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  • Hemophilia Treatment Centers 101
    day long helping families with everything from how to open a factor bottle to deciding whether a child needs to be seen she says Cohen s HTC provides emergency care during office hours A physician is on call 24 hours a day to address concerns or coordinate treatment when a person is en route to the emergency room ER The HTC also makes sure patients have clotting factor on hand at home in case they need to use it in an ER They can bring factor with them because sometimes hospitals won t have clotting factor on the shelf to give to them says Cohen Some HTCs are portable serving patients via temporary traveling clinics in local hospitals before moving on If you re planning a trip your HTC staff can contact their counterparts in other parts of the country or world to let them know you ll be traveling there and may need assistance Education Emphasis HTCs provide more than just treatment Cohen says One of the major roles of the HTCs is education she explains When children begin prophylaxis treatment for example HTCs will teach parents and caregivers and eventually the children how to infuse themselves so they can treat at home This can save time reduce discomfort and increase convenience during a bleed because patients don t have to travel to an emergency room and wait she adds Steps for Living Treat Responsibly Today for a Healthy Tomorrow The educational role extends beyond the patient and family HTC staff often visit schools explaining to teachers staff and school nurses what hemophilia is what activities a child can engage in and how to recognize an emergency This comprehensive approach to care does more than improve the quality of life for people with bleeding disorders According to studies by the CDC people with hemophilia are 40 less likely to be hospitalized for bleeding complications if they use HTCs rather than receiving care elsewhere see Learn More Those who use HTCs are also 40 less likely to die of hemophilia related complications The comprehensive care approach is so effective that the federal government is considering HTCs as a model for care for diabetes sickle cell anemia and other diseases The new buzzword on Capitol Hill is medical homes something individuals with bleeding disorders have long enjoyed Psychosocial Needs HTCs also address psychosocial needs We re there to make sure the whole person is taken into account all the time says Mary Jane Berry MSW a clinical social worker at the Georgetown University Hemophilia and Thrombosis Center in Washington DC During the comprehensive care clinic appointment Berry schedules a short visit with each family to introduce herself and ask if the family needs help with insurance matters She also screens patients about their coping strategies pain management and any job related marital or other problem If parents are worried about how to manage their insurance copayment for example Berry can connect them to their state s Children s Health Insurance Program

    Original URL path: http://www.hemaware.org/print/564 (2016-02-18)
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  • Research Progress Helps Entire Community
    many of our centers and consumers are involved in a federally funded project trying to determine if having hemophilia is protective against heart attacks Amazing For those of us providing services to people affected by hemophilia and other bleeding and clotting disorders we know this is an exciting time But there are still obstacles such as dwindling federal research support Although researchers receive money from various government sources federally funded research into bleeding disorders still does not receive sufficient priority based on the impact our conditions have on the federal budget Further basic science research such as that funded by the National Hemophilia Foundation s NHF s Judith Graham Pool JGP Fellowship Award and the Career Development Award CDA drives innovation See Banding Together page 20 Without the support of foundations such as NHF some of our most exciting clinically significant new products might never have moved from the drawing board to the lab and to the pharmacy To evaluate the success of a funding source look no further than the impact of those who have received career funding from NHF Seven current hemophilia treatment center HTC physicians have been awarded the JGP Fellowship Award Another 17 awardees have contributed

