Hematology Nurse Practitioner
Joint Center for Sickle Cell and Thalassemic Disorders
Brigham and Women's Hospital
revised December 23, 2000
Care Coordination for Patients with Sickle Cell Disease
Sickle cell disease is a chronic, complex syndrome that requires
a multidisciplinary approach in order for patients to receive the best
care possible. Many patients have frequent, regular contact with the healthcare
system and often find it difficult, or even impossible, to coordinate all
the necessary subspecialties without assistance. Because of their holistic
approach to patient care, nurses, nurse practitioners, and physician assistants
(often referred to collectively as mid-level practitioners (MLPs)) are
uniquely qualified to assume the responsibility of patient care coordination.
Coordinating all the necessary subspecialty referrals is only a small
part of overall patient care. Helping patients and their families manage
the daily needs of living with a chronic disease is even more important.
These needs range from pain management, transfusion and chelation therapy
compliance, preventive and primary care issues, to patient education and
education of other health care providers.
MULTIDISCIPLINARY AND SUBSPECIALTY COORDINATION
The MLP serves as the liaison among the patient, the primary care provider
(often the patientís hematologist), and the specialists. Making appointments
for patients, encouraging them to keep their appointments, and following
up and implementing recommendations are all necessary parts of this aspect
of care coordination. Examples of the types of specialists that patients
may require include surgery, for placement and removal of indwelling intravenous
access devices; Ob/Gyn for female patients requiring birth control counseling,
annual Pap smears and, later, menopause management; renal medicine for
annual or semi-annual renal function testing; cardiology for ECHOS and
other cardiac function testing (especially as patients age); psychology
for any counseling needs; and, social services for any issues around welfare,
social security benefits, housing and disability needs.
Every encounter with a patient is an opportunity to reinforce the importance
of preventive health care, and determine the current needs of the patient.
Examinations including physicals, Pap smears, and ophthalmological evaluations
should be done annually. Tracking immunization schedules, EKGs, and pulmonary
function testing is also important and, unfortunately, can often get overlooked
in busy practices. Methods that can help patients remember their annual
preventive care needs include timing appointments with patientsí birthdays
and sending out vaccination reminder letters (for example, sending out
letters to the entire patient panel in the early fall reminding them to
come in for influenza vaccines). Methods that can help providers facilitate
reviewing and tracking all their patientsí needs include using desktop
and/or palmtop computerized databases and using the memo, to do, or reminder
functions of their computer date books.
Preventive care also includes educating patients and their families
to recognize the signs and symptoms of infections and other acute or life-threatening
complications of sickle cell disease. Parents of children with sickle cell
disease need to be taught how to use a thermometer and to palpate the spleen.
The MLP should constantly reinforce the importance of prophylactic penicillin
and monitor parentsí compliance with its proper administration. Parents
should also be taught when to call their childís mid-level or primary provider
and when to seek immediate care either in the clinic or emergency department.
For women with sickle cell disease who are of childbearing age preventive
care includes pregnancy prevention and counseling. Women with sickle cell
disease can and should use birth control. Providing there is no history
of documented thromboembolic events, women with SCD can safely use any
method of birth control including oral contraceptives, Depo Provera and
Norplant. For women on hydroxyurea or Desferal, adequate pregnancy prevention
measures are required (Castro 1999, Shilalukey 1997). Providers should
use every opportunity to ask about the use of birth control measures and
discussions should begin when patients are young teenagers. When they are
ready, teenage girls are more likely to ask for help if they know that
the subject of sexuality has already been suggested. The mid-level practitioner
can either manage patients on birth control themselves, or refer patients
to primary care or gynecology providers. A significant part of managing
these patients is making sure patients are using their birth control options
correctly and consistently, and providing annual Pap smears and STD checks
for women who are sexually active.
Education continues throughout a patientís life. Continue to evaluate
and review patients understanding of their disease. Encourage patients
to take an active role advocating for their own healthcare needs with other
specialists. The MLP can review the patientís medical history with them,
making sure they understand and can explain their own history to other
healthcare providers. Emphasize the importance of regular health care visits,
not just episodic visits for acute needs and encourage patients to make
their own appointments and choose their own primary care providers.
HOME MANAGEMENT OF PAINFUL EPISODES
Painful episodes, or pain crises, are the most common manifestation of
sickle cell disease. Most mild to moderate pain episodes can be managed
at home with prescribed oral analgesics. Other non-medical therapies that
can used for pain relief include hot baths and showers, massage, distraction,
and relaxation techniques. Educate patients to contact their primary or
mid-level provider for any atypical symptoms such as fevers, chest pain,
difficulty breathing, or pain in locations not typical for them or more
severe than usual.
