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  • Total Knee Replacement Surgery | HemAware
    fits on top of the shin bone An insert made of polyethylene fits onto the base plate between the femoral and tibial components providing the hinging motion of the knee The patella has a plastic cap placed on its undersurface so it tracks over the front of the joint in the natural way says Wiedel Cement is used to affix the parts to surrounding bone Potential Problems All surgeries carry a potential risk of complications In 2005 Luck along with co author Mauricio Silva MD published the results of one of the first long term studies of knee replacements in people with hemophilia in The Journal of Bone and Joint Surgery The study of 90 knee surgeries in 68 patients during a 26 year period revealed that the late infection rate from six months to 17 years after surgery was high 16 vs 1 to 2 in the general population This figure is consistent with that found in other studies In patients who underwent bilateral knee surgery the rate of infection was 22 The reason for the increased incidence of late infection is self infusion not the surgery itself says Luck If patients are not meticulous about their technique they get bacteria in the blood stream that go to the knee At the first sign of infection they need to contact their physician he urges A further finding was the refutation of the hypothesis that patients who were HIV positive were more susceptible to infection In the study which compared patients with similar CD 4 counts a measure of the immune system s ability to fight infection the differences were negligible Infection developed in 17 of patients who were HIV positive compared with 13 of those who were HIV negative We have shown clearly that although there is an increased incidence of late infection in patients with hemophilia it is not related to HIV status Luck avers Sepsis a systemic infection of the blood can occur in patients with inhibitors who have the knee surgery Historically bacterial infections have come from airborne spores in the operating room Now the cause is usually a central venous access device Systemic blood poisoning is most common in patients with some type of venous access line especially one that is external Luck says The study also revealed the need for preprocedural antibiotics The use of prophylactic antibiotics before dental work or any other invasive procedure was another important finding says Luck The body s reaction to surgery is to create scar tissue on the skin and in the joint a condition called arthrofibrosis or stiff knee syndrome The surrounding ligaments and tendon stiffen tightening the knee joint capsule restricting the natural motion between the femur and the tibia If ROM exercises are not done soon after surgery some patients can permanently lose full flexion or extension As with any replacement surgery the artificial knee parts can loosen and even fail The artificial components wear out with age which Wiedel says is inevitable Infection which can cause failure of the prosthesis results in its removal However the rates are still relatively low Now that we have better replacement therapies and understand the need for aggressive use of factor replacement loosening and mechanical problems aren t any higher than in the general population Wiedel says Steps for Living Treatment Basics With the advent of better replacement parts the need for revision is now measured in decades not years In individuals who don t abuse the joint by overstressing it the replacement will last 20 years and theoretically could go 30 years says Wiedel Prepping for Surgery At our center we have patients learn all the exercises preoperatively to gain as much strength in the quadriceps and hamstrings as possible beforehand says Sharon Funk PT University of Colorado Denver Hemophilia and Thrombosis Center The more you can do upfront the easier the rehab is afterward The HTC nurse PT and social worker also discuss the procedure encouraging patients to share any fears or concerns We tell them It s going to hurt You are going to have to work hard but each day it will get a little bit easier Patients typically stay in the hospital for two weeks Pain medications and antibiotics are given as long as they are needed The hematology team determines whether the factor infusions are continuous and how long to sustain them after surgery But patients are not resting in bed for long Continuous passive motion machines keep their legs moving to promote blood circulation and prevent clot formation and swelling Most physical therapy begins early and is aggressive Partners in Physical Therapy A Tale of Two Patients Larry Hammerness 45 had a troublesome right knee that required a synovectomy when he was 10 years old It had created so much scar tissue that even moving caused bleeding he says Later he self infused for episodic bleeds caused by his severe hemophilia A Always active he surfs skis swims and cycles the Los Angeles photographer underwent knee replacement surgery in 2002 to relieve chronic pain Steps for Living The Pain Facts Previous surgeries to repair a broken hip and to fuse an ankle led Hammerness to believe he was ready for rehab He remembers the rigorous twice a day sessions the first few weeks falling asleep at home exhausted afterward You have to be really committed to the physical therapy he says I probably did 80 of the work In retrospect I wish I had done the other 20 The result Hammerness has lost flexion in his right knee he can bend it only to a 90 degree angle For physical therapy after knee surgery the truism holds What you put in you get back The goal of the physical therapy is to get full extension passively and actively and to regain flexion Funk says I ve seen as much as 130 to 140 degrees but most people do not get that much She says patients are usually not discharged from the

