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  • Osteoporosis In Kids With Bleeding Disorders
    region of their vertebrae The most likely explanation for the decreased bone density was lack of physical activity specifically weight bearing exercise Screening for osteoporosis was recommended for at risk children with hemophilia who had joint damage from bleeds Patients with inhibitors antibodies to infused factor product are at higher risk for osteoporosis Because their bleeding is often not well controlled they can develop target joints The synovium the membrane lining the joint becomes irritated by the influx of iron and other components of the blood This can lead to synovitis a chronic condition in which the synovium becomes inflamed and thickened prone to even more bleeding Synovitis in turn can impair bone growth Chronic inflammation especially from synovitis can cause the release of tissue necrosis factor or TNF alpha says Bukata TNF alpha is a cytokine a protein produced by the immune system that activates osteoclasts increasing the rate of bone destruction The see saw is then tipped in the direction of bone loss she says When your body is not in balance for calcium it steals it from your skeleton The pediatric patients diagnosed with osteoporosis in the Rush study had a history of synovitis one in his ankles the other in his knees Both had severe hemophilia and inhibitors Compared to their peers on prophylaxis these two bled a lot In a one year period the older teen had dozens of bleeds the younger one had more than 10 But it s not clear if repeated joint bleeds followed by periods of rest and or lack of exercise caused their osteoporosis Simpson says Then there s the issue of weight gain Being overweight taxes joints and limits range of motion in children with hemophilia A 2004 study by the Centers for Disease Control and Prevention showed that nearly one third of the boys and teens treated at the hemophilia treatment centers HTCs they surveyed were overweight or obese Obesity definitely leads to inactivity which then can make osteopenia worse Young says Osteoporosis Diagnosis Symptoms of osteoporosis can be subtle or nonexistent Had the two boys not been in the Rush HTC study Simpson doubts their osteoporosis would have been identified Osteoporosis is a silent disease says Bukata That s why tests confirming osteoporosis are performed the gold standard of which is the dual energy X ray absorptiometry or DEXA scan It uses radiation to measure bone density at sites of frequent fractures the wrist the spine and the head of the femur the long thigh bone If the results are at least two standard deviations below those of healthy peers and the child has a clinically significant history of bone fractures osteoporosis is presumed according to the International Society of Clinical Densitometry However because children s bones are less dense than adults the DEXA scan is not always accurate Other tests may be needed to confirm bone loss Lab tests can provide clues on the levels of calcium potassium which helps the body absorb calcium and vitamin

    Original URL path: http://www.hemaware.org/print/1092 (2016-02-18)
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  • Hemophilia Summer Camps | HemAware
    is called the OK Corral in keeping with the rustic Old West theme of the rest of the camp complex It has five nurses and three physicians and room for a helicopter to land in an emergency With the medical concerns taken care of the camp feels it can focus on other things like building community says Cook We normalize the experience of being a kid with a bleeding disorder With 40 peers who also have bleeding disorders campers often make connections for the first time with other children they can talk to on the same level The kids can finally say It s okay for me to be me entirely Here I m a part of something really big Other camps are founded on a different philosophy They use the one week window of time to push health education self sufficiency and advocacy along with offering social activities Most of these camps are run by NHF chapters hemophilia treatment centers HTCs or other hemophilia associations Education is a very important component of bleeding disorders camp says Val Bias co director of Camp Hemotion in Coarsegold California near Yosemite National Park The camp is sponsored by the Hemophilia Foundation of Northern California an NHF chapter Knowledge about their bleeding disorders which one they have whether it s mild moderate or severe is important information for them to have in terms of managing their lives They need to understand it before they get to be teenagers and are looking at insurance issues Setting the Standard About five years ago Michael Rosenthal former executive director of the Hemophilia Association of Arizona an NHF chapter helped organize the North American Conference of Hemophilia Organizations NACCHO The conference funded by a grant from Wyeth was created to provide best practices and resources for camp personnel in a forum that encourages information and idea sharing One outcome of the conference was a set of health and safety guidelines Health Center Standards for Camps Serving Persons with Bleeding Disorders written by a committee of camp directors and healthcare professionals It incorporates NHF s 1993 publication Hemophilia Camp Program Standards and the American Camp Association s health center guidelines and offers new program enhancements for camps that have the resources to meet higher standards The guidelines were approved by NHF s Medical and Scientific Advisory Council MASAC in April 2006 The basic health and safety standards are divided into four sections site facility policies and documentation program and education and staffing The need for guidelines according to Rosenthal was to create common standards for camps across the country Summer camps are a big deal he says They are the single most important program that chapters run You re taking 100 children with a medical condition away from their parents for a week and you re responsible for them 24 hours a day There is a science to it In the Program and Education section the guidelines state that there should be at least 30 minutes of health

