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  • Factor XI Deficiency | HemAware
    bleeding disorder including FXI deficiency in women But it s easy to miss Many women chalk up their heavy menstrual bleeding to a family tendency without realizing that it s an indicator of an underlying disorder FXI could be diagnosed when you workup a woman who has some menorrhagia or who has some increased bleeding after pregnancy says Hurlet Jensen Usually I do a von Willebrand workup and PT PTT If those are normal sometimes I look at the platelet function A 1999 study published in the American Journal of Hematology AJH reported that 59 of women with FXI deficiency compared with 10 of the general population experienced menorrhagia Of those 65 to 75 bled through protection experienced flooding at night and passed blood clots It is vital that girls with factor XI deficiency who are beginning to menstruate recognize symptoms I will inform the parents to be very careful when a girl has the first menstrual period to watch for increased bleeding says Hurlet Jensen Heavy menstrual bleeding in young girls is often due to hormone imbalances You have to be very careful not to blame it on the FXI deficiency because it could be another diagnosis like VWD Hurlet Jensen says They have to be followed by a good gynecologist to be sure it s nothing else Pregnancy labor and delivery can also be challenging for women with FXI deficiency The 1999 AJH study revealed that FXI levels are inconsistent during pregnancy The authors recommend that FXI levels be obtained during the initial visit and monitored during the woman s third trimester Further the study reported that postpartum hemorrhage in FXI patients was 16 compared with 5 in the general population For all of these reasons it is important for women who suspect they might have a FXI deficiency to be tested and diagnosed before pregnancy Invasive gynecological procedures such as D C dilatation and curettage endometrial ablation for menorrhagia or hysterectomy might not be necessary in FXI patients Our view is not to go to radical surgery in women especially of childbearing age unless it s an absolute emergency say Hurlet Jensen Her center prefers to treat the coagulation problem first Boys Issues A mainstay of the orthodox Jewish faith is circumcision which is performed eight days after birth For families of Ashkenazi origin who suspect their son might have FXI deficiency there are concerns about bleeding It s rare to have bleeding assures Hurlet Jensen It is not necessary to treat prior to the procedure What is necessary though is education of the family and the rabbi about potential complications she says Treatment Options Treating patients with FXI deficiency is not straightforward The best product for each patient depends on the severity of the disorder bleeding history and if surgery is recommended the site of the procedure The variability of bleeding within the same patient also complicates the issue Further the treatments themselves are not yet optimal Depending on the product the side effects can range from allergic reactions to inhibitor development or thrombosis Since most people with factor XI deficiency do not have spontaneous bleeds home infusion of clotting factor is usually unnecessary FXI bleeding happens so infrequently that it doesn t make sense to teach the parents to infuse any type of product says Hurlet Jensen Still some patients in the US would like to have another option FXI concentrate They have treatments for hemophilia C in Great Britain and Europe but not in the US says Beck That s frustrating FXI concentrate was available for a limited period in the US in the late 1980s and early 1990s but was withdrawn because of safety concerns However a FXI concentrate made by a French company could lead to clinical trials in the US in the future See sidebar Concentrate Conundrum Several readily available products in the US can help curtail bleeding from injury surgery or menorrhagia in FXI patients The treatment for major bleeds or to prevent bleeding after surgery or during trauma is to use a plasma product like fresh frozen plasma FFP says Mathew Treatment is given before and after major surgical and dental procedures but there are drawbacks A large volume is needed which could overwhelm the circulatory system and it contains only a small amount of FXI In addition it exposes the patient to potential infectious agents and may cause an allergic reaction in some people To prevent the risk of contracting hepatitis from plasma products or other sources the National Hemophilia Foundation s NHF Medical and Scientific Advisory Council s MASAC Recommendation 128 states that all patients with bleeding disorders should be vaccinated against hepatitis A and B An alternative that I use before surgery is a bypass agent activated factor VII says Hurlet Jensen It s a genetically engineered recombinant product but you have to be very careful not to give too much because it can cause thrombosis The recombinant FVIIa or rFVIIa triggers a burst of thrombin on the surface of the activated platelet bypassing the need for FXI A 2008 study published in Haemophilia reported on the use of rFVIIA in 14 patients with mild or severe factor XI deficiency The patients evaluated had undergone such procedures as molar extraction total hip replacement elbow surgery or vasectomy Hemostasis was classified as effective at all times points in all patients the authors wrote Although this data is promising rFVIIa has not been approved by the FDA for this use Fibrin glue is a topical agent that stops bleeding and helps clot formation in the mouth after tooth extraction and as an adjunct with FFP in circumcision and hernia repair It is made from human plasma that has been treated to protect against viruses Antifibrinolytic agents help hold clots in place by inhibiting plasmin the enzyme that dissolves clots They are useful for preventing and curtailing bleeding in dental extractions nosebleeds surgery and injury The most familiar products in the US are aminocaproic acid Amicar and tranexamic acid

