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Ape Over Hemophilia

During my Uganda trip
in April, I spent two days visiting the amazing Bwindi Impenetrable Forest,
which hosts the largest number of mountain gorillas in the world. These are
different than the ones you see at the zoo, which are Lowland gorillas.
Mountain gorillas are protected by law, and Uganda has done a spectacular job
at warding off poachers and protecting these highly intelligent,
family-oriented animals. With limited numbers of passes each year, I was lucky
to get one and view two different families. Below is my experience with one of
them.
When our guide told
us that gorillas share 98.8% of our DNA, I was shocked. I wondered, can
gorillas get hemophilia? I imagine they could. There are less than 1,000 of
them, living in four national parks in three countries. If 1 in 5,000-10,000
human males are born with hemophilia, perhaps there are no gorillas with
hemophilia… yet?
Thursday, April 28, 2016   Bwindi Impenetrable Forest
The rains are bathing
everything in moisture, which heightens the colors of the emerald forest and
yellow flowers. I hear birds repeating their songs, the constant background of
cicadas, the gentle rains. Every now and then one of the workers at the Gorilla
Forest Sanctuary—Nicholas, Eli, Judith— stops to see how I am, and how the day
was. It has been a marvelous day.
I awoke this morning
in my safari tent to the distinct three-note sound of a local bird, and the
whirring crickets. It’s peaceful here. The day was overcast and cool. After
breakfast, I skipped down the red stones of the Sanctuary, took a left on the
gravel road and within a few hundred feet reached the sloping driveway of the
Ugandan Wildlife Authority. I was the only one from our lodge; a few people
from other outfitters joined us, milling about on the lawn. We were to track
the “Rushegura family” of apes. This is an active group, on the move, comprised
of 19 family members. (Read more about this family here)
Our group was:
Stephanos and Leeza from South Africa; Ali, blonde, from the UK, who had asthma
and was probably my age or a bit younger; a young woman from Israel; Gladys
from the UK, who had a swishy ponytail and riding boots that looked wrong for
this kind of hike; a tall guy, my age, with glasses and was very happy; and a
German lady, tall and blonde.
Our guide was Gad, a
dark-skinned man with a chiseled face and thin mustache, small but wiry,
donning his army green wildlife outfit. As we set out on a long, flat dirt road
for 30 minutes, we had a chat. I asked him how long he had been a guide. He
replied 19 years, which seems a long time. “You must have been here pretty much
when the forest was opened, right?” I surmised. “Yes, back in 1997. I know a
lot of stories about the gorillas.” I asked if he then knew of Thor Hanson,
author of The Impenetrable Forest,
which I had just read.
“I know him!”
exclaimed Gad. “You know when he writes about that family whose bananas were
being eaten by the gorillas?” Yes! I told him, Chapter 16. The gorillas kept
raiding the village farmers’ field, particularly banana trees, as they love the
pulp (and not the bananas, contrary to pop culture). It was delightful to share
my enthusiasm about the book (which is well written) with someone. He shared
some stories about him being attacked by a gorilla, and still carrying the
puncture wounds. We fell in to chatting quite a bit, and I told him he should
write a memoir about his years: how the forest has grown as a tourist business;
who were some of his memorable guests?
We started hitting
dense forest after an hour. The gravely dirt road became a trail, and the real
hike began. I have never experienced any hiking like this. For the next two
hours, we hacked, stumbled, slipped, hauled, crawled and grabbed our way up the
mountainside.
“The gorillas keep
movin’,” one of the guides said, translating from the walkie-talkie
conversation he just had with the trackers. The four trackers—armed with
machetes for the undergrowth, and rifles for the wildlife— were far ahead of
us, scouting for the Rushegura family. This meant we would have to follow them
up the mountainside. It’s not called the Impenetrable Forest for nothing. The
trail very quickly turned into a 45° hike, through thick vegetation designed by
Nature to keep trespassers out. The light chitchat that bounced back and forth
between the trekkers petered out, as each person focused on where each foot
would land. It was an exercise in pedestrian strategy: if you put your foot on
a root as a launch pad, you might slip from its slimy covering, or it might
twist suddenly free and ensnare you. If you fall, you crash on more roots,
downed branches, wild vines. You put your foot up on the hill sideways (you