    Original URL path: http://www.hemaware.org/story/hemophilia-summer-camps (2016-02-18)
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  • Physical Education for Kids With Bleeding Disorders
    It can include gym class considerations If your child has bleeds from certain activities there should be a discussion with the school about other roles the child can play Rousseau Purdy says Those could include serving as equipment manager or referee Put that right in the 504 plan While 504 plans address discrimination they also facilitate the child s well being Children with bleeding disorders can and do excel in physical activities Rousseau Purdy s youngest son Zachary 18 was named MVP of his high school basketball team earlier this year He has severe hemophilia A If I would have told him he could no longer play he would have wondered why he exists Rousseau Purdy jokes Kids have to understand the risks But you also have to talk about what their quality of life will be like if they can t do things they love Getting the benefits without the risks Because of the benefits of physical education medical researchers have been looking into which activities are safe for young people with bleeding disorders One study from Australia published in the October 10 2012 issue of the Journal of American Medical Association looked at how much the risk of bleeding increased with vigorous exercise Study author Dr Carolyn Broderick and her team of researchers rounded up 104 boys with moderate to severe hemophilia ages 4 through 18 with an average age of 9 5 The researchers monitored the boys bleeds and physical activity through telephone interviews for one year Eighty eight participants had bleeding episodes usually in the knees ankles and elbows and most frequently before and after school But researchers found that some sports were associated with a higher risk of bleeds depending on the activity s intensity and how often the child played Activities were categorized by their likelihood of a severe collision broadly based on NHF s publication Playing It Safe Bleeding Disorders Sports and Exercise Swimming for example was a category one activity Basketball was considered a category two activity while sports where collision is inevitable like wrestling were ranked in category three For a child who bleeds five times annually and is exposed on average to category two activities twice weekly and to category three activities once weekly exposure to these activities was associated with only one of the five annual bleeds the study authors wrote Another smaller study found no difference in the risk of bleeding between high impact activities like basketball and baseball and low impact activities like cycling and swimming The study gathered clinical data and information via telephone interviews on 37 young people between ages 6 and 21 with severe hemophilia A or B who were on factor prophylaxis Surprisingly we found that kids with severe hemophilia could take a lot more in the way of impact sports than is often attributed to them said Marilyn Manco Johnson MD of the Mountain States Regional Hemophilia and Thrombosis Center at the University of Colorado in a previous issue of HemAware The

    Original URL path: http://www.hemaware.org/print/1009 (2016-02-18)
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  • Immune Tolerance Induction Can Eliminate Inhibitors
    into play Although inhibitor titers drop at different rates they should do so within three to six months says Young It s important to do monthly testing in the pre ITI phase so that you can get to that less than 10 level as soon as possible Sometimes the inhibitor is stubborn hitting a plateau and not budging for months After five months the titers for Carri Nease s fraternal twins with severe hemophilia A and an inhibitor Connor and Tyin now 6 did the opposite they rose They ended up getting fast tracked for ports for ITI at 11 months old says Carri a receptionist from Essex Maryland The ports were needed for the frequent factor infusions that ITI requires which can be difficult in young children with difficult to find veins You shouldn t be waiting a year to start ITI says Young Read The Pros and Cons of Infusion Devices Rationale and Regimen The rationale behind ITI is similar to allergy desensitization You re infusing very high concentrations of the antigen protein in the hope that the immune system becomes tolerant or accepting of that FVIII says Leonard A Valentino MD medical director of the Hemophilia and Thrombophilia Center at Rush University Medical Center in Chicago It s re educating the immune system Not just any FVIII product will do The standard of care has been to begin ITI with the factor product in which the inhibitor developed says Valentino For most patients that s a recombinant product The best way to infuse the FVIII product for ITI is through a central venous access device typically a port The reasons for using a port in toddlers are legion They are chubby and have baby fat still so it s hard to find a vein says Young And they re strong enough to fight you Most parents find ports to be lifesavers When your child has a port you have the freedom to treat him when you need to Amanda says Good hygiene is a must We ve only had one port infection and that was in Connor this past year Carri says Just pay strict adherence to the protocol Families should be reminded that for any dental work antibiotics must be prescribed to prevent infection of the port from bacteria in the mouth entering the bloodstream No universal standards of care exist yet for ITI but registries of inhibitor patients in the US and Europe show that frequent high dose treatments are most effective in eradicating inhibitors Peyton was started on a daily high dose of 200 units per kilogram U kg says Amanda You can expect to make regular visits for lab work while on ITI Once you start ITI the titer will shoot up when you give a dose of FVIII again but then will start trending down Young says The only way to see that trend is with monthly testing Routine testing for Peyton revealed that his inhibitor dropped to 0 8 during the

