I love discovering new people in our amazing hemophilia community, along with their talents and accomplishments. So I was delighted when Ray Perreault contacted me this year to introduce himself and share a new book he self-published. Ray has severe factor IX deficiency, and his new book, A Life Without IX, is a memoir. To Ray, this is simply a memoir of his life, but his life has been one of unmitigated pain and suffering. He tells this book (mostly) in the third-person, through a character named John. John’s life is Ray’s life. And it is remarkable.
The 173-page book details what it is like to live with chronic, torturing pain, from bleeds treated with plasma, to the “Miracle,” factor IX concentrate. I’ve read people’s stories on pain before, and I have to admit this is the most carefully constructed, detailed recollection of enduring waves of pain that dominate your life that I have read. While reading, you get inside the mind of “John,” experiencing what he experiences. What do people in searing pain think, how do they strategize how to move across the room, what do they dream of at night?
The book covers childhood, high school, adulthood, even a first marriage that brought even more pain to John’s life, then the birth of his child, and at last a second marriage, which brings joy. A book like this will expand your concept of what pain is, and being fresh appreciation for those struggling to survive it minute by minute, day by day.
Ray Perreault is a survivor who deserves the highest respect. His wife summed it perfectly in the book: “You always had the courage to bounce back…it’s the most amazing thing about you.” Resilience in the face of crushing and crippling pain, strength in the face of the negative attitudes and discrimination of “others.” Do not read this book looking for lofty inspiration, though; it reads like the no holds barred memoirs of a war veteran who has seen the worst in battle. But through it all, there is a human spirit that subtly rises above it all, and that embodies itself in this book.
Ray Perreault
I am also an author and editor and so cannot conclude with a few caveats. Self-published books often have their share of problems and glitches, and so it is with this book. Layout: expect many big gaps and white spaces (half page sometimes) in the book where the text flow did not get corrected. Copy: some grammatical errors, many stylistic inconsistencies. Content: the subject matter is vitally important and the story is basically good. But it rambles, and is confusing. It bounces about chronologically. Why Ray chose to tell it in the third person is a bit of a mystery, and the voice changes sometimes from third-person to first-person with no explanation who the narrator is. But most importantly there is a lack of medical information (HIV is barely explained, leaving too many questions; factor IX is incorrectly explained and referenced; hemophilia is not adequately explained).
My advice is to read this for the story of the man, and it is a story that deserves to be told. It’s a story of perseverance and courage, fortitude and hope. With thorough, professional editing, this book would carry even more impact than it already does. Well done for a first book, Ray!
This week marks the first ever International Plasma Awareness Week, an opportunity to recognzie the importance of plasma in our lives, and the need to donate blood to provide the life-saving liquid to those in need. It’s especially important to many with hemophilia throughout the world as donated blood donations can be fractionated into factor VIII and IX for many hemophilia patients who use plasma therapy.
You learned this in high school science class but here it is again: Plasma is the clear, straw-colored liquid portion of blood that remains after red blood cells, white blood cells, platelets and other cellular components are removed. It is the single largest component of human blood, comprising about 55 percent, and contains water, salts, enzymes, antibodies and other proteins. It is a clear, straw colored liquid that is 90% water and serves as a transporting medium for cells and a variety of substances vital to the human body.
In the US, we often think only of recombinant factor when we think about treating hemophilia, but plasma therapies remain the mainstay for many with hemophilia, those with inhibitors and undergoing ITI, and those with von Willebrand disease patients.
In addition, plasma protein therapies are used to in emergency and surgical medicine.Plasma protein therapies are not interchangeable and have been defined by regulators as sole-source biologic products because no generics or substitutions exist. In addition, their biological nature demands storage and handling requirements by specialty distributors that ensure their safety.
Source plasma is plasma that is collected exclusively for further manufacturing through a process called plasmapherisis. Recovered plasma is collected through whole blood donation which has been separated into its cellular components.