    Original URL path: http://www.hemaware.org/story/factor-xi-deficiency-facts?quicktabs_story_page_tabs=0 (2016-02-18)
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  • Factor XIII Deficiency | HemAware
    home Trips to the doctor or the emergency room are necessary With both of these treatments there are some concerns that patients should know about says Silver All products that come from plasma contain proteins and factors that are different from those naturally produced by the person receiving the treatment This can cause an allergic or what we call a hypersensitivity reaction in some people In addition there is a small chance that viruses such as those that cause hepatitis and AIDS could be inadvertently transmitted in the plasma along with the factors All three medical experts interviewed for this story said that donors are screened but that there is still what they called a low but not zero risk of contamination A third treatment Fibrogammin P is available in Europe Canada and Japan It is still in the final stages of clinical trials in the United States and is only available through doctors participating in the Investigational New Drug study This means the medication can only be given at larger medical centers and other places that have experience with the patient safeguards required by the FDA Update On February 17 2011 the FDA approved Fibrogammin P known as Corifact for use in the US This treatment is also made from plasma Unlike the other treatments only factor XIII remains and the medication undergoes an additional cleaning step further reducing the chance of virus transmission There is also a recombinant form of factor XIII undergoing study that formulation carries no real risk of viral transmission My children have also been prescribed Fibrogammin P and it is much easier to use says Kathy It is easily stored in the refrigerator and can be given in the home without having to involve a doctor or nurses so it saves trips to the emergency department The available treatments are all used prophylactically to prevent bleeding episodes It is very important that both physicians and patients understand that prophylaxis is very feasible in this disorder and that it can prevent intracranial hemorrhage or other life threatening events says Manco Johnson Because factor XIII lasts so long in the bloodstream treatment is usually monthly Also as little as 5 of normal can still prevent bleeding Factor XIII Deficiency Research and Support Difficult Because so few people have factor XIII deficiency it is difficult to find information on it and doctors who specialize in treating it Silver for example only knows of two families with the disorder in Northeastern Ohio Nugent treats three patients out of the 5 million people who live in Orange County California Any hemophilia treatment center HTC or the hematology department of a major medical center with a specialist in coagulation disorders will be able to treat factor XIII deficiency says Silver However treatment for the disorder may not always be well understood by healthcare workers outside the HTCs When Kathy s youngest son Mark fell and had bleeding inside his head treatment was delayed The pediatrician who was unfamiliar with factor

    Original URL path: http://www.hemaware.org/story/factor-xiii-deficiency-one-rarest-bleeding-disorders (2016-02-18)
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  • Lucky 13 | HemAware
    menorrhagia heavy menstrual periods and miscarriages Patients are also at increased risk for intracranial hemorrhage bleeding in the brain that can be life threatening A clinical trial of Tretten showed that when used prophylac tically the treatment prevented bleeds in 90 of the 77 subjects Side effects included headache as well as pain at the injection site and in the extremities In 2011 the FDA approved CSL Behring s Corifact a plasma derived product to treat FXIII deficiency Source Novo Nordisk View the discussion thread Life Stages Infants Children Teens Young Adults Adults Aging Research Treatment Hemophilia A B von Willebrand Disease Rare Bleeding Disorders HIV Hepatitis C Surgery Transplants Researcher Profiles Health Wellness Fitness Nutrition Taking Charge Pain Management Parenting Family Parenting Family Hemaware Jr Women Women s Bleeding Disorders Carriers Watchdog Insurance Blood Safety Advocacy Community HemAware Blogs HemAware Videos HemAware Photos Chapters in Action Global Spotlight Community Voices En Español Related Most Viewed 1 Factor XIII Deficiency One of Rarest Bleeding Disorders 2 Understanding Factor 1 Deficiency 3 Factor XI Deficiency Facts 4 Factor V Deficiency 5 A Family Matter The Pros and Cons of Infusion Devices Pumping Iron Head Bleeds and Hemophilia Dogs Priceless Contribution to

