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Lost in transition: Care for adults with sickle cell disease ‘complex puzzle’
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Sickle cell disease once was considered a childhood disorder.
Thirty years ago, fewer than half of children with the condition were expected to survive into adulthood.
Today, because of earlier diagnosis and more effective treatments, nearly 86% of all patients — and more than 97% of those with mild forms of the disease — live until aged at least 18 years.
The improved survival rates create their own challenges, however.
Patients with sickle cell disease who transition from childhood to adulthood continue to develop physical complications, such as acute chest syndrome, infections or pain.
There also are significant societal implications.
A lack of comprehensive care programs for adults with sickle cell disease suggests that these patients often are forced to rely on EDs to manage acute events. Their hospitalization rates also exceed those of the general population, resulting in significantly higher costs.
Janis L. Abkowitz
“These issues can snowball, and they can create a very difficult and complex puzzle,” Janis L. Abkowitz, MD, president of ASH and hematology division head at the University of Washington School of Medicine in Seattle, told HemOnc Today.
Several studies presented at the ASH Annual Meeting in December suggest there are gaps in the continuity of care for patients as they transition from pediatric to adult care.
“While we have made many advances in the treatment of sickle cell disease, this research reveals the important challenge we as physicians continue to face in ensuring that the medical system supports timely access to needed preventive and disease management protocols for our patients,” Abkowitz said. “For health care providers, it is important to consider how we can address the needs of patients transitioning into adulthood. [These efforts] will improve the health care system for everyone.”
Cracks in the system
All babies born in the United States are screened for sickle cell disease. The inherited chronic blood disorder affects an estimated 100,000 Americans.
The condition, particularly common among those whose ancestors came from sub-Saharan Africa or Spanish-speaking regions in the Western Hemisphere, causes a mutation in the hemoglobin gene, resulting in the distortion of red blood cells into a sickle — or crescent — shape. This decreases the cells’ flexibility and can lead to a variety of complications, including anemia and pulmonary hypertension.
Most academic medical centers develop tightly structured approaches to care for children with sickle cell disease. Comprehensive teams often include pediatric and adult hematologists, research nurses, case workers, social workers, specialized nurses and psychologists.
“When it is time to transition patients to the adult clinic, many medical centers do not have a comprehensive approach,” Kenneth I. Ataga, MBBS, associate professor in the division of hematology and oncology and director of the sickle cell program at the University of North Carolina School of Medicine, said in an interview. “Patients are often referred to hematologists or hematologist/oncologists who may not have a specific interest in sickle cell disease.”
Patients typically transition from pediatric to adult care when aged 18 to 21 years. The timing can depend on several factors, such as physical well-being, maturity and health insurance coverage.
Few medical centers offer formal transition assistance. Without the built-in support network, patients are forced to schedule appointments, adhere to medication schedules and manage other aspects of care on their own.
“The increased responsibility can become a real shock to patients when they move to adult care,” Ataga said. “They can easily slip through the cracks at this stage and not receive the required medical care.”
Lack of providers
Adolescents and adults with sickle cell disease often struggle to identify local providers who are willing and capable to treat them.
Jeffrey D. Lebensburger
In a 2012 literature review published in the Journal of Blood Medicine, Jeffrey D. Lebensburger, DO, assistant professor of pediatric hematology and oncology at the University of Alabama Birmingham, and colleagues examined the barriers patients with sickle cell anemia — the most severe form of sickle cell disease — face when they transition from pediatric to adult care.
A survey of leading pediatric sickle cell centers found the most difficult aspect of the transition was identifying qualified adult providers. Only 60% of responding centers indicated they could refer patients to adult hematologists who specialize in sickle cell anemia, and the rate was much lower in rural areas.
“Without local providers with expertise in sickle cell anemia, many patients are transferred to a general internist for their adult care,” Lebensburger and colleagues wrote. “Unfortunately, one-third of general internists surveyed express comfort in being the primary care provider for [these] patients. In addition, the majority of internists believed that they should not assume primary care responsibility for this patient population.”
