Comprehensive Hemophilia Care Region VI - Arkansas, Louisiana, Oklahoma, Texas |
North Texas Hemophilia and Thrombophilia Center - Pediatric Program Children's Medical Center Hematology/Oncology Clinics 1935 Motor Street Dallas, TX 75235 http://www.childrens.com/Specialties/template.cfm?groupid=1&pageid=157 I.Introduction: The Hemophilia and Thrombosis Program at Children's Medical Center Dallas (CMCD) and University of Texas Southwestern Medical Center at Dallas (UT Southwestern) has as its missions patient care, education, research, and advocacy regarding inherited hemorrhagic disorders and thrombosis in infants, children, and adolescents. II.Facilities: The Pediatric Hemophilia and Thrombosis Program is centered at CMCD and UT Southwestern, which are adjacent facilities on the Southwestern Medical Center campus, three miles northwest of the central business district of Dallas, Texas. CMCD (Children's) is a 406 bed academic children's hospital, the only tertiary care pediatric medical center in the northern half of the State of Texas, serving a total population of approximately 7 million. Most of the program's activities take place in Children's outpatient clinic and an adjacent building which is the site of the administrative offices for all but one of the physicians, as well as for nurses, social worker, secretary, and research coordinator. The program's databases are maintained there as well. Outpatient clinics focusing on hemophilia and thrombotic disorders are held every Wednesday morning and afternoon, respectively. Further details are described below. For urgent as well as scheduled visits between the formal comprehensive clinic sessions, the Hematology-Oncology outpatient unit is employed. This facility is shared with other hematology programs in the Center for Cancer and Blood Disorders. Additional outpatient areas frequently utilized by our patients include Children's emergency and physical therapy departments. Hospitalized patients are generally admitted to C10, the 22 bed inpatient unit for the Center for Cancer and Blood Disorders. Children with thrombosis or bleeding disorders whom program members see in consultation are usually hospitalized on other services. The Program's facilities at UT Southwestern include a comprehensive multi- institutional hemostasis laboratory, administrative offices (for the program director and his staff), and the companion Adult Hemophilia Program, described briefly below. III.Patients: The Program is the major provider of diagnostic and management services for children and their families with suspected or confirmed hereditary hemorrhagic disorders and thrombosis in metropolitan Dallas and throughout north and northeast Texas. The Table below summarizes patient statistics, both overall as well as those seen during 2007. A major program activity is evaluation of children suspected as having a bleeding disorder on the basis of a positive family history of hemorrhage, signs or symptoms suggestive of a bleeding disorder, or laboratory abnormalities uncovered unexpectively, often prior to scheduled surgical procedures. Two or three such children are typically seen each week. More than half are found not to have a bleeding problem, usually much to the relief of the parents and the referring physicians. Those found to have a probable or definite condition receive further evaluation, and their parents and their referring physicians are provided with detailed diagnostic and management recommendations. Many of these children then become regular patients followed in the program. Another group of new patients are children with existing diagnoses who move to Dallas from elsewhere. Established program patients are usually seen for comprehensive assessments annually (or more often if necessary), although some with milder disorders are seen less frequently. For acute care, patients are evaluated in the regular weekly clinic, ambulatory facility, or emergency department, as needed. The nurse coordinators, who know most of these patients extremely well, are available during working hours to coordinate care. Hematology/Oncology fellows and faculty are on call during nights and week-ends. The same staff is involved in the care of children with confirmed or suspected thrombosis. These are generally patients in one of two categories. First are those, often with an underlying disorder, who are seen in consultation as inpatients at the time of their thrombotic event and subsequently followed while receiving anticoagulation and thereafter by the Hemophilia and Thrombosis Program. The other group of children, often without a personal history, have a parent or another relative with a suspected or documented hypercoagulable state. Many such patients are seen only one or twice in consultation for purposes of diagnosis and management recommendations. IV:Personnel: The program