Onward and Upward! Q&A about Hemophilia with Jim Brown
Jim Brown wants to improve the lives of people with a bleeding disorder.
Answers4Families recently had the opportunity to have a Q&A with Jim Brown, who works with National Hemophilia Foundation Nebraska and Midwest chapters. Mr. Brown has also worked as an RN and earned his Law Degree through UNL. He now works for a home care company that provides blood factor, ancillary supplies, nursing service and other types of support to persons with bleeding disorders, so that the family can treat the bleeding disorder at home to avoid costly emergency room visits.
Brown was also born with severe Factor IX hemophilia and has a 14 year-old grandson who inherited hemophilia. Brown shared his story as well as a wealth of information concerning bleeding disorders and how treatment has improved throughout the years, education, and misconceptions about bleeding disorders.
Answers4Families: What has been your experience with hemophilia?
Jim Brown: I had an older brother who was also born with severe Factor IX hemophilia (he died in 1985) and when we were born, there was very little in the way of treatment--mostly wrap the affected area, rest it and apply ice--and take aspirin (which we now know is harmful to persons with bleeding disorders). One of the major problems when I or my brother was having a bleed when we were younger was trying to replace the blood loss. To try and get enough blood in us using whole blood created the danger of overloading the circulatory system. My parents were told that neither of us would probably live to be 16, and surely wouldn't live to be 21! Imagine the effect of that kind of prognosis to parents! Both of us far exceeded that prognostication, as my brother was 46 when he died, and I am now 63. The first transfusion I remember was with my father laying on one gurney and me laying on another in the emergency room with tubing going between us to get blood from him to me to try and stop a bleed from a cut.
My brother and I both suffered extensive joint damage because of frequent bleeds into joints--one of the most common sites for bleeds--there was literally not much that could be done other than to wait for the bleed to be over. I've had both knees replaced, and have significantly limited range of motion in many other joints.
I am pleased to say that the treatment and care for hemophilia has come a long way since we were younger. The treatment eventually went from whole blood to plasma to cryoprecipitate to blood "factor". It is now possible to isolate and extract the specific blood factor we are deficient in and make it available for home infusion. The factor comes in a powdered form with sterile saline to reconstitute it. It can be mixed and given at home, at school, on vacations, etc. Now we can treat bleeding episodes immediately for better control of the bleed, less joint damage, better quality of life, less missed school or work, etc. The factor is very expensive though, because it is either extracted from human blood or made by artificial means which requires compliance with all FDA requirements. An average hemophiliac can use approximately $250,000+ of factor ina year! But it is hard to put a price on the improved quality of life, reduced joint damage, etc. During the 1980s, about one third--10,000--of the hemophiliacs died from HIV/AIDS from contaminated blood used in making factor. That was before science knew what caused AIDS and then how to treat blood supplies to make them safe, which also contributed significantly to the cost of factor.
I have a 14 1/2 year-old grandson who inherited my hemophilia, (it is hereditary--passed through the female--so my daughter was the carrier) and I am excited and encouraged by the developments that have been made in the products and treatment available for hemophiliacs todayand in the future. Most hemophiliacs will now be able to enjoy a more "normal" life.
A4F: What do you do for a living? What goals have you yet to achieve?
JB: I work now for a home care company that provides blood factor, ancillary supplies, nursing service and other types of support to persons with bleeding disorders, so that the family can treat the bleeding disorder quickly and most often at home to avoid the delays and additional expense of having to go to an emergency room. If a joint bleed occurs in the middle o f the night, as a result of a child's activities, it can be treated in minutes and with little disruption to the child and family, also resulting in less damage to joints and tissue.
I'm old enough that many of my career goas have been achieved. I am an RN, with a BS degree in Health Education from UNL, and a Law Degree (Juris Doctor) from the University of Nebraska Law College. I've practices each of those professions over the years. My main goals in life now are to try and make the quality of life better for my grandson and other young men and women with bleeding disorders. I want them to have an essentially "normal" life, while living with their bleeding disorders. I'd love it if I work myself out of a job because of developments in treatment and care of bleeding disorders!
A4F: How did you come to be a part of the National Hemophilia Foundation? What is your role with the organization?
JB: My parents helped form the first hemophilia chapter in Nebraska when my brother and I were young, in the early 1950s. There was a National Hemophilia Foundation at that time and many states had local chapters. The Nebraska Chapter of NHF went through cycles and was reactivated in the 1980s. I have served as President of the Nebraska chapter and in other capacities, and currently work on committees of the Nebraska Chapter. I also just finished two terms on the Board of Hemophilia Foundation of Minnesota/Dakotas, and am in my second term on the Board of Hemophilia of Iowa, and was recently elected to the Board of Midwest Hemophilia Association in Kansas City. I also serve on the Board of another national organization--the Hemophilia Federation of America, which many consider to be more consumer oriented than NHF which is considered to be more provider oriented (nurses, doctor, researchers, etc.).
A4F: What are some common misconceptions you often find yourself explaining when presenting or talking with people unfamiliar with bleeding disorders?
