Laurie Kelley

Barbers: A Cut Above

Laurie Kelley June 27, 2021

Today I was walking about Newburyport, Massachusetts, an old seaport, now a lovely tourist town, following an exciting whale watching boat ride. As Doug and I strolled past the sidewalk shops, I noticed an old-fashioned barber pole, with the red and blue stripes.

“Ever wonder how the barber pole got its stripes?” I asked Doug. He had not.

Well, medical science was basically primitive in the Middle Ages. In fact, barbers sometimes performed surgery. After all, they had razors, right? A standard treatment for just about any ailment was “bloodletting,” where the doctors—or barbers—would drain someone’s blood. It was believed that blood sometimes contained bad “humors,” and a build-up of them needed to be released. You can see a decent portrayal of it in the wonderful 1995 Ang Lee movie “Sense and Sensibility.”

The barber pole developed as a symbol of bloodletting. During bloodletting, the patient would grasp a pole to make veins stand out. Then the barber-surgeon would cut into a vein, and blood would drip into a basin. Afterward, blood-soaked bandages would be hung outside as advertisement. Twirled by the wind, they would form a red and white spiral pattern that was later adopted for painted poles.

Eventually the bandages would be replaced by a wooden pole, with painted red and white stripes. Variations of the barber pole appeared in different countries, with some alternating a blue stripe with the red, like the one I saw today.

While the barber pole is recognized around the world, its days could be numbered. The William Marvy Company of St. Paul, Minnesota is the sole remaining manufacturer of barber poles in North America. To date, the William Marvy Company has produced over 82,000 poles, but only sells about 500 a year.

When you spy a barber’s pole, you are seeing an icon that dates to the Middle Ages, and now is quintessential Americana.

Still Advocating at 89!

Laurie Kelley June 13, 2021

Revenge is sweet at age 89, even if it is only the knowledge that you were right and the experts were wrong.

On Thursday I learned my 89-year-old mother, affectionately called “Gram” by all, was rushed to the ER by my brother when she complained of really not feeling well, during his daily visit with her.

For those of you who know my mom, she is an 89-year-old spitfire; a rebel with all kinds of causes; possessing a passionate desire to educate the entire world about everything she has ever read or learned; still raising her 18-year-old great-grandson; and… a fantastic advocate for her own medical care.

Home 24-hours after the procedure:
advocacy in action!

In other words, not someone to trifle with or be dismissive of.

She is generally in excellent health. Absolutely no joint problems, making me look like the infirm as I struggle through some back issues. Active, still driving and driving all the grandkids and great-grandkids around town, going to church almost every day and reading several books at once. And politics? Don’t get us started!

So for her to complain about a health problem was odd. She does have diabetes, which runs in the family on both sides. But that’s been manageable. The latest issue was a severe pain in her lower jaw, that often manifested when she climbed the cellar stairs. And she climbed the stairs slowly, too slowly for her liking, and she had to rest after climbing them. These are the same cellar stairs she has been climbing daily for 60 years.

I suggested going to a dentist to rule out an infection under the tooth. She did and there wasn’t.

She had been asking her primary health care (PCP) physician, who has been her doctor for years. The PCP didn’t think the jaw pain was a concern, or the short breath. She rather dismissed it. From time to time, my mother would ask her again about it. Once my mother said, Do you think it’s related to my heart?

The doctor winced and said, Who told you that?

Well, that was that. My mother, age 89, decided to put her formidable advocacy muscles to work. She fired her PCP and found another medical center. Got a second opinion. This new medical team went right to work with a clean slate of opinions. Two stress tests (one a nuclear one) later, and the team determined she had a blockage in her circumflex coronary artery. Friday, June 11, she had a procedure, with her awake the whole time, to place a stent, to open the artery.

All this because she trusted her instinct, knew her body (she did give birth to nine children, so there’s that), and perceived an issue of sorts with a surly doctor. After months of no answers, finding the right medical team gave her answers in two weeks. That’s advocacy in action!