personnel consist of a core multidisciplinary team of healthcare professionals who devote a substantial part of their activity to the Hemophilia and Thrombosis Program, as well as a group of collaborators and consultants. There are four program physicians. Dr. George R. Buchanan, the Program Director, and Professor of Pediatrics UT Southwestern, also serves as overall director of the Center for Cancer and Blood Disorders, the Hematology-Oncology Program at Children's. His primary interests are hemophilia and immune thrombocytopenic purpura. Dr. Janna Journeycake is Assistant Professor of Pediatrics and Associate Director of the Program, and a former Clinical Research Scholar at UT Southwestern. Her primary interests are diagnosis and management of thrombosis, and her research is supported by an NIH K23 award. Dr. Shelley Crary, Assistant Professor of Pediatrics, has research interests in thrombosis in children with infection and following surgical removal of the spleen. She is a Clinical Research Scholar at UT Southwestern supported by a CTSA grant. Dr. Cindy Neunert also an Assistant Professor, has research interests in hemophilia , immune thrombocytopenic purpura and is also a Clinical Research Scholar at UT Southwestern. All four of the physicians are involved broadly in diverse aspects of the patient care program. Other members of the core team include the nurse coordinators, Kim Spencer is an Advanced Practice Nurse with 14 years of experience in hemophilia. She works full time with the patients and families with hemophilia and other hereditary bleeding disorders and is also involved in research and advocacy regionally and nationally. A second nurse coordinator position is currently vacant. Amanda Cabrera offers comprehensive social work support to the program's patients and their families (as well as to children with cancer). Alice Anderson is a physical therapist nationally known for her expertise in prevention and treatment of musculoskeletal disease. Paula Mason is the program's secretary in charge of appointments, telephone communications, correspondence and reports. Roberto Torres, is a clinical research associate (research coordinator) responsible for maintaining the Lab Tracker database, other databases, and accessing, analyzing, and reporting research data. Many other individuals are directly involved with the management of children with hemophilia and thrombotic disorders. They include physicians (including the 17 other faculty members and 10 fellows in the Hematology-Oncology program at Children's Medical Center Dallas and UT Southwestern), Dr. Maureen Finnegan (orthopedic surgeon with expertise in arthroscopic synovectomy and other surgical approaches to hemophilia patients), the staff at Children's Medical Center's Dental Clinic, genetics counselor Gail Brookshire, and the expert personnel in the hemostasis laboratory at UT Southwestern and Children's. Finally, Ann Fillmore, Dr. Buchanan's lead administrator at UT Southwestern, provides expert oversight of the hemophilia grant from the CDC and MCHB and the necessary subcontracts. Virtually all program members interact on a daily basis regarding patient care, as well as research, education, and administrative issues. In addition, a formal weekly conference is held to review patients seen during the prior week, prepare for patients scheduled for the subsequent week, and discuss other children requiring attention. Emphasis is placed on psychosocial, financial, musculoskeletal, and research issues, thus engaging the participation of all hemophilia and thrombosis team members. Another regular meeting is the hemostasis laboratory conference held weekly in a nearby UT Southwestern classroom. This conference is one of several means of assuring interaction between the pediatric and adult hemophilia programs and the diagnostic laboratory that currently serves both. Special meetings and conferences are also held periodically with Texas Central Hemophilia Chapter officers, pharmaceutical representatives, and others. Annual site visits of the program are conducted by the Region VI coordinator. Hemophilia and Thrombosis program staff members participate in diverse conferences outside of the institution for networking and continuing medical education. These include the annual meeting of the National Hemophilia Foundation, the annual meeting of Hemophilia Region VI providers, and meetings of professional organizations such as Hemophilia and Thrombosis Research Society and American Society of Hematology. V.Linkages and Liaisons: The Hemophilia and Thrombosis Program at Children's has a number of interactive linkages with other personnel and programs in the medical center, greater Dallas-Fort Worth community, the state, and other regional and national programs. These are now briefly described: "Center for Cancer and Blood Disorders(CCBD)- The