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Case Management of Posttransplantation Lymphoproliferative Disorder (PTLD)
Carolyn Rosa, RN
University of Virginia
Cmr9bx@virginia.edu
On my honor I have neither given nor received help on this assignment.
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Posttransplantation lymphoproliferative disorder (PTLD) is a serious
complication that can affect pediatric patients following an organ transplant. It is an
opportunistic infectious disease that arises after transplantation, usually involving
Epstein-Barr Virus (EBV) (Jain, 2002). Although rare, PTLD has a mortality rate
upwards of 50%. Risk of PTLD varies among types of solid organ transplant but is
highest among heart, lung, and small-bowel recipients. Furthermore, PTLD was
positively associated with young age, male sex, and white race. Further risk factors
include EBV seroconversion following transplantation and cytomegalovirus infection
(Morton, 2007). The pediatric patients that develop this disorder post-transplant become
increasingly complex to manage because of the delicate balance between maintaining the
grafted organ and combating the potentially deadly PTLD. Case managers are needed in
this situation because of their strong interdisciplinary component, their ability to advocate
for the patient and their strength in cost containment. PTLD patients are high-risk and
high-cost populations, further reinforcing the need for effective case management to
handle their care (Huber, 2010).
At Georgetown University Hospital, the pediatric transplant floor is an ideal
candidate for a case management project that would assist in care coordination of these
specific patients. Georgetown currently has transplanted 126 livers and 59 intestines for a
total of 185 patients under the age of 18 (United Network for Organ Sharing, 2010). Out
of those patients, five have currently been diagnosed with posttransplantation
lymphoproliferative disorder, resulting in 2.7% of the population being affected. (Two
more patients have been admitted in the past two weeks to work up for PTLD, one is
currently negative and the other is pending results). Despite this small number, 80% of
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those diagnoses have occurred in the past year, with 2 deaths in the past month. Clearly,
this is a medical complication of transplant at Georgetown University Hospital that is
increasing in its visibility and devastation. Also, in a study of 4,000 liver transplant
patients at the University of Pittsburgh, 4.3% were found to have PTLD with a
significantly higher rate found in children of 9.7%. The overall median time in
developing PTLD post-transplant in children was much shorter with a median of 8.1
months versus 15 months for adults. The children with PTLD in this study were found to
be EBV positive 98% of the time, compared with only 68% of adults with PTLD (Jain,
2002). These sobering statistics indicate that Georgetown will have a continued need to
assess, treat and manage children with PTLD.
To further indicate the need for a case management project for these patients, the
management must be further understood. In a situation where a child is both posttransplant and diagnosed with a hematological disorder, the patient will be bounced
between two separate units (transplant and hematology-oncology) at Georgetown
University Hospital, and be followed by a minimum of four attending physicians.
Furthermore, these children are also followed by their primary care providers (PCPs)
upon discharge and have the option of being transferred to an oncology clinic, but not
transplant clinic, closer to home. According to Buchman (2004), intestinal and multiorgan transplant alone are expensive procedures, ranging from $250,000 to $3 million per
case, with anti-rejection medications costing $10,000 annually without the cost of
readmission for infection and rejection calculated in. PTLD does not currently have a
defined estimated cost, but $5,000 a dose for rituximbab, the primary drug of treatment,
is what the nurses on the transplant unit have been told. Although Medicare has now
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approved payment for intestinal transplantation and cost of PTLD, the cost of an
extended hospital stay to the institution and families are going to be astronomical.
Case management solutions for the diseases of PTLD and post-transplant were
not evident from the review of literature, and neither disease singularly appeared to have
a case management model for pediatrics. However, Feudtner (2009) in a study of 186,586
children between the ages of 2 and 18 discharged from one of 38 hospitals found a
readmission rate of 16.7%. These readmissions were most highly correlated with:
The likelihood of readmission was most strongly associated with the patient's specified primary payer, the
number of prior admissions, the diagnosis of a complex chronic condition, and longer lengths of stay
during the index admission (Feudtner, 2009).
Furthermore, readmissions also found communality between complex chronic disease,
with malignancy and neurological diagnoses being the highest reason for readmission.
These specific issues fall directly into the realm of case management, as case managers
are focused on coordination and integration of health services for clients with complex or
costly healthcare problems. Feudtner (2009) suggests using the predictive readmission
model that was described in his study as a tool for case management to find target
populations to decrease costs of readmission. Although not specifically applied to case
management or PTLD, this study provides a structure for case managers who could
potentially apply the predictive readmission model to PTLD patients to discover the
specific reasons behind readmission, and therefore lead to a better way to coordinate
health care services across the continuum (Huber, 2010).
