Laurie Kelley

Time to Rethink RICE? Part 2

Laurie Kelley July 10, 2022

By Paul Clement

RICE—rest, ice, compression and elevation—has been the most often recommended therapy for soft tissue injuries for more than forty years. However, the effectiveness and rationale for the “rest” and “ice” parts of RICE have increasingly been called into question. In part one of this two-part series, we looked at how using ice on a soft tissue injury not only slows the clotting process but may actually delay or prevent proper healing. Now we’ll look at why the “rest” part of RICE is also being questioned.

When you’re injured, the body responds with three sequential but overlapping phases of recovery:

1) inflammation [1 to 3 days];

2) repair [4-21 days]; and

3) remodeling [21 to 365 days or more].

Each phase must be successful, in order for the next phase to be successful. How does “rest” delay or prevent proper healing throughout these phases?

First, what does “rest” in the RICE protocol actually mean? Here, rest refers to immobilization (not moving) and non-weight bearing. So resting actually interferes with completion of both the inflammation phase and the repair phase of recovery!

After you’re injured, fluid and waste products produced by white blood cells accumulate around the damaged site. To remove this fluid, the body relies on lymphatic vessels (part of the lymphatic system). The lymphatic system has no heart, like the circulatory system, to pump fluid—it’s a passive, one-way mode of transportation that relies on the contraction of muscles, which in turn squeeze the lymph vessels, . If you rest a long time, you greatly reduce the movement of lymph fluid, which then allows an accumulation of waste products and excess swelling. Your damaged tissues will not recover well.

Resting (along with swelling and icing) also delays the repair phase. Resting decreases blood flow to the injured site. This delays the formation of new blood vessels that help rebuild tissues, which occurs in the remodeling phase. Stresses to the injury site which occur during movement also help collagen fibers  align themselves correctly, so proper healing can occur. Resting also starves cartilage cells in joints of nutrients, because the synovial fluid in the joint which lubricates and nourishes the cartilage, requires movement of the joint to flow.

No one is advocating you walk on a sprained ankle. Rest is important, but in moderation. (Of course, in addition to “factoring up,” all serious injuries should be evaluated by your hematologist!) The key is early mobilization, to regain or maintain range of motion, prevent muscle atrophy, and stimulate the movement of lymphatic fluid. Even simple motions such as wiggling your toes (in the case of an ankle soft-tissue injury) or light stretching are beneficial. Your hemophilia treatment center physical therapist can help you establish an appropriate balance between rest, early mobilization, and weight-bearing. This will help prevent delayed healing and unwanted complications associated with immobilization, while simultaneously minimizing the risk of rebleeding.

And the good news is that elevation (above the heart, for lower extremity injuries) and light compression can both help in healing: elevation by using gravity to assist in the movement of lymph fluid and compression in preventing excessive swelling. So keep these parts of RICE in your treatment plan!

Time to Rethink RICE? Part1

Laurie Kelley July 3, 2022

by Paul Clement

If you’ve ever sprained or strained an ankle or knee, or suffered from a muscle or joint bleed, you’ve probably heard that a couple of days of rest, ice, compression and elevation—RICE—is the surest route to recovery. But this simple at-home treatment has increasingly been called into question, especially the “rest” and “ice” parts. Many physicians and researchers now believe that rest and ice may actually delay or prevent proper healing.

RICE guidelines have been recommended by coaches and healthcare providers for over four decades—ever since the term was coined by Dr. Gabe Mirkin in his 1978 publication Sports Medicine Book. The intent of RICE was to speed the recovery process by reducing inflammation, swelling and pain. However, subsequent research demonstrated that icing actually prolongs the healing process. Dr. Mirkin recanted his original position in 2015, and today, he advises against icing an injury.

How can icing delay or prevent proper healing? When you’re injured, the body responds with three phases of recovery: 1) inflammation, 2) repair, and 3) remodeling. Each phase must successfully occur in order for the next phase to proceed and be successfully completed. That means inflammation must occur before the repair process can begin. If you reduce inflammation, then you delay the healing process.

Why is inflammation good? Immediately after an injury, blood vessels in the damaged tissue constrict (called vasoconstriction) to reduce blood flow; simultaneously, the blood clotting process (called hemostasis) occurs to reduce blood loss. Immediately after vasoconstriction, a type of immune cell in the damaged tissue, called mast cells, release inflammatory chemicals, such as histamines, which cause the blood vessels to expand (vasodilation) and become more permeable. This initiates the inflammatory process and allows other immune cells, called neutrophils, which are attracted to the site of the injury, to leave the blood vessels and enter the space between the cells, where they destroy damaged cells and mediate the inflammatory process. This sets the stage for another type of immune cell—macrophages—to come in and remove dead cells and release growth factors which are necessary for healing. When this process is complete, the neutrophils self-destruct, ending the inflammatory phase of the healing process.

The inflammatory process is extremely complex and researchers are still learning about it at the molecular level. We  know that the inflammatory process is not always good: in some auto-immune diseases such as rheumatoid arthritis, neutrophils that enter a joint during inflammation do not self-destruct, ending inflammation, but hang around and attack the cartilage lining the joints, destroying the joints.