Hemophilia and Thrombosis Program is an integral component or sub-program in Center for Cancer and Blood Disorders, the nationally known Hematology-Oncology program at Children's and UT Southwestern. "Adult Hemophilia Program- The medical center's Adult Hemophilia Program has for years assumed the care of the majority of pediatric program patients once they reach 18 -19 years of age. This companion program, directed by Dr. Cynthia Rutherford, Professor of Internal Medicine at UT Southwestern, includes a second faculty hematologist (Dr. Yu-Min Shen), a full time nurse coordinator (Patricia Dunnagan), administrative coordinator (Nona Capers), and part-time social worker (Katherine Lipsky), as well as other participating providers. The personnel of the Pediatric and Adult Programs are in contact with one another regularly regarding individual patients, as well as policies and procedures. Quarterly meetings between the Pediatric and Adult Programs ensure interactions and coordination. An effective transitioning program for adolescents entering the adult hemophilia arena includes several members of the adult team ( usually the nurse coordinator and social worker) attending the patient's last pediatric clinic visit. "Other Regional Partners- Our program interacts closely with other Hemophilia Centers in Region VI, including Fort Worth, San Antonio, Houston, Oklahoma City, Little Rock, and New Orleans. Areas of interchange include sharing of patient information (since children with hemophilia often move from one regional center to another ), and working together in areas such as Camp Ailihpomeh, regional meetings, and retreats and working groups involving nursing and psychosocial staffs. "National Hemophilia Foundation- The program has a close working relationship with the Texas Central Chapter of the National Hemophilia Foundation, many of whose officers are the parents of our pediatric patients. The chapter and Hemophilia and Thrombosis Program carry out a number of collegial educational, fund raising, and advocacy activities. The Program is also involved with NHF at the national level, with several team members attending each annual NHF meeting and serving as speakers at meetings and on national committees and task forces. "Camp Ailihpomeh- A program highlight is Camp Ailihpomeh, a one week camping experience for children with hemophilia and related disorders held each July, 90 miles southwest of Dallas at Camp John Marc. Twenty five to thirty Dallas program patients attend each year. Several members of our team are on the Camp Ailihpomeh board. Each year the Camp serves approximately 120 children from all hemophilia centers in Texas. This camp is a joint project of all of the hemophilia treatment centers in the state. "Research Collaborations- Research is a major objective of the program. Each of the physicians and several of the other personnel are involved in local institutional and multi-center collaborative research projects, described more fully below. Research results are often presented at annual meetings of organizations such as the American Society of Hematology, American Society of Pediatric Hematology-Oncology, Hemophilia and Thrombosis Research Society, and International Society of Hemostasis and Thrombosis. Program physicians are active members of each of these organizations. VI.Research: A number of investigator initiated and collaborative research projects are being conducted in the program, and they are now briefly described: "Central Venous Catheter Associated Infection and Thrombosis- For over a decade the program's investigators (Drs. Buchanan and Journeycake and Kim Spencer, RN, CPNS) have been national leaders in characterizing the etiology, risk factors, and outcomes of central venous catheter related infection and thrombosis in patients with hemophilia and cancer. Numerous presentations and publications have resulted from the efforts of this research team, which is currently being led by Dr. Journeycake. "Post-Thrombotic Syndrome Measurement Instrument- One risk of catheter related thrombosis is the development of post-thrombotic syndrome. Program investigators have developed and recently published details regarding the first instrument to quantitatively measure this complication. "Arthroscopic Synovectomy- For the past 17 years the program has been known for its interest and expertise in this procedure for children with hemophilic arthropathy. Our publication describes one of the largest series of such patient,s and we continue to collect data on the efficacy of this approach. "Rituximab for Inhibitor Patients - The TMH Clinical Trials Network of the National Institutes of Health has opened a multicenter study of rituximab in hemophilia patients with refractory high titer inhibitors. Dr. Journeycake is the program's principal investigator for this important study, which is currently