can’t often go straight on because the angle is too steep), ducking to avoid
creepers, vines, branches above you and at face-level; you reach up, dig your
walking stick with the carved gorilla head on it into the muddy ground above
you, pull and push, then steady yourself by grabbing a tree to hoist yourself.
Ouch! The trees are covered with moss and usually thorns from some of the
creepers. You quickly learn which trees and creepers to avoid. All the while
you ascend, more and more, higher and higher. Sweat pours off you, the trees
above drip on you, the mud splatters you. “Trampled Underground” played in my
head.
The trail, barely
qualifying for that title, is strewn with rotted vegetation: brown leaves,
moldy green ones, moss, and a cat’s cradle of vines that twist and ensnare. At
times you lose your balance, and start to waver downwards. When you
overcompensate and pitch forward your foot most likely is entrapped in the
vines and roots, holding you back, so you start to fall faster into the muddy
hillside. I lost my balance many times and once fell forward on the narrow and
treacherous hillside trail, but couldn’t right myself because anywhere I put my
hands I hit thorns. “Give me a hand up,” I pleaded to the South African couple
near me and they righted me. We then had to stamp on the vindictive vines until
my foot was free.
This was just one
small step for mankind. We still had two more hours of this just on the way up.
As each of us focused
on the botanical traps at our feet, we were surrounded by a primeval forest,
which only knew how to attack and defend. Creepers encircled trees, trying to
reach the top of the canopy to get sun, and strangling some trees over time.
Certain trees grew immensely big, while saplings sprung up like mushrooms
underneath. Every type of green thing known to humankind seemed to grow here,
with no inch spared for space, except for what we hacked.
The trackers thwacked
away with their two-foot long machetes, called pangas; branches lopped off trees in seconds. It seemed horrifying
to watch them mutilate trees this way; in the States we try not to harm our
trees. But you soon want a machete yourself to protect your arms and hands from
the insidious thorny trees and stinging nettles.
I saw a sort of slo-worm,
plump, blind and primitive, lying on the sodden trail. I pointed it out and our
guide told us it was a neera. To
amuse and shock them I picked it up; it worked. Suddenly my hands felt stinging
and I hastily put it back. I couldn’t imagine why it stung me until I saw the
safari ants crawling all over it. Later I saw a white shell, a smaller version
of the ones we find at the beach, curled over and over to form a funnel. It was
empty, and once belonged to a snail, probably a long forgotten victim of the
safari ants as well.
We stop only once
briefly, to catch our breaths as we are all heaving now, to sip water, and to
listen to the walkie-talkie chatter of the trackers ahead. Gorillas still on
the move. We wipe off sweat and start again. There is no small talk from anyone
now; we are in our own little endurance trek. Ali leaned against a tree,
seemingly defeated. We still have an hour to go. “Altitude sickness,” she says, but I’m not sure that’s it. We are up a high
mountain for sure, but not the 13,000 feet where this sickness would manifest.
It’s just a very strenuous hike and someone should be relatively fit to tackle
it.
The hired porters
kindly hold hands with the women in the group. My porter, Stifa, is a girl
younger than my youngest and I hired her as a community investment. Local
porters depend on tourist dollars to help their community.
Eventually we meet up
with the trackers on flat ground, and they look as exhausted as we do. Now we
are all gathered in a small clearing; there are big potholes of mud around us.
“Elephant,” one of the trackers said. Yes, elephants live up this wild
mountain! Now the trackers hack the vines and branches trying to keep us from
the gorillas, with a vengeance. They feel the gorillas are close by. Another 30
minutes of hacking, stepping, climbing.
At long last, after three solid hours, we hear the grunting
of the gorillas. Sure enough, around a tree caged with vines and smaller trees,
is a lone gorilla, stripping leaves off a branch with his teeth. We circled
about and watched as he munched. He was not concerned with us; behind us, the
Rushegura group. The silverback, “Mwirima,” lay on his back, one foot high in
the air. Two babies, just a few months old, their wobbly heads trying to focus
on us, lay in their mothers’ arms. One had a wildly wooly mane of hair. The
struggle to get here took the wind out of us, and the gorillas were somnolent
and lazy. But we stood about, snapping photos, quiet and respectful. None came
near us. They are habituated to humans, meaning that over time, the park