Safety and quality of plasma protein therapies is the top priority of the plasma protein therapeutics industry. Both collectors and manufacturers adhere to strict regulatory policies and have instituted Good Manufacturing Practices in every step of plasma collection and manufacturing processes.
Plasma-derived therapies depend on the generosity and commitment of healthy donors. Source plasma is collected in over 450 specialized donor centers in the U.S., Canada, Germany, Austria and the Czech Republic. Source plasma collection in the U.S. is regulated by the Food and Drug Administration and by the European Medicines Agency and national regulatory authorities in Europe. Additionally, 436 plasma collection centers are also certified by the International Quality Plasma Program (IQPP), a rigorous, voluntary program that goes beyond regulatory requirements to help ensure donor safety and further improve the quality of plasma used to manufacture therapies.
This is a good week to donate blood then! If you can make the time, please visit your local blood donation center and give the gift of life.
Manufacturers set up large booths to view new
programs and products
I’m
still in Anaheim, about to enjoy the sunny California weather for two more
days. I’ve already
enjoyed the past five days with my bleeding disorder
community at the 65th annual National Hemophilia Foundation meeting,
with about 3,000 colleagues, patients, families, corporations and friends.
These just keep betting better and better!
Consumers get to browse many booths
The
theme was “United in Progress,” aptly named as all the divergent groups in our
community work together to push our agenda through obstacles such as funding
cuts and insurance threats. While I could only attend a couple of sessions,
they were powerful.
Stakeholders’ Meeting
Laurie Kelley with Kevin Shaughnessy
The
first was NHF’s Stakeholder Meeting, a comprehensive three-hour session that brought together
key opinion leaders in the US.
Laurie Kelley with Debbi
Adamkin of the Florida Chapter
Dawn
Rotellini of NHF first discussed the need to establish conflict of interest
boundaries and policies for chapters working with industry (pharmaceutical
companies and specialty pharmacies). There’s even a need to define “industry,”
as HTCs, half of which sell factor, don’t like to be included as industry. 51
chapters have different guidelines so there’s a need to standardized policies
to industry. A lively debate ensued about how to create a template and work with industry’s legal system to approve this.
Laurie with Julie Birkofer and Jan Bult
of the PPTA
Next,
we had a federal update from Ellen Riker, of MARC Associates in Washington DC. As we all know, the federal fiscal year began October 1. And there is no
appropriations bill for health programs or agencies. So now there is an extension
of current funding levels, with a sequester likely to occur again in 2014. I was
amazed at the low level of funding for HTCs. HRSA/MCHB funding remains flat at
$4.9 million for HTCs and coordinating centers. The CDC receives approximately
$7 million (not a lot) for hemophilia for surveillance, research, outreach and
education (grants to HTCs and patient organizations, all impacted negatively by the 2013
sequester). Most alarming is that the head of CDC doesn’t think chronic disorders
should be in CDC to begin with! So new cuts are a threat. Ellen reminded us
that we would not have gotten through our HIV days without CDC; we need each
other.
An
update on HR 460 Bill, which NHF is supporting: this prevents private insurance
plans from requiring cost-sharing for drugs on specialty tiers. Only 14% of
health plans using specialty tiers; specialty drug spending represents only 2%
of overall health plan spending. NHF wants to prevent insurance plans from
putting factor in the specialty tier, where copays could be astronomical.
The legendary Barry Haarde!
Michelle
Rice gave an update on the Affordable Care Act “Marketplaces”—those websites
that will allow consumers to compare health care plans to find the right one at
the lowest cost. We learned that the Marketplaces went live just the day
before, and many crashed as huge numbers of consumers logged in! Her own staff
went on the federal site and it was confusing. Michelle presented a great spreadsheet
that will help breakdown the Marketplace by level (denoted by “metal”—platinum,
bronze, etc), by state, etc. This will be a vital and helpful guide for those with bleeding disorders using the Marketplace. Glitches in the Marketplace include: some had navigators but called them different things, which was confusing. Some organizations
are putting up tools based on what feds said would be there but the tools
aren’t right because the feds haven’t followed through with what they said they
would provide!
