Delivering Integrated Care and Cost Management
The IHTC works collaboratively with payors to optimize care. We ensure that the patients and families we serve have access to care and therapies, thereby helping to contain costs and reduce both bleeding events and utilization of resources.
The IHTC Pharmacy’s ability to purchase clotting factor through the Public Health Service 340B discount program and our overall pricing structure benefit payors and patients by dispensing clotting factor at significantly reduced prices.
- What is Von Willebrand Disease (VWD)?
- Types of VWD
- Prevalence of VWD in the US Population
- Causes of VWD
- Signs & Symptoms of VWD
- Diagnosis of VWD
- Treatment of VWD
- Treatment Adherence in VWD Care
- Hemophilia Treatment Centers and Comprehensive Care
- IHTC’s Disease Management Program: Optimizing Patient Care While Minimizing Costs
- Special Considerations
- What Can IHTC Do for You?
VWD is the most common bleeding disorder in the general population, affecting males and females equally. Based the findings of population screening programs, it has been estimated that approximately 0.6% to 1.3% of people may be affected with some form of VWD.1 VWD is caused by deficiencies or abnormalities in the Von Willebrand factor (VWF), a large protein made up of multimers, or protein subunits. VWF binds to coagulation factor VIII (FVIII) and protects it against proteolytic degradation. In the process of hemostasis, VWF contributes to clot formation by facilitating platelet-platelet binding and platelet-endothelial cell adhesion at the injury site, leading to formation of a hemostatic plug. When VWF is defective or present in inadequate quantities, a person may exhibit symptoms such as difficulty forming a clot after an injury.
Several subtypes of VWD can occur with a variable range of severity.2 Genetic mutations in the VWF gene have been identified for some of these subtypes.
- VWD Type 1: The most common and often mildest subtype of VWD, in which VWF levels (and sometimes FVIII) levels are lower than normal;
- VWD Type 2: The second most common subtype, featuring structurally abnormal VWF, and resulting in moderately severe disease. Type 2 is further divided into smaller groups, types 2A, B, M, and N:
- Type 2A is characterized by a reduction in platelet binding due to a deficiency of high and intermediate weight VWF multimers;
- Type 2B demonstrates an actual increase in binding of VWF to platelets due to an increased affinity of VWF for the platelet glycoprotein 1b receptor, leading to rapid clearance of the platelets and loss of the high molecular weight multimers in the plasma. This results in abnormal function.
- Type 2M, a rare type of VWD, is characterized by greatly reduced platelet adhesion despite normal distribution of all multimers.
- Type 2N, which is rare, demonstrates a markedly decreased affinity for FVIII.
- VWD Type 3: The least common and most severe form of VWD, in which an individual inherits an altered gene from both parents. VWF may be almost completely absent in these patients, and they often have markedly decreased FVIII and ristocetin cofactor activity levels.
- Pseudo (or platelet-type) VWD: Clinically similar to VWD Type 2B, pseudo VWD is caused by defects in the platelet receptor for VWF rather than a defect or deficiencies of VWF. Platelet glycoprotein Ib receptor has increased affinity for VWF leading to increased binding of platelets to VWF, subsequent loss of high molecular weight multimers with altered VWF function, and increased platelet clearance.
The Centers for Disease Control and Prevention collect data on patients with bleeding disorders as part of the Universal Data Collection (UDC) Project. This database provides the prevalence of different subtypes of VWD seen among patients treated at hemophilia treatment centers in the United States. This database (accessed April 04, 2010) currently includes 5505 patients with VWD, of whom 83.5% (4597) have Type 1, 10.8% (595) have Type 2, and 5.7% (313) have Type 3.
VWD is a hereditary bleeding disorder caused by quantitative (Types 1 and 3) or qualitative abnormalities (Type 2) of the Von Willebrand Factor (VWF).3 VWD is transmitted on an autosomal (non-sex linked) chromosome, meaning that children of both genders are equally likely to inherit the gene. There are subtle differences in inheritance patterns depending on the type of VWD:
- VWD Type 1: If either parent has an altered gene resulting in VWD Type 1, there is a 50% chance that each child of that parent will inherit the VWD gene. However, for reasons not yet understood, in some cases the VWD gene is present but “non-penetrant”, meaning that it does not result in clinically evident VWD in the individual. However, there remains a 50% chance that the individual will pass the gene on to offspring who may (or may not) express VWD Type 1.
