December 2014

A Christmas Legacy

My brother Tim sent me this story of a fellow Massachusetts family, which may have otherwise gone unnoticed in our community. I’ve never heard of the Pastorellos; any of our readers heard of them? It’s a beautiful story just made for Christmas.

Son Rekindles Family’s Holiday Tradition

SOUTH HADLEY, Mass. (WGGB) — John Pastorello made it his mission to rekindle his family’s beloved holiday tradition.
This year, Pastorello brought back his family’s Christmas light display — something they started together nearly 20 years ago.
“I always loved the tradition of always putting all the lights together with my family,” explains Pastorello. “And, I just felt like something I needed to do.”
More than 1,000 Christmas lights and 100 lawn ornaments now adorn Pastorello’s front-and-back yard. He told ABC40 why this event is so significant.
“My dad’s brother was a hemophiliac who got a bad blood transfusion,” explains Pastorello. “So, my dad put up a couple of Christmas lights [in 1989] and was thinking about his brother a lot because he spend a lot of time in hospitals. And as a child in hospitals, he really loved Christmas.”
The holiday season was his uncle’s favorite time of year, which made this Christmas an emotional one.
“It was nice to see them out again — it’s still a lot of work,” says Richard Pastorello, John’s father. “But to see them out, the lights on and everything — it brings back the memories and everything that we did. “We wish that — our kids were pretty young — could see their Uncle Roy because it kinda meant a lot.”
Many of the lawn ornaments are original. Some, came from Richard Pastorello’s home in Belmont — where he grew up.
The planning process started in October and then the Pastorello family worked hard until the last week of November to set up the display.
Not only did Pastorello take on the tradition, but also the hefty $500-a-month electricity bill.
“I actually delivered newspapers for almost a year — and I was trying to collect money, in order to update my electrical and all that,” says Pastorello.

The family will celebrate the end of the holiday season on New Year’s Eve, when the light display will go dim — until next Christmas.

Great Book I Just Read
All the Light We Cannot See [Kindle]
Anthony Doerr
This wonderful fictional story, set against the backdrop of World War II France and Germany, is about Marie-Laure, blind since age six, daughter of a locksmith for the Museum of Natural History in Paris. Parallel to her life story is that of Werner, a bright and curious orphan German boy with a knack for electronics, who happens to listen to radio science programs, told by coincidentally Marie’s great-uncle. The children grow, and as WWII engulfs their countries, each tries to survive–Marie by moving in with her great-uncle, and Werner in the German army– while all the time are on a path of destiny to meet one another. A subplot involves a rare and precious diamond, secreted away by Marie’s father. The conclusion brings all the intricate subplots together magnificently. A national book award finalist, the writing is superb and character development sublime. Sure to be a Hollywood movie someday. Four/five stars.

Hemophilia Leadership: Jesus’s Example

Christmas is celebrated this week, and has me thinking of leadership. I have worked with many emerging leaders in the international hemophilia community, who are volunteering to help their country’s people with hemophilia. It’s a daunting task: these volunteers are typically mothers or fathers who struggle daily to make ends meet, and raise with a child with hemophilia without factor. To take on the mantle of leadership, and the challenge of forming a nonprofit without experience, money, resources or even much time, is sheer bravery. We here in the US cannot even begin to imagine their daily lives. Every step is hardship.
Caravaggio’s Adoration of the Child, in the Uffizi Gallery, Florence, Italy

I sometimes think of the example of Jesus when I approach new leaders, or try to inspire new leaders. Jesus was born into poverty too. He had no earthly resources. He wasn’t trained to be a leader–he was a carpenter. He saw the suffering around him; indeed, his very birth triggered the “Slaughter of the Innocents.” Imagine knowing that fact as you are growing up. He was gifted with the ability to heal. And he had to sacrifice much: leave his mother and father, travel far from home, find disciples, spread his message without any use of newspapers, radio or cell phone. No slogans, no banners, no World Jesus Day. It all came down to him.

Our leaders in Africa, South America and Asia also face much sacrifice and struggle; they need to find disciples; they need to educate the masses. On top of it they have a child with a painful and life-threatening disorder. But these Hemophilia Leaders follow the same style of leadership that Jesus exemplified. They are–or should be–servant leaders.

