revised April 19, 2002
Development of a Comprehensive Care Program for Patients with Sickle Cell
Disease
Need for Comprehensive Care in the Management of Sickle
Cell Disease
Sickle cell disease is a chronic illness that affects
multiple organ systems. While often viewed as an episodic process, the
true nature of the disease in adults is one of chronic slow deterioration
of multiple organ systems due to sickling of red blood cells and sickling
relation phenomena. The disease should be viewed as a complex, multi-system
illness rather than a simple disorder involving deranged red blood cells.
The most important aspect of comprehensive care for
patients with sickle cell disease is early intervention for preventable
problems. The known pattern of organ injury in people with sickle cell disease allows timely intervention to circumvent problems.
Retinal hemorrhage, which can occur in young adults with sickle cell disease
with no warning, highlights the issue. Retinopathy does not correlate wth sickle
cell disease severity and can occur in people who are otherwise virtually asymptomatic.
Yearly retinal examinations by an
opthamologist can detect early changes of sickle cell retinopathy allowing
interventions to prevent the complications of this devastating problem.
Members of the Comprehensive Care Team
-
Physician
The comprehensive care team requires a physician
who is dedicated to the care of patients with sickle cell disease. This
means that the physician should become familiar with the multiplex complications
and presentations of the disorder. The physician plays a crucial role in
care coordination and patient oversight.
While a hematologist is a logical choice for the
role, the physician involved in sickle cell management does not necessarily
need a hematology background. Sickle cell disease as a chronic illness
manifests problems and complications that are common to internal medicine
in general. A well trained internist who takes the additional time to learn
about the management and issues involved in patients with sickle cell disease
can be as effective as a hematologist in the oversight and care of these
patients.
-
Physician Extender
The physician extender is crucial to the program.
Ideally, a nurse practitioner or a physician assistant would fill this
role. Some programs have worked with an RN who has developed a special
expertise in the care of patients with sickle cell disease.
The chronic nature of sickle cell disease means that
a plethora of issues will develop while caring for many of these patients.
These include problems such as leg ulcers, cardiac dysfunction and avascular
necrosis of bone. The physician extender plays a crucial role in helping
the patient to cope with these problems and arranging appropriate consultative
evaluations for these issues.
The primary manifestation of sickle cell disease
is recurrent and often chronic severe pain. As a result, opioid analgesic
management is a major part of the care program. Opioid analgesics are often
effective in relieving pain, but do not alter the underlying course of
sickle cell disease. The management personnel, namely the physician and
the physician extender, must be aware of the limitations of these medications,
as well as possible complications of opioid medication use.
One of the major issues that develops in the management
of patients with sickle cell disease is under-medication during pain crises.
This most often reflects the development of tolerance (which is distinctly
different from addiction) to opioid medications. Patients consequently
require larger doses of these medications than most physicians and nurses
are accustomed to providing. A close working relationship between the patient, physician
and physician extender
provides the foundation for understanding how best to manage sickle cell pain
both in hospital and out of the hospital.
-
Social Service Coordination
The complications of sickle cell disease such as
avascular necrosis of bone or recurrent episodes of pain disable many patients
to varying extents. The consequent issues, including unemployment or underemployment
and sub-optimal education places these patients at a socioeconomic disadvantage.
A host of problems can arise such as paying heating bills, light bills,
and transportation for medical care. These burdens can become extremely
oppressive for patients with sickle cell disease. Sickle cell pain is real
but is impacted by the psychological state of the patient. A positive frame
of mind for patients reduces the possibility of complications flaring out
of control because of the intersection of the medical problem with psychosocial
issues.
Facilities for Patient Care
-
Outpatient Care
Most medical interactions for patients
with sickle cell disease consist of outpatient care. The model that works
best is one in which the care of these patients is clustered around the
physician and physician extender. Although many members of the staff, including
the general medical staff, can provide care for these patients, an understanding
of the issues involved and the chronic nature of the problems means that
these are handled most efficiently by a dedicated outpatient team.
Often the comprehensive care team become de facto
primary care physicians for these patients. The patients have far more
contact with individuals in the comprehensive care setting and therefore
defer to these providers regarding many of their primary care problems.