During the procedure, her hand started to turn a dark blue (the catheter was inserted in her wrist area), and the team was apologetic and assured her it would clear quickly. She replied, I don’t care! You can cut my hand off! It’s worth it to know I was right!

I never once wondered where I got my advocacy muscles from!

Remembering Our Veterans

Laurie Kelley May 31, 2021

Today is Memorial Day in the US, when we remember those who gave their lives in service of their country. I had an uncle, Jim Morrow, who died in Vietnam, on October 15, 1967. His name is inscribed on the Vietnam Memorial wall in Washington DC. Those we remember are those who willingly joined the service as career veterans, or were called to active service in a time of war.

I often think of our bleeding disorder community on Memorial Day. We were at war at one time, from the late 1970s through the 1980s, fighting the scourge of HIV. My son with hemophilia was born in 1987, at the height of the HIV battle. The Ray brothers had just been bombed out of their home in Florida. Our “soldiers” were gathering for a battle against pharma and the US government, to hold them accountable for the contamination of our nation’s blood supply. It was a scary time, especially for a new parent. I attended my first NHF meeting in 1992, and witnessed so much hostility and anger, directed at the displays by pharma, and at each other. Some of our community members were taking a legal approach, hoping for compensation through negotiation; others wanted a more militant approach to solving the accountability problem. So many had died. So many.

I was on the “other” side; my baby had escaped the dreaded HIV infection by only a year or two. When I met these leaders and soldiers in the community, I was struck by their dedication, knowledge and courage. Some were dying already, but fought the good fight to win compensation for all. That would be our only freedom. They were victims of this insidious infection, but became soldiers to defend us, the ones who barely escaped. Their efforts ensured a safer future for all.

Many of those soldiers have died now, but I still remember them. I feel honored to have known them. And I always appreciate how by fate, my child escaped their fate by mere months. I keep photos of some of them on a shelf at home, and have for the past 20 years. Names like Michael Davon and Tom Fahey, co-founders of the Committee of Ten Thousand (COTT); Dave Madeiros, a visionary leader who sought to provide funds to the community by owning a specialty pharmacy—his insights into insurance changes and prophecy about managed care and restrictions all came true.

And some photos are of patients who died, who inspire me all the time. It’s important we remember them all, and carry on their legacy. We are of a new generation now, and our community may be forgetting these veterans.

How can you get to know them and remember them? Read And the Band Played On (also an HBO movie). Watch the movie “Bad Blood: A Cautionary Tale.” Buy the book Dying in Vein. If you have hemophilia, or a child with it, knowing your community history is every bit as important as knowing American history this Memorial Day.

Factor Products: How Factor is Made?

Laurie Kelley May 23, 2021

Safety and purity are considered along every step of the factor manufacturing process. For most factor concentrates, the manufacturing process has four basic steps:

1. Sourcing factor

2. Viral removal and inactivation

3. Purification

4. Final formulation

Sourcing Factor

Plasma-derived products come from human blood plasma. Plasma donors undergo strict screening for disease risk factors, and their plasma is tested for several viral diseases. Recombinant products are not derived from blood; they originate from genetically engineered mammalian cells containing the human gene for factor. Recombinants are produced in large bioreactors, with human and animal proteins used in the culture medium in first- and second-generation recombinant factor. Third-generation products contain no human or animal proteins in the culture medium or the final product. Fourth-generation products are made from human cell lines.

Although plasma-derived products potentially risk transmitting blood-borne viruses, all US factor products, whether plasma derived or recombinant, are considered safe by the FDA. (See last week’s blog on purity vs safety to learn more)

Viral Removal and Inactivation

These methods remove or inactivate most blood-borne viruses including HIV, hepatitis A, hepatitis B, and West Nile virus, making them noninfectious. But no viral inactivation method used on factor concentrate can inactivate all viruses. The two most common viral inactivation methods are heat treatment (pasteurization) and chemical

inactivation. Heat treatment involves exposing the factor to a high temperature for 30 minutes to 72 hours, depending on the method.

