A Consultative Network for Sickle Cell Disease



Sponsored by the NHLBI and Office of Rare Diseases, NIH

The aim of this conference was to pursue a high priority recommendation from an NHLBI workshop on "Adults With Sickle Cell Disease: Meeting Unmet Needs" held at the NIH on June 13-14, 2002 and meetings with consumers that followed in 2002-3. Over the years, medical advances have helped to increase the lifespan of sickle cell patients. Comprehensive care for children with sickle cell disease is generally more available than for adults because there are relatively fewer providers with expertise and willingness to care for adults with sickle cell diseases. The NHLBI therefore was asked to form a consultative network that could provide expertise to community providers who care for adults with sickle cell disease.


Rationale for the Agenda:

To provide remote consultations, the possibility of telemedicine was explored initially. This modality provides the most information, and has been used within states, rural areas, the Department of Defense, and the Veterans Administration. Across state lines, however, telemedicine requires multiple licenses, a barrier that federal programs do not face, so they are not good models. In addition, telemedicine uses expensive technology which is not widely available. Thus, this meeting focused on telephone calls, a long accepted form of consultation for which national hotlines exist; websites which may have enhancements like electronic newsletters and live chat; and e-mail which can include listservs. The latter two options require computer access. In any case, one serves either patients directly or via their primary care providers. The program presented several approaches to cover different scenarios:

1. A physician may need an emergency consult.

Dr. Henry Rosenberg and Dianne Daugherty spoke about a model called the Malignant Hyperthermia Hotline ( is external)) established in 1982. A provider calls 1-800-98-MHAUS to get realtime advice on how to treat this life-threatening emergency. The number operates 24 hours a day, 7 days a week, 365 days a year and is accessed via a collaborative agreement with a poison control center. The triage staff are nurses and toxicologists trained by publications and anesthesiologists; they listen in and write up the calls to ensure accuracy and improve follow up. If the request is for information only, the caller will be read the answer from printed information, referred to the MHAUS number or to the website. A specific call about patient management will be connected to one of three consultants. A hotline coordinator schedules the three consultants from a pool of about 30 anesthesiologists who serve for two week periods on call. The coordinator also helps to recruit and train new consultants, ensures quality data collection, and prepares cases for review. The coordinator is paid, but the consultants are not. They must maintain an interest in malignant hyperthermia, have their own beeper or cell phone, and agree to fill out a data form on the website or a paper report after each call. New recruits are recommended and mentored by other consultants, and are motivated by public service, growth in personal knowledge, and peer recognition. The consultants develop camaraderie as they review problem cases quarterly and give awards for service at their annual meeting. The total budget for this hotline is about $40K per year, and consists of: $27K for the coordinator, $7800 for triage ($16/call), $813 for the 800 number, $2K for malpractice coverage ($1M policy with a $7500 deductible), and about $500 for supplies. Support comes from corporations, professional groups, individuals, membership, and publications. With regard to the remote legal risk, the consultants must have their own malpractice insurance, and inform their institution of this activity. Over the years, MHAUS received only two subpoenas, both of which were resolved easily. Call activity for a year consisted of 2707 calls, 343 (13%) answered by nurse, 346 (13%) connected to consultant, of which 286 (11%) were case consultations, and 60 (2%) were unusual questions. Most importantly, the hotline is publicized at meetings, through publications, stickers, etc. for outreach. Another hotline is 1-800-NO-CLOTS, which deals with blood coagulation problems. It is staffed by a receptionist (or answering service after hours) that pages a doctor who rotates with 1-2 others on call. The service is based in Canada, so the (mal)practice situation may not apply to the U.S. Our country makes about 10% of the 500-1000 calls per year, and the program operates on about $20K per year.