- VWD Type 2: As with Type 1, if either parent has an altered gene resulting in VWD Type 2, there is a 50% chance that the children of that parent will inherit the VWD gene. However, unlike the Type 1 gene alteration, the gene for Type 2 is always expressed when it is present, such that the person with the gene will have clinical evidence of VWD Type 2.
- VWD Type 3: The most severe form of VWD, Type 3 occurs when both parents have an altered gene resulting in some form of VWD and their child receives an altered VWD gene from each parent. When both parents have an altered gene for VWD Type 1, there is:
- 25% chance that each child will have VWD Type 3;
- 50% chance that each child will inherit a gene for VWD Type 1;
- 25% chance that each child will not inherit an altered VWD gene.
To see pictures of inheritance patterns in individuals with VWD, visit the Genetics Home Reference, a service of the US National Library of Medicine at http://ghr.nlm.nih.gov/handbook/inheritance/riskassessment.
Many of the signs and symptoms of VWD resemble those observed in patients with platelet defects. Bleeding symptoms in VWD tend to be mucocutaneous in nature. The most common symptoms in persons diagnosed with VWD include4:
- Nose-bleeding (epistaxis)
- Abnormal or easy bruising (ecchymoses or hematomas)
- Gingival bleeding
- Postoperative bleeding
- Gastrointestinal bleeding
- Urogenital tract bleeding
Patients are brought to the attention of a hematologist due to abnormal bleeding after surgical procedures such as:
- Bleeding after tonsillectomy and adenoidectomy
- Bleeding after dental extractions, such as wisdom teeth
- Bleeding after urogenital surgical procedures
Women may experience bleeding complications related to menstrual cycles and labor and delivery. Prevalent symptoms in women may include:
- Menorrhagia – abnormal, excessive and/or prolonged menstrual bleeding; a critically important clinical issue in females, affecting up to 95% of women with VWD.1,5
- Metrorrhagia – bleeding between periods
- Postpartum bleeding: Heavy bleeding and/or hemorrhage during or after labor or delivery
VWD Type 3 is characterized by a virtual absence of VWF and may share the clinical symptoms and complications associated with moderate to severe FVIII deficiency. For instance, patients with Type 3 VWD, may experience hemarthrosis (bleeding into the joints or joint spaces)3, because of associated deficiency of FVIII. For more information on FVIII deficiency (hemophilia A), click here.
If your patients with a bleeding disorder need to visit the emergency department (ED), encourage them to contact the IHTC so that the center can help them get the best possible emergency and follow-up care. Visit our webpage on Emergency Care to learn about what patients and their families should do, what they need to take to the ED, and what information they need to provide to the ED staff. Inform your patients about the IHTC’s useful Emergency Care Tips.
Accurately diagnosing VWD Type 1 can be a challenge. Many bleeding symptoms associated with VWD, including menorrhagia, are common in unaffected populations, making it difficult to differentiate between normal and abnormal bleeding. Moreover, because VWD runs in families, symptoms such as easy bruising, oral bleeding, and menorrhagia seem “normal” to family members and are therefore not recognized as potential symptoms of a bleeding disorder. Clinicians should obtain a comprehensive patient and family history to identify abnormal bleeding symptoms. In general, clinicians need to take into account 4 components:
- Personal history of bleeding
- Family history of bleeding
- Physical examination
- Laboratory testing
Initial tests commonly used to evaluate individuals for a bleeding disorder include:1,4:
- Complete blood count (CBC) and platelet count
- Prothrombin time (PT)
- Activated partial thromboplastin time (aPTT)
- Thrombin time (TT) and/or fibrinogen testing
These tests are insensitive to detect VWD; therefore, specific tests are required if the patient or family history is suspicious. 1,4:
- Platelet function (PFA-100 bleeding time)
- FVIII activity
- Ristocetin cofactor activity (VWF:RCo) – VWF functional analysis
- VWF antigen level (VWF:Ag)– VWF quantitative analysis
- VWF multimers – VWF qualitative analysis
- Blood type
Calculations such as the ratio of VWF:RCo to VWF:Ag may be helpful in the differentiation of subtypes of VWD Type 2 (2A, 2B, 2M).1 Similarly the ratio of VWF:Ag to FVIII levels may help indicate Type 2N VWD.