    Original URL path: http://www.hemaware.org/print/1013 (2016-02-18)
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  • La barrera para portadoras
    jugábamos afuera todo el tiempo y siempre teníamos moretones dijo Danielle La madre de las niñas Vickie una enfermera neonatal le preguntaba al pediatra constantemente si sus hijas tenían hemofilia B como su padre Me insistía que No las mujeres no tienen hemofilia dijo Vickie Cinco años después Danielle sangró durante 10 días cuando le extrajeron cuatro dientes Su ortodoncista y cirujano dental intuyeron que algo pasaba y la remitieron al centro para el tratamiento de la hemofilia HTC en Cleveland Los exámenes del nivel del factor confirmaron la corazonada de Vickie El nivel de mi factor era 8 o 9 cuando me diagnosticaron hemofilia B leve dijo Danielle El de Heather era de aproximadamente 40 Esos diagnósticos tomaron más de una década Durante la Reunión Anual de la NHF en Chicago en noviembre del 2011 Koerper y Vickie realizaron una sesión educativa para mujeres con hemofilia leve Muchas mujeres contaron experiencias similares Ellas sabían que estaban sangrando demasiado dijo Koerper Pero sus doctores no prestaron atención a sus inquietudes y le dijeron Tienes un moretón enorme porque te golpeaste la pierna Cuando se someten a cirugía sin un plan de tratamiento surgen los problemas Les sacan la vesícula y se presentan complicaciones hemorrágicas dijo Koerper Davenport le advirtió a su nuevo hematólogo quien no tenía experiencia en el tratamiento de mujeres con trastornos hemorrágicos que el nivel de su factor tal vez se iba a elevar por el estrés que tenía antes de una histerectomía pero que luego disminuiría mucho Como el nivel de su factor se elevó a 80 el hematólogo no tomó precauciones Davenport se despertó en la sala de recuperación y escuchó que su médico le decía al personal que no sabía lo que estaba causando la hemorragia porque ella no tenía hemofilia Cuando te llaman portadora sintomática y así te etiquetan hay la tendencia a no tomarte en serio dijo Davenport Para ella el resultado fue una hemorragia después de la operación Para otras puede significar daño articular y artritis por hemorragias que no recibieron tratamiento o complicaciones graves durante el parto Efecto emocional y en la conducta Se documentaron las consecuencias emocionales y conductuales de las mujeres con hemofilia leve que notaron errores en su atención médica en un estudio publicado en Haemophilia que realizaron en el 2011 Nisa Renault e investigadores de la Universidad Dalhousie en Halifax Nueva Escocia Canadá Las 11 mujeres entrevistadas dieron 264 reacciones negativas incluidas ira duda y desconfianza La incredulidad del doctor puede causar que la paciente se decepcione Es frustrante explicarles esto a los médicos pues espero que sepan algo sobre trastornos hemorrágicos dijo Vickie Para Davenport fue un shock cuando su médico negó que esta tenía hemofilia tras su histerectomía Fue muy duro para mí dudar de un hecho en el que creía desde hacía 35 años Simplemente no estaba bien dijo El dolor fue profundo pues Davenport perdió una conexión familiar que le proporcionaba consuelo Eso me quitó mi identidad todo lo que tenía de

    Original URL path: http://www.hemaware.org/print/1120 (2016-02-18)
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  • Test Takers
    the extensive screening to come If the prescribing doctor suspects the hemophilia was caused by an inversion of the genetic code the lab tech places the strands of DNA on a gel and passes an electronic current through it to separate the size of the fragments How the strands break into pieces determines whether an inversion is present says Miller If an inversion is not suspected or known the lab tech applies primers manmade polymers that attach to different portions of the DNA double helix to isolate the part where the mutation might exist The genetic mutation is identified when that section of DNA is compared with a normal section of DNA Deciding Whether to Test All women do not need to be tested to confirm their hemophilia carrier status Enid Carter 43 a massage therapist from Dearborn Heights Michigan knew she was an obligate carrier the daughter of a man with hemophilia Daughters of men with hemophilia are carriers or have hemophilia themselves Still she felt the test was important to explain why she had blood in her urine which turned out to be from bleeding in her kidney Further she was able to determine the mutation for her entire family line I realized as an obligate carrier the testing was valuable for those I might pass the information along to she says As women we really need to start writing our own histories down Other women who may want to have a genetic test include those with a family history of bleeding disorders who are considering pregnancy or who are already pregnant Nuñez says Known symptomatic carriers with no family history may also want to know their mutation according to Hemophilia A Practical Approach to Genetic Testing The article was published in Mayo Clinic Proceedings in 2005 by Rajiv Pruthi MBBS associate professor of hematology at the Mayo Clinic in Rochester Minnesota In symptomatic carriers with no family history of hemophilia and thus no information on clinical phenotype genotyping of the carrier and or fetus or child can predict severity of the disease Pruthi wrote This is also true for adopted women who are carriers and unaware of their family history What s more some women can have normal factor levels but still experience bleeding symptoms says Danielle Nance MD fellow in hemostasis and thrombosis at the Puget Sound Blood Center and University of Washington in Seattle She is a symptomatic carrier from a spontaneous mutation and mother of a son with severe hemophilia A A March 2011 study in the journal Haemophilia found that women who are carriers of severe mutations inversions or deletions in their FVIII genetic code are two to three times more likely to have bleeding problems even if their FVIII levels are in the normal range of 40 to 80 That result wasn t something we expected says Nance We always knew that if a woman s factor VIII level was below 30 or 35 she had an increased chance of bleeding Now