2. A patient needs information or a second opinion.

Dorothy Knight spoke about consolidation at the Centers for Disease Control, e.g. the AIDS/STD hotlines at is external) into one number, 1-800-CDC-INFO. Over 40 toll-free numbers that respond to 2M+ inquiries per year at a cost of $25M will be routed to a single resource to increase efficiency, assess customer satisfaction, and improve accountability. Senior management agreed to check the NCI, CMS, SSA, and IRS to see how they handle first contacts with the public and provide personal services. These ideas and industry input contributed to a workscope with 24/7/365 multi-channel contact, multi-lingual operator and e-mail responses, web integration, self-service, referrals to local health facilities, and rapid responses to changing events. Other technologic features use communications by the FTS network, call monitoring, intelligent routing, multi-tier support, advanced search functions, web chat, content/data/knowledge and workforce/customer management. Good business practices include data collection, quality assurance, knowledge integration, and promotion. These components may be desirable to deliver information to patients. The hotline will be funded by a 7 year performance-based contract with defined measures, a cost plus fixed fee and incentive award, hopefully to reduce the cost to under $50 a call. Vendors will be rated on responsiveness, performance, quality, accuracy, completeness, manner of delivery, continuous improvement, customer satisfaction, and cost management. It may be possible to link this system to sickle cell information as that provided by Emory (see below).

3. Tools used to improve the accuracy of communication

This part of the meeting dealt with some tools used at the NIH to augment communication. The first was presented by Kathy Kranzfelder from the NIDDK. As part of a collaboration between the National Kidney Disease Education Program and the Renal Physicians Association, they developed a Nephrology Consult Letter Template based upon practice guidelines ( It is a web-based form that a nephrologist fills out with a patient?s data and that generates a letter to the primary provider to define who will take care of what health problems. This coordination will decrease duplications and omissions. The data is not stored on the NIDDK website, and a disclaimer states: ?Medical or health information provided on this site is designed to support, not replace, the relationship that exists between a patient or site visitor and his/her physician. With respect to documents available from this website, neither the United States Government nor any of its employees make any warranty, express or implied, including the warranty of fitness for a particular purpose, or assume any legal liability or responsibility for the accuracy, completeness, or usefulness of any information, apparatus, product, or process disclosed, or represent that its use would not infringe privately owned rights. The physician utilizing this letter template is solely responsible for reviewing the generated letter for accuracy before transmittal.? It is possible that a similar tool also could be designed to convey and capture essential data when a patient is referred to a consultant to reduce errors. A second tool was described by Annette Galassi from the NCI Cancer Information Service (CIS). For years they have run the Physicians? Data Query (PDQ) via a hotline at 1-800-4-CANCER, which operates in the U.S. Mon.-Fri. from 9-4:30 local time. It fields about 200-300K calls per year. More recently, it started a LiveHelp service (, where users can type questions into a web browser and have them answered in real time by an information specialist. While the service does not provide medical advice, it is available internationally and until 10PM EST. They receive about 13K chats per year at a cost of about $16 per session. This modality may be suitable for subacute situations where patient-specific advice is not needed.

4. Websites that could serve as key information sources

Several websites provide information on sickle cell disease, e.g. the Sickle Cell Disease Association of America ( is external)); Information Center for Sickle Cell and Thalassemic Disorders ( is external)); and the Sickle Cell Information Center ( is external)). Dr. James Eckman spoke about the latter, developed by Allan Platt, and has no commercial ties. (It is sponsored by the Georgia Comprehensive Sickle Cell Center; Dept. of Pediatrics and Winship Cancer Institute, Emory University School of Medicine; Sickle Cell Foundation of Georgia Inc.; Grady Health System; Morehouse School of Medicine; and Atlanta Sickle Cell Consortium.) The site provides patient and professional education, research updates, and other resources. While it also offers counseling, the "information provided on this site is designed to support, not replace, the relationship that exists between a patient/site visitor and his/her physician." The site maintains individual privacy, and gathers no personal information. The content is user-specific for patients, families or providers, and includes responses to FAQs, which are reviewed by the center?s Advisory Board. The text of Sickle Cell Problem Oriented Clinical Guidelines by Eckman and Platt is online, and can be downloaded onto a personal digital assistant (PDA). The website has over 40K hits and 5K page views per day, plus 30K unique visitors a month. Access data to specific pages help to improve the site, but there is still need for tailored information. Dr. Eckman still tries to give personal consults by e-mail, but warned that many issues of licensure, liability, and reimbursement for cybermedicine are not resolved. Useful articles are by: Pong ( is external)), Straus ( is external)), Wachter ( is external)), and Martin ( is external)). [Many of these problems do not exist for local telemedical systems ( is external)) or ones that extend internationally (,2510,404_2022_5174,00.html(link is external) and,2564,455_3817_5856,00.html(link is external)).] Reimbursement is still limited ( is external)), but is being tried for e-mail.