Adults with sickle cell disease often experience daily, chronic pain
as well as episodic, acute painful events. Many of these patients manage
their chronic pain with daily use of prescribed oral opioids. The mid-level
providerís role in home management of chronic pain is crucial. Providers
must be vigilant in tracking their patientsí daily use of oral opioids
and intervening if there is any suggestion of over-medicating or misuse
of pain medications. Some suggestions for facilitating the tracking of
medications include the use of a computerized prescription database that
can be analyzed over time, and having as few providers as possible writing
prescriptions for a patient. When patients know they can only receive prescriptions
from one or two providers, they are less likely to try "shopping around"
for multiple prescriptions.
Providers must also educate patients and their families about basic
pain management principles, proper use of both long-acting and immediate
release opioids, concomitant use of non-opioid medications (such as NSAIDS
and anti-depressants), and the signs and symptoms of medication overuse
or abuse. A team approach including the patientís primary providers, pain
management experts, and a social worker is ideal. All of these providers
can work together to monitor patientsí ability to manage their daily pain
and to lead productive lives despite chronic pain.
DAY TREATMENT CENTER MANAGEMENT OF PAINFUL EPISODES
When a painful episode no longer responds to the prescribed oral analgesic
regimen, patients should be encouraged to go to the day treatment center
or emergency room for further care. Many hospitals around the country have
found they can offer excellent, consistent and cost-effective care in a
day treatment facility (Benjamin 2000; Ware, 1999). Hospitals with large
populations of sickle cell patients have dedicated sickle cell day treatment
clinics. In hospitals with smaller populations, sickle cell patients can
be treated along side oncology patients in chemotherapy infusion suites.
Both options for outpatient day treatment have several advantages over
the use of emergency rooms. First and foremost, a staff of nurses, midlevel
providers and physicians who are familiar with the patients treat them
on a regular basis. Intravenous or intramuscular pain medications are usually
given quicker and more aggressively than in the emergency room. Aggressive
outpatient treatment of moderate to severe painful episodes and consistent,
supportive care from familiar staff can often prevent the need for inpatient
admissions for these same crises.
The MLP continues to act as a liaison and resource for the day treatment
center staff, providing key information and recommendations about each
individual patientís needs. The MLP can also be responsible for assessing
the patient for any complications or symptoms requiring referral to a physician
or admission. Again, take the opportunity to review the patientís need
for preventive services, prescription refills or any other general health
INPATIENT MANAGEMENT OF PAINFUL EPISODES
When hospitalization is necessary for a painful episode, or any other sickle
cell disease complication, consistent admission to the same unit and primary
nursing is recommended when available. Patients should be treated with
intravenous opioids, by patient-controlled analgesia pumps or even by patient-controlled
epidural analgesia pumps if available and recommended.
The MLPs involvement continues to be crucial for optimal continuity
of care. Just as with the day treatment center staff, the MLP acts as a
liaison and resource for the
house staff and nursing staff. As the provider who knows the patient
best, being able to offer information about the patientís usual treatment
regimen, usual length of stay and even about the patientís usual behavioral
patterns will greatly enhance the patientís experience of the hospitalization
and make caring for the patient a much better experience for the house
staff. The MLPís input can make the difference between the patient being
treated appropriately and effectively or being under treated or even viewed
as "drug seeking" or "difficult". Regular phone contact or attending rounds
with the house staff provides ongoing education opportunities and ensures
the house staff gets accurate, consistent and timely information about
patient care. Offering in-services on sickle cell disease management to
new nurses is also a part of the MLPs responsibility. Providing education
for nursing staff will allow them to provide consistent, optimal care and
advocate for their sickle cell patients with the house staff.
EMERGENCY DEPARTMENT CARE
By the time they are adults, all sickle cell patients have experienced
many emergency department visits. The most common complaint of sickle cell
patients regarding emergency room care is the often inappropriate treatment
of their disease (Maxwell 1999; Shapiro 1997). Again, the MLPs can educate
emergency department staff members about the natural history of sickle
cell disease, recognition and treatment of life-threatening complications,
and proper, timely and effective pain treatment. Usually, emergency physicians
under or over treat painful episodes not because of their lack of knowledge
about pain management principles or treatment of sickle cell disease in
general, but because of their unfamiliarity with the individual patients.
Given the pace of activity in most urban emergency departments, quick and
easy access to individualized care plans specific to each patient is imperative.
Hospital-wide computerized records (if available), wallet-sized patient
"identification" cards, and a phone call to the patientís primary sickle
cell provider are all options for encouraging rapid communication between
the patient and ED personnel.
A two-sided identification card (Mandell 1997), which is the size of
a standard business card, can contain enough information to ensure the
prompt initial treatment for most sickle cell patients. This card can contain
pertinent medical history, baseline labs, allergies, outpatient medications,
the usual treatment plan for the patientís specific pain crises, and the
name and contact number of the patientís primary sickle cell providers.
The card can be signed by the patientís sickle cell provider (lending it
the authority of a medical record), laminated, and carried at all times
by the patient.