Establishing a diagnosis of VWD Type 1 may require repeated testing. Plasma levels of VWF can fluctuate dependent on a variety of factors, including physical or emotional stress, exercise, systemic inflammation, pregnancy, and administration of oral contraceptives. These variables have the potential to elevate VWF levels and cause a false-negative result. Establishing a diagnosis of VWD Types 2 and 3 is more straightforward.1
The Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation (NHF) advises that VWD and other inherited bleeding disorders should be considered in the differential diagnosis of all females presenting with menorrhagia.6 Project Red Flag, the NHF’s public awareness campaign, estimates that more than 2.5 million women in the United States have an undiagnosed bleeding disorder.4 Moreover, particularly in women, there is a significant delay between initial clinical symptoms and diagnosis, with symptoms starting at a median age of 12 years and diagnosis occurring at a median age of 16 years (P=0.0049).5
Treatment and management of VWD is determined by the subtype and severity of disease, as well as the patient’s age and symptoms. Prevention of bleeding is a primary treatment aim. Specific treatment options are available to increase the levels of VWF. In addition, supportive therapies can be used to prevent or control the bleeding symptoms. The main treatment options include:7,8
- Desmopressin acetate (DDAVP; Stimate) nasal spray or injection
- Synthetic hormone that increases the release of endogenous stores of VWF.
- Indicated for use in patients with Type 1 VWD and in hemophilia patients with mild factor FVIII deficiency (>5%).
- Nasal spray recommended only for patients >3 years old and weighing more than 20 kg
- To use desmopressin, patients should undergo a DDAVP challenge test to determine if they exhibit a hemostatic response, because it is not effective in all patients.
- VWF-containing concentrates (eg, Humate-P)
- These concentrates are derived from human blood that has undergone viral inactivation.
- These concentrates are administered via intravenous route to increase the level of VWF.
- Indicated for:
- patients with VWD Type 2A, 2B, 2M and 3
- Type I with severe bleeding
- patients who are unresponsive or have contraindications to desmopressin (DDAVP);
- patients undergoing major surgical procedures involving areas of fibrinolysis, such as oropharyngeal region or urogenital area
- Aminocaproic acid
- Antifibrinolytic agent indicated for use in patients with bleeding disorders
- This agent prevents the breakdown of a blood clot and helps to maintain hemostasis
- Used to treat mucous membrane bleeding
- Commonly used as an adjunctive treatment along with DDAVP/Stimate or VWF-containing concentrates
- Oral contraceptives
- Indicated for use in women whose primary symptom is menorrhagia or metrorrhagia
- Other Adjunctive therapies
- May include local fibrin glue, microfibrillar collagen, or tranexamic acid.
- Other measures that may be taken to reduce complications associated with VWD include the following:
- Advise patients to avoid medications that may increase bleeding, such as aspirin or other antiplatelet drugs
- Use precaution when administering recommended immunizations: give injections in the thigh or other large muscle group(s) using the smallest gauge needle
- No more than one immunization in one muscle group per visit
- Apply ice and pressure after immunization for 3 to 5 minutes each
- Vaccinate patients against hepatitis A and B if not immune or not previously exposed
- Advise people with bleeding disorders that they have an increased risk of exposure to blood and blood products
- Identify bleeding problems early and treat as soon as possible
- Advise patients to maintain an ideal weight and an active lifestyle to increase muscle tone and preserve joint function.
For MASAC’s recently updated recommendations on the treatment options for VWD and other bleeding disorders, please refer to http://www.hemophilia.org/NHFWeb/Resource/StaticPages/menu0/menu5/menu57/masac190.pdf.