5. Enhancement of an Sickle Cell Adult Provider Network (SCAPN)

Dr. Kathryn Hassell described the SCAPN, founded in 2002 by providers interested in adult sickle cell disease. Its goal is to create a forum for communication and a directory of adult care providers who can post questions and cases on a listserv for discussion and education. It also can link providers with similar interests to promote clinical care or research opportunities. Currently there are 123 providers (78 MD/DO, 18 RN, 5 NP, 4 MSW, 3 PAC, 2 PhD, 1 OTR) from 28 states, Brazil, Canada, and India. Recent activities include: (1) Case studies followed by 2-4 questions (e.g. pregnancy, pulmonary hypertension, transfusion management), and e-mail or fax responses summarized and discussed with bibliography sent to all participants; (2) Collaborative data collection of unusual adult events (e.g. adult stroke), health maintenance practices, and support services; and (3) Participant questions, such as appropriate use of pain medications and unusual complications. The value of this listserv is that providers can share their experiences when no definitive treatments exist, and perhaps work together to develop some guidelines. The network members try to meet regularly at national meetings, but in reality, only about 7-10 of the 123 participate routinely in the online cases or questions. More appreciate the shared information and educational benefit. Dr. Ted Wun envisions the SCAPN to grow into regional centers with experienced staff, not necessarily hematologists, to care for adult sickle cell patients. They would provide remote consultations and accept referrals; perform annual comprehensive evaluations and clinical trials; and help to develop practice guidelines. Hemophilia Treatment Centers may be good models, even though they also have a shortage of specialists. Comprehensive Sickle Cell Centers could serve as anchors to areas that do not have facilities. Team members would include: an M.D., R.N., social worker, and pulmonary, nephrology, neurology, cardiology, ophthalmology, transfusion, or liver experts as needed. If possible, the group should involve the patient?s pediatric caregiver to ease the transition. Presently, the SCAPN requires only an interest to participate, but encourages more action to develop practice guidelines and collaborate in multicenter surveys and trials. Tangible products are important to create legitimacy and prestige as a sickle cell center that should receive NIH grant support.

6. Sickle Cell Disease Management in the Information Age

Dr. Kenneth Bridges presented his perspective as the Director of the Joint Center for Sickle Cell and Thalassemic Disorders ( is external)). Maldistribution of specialists is the main reason for poor availability of good care. The NIH funds 10 Comprehensive Sickle Cell Centers in major cities, which attract providers. Other large cities have good SCC, but some do not, and many patients live beyond urban centers. The provider knowledge base is outdated, and often primary caregivers cannot get CME due to limited time or cost. Thus the Internet is a good way to provide consults if a computer is available, and when a hotline is not needed for dialogue in an emergency. The Internet also requires few personnel because they or the website can screen FAQ and forward only the unique ones. While responses are uniformly good, the information from the requester may not be accurate, complete, or current, so the quality of the ?virtual? interaction is uncertain. Nevertheless, the contact or data can be captured, and the availability of the information seems to benefit patients. His website has about 2000 unique visits a month, which generates about 30 inquiries. About 15 are from patients, 10 from providers, and 5 from schools. Some general advice about e-mail communication is to omit patient identifiers, use a disclaimer in the response, which should be to the general thrust of the question. One should give accurate information without hurting the patient?s relationship with the provider, address alternatives reasonably without bias, and avoid undue optimism or pessimism. Approaches to reimbursement would be to convince insurers that electronic consults improve healthcare, apply for grants from HRSA, or get ASH to back projects to improve hematology care.

Conclusions and Recommendations

Dr. Cage Johnson suggested that SCAPN members collect data on the number and types of calls they receive to assess the needs of both providers and patients.

