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Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 1 July 2014 Advances for Hemophilia B. Hemophilia A not far behind. Inside This Issue By: Barbara A. Konkle, M.D. Hematologist, Hemophilia Care Program 1 Advances for Hemophilia 2 Nutrition Tips 4 Does Hemophilia Run in Your Family 5 5 Essential Summer Camp Tips 5 Welcome Dr. Rebecca KruseJarres Director, Clinical and Translational Research The first of several new clotting factor products was just approved by the FDA in April 2014. This is a factor IX (9) product for Hemophilia B that is modified so that it stays around longer and requires less frequent infusions. For prophylaxis this means a treatment once every week or 10 days instead of twice a week. The newly approved product, Alprolix®, has a portion of a recombinant protein (Fc) attached which keeps the factor in the blood for a longer period of time. Two other products with extended half-life are under study, one with an albumin tag and the other with PEGylation, both appear to extend half-life similarly to 6 How to prepare your child for Alprolix®. Patients using a longer-acting product will require less frequent infusions but also Camp? will likely spend more time with a lower factor level while on prophylaxis. Alprolix is priced 7 Walking program higher than BeneFIX® and Rixubis®, the two unmodified recombinant FIX products, with 8 Back to School Tips anticipated cost of a prophylaxis treatment regimen to be approximately the same. If you would like to learn more about these products please call the Hemophilia Care Program at 206-689-6507or plan to discuss this at your next HCP visit. For patients wanting to switch, we recommend testing with the new product to see how your factor level responds. Gene therapy to treat Hemophilia B continues to advance. Sustained levels of factor IX in the 5-10% range were reported in late 2011 in patients treated in the United Kingdom. PSBC is participating in a trial where the factor IX is more active so that less has to be used in the gene therapy. Longer acting factor VIII (8) products for Hemophilia A are under study and PSBC is participating in two of those studies. These keep the FVIII in the blood stream about one and a half times longer than do the regular FVIII products. Gene therapy for Hemophilia A is advancing but is still not ready for testing in humans. The factor VIII gene is much larger than the factor IX gene, and that and other issues, have made advances more challenging. Barbara A. Konkle, M.D. If you have questions about the new products or research, please call the PSBC HCP at 206689-6507. You will be directed to the clinical or research team to answer your questions. Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 2 Nutrition Tips for People with Bleeding Disorders By: Liz Adams, PhD, RD Hemophilia Center Child Development and Rehabilitation Center Assistant Professor, Public Health and Preventive Medicine Oregon Health & Science University Joanna Helm, MS, RD, from OHSU Graduate Programs in Human Nutrition, 2014. Following a diet consistent with US Dietary Guidelines is recommended to promote optimal health for the US population, including people with bleeding disorders1. These guidelines are also important for prevention of age-related health outcomes recognized to be priorities in the bleeding disorder population including: maintaining a healthy weight, thereby reducing risk of heart disease, high cholesterol, Type II diabetes; and prevention of osteoporosis2. Maintenance of adequate iron stores is also essential for health, and requires special attention for people with possible ongoing blood loss. For long term bone health, be sure to get enough vitamin D and calcium every day from your diet and supplements. US Dietary Guidelines for Optimal Health: 1) Balance calories with physical activity to manage weight. 2) Consume more foods and nutrients such as fruits, vegetables, whole grains, fat-free and low fat dairy products, and seafood. 3) Consume fewer foods with sodium (salt), saturated fats, trans-fats, cholesterol, added sugars and refined grains. Recommendations for People with Bleeding Disorders: 1. For long term bone health, be sure to get enough vitamin D and calcium every day from your diet and supplements3. (See Table 1. Dietary Reference Intakes for Calcium and Vitamin D.) 2. Maintain a healthy weight to reduce risk for joint disease associated with bleeding disorders. Maintaining a healthy weight also reduces risk for chronic conditions such as diabetes and heart disease. (See Table 2. Body Mass Index (BMI) Guidelines for Children and Adults1.) 3. Iron is essential for health. People with bleeding disorders have increased need for iron if experiencing ongoing blood loss. Iron needs depend upon age and gender. More iron is needed at times of rapid growth such as pregnancy, early childhood and adolescence. Women 14-18 years old need 15 mg of iron/day, women 19-50 need 18 mg, and pregnant women need 27 mg. (See Table 3. Choosing Iron Rich Foods4.) 