    Original URL path: http://www.hemaware.org/print/897 (2016-02-18)
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  • La inducción de inmunotolerancia puede eliminar inhibidores | HemAware
    IIT con el producto de factor en que surgió el inhibidor aseguró Valentino Para la mayoría de los pacientes se trata de un producto recombinante La mejor manera de realizar una infusión con el producto FVIII para la IIT es por medio de un dispositivo central de acceso a la vena típicamente una vía Los motivos para usar una vía en niños pequeños son muchos Son regordetes y todavía tienen grasa de bebé por lo que es difícil encontrar una vena afirmó Young Y son suficientemente fuertes como para pelear contigo A la mayoría de los padres las vías les parecen caídas del cielo Cuando tu hijo tiene una vía tienes la libertad de darle tratamiento cuando es necesario dijo Amanda La higiene es esencial Solo hemos tenido una infección de vía y eso fue con Connor el año pasado dijo Carri Simplemente hay que seguir estrictamente el protocolo Se debe recordar a las familias que para cualquier tratamiento dental se deben recetar antibióticos a fin de evitar que la vía se infecte con bacterias de la boca que ingresan a la sangre Todavía no existen estándares universales de atención para la IIT pero registros de pacientes con inhibidor en Estados Unidos y Europa indican que los tratamientos frecuentes con una dosis alta son los más eficaces para erradicar los inhibidores Peyton comenzó a tomar una dosis alta de 200 unidades por kilo U kg indicó Amanda Hay que ir al médico con frecuencia para análisis durante la IIT Una vez que se inicia la IIT el título aumentará mucho cuando se vuelve a dar una dosis de FVIII pero luego comenzará a disminuir afirmó Young La única manera de notar esa tendencia es con pruebas mensuales Una prueba de rutina en Peyton reveló que su inhibidor había bajado a 0 8 durante el primer mes con la IIT por lo que la dosis del factor se redujo a la mitad a 100 U kg Pero la reducción no siempre es uniforme el nivel de inhibidor puede fluctuar Durante su IIT los mellizos recibieron infusiones diarias con una dosis alta de FVIII recombinante Ambos llegaron a cero un par de veces pero luego a Connor le volvía a subir dijo Carri El nivel máximo de Tyin fue de 7 5 el de Connor fue casi 20 veces más alto 139 Ya que cada paciente responde de manera diferente a la IIT la duración del tratamiento varía El tiempo mínimo es 33 meses lo que se ha comprobado con estudios del registro dijo Valentino El tratamiento de Tyin duró aproximadamente ese tiempo Tenía 3 años y medio cuando su inhibidor simplemente desapareció dijo Carri Se declaró que Tyin había adquirido tolerancia y recibe profilaxis cuatro veces por semana Pero la IIT no es solo para niños Vale la pena por lo menos probarlo con pacientes mayores que nunca han recibido tratamiento de inmunotolerancia dijo Valentino Sin embargo hay cierta inquietud sobre inhibidores arraigados Eso es cuando el sistema inmunitario ha estado

    Original URL path: http://www.hemaware.org/node/1118 (2016-02-18)
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  • Genetic Differences Influence Inhibitor Development | HemAware
    shown in a previous study on people without hemophilia that blacks and whites differed in the haplotypes or naturally occurring genetic variations involving the FVIII chromosome Although both displayed H1 and H2 haplotypes only blacks had three other haplotypes H3 H4 and H5 which is rare A sixth haplotype H6 was found in Asians It turns out that haplotypes may matter when it comes to factor product When the patients in the HTCs were divided into two groups based on haplotype the data revealed that those in the H3 H4 group 24 of the subjects were 3 4 times more likely to develop inhibitors than those in the H1 H2 group Inhibitor development was not related to the severity of hemophilia or the age of the patients The triggering agent may be the factor product itself Because all current recombinant and plasma derived products are made from H1 H2 versions of the FVIII protein the immune systems of black patients with the H3 H4 haplotypes could recognize it as new and foreign To defend itself against what it perceives as an attack by the unfamiliar H1 H2 haplotypes the immune systems in black patients produce inhibitors antibodies to the infused product These preliminary results suggest that mismatched factor VIII replacement therapy may be a risk factor for the development of anti factor VIII alloantibodies the researchers wrote But before manufacturers change the FVIII formulations more data need to be collected This is the first report of how the type of naturally occurring haplotype might be important in making a product says Thomas C Abshire MD one of the study investigators He is director of the Emory Children s Comprehensive Hemostasis and Thrombosis Program in Atlanta It should be one of the things that might be taken into consideration along with