  • A hotline may be useful for acute management problems or urgent referrals to patients. If a facility can be found, providers or patients could speak with an SCAPN consultant directly. In addition to the malignant hyperthermia network, pediatric ICU/ER models may be applicable. An SCD hotline could be spliced with an existing one.
  • Less emergent issues, such as advice or second opinion, could be handled by a website, e-mail, or the SCAPN listserv. The latter helps to share experience and builds closer relationships.
  • Initially, documentation of contacts could be fairly general, like that used for case exercises. As experience grows, a more detailed case report form could be developed to improve accuracy of patient data and completeness of response
  • The SCAPN (under the aegis of ASH, AHRQ, HRSA, or NIH) could check the ITP experience to help find consensus, and draft practice algorithms or guidelines, which it could publicize and offer CME among primary care and emergency medicine organizations.
  • The SCAPN should consider these main topics: (1) surveillance of adult complications, including pregnancy; (2) clinical trials, e.g. pain management; and (3) outcomes research, such as quality of life.
  • Training is part of the Comprehensive Sickle Cell Centers, but additional fellowships could be offered at other facilities.
  • Legislation may be needed to clarify consults vs. practice across state lines or to shield providers from liability when they act as ?Good Samaritans? for the treatment of rare diseases. In addition, a mechanism is needed to harmonize application procedures across state-run Medicaid programs to get consults on a younger population with rare diseases listed for reimbursement.
  • Since resources exist in different parts of the country, it seems reasonable for each one to apply for grants (e.g. NIH R18 for Demonstration & Dissemination) to expand its service nationally. Then perhaps the resources can be linked together or duplicated to make complete packages for different regions.

P.S. The Sickle Cell Treatment Act (S. 874/H.R. 1736) became part of the American Jobs Creation Act (H.R. 4520), and was signed by the President into law on Oct. 22, 2004 (, Title VII, Sec. 712, pp. 375-385. Inclusion of Primary and Secondary Medical Strategies for Children and Adults With Sickle Cell Disease as Medical Assistance under the Medicaid Program). On November 19, 2004, the Appropriations bill allocated $200K to the Health Resources and Services Administration (HRSA) ?to establish a demonstration program and a National Coordinating Center to develop systemic mechanisms for the prevention and treatment of sickle cell disease as authorized in Section 712 of Public Law 108-357. The Sickle Cell Demonstration Program is designed to improve and expand patient and provider education and the continuity and coordination of service delivery for individuals with sickle cell disease through grants to eligible entities as provided for in the legislation. The conferees encourage HRSA to work with patient and provider organizations to develop these programs and prepare a program final plan for fiscal year 2006 within six months of enactment.? A Sickle Cell Disease Clinical Research Network is under development (RFA HL-05-006).

Invited Participants:

  • Lennette Benjamin, M.D., Dept. of Medicine, Montefiore Medical Center, Bronx, NY 10467
  • Kenneth R. Bridges, M.D., Partners HealthCare, Harvard Medical School, Cambridge, MA 02139
  • Dianne Daugherty, Malignant Hyperthermia Assoc. of the U.S. (MHAUS), Sherburne, NY 13460
  • Violet L. Dease, M.Div., Abyssinian Baptist Church, New York, NY 10030
  • James R. Eckman, M.D., Emory School of Medicine and Comprehensive Sickle Cell Center, Atlanta, GA 30303
  • Annette Galassi, R.N., M.A., Cancer Information Service, National Cancer Institute, NIH, Bethesda, MD
  • Kathryn Hassell, M.D., Colorado Sickle Cell Center, Univ. of Colorado Health Services Center, Denver, CO 80262
  • Cage Johnson, M.D., Comprehensive Sickle Cell Center, Univ. of Southern California, Los Angeles, CA 90033
  • Dorothy S. Knight, MBS, Ed.S., Consumer Response Services, Centers for Disease Control, Atlanta, GA 30333
  • Kathy Kranzfelder, M.A., National Kidney Diseases Information Clearinghouse, NIDDK, NIH, Bethesda, MD 20892-2560
  • Henry Rosenberg, M.D., CPE, MHAUS and Dept. of Medical Education, St. Barnabas Med. Center, Livingston, NJ 07039
  • Ted Wun, M.D., FACP, Dept. of Medicine, UC Davis Cancer Center, Sacramento, CA 95817

NHLBI Staff: 

  • Duane R. Bonds, M.D. 
  • Henry Chang, M.D. 
  • Greg Evans, Ph.D. 
  • Wanda Ware, Conference Coordinator