    Original URL path: http://www.hemaware.org/story/total-knee-replacement-surgery-takes-team (2016-02-18)
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  • Elbow Surgery for Hemophilia | HemAware
    combined the researchers looked at the efficacy of elbow replacement surgeries in 12 patients with hemophilia About six of them had major complications including infections that s a main concern says Silva One had a vein thrombosis and another had a nerve injury At this time that procedure is not our choice The surgery Silva does choose however is radial head excision It effectively removes the mechanical blockage created by the oversized radius To rotate your elbow the radial head has to join an articular surface on the ulna Because this radial head is so enlarged and irregular every time you try to rotate that irregularity is stopped by the proximal ulnar facet says Silva Once you remove the radial head surgically you have removed the mechanical blockage and the patient can return to rotation Radial head excision surgery is often accompanied by a synovectomy They are usually done together says Silva One you take the radial head out if you find a very thickened synovium it s a golden opportunity to take it out A 2007 research study co authored by Silva and James V Luck Jr MD published in The Journal of Bone and Joint Surgery reviewed 40 radial head excisions performed in men with severe hemophilia A or B during a 35 year period from 1969 to 2004 Patients referred for surgery had severe pain limited range of motion and chronic bleeding in their elbows The results showed that the surgery successfully reduced pain in 70 of patients of those 30 reported complete pain relief Further bleeding frequency was decreased and there was a low rate of complications One patient developed nerve palsy after the surgery but it resolved within six months In addition there was a mean gain of 63 degrees in the pronation supination arc in the forearm which Silva calls a significant improvement Preferred Patients When pain and immobility interfere with daily living people are ready to consider radial head excision surgery Anything I enjoyed doing was limited says Greer When you have that much limit to your joints it pretty well curtails your activities I had about 20 degrees of motion in my left elbow when I had the surgery done While there is no typical patient there are populations of patients for whom surgery is the best option Generally the patients I see at my center are between their mid 20s and early 50s says Sue Geraghty RN MBA of the Mountain States Regional Hemophilia and Thrombophilia Center in Aurora Colorado The degree of debilitation they have is dependent on the frequency of bleeding into the joint Someone who develops a target joint as a child and has an average of four to eight bleeds into the elbow by the time he is in his 20s will have much more degeneration she says than one who has his first elbow bleed in his teens Many people with limited movement in their elbow find ways to manage What we see a lot of times is that they ve learned to compensate says Geraghty If you watch they re opening the door with their whole body leaning over to get their hand to open the door as opposed to the motion coming from the elbow The yardsticks Silva uses to determine the need for elbow surgery in people with hemophilia are pain radial head enlargement and limitations in pronation and supination in the forearm If you cannot rotate your forearm it s very difficult to perform personal hygiene or feed yourself he says If we can improve it with surgery that is a very important gain in your quality of life On the other hand not all patients with limited movement in the elbow and arm are candidates for surgery If I have a patient who has a bad elbow it looks terrible on the X rays the joint is bad the radial head is bad but there is no limitation for pronation supination I will be a little reluctant to excise the radial head because the patient will not gain much from the surgery Silva says People who want to improve flexion and extension are told the same thing This procedure is not going to improve that Silva says Patients need to understand that so the goals are clear The research he conducted with Luck showed that after surgery only two degrees of flexion at best were gained in the elbow Also Silva will not perform elbow surgery on children under the age of 15 because they are still growing I will not do it on a patient with an open physis growth plate he says emphatically The proximal aspect of the radius can regrow and can become a problem again The total length of the radius can be altered and can actually change the distal radial ulnar joint Surgery performed on a child who is not done growing could lead to uneven arm lengths down the road Pre op Workup and Post op Workout Candidates for radial head excision need to have a workup prior to the surgery That includes a full musculoskeletal evaluation measuring the degrees of flexion and extension pronation and supination and strength testing Depending on the surgeon radiological evaluations usually include X rays and an MRI Taking a bleeding history is also important We ask them such things as the age at first bleed into the elbow joint frequency of bleeding in the past current frequency of bleeding and whether they are on an anti inflammatory medication says Geraghty These help us determine whether someone has significant arthritic changes versus whether they are still bleeding into the joint The combined information provides the surgeon with the most accurate picture of the state of the elbow before surgery Silva says the surgery is relatively simple We approach the elbow through the lateral side spread the muscles apart and open the capsule That provides the ideal position to expose the radial head which is then removed through a cut just

