archive-org.com » ORG » H » HEMAWARE.ORG

Total: 1100

Choose link from "Titles, links and description words view":

Or switch to "Titles and links view".
  • The Pros and Cons of Infusion Devices | HemAware
    the infection At highest risk for infection are children younger than two years old and those whose ports are accessed more often says Leslie Raffini MD assistant professor of hematology at The Children s Hospital of Philadelphia Poor oral health can also trigger infections by sending bacteria from infected gums or decaying teeth into the bloodstream Failure to adequately sterilize the port site before and after infusion can also cause infection A hematoma or bruise can occur on the surface or septum of the port device It is caused by leakage of blood from the port to underneath the skin when the needle is removed from the port If this occurs the port should not be used since the accumulated blood is an excellent growth media for bacteria and may lead to an infection A hematoma is more likely with frequent such as daily access or in individuals with inhibitor s However this can easily be prevented by applying direct pressure over the puncture site once the needle is removed Pressure should be applied for 10 minutes Clotting is another common problem with access devices Up to half of patients with ports develop blood clots in the vein accessed by the port s main line according to studies Journeycake has done at her clinic Some patients develop clots in the device itself Flushing the port with a blood thinning medication can help prevent clots and possible infection Physicians may also need to use blood thinners to treat clots In some cases the only way to break up the clot is to remove the port Other problems can develop when a port s line or catheter separates from its reservoir As a result factor can leak into tissue surrounding the port The port s line can also get twisted in which case the entire device must be reinstalled As children grow ports can also get displaced and cut off circulation in affected tissue This happened to Kristie s son Saverio on the brink of his third birthday A venogram showed the port was pushing on nearby veins so doctors removed it After that the Ciminos learned how to infuse Saverio s arms Decision time When it s time to decide if your child should have a CVAD consider the following questions suggest Rueleen Lavergne RN a quality assurance nurse at Hemophilia of Georgia in Atlanta and Jennifer Maahs MSN PNP a nurse practitioner at the Indiana Hemophilia and Thrombosis Center in Indianapolis Is the hemophilia severe Is there an inhibitor Children with mild or moderate hemophilia generally do not require the multiple infusions that warrant a CVAD the nurses say Most CVAD candidates have severe hemophilia Those with severe hemophilia B are less likely to need a port because they infuse less often than those with hemophilia A twice a week versus three times a week or every other day typically However a person who develops an inhibitor will likely need a CVAD regardless of severity because of the need for

    Original URL path: http://www.hemaware.org/story/pros-and-cons-infusion-devices (2016-02-18)
    Open archived version from archive

  • Anemia and Bleeding Disorders | HemAware
    chronic disease This type of anemia results when a long term medical condition affects the production and lifespan of red blood cells According to the NAAC 67 of people with hepatitis C who are on the combination therapy ribavirin interferon develop anemia Typically we see people present with fatigue and tiredness although very often they can be confused with the symptoms of hepatitis C or even interferon says T Jake Liang MD chief of the Liver Diseases Branch of the National Institute of Diabetes and Digestive and Kidney Diseases NIDDK one of the National Institutes of Health in Bethesda Maryland Physicians who treat patients with hepatitis C need to be vigilant about the impact of anemia on the symptoms Ribavirin is the culprit causing hemolytic anemia an abnormal destruction of red blood cells about four weeks after therapy begins But how it destroys the cells is unknown It may have something to do with the way the drug gets into the body accumulates in the red blood cells and becomes toxic Liang says If a blood test reveals the patient is anemic reducing the ribavirin dose or discontinuing it for a few weeks often allows the hemoglobin levels to rise Unfortunately in about 5 to 10 of patients you have to stop the ribavirin or keep it at a very low dose which tends not to be very effective says Liang An alternate therapy is gaining popularity the use of erythropoietin stimulating agents ESAs that prompt red blood cell production They work pretty well keeping the blood counts at a reasonable level so patients can continue to receive ribavirin at an effective dose Liang says Although this use of ESAs is considered off label says Liang it is generally accepted in the medical community However studies show that ESAs can cause cardiovascular complications including heart attack heart failure and stroke Further they are expensive Recent data suggest that erythropoietin growth factors can also cause tumors so we try not to be too cavalier about prescribing them Liang says We don t use them for an extended period usually only for a few weeks The US Food and Drug Administration issued a safety announcement in February 2010 requiring prescribers to complete a training module In HIV AIDS infection HIV attacks the platelets directly but its effects on red blood cells are indirect causing chronic disease anemia Anemia in these patients can also be caused by chronic infections such as atypical tuberculosis and certain drugs such as AZT azidothymidine Doses are adjusted to avoid this outcome Anemia is very uncommon in those who receive HAART highly active antiretroviral therapy says Margaret Ragni MD MPH professor of medicine at the University of Pittsburgh and director of the Hemophilia Center of Western Pennsylvania If you are co infected with HCV you can also develop a mild hemolytic anemia due to ribavirin That s why we need careful monitoring of blood counts on all individuals with HIV and or HCV infection Blood counts provide a