JB: There are several. First is probably the notion that hemophiliacs will bleed to death with the slightest cut, and that hemophiliacs can't do a lot of activities because of the danger of bleeds. Hemophiliacs bleed longer but not faster than any other person because they are missing or deficient in one of the factors involved in blood clotting. The best illustration I have heard of is comparing it to a row of dominos standing up in a row--you tip one over, and it tips the next, and so on. With blood clotting, it is said there are 15 "factors" involved in the clotting process. One activates or precipitates the next, which precipitates the next and so on. If one factor is missing or deficient, it stops the process--thus the "row of dominos" stops toppling and the clotting process is disrupted. By giving the missing factor through infusion, we can fill in the missing "domino" at least temporarily to facilitate the clotting process and form a clot or otherwise stop the bleeding.
There are different severities of hemophilia--mild, moderate and severe--depending on the amount (or lack of) factor being produced in the individual. Obviously, the severe hemophiliacs are likely to have more, and more severe bleeds, but these too are lessened with factor. So the second misconception is that hemophiliacs can't participate in any "dangerous" or strenuous activities. I know of hemophiliacs that have played collegiate hockey, high school football, high school wrestling, high school and college baseball, competitive international weight lifting, etc. Our participation is almost unlimited--as long as we exercise some caution and common sense, and with factor available at any time for self-infusion when and if we are injured. My hobby is building street rods!
A third misconception is that cuts are the worst type of bleed. Actually, just the opposite may be the case. It is said that it is not the bleeds you can see that are the problem, it is the ones you can't see. If you can see a cut, for example, you can put a bandage on it, put pressure on it, suture it up, etc. A bleed that you can't see (a silent bleed) is on that may be occuring internally in a joint, in the abdomen, in the head, etc. Because it is unseen, it can go on for longer and become more serious before it is discovered and treated.
A fourth misconception is that only males have bleeding disorders. Certainly the highest incidence of hemophilia is in males, probably 95%+, but occastionally a female can be diagnosed with hemophilia. There is another bleeding disorder--von Willibrand Disease--that occurs in both males and females, but is considered the most undiagnosed or underdiagnosed bleeding disorder. Many times it is just felt that females have difficult or long periods when in fact it is vWD. The greatest increase in the utilization of Hemophilia Treatment Centers is now for women with bleeding disorders.
A final misconception--for the purposes of discussion--is that all cases of bleeding disorders, especially hemophilia, are inherited. It is often called the "Royal Disease" because it was somewhat prevalent in the monarchies of Europe. However it is now known that approximately 25-33% of bleeding disorders are the result of spontaneous gene mutation--that is, there was not family history of hemophilia or vWB. This is sometimes harder for parents to accept when it is discovered, it comes out of the clear blue. Whereas when it is inherited, families have some awareness of what hemophilia is all about, and it is known that it could occur in future generations.
A4F: Let's say you are teaching a nurshing class, or a class for caregivers, what is something about bleeding and clotting disorders you would teach them that they wouldn't find in textbook or lecture?
JB: Six things. First, listen to the person with a bleeding disorder or their parent! They have lived with hemophilia, they know what is going on with their body. They know what works for them. Second, treat first, test later. Too often emergency doctors want to run all their diagnostic tests first. It is imperative that the person with a bleeding disorder be treated with factor and other measures first. That way, the blood clotting factor can begin working to reduce the severity and damage of the bleed while the toehr diagnostic tests are being done--X-rays, etc. Third, be particularly concerned about the possibility of internal bleeds. These can be the more dangerous ones because they may be harder to detect or diagnose. Fourth, understand that the psychological/emotional well being of the person with a bleeding disorder may be as important, or more important than the physical well being. I marvel to this day at how my parents let my brother and me do things they were told we shouldn't do, so that we were not seen as misfitswho couldn't do the things a normal child could do. I have no doubt that our psyches are better adjusted, and we adapted better to life than had we been placed in a bubble and not allowed to experience life to its fullest. Fifth, be aware of the distinct possibility that a female experiencing difficult menstrual periods may in fact have a bleeding disorder. Sixth, when a bleed is happening or suspected, practice R.I.C.E (Rest, Ice, Compression, Elevation). This can help reduce the severity of a bleed until factor or other treatment can be initiated. This is good practice for many traumatic injuries.
A4F: What is your personal mission statement:
JB: My mission is to improve the lives of anybody with a bleeding disorder. Beyond that, I consider myself to be truly blessed and I try to live my life by two mottos. The first is Onward and Upward! I feel that all of life's activities should be directed to moving forward with life and improving life for others--that we leave the world a better place. The other is a motto my brother often quoted: "I complained because I had no shoes until I met a man who had no feet." We often think we have a lot to complain about--until we see somebody that has greater limitations and needs than we do. We should be thankful for what we have, and make the most of whatever abilities and opportunities we have, especially to help others. We need to be careful we don't confuse "I can't" with "I won't". A Chinese proverb reads: A journey of a thousand miles begins with a single step. Too often we are reluctant to make that first step on a life journey of helping others that will take us a thousand miles.