Icing an injury delays healing by slowing inflammation. It slows the activity of clotting factors, slowing the formation of a clot. It prevents vasodilation, which slows the movement of immune cells into the damaged tissue, and the movement of waste out of the damaged tissue. It prevents the release of growth factors necessary for healing. And it increases the permeability of the lymphatic system, allowing fluids to flow in the wrong direction: from the lymph vessels into the injured area, increasing the amount of local swelling.

Icing can help reduce pain, but many physicians now recommend using acetaminophen (Tylenol) to reduce pain, and not ice. Finally, do not use anti-inflammatory drugs, such as ibuprofen (Motrin), to reduce pain: these drugs also reduce inflammation and slow healing—in addition to interfering with the clotting process, which may prolong bleeding.

Next week: Why movement helps in the healing process.

Remembering Fathers in Hemophilia: Samuel Appleton

Laurie Kelley June 19, 2022

A tribute on Father’s Day to the first known father of a son with hemophilia in the Americas

by Richard Atwood

I search for intriguing stories about people with bleeding disorders. By discovering those stories, including historical ones, I always learn something valuable. Often, I find inspiration in the stories of other family members, as in the case of the Appletons, who were connected with the powerful origins of our country.

One father of a child with hemophilia was Major Samuel Appleton (1625–1696). His son, Oliver Appleton, was the first person identified with hemophilia to be born in the American colonies.1 Samuel spent a lifetime in public service fulfilling legislative, judicial, and military roles. He stuck to his principles about the illegality of improper taxation, and he remained calm in times of distress—during battle, and during the infamous Salem witch trials.

Samuel was only 11 when his family left England to settle in the Massachusetts Bay Colony in 1636. His father, also named Samuel, was one of the original settlers of historic Ipswich. The family owned a house and eight acres in town, and a 400-acre farm on the Ipswich River. 2

One of five children, Samuel grew up to help run the family farm and businesses. He married Hannah Paine in 1651, and they had three children. After his wife’s death, in 1656 he married Mary Oliver (1640–1698), a hemophilia carrier, and had eight more children, including Oliver in 1677. Oliver’s bleeding disorder was noted by family members, but probably not as a genetic condition. Only later, in retrospect, did family members realize the distinctness of the bleeding.

Due to periodic threats of Indian attacks, Samuel Appleton led the local militia. From lieutenant in 1668, he rose to the rank of captain during King Philip’s War, and commanded an infantry of 100 men. At the decisive battle near Hatfield along the Connecticut River in 1675, Samuel was commander-in-chief of more than 500 men. A turning point for the colonists, this battle proved that the Indian warriors could be defeated. During the fighting, a bullet passed through Samuel’s hair. If he had died then, his son Oliver with hemophilia would never have been born.

Samuel held several elected offices. As a legislator, he was a commissioner of Essex County in 1668. He was a representative of the General Court from 1669 to 1680. And he served on the Governor’s Council from 1681 to 1692. Appleton opposed the government of the colonial governor, Sir Edmond Andros. When in 1687 Andros levied a tax of one penny on a pound, the town of Ipswich refused to collect the tax, stating that it was against the rights of Englishmen for any taxes to be levied without consent of an assembly chosen by landowners, or “freeholders.” An arbitrary and illegal warrant was issued for the arrest of Samuel and other leaders in the opposition to the tax. Samuel took refuge in Saugus, where he stood on a rock and denounced the government. A Massachusetts historical marker now acknowledges the site as “Appleton’s Pulpit.” Refusing to apologize, Samuel was imprisoned in November 1687. He petitioned in January for his release due to his age and weakness, but wasn’t freed until March 1688, when he posted a 1,000-pound bond.

In 1689, during the coup of crown-appointed Governor Andros, Samuel and other leaders in the Massachusetts Bay Colony put Andros on a boat to the island prison in Boston Harbor. Colonial revolutionaries 100 years later simplified the opposition to taxes with the slogan “No taxation without representation.” But it’s important to remember that the ideas for the American Revolution began long before 1776: to be properly recognized, Ipswich adopted the motto “The Birthplace of American Independence 1687.”

Samuel Appleton served on the judiciary. He was a deputy to the Massachusetts General Court from 1668 to 1681. As a member of the Council of Assistants from 1681 to 1686, Samuel attended the examination of accused witches in Salem on April 11, 1692. His role may have been minor; he isn’t always listed as one of the seven judges. And apparently he did not serve as a judge in any of the trials that executed 20 alleged witches in 1692. On May 2, 1693, the first Supreme Court convened in Ipswich to try Andover residents charged with witchcraft. As a judge at that hearing, Samuel cleared everyone accused of witchcraft, ending the infamous witch trials and demonstrating his rationality. During the hysterical witchcraft proceedings in Salem, Oliver Appleton was a 15-year-old with hemophilia living at home in Ipswich.

The story of Major Samuel Appleton reveals essential information about colonial America. Some of our defining principles that we cherish today were sown by the colonists years before the revolution for independence. We need to honor those colonial leaders for their contributions, and remember that Major Samuel Appleton also raised a son with hemophilia.