ongoing. "Other Thrombosis Research- Our physicians are conducting a variety of research studies involving the identification and management of thrombosis in various pediatric patient populations. Current research areas include a prospective study of catheter-related thrombosis in pediatric cancer patients who experience catheter occlusion and/or bacteremia, and study of thrombosis in individuals with hereditary spherocytosis and other conditions for which splenectomy may be performed. "Alliance of Four- All of the physicians in the Program are actively involved in the "Alliance of Four," an informal research group consisting of experienced and junior investigators in our center and at The Hospital for Sick Children (Toronto), Children's Hospital of Philadelphia, and Emory University (Atlanta). The Alliance meets on an annual basis to share research ideas. VII.Program Financial Support: The Hemophilia and Thrombosis Program at CMCD and UT Southwestern receives financial support from a number of sources. The major expense of the program is the salaries of program personnel. The primary source of such support for the physicians are fees collected for professional services that are rendered, through UT Southwestern's Medical Service Plan. Salaries of program personnel at Children's are covered primarily by hospital operating funds derived from general revenues. The institution has a 340B program that also generates income for the program. Another important source of program revenue is grant support through the federally funded comprehensive hemophilia program network. This support is derived from the Centers for Disease Control and Prevention (CDC) and from the Maternal and Child Health Bureau (MCHB) of the Health Resources and Services Administration(HRSA). These two Hemophilia Center grants are administered in Region VI by the Gulf States Hemophilia Center in Houston. Funds are then subcontracted to the "North Texas Comprehensive Hemophilia Center," the official name of the combined pediatric and adult programs at UT Southwestern. Approximately half of the grant money directed to Dallas supports the pediatric program and the adult program. The Pediatric Hemophilia and Thrombosis Program also receives research grant support in the form of NIH K23 Mentored Career Development Award to Dr. Journeycake NIH -supported Career Development Awards supporting Dr. Neunert and Dr. Crary,as part of UT Southwestern CTSA a T32 institutional training grant with (Dr. Buchanan as principal investigator), and a grant from the National Heart, Lung, and Blood Institute supporting our institution's participation in the TMH Clinical Trials Network. VIII.Summary and Future Plans: Early in 2008 the program moved into a new clinic facility which increased the amount of space and enhanced patient flow and privacy. Steps have also been taken recently to initiate a major expansion program of both the inpatient and outpatients units during the next several years. In addition, Children's Medical Center has opened a new facility clinic and inpatient unit at Legacy in Plano approximately 25 miles north of the main campus near downtown Dallas. Patients from the northern suburbs referred for hematologic disorders, including hemophilia and thrombosis, can now be seen there rather than at the main campus. In 2009 we hope to begin periodic hemophilia-thrombosis clinics in the Legacy facility. Other plans for 2008 include a Saturday educational and networking meeting for the parents of our hemophilia patients, co-sponsored with the Texas Central Chapter. V.Program Statistics: (see attached) 2003 2004 2005 2006 2007 New Patients Evaluated 88 81 75 77 74 von Willebrand disease 4 7 10 6 6 Factor VIII deficiency 15 11 13 12 6 Factor IX deficiency 2 4 2 3 0 Bleeding disorder ruled out 6 59 45 52 57 Other 6 0 3 3 5 Hemophilia Patients in Database 246 323 331 324 336 Active Patients 157 158 155 157 160 Comprehensive Visits 117 139 154 169 150 Laboratory Evaluations 34 62 81 53 22 Education Visits 23 18 21 27 16 Acute Visits/Treatments 165 199 186 109 64 INPATIENT STATISTICS Day Surgery Procedures 16 7 10 14 14 Dental Rehabilitation 4 0 3 2 2 Port Procedures 7 5 3 4 7 T&A 1 1 0 2 0 Other 4 1 4 6 5 Inpatient Admissions 19 35 37 27 30 Control of Bleeding/Pain 13 27 27 19 15 Synovectomy 2 3 1 3 2 Port Procedures 0 1 2 2 3 T&A 0 1 3 0 0 Miscellaneous 4 3 4 3 10 THROMBOSIS PROGRAM STATISTICS Outpatient New Patient Evaluation 2006 2007 Acute Thrombotic event (DVT/Stroke) 34 44 Acute Thrombophilia No Clot 12 9 Ambulatory Follow-up Visits 94 23 Anticoagulation lab (CMCD) 34 181 Total Active Patients 7 5 Inpatient Consults 31 N/A Acute venous thrombosis 21 N/A Stroke 0 12 Anticoagulation 7 N/A Thrombophilia evaluation 3 N/A This summary was updated by hemophilia and thrombosis program staff at Children's and UT Southwestern on September 24, 2008. |