    Original URL path: http://www.hemaware.org/print/788 (2016-02-18)
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  • The Benefits Of Knowing Your Genotype
    one missense One of the mutations which results in the absence of FXI is also a risk factor for the development of antibodies if FXI is administered as plasma or FXI concentrate says Paula Bolton Maggs DM consultant hematologist Department of Clinical Haematology Manchester Royal Infirmary UK Family Planning Women who are carriers of hemophilia A or B can also benefit from genotyping In the past a variety of carrier testing methods were used with varying degrees of accuracy says Bolton Maggs DNA analysis is now the gold standard as in the majority of cases it can give a definite answer she says Since the same mutation runs in the family the genotyping for the index patient can help with other family members in terms of family planning says Soucie See Family Planning for Carriers HemAware November December 2007 p 50 Only one region of the gene then needs to be evaluated he says making the testing simpler less expensive and more targeted Val D Bias NHF s CEO is an index patient As an individual with a bleeding disorder and having had my own genotyping done when carriers in my family have become pregnant they have been able to test against my genotype and figure out whether they re carrying a child with a bleeding disorder Knowing whether a pregnant woman is carrying a baby boy with hemophilia helps the labor and delivery team modify its plan See Pregnant Pause HemAware Winter 2010 National Initiative All that s needed for genotyping is a blood sample which is sent to a lab that can analyze mutations using gene sequencing or sequence analysis The first test determines the presence of the most common types of mutations insertions missense or nonsense If these types of mutations are absent a second test is performed to locate deletions or duplications Results are obtained within three to eight weeks and sent to the patient s physician or hematologist In the near future NHF expects to offer free genotyping for all people with bleeding disorders in the US Some of the donations given to its new fundraising campaign Research Is Our Future will be earmarked for genotyping As the funds come in we will make the testing available to patients through a network of labs nationally Bias says He estimates it will take three to five years to complete the project NHF is consulting with the Division of Blood Disorders at the CDC as it develops this project Before genotyping gets under way though NHF and the CDC will take time to troubleshoot We are working with the CDC to address some of the ethical and informational issues Bias says Other nonprofits that have genotyped their populations such as people with cystic fibrosis will be consulted as will the Genetic Alliance a nonprofit health advocacy organization One of the challenges is to protect patient information Bias says For patients who develop an inhibitor during a clinical trial the US Food and Drug Administration FDA can penalize

    Original URL path: http://www.hemaware.org/print/469 (2016-02-18)
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  • Mild Hemophilia
    then we do the cord blood testing on those infants just like we would on a known severe patient says Geraghty When there is no prior history a partial thromboplastin time test PTT can be ordered a preliminary measure of the time it takes blood to clot For a patient who was presenting with bleeding the PTT would be part of a bleeding evaluation says Christine Kempton MD MSc assistant professor Aflac Cancer Center and Blood Disorders Service of Children s Healthcare of Atlanta and Department of Hematology Oncology Emory University in Atlanta A normal PTT doesn t exclude mild FVIII deficiency as different PTTs reagents may be insensitive to very mild FVIII deficiencies The normal range can vary by lab she says According to the Merck Manual a typical PTT is 28 34 seconds In Kempton s lab the upper limit is 39 seconds When Johnson was in the ER his PTT was 53 Factor assays are more diagnostic The FVIII activity assay will give you their FVIII level and tell you whether that s low or not Kempton says If the level is low and there is a family history of mild hemophilia the diagnosis is confirmed However for a new diagnosis the doctor may want to differentiate the low factor level from other causes For instance patients with von Willebrand disease type 2N also have low FVIII levels as do those with combined FV FVIII deficiency See sidebar Combined FV FVIII Deficiency in FV Deficiency Low FIX levels on the other hand can be caused by vitamin K deficiencies Treatment DDAVP desmopressin acetate a synthetic version of the natural antidiuretic hormone vasopressin is often the product of choice for patients with mild hemophilia A DDAVP is not recommended for patients with mild hemophilia B It produces a rapid three fold increase in circulating FVIII and von Willebrand factor VWF Plus it comes in a handy nasal spray called Stimate DDAVP is used pre and post operatively for joint and muscle bleeds and for mucous membrane bleeds in the nose and mouth But DDAVP Stimate has drawbacks Usually mild hemophilia patients whose levels are greater than 5 but below 10 are not good candidates for DDAVP Stimate Geraghty says However some patients in this range will respond to Stimate DDAVP and their physician will recommend it following a challenge The Stimate challenge involves exposing the patient to a sample dose to see how the body s VWF level changes later in the day and determine if the patient is a candidate for it In rare circumstances and in young children DDAVP causes the body to retain fluid resulting in hyponatremia an abnormally low concentration of sodium in the blood Further its effectiveness decreases after a few doses Infusions of factor product are sometimes needed for patients with mild hemophilia A or B For big surgeries including hysterectomies or any other abdominal surgery heart surgery brain or spinal cord surgery or joint replacements most times I would want to