    Original URL path: http://www.hemaware.org/story/lucky-13 (2016-02-18)
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  • Factor V Deficiency | HemAware
    is fresh frozen plasma says Huang For mucosal bleeding that s not severe the antifibrinolytic agents like aminocaproic acid can be used If you have menorrhagia hormonal therapy might be an adjunct to minimize bleeding For the D Ambrosios obtaining FFP was not so straightforward Even with standing orders to receive it sometimes the family ran into roadblocks Once after phoning in the order ahead of time it takes two hours for the plasma to be secured and thawed upon arriving at the hospital the family was told the standing orders had expired Another time an urgent care physician said We re going to see what s going on here We don t just order blood products The diagnose first treat later philosophy did not sit well with the D Ambrosios We can t wait six hours for plasma says Cheryl s younger stepdaughter Cheryl learned quickly to argue with the staff or question procedures to get people to understand that Now that the family relies on the Puget Sound Blood Center PSBC their hemophilia treatment center HTC as the liaison the process is smoother If we do have some negotiations or if the doctor hesitates we make the HTC intervention call from our cell phone says Cheryl Once the doctor has spoken directly with the HTC he or she will say I ll order it I ll take care of it right away Plasma Safety The reticence of some physicians to order FFP is related to safety Plasma is derived from the pooled blood of donors Our blood supply is as safe as it s ever been but there still is that possibility of infection and allergic reactions Huang says The causes of such allergies while not well understood can be due to the presence of normal blood constituents he says In addition it is conceivable that donors could passively transfer proteins from food they ate such as peanuts to which the recipient may be allergic says Huang Mike Murdock American Red Cross Fresh frozen plasma derives from pooled blood of donors and is used to treat factor V deficiency Once Cheryl became involved in her stepdaughters medical issues the D Ambrosios had to face the plasma dilemma head on Do you know what you re putting into these girls Tony the girls father asked her pointedly They could get HIV They could get hepatitis They could die from it Waiting however had its own downside Instead of two units of FFP at the outset a delay could create a situation where six units were needed instead You do the math and finally change your paradigm Cheryl says When you nip it in the bud there are fewer units used and less risk Cheryl and Tony now agree on this Both daughters have experienced occasional itching from mild allergic reactions after FFP treatments Over the counter antihistamines provide relief A concern for patients receiving routine transfusions is transfusion related acute lung injury TRALI TRALI occurs in approximately one in