rangers have approached the group every day for an hour, for years, to get them
accustomed to humans. They are wild, no doubt, and dangerous. One gorilla could
easily kill you. The silverback weighs about 400 pounds. But they have been
trained to be in the presence of humans and tolerate them. To visit them you
must be quiet, move slowly, don’t go near them (though they might come near
you), do not set off any flash photography (it frightens them, reminding them
of lightning). And you can’t trek to see them even if you have a bad cold.
Because gorillas share 98.8% of our DNA, they are susceptible to catching our
germs. This made me wonder about hemophilia; can they develop it? No one seems
to know.
The gorillas had
feasted on the forest and were sleepy. Only the infants tried to break away and
were very curious about us. Each time they tried to climb down off their sleepy
mothers, the mothers gently took their arm and placed them back in their hold.
The mothers were incredibly tender towards the infants.
A crackling noise
revealed Ali made it after all! I quietly congratulated her and she immediately
credited Gad for getting her there. “I have been a guide for 19 years,”
reminded Gad. “I know people want to come see the gorillas. So I know how to
make them come up the hill.”
Eventually, thunder
burst overhead, warning us that a downpour was imminent. Gad politely suggested
it was time to move. We all donned rain gear, which would prove useless within
minutes. We bade the gorillas silently good-bye and began a slow march down the
mountain. It took us only 90 minutes, half the time, but was every bit as
treacherous. The rain burst like water sliced open from a huge camelback
bladder in the sky. It hammered us relentlessly. My hood—I had forgotten to
flip it up after I put on my trekking hat. When I did, of course it was filled
with cool rainwater, which slid down my back uncomfortably. Within 15 minutes
we were soaked to the skin, and soon no one cared anymore that we were wet. We
had to focus on our feet again. Gravity pulled us, but the roots and vines
weren’t done yet. Somehow I sped ahead of the group, keeping up at all times
with the trackers. Through the rain, I watched as the lead tracker followed a series
of yellow slashes on the trees, and neatly halved saplings; these were his
markings for finding our way out. Otherwise, how would he know? They don’t use
GPS or even compasses.
At one time he held
onto a sapling as he walked, pulling it toward him then releasing it suddenly.
It snapped back and whipped my left thumb painfully. Another time, I slipped in
the mud—no surprise as now a stream of mud, clogged with decomposing leaves,
became our trail, and the sudden thought that these beheaded saplings could
easily impale someone. I vowed not to fall again.
There was time to
enjoy the beauty of the rainforest. Everywhere you look, there was green: jade,
emerald, forest… all shades and types. Ants crawled on the ground, birds
occasionally chirped, but mostly the forest was deathly quiet, except for the
rush of rain. The stinging nettles still caught on my pants relentlessly, and you released them like a cat’s claws, pulling them back first
and then letting go. All of our clothing had minute holes in them from the nettles,
and the next day, I would awake with dozens of small bumps on my legs, itching
furiously where the nettles stung.
I descended rapidly,
well ahead of the group. We eventually came to the waterfall, about 40 minutes
from the Uganda Wildlife Authority building. The rain tapered off and we took
photos in front of the falls. The falls pounded the rocks like white foamed
fists. Nicholas had dashed ahead of us, and had stripped off his shirt to clean
the mud off him in the pool. He looked so happy, and there was a certain
simplicity about everything. Wet, muddy, sweaty, we were happy. Gad wanted to
know what we wanted to do: eat lunch in our raincoats, in the rain, next to the
waterfall, or continue? I wanted to continue on, not interested in eating.
Everyone stood about, not able to make a decision. I simply said, all in favor
of eating lunch here? The South African couple raised their hands hopefully.
All in favor of moving on? Everyone else raised their hands. “Sorry!” I offered
not apologetically. On we trekked!
The rest of the
excursion was easier, even in the now light rain. The trail evened out but
remained muddy, rocky, slippery, serpentine. It eventually spit us out on the
main road again, that led to the UWA. Gad exclaimed, “You’re fast! Do you want
to be a tracker?” Of course, I replied!
When we got back to
the main building, I thanked Stifa, gave her most of my lunch, a big tip and
pay, and then walked back to the Sanctuary, soaked, muddy and sweaty… and
thrilled.
See photos of the
gorillas here.
 