Utilization of care
The lack of established transition programs may explain why patients in the post-transition period utilize ED care at much higher rates than pediatric patients.
George S. Rust, MD, MPH, a professor of family medicine and director of the National Center for Primary Care at Morehouse School of Medicine in Atlanta, and colleagues examined the rates of hospitalizations, outpatient visits and ED visits among 19,168 children and adults with sickle cell disease in 14 states.
All patients were enrolled in Medicaid, with eligibility for 2006 and 2007. The mean age of the patients was 22.06 years.
The researchers — who presented their findings at ASH — reported 94,891 outpatient visits, 61,762 hospitalizations and 146,163 ED visits within the 2-year study period.
Utilization of care changed significantly as children transitioned into adulthood, Rust and colleagues found. Outpatient visits decreased, whereas ED visits and hospitalizations increased and peaked in young adults.
Patients aged 21 to 30 years accounted for the most ED visits (n=45,820; 31.3% of total) and hospitalizations (n=17,735; 28.7% of total) of any age group (P=.0001). Patients aged 11 to 20 years accounted for the second-highest rates of ED visits and hospitalizations, followed by patients aged 31 to 40 years.
Patients aged 10 years or younger accounted for the most outpatient visits (n=30,193; 31.8% of total). The frequency of outpatient visits declined with age, with patients aged older than 50 years accounting for the fewest percentage of visits (n=4,184; 4.4% of total).
“There is clearly a problem in the child-to-adult care transition of patients with sickle cell disease,” Rust told HemOnc Today. “The dramatic rise in emergency department use relative to other care during the teen and young adult years — despite being insured by Medicaid — suggests a significant opportunity for care coordination to assure that each patient finds an effective medical home for ongoing care.”
Age-dependent changes
Morey A. Blinder, MD, associate professor in the division of hematology at Washington University in St. Louis, and colleagues investigated age-related patterns of ED reliance among patients with sickle cell disease. They also calculated associated health care costs.
Blinder and colleagues reviewed Medicaid data from 3,208 patients in five states. Evaluable patients included those who were transitioning from pediatric to adult care.
The results, presented at ASH, showed the average number of ED visits per quarter increased from 0.76 to 2.29 from aged 15 to 24 years. The rate peaked at 2.9 per quarter at age 36 years.
Regression analysis showed patients who were in the post-transition period — defined as aged older than 18 years — were more likely to have high ED reliance, defined as more than 0.33 visits per quarter (P<.001).
“There is an age-dependent change in medical care where the patient relies on the emergency department for their care as opposed to other outpatient settings, such as PCPs or clinics,” Blinder said in an interview. “On average, the number reached approximately one emergency room visit per patient, per month. That struck me as being the most dramatic finding.”
Patients with high ED reliance incurred significantly higher ED and inpatient costs than those with low reliance, Blinder and colleagues found.
Study results showed an unadjusted mean quarterly cost difference of $7,376 (P<.001) between patients with high and low ED reliance.
The findings highlight “the need to improve transition-related support, including better access to primary care and increased engagement with sickle cell disease patients,” Blinder and colleagues concluded.
Rehospitalization rates
Thirty-day rehospitalization has become an established clinical indicator of the quality of care patients receive for a specific disease.
A retrospective cohort study by Brousseau and colleagues published in 2010 in the Journal of the American Medical Association found an estimated 41% of patients with sickle cell disease aged 18 to 30 years who were hospitalized in acute care were re-admitted to the hospital within 30 days.
That rate was higher than for any other disease, including diabetes (20%) and heart failure (16%).
Some researchers use large administrative databases to broadly examine health care utilization. However, administrative databases may misrepresent clinical care due to coding errors, according to Hung Tran, MD, a third-year resident in pediatrics at the University of California at Irvine, and colleagues.
Tran and colleagues conducted a retrospective chart review to assess acute health care utilization in one community-based health care system that offers comprehensive sickle cell disease care for patients aged 21 years or younger, but does not have a dedicated adult clinic.