A servant leader puts the needs of his people over his own desire for control, power, acquisition, or ego. He is attuned to the needs of his people, and seeks to meet them. In hemophilia, this means helping find those who suffer, putting them in touch with a treatment center, educating them about their disorder, and above all, getting them factor.

So in a way, Hemophilia Leaders can “heal”: they learn who to reach out to (doctors, WFH, Project SHARE) for help, for factor. I’ve witnessed so many amazing leaders, some young, some beyond retirement, dedicate every minute of their free time to helping the suffering in their countries. And this while they themselves often need help. Imagine a Hemophilia Leader contacting us at Project SHARE, while they themselves are in pain from a bleed, requesting help for one of their patients. That’s servant leadership.

They are stoic, strong, driven with an inner passion, rise above their own pain and suffering, and above all things, have faith. Faith and trust that someone will help them, that they can change the situation in their countries to make it better for a new generation. Faith in themselves, because they often believe in a higher power helping them, whether a deity or an organization, or both! They have a vision that compels them. They are driven and dedicated. Meditating on this at Christmas, I am in awe of these Hemophilia Leaders–patients, parents, doctors–and I cannot wait to serve them in 2015. What a gift they are to us at Christmas time and throughout the year.

Book I Just Read
Jesus, CEO
Laurie Beth Jones 1995

What skills, attributes and style did Jesus possess that made him a success and a “fisher of men” that leaders today can emulate? That’s the goal of this book, broken down into easy-to-read chapters that pose personal questions to consider at the end of each. The book may be good for leadership novices, but lacks a sharp, in-depth comparison of Jesus’s life and skills compared to those of a modern-day business person (such as provided by Margaret Morrell in Shackleton’s Way). This is a superficial, often trite comparison of Jesus’s skills, mindset and style to modern day leaders. Some are stunningly obvious: He planned. He formed a team. He had a vision. He expressed himself. He forgave them. Simplistic, and questionable at times. (“He came from left field.” “God surprised people.” What? I thought his coming was prophesied, like for centuries?)
There are much better books on Jesus and leadership. Try John Maxwell’s books on leadership and skip this outdated one. It still sells for $10 on Amazon! One/five stars.