    Original URL path: http://www.hemaware.org/story/when-elbow-surgery-best-option (2016-02-18)
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  • Ankle Surgery for Hemophilia | HemAware
    walk the same way He waited three years before he had his first ankle surgery in April 2005 Gilbert says that by the time most people undergo fusion surgery they probably have little to no motion in the ankle but don t realize it They think they re having some motion but that happens to be motion in the bones below the ankle which allows the foot to look as if it s going up and down and in and out says Gilbert They think they re having tibiotalar true ankle joint motion what they re really having is subtalar motion movement of the joint below the talus bone The Surgical Procedure The typical fusion in the ankle occurs between the tibia and talus joints and is called tibiotalar fusion although subtalar fusions are also done when needed In the arthroscopic procedure the surgeon removes the remaining cartilage on the talus and tibia exposing the bones and then aligns them for proper fusion You then have two raw ends of bones says Gilbert The body identifies it as a fracture and determines to heal it So the bones fuse together Although there are many methods used typically metal screws pins plates or rods are put in place to anchor the joint until it heals In a crossed screw fixation one goes diagonally through the tibia on the inside the other diagonally through the fibula on the outside into the talus to hold it A cast keeps the ankle immobilized while the bones fuse protecting the joint Most patients are in the cast for six weeks without bearing weight on it After that the cast is removed and the patient is in an ankle brace commonly a boot with Velcro straps bearing weight on it while on crutches for another six weeks In most cases 12 weeks is the minimal amount of time needed for healing Full recovery and returning to activities can take up to nine months depending on the patient Post op and PT Once the cast is removed some physicians recommend physical therapy PT for their patients If everything is fine on the X ray and looks healed I refer people to PT because they need to be retrained on how to walk says Silva Range of motion is not our concern anymore because we took that away with the surgery What the patient needs is strengthening exercises for the muscles around the ankle After being in a cast for so long those muscles are quite atrophic Changing the bones of a joint isn t all there is to the procedure says Ruth Mulvany MS PT at the University of Tennessee Memphis You ve got to rehab the muscles and soft tissues and the other joints that are also affected It comes down to good biomechanics Mulvany says helping someone regain good posture and joint alignment If a person has walked abnormally because of an ankle problem then the knee and hip also have problems she says We

    Original URL path: http://www.hemaware.org/story/ankle-surgery (2016-02-18)
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  • NHF-Baxter Clinical Fellow Profile: Rachael Grace, MD, MMSc | HemAware
    research and clinical care The fellowship allowed me to participate in our hemophilia program in ways that I wouldn t have otherwise been able to she says In addition she plunged deeper into research on immune thrombocytopenia ITP a rare bleeding disorder in which the immune system destroys platelets Are you still engaged in the clinical aspects of patient care or bleeding disorders research In what aspect of care are you most interested As director of the hematology clinic at Boston Children s Hospital Grace spends about 25 of her time with patients and 75 pursuing research In 2012 she launched the Pediatric ITP Consortium of North America a collaborative research consortium of more than 20 investigators from the US and Canada Did your NHF Baxter Clinical Fellowship assist in advancing your own position at your institution Or did it serve as a building block to further your career in coagulation The fellowship helped Grace succeed in both areas she says With dedicated time for research the fellowship paved the way for her current K12 grant from the National Heart Lung and Blood Institute NHLBI It allowed me to make progress in my research and also to become a better clinician for patients with bleeding and clotting disorders she says Where do you think bleeding disorders research and clinical care may be headed in the near future In the next decade Grace believes that longer acting factor products and gene therapy developments are coming in the foreseeable future But she also predicts that patient reported outcomes will be more highly valued in the short term I think we ll be listening more to what patients have to say about how treatments and approaches make them feel When you are not working how do you relax or escape from your work

    Original URL path: http://www.hemaware.org/story/nhf-baxter-clinical-fellow-profile-rachael-grace-md-mmsc (2016-02-18)
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  • NHF-Baxter Clinical Fellow Profile: Brian Branchford, MD | HemAware