Amanda M. Termuhlen
“The long-term question is, can we establish an outpatient medical home and a transition program for patients that could decrease utilization of inpatient hospital stays and ED visits,” researcher Amanda M. Termuhlen, MD, medical director of the Jonathan Jaques Children’s Cancer Center at Miller Children’s Hospital Long Beach, and professor of clinical pediatrics at Keck School of Medicine at the University of Southern California, told HemOnc Today.
To assess clinical care, the researchers used discharge codes to identify patients with sickle cell disease who had been admitted to Miller Children’s Hospital and Long Beach Memorial Medical Center from 2009 to 2010. The investigators then examined 14- and 30-day readmission and ED visits in that patient population.
Tran and colleagues identified 174 unique patients (median age, 15 years; range, 7 days to 65 years). They accounted for 563 acute care encounters, defined as readmissions plus ED visits, during the 2-year study period.
The study results, presented at ASH, showed patients aged 21 to 30 years were most likely to require 14- and 30-day readmission.
Forty-one percent of patients in that age group required a 14-day readmission, nearly six times the rate required by patients aged 10 years or younger, and about four times the rate required by patients aged 11 to 20 years.
Researchers said 51.3% of patients aged 21 to 30 years required a readmission within 30 days, about 3.5 times the rate of those aged 10 years or younger, and three times more than patients aged 11 to 20 years.
The median number of acute care encounters within 30 days of discharge was 2.41 for patients aged 21 to 30 years compared with 1.62 for the entire patient population, study results showed.
Of the patients who had 11 or more acute care encounters in the 2-year study period, 67% were aged 21 to 30 years, researchers found.
The findings confirmed those of previous database studies that suggest, among patients with sickle cell disease, those aged 21 to 30 years account for the highest rates of hospital readmissions and acute care encounters. Further investigation into the explanations for those higher rates may contribute to decreased acute health care utilization among that patient population, the researchers concluded.
“The number of patients actually able to go home after emergency room treatment was very small,” Termuhlen said. “That was very surprising. Are patients being discharged appropriately? Do they have that support to stay out of the hospital once they are home? If they do visit the emergency room, what can we do to actually get them home rather than readmitted again?”
Role of Medicaid
Over-reliance on EDs for sickle cell care is not the way to achieve the best and most effective outcomes, Rust said.
Medicaid, however, may be an important tool, “using emergency visit claims as both a surveillance tool for adverse events and a rapid-cycle feedback loop for providers whose patients are experiencing suboptimal outcomes,” he said.
The Affordable Care Act will reduce barriers to coverage by eliminating the categorical eligibility requirements in Medicaid, Rust said. If states embrace Medicaid expansion, a teen with sickle cell disease who qualifies for Medicaid based on income at age 17 years still will be eligible at age 22 years.
“Medicaid is an important source of publicly funded coverage for patients with sickle cell disease, so we all have a stake in helping these patients achieve the best possible outcomes through high-quality care in the most appropriate settings,” Rust said. “State Medicaid programs are working at varying degrees of success to move from paying bills to medical providers to actually being responsible for Medicaid population health outcomes. Nowhere is the opportunity more obvious than in this group of … sickle cell patients.”
A lack of plans
The three studies presented at ASH focused on the economic consequences of gaps within the continuum of care for young patients with sickle cell disease.
“The economic argument is critical, but are we doing a good enough job of delivering care to these young patients with chronic and debilitating illnesses? That question is even more paramount,” Abkowitz said.
The challenges caused by the lack of care options for older patients with sickle cell disease are compounded when children and adolescents do not receive sufficient guidance for managing their own care in adulthood.
When Lebensburger and colleagues conducted their literature review in 2012, one study focused on sickle cell programs at three pediatric centers in a large Midwestern city. None of the programs implemented a formalized transition of care plan.
Eleven of 18 patients interviewed in that study did not receive transition preparation, and the seven other patients had limited preparation that included a one-time call to an adult care provider, according to the study.
One survey of 30 major sickle cell centers across the country showed that 72% arranged for planned transition discussions with adult care providers. Another survey showed 67% of providers offered at least one mechanism to demonstrate transition, with most transition plans coordinated by nurse practitioners.