Hospital Advocacy 101–A Refresher Couse

Character cannot be developed in ease and quiet. Only through experience of trial and suffering can the soul be strengthened, ambition inspired, and success achieved. —Helen Keller
Helen Keller’s quotation could be a the tagline for Advocacy 101, a class I feel I attended this weekend. I received a text Friday morning from my brother Tim, telling me our 82-year-old mother had been admitted to the hospital in the early morning hours. Apparently she had fallen in the night and couldn’t get up–yes, just like the commercial says.
When I finally arrived in Springfield, I went straight to the hospital observation ward. My mother was very ill, that was clear. The doctors were running tests on her to rule out arterial blockage, stroke or neurological issues. Her main symptom was extreme vertigo, as she had no balance at all, which left her exhausted and nauseated, and shaking. She had not been able to eat all day. Fortunately, when I left her three hours later for the evening, she was perking up and looking and feeling better. The doctors were very helpful, and explained everything they could, in the best way they could. The nursing staff was excellent and we felt she was getting top-notch care.
As I left, I realized I had not been in a hospital, in a medical situation, in a very long time, probably more than four years ago, when my son was last admitted with me around (appendicitis). My advocacy skills were a bit rusty.
Well, the great care she received Friday was from the day staff. The next day I returned with my sister-in-law Lee, and my mother looked like she was suffering, having had an ordeal through the night, including a 3 am MRI. She was curled up on one side, unable to lift her head, nauseated to the extreme. She had not had anything to eat or drink in forever. She said the noise from the admin desk (just a few feet away) from the “girls” at the desk was loud, obnoxious, keeping her tense and unable to rest. She rang and rang the call button, but no one came to assist her. She listed all her frustrations.
Lee poked her head out of the curtain that barely screened her from the rest of the ward and ordered, “She needs a room, now. She is very uncomfortable.” And this lead to, when are we getting the room? Do you know, within 15 minutes they had a room for her. They gave her a pill for nausea, changed her, wheeled her upstairs and brought her delicious broth to drink. My mom felt better within 30 minutes. She sat up and ate the soup.
Lee also discovered that the nurse did not have it noted on her charts that my mother is diabetic: staff should really not be giving her muffins with high glucose content! Noted. And her diet was changed.
I was proud of my sister-in-law for being there, and for so immediately requesting an improvement. That’s true advocacy in action. It was a reminder of everything I learned 27 years ago when my son with hemophilia was born: how to politely make my medical rights known, to make my needs known, to speak up for a baby who cannot speak for himself. And not to back down when you know you are right. These skills weakened over time when not used; I was slower to advocate for my mother than my sister-in-law was!
I just learned that she has been discharged; so we will arrange to have her brought home, just in time for Christmas. And from where did I first learn medical advocacy? It wasn’t really hemophilia; it was from mom of course. As one of seven children, I watched her handle hospitals and doctors many times with my active brothers. And I saw her get action when she spoke up. The nurse mentioned to me that my mother is refusing a script for a walker, which we all wanted her to have. That’s advocacy and pride. That’s my mom, my first advocacy teacher.
Interesting Book I Just Read
Metallica This Monster Lives: The Inside Story of Some Kind of Monster [Kindle]
Joe Berlinger and Greg MilnerA story about the making of a documentary of one of the most successful metal bands, which was going through a crisis that threatened to destroy itself. In 2001 Metallica was reeling from losing its bass player, yet had to produce a new album, its first in years. The film team that produced two excellent documentaries, Brother’s Keeper and Paradise Lost (and the widely panned Blair Witch Project 2), now documented the band composing, fighting and enduring therapy with a performance coach. 1600 hours of footage turned into a feature documentary, and entered the Sundance Festival. But the book–an oddity. More about the filmaker and his career. Berlinger is too close to his subjects to be objective, and at times, revels giddily in his chumminess with Lars, the drummer. But I enjoyed it, even if it is an unevenly told story; Berlinger comes across as a very nice person, sincerely trying to do his best as the game plan keeps changing radically. Lars does not come across well. Fans may cringe at hearing these thrash metal superstars bare their feelings towards each other. A great book for psychotherapists or filmakers in training. The Kindle version is riff with missing periods at the end of sentences. Two/five stars.

What a Pain

I was just catching up on my weekly news magazines today and caught this stat about prescription addiction: 46 people die each day from overdoses of prescription opioids, like Percocet, Vicodin and Oxycontin. Up to 90% of those with chronic pain are prescribed an opioid, and up to 25% become addicted. Some people in our bleeding disorder community suffer from chronic pain, especially those with inhibitors and chronic joint damage. I thought it would be good to run this excellent article published in my latest edition of PEN by Paul Clement, our science writer, in our column Inhibitor Insights, sponsored by Novo Nordisk.