Laurie Kelley withAndy Matthews
and Chad Frederickson
The
primary message was: be patient. We all have until December 15 to pick a plan for
January 1. Wait before accessing the Marketplace so the bugs can be fixed.
Laurie with hemo moms Elizabeth Pulley
and Julie Heinrich
Uniting the World
Laurie with friends from Nigeria
Another
session I attended was about the twinning program of the World Federation of
Hemophilia. Present was Megan Adediran,
my friend and colleague from Nigeria. Megan is the president and cofounder of
the Haemophilia
Foundation of Nigeria, and mother of
two boys with hemophilia. She was present with husband Thomas and another
mother of a child with hemophilia. She gave a fiery speech about the importance
of the twinning program with NHF, to close the gap between two countries, two
worlds, two different approaches to hemophilia. She related how hard the HFN is
working to reach its people in need. Nigeria is a huge country, where only
about 150 people with hemophilia out of approximately 10,000 have been
diagnosed, and where thousands more wait to be identified and helped. It could
take a lifetime to find them, but the twinning assures that we are in this
together for the long haul, United in Progress.
Laurie Kelley with the
lovable Tater, who has hemophilia
It
was a great meeting, with sessions on social media, von Willebrand Disease,
hepatitis C updates, gene therapy research, obesity, pain management and even
dating when you have hemophilia. There was something for everyone.
We’re really grateful to NHF also for allowing Save One Life, my nonprofit that sponsors children with bleeding disorders in developing countries, a table to help us gather more sponsorships. We did pick up a lot of new sponsors, hopefully helping us to reach 85 new sponsorships before December 31. Go to www.saveonelife.net if you can sponsor a needy child in poverty with a bleeding disorder!
With Debbie de la Riva, former ED of
Lone Star Chapter
It
is always wonderful to meet up with families I’ve known for so long, going on
23 years for many. One special visitor was 25-year-old Emmanuel, from Ghana,
Africa. I’ve known “Kofi” for about 5 years, when I first went to Ghana, and
now he is attending college in UC Riverside. Living so close, he was able to
take a bus, and was invited in as a guest of NHF. He has factor VIII
deficiency, and is bright, articulate and energetic. It was wonderful to have
him here, especially as he could meet the Nigerians! But I always wish more of
our own US families could attend this remarkable event. I’d like to see a way
to spend less on booths, and more on airfare and hotel for as many families as
possible, to make this truly a community event.
Laurie and Patrick Schmidt,
CEO FFF Enterprises and sponsor of
88 children with Save One Life!
NHF
did a splendid job as always of organizing this mammoth event, and we thank
everyone involved, including sponsors and supporters, for mailing this
available. See you in Washington DC next year!
Rich Pezillo, Sonji Wilkes and
Jane Smith
Laurie Kelley with Laurel McDonnell
and Paul and Linda Clement
Janie Davis of Baxter with Emmanuel
of Ghana
Laurie and Chris Bombardier at
Save One Life table
Ellen White, RN, who first “discovered”
Ghana as a country in need of our help
Save One Life sponsors gather
for coffee
Factor Support Network displays
our books!
Bayer booth
With long time friend
Barbara Chang
Laurie Kelley with
Reid Coleman of NC
Laurie and Zoraida taking
a break
Yeah, I met Iron Man!
No trip to California
without visiting Jim Morrison in
Venice, where it all began for
the Doors!
Kudos to some young people with hemophilia with great ideas. Billy Conde Goldman and Matt Tache have recently announced “Blood Vibrations.” This is an ongoing music project created by people in the bleeding disorders community.
In their own words: “The goal of the project is to provide a forum for creativity, expression and sharing. Anyone with a bleeding disorder (boys and girls, men and women) is welcomed to participate. We encourage collaborations and group works. Proceeds from the project will be donated to the bleeding disorders community. Pay it forward.”
They write that this project is run and funded by the community, for the community. There is no affiliation with or funding from any private or public organization or company.
Aspiring musicians with bleeding disorders, Billy and Matt look forward to your involvement!