A Source of Support and Strength

Trying
to “go it alone” is a noble and brave course in the face of adversity, but not
always an ideal one, especially if you have a bleeding disorder. There is a
huge community filled with caring people, knowledgeable professionals and
valuable resources to help you. This week’s blog shares the experience of
Casey, who realizes how much he needs the support of the community, especially
when it comes to selecting a product that he and his physician believe is right
for him and his body’s individual needs.

Indications

ALPROLIX is an injectable
medicine that is used to help control and prevent bleeding in people with
hemophilia B. Hemophilia B is also called congenital Factor IX
deficiency.

Your healthcare provider may
give you ALPROLIX when you have surgery.

Selected Important Safety Information

Do not use ALPROLIX if you
are allergic to ALPROLIX or any of the other ingredients in ALPROLIX.


Please see ALPROLIX.com for the Indications and Important SafetyInformation , as well as the full Prescribing Information.

Growing
up, I usually didn’t talk about my hemophilia B. I was the first person in my
family to have hemophilia; diagnosed in early childhood. At school, when I would
have bleeds, I remember thinking that none of the other kids did. Without a
mentor, I kept my emotions to myself, no matter how vulnerable I felt.

My
dad gave me my treatments 99% of the time. Always getting the vein on the first
stick. However, my mom was a nervous wreck. One time she had to give me a
treatment, she tried again and again with no luck. She was in tears. In that
moment, she became my motivation to learn how to self-treat. So at the age of
10, I began managing my own hemophilia infusions. It was a liberating feeling.
I wasn’t so vulnerable after all.

It
was one of my nurses who told me about ALPROLIX. After discussing the risks and
benefits with my doctor, he explained that the recommended starting prophy
regimens are either 50 IU/kg once weekly, or 100 IU/kg once every 10 days. And
that the dosing regimen can be adjusted based on individual response. No two
people with hemophilia are alike, so it made sense to me that medication could
work differently in everyone. I started on a 10-day schedule, but when I had my
labs taken, my doctor told me that I could extend my infusions to once every
two weeks. That really fits my busy schedule. Thinking back on my childhood, I
never believed infusing every 14 days would be possible.

I
didn’t start taking ALPROLIX just for myself. I was also thinking of my two
nephews who also have hemophilia. After seeing my experience and talking with
their doctors, they started on ALPROLIX, too. I try to help with my nephews
when I can. I’ve even occasionally been their caregiver and given them a couple
treatments. I make sure they know they have me to look up to if they need
advice.

Watching
my nephews grow up brings back so many memories of my own childhood. We have a
special connection because I understand some of the challenges they face. They
have an advantage I didn’t have growing up: a close family member who knows
what they’re going through. I can help my nephews in a way no one could help
me. My goal is to help them find confidence in themselves and encourage them to
have fun being kids.

I
hope that by sharing my experiences, I might encourage others to figure out
what they have learned from their own experiences and take control over their
choices.

Thank
you.
To hear from other people
living with hemophilia B, check out thesevideos.
Talk to your healthcare team
about whether ALPROLIX may be right for you.
This blog was sponsored by Biogen, for educational
purposes.
FIX-US-0807   
06/2016