The effectiveness of treatment of VWD depends on two important variables: the physician recommending an appropriate treatment plan for the patient’s condition, and the patient following the recommendations provided. Treatment adherence is the extent to which a patient’s behavior coincides with medical or health advice. Individuals with type 3 VWD may share the clinical symptoms and complications associated with moderate to severe hemophilia. Because individuals with type 3 VWD often require infusion therapy at home, they must be actively involved in working with physicians to plan and manage their treatment regimen.
Poor adherence to a treatment plan may reduce treatment effectiveness and contribute to long-term disability and decreased quality of life in patients with type 3 VWD. Nonadherence to on-demand/episodic therapy may result in bleeding episodes of longer duration, recurrent bleeds in the same location, joint damage and subsequent disability. Nonadherence to prophylactic therapy may cause suboptimal prevention of bleeding events and lead to the poor outcomes. Because clotting factor and other medications for the treatment of type 3 VWD are expensive it is especially important for patients to adhere to treatment recommendations to prevent complications and reduce waste of valuable medical resources.
While most healthcare providers agree that treatment adherence is an important issue in health outcomes, historically there has not been an effective way to measure treatment adherence in individuals with bleeding disorders. Different studies of individuals with hemophilia have used infusion logs, clotting factor dispensations, joint health, and patient interviews to evaluate adherence. However, none of these studies resulted in a standardized method to measure adherence to hemophilia treatment. In response to the need for a standardized adherence measure, the IHTC developed and validated two scales to measure treatment adherence – the Validated Hemophilia Regimen Treatment Adherence Scales (VERITAS) for episodic treatment (VERITAS-PRN) and for prophylactic treatment (VERITAS-Pro).
VERITAS: IHTC’s Validated Treatment Adherence Scales
Each VERITAS scale includes 24 questions divided into six 4-question subscales. The subscales are designed to measure different aspects of treatment adherence, including the dosage and timing of infusions, the patient’s tendency to forget or skip infusions, and the frequency of communication between the patient and the HTC. Using the individual subscale scores as well as the total score allows care providers to evaluate multiple dimensions of adherence and to identify areas that should be addressed to improve adherence and optimize long-term health outcomes for the patient.
The validation process involved developing these questionnaires and testing them in patient groups to demonstrate that the scales measure what they purport to measure, i.e., that they measure actual treatment adherence and not another aspect of treatment. We demonstrated that the scales reliably measure treatment adherence and represent a valuable tool for use in patient care. The scales take less than ten minutes to complete and are a simple and highly informative addition to a patient’s clinic visit and care.
Both scales were published in Haemophilia, a leading peer-reviewed journal, in early 20109,10 and have been well received in the hemophilia community. To date, the IHTC team has presented the scales at the American Society of Hematology, World Federation of Hemophilia, the International Network for Pediatric Hemophilia, and the first Conference on Blood Disorders in Public Health. The VERITAS-Pro is currently undergoing translation into Spanish, Italian, and French, has been introduced to multiple clinic settings and added to several pharmaceutical study protocols. It is our hope that the VERITAS scales will improve our understanding of the link between treatment adherence and health outcomes, and thus significantly influence our ability to help patients achieve the best long-term outcomes possible.
Please use the following links to read the VERITAS study abstracts in the journal Haemophilia.
For additional information about the tools themselves and about licensing the scales for use in research and clinical practice, please contact the IHTC disease management team at 1 317 871 0011 (ext. 273).
The federal Office of Maternal and Child Health and the Centers for Disease Control and Prevention (CDC) support a network of specialized healthcare centers dedicated to treating blood disorders.11 Currently, the network includes approximately 140 HTCs. The IHTC is Indiana’s only federally recognized HTC.