I did it! Yessir, I’ve gone from cycling a paltry 21 miles back in June (to which Barry Haarde prodded me to go further) to 105.5 miles yesterday. Not bad for a 55-year-old mom. I’d jump up and down but my left knee is stiff.
Brother Tim Morrow, wife Lee, and Laurie Kelley
On Saturday, I completed my first “century,” riding over 100 miles across Massachusetts. Many lessons learned from this event, and many parallels to our hemophilia community. In fact, I thought about our special community the whole way.
Our police escort, all 105.5 miles
The first annual “Ride to Remember” honored two fallen police officers killed in June 2012 in the line of duty: Kevin Ambrose of Springfield, and José Torres of Westfield. My brother Tim Morrow, a K-9 officer in Springfield, my hometown, was a close friend of Kevin Ambrose. The ride raises
awareness of the perils of our men and women in blue, and also raises money for law enforcement families. So I couldn’t help but think of my friend Barry Haarde, who rode across America, both last year and this year, to raise money for Save One Life, and to raise awareness of our “fallen,” those with hemophilia who died from HIV, contracted in the late 1970s and early 1980s from the blood-clotting products they used. Barry posted a photo each day of his ride on Facebook of someone who had passed away, including his own brother, to honor their memory. Like our police officers, these young men sacrificed their lives so that others, including all our children today, can have a safer life. Only, they did not go willingly into this service. No matter. Heroes all.
About 250 police officers, colleagues, and family members, like me, gathered in Springfield, Massachusetts Saturday morning, September 21, at 6 am in the mildly chilly air. We were blessed with excellent weather: no rain, slight overcast skies for a while. While we milled about, eager to get going, like race horses twitching in their gates, we were reminded by Sgt. Delaney, who organized the event, this was not a race. We would stay
together as much as possible—very tough, as the roads were often one lane each way as we weaved our way through the charming towns of New England. My brother Tim and I are naturally competitive and wanted to break away (well, he later did!) but we complied as much as possible. He had trained weekly with his colleagues; I trained solo. I’ve never ridden in a group before. I looked about and saw a lot of hardware—expensive bikes, wheel, spokes, cables—and software—arms, legs, heads. I was a bit wary of riding too close to anyone due to my own inexperience in group riding.
We gulped down bagels and bananas and at 7 am shoved off, herded by a police escort of about 20 motorcycles to keep us in formation, much like a pack of helmeted border collies. What would get us through 105 miles to Boston? Not just bagels and bananas.
“Take it easy, baby…” (Take It As It Comes)
Music helps pass the time and motivates, so I thought of my favorite songs from my favorite band: the Doors, of course. I was amused at how many songs and lines seemed appropriate for this journey.
I was shivering in the misty cool air, but thought of our guide Jacob on Mt. Kilimanjaro: “Pole, pole…(slow, slow)” A journey of a thousand miles starts with a few steps. We were cautioned to go at a slow pace, 13 to 15 miles per hour. Now, 15 miles per hour is my regular speed, but here it was like crawling. It was challenging to have riders blocking my view, causing me to swerve, weave, wiggle
my front wheel to keep balance. The first rest stop was only 18 miles away, which also seemed too soon. We’re used to going 20-25 without a break.
We took it easy and it was. My fingers and palms would go numb occasionally from the morning chill but that would ease once
the sun rose. Stop #1: Palmer. So far so good. We laughed, joked, ate more
bananas and guzzled water. Tim and I would later mention we have never eaten so much in one day in our lives, but you have to eat. It’s the one piece of advice I
took seriously: eat every 15-30 minutes, drink constantly. My sister-in-law Lee made delicious sandwiches that went down fast. On we go!