    Original URL path: http://www.hemaware.org/print/567 (2016-02-18)
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  • Where the Girls Are
    They all felt like that week at camp is a wonderful opportunity to talk with nurses or counselors and older girls with bleeding disorders to help them understand Infusion of Girl Power One of the complications of having a bleeding disorder is menorrhagia long and heavy periods It s a topic that just about every girl avoids discussing But it s essential for girls with bleeding disorders to learn to talk about their periods and find out how best to treat them For instance many young women use hormone therapy to regulate their periods Others infuse around their periods to prevent excessive bleeding All girls should learn to infuse for themselves if they need it but if not for their loved ones says Marion A Koerper MD former director of the Hemophilia Treatment Center at University of California San Francisco and co founder of Camp Hemotion the northern California bleeding disorders camp Camp has two important functions education and empowerment says Koerper who is also NHF s medical advisor Once girls understand their disease they take control of it Even though not every girl needs to self infuse we let all the girls who want to learn how Changing Times At the annual meeting of the National Association of Camps for Children with Hemophilia and Other Bleeding Disorders NACCHO this year Shelly Mattson 26 who has VWD gave a presentation about how camp counselors can talk to girls about their bleeding disorders and their periods Her advice Make it private listen more and try to have an adult woman with a bleeding disorder there to share her experience NHF s Victory for Women Task Force is creating a toolkit to help camp staff talk to girls about their periods Campers themselves are taking action too Last year when Cecil was

    Original URL path: http://www.hemaware.org/print/889 (2016-02-18)
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  • College Health Project
    of 1 243 women ages 18 to 24 across the country researchers asked where they would seek care if they thought they had symptoms of a bleeding disorder Approximately 20 said they would seek care at a college health clinic Further of the 2 of respondents who had been diagnosed with a bleeding disorder 24 had received care at a college health clinic Based on those results NHF organizers targeted their efforts accordingly Young women with symptoms often turn to their college health clinics for help so that seemed a good place to start says Thomas The NHF CDC College Health Project targeted women s health clinics on four college campuses Florida International University FIU and University of Miami UM both in Miami Michigan State University MSU in East Lansing and University of Pennsylvania in Philadelphia HTC staff facilitated training for providers at each college health site The training included the basics of bleeding disorders challenges faced by women with bleeding disorders and assessing women with specific symptoms of a bleeding disorder NHF provided pretests and posttests that measured changes in the college health providers knowledge After the educational training sessions personnel at the college health sites were asked if they had adopted any new policies for screening women for bleeding disorders They were also asked if they had witnessed an increase in identified women who had then been referred to the HTC near campus for further testing Reaping results One of the most important results of the project was simply building connections between the college health clinics and the HTCs nearby Many HTCs are located on or near college campuses But this type of education and interaction with college health services had not been done before on the campuses we contacted says Patti Rhynders PhD MPH MCHES She is

    Original URL path: http://www.hemaware.org/print/1303 (2016-02-18)
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