The purpose of HTCs is to prevent and reduce problems related to blood disorders by providing optimal and comprehensive care to persons with bleeding disorders, thrombosis, thrombophilia (clotting disorders), and other blood disorders. The centers use multidisciplinary clinic teams, and clinical, outreach, and education programs. The multidisciplinary team may include:
- adult and pediatric hematologists
- triage and clinic nurses
- social workers and other mental health professionals
- dental hygienists
- research coordinators
- physical therapists
The teams work closely with local healthcare providers to meet the specific needs of the population to improve their quality of life. The care provided at HTCs has been shown to significantly decrease complications for persons with hemophilia. Mortality rates are 40% lower in persons who use HTCs than in those who do not, despite the fact that the more severely affected patients are the ones who typically visit these centers.12 HTCs place a premium on preventive care because of the difficulty and expense of treating complications of bleeding disorders, which can reduce the patients’ quality of life.
The IHTC exceeds national staffing standards and offers an extensive spectrum of care and services through dedicated trained professionals. In addition to the core services provided at all HTCs (e.g., hematologists, clinical nurses, social workers, physiotherapists), the IHTC staff also includes a career counselor, risk reduction specialists, a registered dietitian, genetic counselor, dental hygienists, and clinical research professionals.
For more about the value of comprehensive care, go to IHTC’s informational brochure, “Why Attend Comprehensive Clinic?”
Since October 2004, the IHTC has administered a disease management program (DMP) for persons with bleeding disorders who are insured by Indiana’s high-risk insurance pool, the Indiana Comprehensive Health Insurance Association (ICHIA). Indiana code I.C. 27-8-10-3.5(e) mandates that ICHIA-insured individuals with chronic diseases participate in an ICHIA-approved DMP. Because of IHTC’s specialized focus on bleeding disorders and commitment to excellence in patient care, it was selected as the disease management entity for ICHIA-insured individuals with bleeding disorders.
The purpose of the DMP is to optimize patient care and health outcomes while containing or reducing costs of care. The program brings together expertise, experience, and advanced technology in a patient-focused, research-oriented approach. Included elements of the DMP are targeted toward the most accurate, effective, and efficient methods to diagnose and treat bleeding disorder complications. Through targeted interventions and enhanced quality assurance, this program is expected to result in improved patient care and cost stabilization/savings.
Patients enrolled in the program are required to do the following:
- Attend an annual comprehensive clinic at the IHTC or at an outreach location;
- Have prescriptions for clotting factor concentrate and other bleeding disorder medications signed by an IHTC physician;
- Obtain outpatient clotting factor and related products through one of the ICHIA DMP Public Health Service vendors, such as the IHTC Pharmacy Program;
- Engage in periodic telephone contact with IHTC/DMP nurses and staff regarding ongoing health issues, psychosocial concerns, and educational or occupational needs;
- Complete DMP questionnaires and assessments upon request;
- Use the ATHNadvoy web-based infusion reporting system or other approved infusion reporting system to log infusions.
Program enrollees also have the option to participate in the research component of the DMP, whereby program results may be presented to other HTCs and/or published in academic journals. Patient privacy is always an IHTC priority. Any data that are published or presented are de-identified and aggregated so that no individual patient can be linked to the information.
Evaluation of Progress
The following tools are used to evaluate program progress:
- Monthly meetings of the multidisciplinary team to review DMP patients’ health status, progress, and needs;
- Pharmacoeconomic analysis of claims data – i.e., annual insurance claims are reviewed from year to year, with a special focus on costs of care, costs of clotting factor, source of care (e.g., HTC, emergency department), and changes in costs and utilization from one year to the next;
- Annual reporting of specific cost and utilization outcomes:
- Average costs of clotting factor, medical, and hospital care;
- Average number of clotting factor units dispensed, emergency department visits, and inpatient hospital days;
- Regular patient assessments:
- Diagnosis-specific knowledge assessments – to assess a patient’s knowledge of his or her own condition, and to assess changes in knowledge over time;
- Quality of life survey – to assess a patient’s health-related quality of life, and to assess changes in quality of life over time;
- Treatment adherence scale – VERITAS-Pro or VERITAS-PRN for patients who use a home infusion regimen.