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A study involving a perspective on comprehensive care programs as applied to
pediatric cardiomypothay as a chronic disease by Bublik (2008) provides further
guidance for case management in chronic disease. The interdisciplinary approach of
coordinating nurses, social work, psychologists with funding from a regional insurance
company used by the University of Rochester in the late 1980s showed a decreased in
inpatient charges for children with chronic conditions from $26.1 million to $14.6 million
with a mean length of stay that decreased from 83.9 to 10.6 days. Also, three intensive
case management services for children with diabetes resulted in substantial savings as
well. These initiatives of specialty education, round-the-clock telephone access to an
educator and quarterly educator assessment of self-management skills resulted in the total
cost of a nonparticipant in this program being 125% greater than a participant. Bublik
acknowledges Wagner’s Chronic Care Model that integrates all medical and auxiliary
services that are designed for the care of chronic conditions, however does point out the
limitation in that it is specific for adults. However, the overview of the two separate
projects by Bublik (2008) does provide a guideline for case managers to initiate programs
in relation to PTLD which could help control costs, decrease readmissions and by
association with that, increase the quality of life for children and their families who have
already had an immense struggle with multiple chronic illnesses.
However, the model that resonates most with the needs of this particular
population is the medical-social case management model, specifically the primary
therapist model, as the case manager’s relationship to the client is one-on-one and
involves therapeutic interventions as well as a coordination of care services for the
patient. This model focuses on long-term care for patients that are at risk for frequent
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hospitalization. It focuses on using resources to keep the patients medically managed at
home and in the community. The family can relate to one person about treatment, service
access and case coordination (Huber, 2010). This model would provide the emotional
support needed for families who have just undergone a life-alternating transplant that
they hoped would improve the quality of their child’s life, only to then discover their
child has PTLD. The primary therapist model is only applicable when the case manager
has a master’s degree in psychology, social work, psychiatry or psychiatric nurse
specialties. This particular method has been found useful in small communities; however
has positive outcomes with coordination of care in larger institutions as well. The biggest
fault of the primary therapist model is that case management is occasionally ranked as
second priority by these managers, however the incentive to provide excellent continuum
of care is high for therapeutic reasons (Cohen, 2005).
For the Georgetown University Hospital pediatric transplant patients that are
diagnosed with PTLD, this particular model would be ideal for a variety of reasons. The
community of those diagnosed with PTLD post-transplant is small and close knit,
however it is situated within the larger institution of the transplant and hematologyoncology pediatric units of Georgetown University Hospital. Furthermore, based on the
available research of patients who develop PTLD post-transplant, statistically
Georgetown can expect future cases of PTLD to occur. This particular model would
allow one person to be the contact point for patients with PTLD whether they are inpatient or out-patient, providing a continuum of care in regards to follow-up transplant
and hem-oncology labs, changes in outpatient treatment, admission to either unit, needs
for out-patient assistance such as feeding pumps, infusion pumps, in-home nursing care,
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food stamps, ect. Furthermore, there is no current data in regards to case management for
PTLD. By providing a primary therapist case management model, run by one specific
case manager, a program could be developed and evaluated that could potentially be
applied to other pediatric cases of PTLD, and perhaps modified for the adult world at
GUH as well.
For development of this program an initial assessment would need to be done of
the current patient with PTLD and their families. Due to the small number of families,
personal one-on-one interviews could be conducted for the dual purpose of collecting
data and the case manager beginning to establish a relationship with the families. Data
collection should also be done in survey form for easy evaluation later, focusing on the
families’ view of their child’s quality of life. Further data collection should be obtained in
regards to length-of-admission, number of readmissions to Georgetown University
Hospital as well as outside institutions (that potentially follow the child’s course of PTLD
but not necessarily the transplant), and total cost of disease starting from initial diagnosis
of PTLD. Data could also be collected from the two families who experienced the death
of their child due to PTLD to have a better understanding of issues that occurred for the
family at end-of-life and frustration with the overall transplant/PTLD process. Once
needs are identified, the case manager could begin to plan and implement a
comprehensive program based on the primary therapist model. Families with PTLD could
be met with one-on-one during inpatient stays three times a week to provide emotional
support, specialty education and on-going discharge teaching as referenced in the Bublick
assessment of programs. The case manager would also be working on assuring a smooth
discharge, that outpatient services will be provided, and that the interdisciplinary teams of
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transplant and hem-onc are actively coordinating care and appropriately updating the
patients and each other. Upon discharge, a follow-up phone call within 24 hours would be
made to assure proper delivery of all medications, home equipment and that there were
no further complications. Following the initial phone call, weekly phone calls would be
made to discuss lab values, changes in medications and any concerns or frustrations the
family is experiencing. These phone calls could be further spaced depending on the
family’s needs. The cycle would restart every-time a patient was readmitted to
Georgetown University Hospital, with tri-weekly phone calls specifically to the families
and teams at outside institutions if the patient is readmitted somewhere else. The case
manager, no matter the stage of this process, would continue to advocate for necessary
funding and appropriate treatment for the patient, as well as timely coordination between
the two teams and multiple care providers(Huber, 2010).
