HazelBecker.net

When the FDA approved a new treatment for some hemophilia patients, including those (like me) with Factor XI deficiency, a few people asked what that would mean for me. I emailed Carolyn Francis, the hemophilia coordinator at Georgetown University Hospital, to find out, and her response was – “it’s complicated.” Here’s the story, as sI understand it.
The new treatment, called Octaplas®, is administered in much the same way as the fresh frozen plasma (FFP) I have received in the past. (See below for the back-story, if you don’t already know it.) Octaplas® is made of pooled human plasma that’s treated with solvents and detergents to scrub out viruses, including hepatitis A, herpes simplex, and parvovirus. In its current iteration, it has been used in Europe since 2006. FDA’s action last week means it will be marketed in the United States.
Along with scrubbing and cleansing, the manufacturer says the fact that its product is made of pooled plasma has an additional benefit: the potential to dilute antibodies in a donor’s plasma that, if present in large numbers, can cause allergic reactions, including swelling, hives, and anaphalaxis. The last time I had a plasma infusion, I had a severe reaction that had to be treated with steroids, so I would welcome this relief. With a pooled product, perhaps whatever made me swell up would have been diluted so that at least the reaction would not have been so sever.
But let’s not all jump for joy at the FDA’s approval of Octaplas®. The agency’s action was only one hurdle, Carolyn told me. The head of the hospital’s blood bank says the facility has not decided whether to bring the product into its supply. First, it would have to be approved by a committee of people who will consider cost, benefit, and risk before deciding. Even before that, it likely would have to be approved by Medstar, Georgetown’s parent company.
Cost is critical because the substance reportedly is expensive. The Georgetown committee probably will wait and see whether Medicare approves its use before going on to consider whether its benefits outweigh the risks of using it. If not, they are not likely to approve it either.
Even if they decide to include Octaplas® in their blood supply, my case might not warrant its use. The first group most likely to use it would be those with thrombotic thrombocytopenic purpura (TTP), a rare blood disorder that suppresses an enzyme and thereby causes clots to form in small blood vessels. TTP is generally treated by plasmapheresis, a process in which the plasma is removed from the patient’s blood and replaced with FFP. These plasma exchanges sometimes need to be repeated daily for one to eight weeks, increasing the patient’s chance of having a transfusion reaction.
People who have had reactions to past infusions might (or might not) be treated with Octaplas®, and cost might not be the only consideration. One severe side effect of the substance found during clinical trials was anaphylactic shock, which also is a complication seen in FFP treatment. If we weren’t eliminating that risk, what would make this new treatment worthwhile?
I’m only writing this to show what makes this decision so complicated. I’m not the one who will decide – that task will be left up to my hematologist and his blood supplier at Georgetown. Let’s hope we don’t have reason to find out anytime soon!
My back-story
For those who don’t know, here’s the background: I am Factor XI-deficient big-time. In past tests my Factor XI level has been measured at less than 0.5% of normal. This genetic disorder is autosomal dominant, meaning it’s carried on the X chromosome and can affect both males and females. My daughters have been tested and found to have between 50% and 60% of normal levels, which is generally thought to be “enough to get by” without pretreatment. My case is more severe (as we saw when Allison was born and I bled a lot) and I am pre-treated before surgical procedures and invasive tests.
My bleeding condition was know when I was young (I bled a lot after a tonsillectomy) but not diagnosed until the 1970s, when I happened into the care of Alan Klatsky in Hartford. Before I got pregnant he had me tested for clotting factors after hearing previous physicians’ suspicions that I had Christmas disease (Factor IX deficiency).
For a long time now I’ve been treated by Craig Kessler, head of Georgetown’s Coagulation Laboratory. I always appreciate the time he and Carolyn are willing to take to help me learn. 