Patients who are hospitalized frequently and who are also frequently seen
in the outpatient setting are understandably reluctant to add another set
of visits to a primary care physician. In fact, the PCP often plays a very
secondary management role because so many of the medical problems relate directly to sickle cell disease. The exact logistics of the interface with primary care
physicians varies between institutions depending on issues such as reimbursement
and the general structure of outpatient care in the medical system.
-
Inpatient Care
Patients with sickle cell disease who require hospitalization
should be cared for by a select staff to the degree possible. The practice
of spreading patients with sickle cell disease among several medical teams
on different floors has the advantage of allowing more professionals to
work with these patients. It has the profound disadvantage that these professionals
frequently are unfamiliar with sickle cell disease as a whole and are unfamiliar
with the patients as individuals. Knowing the opioid medication tolerance
of a particular patient can, for instance, allow providers to intervene
in a way that gives the greatest degree of patient relief while reducing
stress on the providers in the system.
Day Hospital Concept
The concept of a day hospital for the treatment of sickle
cell disease has been one of the major innovations over the last twelve
to fifteen years. A number of institutions have developed excellent models
for day hospital management, including the program at Montifiore Hospital
created by Dr. Lennete Benjamin (1) and that developed at Emory
by Dr. James Eckman.
The day hospital is a facility in which patients
with sickle cell disease who have acute vaso-occlusive pain crisis without
other obvious complications can be treated in an outpatient setting without
going to the emergency room. Emergency room care almost uniformly is unsatisfactory
for patients with sickle cell disease. This reflects the nature of an emergency
room, in which patients with trauma, myocardial infarctions, septic shock
and other major, life-threatening events must take priority over patients
who are in severe pain, but whose lives are not threatened. As a result,
patients with sickle cell disease can wait hours before receiving treatment.
Disadvantages of this arrangement include frustration for both patient
and family who come seeking treatment of often excruciating pain. Further,
the level of pain often increases during the wait necessitating
larger medication doses when the patient finally is seen.
Day hospital facilities can be arranged in several
different ways. At institutions with a large number of patients with sickle
cell disease, for instance more than 200 to 300 patients, a dedicated facility
that can provide analgesics, hydration and management during the day can
be very successful. Many institutions, however, do not have enough patients
with sickle cell disease to justify the creation of an entire establishment
for management of acute pain.
In this instance facilities, including the program
at Brigham and Women's Hospital in Boston, use the outpatient chemotherapy
infusion suite of the oncology service as an alternative to the emergency room. The chemotherapy
infusion suite has highly skilled nurses who are accustomed to handling
patients with severe illness and severe pain. The ability to move patients
quickly into a setting where they can be evaluated and begun on treatment
is a major advantage. Clearly, flexibility by the staff in the chemo-infusion
suite is of paramount importance since the onset of sickle cell crises
is unpredictable. Patients receiving chemotherapy are on a regular schedule
and visits are planned ahead of time. This is not the case in general for
patients with sickle cell disease. If flexibility in the system can be
developed, an excellent program can move forward for the care of
patients with sickle cell disease.
Pain Service
Sickle cell disease is a multi-system chronic illness.
The major manifestation of sickle cell disease, however, is severe, recurrent
episodes of pain. Patients who are hospitalized should have their care
coordinated by the comprehensive care team in conjunction with the pain
service. Most pain service programs are geared to manage post-operative
patients. The pain that sickle cell patients experience is quite different
and follows a different clinical pattern. Therefore, the pain service must
be aware of these differences and willing to make the appropriate changes
to allow optimal patient care.
In some institutions, the pain management is overseen
completely by the physician from the comprehensive care program along with
physician extender. Opioid analgesics administered by PCA pump is the preferable
means of providing pain relief. Input into management of PCA pumps by the members
of the comprehensive care team is an extremely important issue. At some
institutions, oversight of PCA pumps is solely the province of the pain
service and the anesthesia department. Patients with sickle cell disease
benefit enormously when members of the comprehensive care program are part
of the management team.
Uniform Patient Data Collection and Management
Like all people afflicted with chronic illness, patients with sickle cell disease
can acquire staggeringly complex hospital records. An 18 year old with a record comprising
10 or 11 volumes exists in every practice with a substantial number of patients. Paper records of this
magnitude are very difficult to sort and review. Important information often is buried
in the mass of paper, frustrating providers and compromising the quality of patient
care. The advent of computerized hospital records and data storage has improved but
not eliminated the problem.