    Original URL path: http://www.hemaware.org/story/factor-v-deficiency (2016-02-18)
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  • Factor V Deficiency: How Our family Learned to Deal With Rare Bleeding Disorder | HemAware
    sure the girls didn t create a situation that would result in a bleed You might call that being a control freak Even though I wanted the girls to have a fun life and enjoy things frequently when I planned something special it seemed like one of the girls was injured amidst the fun and I regretted planning the activity Also because I came into their lives during their preteen years I had difficulty integrating myself as part of the care team Tony and the girls had already established their routines during a bleeding episode We finally sorted out that during hospitalizations we would trade shifts When it was my shift I took the lead and when it was his shift he took the lead Some hospitalizations would last a week or many weeks and one parent could not do it alone Finding a Way for Safe Activities When the girls were young I accompanied them to a camp for families with bleeding disorders called Camp I VY One day the camp staff transformed the mess hall into a carnival for kids of all ages Both girls entered the pie eating contest The oldest entered twice and her younger sister entered three times The photos of the girls with whipped cream all over their faces was all the evidence I needed to show how much fun the girls had that week Despite the fun of camp we had to eventually go back to our real lives where it is an exhausting effort trying to lead a reasonably normal life But I finally got it When I first met Tony I couldn t understand why we couldn t plan trips and have adventures like most families Now I understand fully why every activity too far from our hemophilia treatment center is nothing more than a wish for disaster You might say I have become an official card carrying member of the party pooper club After our oldest girl was knocked over at a Special Olympics event I drove her to a local hospital where I learned they treated only their own military patients We then took off for Group Health in Bellevue Washington about two hours away I drove her to the ER and after a brief discussion the staff ordered FFP Once again I shook my head I should never have taken her so far from her treatment facility and I shouldn t have put her at risk around excited kids But the damage was done She had a bleed that traveled from her back into her abdomen and she needed transfusions throughout the summer We were lucky she didn t require surgery Until things healed her mobility was severely impaired Bleeding episodes have a way of taking so much control over your life I have heard others say they don t but that has not been our experience When we took a vacation to Kansas City Missouri our youngest girl began her first menstrual cycle Keeping an eye on

    Original URL path: http://www.hemaware.org/story/family-matter (2016-02-18)
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  • RiaSTAP Approved for Factor I Deficiency | HemAware
    usually diagnosed at birth when newborns bleed from their umbilical cord site Other symptoms include bruising joint bone and tissue bleeds and excessive bleeding following an injury RiaSTAP is approved to treat acute bleeds in people with afibrinogenemia who have no fibrinogen or low levels of it It is also approved for people with hypofibrinogenemia whose fibrinogen levels are below 50 mg dL RiaSTAP is not approved for people with dysfibrinogenemia who may have normal fibrinogen levels but defective fibrinogen function these individuals are at risk for both bleeding and clotting complications RiaSTAP is contraindicated for people who have had severe immediate hypersensitivity reactions including anaphylaxis to it or its components This product offers a much needed treatment for the small number of patients with congenital fibrinogen deficiency says Jesse Goodman MD MPH director of the FDA s Center for Biologics Evaluation and Research If bleeding occurs in the brain or other organs and is left untreated it may lead to blood loss organ damage and death RiaSTAP is an intravenous fibrinogen concentrate made from the pooled plasma of healthy human blood donors According to CSL Behring the drug undergoes viral inactivation and removal for safety assurance The FDA approval is based on data from a Phase II study of 15 patients with afibrinogenemia who reached the target level of fibrinogen expected to prevent bleeding after receiving 70 mg kg of the drug Plasma from 14 of the 15 patients also showed increased maximum of clot firmness a marker the FDA believes is likely to predict clinical benefit Chills fever headache and nausea and vomiting were the most common adverse reactions that were observed Postmarketing studies will include patients with afibrinogenemia and hypofibrinogenemia Learn More Visit the RiaSTAP Web site www riastap com View the discussion thread Life Stages Infants

    Original URL path: http://www.hemaware.org/story/riastap-approved-treat-factor-i-deficiency (2016-02-18)
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  • Ask HANDI
    only and does not give medical advice or engage in the practice of medicine HANDI does not recommend particular treatment for specific individuals and recommends that you consult a physician before pursuing any course of treatment Send your question below

    Original URL path: http://www.hemaware.org/print/130 (2016-02-18)
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  • Send page by email | HemAware
    Fitness Nutrition Taking Charge Pain Management Parenting Family Parenting Family Hemaware Jr Women Women s Bleeding Disorders Carriers Watchdog Insurance Blood Safety Advocacy Community HemAware Blogs HemAware Videos HemAware Photos Chapters in Action Global Spotlight Community Voices En Español Need info Ask NHF Contact HANDI NHF s resource center for additional information on bleeding disorders Home Glossary Site Map Donate Advertise Privacy Policy Subscribe Contact Us Life Stages Infants Children

    Original URL path: http://www.hemaware.org/printmail/130 (2016-02-18)
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