After initiating the primary therapist case management program, an evaluation
would be done in six months comparing readmission rates and cost of management of
PTLD in these patients. This would provide an economic overview of whether or not the
case management program has been effective. The exact same survey that focuses on the
quality of life for their child should be re-distributed to patient’s families to see if their
has been an improvement related to management. Both of these results have the potential
to be skewed, as it is likely these patients will be further along in their PTLD treatment
and further away from their inpatient post-transplant stay. The ideal way to evaluate the
effect of this program would be to follow a child diagnosed with PTLD from day one of
diagnosis, implement the program, and compare outcomes with this child from the initial
data collected concerning readmissions, cost of treatment and quality of life.
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The resources in this chosen area are very slim. As PTLD is a rare complication
of transplant, there is very little literature available at how to appropriately coordinate the
care of these patients. However, the nurses who care for these patients on a regular basis
are in an excellent position to consider pursuing a case management degree to further
assist these patients. The transplant and hem-onc nurses at Georgetown University
Hospital have close relationships with the PTLD patients as well as their interdisciplinary
team and have a strong understanding of the challenges and needs of this particular
population. Furthermore, the approval of Medicare of intestinal/liver transplantation and
the coverage of complications (Buchman, 2004) allows the case manager monetary
assistance for various needs of this particular population. Another available resource is
simply the families themselves who have undergone this ordeal and have been largely
ignored by conventional medical management studies. Lastly, the transplant and hem-onc
programs individually have dedicated social workers, case managers and coordinators
that would be available to collaborate in the initiation of such a program due to their
specialty knowledge.
The complexitity of posttransplanation lymphoproliferative disease, along with
the financial burden and emotional toll that this disease creates provides an excellent
platform for a case management program. A program of such nature would make the
disease more manageable not only for the institution, but for the patients and their
families who must suffer through it.
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References
Bublik, N., Alvarez, A. & Lipshultz, S.E. (2008). Pediatric Cardiomyopathy as a Chronic
Disease: A Prespective on Comprehensive Care Programs. Progressive Pediatric
Cardiology 25(1), 103-111.
Buchman, A.L. (2004). The Medical and Surgical Management of Short Bowel
Syndrome. Medscape General Medicine 6(2), 18-20.
Cohen, E.L. & Cesta, T.G. (2005). Nursing case management: From Essentials to
Advanced Practice Application. St. Louis, Missouri: Elsvier Mosby
Feudtner, C.M., Levin, J.E., Srivastava, R., Goodman, D., Slonim, A.D., Sharma, V….
Hall, M. (2009). How well can hospital readmission be predicted in a cohort of
hospitalized children? A retrospective multi-center study. Pediatrics 123(1), 286293.
Jain, A., Nalesnik, M., Reyes, J., Pokhama, R., Mazariegos, G., Green, M.…Fung, J.
(2002). Posttransplant Lymphoproliferative Disorders in Liver Transplantation:
A 20-Year Experience. Analysis of Surgery 236(4), 429-437.
Morton, L.M., Landergren, O. Nilanjan, C., Castenson, D., Parsons, R., Hoover, R.N. &
Engles, E.A. (2007). Hepatitis C virus infection and risk of posttransplantion
lymphoproliferative disorder among solid organ transplant recipients. Blood,
1528-0020. Retrieved from:http://bloodjournal.hematologylibrary.org
Huber, D. L. (2010). Leadership and Nursing Care Management. Maryland Heights,
Missouri: Saunders Elsvier.
United Network for Organ Sharing. (2010). Transplant in the U.S. By Recipient Age.
Received from http://www.unos.org/donation/index.php?topic=data)
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