If you don’t live in the District of Columbia, you may not understand the significance of President Obama’s decision to display D.C. license plates inscribed “Taxation Without Representation” on his vehicles used for the inauguration and throughout his second term. In fact, some folks may believe the status quo – in which D.C. residents pay federal and municipal taxes but are not represented in Congress – is correct.
As a longtime D.C. resident, I have somewhat mixed feelings in this whole debate. One the one hand, I know that my citizenship in this country is not equal to that of residents of any of the 50 states because we do not have voting representatives in either chamber of Congress. We pay federal taxes at the same rates as state residents – we don’t get a rebate or exemption just because we don’t have a say in enacting laws that apply to us the same way they do to everyone else in the country.
Moreover, our elected municipal government levies taxes that we must pay but does not have the final say in how D.C. tax money is spent. Congress can override our laws, including budget provisions, and frequently uses these votes to impose social dictates that go against the will of the city’s population. Among the social issues that have been addressed in this way in the last few decades are school choice, gun control, and AIDS treatment and prevention, just to name a few. The issues change with the leadership of the two chambers and their committees with jurisdiction over D.C. matters. What doesn’t tend to change is their idea that they can use the District as a laboratory for new ideas or a step toward achieving social goals that are outside the norm in the country as a whole and against the wishes of the only citizenry to which the measures apply.
In all probability our nonvoting delegate in the House of Representatives, Eleanor Holmes Norton, provides constituent services to D.C. residents as well as state representatives, and if she’s not doing so, we certainly could decide to vote her out. This is where my “mixed feelings” come in – part of me says, “what difference does it really make?” Sometimes I believe it would be just as well for us to be exempt from paying federal taxes, like Puerto Rico, Guam, and other U.S. territories.
But then I think of the unfairness, the lack of equality I feel with U.S. citizens who live in a state. When the system was set up, D.C.’s population was nowhere near the hundreds of thousands who inhabit the city today. The changes Congress enacted in the 1960s and ‘70s allowing us to vote in Presidential elections and for municipal leaders don’t go far enough in acknowledging that our city is no longer the small borough it was in the early 1800s when the federal government set up shop here. Our young men must register with the Selective Service System and were subject to conscription until the draft was eliminated after the Vietnam War. We pay federal taxes, and we don’t have a vote when Congress decides how we are to spend our local tax money. Fundamentally, something is wrong here.
 I remember having this discussion with my Uncle Dan several years ago. He asked me why I held the political view that D.C. residents should have voting representation in Congress when the Constitution established the District of Columbia as a “federal city” under the exclusive control of the Congress. I’m not sure exactly how I presented my case, but I was surprised to hear him acknowledge afterwards that my arguments were causing him to rethink his position.
I’m sure there’s a political side to the change in President Obama’s stance on displaying D.C. license plates that say “Taxation Without Representation” on his cars – it’s not just that someone made a good case to him, as I must have all those years ago in discussing this with my uncle. Even so, I have to take it as a hopeful sign – a step in the right direction. First, Dan Schusterman. Now, Barack Obama.
Who’s next?

Those who don’t know yoga may have been puzzled by my recent Facebook status update: “A full bind!” Those who know yoga and also know me may not have believed it. That’s OK – I didn’t either! I’m amazed at how far I have come in the last few years, fitness-wise.

This story goes back almost six years now. In my last few months at BNA I worked part-time, and I joined a health club not far from home. I got into a two-day-a-week routine and signed up with a personal trainer, Jen Young, and in short order I was back in physical therapy after stretching too far in the wrong direction trying to do a yoga pose she had showed me. It’s a twisting thing that gave me an incredible release, and I wanted to feel that again – so I tried on my own. Mistake. Not only was I a weakling and an idiot when it came to muscles and coordination, but I had no balance or flexibility. I could do aerobic exercise comfortably on a recumbent bike or a treadmill – that was about it.
After a few months of physical therapy, I was ready to train again, and Jen got me started with exercises the PT thought would be helpful. I also had a habit of rolling my foot to the outside and, sometimes, falling because of that. As a remedy, the therapist assigned exercises designed to strengthen my lower abdominal muscles and the quadriceps right above the knee cap. Jen took it from there, and within a few months I understood how various muscles worked and how to isolate them. Eventually I reached a plateau and decided to work out on my own until I was ready for more challenge.
I made progress on the weight machines and eventually switched to the ellipticals for aerobic exercise. These machines sat just outside the group fitness studio, where I saw a few friends – mostly younger than I was but including one woman my age. The first day I joined them, I fell off the resistance ball and bruised my tailbone! And, standing on one foot? No way that could happen.
My first class was Abs and Stretch, and I went to it twice a week. I learned that “core” didn’t mean the center of an apple. Eventually I was able to hold a plank for 45 seconds. And I never fell off the ball again.
The instructor, Judy Martini Abshire, taught an earlier class that included movement and weight-lifting. I started going into the studio in the middle of that class and found that I was getting a great aerobics work-out in there. Not being a particularly good dancer, I didn’t know what a “mamba” was, and being directionally challenged made things difficult for everyone around me when I went to the left while they went to the right. But after a time I extended my workouts to include all of both classes – an intensive two-hour session that I generally attended twice a week.
Somehow, Judy can teach a class of 45 people ranging in age from 12 to 80 and keep everyone working at his/her own level. The modifications she suggests for people with this weakness/injury or that present options that have helped me work on every part of my body. Over time I gained strength. I have increased my weights from 1s and 3s to 5s and 8s. I became a little more flexible. I stopped telling myself I couldn’t or shouldn’t try some of the exercises. And, I found that I could reach things more easily at home, pick up and carry heavy things, and generally take care of more things on my own. Needless to say, that feels good – and I don’t remember the last time I fell without tripping over something in the way.
After changing gyms in mid-2011, I also started working out almost every morning. My twice-a-week two-hour workout was no longer available, so I started trying other classes. The one that made a difference for me is called Yoga for Jocks. It’s not really what it says – I don’t think very many of the people in the class are athletes. But it is a very athletic class, without the spiritual focus that some yoga classes have. Instead, the focus is on breathing, strength, and flexibility. I have learned to stretch my muscles and, recently, have begun to improve my balance.
Now, about the full bind. I was, indeed, doing a side angle pose, and I was stretched beyond my imagination. My balance was good that day, and when Kelli said some of us should reach around and try it, I did. And, I did it!
Today it would not have happened – no balance at all. But I’ve promised myself I’ll try again. And someday, who knows? It might even happen again.