4. Fish and fish oil supplements. Large doses of omega-3 fatty acids (DHA and EPA) from fish and fish oil supplements may interfere with blood clotting. People who have a bleeding disorder should check with their provider to determine a safe dose. Take control of your food supply to help achieve the diet you want. We live in a world that makes it challenging to choose a healthy diet. We have food companies, TV advertising, and often a food supply that is not focused on health. Continued on page 3. Liz Adams, PhD, RD Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 3 Nutrition Tips...continued from page 2. It can be hard as an individual to navigate this food environment. But you have control over the kinds of foods that you bring into your house, and the kinds of foods you may eat when out of your home. Include more fruits and vegetables and whole grains in the foods you buy and prepare. Watch out for “portion distortion” of restaurant servings. What steps can you take to make the right kinds of foods easy to choose? Table 1: Dietary Reference Intakes for Calcium and Vitamin D3 Age Infant (0-6, 7-12 months) Child (1-3, 4-8 years) Adolescent (9-18 years) Adult (19-50 years) Vitamin D Amount per day 400 IU 600 IU 600 IU 600 IU Calcium 200, 260 milligrams 700, 1000 milligrams 1300 milligrams 1000 milligrams Table 2: Body Mass Index (BMI) Guidelines for Children and Adults1 Category Children and adolescents (BMI for age Percentile range) Adults (BMI) Underweight Less than the 5th percentile Less than 18.5 kg/ m2 Healthy weight 5th percentile to less than the 85th percentile 18.5 to 24.9 kg/m2 Overweight 85th percentile to less than the 95th percentile 25.0 to 29.9 kg/m2 Obese Equal to or greater than the 95th percentile 30.0 kg/m2 or greater Table 3: Choosing Iron Rich Foods4 Whole grains and enriched breads and cereals are good sources of iron. Iron in meat, fish and poultry is especially well absorbed. Dark meats have more iron than lighter meats. Eggs, dried beans and nuts provide more iron than poultry. Dark green leafy vegetables can make important contributions to your total iron intake. Fruits and vegetables are good sources of vitamin C, and help increase iron absorption from other foods. Iron is better absorbed when combined with foods rich in vitamin C, such as orange juice or tomatoes. Milk, yogurt and cheese are good sources of calcium, but provide little iron. Dairy products, calcium, antacids, foods high in fiber and foods high in caffeine interfere with iron absorption and should not be taken at the same time as iron supplements 5. Include more fruits and vegetables and whole grains in the foods you buy and prepare. Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 4 Nutrition Tips...continued from page 3. References: 1. US Dietary Guidelines for Americans, Dietary Guidelines for Americans, 2010; accessed at http://www.health.gov/dietaryguidelines/2010.asp, February 16, 2014. 2. World Federation of Hemophilia, Guidelines for Management of Hemophilia, 2012. 3. National Research Council. Dietary Reference Intakes for Calcium and Vitamin D. Washington, DC: The National Academies Press, 2011. 4. Virginia Cooperative Extension. Iron: Build Strong Blood, May 2009; accessed at http://pubs.ext.vt.edu/348/348-371/348-371.html, February 16, 2014 5. Medline Plus, A service of the US National Library of Medicine, NIH. Taking Iron supplements; accessed at http://www.nlm.nih.gov/medlineplus/ency/ article/007478.htm, March 14, 2014. Does Hemophilia Run in Your Family? By: Barb Oldenburger, RN Pediatric Hemophilia Nurse Hemophilia Care Program Who gets Hemophilia? How is it passed down? Can women have Hemophilia? How do you know if you are a carrier? How do you answer these questions for yourself or your family? Hemophilia is a genetic bleeding disorder. It is not contagious or environmental. The two diagrams show the possibilities of passing down the affected gene that causes Hemophilia. If a man has Hemophilia and has children, all of his daughters will automatically be carriers. His sons will not be affected. If his daughters have children, her sons and daughters each have a 50/50 chance of receiving the affected X chromosome that carries the gene for Hemophilia. The daughters have two X chromosomes, one from mom and one from dad. Because of this most of the time, a daughter who is a carrier has a backup system in place for normal factor production. Occasionally though, her factor level will be low and she will have mild Hemophilia. She may notice symptoms such as heavy bleeding with her periods, excessive bleeding with surgery such as tonsillectomy or tooth extractions for example. Knowing about the Hemophilia that runs in your family is very important, especially in planning for the future. We do carrier testing here at the Hemophilia Care Program. We work with women who are carriers and their OB/GYN regarding specific recommendations when planning for a safe delivery. We help women with mild Hemophilia plan for invasive procedures. There are many treatments to control or prevent bleeding so that you can lead your most productive life. If you have any questions, please do not hesitate to contact us at 206-689-6507. We would be more than happy to speak with you. Knowing about the Hemophilia that runs in your family is very important, especially in planning for the future. Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 5 5 Essential Summer Camp Tips Fun is right around the corner. Here are 5 essential tips for summer camp: 1. Be prepared. Bring your factor and double check for what to bring and not to bring for camp. It’s always a good thing to be prepared in case of an emergency. 2. Reconnect with friends and make new ones. Summer camp is a great opportunity to reminisce with old friends and possibly make a few new ones! Reconnect with friends and make new ones. 3. Lead by being a good example. You could be someone's role model and not even know it! Remember that younger kids may look up to you and are watching your attitude and behavior. 4. Help others. It feels good to help others in need. There will be opportunities to help out at meal times or pitch in to help a counselor or leader with activities. 5. Have fun! Camp is the best place to have fun, try new things, and enjoy the great outdoors with friends. We look forward to seeing you at Camp I-VY! Welcome Dr. Rebecca Kruse-Jarres to PSBC! We welcomed Dr. Rebecca Kruse-Jarres as the new PSBC HCP Director June 30, 2014. Dr. Kruse-Jarres comes to our Program from Tulane University where she has been the Associate Director of the Bleeding Disorders Program, providing care for adults and children with bleeding disorders. She has a message for you! Dear Hemophilia Community, I am very eager to be joining the Puget Sound Blood Center as the new Director of the Hemophilia Care Program this summer. Hemophilia is facing some exciting times and I am very much looking forward to continuing to provide excellent care to you and discussing new treatments as they are emerging. In delighted anticipation of meeting you, Rebecca Kruse-Jarres Welcome Dr. Rebecca Kruse-Jarres! Dr. Rebecca Kruse-Jarres Director of the Hemophilia Care Program Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 6 How to prepare your child for Camp By Cat Stulik, LICSW Well, it’s almost that time of year again. The Bleeding Disorder Foundation of WA is gearing up for another wonderful Camp I-VY experience this July! In recognition of that, I thought I might write about preparing our first time campers for this edition of Factor Fusion. Camp can be a wonderful opportunity for kids to experience spending time away from their family and their community while they commune with nature and other campers. At Camp IVY, staffers work extra hard to ensure that the kids have the best camp experience they can. With that said, there are some things campers and their families can keep in mind to help them have a great camp experience. When making your list of things to pack for camp, I’m sure I don’t need to remind anyone that their clotting factor goes at the top! Campers are responsible for making sure they have enough to last the five days of camp. Also, be sure to include lots of extra supplies: Coban, band-aids, syringes, alcohol wipes, tourniquet and anything else you like to have handy for infusions. Once you’ve got all of your bleeding disorder supplies, remember to bring any other medications/vitamins/supplements you might need on a daily basis. Clothing for camp can vary, I would suggest bringing something waterproof that will fit over your clothes; a lightweight, roomy windbreaker or shell is great for those rainy days and can be easily tied around your waist when the sun comes out. A nice warm hat is good to have for campfire and chilly nights. Bathing suits for swimmers are a must! The pool is heated and morning swims are routine for Camp I-VY. Another important item is a good pair of walking shoes; they should be fairly lightweight and waterproof is better! Keens and Tevas make great camp shoes and are waterproof. There are lots of copycat styles that work great for being in the outdoors and they tend to be less expensive. Being independent and away from the comforts of home while at camp is quite the rite of passage and something that should make you very proud. However, for first time campers, homesickness can be an issue. Please know that there will be other people who are first time campers and that they will understand. Remember to use your supports like your counselor, camp staff and fellow