The DMP is currently in its sixth year of administration. Five years after implementation, all costs, as well as emergency department visits, have remained below pre-implementation levels. Inpatient hospital days also have remained below pre-implementation, although a few spikes in costs for medically necessary surgeries have occurred. Program patients have benefited from the focus on proactive, preventive care and personalized interventions. A major strength of the IHTC DMP is that it is administered by healthcare providers rather than a third-party disease management entity. This ensures that program staff has direct access to the multidisciplinary medical team, physicians, and pharmacy. This model strongly supports the implementation of provider-administered DMPs in managing costly chronic disease and addressing the rising healthcare costs in the United States.
The IHTC team published the initial results of its DMP in Haemophilia, a leading peer-reviewed, academic journal. To access the abstract of the published article, click here.
It is important for primary care providers, gynecologists, surgeons, and dentists to obtain a bleeding history, and particularly a history of menorrhagia, prior to surgery or a procedure. In one study,5 7 out of 27 participants with menorrhagia underwent hysterectomy to control bleeding; yet in 6 of the 7, a diagnosis of VWD was not known preoperatively despite a previous bleeding history in all.
An international group of experts on the care of women with bleeding disorders recently published a consensus statement on how to specifically identify and manage bleeding disorders in women. To download a free copy of the consensus statement, visit http://download.journals.elsevierhealth.com/pdfs/journals/0002-9378/PIIS0002937809004104.pdf
Women with VWD who become pregnant or plan to have a child should receive follow-up care from a hematologist and obstetrician who are skilled in the management of VWD. The obstetrician should work with the hematologist to develop a plan to prevent bleeding and to manage bleeding if it does occur. Pregnancies in women with VWD are often considered to be high risk; and delivery should occur at a facility with the capability to manage potential bleeding complications. The National Hemophilia Foundation’s MASAC recently updated its guidelines for perinatal management of women with bleeding disorders and carriers of hemophilia A and B. These updated guidelines may be viewed at http://www.hemophilia.org/NHFWeb/Resource/StaticPages/menu0/menu5/menu57/masac192.pdf.
Recombinant VWF is available in clinical trials but is not available for routine clinical use. This means that patients with VWD must use plasma-derived concentrates, which may have an increased risk for transmitting blood-borne infections such as hepatitis A, B or C, and HIV. However, intensive screening, testing and advanced purification processes have greatly reduced this risk. IHTC’s risk reduction specialist counsels individuals with bleeding disorders about steps they need to take to reduce or prevent complications related to their bleeding disorder or its treatment.
Full details of current guidelines for the management of VWD are available in the National Heart Lung and Blood Institute publication, “The Diagnosis, Evaluation, and Treatment of von Willebrand Disease”1. This is available to download for free at: http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. The guidelines have also been published in the journal Haemophilia.13
What Can IHTC Do for You?
- As Indiana’s only federally recognized HTC, the IHTC is committed to working with other healthcare providers throughout the state to serve Indiana’s bleeding disorders population. We are available to answer any questions you might have about VWD in general and to consult about cases of suspected VWD or other bleeding disorders.
- IHTC will provide surgery plans for patients who need a surgical procedure. We gladly take referrals to our center and, based on the benefits of receiving care at an HTC,12 we encourage providers to involve the IHTC in the overall care of persons with VWD and their families.
- The IHTC exceeds national staffing standards and offers an extensive spectrum of care and services through dedicated trained professionals. In addition to the core services provided at all HTCs (e.g., hematologists, clinical nurses, social workers, physiotherapists), the IHTC staff also includes a career counselor, a risk reduction specialist, a registered dietitian, geneticist, dental hygienists, and clinical research professionals.
- Our Public Health Service 340B IHTC Pharmacy Program benefits payors and patients by reducing the cost of treatment, coordinating care, communicating with patients and their families to manage medication issues, and helping patients enroll in patient assistance programs so that patients have access to therapies during a lapse in insurance coverage.
- Genetic counseling is available through the IHTC for individuals and families with VWD.
- Our social workers help individuals with bleeding disorders and their families handle insurance issues.
- Dental care: IHTC’s dental hygienists will work with you and your patients to make sure all the necessary precautions are taken in preparing for dental procedures and for long-term dental care. Visit IHTC’s webpage on dental care to learn more about the special considerations for dental care in persons with bleeding disorders.