TRANSFUSIONS AND CHELATION THERAPY
Chronic transfusions are a fact of life for many people with sickle cell
disease, both children and adults. Mid-level practitioners can assume the
responsibility of coordinating the transfusion-related activities of their
patients. These activities include scheduling the transfusions, and monitoring
for iron overload. People with sickle cell disease who have been chronically
transfused since childhood often experience few pain crises and are, therefore,
either in school or working full time. Ideally, transfusions should be
scheduled at the patientís convenience so as not to interfere with their
school or work activities. To this end, many sickle cell centers offer
evening and weekend hours for transfusions. When transfusions can only
be scheduled during regular business hours, many patients will require
some gentle guidance and empowerment to help them plan regular time off
and to discuss these issues with their employers and/or school staff.
When iron overload is documented, patients should be started on iron
chelation therapy. Parenteral deferoxamine mesylate (Desferal) is the only
iron chelator available at this time and is used primarily as a subcutaneous
daily infusion. MLPs should educate children and their families about the
serious complications of iron overload, and carefully monitor compliance
with chelation therapy. Starting children on Desferal soon after beginning
chronic transfusions may make compliance with Desferal during the difficult
teen years somewhat easier. Children and their families should understand
that iron chelation is a routine and integral part of their transfusion
therapy, not something that can be ignored or dismissed because of itís
inconvenience. Adult sickle cell patients should also be educated about
the importance of iron chelation therapy, although compliance is often
Sickle cell patients should be transfused with leukocyte-poor, antigen
matched blood to reduce the frequency of transfusion reactions and the
development of minor antibodies (Steinberg, 1999). Finding antigen-matched
blood is often difficult in areas where the blood donor pool is primarily
made up of Caucasian donors. Providers should make it a routine part of
their patient, family and community education efforts to encourage blood
donations by the African American communities.
Women with sickle cell disease can successfully carry pregnancy to term
or near term. If a patient reports that she is interested in, or already
trying to conceive, hydroxyurea should be immediately discontinued. A patientís
pregnancy should be co-managed by high risk obstetrics and the primary
and mid-level sickle cell providers. Painful episodes during a pregnancy
should be managed as they would for non-pregnant patients with adequate
hydration, oxygenation and analgesia. Opioid analgesics should not be withheld
from a patient just because she is pregnant since fetal or newborn addiction
is rarely an issue. More importantly, the patient herself should remain
as healthy and comfortable as possible throughout the pregnancy.
Prophylactic transfusions during pregnancy are not indicated unless
there are complications which would ordinarily be treated with transfusions,
such as acute chest syndrome. Studies have shown that there is no overall
decrease in crisis frequency, premature labor or premature deliveries for
women who are prophylactically transfused throughout their pregnancy (Koshy,
The role of the mid-level provider is to educate the obstetric staff
about the current standards of treatment for sickle cell disease during
pregnancy, and to advocate for patients with OB physicians and antenatal
nursing staff to ensure proper, consistent and timely treatment of pain
crises. Patients should be seen frequently in the sickle cell clinic, every
few weeks to monthly, to be able to reinforce healthy behaviors and to
ally fears regarding use of opioid analgesics during pregnancy. She should
be encouraged to call the MLP with any questions or concerns. Educate the
patient and her family that the severity and frequency of pain crises may
increase during her pregnancy. Pain medication dosages may need to be increased.
Reassure her that you will work together to keep her as comfortable as
possible and that her narcotic use during pregnancy does not jeopardize
the health of her baby. Explain that if high doses of opioids are necessary
late in the pregnancy, her newborn may require a period of opioid weaning.
Hopefully, a NICU staff familiar with this process will be available to
care for the newborn.
EDUCATING FAMILIES AND THEIR COMMUNITIES
As shown throughout this chapter, family education is an integral aspect
of providing excellent care for patients with sickle cell disease. Encouraging
patients to become independent in their healthcare requires that family
members begin to relinquish some control over their childís involvement
in the healthcare system. This letting go process is not easy and offering
counseling and supportive assistance to families is important. Sickle cell
providers should get involved with efforts to educate their patientsí communities
about sickle cell disease, offering themselves for workshops and speaking
engagements. Providers should also stress the importance of community involvement
with efforts such as social support, offering part-time employment for
patients and regular participation as blood donors. Educating school nurses,
teachers, college professors and health services and, later, employers
will become necessary as patients go through their various life stages.
MLPs want patients to be independent, well-informed and active participants
in their own healthcare. Therefore, coordinating care must balance between
fostering this independence and ensuring that patients receive optimal
and appropriate healthcare. Advocating for patients in the confusing setting
of todayís healthcare environment is a rewarding aspect of the mid-level
practitionerís daily experience of working with sickle cell patients.
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Shilalukey K, Kaufman M, Bradley S, et al. Counseling sexually active teenagers
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