he explained. “A large number of them cannot answer these questions, which is not surprising because their parents have been their primary caregivers. The first step for these patients to successfully transition is to have adequate knowledge about their history and the challenges they might face in the future.”
A joint algorithm released by the American Academy of Pediatrics, American Academy of Family Physicians, and the American College of Physicians suggested familiarizing patients with the idea of transition at around 12 to 13 years of age, which is considered an important development time.5 These discussions should introduce patients to the adult health-care system, covering topics such as how to call for an appointment, showing up early for appointments, arranging transportation to appointments, and how to handle unexpected health emergencies.
“You have to start the discussion early,” Dr. Sobota said. “For many teenage patients, it never occurs to them that they will have to leave pediatric care.”
For patients with SCD, the transition to adult care may also come with social stigmatization. Young adults with SCD are often labeled as “drug-seekers” when they request specific medications to help manage their pain, which can sometimes lead them to avoid seeking medical care. Dr. Sobota recalled watching a video from Johns Hopkins, titled “CRISIS: Experiences of People with Sickle Cell Disease Seeking Health Care for Pain,” in which an adult woman with SCD explained what she had to go through to get medical care.6 “She makes sure she is professionally dressed and that her hair looks good before going to the emergency room so that she won’t be thought of as a drug-seeker,” she said.
Dr. Sobota tries to prepare her patients for the realities of having a condition that many physicians do not know much about. Patients have to be empowered to advocate for themselves, she said, adding that she recommends that patients have a medical summary with them whenever they seek care.
Transitioning from a pediatric patient to an adult living with a hematologic condition and being treated in the adult health-care system demands that patients be more proactive and feel confident enough to speak up and ask questions, Dr. Advani said.
“There are resources for young adults available within the adult health-care system, but patients may have to ask about them specifically if they want information, for example, about fertility or sexual function,” Dr. Advani said.
During this time, adult providers should be prepared for a patient’s family to remain active participants in the patient’s health care. Although she has seen some patients who lack social support in the adult clinic, Dr. Advani said there are often others whose parents are very involved, even if the patients are living on their own or married.
Dr. Gupta added that the transition to adult care is often as much about the patient’s family or caregivers as it is the patient.
“It is hard for family members who have spent five, 10, or 15 years taking care of a child and being in charge of the medical decision making to transition out of that role as the patient achieves greater independence,” he said. “The young patient will never be ready if the parent is still answering all the questions or taking responsibility for making appointments or getting medications.” —By Leah Lawrence
- Brousseau DC, Owens OL, Mosso AL, et al. Acute care utilization and rehospitalization for sickle cell disease. JAMA. 2010;303:1288-94.
- Vichinsky EP, Neumayr LD, Gold JI, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2012;303:1823-31.
- Suh E, Daugherty CK, Wroblewski K, et al. General internists’ preferences and knowledge about the care of adult survivors of childhood cancer. A cross-sectional study. Ann Intern Med. 2014;160:11-7.
- DeBaun MR, Telfair J. Transition and sickle cell disease. Pediatrics. 2012;130:926-935.
- American Academy of Pediatrics. Clinical Report —Supporting the health care transition from adolescence to adulthood in medical home. Pediatrics. 2011;128:182-200.
- Johns Hopkins University, “CRISIS: Experiences of People with Sickle Cell Disease Seeking Health Care for Pain.” Accessed July 26, 2016 from https://www.youtube.com/watch?v=FuelQDBOxXI.
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 the October issue.
The American Society of Hematology (ASH) recently launched a toolkit designed to help health-care professionals transition young adults with hematologic conditions into the adult care system. The toolkit provides both an assessment and summary form that are designed to facilitate conversations between patients, parents, and physicians as the patient transitions to adult care.
All forms in the toolkit were developed as part of a broader initiative led by the American College of Physicians (ACP) and included several member organizations from the ACP Council of Subspecialties (CSS), including ASH. A broad ACP work group designed the general form templates and the Society worked to customize the forms for hematology. The tools were reviewed by both pediatric and adult hematologists and ASH’s Committees on Practice and Quality, as well as the Society’s Officers before being reviewed by the American College of Physician’s Steering Committee and the American Academy of Pediatrics. In the future, ASH hopes to expand on these tools and develop additional toolkits for other hematologic conditions.
Readiness Assessment Forms
The toolkit includes general hematology, hemophilia, and sickle cell disease (SCD) transition readiness assessment forms for the patient to complete. The forms were designed to assess patients’ readiness for transition by testing their knowledge and skills related to their condition and its management. For adolescents about to make the transition, this form can be used as a way to figure out what additional education needs the patient has before they can be successfully transitioned. For adult patients, the form can assess any remaining gaps in self-care knowledge and skills.
Clinical Summary Forms
The toolkit also includes clinical summary forms for general hematology, hemophilia, and SCD that provide a medical record summary, including essential clinical information related to the hematologic condition. The forms should be completed, signed, and dated on the last page by the referring provider and patient/family, reviewed by the patient, and given to the new adult provider.
More information on the toolkit is available at hematology.org/transitions.