The data on patients followed in a comprehensive care program should be organized around several principles with
the aim of enhancing the quality of the care that is delivered.
- Ease of access.
Data should be readily available to providers. Paper medical
records in which old volumes are (necessarily) placed in storage for the sake of
space is the antithesis of this principle.
- Flagging of major events
. An example of this principle is the need to
know when patients were last immunized with pneumococcal vaccine. Most patients do
not remember medications and vaccinations from the past. Finding documentation of
vaccinaiton in old paper medical records is nearly impossible.
- Flagging of upcoming anniversaries
. This is a corollary of the preceeding
point. Opthalmology follow-up is often overlooked, for example, because the patient
and the provider forget that the anniversary of the most recent examination has passed.
Although computerized records and data storage are not de facto solutions
to these issues, digital technology lends itself to several approaches to the problems.
Most large medical facilities now have records that are computerized to some extent.
Often the data are in multiple databases (e.g., a blood bank data base and
a clinical chemistry data base) which can frustrate an effort to create a profile
pertinent to patients with sickle cell disease. The hospital system is designed to
be as broadly encompassing as possible, while specific data are needed for people with sickle cell
diseae. A number of options exist:
- Spreadsheets.
Spreadsheets are powerful tools for organizing data of all
types. They allow rapid data recall and highlight missing information. Empty data cells are
easy to spot and thus are a mnemonic for providers. Most spreadsheets provide
graphic data displays that can be very useful in spotting trends that occur over
extended periods of time. A creatinine of 1.0 mg/deciliter may not appear alarming. However, a
graphic representation of a rise from 0.4 to 1.0 mg/deciliter over the course of a year is very
striking and alerts the provider that investigation of the trend is warranted. Information
on antibodies to minor red cell antigens is quite useful as these can disappear over
time, but return in the form of a delayed hemolytic reaction following transfusion
with cataclysmic results. Spreadsheets
can store both text and numerical data, which extends their usefulness even further.
- Relational Databases.
Relational databases are powerful tools most useful for research.
These programs allow groupings and comparisons of data from multiple and variable
fields from any number of patients. The platform must be carefully designed before
data are entered into the fields. Once in place, relational databases have almost
unlimited potential in patient assessment and management.
- Personal Data Assistants.
The digital revolution was intially limited
by the stationary nature of the data. Desktop computers are not mobile, and even
laptop computers are limited in that regard. Personal Data Assistants (PDAs) provide
one solution to the issue. A number of databases are specifically designed for PDAs.
As the computing power of these devices has increased, some now can support modified
versions of potent desktop databases, such as Microsoft Excel. PDAs allow all the
providers in the group to have the same up-to-date information on patients. PDAs
are particularly useful for physicians who are covering the primary providers since
they
often are unfamiliar with the details of a particular patient's history. A PDA can
save the physician the time and effort needed to travel in to the hospital to review
records needed to make informed recommendations. Some
PDAs can now communicate wirelessly with servers to obtain the most recent patient
data and profiles.
- Patient Information Cards.
Patient information cards are easy to
produce and can be tailored to provide essential information for treating physicians,
such as those in the emergency room. A typical patient information card contains
the names of the primary physicians and their contact information, key laboratory
data (e.g., baseline hemoglobin and white count), medications used at home
and medications typically used to control crisis pain. Information on allergies,
blood type and antibodies is easily included on such a card. An information
card often is invalubale to providers when patients are seen outside their home institutions.
Allied Medical Services
The chronic nature of sickle cell disease and the injury
to various organ systems requires the coordinated care with other medical
subspecialties. Standing relationships serve patients far better than do ad
hoc arrangements.
Orthopedics
Patients with sickle cell disease often suffer avascular
necrosis of bone involving, most often, the hips and shoulders. This problem
is extremely difficult to treat and requires close coordination with the
orthopedic surgeons. Some patients unfortunately come to joint replacement,
most often the hips. An orthopedic surgeon who takes a special
interest in the management of patients with sickle cell disease is ideal
for this kind of program.
Nephrology
Patients with sickle cell disease suffer renal injury
beginning early in life. Patients lose renal concentrating abilities by
approximately the age of 18 months and are at higher risk of developing
chronic renal failure. Close coordination with the nephrologist is an important
issue. Many care providers do not realize that patients with sickle cell
disease have a low serum creatinine usually in the range of 0.5 mg/deciliter relfecting
their high glomerular filtration rate.