How to Use Pain Meds Safely             by Paul Clement
Pain is no stranger to people with hemophilia: joint bleeds are painful, and repeated
bleeds into a joint cause damage to cartilage, resulting in a painful form of
arthritis called hemophilic arthropathy. A trip to your medicine cabinet
usually yields some form of painkiller (analgesic) to get you through the worst
of the pain. But when you have inhibitors, you may need something stronger for prolonged
bleeds or chronic arthropathy.
Most people don’t think twice about taking analgesics. But these meds can potentially kill:
every year, many people overdose accidentally. People with inhibitors frequently
need painkillers and may be at higher risk of serious side effects. When was
the last time you thoroughly read the package insert on your pain med? What do
you need to know to be safe when taking pain meds?
Two Types of Pain
Pain is either acute or chronic. Acute pain lasts hours or days. Chronic pain lasts six months or longer. Acute pain is considered necessary, even beneficial—alerting our bodies to danger or injury, and prompting us to protect ourselves or get treatment. But chronic pain is a disease state in itself, and is often destructive and debilitating, harming our general well-being.
If you have hemophilia, your acute pain is usually caused by bleeding that leads to swelling in joints and muscles. Chronic pain, by contrast, is usually caused by arthritis in joints, a result of repeated bleeds that have damaged the joint’s cartilage—a common problem for many people with inhibitors. The two types of pain require different treatment approaches and different pain meds.
Proper treatment of pain depends on the type you have. Pain meds are divided into two broad groups: opioid and non-opioid. Mild acute pain—like a headache—is often treated by non-opioid over-the-counter (OTC) pain meds, available without a doctor’s prescription. There are two basic
types of OTC pain relievers: (1) acetaminophen and (2) a broad class of drugs called non-steroidal anti-inflammatory drugs (NSAIDs), such as naproxen and ibuprofen.1
Opioids, such as morphine and oxycodone, are available only with a prescription. These meds are used to treat moderate-to-severe acute pain and chronic pain.
When used as directed, pain meds are usually safe and effective. But when misused, all pain meds can be dangerous and even deadly. Most drug overdoses are not intentional, but are caused by ignorance. People don’t read product inserts carefully, or they combine multiple drugs, not realizing that this could result in an overdose or make their normally safe medication toxic. Also, many people believe that because OTC drugs are available without a prescription, they must be safe and can be taken without harm. Not true! In fact, OTC pain meds are powerful drugs that can be deadly, and they should be used with caution.
What do you need to know about pain meds to keep yourself or your child safe? In the first of this two-part series, we’ll look at the risks of acetaminophen, the most commonly recommended pain med for people with hemophilia.
For hemophilia, acetaminophen (Tylenol®, Excedrin®, Anacin®) is the most often recommended drug for mild to mild–moderate
pain because it’s generally effective, and it doesn’t affect the blood’s clotting ability, like almost all NSAIDs do. Acetaminophen reduces pain and
fever and won’t cause gastrointestinal bleeding, as NSAIDs can. But acetaminophen has no anti-inflammatory properties, as NSAIDs do, to help reduce swelling in joints and muscles.
When taken as directed, acetaminophen is generally safe. Over 50 million Americans use acetaminophen weekly, but it can cause liver damage. Don’t take a higher dose than is recommended. Don’t take acetaminophen for more than ten days or while drinking alcohol.
Acetaminophen overdose is the leading cause of calls to US poison control centers—more than 100,000 instances annually. Every year, acetaminophen overdose is responsible for more than 56,000 emergency room visits; 26,000 hospitalizations; and an estimated 458 deaths due to acute liver failure.2 In fact, acetaminophen is the number one cause of acute liver failure—placing it above viral hepatitis as a cause.
About half of acetaminophen overdoses are intentional, as in suicide. The other half are unintentional. Unintentional overdose often occurs because people are either unaware that acetaminophen can be dangerous, or they mix drugs. Even if you’re aware of the dangers of acetaminophen, you may not know what’s in the other drugs you’re taking at the same time. Acetaminophen is found in over 600 drugs,
including many cold medications, and this may not be prominent on the label. Consumers may also be led astray by a label’s wording: sometimes acetaminophen is abbreviated “acet” or “acetamin,” or is listed in the ingredients not by its common name but by the abbreviation of its chemical name: APAP.3 If you’re traveling abroad, know that in countries outside the US, Canada, and Japan, acetaminophen is called paracetamol.
Unfortunately, overdosing on acetaminophen is easy—there isn’t much difference between the maximum recommended dose and a potentially dangerous dose that is toxic to the liver. FDA guidelines limit the daily maximum dose of acetaminophen to 4.0 grams (g), or 4,000 milligrams (mg). (1 g equals 1,000 mg.) But for some people, even taking acetaminophen at below the FDA recommended maximum dose may be dangerous—a small percentage of people who take acetaminophen can’t efficiently metabolize (break down) the drug, and they suffer liver damage even
though they are taking less than the recommended maximum daily amount.