    Original URL path: http://www.hemaware.org/story/pumping-iron (2016-02-18)
    Open archived version from archive

  • Head Bleeds and Hemophilia | HemAware
    affected says Young Subdurals have the potential to lead to brain damage in the local area where the bleeding is occurring The damage could include paralysis vision loss difficulty speaking weakness on one side of the body and strokelike symptoms For significant subdural hemorrhage neurosurgery might be required Since the bleeding is in an enclosed space a neurosurgeon may need to make a hole in the skull or remove part of it to allow the pressure to be released says Young That gives the brain needed space to expand and restores blood flow to other areas of the brain In addition the neurosurgeon may try to remove the blood that is causing irritation and damage Spontaneous bleeds into the head tend to be intracerebral within the brain tissue itself says Young They can result from arteriovenous malformations congenital weaknesses in blood vessels in the brain that cause them to rupture resulting in bleeding and widespread swelling Because the bleed is deeper in the brain it is not as easily accessed You may still need the neurosurgical procedure to relieve the pressure but you re not going to be able to remove the blood Young says Significant local brain damage can result from this type of head bleed Precaution and Prevention Protecting the head seems logical especially in toddlers with severe hemophilia who are learning to walk But not all healthcare providers subscribe to the helmet every head philosophy If a child is on prophylaxis three times a week and the risk of intracranial hemorrhage is very small then there s no need to wear a helmet says Young It stigmatizes him But Young makes exceptions For a toddler with an inhibitor engaging in an activity that may lead to trauma like going to the playground I would suggest a helmet He also recommends helmets and protective gear for activities that have a high rate of injury in the general population Any child who s riding a bicycle skateboard or scooter needs to wear a helmet says Young In many states it s a law Helmet rules apply to all children with or without hemophilia Recommending helmets for children with hemophilia who want to play certain sports depends on several issues says Young For older children playing sports like baseball and soccer I don t recommend wearing helmets unless they re required for the sport such as batting in baseball or softball But a child with hemophilia who wants to be a soccer goalie needs to take extra precautions In this situation having him wear a helmet is not a bad idea Choosing safe sports is vital for children with hemophilia especially where the risk of head bleeds is heightened NHF s Sports and Exercise booklet is a useful guide for parents They should look at it say Young In it sports and activities are ranked by risk of injury on a color coded scale from 1 to 3 where 1 is safe and 3 is dangerous and not recommended by NHF There are only a handful of sports I consider taboo tackle football wrestling boxing ice hockey and lacrosse says Young For high level competitive sports such as soccer he talks with patients about preventive strategies I tell them that if they re going to play competitively there is a risk of blunt trauma of butting heads with an opponent The preventive strategy is that they have to infuse factor before the game says Young That may mean daily infusion throughout the season That way the player has adequate factor product in his system virtually the same as other players on the field and the risk of a head bleed is decreased Teens Will Be Teens Parents of teens with bleeding disorders would like to believe that Jared will never ride a scooter without a helmet and that Jenny will always wear a seatbelt when she catches a ride with friends But teenage independence and rebellion manifest in many forms one of which is risk taking Injuries tend to happen when teens are doing things they know their parents don t want them to do but they want to be one of the guys or girls says Nancy Roy MSN FNP C nurse coordinator at the Hemophilia Center of South Carolina an HTC in Columbia In younger teens it can be riding bikes without helmets or skateboarding without protective gear In older teenagers it might be a night of drinking with friends They bang their head are unaware of it and don t come into the clinic until a couple of days later By that time they have a major head bleed Communication and education are essential components of head bleed prevention says Roy I try to make things as nonthreatening as possible such as using the American Academy of Pediatrics AAP guidelines she says That way if a child knows that this is something that s recommended for everybody regardless of their hemophilia it does make it a bit more acceptable See sidebar Using Your Head Choosing Safe Sports and Activities The HTC staff encourages open communication with patients before children hit the teenage years That way parents can tell their children to call the HTC and see if the sport or activity is approved We try to have time alone with the teen so they can tell us anything they want to says Roy She is blunt she says because it is vital that she knows what they are doing I tell them Don t tell me what you think I want to hear Tell me the truth and let s start from there If a teen has had a head bleed and is on prophylaxis once he starts feeling better he may stop taking it and start taking risks says Roy Some teens have rejected her advice saying I don t care what you told me But many teens do listen to the HTC staff My experience has been that they ultimately come to the decision that