    pathologic process better in hematology than in other specialties It may have been directly related to seeing the movie Fantastic Voyage at an early highly impressionable age he says How did the training and mentorship you received as an NHF Baxter Clinical Fellow affect your decision to pursue clinical care and or research in bleeding disorders One of Branchford s mentors for the fellowship Neil Goldenberg MD got him interested in evaluating risk factors for pediatric thrombosis When I started working with him I found out just how much potential there is to move the field forward Evaluation management and prevention of such blood clots have become Branchford s clinical niche today Are you still engaged in the clinical aspects of patient care or bleeding disorders research In what aspect of care are you most interested Although Branchford does six weeks of inpatient service at the Children s Hospital every year seeing all manner of patients the majority of his clinical time is spent with pediatric patients who have bleeding disorders or blood clots Most of my patients have had blood clots in an extremity or pulmonary vessel he says The cause could be anything from trauma or injury to surgery or infection or to a central venous access device I m leading a large group at our hospital as well as a 74 hospital national group that are both trying to institute prevention standards for this Did your NHF Baxter Clinical Fellowship assist in advancing your own position at your institution Or did it serve as a building block to further your career in coagulation The latter probably led to the former says Branchford At his institution the fellows doing oncology research during their second and their years are covered by a training grant from NIH but not so for the fellows doing hematology research The NHF Baxter Clinical Fellowship secured his position financially It involved research in the lab of his mentor Jorge DiPaola MD that was recognized on national and international platforms reflecting positively on Branchford s institution The past record of my successful application for this clinical fellowship and the high quality data soon to be published that were generated during its course probably made my section chief more likely to hire me for a faculty position after the fellowship Branchford says Where do you think bleeding disorders research and clinical care may be headed in the near future In the next decade The big new thing is long acting factor replacement products and figuring out what the best options are for particular patients says Branchford In the next 10 years he s placing his bet squarely on gene therapy It has the potential to provide an actual cure for the disease he says His institution is planning to take part in a gene therapy trial headed by Paul E Monahan MD at the University of North Carolina at Chapel Hill What profession would you be in if you weren t in medicine Without hesitation Branchford

    Original URL path: http://www.hemaware.org/story/nhf-baxter-clinical-fellow-profile-brian-branchford-md (2016-02-18)
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  • NHF-Baxter Clinical Fellow Profile: Salley Pels, MD | HemAware
    hemophilia community was through Diana but with Michael I was able to see how these bleeding conditions affect women throughout their life cycles she says Although Pels enjoyed her time in the lab the influence of these three mentors confirmed her calling in the clinic For me it has always been and always will be about the patients she says Are you still engaged in the clinical aspects of patient care or bleeding disorders research In what aspect of care are you most interested As associate medical director of our HTC at Yale I am still very involved in clinical care Pels says Having engaged in basic research she has now transitioned to several clinical projects all in early stages some of which focus on the quality of patient care in the hemophilia population Did your NHF Baxter Clinical Fellowship assist in advancing your own position at your institution Or did it serve as a building block to further your career in coagulation The answer says Pels is both The added training through the fellowship helped solidify Pels expertise and experience she says I ve been able to establish myself among my hematology oncology colleagues as someone with hemostasis and thrombosis expertise and also within the institution as someone to go to for these kinds of questions and concerns with the patients For Pels the building block aspect has to do with involvement on a larger scale She is on the board of directors of the Hemophilia Alliance and on the quality improvement committee with ATHN Having been an NHF Baxter fellow I ve been able to use that in a couple of different ways says Pels It has certainly helped me get to where I am today Where do you think bleeding disorders research and clinical care may be

    Original URL path: http://www.hemaware.org/story/nhf-baxter-clinical-fellow-profile-salley-pels-md (2016-02-18)
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  • NHF-Baxter Clinical Fellow Profile: Mindy Simpson, MD | HemAware