In 2011, McNeil Consumer Healthcare (a Johnson & Johnson company, the manufacturer of Tylenol and largest seller of acetaminophen) voluntarily lowered its recommended maximum daily dose from 4.0 g to 3.0 g, that is, from eight extra-strength (500 mg) tablets to six extra-strength tablets daily.
People may unintentionally overdose on acetaminophen by taking more than one drug containing acetaminophen. Suppose you’re taking extra-strength Tylenol (500 mg) for a joint bleed, and then on top of that, you take NyQuil® Nighttime Relief (containing 650 mg of acetaminophen) to get some sleep because you also have a cold. This combination of meds (1,150 mg of acetominophen) may push you over the 4.0 g daily limit.
It’s also easy to overdose on acetaminophen by taking repeated doses that are only slightly over the recommended maximum dose. Some people fall into the trap of thinking that more is better, and knowingly take a little more than the recommended dose—because it’s “close enough” not to cause problems, right? This is dangerous thinking! Taking multiple small overdoses, called staggered overdosing, is often more life-threatening than taking a single, large dose. Why? It’s harder for physicians to detect staggered overdosing because blood acetaminophen levels are low (as opposed to a single large dose, which is easily detected); and often, people often don’t go to a hospital for help until after the damage is done.
Why would anyone not go to a hospital for acetaminophen overdose? Often, because they don’t know they have overdosed! An overdose of acetaminophen doesn’t typically produce immediate symptoms, and when symptoms do appear, they often mimic those of the flu—often, the reason people are taking acetaminophen in the first place.
Symptoms of drug-induced liver damage, which may not show for several days or more, include loss of appetite, nausea, vomiting, fever, and abdominal pain. In more serious cases, urine may be dark (indicating blood in the urine), and the skin and eyes may be tinged yellow (called jaundice, an indication that the liver is not effectively removing the breakdown products of red blood cells). Usually, if the damage is not severe and caught early, the liver recovers once the drug is stopped or with medical treatment. But if the damage is severe, the liver will stop functioning effectively. This is acute liver failure, and without a transplant, it will cause death. .
How to Make Sure You Take Acetaminophen Safely
1. Always read the package insert! If the product you’re taking contains acetaminophen, check the package insert for correct dosing.
2. More is not better! Take the lowest possible dose of acetaminophen you need to control your pain.
3. Don’t take more than one product at a time that contains acetaminophen! And never drink alcohol when taking medicines that contain acetaminophen.4
Here are more ways to stay safe:
Keep your daily acetaminophen dose below 3,000 mg (3 g). That’s six extra-strength 500 mg pills, or ten regular-strength 325 mg pills. (This is less than the FDA recommendations.
Do not take acetaminophen for more than ten days unless you are under a doctor’s supervision; this increases your risk of liver damage.
If you have viral hepatitis, ask your doctor before using acetaminophen; you may. be at higher risk of drug-induced liver damage due to previous liver damage.
Consult your doctor if you take carbamazepine (anti-seizure medication) before taking acetaminophen. This drug can put you at higher risk of severe skin reactions to acetaminophen.
Be aware of these rare but serious and potentially fatal skin reactions caused by acetaminophen: Stevens-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP). If you develop a skin rash or reaction while using acetaminophen, stop taking the drug and seek medical attention immediately.
If you use warfarin (blood thinner, brand name Coumadin®), ask your doctor before taking acetaminophen, which interacts with warfarin, increasing
bleeding time.
If your child uses acetaminophen:
Never give your child adult doses.
Dose children based on weight, not age. McNeil Consumer Healthcare recommends consulting a physician before giving Tylenol to children under age two.
To measure liquid acetaminophen for a child, use the measuring device that comes with the child’s medication. Don’t use household teaspoons, which can vary widely in size.
Keep all pain medications out of reach of children, and securely replace childproof caps.
In case of overdose, contact the Poison Control Center: (800) 222-1222
1. High-dose NSAIDs for moderate acute pain are available by doctor’s prescription.
2. In a 2013 telephone poll of approximately 1,000 adults conducted by Princeton Survey
Research Associates International, 51% of respondents were unaware of any safety warnings associated with
Tylenol. The poll also found that many Americans also believed it was safe to
take several different meds containing acetaminophen at once. For example, 35%
of respondents said it was safe to combine the maximum recommended dose of extra-strength
Tylenol with NyQuil®, a cold remedy that also contains
acetaminophen. According to the FDA, this is not safe.
3. Fortunately, this is now uncommon. With greater awareness of the potential of liver damage from acetaminophen, most
drug manufacturers now prominently list acetaminophen in the active ingredients of the drug facts panel of their products, often in bold type with highlighting in bright yellow.
4. More than three alcoholic drinks a day significantly increases your risk of liver damage by decreasing the ability of your liver to properly metabolize