    Original URL path: http://www.hemaware.org/story/head-bleeds-and-hemophilia (2016-02-18)
    Open archived version from archive

  • Dogs' Priceless Contribution to Hemophilia Research | HemAware
    than 25 years The people who work here love animals and are extremely knowledgeable about them says Nichols At UNC the dogs have indoor outdoor runs and an acre of outdoor play area to romp in They re not kept in cages but in runs that are cleaner than our hospital says Roberts partially joking They re out in paradise next to University Lake The dogs are socialized with each other and receive plenty of human contact Just like family pets they are taught commands and receive treats as rewards The dogs are in the lab all the time with people says Nichols Since they stay there for years they become more than just lab animals They become companion animals says Lillicrap They are very well looked after and nurtured The dogs have names At the Canadian facility litter mates receive names starting with the same letter of the alphabet Mindy and Matteo for instance are siblings At the UNC lab the dogs are named for personality or looks One of our inhibitor dogs Cliffy named for Clifford the Big Red Dog is the lab prince right now One of the vet techs is a dog groomer and gives Cliffy s black and white coat special attention He is groomed to look like he has a great big mustache Nichols says with a laugh Most of the dogs live out their lives at the labs being used for research in middle and older age Lillicrap says We don t breed for a specific experiment and then euthanize the dog We now have dogs that are 13 years old Other Animal Models Researchers cannot limit their animal subjects to only one species Protocol requires them to progress from rodents for instance to larger animals if the experiment is successful at each stage Larger animals help researchers obtain the proper scale in terms of physiology and anatomy Eventually experiments are approved for human studies called clinical trials See Clinical Trials 101 This progression from one animal model to another and eventually to humans can take years Michele Calos PhD professor in the genetics department at the Stanford University School of Medicine in California is a bleeding disorders researcher who uses a variety of lab animals A huge amount of work has been done on mice throughout genetics because they are less expensive have a shorter life span and you can do experiments with such larger numbers she says Using mice helps researchers build confidence about treatments and techniques but they have limitations Their size and blood volume are two factors so is their genetics For most if not all gene therapies the delivery is a barrier says Calos Even with the sometimes successful delivery in mice however the results do not translate well You cannot go from a mouse model to humans she says It s too risky The intermediary can take the form of anything from pigs to primates The UNC lab has housed pigs with severe type 3 VWD since

    Original URL path: http://www.hemaware.org/story/dogs-priceless-contribution-hemophilia-research (2016-02-18)
    Open archived version from archive