However, outcomes assessment measures were lacking, Lebensburger and colleagues found. Forty-six percent of centers did not evaluate their transition programs, and 61% did not assess patient satisfaction with transition planning, results showed.
“For those centers that have a defined transition program, an important component of their plan should include an outcomes assessment measure,” Lebensburger and colleagues wrote.
A smooth transition
Transition support services are routinely offered to young adults and adolescents with congenital, genetic and pediatric-onset disorders, such as cystic fibrosis, spina bifida and heart disease.
As patients with sickle cell disease live longer, such programs will become essential to ensure they benefit from continuity of care, clinicians said.
The three primary elements of a formal transition-of-care plan are transition education, efforts to understand and address social issues, and access to adult-care providers, Lebensburger said.
Limited literature exists regarding how — and when — physicians can appropriately engage transitioning patients. Future studies must focus on the development of educational programs that will encourage patient participation in the transition process, Lebensburger said.
“Transition education cannot focus solely on teaching a child his medical information and providing the name of an adult provider,” Lebensburger said. “Transition education must address patients’ fears about transition, educate them about overcoming social barriers experienced by adults with chronic diseases, and develop strategies to manage the adult health care system.”
Pediatric providers can identify barriers that are unique to their programs through interviews or surveys with former patients.
“Once barriers have been identified, transition education programs can be developed that accurately addresses issues within their population,” Lebensburger said.
Comprehensive disease management
Miller Children’s Hospital hired a social worker to help coordinate transitions of pediatric patients in several target populations, including those with sickle cell disease.
“We realized many of the issues concerning transition were related to education, vocation and how to navigate the insurance side of care,” Termuhlen said. “Physicians do not necessarily have the expertise in those areas. Social work is the ideal field to fill that piece of the puzzle.”
Young patients with sickle cell disease frequently encounter obstacles related to insurance, such as difficulty paying for their own coverage or finding providers who accept the insurance plans they can afford. Social workers have experience connecting with insurance companies and identifying the barriers specific to each patient, Termuhlen said.
Transition planning begins in the patients’ early adolescent years.
“Every year the patients come to see us, we’ll build on teaching them about their disease,” Termuhlen said. “They’ll learn how to advocate for themselves and their own health care needs. They will learn how to navigate their insurance when they turn 18.
“It will help them manage their disease in a much more comprehensive way,” she added. “In a few years, we will redo our initial analysis and examine the impact a comprehensive transition program had on this patient population in the community setting.”
Tailored treatment
Clinicians at St. Jude Children’s Research Hospital in Memphis, Tenn., also recognized the growing need to help adolescents with sickle cell disease identify adult care providers in their area and learn how to navigate the health care system on their own.
“We established our program for the same reasons other programs are popping up,” Jane S. Hankins, MD, MS, associate faculty member in the department of hematology at St. Jude, told HemOnc Today. “It hits close to home when you know someone for 18 years, and then 3 years later they’ve passed away.”
Jane S. Hankins, MD, MS, and colleagues at St. Jude Children’s Research Hospital launched a program in 2008 to help adolescents with sickle cell disease transition to adult care. Since then, the percentage of patients who establish care with an adult provider within 3 months of leaving pediatric care increased from 33% to 75%.
Source: Photo courtesy of Jane S. Hankins, MD, MS.
In 2008, Hankins and colleagues developed a program that focused on teenagers who were approaching the age when they should transition to adult care.
Patients begin the program at age 12 years, and clinicians see patients at least once every 6 months.
In the beginning, members of a transitional team — which includes a social worker, physician assistant, academic coordinator, psychologist and nurses — educate patients and their families through literature, games and videos.
During the preparation period, the team works to tailor treatment options that address the needs of patients beyond medical care, such as insurance issues, lack of follow-up adult care and family concerns.
When patients reach age 15 years, parents are asked to stay out of the room for most of their visits so that teenagers get accustomed to answering questions on their own. Nurses also teach patients how to complete personal health records.
A month after a patient reaches age 17 years, members of the multidisciplinary team meet to discuss the individual’s academic, social and medical progression. Each patient must meet benchmarks in each domain to be deemed eligible for transition into adult care.