    about coagulation Simpson describes Marilyn Manco Johnson MD director of the hemophilia treatment center at the University of Colorado in Aurora as a fantastic mentor During her two years there Simpson started work on global assays which measure clot formation and breakdown She developed a simultaneous thrombin and plasmin generation assay and her work was published in Thrombosis Research She also observed role models for what she is doing now at Rush I was exposed to people who were able to make it work doing good clinical care and good research Are you still engaged in the clinical aspects of patient care or bleeding disorders research In what aspect of care are you most interested Simpson divides her time between clinical care of pediatric patients and research She sees the value in each I love clinical medicine so I will never leave the patient part of it But the research aspect has to take such a large role in what we re doing because there s still so much to improve on Translational research based on patient concerns and questions drives Simpson s lab work to provide answers explanations and improvements Simpson believes that the research she and others are conducting using global assays may help explain the variability in bleeding between patients with identical diagnoses and factor levels These global assays are trying to look at the process of clot formation and clot breakdown she says We are trying to find what is somebody s individual ability to make a clot and have that clot break down Using the assays to measure levels of different proteins in the blood and thrombin which helps build a clot could provide more answers We re trying to improve upon how we define who s going to bleed more and who s going to bleed less who needs more treatment and who needs less Simpson says Did your NHF Baxter Clinical Fellowship assist in advancing your own position at your institution Or did it serve as a building block to further your career in coagulation The fellowship was a building block for Simpson s career Without it I probably wouldn t be where I am today Where do you think bleeding disorders research and clinical care may be headed in the near future In the next decade In the short term Simpson says trials of longer acting factor products are popular among patients Here in my world of clinical research that s where we are enrolling our patients They all want to be able to infuse less often but have good effects Down the line Simpson is banking on global assays to personalize medicine We re hoping to individualize the treatment options and the decision making for what s best for each patient When you are not working how do you relax or escape from your work When Simpson is away from patients and the lab she is completely focused on spending time with her husband and her son Brian We are doing

    Original URL path: http://www.hemaware.org/story/nhf-baxter-clinical-fellow-profile-mindy-simpson-md (2016-02-18)
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  • NHF-Baxter Clinical Fellow Profile: Michael Callaghan, MD | HemAware
    as an NHF Baxter Clinical Fellow affect your decision to pursue clinical care and or research in bleeding disorders There are a lot of demands on fellows and junior faculty to do a lot of patient care in different areas says Callaghan Having the NHF Baxter Clinical Fellowship allowed me to focus more of my time and energy on research in hemophilia and on hemophilia care Plus he was welcomed as part of the hemophilia team early on and received invaluable training he says Callaghan worked in the lab of Randal J Kaufman PhD at the University of Michigan in Ann Arbor on the unfolded protein response That process is very important in inhibitor antibody development he says We did high throughput drug screening to find what targeted that pathway Drug candidates were then tested on mice with hemophilia and inhibitors Are you still engaged in the clinical aspects of patient care or bleeding disorders research What aspect of care are you most interested in Callaghan spends about 75 of his time in the lab focusing on immune tolerance therapy to eradicate inhibitors The rest of his time is devoted to patient care I like to see all comers from people who are just being worked up for bleeding disorders to hemophilia von Willebrand disease even platelet disorders and ITP idiopathic thrombocytopenic purpura Although most of the patients are children some are a bit older We do have several kind of big kids who stretch out into their 20s Callaghan says Did your NHF Baxter Clinical Fellowship assist in advancing your own position at your institution Or did it serve as a building block to further your career in coagulation Receiving the grant helped Callaghan on both fronts Obtaining funding helps secure a position at your own institution and helps you get future funding he says The time and resources it provided him were very very helpful Where do you think bleeding disorders research and clinical care may be headed in the near future In the next decade Callaghan says this is an exciting era for bleeding disorders research and drug development After decades of research clinical trials are now closer to yielding factor products with longer half lives for instance We expect that s going to be integrated into clinical practice very soon he says Decades of research using gene therapy techniques are also advancing in clinical trials currently There s been successful gene therapy in a few patients with hemophilia B says Callaghan That s very exciting A third area in which research is making headway is inhibitors Callaghan says Hopefully there ll be some breakthroughs in new bypassing agents with different mechanisms for inhibitor patients as well When you are not working how do you relax or escape from your work With three daughters ages 5 6 and 8 Callaghan says work is his escape It is really kind of relaxing for me When he s home he is an active dad biking or swimming with his

    Original URL path: http://www.hemaware.org/story/nhf-baxter-clinical-fellow-profile-michael-callaghan-md (2016-02-18)
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