“Keep your eyes on the road, your hands upon the wheel…” (Roadhouse Blues)
The pack was still thick as we kicked off to the next segment of the ride. I had to constantly monitor who was riding up on my right or left, who was slowing down in front. Hills. So many hills. Steep and
long. I’m not a big person, and I don’t have the quadriceps that some of these cops have, but I noticed on the hills I blew past a lot of the guys who could outgun me on a straightaway. I attribute this to my cross training and core workouts with my trainer, Dan. When the quads burn out, and they will quickly on these hills, the body kicks in other parts, like back and abs. Mine were primed and
ready to take over on hills. This is why cross training is so important: you can get serious back problems from overtraining one part of the body and not all parts. Yahoo! Up the hills I went, happy.
Laurie coasting; Tim behind with raised fist
We passed through such quaint and picturesque New England towns: congregational church steeples piercing the blue skies above pumpkins plopped next to hay bales, antique shops. This is Sturbridge, our next stop and a historic town. I am baffled that I have never toured Sturbridge
before this!
Break On Through
Nothing can explain why, at the third stop in Charleton, I hit some sort of wall. I routinely ride 70 miles every Sunday; what was this all about? My mind felt fuzzy, quads buzzing with fatigue. I kind of stumbled in and grabbed some food. I resented having to stop so often, but you know, in the end, it was good the organizers made us stop. Otherwise we would burn ourselves out. I hardly saw Tim; he seemed to know everyone, and was busy chatting with his comrades. I actually am used to riding alone, and being alone, and didn’t really know anyone. I made a few friends, but preferred to use my rest time to stretch, eat and hydrate. Time to break through this wall. Maybe I should rename this section Pink Floyd’s The Wall?
Light My Fire
Well, things change! Back on the road, muscles got warmed up, and we hit a huge, long hill right off the bat. That was tough but again, I found the hills not a problem. I felt more motivated and competent and pushed it. Looking at the MapMyRide app, which charted my whole ride, I hit
18 mph at some points on this ride. Maybe that was down hill? Naw, because my speedometer said 35 mph going downhill. We were kicking it! This felt great
now. I learned something important about group riding, which I now prefer to
call team riding: you can draft. This means you ride behind another cyclist, who absorbs the brunt of the wind. You ride faster with less effort. I usually
can’t get above 18 mph, and even then only for a short time; now I was easily doing 19 mph with minimum effort. A lesson for teamwork, a lesson for our community. Stick together; lead; follow; be efficient; allow other leaders to take the helm when you get fatigued; listen to the leader, who spots danger first—Slow! Pothole!
Occasionally I would reach behind me and grab Snickers bars from the back pockets on my shirt, or Shot Blocks, or Gu gel, which give instant energy. Whatever I was doing it was working. Felt great!
Queen of the Highway
After 5.5 hours, we hit Grafton, next stop, about 60 miles in. We all checked in with each other: “How you feeling?” We lost a few riders and I watched the noble bikes sadly get hung on the inside walls of a truck, feeling badly for the riders. There were a couple of crashes too; riders who got too close to one another, or perhaps hit a pothole. My brother Tim even crashed. Flying downhill, probably at speeds over 35 mph, there was a sudden left hand turn at an intersection; he couldn’t slow down fast enough because the riders in front of him were going slower, so he thoughtfully went straight across the intersection, into a field and catapulted over his handlebars! Luckily, he hit soft ground and despite this spectacular landing, didn’t even have a scratch.
Only 40 something miles to go. This was so doable and easy! I plugged in my headphones now, and cranked up my music. We had a full police escort the whole way. The traffic in the oncoming lanes was stopped; all traffic in intersections was stopped. The world stopped for us!
Occasionally we had folks coming out of their homes to stand by the side of the road to cheer us on. Sweet. I felt unstoppable. Finishing was not going to be a problem!
I noticed that when hills appeared on the horizons, the riders, some 100 or more ahead of me, would swarm suddenly and become a huge pedaling mass. This is when I took to the incoming traffic lane,
as I could pedal pretty well up the hills, blowing by the big guys (and some petite women) who puffed and struggled. I started riding on the incoming traffic lane; I didn’t have to get too close to other riders, and had lots of room. Fun!
Stop 5: Ashland High School. Eat, eat, eat, hydrate. Stretch. Channel 22 news was interviewing riders, and providing live coverage. This was our last stop till Boston! About 30 miles to go.