At age 17½ years, patients take tours of adult providers in their area. They also visit local public hospitals and learn which ones accept various insurance policies.
A joint effort between St. Jude and Methodist University Hospital, also in Memphis, allows patients aged 18 to 25 years the opportunity to continue to see Hankins for care, albeit with a different curriculum geared toward adults. The effort is called the St. Jude-Methodist Sickle Cell Disease Transition Clinic.
Improved outcomes
Outcomes have improved significantly since the creation of the transition program at St. Jude.
In 2007, the year before the program was established, 33% of patients with sickle cell disease successfully established care with an adult provider within 3 months of leaving pediatric care, Hankins said. In 2012, 75% did so.
Hankins said she and colleagues hope the program becomes a national model for efforts to encourage and facilitate a smooth transition from pediatric to adult care.
“The program can be helpful anywhere if you have the trained personnel,” Hankins said. “It may be more difficult to find specialized physicians in remote areas, but the training to become an educator is not that complex. It’s adaptable and resources are minimal. It’s not expensive. You just need the people to make it work.” – by Anthony Calabro
References:
Brousseau DC. JAMA. 2010;303:1288-1294.
CDC. Sickle Cell Disease Data & Statistics. Available at: www.cdc.gov/ncbddd/sicklecell/data.html. Accessed on Feb. 11, 2013.
Lebensburger JD. J Blood Med. 2012;3:105-112.
Platt OS. N Engl J Med. 1994;330:1639-1644.
Quinn CT. Blood. 2010;115:3447-3452.
Quinn CT. Blood. 2004;103:4023-4027.
Wojciechowski EA. J Pediatr Nurs. 2002;17:18-27.
Yanni E. J Pediatr. 2009;154:541-545.
The following were presented at the ASH Annual Meeting and Exposition; Dec. 8-11, 2012; Atlanta:
Ansa B. Abstract #3181.
Blinder MA. Abstract #475.
Tran H. Abstract #4246.
For more information:
Janis L. Abkowitz, MD, can be reached at Department of Medicine, University of Washington, P.O. Box 357710, 1705 NE Pacific St., Seattle, WA 98195-7710; email: janabk@u.washington.edu.
Kenneth I. Ataga, MBBS, can be reached at Department of Medicine, University of North Carolina, 170 Manning Drive, Chapel Hill, NC 27599; email: kenneth_ataga@med.unc.edu.
Morey A. Blinder, MD, can be reached at Washington University in St. Louis, Campus Box 8125, 660 S. Euclid Ave., St. Louis, MO 63110; email: mblinder@dom.wustl.edu.
Jane S. Hankins, MD, MS, can be reached at St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105-3678; email: jane.hankins@stjude.org.
Jeffrey L. Lebensburger, DO, can be reached at University of Alabama Birmingham Children’s Hospital, 1600 Seventh Ave. South, Birmingham, AL 35233; email: jlebensburger@peds.uab.edu.
George S. Rust, MD, MPH, can be reached at National Center for Primary Care at Morehouse School of Medicine, 720 Westview Drive SW, Atlanta, GA 30310-1495; email: grust@msm.edu.
Amanda M. Termuhlen, MD, can be reached at Miller Children’s Hospital Long Beach, 2801 Atlantic Ave., Long Beach, CA 90806; email: atermuhlen@chla.usc.edu.
Disclosure: Blinder and colleagues report research funding from, as well as advisory board and employment relationships with, Novartis Pharmaceuticals. Abkowitz, Ataga, Hankins, Lebensburger, Termuhlen and Rust report no relevant financial disclosures.
Perspective
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Harry S. Jacob, MD, FRCPath(Hon)
This extensive article might be briefly summarized; to wit: (1) Social workers trained in the disease are the key to effective transition; and (2) Medicaid expansion associated with the enactment of the Affordable Care Act will likely reduce the role of outrageously expensive EDs for adult sicklers. However, ironically, most Southern governors have — so far — opted out of government-supplemented Medicaid expansion plans (sigh!).Click Here to Manage Email Alerts