Together we can FACTOR IT FORWARD™

The following is a paid public service announcement.
LA Kelley Communications always advises you
to be a savvy consumer when contacting any company; do not reveal personal
information (PI) or protected health information (PHI) carelessly or against
your will.
Every day, I’m reminded of how much I have to be thankful for. Like
the life-changing support I’ve had the privilege to both give and receive within
the hemophilia community.
As a parent personally touched by a
bleeding disorder, I’ve witnessed first-hand the power a strong support system
can provide.
This truly is a community that cares, and I’m proud to be a part
of it.
That’s why today, I’m excited to spread the word about a program that
embraces the spirit of this community—FACTOR IT FORWARD.
With FACTOR IT FORWARD, the people behind IXINITY® [coagulation
factor IX (recombinant)] have partnered with AmeriCares, a non-profit global
health organization, to donate IXINITY to people with hemophilia B who can’t
afford to be without it right here at home.
And it doesn’t end there. Visit www.factoritforward.com or read on to find out how you can help.
FACTOR IT FORWARD and help lead
the way
As part of their commitment to the hemophilia B community, the
makers of IXINITY will donate 10% of IXINITY used to nines who need it most
here in the U.S. That means:
For
every 10 units of IXINITY used, 1 unit is donated to those in need in the United
States.
So when you use IXINITY, you help
give back to the hemophilia B community.
You can try IXINITY for free to see how it works for you. With
your IXINITY FREE Trial, you’ll receive 1 month of treatment up to 20,000 IU. Learn more about the FREE Trial, download the FREE Trial Form, and talk to your doctor. 
Plus, there are even more ways to join in and help FACTOR IT
FORWARD in addition to using IXINITY.  
Get involved today, right now, when you take the giving quiz at www.FactorItForward.com. You’ll earn points that will contribute to
factor donations just by answering a few quick questions.
Or chat with an IXINITY rep
at a local chapter event and snap a
selfie with your giving pledge. Share the pic on your favorite social media
channel to get points just for posting.  
More points equal more factor donated, making it even easier for
everyone to do their part!
And since there’s no limit to the number of IUs donated, every IU
of IXINITY used and every point earned count toward that end goal: to leave no
nine behind.
Visit www.FactorItForward.com
 today
to learn more about the program and how you can participate, and talk to your
doctor to find out how you can try IXINITY for free.   
IXINITY
INDICATIONS AND IMPORTANT SAFETY INFORMATION
What is IXINITY®?
IXINITY [coagulation factor IX (recombinant)] is a medicine used
to replace clotting factor (factor IX) that is missing in adults and children
at least 12 years of age with hemophilia B. Hemophilia B is also called
congenital factor IX deficiency or Christmas disease. Hemophilia B is an
inherited bleeding disorder that prevents clotting. Your healthcare provider
may give you IXINITY to control and prevent bleeding episodes or when you have
surgery.
IXINITY is not indicated for induction of immune tolerance in
patients with hemophilia B.
IMPORTANT SAFETY INFORMATION for
IXINITY®
·    
You should not use IXINITY if you are
allergic to hamsters or any ingredients in IXINITY.
·    
You should tell your healthcare provider if
you have or have had medical problems, take any medicines, including prescription
and non-prescription medicines, such as over-the-counter medicines,
supplements, or herbal remedies, have any allergies, including allergies to
hamsters, are nursing, are pregnant or planning to become pregnant, or have
been told that you have inhibitors to factor IX.
·    
You can experience an allergic reaction to
IXINITY. Contact your healthcare provider or get emergency treatment right away
if you develop a rash or hives, itching, tightness of the throat, chest pain,
or tightness, difficulty breathing, lightheadedness, dizziness, nausea, or
fainting.
·    
Your body may form inhibitors to IXINITY. An
inhibitor is part of the body’s defense system. If you develop inhibitors, it
may prevent IXINITY from working properly. Consult with your healthcare provider
to make sure you are carefully monitored with blood tests for development of
inhibitors to IXINITY.
·    
If you have risk factors for developing blood
clots, the use of IXINITY may increase the risk of abnormal blood clots.
·    
Call your healthcare provider right away
about any side effects that bother you or do not go away, or if your bleeding
does not stop after taking IXINITY.
·    
The most common side effect that was reported with IXINITY during
clinical trials was headache.
·     These are
not all the side effects possible with IXINITY. You can ask your healthcare
provider for information that is written for healthcare professionals.
For more information about IXINITY,
please see accompanying full
 Prescribing Information, including
Important Patient Information.
You are encouraged to report side
effects of prescription drugs to the Food and Drug Administration. Visit 
www.fda.gov/medwatch, or call
1-800-FDA-1088.
 