    Original URL path: http://www.hemaware.org/story/genetic-differences-influence-inhibitor-development (2016-02-18)
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  • The 411 on 504 Plans
    what to do in case of emergency and provide the school staff with all of the appropriate emergency phone numbers Parents should consider the severity of their child s condition and the accommodations needed before deciding if a legal document like a 504 plan is the best way to ensure their child s participation in school Rousseau Purdy adds that the most important thing is that school staff members know what to do in an emergency They don t want to see your child hurt and they are not doctors she says Not knowing what to do in an emergency is the worst situation for anybody In addition to consulting with a member of your child s hemophilia treatment center HTC team the school nurse is often a good person to start asking about an IEP or 504 plan The nurse may be able to ensure that the school staff knows what s necessary and not necessary to prevent and address health related issues She can also educate the school staff on bleeding disorders by clarifying concerns and debunking myths Currently about 75 of schools in the US have a part time or full time school nurse according to the National Association of School Nurses NASN However recent cuts in government spending could jeopardize them Digital Vision Jupiter Images The school nurse can serve as a bridge between the school the child s physician or HTC staff and the family However contact with the school nurse should begin before the first day of school If the school nurse can translate what the parent is saying about the child s needs and what that means for the child s education then she can make sure that barriers are removed says Linda Davis Alldritt MA RN president of NASN This may include explaining to teachers how certain medications can affect the child s performance in class or how to ensure that missed school days are made up The school nurse also keeps tabs on additional resources available in the school district from transportation for treatment and clinical referrals to financial assistance Each of these provisions protects the child s health without interruptions in academic progress The most important function of the school nurse is to facilitate communication especially between the school and the child s physician or HTC team When miscommunication happens it can be really challenging Davis Alldritt cautions She has encountered parents who for example do not allow the school nurse to confer with the child s other healthcare providers and may feel like their knowledge as a parent is being questioned Parents need to know that the school nurse will need to be in contact with the child s physician about questions related to the child s care at school Joanna Whitaker 26 a stay at home mom from Pang burn Arkansas didn t know what a 504 plan was until the school nurse called her requesting a meeting to discuss her son Weslee 9 who has moderate hemophilia

    Original URL path: http://www.hemaware.org/print/886 (2016-02-18)
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  • Información sobre planes 504 | HemAware
    una emergencia es la peor situación para cualquiera Cuando comience a averiguar sobre un IEP o plan 504 además de consultar con un miembro del equipo del centro de tratamiento de hemofilia de su hijo el enfermero escolar a menudo es una buena fuente de información Quizá pueda asegurarse de que el personal escolar sepa qué es necesario y qué no para prevenir y resolver problemas médicos También puede informar al personal escolar sobre trastornos hemorrágicos y así disipar inquietudes y aclarar mitos Actualmente aproximadamente 75 de las escuelas en Estados Unidos tienen un enfermero escolar a medio tiempo o tiempo completo según la Asociación Nacional de Enfermeros Escolares NASN en inglés Sin embargo recortes recientes en gastos del gobierno podrían afectar eso El enfermero escolar puede actuar como puente entre la escuela el médico o personal de tratamiento del niño y la familia Sin embargo se debe iniciar contacto con el enfermero escolar antes del inicio de clases Si el enfermero escolar puede interpretar lo que el padre está diciendo sobre las necesidades de su hijo y lo que significa para la educación del niño entonces puede asegurarse de que se eliminen barreras indicó Linda Davis Alldritt MA RN presidenta de NASN Esto puede incluir explicarles a los maestros qué impacto tienen ciertos medicamentos en el desempeño escolar del niño o formas de asegurarse de que se ponga al día si se ausenta El enfermero escolar también se mantiene al tanto de los recursos educativos en el distrito escolar desde transporte para recibir tratamiento y remisiones clínicas hasta ayuda económica Cada medida protege la salud del niño sin interrumpir su progreso académico La función más importante del enfermero escolar es facilitar la comunicación particularmente entre la escuela y el médico o equipo de tratamiento del niño Cuando hay un malentendido puede ser realmente difícil advirtió Davis Alldritt Ha tratado con padres de familia que por ejemplo no permiten que el enfermero converse con los proveedores de servicios médicos de sus hijos y a quienes quizá les parezca que se pone en duda sus conocimientos como padres Los padres deben saber que el enfermero escolar necesita comunicarse con el médico de su hijo con preguntas relacionadas a la atención del niño en la escuela Joanna Whitaker ama de casa de 26 años de Pangburn Arkansas no sabía sobre los planes 504 hasta que una enfermera escolar programó una reunión para hablar sobre su hijo Weslee de 9 años quien tiene hemofilia B moderada La enfermera invitó al director auxiliar de enfermería y la principal maestra de Weslee a la reunión donde hablaron de todo desde qué hacer en caso de hemorragia hasta qué actividades Weslee debía evitar y hasta su marca preferida de compresas de hielo Luego la enfermera le dio a Joanna su número de celular y se mantienen en contacto durante todo el año escolar Me dijeron que estarían al tanto de él y sé que lo harán porque me escucharon cuando les dije qué hacer dijo Whitaker

    Original URL path: http://www.hemaware.org/node/1111 (2016-02-18)
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