  • Nutrition Info for People With Hemophilia | HemAware
    eating habits such as the ones mentioned here are most likely to yield long term results Be aware of portion sizes read labels and avoid snack foods Steer clear of foods that are high in fat and calories and offer little in the way of nutritional value Be mindful of hidden or overlooked sources of excess calories such as large glasses of juice and soft drinks Increase your consumption of fruits and vegetables Dark leafy greens and orange fruits and vegetables are excellent sources of vitamins and minerals They are also a rich source of fiber which helps you to fill up and maintain intestinal health Try to eat at least half of your grains from whole grain products Try oats barley whole wheat flax seed bran brown rice and pasta Whole grains help to satiate the appetite lower cholesterol and stabilize blood glucose levels to avoid an afternoon slump Eat baked snack items instead of fried Use low fat dairy sources such as 1 or skim milk and low fat cheeses These contain plenty of vitamins and minerals without the fats that have been linked to heart disease If you cannot consume milk look for other sources of calcium such as calcium fortified orange juice Bake broil or grill lean meats poultry and fish Avoid heavy gravies sauces and toppings that are often full of calories from fat Include some meatless meals during the week by incorporating dried beans nuts and seeds into your menus Dried beans provide an excellent source of protein and are high in fiber and low in fat with no cholesterol Cholesterol is found only in animal derived food sources such as meats eggs and milk Reduce fat intake and increase the use of good fats These include olive and canola oils Limit the use of butter stick margarine shortening and lard A few other nutritional suggestions are especially important for people with hemophilia In addition to maintaining healthy weight people with bleeding disorders need to maintain normal blood volume and blood cell production There are several nutrients involved in blood cell production such as iron protein copper vitamin C vitamin B12 vitamin B6 vitamin E and folic acid But it would be wise for patients to avoid vitamin E supplements since they may increase the risk of bleeding A diet that incorporates all of the food groups should provide nutrients adequately During a bleed it is estimated that a miniscule amount of iron roughly 75 micrograms is lost with each tablespoon 15 ml of blood Maintain your iron levels by eating naturally iron rich foods such as liver lean red meat and poultry all of which provide the best and most readily absorbed iron sources Other excellent sources of iron are leafy green vegetables broccoli dried beans grains and raisins Combining iron rich foods with good sources of vitamin C such as orange juice can enhance iron absorption Being realistic you know that you re not going to make perfect choices at every meal

    Original URL path: http://www.hemaware.org/story/making-better-food-choices (2016-02-18)
    Open archived version from archive

  • Think Again | HemAware
    on the actual cause of the pain A 2010 study in the journal Emotion found that long time meditators actually reduced the intensity of pain Joshua Grant PhD a postdoctoral candidate at the Max Planck Institute in Leipzig Germany studied 17 longtime Zen Buddhist meditators asking them to sit in meditation while researchers applied extreme cold to their skin to study the effect of pain on the meditators What he found was that meditation may change the structure of the brain In longtime meditators the anterior insula a part of the brain involved in feelings is thicker as is the anterior cingulate cortex a part of the brain with many roles including attention and emotion What we think happens is meditators disengage regions of their brains that interpret the experience as pain Grant says He conducted the Emotion study They still feel it but it s somehow disconnected from the notion of hurt or suffering And it s not just veteran meditators who get results The April 2011 study in the Journal of Neuroscience found that those who had only received four days of meditation training were able to reduce their perception of pain The benefits of meditation seem to be available to everyone Grant says Meditation has been found effective not only in addressing how our brains process and perceive pain but also in how we deal with the emotions that chronic pain creates Diane Dimon DrRS calls this catastrophizing She is a doctor of religious studies and founder of Matters of the Mind a mind body center in Fresno California She speaks on mind body skills for anxiety stress reduction and pain management in hemophilia often at NHF chapter events Pain is complex says Dimon but our perceptions often influence our experience of it Pain triggers your primitive brain the fight or flight response in the amygdala responsible for assessing threats When set off this response shoots the body full of stress hormones and opens pain receptors in the spine both of which intensify the experience of pain When a person sits in meditation he or she can learn to discern whether there s really any danger Dimon says As long as there s no real danger meditation helps people view pain as a challenge instead of a danger she says In other words meditators learn to relax instead of tensing and resisting which make pain worse Depending on their skill level meditators can short circuit the stress response Then there s the effect of meditation on a person s stress level in general Chronic pain can wreak havoc on everything from sleep cycles to exercise routines making it difficult for a person to bounce back Meditation offers an antidote through what Benson calls the relaxation response The heart rate slows blood pressure goes down breathing slows oxygen intake increases and muscle tension decreases When you meditate you produce the exact physiological opposite of the fight or flight response Dimon says Your heart rhythms your biochemistry your whole

    Original URL path: http://www.hemaware.org/story/think-again (2016-02-18)
    Open archived version from archive