“The future’s uncertain…” (Roadhouse Blues)
I was zooming along, when at mile 93—almost done—something kind of popped. In a second I had pain in my left knee, and couldn’t get it to work. I went from 19 mph to 9 mph in seconds. I could not
push that knee for anything. I think I was right on Rt 9, police motorcycles swarming around me, riders now zooming by me. I had been popping Tylenols the whole ride to help with general muscle soreness and specifically my neck, which has some arthritis in it. But nothing helped this. I hobbled along, dropping further and further behind in the pack.
I now lost focus on the surroundings and barely noticed the spectators cheering us on; we went through Wellesley, then Newton. I had my eyes down and was pushing my right leg hard to compensate. Don’t quit on me now! Thankfully, there was a final stop at Boston
College (that didn’t show on our ride map), where we all gathered in a huge, heaving, blue mob.
Do It
The famed Citgo sign; the end is near
I limped over to Tim, who was straddling his bike and gripping his handlebars, even though this was a 30-minute rest, like he was ready to bolt. He took me to the emergency team where they taped my knee. It
was the least we could do, and the most they could do. We waited on the grass
till 5 pm, when the organizers grouped us for the final push into Boston. There
was a ceremony waiting for us.
That was a painful ride. My beautiful adopted home city of Boston, the birthplace of America, all the familiar sites—the Citgo sign! Seeing that made us believe we can do it. But I couldn’t keep up
well. I felt I was the last rider (though Lee assures me I wasn’t). The streets were lined with thousands of people waving, cheering, with flags and banners for the fallen officers and for us. I was in a surreal zone mentally. Through Fenway, down a tunnel, popping up to Beacon Hill and our beautiful state house
with the gold dome forged by Paul Revere himself.
“This is the end, beautiful friend….”
Laurie rides in to the State House
It was done. 105.5 miles. As I sailed to the back of the State House, I saved my GPS map and stats, and then and there my second and last power pack died—perfect timing. Everyone had that “high” so familiar when you do something athletically great, and are so tired but so euphoric. My
brother Tim and I hugged; this is the first time we ever did anything like this
together, let alone apart (although he is quite a competitive athlete). My
sister-in-law Lee gets the credit for providing our gear when we needed it,
giving us fuel to keep us going, and being moral support!
A beautiful ceremony ensued, with full honor guard, and the heads of state of the law enforcement, including Attorney General
Martha Coakley. Though we were exhausted and hungry, we stood more or less silently for 90 minutes while the dignitaries went through speeches and read the names of every single officer killed in action in Springfield. Very sobering, very sad. Such heroes.
And while standing there, at this beautiful memorial, where the names of Officers Ambrose and Torres had already been added, it reminded me that we in hemophilia do not yet have our memorial. Our shrine, where names can be added, where people can come and pay their respects. Almost 10,000 innocent people with hemophilia and HIV died, becoming the sacrificial lambs for future better and safer treatment of hemophilia. Barry did an astounding ride last year and this year to honor them. Maybe it’s time to think about our own memorial to our own “Fallen But Not Forgotten.”
My brother Tim told me that the activity with the highest fatalities when you are a cop is approaching a vehicle, whether for a routine traffic stop or for suspicious behavior. You don’t know if or when the driver will pull a gun or if you will be struck by another car. That amazed me; how dangerous is their work! And for us, it used to be that injecting factor was as dangerous; you didn’t know which vial had HIV.
Now, we don’t have those worries. We have those fallen to thank. I hope someday we can, through our own memorial.
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I love discovering new people in our amazing hemophilia community, along with their talents and accomplishments. So I was delighted when Ray Perreault contacted me this year to introduce himself and share a new book he self-published. Ray has severe factor IX deficiency, and his new book, A Life Without IX, is a memoir. To Ray, this is simply a memoir of his life, but his life has been one of unmitigated pain and suffering. He tells this book (mostly) in the third-person, through a character named John. John’s life is Ray’s life. And it is remarkable.