A patient with a serum creatinine of 2.0 mg/deciliter in fact has substantially impaired
renal function. Therefore, a nephrologist who understands and is interested
in the differences between nephrology care for normal individuals and individuals
with sickle cell disease is an extremely valuable asset to a comprehensive
care program.
Cardiology
Patients with sickle cell disease invariable have
a dilated heart. Initially heart function is normal with an ejection
fraction of 60 to 70% in most patients. Patients can develop high output
cardiac failure because of the low hematocrit and the work that their heart
performs. This problem must be monitored closely by the cardiologist. In
addition, there is increasing recognition of problems such as right atrial
dysfunction that occurs in patients often at a relatively young age.
Ophthalmology
Yearly ophthalmology examinations are mandatory
for all patients with sickle cell disease. No correlation exists between
sickle cell pain or other complications and development of sickle cell
retinopathy. The retinopathy exists in the periphery of the eye grounds
and can be visualized only by a thorough retinal examination by an ophthalmologist.
This should be done on a yearly basis to prevent sudden vision loss due
to retinal detachment or bleeding into the vitreous humor.
Implementation Schedule
Not all components of the comprehensive care program
for patients with sickle cell disease can be implemented simultaneously.
A newly formed program must go through a series of developmental stages
in order to provide a broad based care program for patients with sickle
cell disease.
Primary Providers
The physician and physician extender are the initial
"components" that must be incorporated within the comprehensive care program.
Physicians and physician extenders unaccustomed to working with patients with sickle cell disease generally require some
time to become familiar with the problems specific to sickle cell disease. With time and dedication, any professional can fill the role
Consolidation of Patient Oversight
The primary care of patients with sickle cell disease
must be concentrated under the management of the physician and physician
extender in order to have a functioning comprehensive care program for
the patients. The initial steps should involve the formation of a clinic
program where patients are seen at discreet times in the outpatient
clinic. The management of the patients should be streamlined and made as
uniform as possible.
Hospitalized patients should also fall under the
aegis of the sickle cell care physician and physician extender. Because hospital care programs vary tremendously between institutions, some
accommodation likely will be necessary for individual institutions. The
most important aspect of the hospital care, however, is that a line of
communication remain open between the physician and physician extender
and the physicians of record, who are directly involved in the oversight
of patients while in the hospital.
Day Treatment Facility
The day treatment facility is a crucial component
to a comprehensive care program for patients with sickle cell disease as
noted above. The day treatment facility cannot work effectively until patients
are consolidated and a fairly uniform standard of management exists.
The hospital administrators as well as the
members of the Department of Medicine who are in a decision making capacity
must decide on how best the day treatment facility should be arranged.
As noted above, the number of patients will have a major impact upon the
arrangements that are most efficient for the sickle cell patients at a
particular institution. Nonetheless, the overall goal must be the same
irrespective of the logistics of the day treatment facility. Rapid management
of vaso-occlusive of pain crisis with adequate analgesic treatment and
review for other sickle cell complications are key to the proper functioning
of this facility.
Social Service Coordination
Social service issues are of great importance in
the lives of patients with sickle cell disease. This is particularly true
for patients who have more severe manifestations of their disease which
require frequent contacts with the medical establishment. A social worker
dedicated to this cohort of patients, in addition to his or her other functions,
is essential to the smooth running of the facility. Patients with sickle
cell disease ultimately will be managed more efficiently with input
from a skilled social service provider.
Summary
Patients with sickle cell disease present
a major challenge to medical care providers. The lifespan of patients with
sickle cell disease is increasing with better general medical care. Despite
the lack of a cure for sickle cell disease (with the exception of bone
marrow transplantation), patients with sickle cell disease can expect further
improvement in the quality and length of their lives. A comprehensive program,
similar to those that exist for patients with other chronic illnesses such as Type II diabetes or
cystic fibrosis, will further improve the quality of life for this very
vulnerable group of patients.
References:
-
Benjamin LJ, Swinson GI, Nagel RL. 2000. Sickle cell anemia day hospital:
an approach for the management of uncomplicated painful crises. Blood 95:1130-1136.