cabin mates to ward off the homesick blues. You can bring something small from home; a photograph or stuffed animal, something that is familiar and comforting. Some people find it also helps to write a letter to loved ones they are missing, whether they end up sending it or not. Having said all of this, you’ll probably be so busy having fun that you won’t have much time to think about missing home. Lastly, make sure you get the camp forms sent in plenty of time! That way, if you’ve forgotten anything and Luke needs to give you a call to let you know they need more information from you, you’re not rushed to get things in on time. Space is limited, so sending forms in early, helps you and your parents stress. We look forward to seeing you at camp and we will do our best to make it an awesome experience! See you in the trees! Cat Once you’ve got all of your bleeding disorder supplies, remember to bring any other medications/vitamins/supplements you might need on a daily basis. Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 7 Excited to join the 2014 Bloody Fun Walk, but unsure how to start a walking program? By: Lorraine Flaherty, PT Here are some tips to get you started on the road to success: 1. Get the all-clear. If you are over 40 and/or haven’t exercised in a while, you may want to talk to your doctor or take this quiz and see if you need to before getting started: http://www.startwalkingnow.org/documents/Questionnaire.pdf 2. Start slow. You have lots of time to get ready for the October walk if you start now. There are a variety of beginner schedules available to help you. Here are some good ones: http://www.thewalkingsite.com/12week.html http://www.mayoclinic.org/healthy-living/fitness/in-depth/walking/art-20050972? Try to commit to a plan that you think you can be successful at 75% of the time or more. footprints=mine http://www.heart.org/idc/groups/heart-public/@wcm/@fc/documents/downloadable/ ucm_449261.pdf You’ll notice that the walk programs will range from 6 week to 12 weeks and progress to 3060 minutes of walking- but they all start out with just 5-10 minutes! 3. Set realistic goals. Try to commit to a plan that you think you can be successful at 75% of the time or more. Don’t set yourself up for failure. Ask yourself where and when you are most likely to walk, (mornings are a good, no excuses time for many people- though it takes some getting used to!) 4. Dress the part. It is often helpful to wear layers, a hat to protect you from the elements, sunglasses and good walking shoes. Good online resources for selecting walking shoes can be found here: http://walking.about.com/cs/beginners/a/blhowshoes.htm http://www.mayoclinic.org/healthy-living/fitness/in-depth/walking/art-20043897 http://www.thewalkingsite.com/shoes.html Don’t forget to carry water. 5. Have a plan B. Is it starting to get dark before you get home from work? Did Joey cancel on you? Is it raining again? Even if you can’t do your walking the way you would prefer to, try to stick to your habit and do some walking anyway. Maybe you can walk indoors at the mall, at a gym or community center or even in a long hallway. Maybe you can listen to music or audiobooks if Joey can’t keep you company. You may want to consider investing in a treadmill. Then you can walk any time of day, in any weather, while you talk, read or watch a movie. 6. Be patient with yourself. Remember that making a change is often the hardest in the beginning. It will get easier over time when it becomes a habit. Trying to do a short walk almost every day will help most people with this. Continued on page 7. Rio Jones and Lorraine Flaherty, PT Factor Fusion: Connecting with the Bleeding Disorder Community. PAGE 8 Excited to join the 2014 Hemophilia Walk...continued from page 7. 7. Expect challenges. Even in the best case scenario you may have struggles as you try to increase your fitness. You may have pain or get injured, you may get out of your routine, you may get bored. That is all perfectly natural. Try to remind yourself that most difficulties can be surmounted if you are willing to try again and get help when you need it. 8. Get support. Tell your friends, family and co-workers about your plan. Go online to find walking routes or clubs: http://www.startwalkingnow.org/home.jsp. Exercising with others is the key to consistency for many people. And don’t forget, your friendly physical therapist is available at 206-689-6507 to help you with questions about stretching, pain, shoes, and even how to make exercise more interesting and meaningful for you. Good luck! See you at the 2014 Bloody Fun Walk if not before! From left to right, back row: Brian Flaherty, Sarah Galdzicka, Bob Gleason. Front row: Amy LoTurco, Lorraine Flaherty and Carissa Flaherty. If your child is starting a new school this year, talk with your pediatric nurse to see if a school visit is needed.