Chemical inactivation involves mixing the liquid factor in a tank with a solvent-detergent (SD) wash for four to six hours. SD viral inactivation is very effective against certain types of viruses, such as HIV and hepatitis B and C, but is ineffective against hepatitis A and some other viruses. Viruses are also removed by the purification process, especially

immunoaffinity (monoclonal) purification. Viruses can also be filtered from factor IX through a process called nanofiltration.

All plasma-derived products use one or more viral inactivation processes, and so do some recombinant products. Yet other recombinant products use no viral inactivation process. Why? The risk of viral contamination is only theoretical, because the product is not exposed to blood plasma.

Purification

This step separates the desired factor from unwanted viruses, proteins, and other foreign substances, to get the purest product containing only the factor you need. For example, when plasma is processed to make factor VIII concentrate, the serum may also contain von Willebrand factor (VWF), factor I, and other proteins. The higher the listed purity of a product, the fewer the unwanted proteins.

Monoclonal products have a higher purity than intermediate products. Recombinant concentrates have the highest purity of all products.

Final Formulation

Even if the viral inactivation and purification processes create a safe and highly pure product, the final formulation—the way a product is packaged and prepared for market—may alter it. In this final step, other components may be added into the concentrate. For example, albumin is added into the final formulation in the last manufacturing steps of first-generation recombinant products. Albumin helps to stabilize and bulk up the product.7 In second-generation recombinant factor, sugar is added in place of albumin at the final formulation step to stabilize the product. In third-generation products, sugar is added to stabilize the final product, and no human blood component or animal proteins are used in the culture medium.

Why would anyone intentionally choose a plasma-derived product instead of recombinant? After all, recombinant factor is the product recommended by NHF’s MASAC. Why choose an intermediate product and not an ultrapure one? Why inject anything other than the missing factor into your child?

For some people, it’s all about cost. Plasma-derived factor, especially intermediate purity, is less expensive than recombinant factor. People who have high out-of-pocket expenses need safe products, but may choose less expensive ones. Sometimes, the decision depends on the type of bleeding disorder being treated. For example, intermediate purity factor VIII products contain factor VIII combined with VWF (the way it’s naturally found in the blood) and are useful in treating von Willebrand disease.

When choosing your factor, always discuss options with your HTC! Use our factor product guide here, bring it with you, and ask what’s best for your loved one or you.

Factor Products: Gen H… for Hemophilia

Laurie Kelley May 16, 2021

Do you use a recombinant factor product? Do you know how it’s made?

Recombinant products are not produced from human blood plasma. They are produced in large stainless steel tanks, called bioreactors, which contain trillions of cells. Into each of these cells, a gene for human factor has been inserted, or “recombined”—the origin of the name recombinant. These genes produce human factor and release it into the culture medium—a nutritious liquid that keeps the animal (or host) cells alive and growing. Although the source material is not blood, some recombinant products contain extraneous human or animal proteins introduced during the production process or added to the final product.

To distinguish between the various production processes, recombinant products are classified according to generation. Generation refers not only to when the products were first developed and commercially available, but also to the presence of animal or human proteins used in the production process or the final product.

First-generation recombinant products, introduced in 1992, use human or animal proteins in the growth medium. These products also contain human albumin added at the final production stage to help stabilize and bulk up the product.

Second-generation recombinant products contain no human albumin added to the final product, but do use human or animal proteins in the growth medium.

Third-generation recombinant products, first available in 2003, contain no human or animal proteins in the growth medium or added to the final product. They have the lowest risk of transmitting viruses.

And while MASAC (NHF’s Medical and Scientific Committee) has not yet confirmed the nomenclature of fourth generation, Octapharma and Sanofi Genzyme each created a recombinant factor product that is created from a human cell line, not animal. The two companies are calling their products, Nuwiq® (Octapharma) and Eloctate (Sanofi Genzyme) fourth generation.

If you are using recombinant product, what generation is your factor? Download our Factor Comparison Chart and find out!