1. “The Appletons: America’s ‘First Family’ with Hemophilia.” PEN, Nov. 2002.

2. That farm still exists today. Called Appleton Farms, it is the second oldest continuously run farm in America, now administered by the Trustees of Reservations, a nonprofit conservation organization in Massachusetts.

© 2016 LA Kelley Communications, Inc. Reprinted with permission. All rights reserved.

A Circle Complete

Laurie Kelley June 12, 2022

Big changes happening this week: after 20 years, we have officially closed our office. Not our business… just the physical office as we downsize. Like all my subcontractors, I’ll be working from my home.

And why not? My kids are gone, I have the space, and it will save money. I opened my first office for one reasons only: to have a place to store factor and prep it to ship overseas. I started my business from my home, so I could be around for my children. And when the first donations of factor started arriving in 1996, I could handle it from my home. But one day, 1 million units arrived, and I knew we had to find a place to store it, other than my home.

My first office: in the basement, circa 1998

Luckily there was a place 2 miles from me—and it was in-between the two schools where my children went. In fact, the high school kids often stopped by to ask “Mrs. Kelley” for some money so they could go to Dunkin Donuts. Or if they needed a ride home.

A new owner jacked up our monthly rent, so we moved down the street to a much nicer office, bigger, where each employee got their own office. And the owner of the building was a local guy, who was very supportive of our work. Eventually, he sold the building and again the rates went up. So we scouted out another building, also down the road. The Eagle Building was aged, with no elevator, but we managed. We decided to downsize a bit and keep only what we really needed. We were at this building until today, for 9 years.

The pandemic radically changed our lives, some of it temporarily and some permanent. For me, two years of not traveling internationally gave me a tremendous amount of time to think. It was time to go minimalist: sell the bookcases, ditch anything we had not used in a few years. Giving Project SHARE and all the factor over to Save One Life reduced our workload tremendously. And somehow, this past week I fit our entire office into the very tiny room in my basement where I first had my business. It was fun having an office, employees, office parties, and visitors, but it’s better now to be lean, light and mobile. Back where I started… a circle complete.

My first professional office: 68 East Main
My second pro office: 65 Central Street
A whole room just for factor!

Coming Changes: Kogenate® FS Discontinued

Laurie Kelley June 5, 2022

It was bound to happen sooner or later: in a community flush with hemophilia A therapies, one of them would have to give.

This past week Bayer announced that it would discontinue production of Kogenate® FS (Antihemophilic Factor (Recombinant), which has been in use since 1993. I’m nostalgic about it leaving; when it appeared, my second born was only 3. We used it at one point. We educated the community that it was the exact same product as Helixate®FS… though many parents tried to argue with me that they had different names and were from different companies, and were therefore different. They were not.

But times are truly changing. We have extended half-life (“long lasting”) products now; we have products made from a human cell line, and not hamster cell lines. We even have transgenic animal therapies. And we have Hemlibra, an injectable with a half-life of over 600 hours.

But above all, we have probably too many factor products for hemophilia A patients, who number around 20,000 in the US. How will the market justify all the products?

Our factor chart below shows 12 products for hemophilia A that are recombinant, and five products that are plasma-derived (from human blood). Some manufacturers, like Bayer, Novo Nordisk and Takeda, have multiple factor VIII products. It’s like they are in competition with themselves. Some are standard factor products and others are extended half-life.

Bayer’s press release says: “Recognizing the growing shift in patient use toward more recent products, such as Kovaltry® (Antihemophilic Factor (Recombinant)) and Jivi® (Antihemophilic Factor (Recombinant), PEGylated-aucl), Bayer has made the decision to discontinue Kogenate® FS (Antihemophilic Factor (Recombinant)) in the United States.  

“Keeping the needs of patients in mind, Bayer is keenly aware that they will need sufficient time to work with their HCP to make decisions about their next treatment. The timing of discontinuation will vary by Kogenate FS vial size. Customer demand may lead to depletion of the larger vial sizes of Kogenate FS during the fall of 2022. Remaining Kogenate FS vial sizes are anticipated to be available into 2023.

“As Kogenate FS patients and their caregivers embark on the next step of their journey, Bayer is committed to supporting them. Attached, please find our discontinuation announcement for your reference. Additionally, we have set up a website (explore.bayer.com) and a dedicated Kogenate FS hotline for anyone who has questions regarding this discontinuation, (1-833-40-BAYER), which is available Monday – Friday, 8:30am – 8:00pm ET.” See the full press release here.

If you are a Kogenate FS user, it’s time to contact your HTC staff and discuss next steps. We’ve been through this before over the decades: we know that some patients want to stay with the manufacturer, and will switch to their other products. Other patients will take this as an opportunity to learn more about other products from other manufacturers. Whichever you are, be sure you make your decision with your HTC staff… not the internet! Not even with me. Call the manufacturer and speak with your HTC staff, learn more about these products, and then choose which is right for you.

And probably more change is coming, in products, manufacturers… and eventually gene therapy.

Resolve to be a master of change rather than a victim of change.— Brian Tracy

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