There’s an interesting article in yesterday’s Washington Post about how atheists talk with their children about terrible things like the Newtown shootings. It made me stop and think about my own beliefs – which, I think, are no different from those espoused by parents in the article. I touched on my “religious” practices in a post I wrote last week about why I did not attend the memorial service for a BNA colleague. In this post I’ll explore my own “religious beliefs” a little more.

First let me say, I identify myself as Jewish – no question about that. For many years Robert and I have been active in a small Jewish congregation in the Washington area, Kehila Chadasha – a “congregation without walls” that meets here and there, on a regular schedule, to celebrate holidays together and offer Jewish education for children and adults. We joined Kehila when our daughters were young to provide a more active Jewish environment in which they could learn the traditions we were raised with and become b’nai mitzvot if they wanted to. We’ve stayed active because Kehila is a close-knit community of families we feel close to and are comfortable with.
For me, at least, religion has nothing to do with it. I don’t believe in a “higher power,” with or without the miracles and unimaginable “explanations” for earthly phenomena (floods, tsunamis, spontaneous fires, plagues) that fill our history books, including the “holy” books. I’m not agnostic – I don’t question, I just don’t believe it. At Kehila’s services and holiday celebrations I’ve grown to love the music accompanying the prayers, which I sing because it feels good to join others in song. But I can’t bring myself to twist it all up into a “G-d” or force beyond “that’s just the way it is.”
And the food! Combining Robert’s family recipes with a few of mine, plus others we’ve explored from books and Kehila cooking demonstrations, we eat well – and, well, Jewish! Oh, we’ve embraced cooking from other cultures, and perhaps no holiday tops Thanksgiving for me with its lack of religious connotations – unless you consider our worship of food traditions on that day to be religious. But our year goes ‘round with the foods we love: plum cake for Rosh Hashanah, honey cake for Yom Kippur, homemade applesauce for Sukkot and with or without latkes at Chanukah, hamentashen not only at Purim but sweetening our lot all year … and a Passover table with too many traditions to mention in a blog post about something else.
So yes, I am certainly Jewish, food-wise. And I think (hope) we used the Jewish holidays and teachings well in raising our children to treat other people with respect and dignity. I think there’s something in there, too, about personal responsibility – owning up to their mistakes, striving to be the best people they can, and caring for others. Remembering and forgiving, themselves as well as others. Caring for and safeguarding the natural world. I think those are the main ones, for me at least. That probably qualifies me as more than just “raised Jewish,” as some others have chosen to say.
I’m not sure yet whether I think there’s a conflict between my personal brand of Judaism and being an atheist. Perhaps others would dispute that, saying that the belief in “one G-d” is fundamental to Judaism. But there’s also the perspective that I don’t worship idols and don’t believe in the Christian trinity, which the Jewish one-G-d declaration has countered for centuries.
So, there it is – at least as far as I’ve taken it so far. Perhaps there will be more on this subject in the future. Or perhaps, for me, that is enough.

I could not, for some reason, bring myself to go to a memorial service today for someone I worked with for nearly 30 years. Instead, I fell back on an “escape” from organized religion that I used quite often in my 20s – going to nature.

I’m not sure why I couldn’t go today. Perhaps it was that I wasn’t that close with Cindy. It would have been nice to see some of my BBNA folks, to let them know I share their sense of loss. But maybe that was my stubborn side’s point in not letting me go: if I want to see them, I should arrange it on my own and not take the opportunistic route. Someone’s memorial service is not, after all, a time to be social.
I don’t think it’s the cancer thing. At least, not Robert’s cancer – that nasty stuff still lurks in the back of my mind all the time, but I don’t let it get in the way. And, I know there’s no connection between Cindy’s death and Robert’s wellness. More likely, it would have something to do with all my BNA people who are no longer with us. I don’t even want to think back through the list – it’s too long, and it makes me too sad.
I think the real reason is the rituals I could not bring myself to go through. Not the Christian ones – I’m sure I would have been interested in the Presbyterian liturgy – I found myself surprisingly comforted by the pastor’s words at the Lutheran service when Susan died a couple of years ago. It’s more the social rituals – seeing the BNA people under these same circumstances, yet one more time. Acknowledging each other’s presence, shaking our heads in disbelief that another of our colleagues had died too soon, too young, with too much left to do in this world.
So, on from the maudlin: I took Chewey to Rock Creek Park instead.
I thought a lot about Cindy – her wit, her perception, her focus. I remembered all the times she patiently answered my questions at BNA shareholders’ meetings, keeping the focus on her duty as a director and corporate secretary to look out for the best interests of the company’s owners. I also remembered the times she brought humor into what otherwise could have been dull and dry meetings, making sure that we laughed and had fun even while we attended to serious business.
I can’t help but think she would have been pleased to be remembered in nature – a setting where I seldom saw her, but one in which I’m sure she felt at home. What could be more religious, more awe-inspiring than clear, crisp air and a vista showing Rock Creek and the hillside beyond?
So, this is for you, Cindy Bolbach. I hope you are smiling still.