Elyse Mandell, MSN, RNCS
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.


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.


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.


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 issues.


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.


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.


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 easier.

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, 1988).

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.


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.