When Hemophilia Prevents Vampirism*

I love reading books, and have already hit my target for 35 books this year. History, nonfiction, biography, exploration and rock bands seem to attract me the most. I hope to cram in about two more before the year is out. One I may try is Mari Mancusi’s Blood Ties: A Blood Coven
Vampire Novel. 
Our friend and colleague Richard Atwood sent me a synopsis and commentary on this “young adult” book, which includes a main character who has hemophilia. That fascinates me and makes me want to read it.  I can easily accept the fantasy part (vampires and fairies) but have a hard time when someone mangles the science. I mean, science is science.  Here’s the rather convoluted plot:
Sunshine McDonald, or Sunny, a teenager with a freckled nose and long dirty blond hair, is enrolled in Las Vegas High School. Her formal name is Princess Sunshine of the Sidhe Light Court of Tir na nOg. Sunny is the royal daughter of Queen Shrinking Violet. Sunny has wings and can
fly because she is a full-blooded fairy. She also has a twin sister named Rayne with black hair.
Rayne, the only known vampiric fae in existence, wants to be a vampire slayer and recently attended a two-week vampire 12-step rehab program. (You gotta love that) Sunny was bitten by a vampire – she turned into one for six days due to mistaken identity with her sister and then became mortal again, or was cured, after drinking blood, whose antibodies help the human cells, from the Holy Grail in England. There she met Lord Magnus, Master of the Blood Coven. Sunny loves Jayden, a mortal with green eyes and black hair who earlier saved her life. Jayden has hemophilia. (How does that relate to the plot? I’m sure I don’t know!)
Jayden is bitten by a vampire, and to save him, Sunny allows herself to be bitten again (who is doing all this biting?) to provide blood for the first time. Jayden is ill. Lord Magnus explains Jayden’s condition to Sunny: “He’s stable now,” he replies. “But I don’t know for how long. I don’t know if it’s because of the manner in which he was bitten or his hemophiliac blood disorder—but his human cells aren’t properly bonding with the vampire ones. Meaning he’s not really a vampire. But he’s not really human either.” Maybe hemophilia interferes with turning into a vampire?
Jayden needs transfusions for his vampirism (not for his hemophilia). Sunny provides more of her blood using a syringe and blood bag (sadly Sunny did not follow universal safety precautions!), but the cure for Jayden is to drink from the Holy Grail. The trio set off to England
but the Holy Grail is stolen and taken to Tokyo for the impending war with the vampire Consortium. In Tokyo Sunny rescues her
abducted twin sister and prevents the war between the vampire factions. Good job, Sunny. Before the Holy Grail is blown up in the Japanese temple
by the Consortium vampires, Sunny transfers to Jayden some of its blood by kissing him, thus curing him of being a vampire. Sunny asks Jayden how he is feeling once they return to Las Vegas: “One hundred percent,” he says, looking slightly bashful. “Except for the fact that somehow in the transition my hemophilia went away.” What?!
Richard writes: This description of hemophilia is just as unrealistic and unbelievable as the rest of the story, but that will not deter its popularity
with teen readers as they somehow relate to a romantic fairy princess who kicks butt. The YA novel is part of on-going series about these vampire and fairy characters, including the character with hemophilia. The Emmy Award winning author and television producer lives in Austin, Texas
with her husband.
And I’d like to know: does Mancusi know anyone in Texas with hemophilia? Who’s the real life model for Jayden? Inquiring minds want to know. I’m pretty sure Mancusi didn’t read my book on hemophilia, which has no section on vampires.
Maybe a good stocking stuffer for your young adult reader with hemophilia? Blood Ties: A Blood Coven Vampire Novel by Mari Mancusi, 2011, New York, NY: Berkley Books. 231 pages.
*Just kidding folks. Hemophilia does not protect against vampirism! Buy yourselves some garlic.
Great Book I Just Read
The Little Prince
Antoine de Saint-Exupery
A fable developed during the real-life crash in the desert of Saint-Expury’s plane, this story has become a beloved classic. The little Prince is a visitor from another planet who appears in the desert, keeps the aviator company until he is rescued, and dispenses worldy wisdom with simple observations and visits to other planets, each small and occupied by only one person or animal. Lessons include:  to think outside the box, look at the world and its people with your heart, and my favorite, “You become responsible, forever, for what you have tamed”.  Five/five stars.
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