Manufactured by Cangene Corporation, a subsidiary of Emergent
BioSolutions Inc. and distributed by
Cangene bioPharma, Inc., a subsidiary of Emergent BioSolutions Inc.
IXINITY® [coagulation factor IX (recombinant)], FACTOR IT FORWARD, and
any and all Emergent BioSolutions Inc. brand, product, service
and feature names, logos, slogans are trademarks or registered
trademarks of Emergent BioSolutions Inc. or its subsidiaries in the United
States or other countries. All rights reserved.
©2016 Emergent BioSolutions Inc. CM-FIX-0048

The Most Powerful Man in America–with Hemophilia No Less

The man who built Fox News into a political media giant has been called the most powerful man in America by President Obama… and also happens to have hemophilia! Roger Ailes. 
During this raucous political season, it’s worth looking at the man who helped create the news outlets for conservative voters in America.

The following is a book review by Richard Atwood, our columnist for PEN: Gabriel Sherman’s 2014 biography The Loudest Voice in the
Room. How the Brilliant, Bombastic Roger Ailes Built Fox News — and Divided the
Country
is fascinating for its look at this interesting man, who once sat on the National Hemophilia Foundation board of directors.
Roger Eugene Ailes was born on May 15, 1940 in
Warren, Ohio. When two, he bit his tongue and bled, leading to a diagnosis of
hemophilia. Ailes attended Ohio University where he enrolled in Air Force ROTC
for two years, but his eyesight and other physical problems were an issue. He
even tried to enlist. Not knowing how long he would live, Ailes did not openly
discuss his hemophilia with most of his classmates. His older brother, a
physician, linked Ailes’ temper to his medical condition. His parents divorced
while he was in college. Ailes married Marjorie White in 1960. Beginning in
1961, he worked as a producer for The Mike Douglas Show at a television
station in Cleveland. Ailes kept his hemophilia a secret. When the show moved
to Philadelphia in 1966, Ailes was its executive producer. He treated politics
as entertainment. After an interview with Richard Nixon in 1968, Ailes worked
as a media advisor, or image consultant, for Nixon’s presidential campaign
before being fired by the RNC in 1970. While separated from his wife in 1972,
Ailes was agent for, and romantically involved with, the actress Kelly Garrett.
As a Broadway producer in the 1970s, Ailes had both successes, such as Hot l
Baltimore
, and several flops. Ailes took risks with sky diving, tearing his
ankle ligaments, while also becoming paranoid about his physical safety. He had
brooding moods, increased weight and pent up anger. He demonstrated his
conservative political beliefs when he was a consultant and news director of
TVN (Television News Inc) for two years before it shut down in 1975. Ailes
broke up with Garrett in 1977, then became romantically involved with Norma
Ferrer who had two children. He divorced White that year and married Ferrer in
1981. 
Ailes was most successful as a political consultant from 1980 to 1986.
After a fist fight, he had swollen hands. He was known to make sexual
approaches to female employees when he was executive producer for the Tomorrow
Show
with Tom Snyder. His father died of Alzheimer’s in 1983, and his
weight ballooned again. Ailes published his book of self-help wisdom, You
Are the Message
, in 1988. He became an executive producer for Rush Limbaugh
in both radio and television. He worked for 28 months at CNBC, beginning in
1993, and learned how to turn news into entertainment. He moved to the America’s
Talking
cable channel in 1994. He divorced Ferrer in 1995, this time a more
expensive change. Ailes now walked with a limp. He moved to MSNBC in 1995, and
left a year later. Roger Murdoch hired Ailes in 1996 to be chairman and CEO of
Fox News. Ailes’ book became the cable channel’s sacred text. His fear for his
personal safety increased. He married Beth Tilson, a divorced producer whom he
met at CNBC. Ailes’ son, Zachary Joseph Jackson Ailes, was born on January 1,
2000. Ailes hired Bill O’Reilly, his brightest star. Fox News passed CNN for
good in ratings in 2002 after the Monica Lewinsky affair in 1998 and the 2000
elections.  Ailes hired Glenn Beck
from CNN in 2008 and supported the Tea Party in 2009. Many conservative
politicians became employees of Fox News. 
After building a mansion in Garrison,
New York, 46 miles north of NYC, Ailes bought the Putnam County News &
Recorder
in 1988, installed his wife as editor, and changed the local paper
to reflect the conservative views of Fox News. Ailes continued to demonstrate
his fear for personal safety and his paranoia, and he shared his conspiratorial
worldview with his wife. Ailes also was in charge of Fox Business Network that
was launched in 2007. 
Ailes remembered his father’s lessons and had a ‘Rosebud’ story.
“The cruelest lesson Roger would speak of occurred in the bedroom Roger shared
with his brother. Roger was standing on the top bunk. His father opened his
arms wide and smiled.
   “Jump Roger, jump,”
he said.
   Roger leapt
off the bed into the air toward his arms. But Robert took a step back. His son
fell flat on the floor. As he looked up, Robert leaned down and picked him up.
“Don’t ever trust anybody,” he said.” (p. 7).
This biography covers Ailes’ childhood rather
quickly, but his hemophilia is stressed more often during his younger years.
Yet a specific diagnosis and family history of hemophilia is never provided.
The book is more an analysis of Fox News than a life story of Ailes for his
older adult years. There are 6 pages for A Note on Sources, 98 pages of Notes,
13 pages for Selected Bibliography, and 20 pages for the Index. Hemophilia is
cited 10 times in the text, and is listed twice in the Index, under ‘Ailes,
Roger’ and by itself. Oddly, there are no photographs. The author interviewed
614 individuals in addition to referencing the written sources. Ailes was not
subjected to a psychological analysis, yet he was frequently described as
impulsive, paranoid, aggressive, and a ruthless competitor, all influenced by
his ego and temper. He was also quite successful. The author, who never
received permission to interview Ailes, is a journalist and editor living in
New York City with his wife.