  • Music Therapy Used for Pain Management | HemAware
    Coupled with imagery of a patient s favorite spot to unwind it can redirect thoughts Recovering from surgery you can use the music to help you relax distract you and give you something else to focus on she says When Gallagher approaches patients she finds out what they prefer What a person likes is what s going to be best for them Gallagher uses a portable electronic keyboard to conduct music therapy sessions She might also use a guitar percussion instruments autoharp or Qchord an electronic autoharp that mimics the sounds of a guitar piano stringed instruments and percussion How Music Therapy Works Raffi Tachdjian MD MPH presents a young boy with his request a guitar just like his dad plays Music s ability to affect the perception of pain relates to the gate control theory Gallagher says If you re focused on something like playing an instrument singing or listening to music that s what s going through the neurotransmitters which relay the pain message to the brain at the time It shuts the gate on the pain because it s hard to focus on two things at once The body produces its own pain relievers serotonin noradrenaline and endorphins all of which can decrease or block pain signals Music can set off the endorphins and you can actually in some cases feel happier and better Gallagher says Pain Relief The anticipation of pain combined with fear and anxiety can increase the perception of it Music therapists can help by redirecting a patient s thoughts and reactions For infusions the music therapist could be there with the patient as the IV is popped in The patient could be playing an instrument through it or singing through it visualizing that the pain anxiety or whatever s bugging them is going away Tachdjian says After that the patient can associate an infusion with the music and the calm relaxed environment that was created It helps give patients control over what can seem an out of control experience This is empowering in the end because patients learn that they don t have to reach for a pill every time this kind of pain comes up Recorded music can be soothing and comforting but it has limitations Live music is always better because it can be controlled Gallagher says If the patient is short of breath or agitated or really anxious I might start playing at the tempo they re breathing By gradually slowing the tempo Gallagher helps slow the patient s breathing which eventually matches the rhythm of the music Music therapists are like quarterbacks who can change the play while it s happening Tachdjian says They can shift the cadence or tone or sound quality to help the patient Studies have shown that music therapy produces positive effects Gallagher s 2006 study of 200 adult palliative medicine patients assessed pain anxiety depression shortness of breath mood and some behaviors All of the eight factors we looked at improved with music therapy

    Original URL path: http://www.hemaware.org/story/music-therapy-used-pain-management (2016-02-18)
    Open archived version from archive

  • Treatment Breakthroughs | HemAware
    developing a recombinant porcine pig FVIII product for patients with inhibitors You can give a dose and get the measurable level of FVIII That s a distinct advantage when there s a life threatening bleed like a head bleed intracranial hemorrhage or a limb threatening bleed in someone with a compartment syndrome increased pressure in a muscle in an enclosed space Koerper says But because 80 of patients developed antibodies to plasma derived pig factor within five days or after five doses it is possible that a similar scenario might occur with the recombinant product Results of the clinical trials will provide more data but its use will probably be restricted The longer lasting products may have an added benefit for inhibitor patients Some forms of PEGylation strategy and possibly even some of the fusion proteins may result in reduced risk for inhibitors says Pipe Another product now being tested Octapharma s recombinant human cl rhFVIII may reduce the rate of inhibitor development because it uses proteins from human cells not the typical hamster cells Recombinant VWD Product at Last Recombinant products to treat FVIII and FIX were approved in 1992 and 1998 respectively not so for von Willebrand factor VWF It has bothered me for almost 20 years that I couldn t offer a recombinant VWF product to my VWD patients says Koerper That need will be fulfilled once Baxter s recombinant VWF product goes through FDA approval and licensure It will be targeted to patients with type 3 VWD the most severe form and those unresponsive to DDAVP a synthetic hormone used to prevent or stop bleeds Gene Therapy Revisited Researchers can now create precision drugs that treat diseases caused by specific genetic mutations One such drug in phase 2 trials is Ataluren PTC 124 manufactured by PTC Therapeutics Inc It will be used for the approximately 10 15 of patients with hemophilia A and B with a nonsense mutation which halts factor production early Ataluren introduces a molecule that allows the cell to read through the stop signal making more clotting factor It comes in a powder that is mixed in water Something that you can swallow is going to be a huge advantage because there are no needles involved Koerper says See sidebar The Allure of Ataluren in What s Your Genotype HemAware Spring 2010 p 29 Rare Bleeding Disorders on the Radar Patients with rare factor deficiencies know that being one in a million is hardly a cause for celebration People forget that there are other clotting factor deficiencies that in some cases have no treatment says Bias But hope is on the horizon Companies that fractionate or separate plasma are interested in getting as many products out of it as they can says Pipe Developing new markets for new plasma derivatives such as the new FXIII product Corifact approved by the FDA in March 2011 and RiaSTAP a fibrinogen concentrate to treat FI deficiency indicated for patients with congenital fibrinogen deficiency including afibrinogenemia and

    Original URL path: http://www.hemaware.org/story/treatment-breakthroughs (2016-02-18)
    Open archived version from archive



  •