- Partners in Bleeding Disorders™ Program: The Partners™ program is the longest-running curriculum-based national educational program for HTC professionals. The program offers in-person and online comprehensive education for HTC staff and is expanding to include directed educational components for HTC disciplines such as physical therapy and social work. To learn more about these professional development activities, visit the IHTC Reading Room
- Outreach comprehensive clinic: As Indiana’s only federally recognized HTC, the IHTC is dedicated to serving persons with bleeding disorders throughout the state. In addition to seeing patients at our clinic, centrally located in Indianapolis, IHTC’s multidisciplinary medical team travels to several Indiana communities each year to conduct comprehensive and follow-up clinics for patients living in other parts of the state.
- IHTC partners with Hemophilia of Indiana Inc. (HII) in hosting Camp Brave Eagle, Indiana’s summer camp for children with bleeding disorders. The week-long camp is open to all Indiana children with bleeding disorders, as well as their siblings, ages 7–15 years. For more information on this camp, please refer them to Reading Room or www.campbraveeagle.org (Camp Brave Eagle website).
- The Doug Thompson Teen Leadership Program, a program of the IHTC and HII, provides teen members of the bleeding disorder community an opportunity to develop and refine life and leadership skills while participating in an adventure camp experience. Teen camp teaches young men how to mentally and physically prepare for nearly anything, while affirming the choice to not participate in activities that may put them at risk mentally or physically. For photos of the Thompson Outpost Teen Camp, see IHTC’s Summer 2014 newsletter in our Reading Room.
- To answer additional questions that your patients may have about VWD care, please see IHTC’s VWD FAQs.
Project Red Flag, National Hemophilia Foundation’s public awareness campaign for women with bleeding disorders.
- National Heart Lung and Blood Institute. The Diagnosis, Evaluation and Management of von Willebrand Disease. 2006. (Available at http://www.nhlbi.nih.gov/guidelines/vwd/3_diagnosisandevaluation.htm.). Accessed April 15, 2010.
- Lillicrap D. Von Willebrand disease–Phenotype versus genotype: Deficiency versus disease. Thrombosis Research 2007;120:S11-S6.
- Sadler J. von Willebrand Factor. The Journal of Biological Chemistry 1991;266:22777-80.
- National Hemophilia Foundation. Project Red Flag: Real Talk About Women’s Bleeding Disorders. 2009. (Available at http://www.hemophilia.org/projectredflag/index.htm.). Accessed April 15, 2010.
- Ragni M, Bontempo F, Hassett A. von Willebrand disease and bleeding in women. Haemophilia 1999;5:313-317.
- National Hemophilia Foundation. Medical and Scientific Advisory Council (MASAC). MASAC Recommendation #185: Regarding Women with Inherited Bleeding Disorders. (Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=1192.). Accessed April 15, 2010.
- National Hemophilia Foundation. MASAC Recommendation #186: Regarding the Treatment of Von Willebrand Disease. 2008. (Available at http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=57&contentid=689.). Accessed April 15, 2010.
- Berntorp E, Abshire T. The von Willebrand Disease Prophylaxis Network (vWD PN): Exploring a treatment concept. Thrombosis Research 2006;118:S19-S22.
- Duncan NA, Kronenberger WG, Roberson CP, Shapiro AD. VERITAS-PRN: a new measure of adherence to episodic treatment regimens in haemophilia. Haemophilia. 2010;16:47-53.
- Duncan NA, Kronenberger WG, Roberson CP, Shapiro AD. VERITAS-Pro: a new measure of adherence to prophylactic regimens in haemophilia. Haemophilia. 2010;16:247-255.
- Centers for Disease Control and Prevention. Blood Disorders: Specialized Health Care. 2009. (Available at http://www.cdc.gov/ncbddd/blooddisorders/treatment.html.). (Accessed April 15, 2010.)
- Soucie J, Nuss R, Evatt B, Abdelhak A, Cowan L, Hill H, et al. Mortality among males with hemophilia: relations with source of medical care. Blood 2000;96:437-442.
- Nichols W, Hultin M, James A, Manco-Johnson M, Montgomery R, Ortel T, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008;14:171-232.