It’s worth Googling Ailes to read about his work, his attitudes and thoughts about the political candidates; a fascinating man with hemophilia!

Another book read by Richard, but not as good, is Zev Chafets’s 2013 book Roger Ailes: Off Camera.
New York, NY: Sentinel. 258 pages.

Standing on the Shoulders of Giants

Dr. Olaf Walter reviews Octapharma’s history
I
was privileged this week to be invited to a special scientific symposium in
Heidelberg, Germany, sponsored by Octapharma. Octapharma is the largest
privately owned plasma manufacturer in the world. It recently launched  the first and only fourth generation recombinant factor VIII (FVIII)  produced in a human cell line without any chemical modification or protein fusion, NUWIQ®, now available in the
US. This unique product is important as it is hoped that a recombinant product
that originates from a human cell line might have low immunogenicity—in other
words, it might not trigger an inhibitor, which is the greatest complication of
hemophilia.
Octapharma
was founded in 1983 by Wolfgang Marguerre; the name comes from the Greek
for the word “eight.” Octapharma manufactured the world’s first plasma-derived FVIII that employed a viral inactivation step using solvent-detergent. I cannot overestimate how important this process is. My own son used product
initially that had no SD or pasteurization and miraculously escaped HIV and
hepatitis C infection.
 
Octapharma
now serves patients in 105 countries with 6,200 employees in 32
countries, of which half are in the US, the largest market for hemophilia
products.
Laurie Kelley with Marcia Boyle,
executive director of the IDF
The
Saturday symposium featured speakers from the US, Denmark, Canada, and Germany,
including our own Dr. Craig Kessler from Georgetown University. While all the speakers
were excellent, I learned a tremendous amount from Dr. Roger Kobayashi, of UCLA
School of Medicine, who is an immunologist. Patients with immune deficiency
share so many of the same concerns as those with hemophilia. Our need for
constant products, safe products, easy to administer and affordable. Dr.
Kobayashi reminded us that physicians like him, and companies like Octapharma, “stand on the shoulders of giants”–those researchers who have come before us. He told us that the very first Nobel Prize was awarded to a doctor who
discovered gamma globulins, proteins that can be used commercially to treat
infections in those with malfunctioning immune systems. He described the pain patients
felt years ago when intramuscular injection were given; the poor children! They
could not walk for days. Finally, intravenous infusions were manufactured and
successfully treated patients with immune deficiency. While he spoke, I had the honor
to be sitting next to Marcia Boyle, the executive director of the Immune Deficiency
Foundation, an exceptional leader who for 32 years has provided education,
advocacy and help to those with immune deficiency in the US. Indeed, Dr.
Kobayashi mentioned her three times in his speech!
We
learned about Alzheimer’s (discovered here in Germany), and Octapharma’s research into treating this
insidious disease, the fifth leading cause of the death in the US. There is
currently no treatment and after hearing Dr. Shawn Kile’s excellent presentation,
I pray that this company discovers a treatment soon. I recently witnessed the devastating
effects of this disease in a dear elderly friend. It strips a person of their
very identify and almost of their humanity. How long will we wait for a
treatment to slow it or stop its progression? Companies like Octapharma work
daily to answer this.
But
I was most interested in Dr. Craig Kessler’s speech on personalized
prophylaxis. Dr. Kessler described how half-lives differ dramatically among
patients and only a pharmacokinetic (PK) test can determine each person’s half-life. That’s the number one place to start with a proper treatment regimen. In
the “old” days, like when we were raising a child with hemophilia, we dosed
by weight and bleed severity only. Now we realize this is not enough. Each person
will have a different half-life. He displayed one slide that showed 66 patients
with half-lives varying up almost 5 hours. Once you know your half-life, you
can find the proper prophylaxis treatment. This might include looking through the vast
array of factor products. Dr. Kessler reminded us that patients want 1) reduced
number of infusions, 2) reduced inhibitor development and 3) to adopt prophy to
their own lifestyle.
Dr.
Kessler reported that NUWIQ® is Octapharma’s new fourth generation recombinant FVIII from a human cell line—the
first such product. Because it is entirely from humans, and not animal cell
lines, this could potentially reduce the rate of inhibitor development and avoid possible
allergic reactions, and allow personalized prophylaxis with fewer
infusions.  He described several
clinical studies with NUWIQ®, including one with 135 previously treated patients,
none of whom developed an inhibitor. We also know that inhibitors can develop
in about 30% of previously untreated patients with factor VIII deficiency, usually
within 20 exposure days. Octapharma designed NUWIQ® to be less immunogenic by using a human cell line. I will want to follow clinical studies closely on the
product to see what else they uncover regarding this.
Laurie Kelley with Octapharma friends

I
was also interested to hear from Dr. Olaf Walter, senior vice president, and MC for the day, that Octapharma is conducting research for a  subcutaneous (under the skin) delivery of factor. I know of two other companies
that are also working on this, and it will again be interesting to watch
developments. As we write in our May issue of PEN, this is the most exciting
time in hemophilia!

The symposium
closed with a delicious lunch and then a black tie gala that evening at the
Heidelberg Castle. It was not only the 33rd anniversary of
Octapharma, but also the 75th birthday of founder and chairman Wolfgang
Marguerre. I have a special indebtedness to Wolfgang, as he is Save One Life’s
largest supporter and also sponsor the largest number of children, 120.

Laurie Kelley with Octapharma Founder and Chairman
and Save One Life’s leading donor Wolfgang Marguerre



The evening was
dazzling, with over 40,000 flowers, 500 guests including Octapharma employees from around the world, 150 wait staff, at least two high quality bands, delicious food and drink. This was a wonderful way to celebrate
the accomplishments of this company and its indefatigable and brilliant
founder. I met up with my hemophilia colleagues, including three of our
top sponsors for Save One Life: Wolfgang, and also Patrick Schmidt, CEO of FFF
Enterprises, Neil Herson, president of ASD Healthcare, and Paolo Marcucci of Kedrion S.p.A.

Together, all three sponsor
about 280 children with hemophilia in poverty annually. Many of the other
attendees also sponsor children, including hemo-mom and Octapharma employee
Sherri Rojhani, who just sponsored a child in honor of Wolfgang Marguerre’s
birthday.

The evening
ended with spectacular fireworks, accompanied by songs representing many
countries, but the final song was a favorite of mine, the Irish pub-song The
Wild Rover.

When I thanked
Wolfgang for such a lovely evening, I told him he needed to manufacture a new
product—his energy! I want an infusion of whatever he has to keep me going strong
at 75 to do the humanitarian work I love to do. If only!
Thanks to Octapharma for a fascinating
symposium, such amazing festivities, and for all its support for Save One Life.

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