In July 2004, Richard Bell was a newlywed who had just moved from London to California, purchasing a new home with his wife, Claire. When severe back pain kicked in, he attributed it to some heavy lifting along the way. After what Bell calls typical male procrastination, he visited a doctor in September. A CT scan revealed a large mass on a vertebra in the middle of his back. The next day, following the advice of the radiologist, Bell consulted an oncologist, who suspected multiple myeloma.

Not yet fully established in the United States and without health insurance, Bell had no choice but to head back to London for treatment. He remained there for a year and a half. The diagnosis, he says, forever changed his life. It also connected him with the Moving Mountains for Multiple Myeloma program, a partnership between CURE® and the Multiple Myeloma Research Foundation (MMRF), which runs endurance events several times a year to raise awareness and funding for the disease. In an interview with CURE®, Bell details hearing that he had months to live, relearning to walk and taking the climb of a lifetime.

CURE®: Can you describe your multiple myeloma diagnosis and treatment?

Bell: In a lot of cases, I was very lucky that I had this tumor on my T7, because I got a fairly quick diagnosis. It was within six months. My oncologist said, “Look, if you don’t do anything about this, you’ve got six months to live. I think it’s multiple myeloma.” I had never heard of multiple myeloma. He didn’t let me out of his sight. He put me in a wheelchair and took me across the parking lot to the hospital where they did a bone marrow biopsy that evening. (The next) morning, we got the results confirming multiple myeloma. I immediately had three weeks of radiation at Mission Hospital, a targeted treatment to shrink the tumor. The tumor had impinged on my spinal cord, and I lost all feeling from the waist downward. I had a lot of problems with walking and not feeling anything. At that time, I hadn’t been in the states for a year, and I didn’t have any medical insurance. I hadn’t set up my business, which I had planned to. I went back to the UK and had my treatment there.

What was it like to hear “You have multiple myeloma”? 

Pretty dramatic. When someone tells you that you have six months to live unless you do something, it’s kind of a kick in the teeth. It’s twofold. It’s something that you never heard of before. If they said “leukemia,” you’d go “Oh yeah, leukemia — that’s a blood cancer.” But multiple myeloma, what the hell is that? So, you do what I imagine 99% of people do, which is go to the (internet) and get frightened to hell. It’s an incurable blood cancer. At that time, rates of survival weren’t particularly good. Amazing things have happened since 2004. It’s a life-changing moment &mdash; there’s no way around it &mdash; and it was upsetting for me because I was upset for Claire.

You recently climbed Mount Washington as part of the Moving Mountains for Multiple Myeloma program. How did you get involved with this?

I’m an unusual myeloma case. I was young and was able to have an allogeneic transplant with my sister. There are so few people around who are in my position. Over the years, I gradually fell away from going to group meetings and being involved in myeloma in any way, but that didn’t stop me from receiving emails and reading up on treatments. How I got to Moving Mountains for Multiple Myeloma was in my normal way of keeping up to date.

When I found the videos, I was really impressed. And I thought, “I walk every day.” Part of my therapy and recuperation was walking. I pretty much had to learn to walk again after my bone marrow transplant because I was so weakened by being in bed and from treatment. A simple thing of walking was a huge deal. The walking and hiking aspect was very appealing.

It was fabulous to be with like-minded people. In the end, the trip didn’t go quite as planned, because we couldn’t get to the top of the mountain. But in some ways, it was almost a tougher hike than the original plan, which would have been up (the mountain), stay the night, and come down the next day. We walked about seven miles. The comradery was fantastic. And (it was nice) hearing (about others’) experiences.

I trained very hard for it. I had been doing nine miles a day, every other day and walking up a very steep local hill. I would walk up it four times. I think if I hadn’t done that, I certainly would have been a lot more tired than I was. It was a hell of a climb down.

What did the Mount Washington climb mean to you?

(It was about) giving something back to all the people who helped me. My treatment was unusual, so I haven’t been able to advise many people in groups or give back an awful lot. I was happy to be able to raise money to help other people. Part of it was also for a specific guy, Ray, who was in the fire service with me and who also had myeloma. He died while I was having treatment. I thought about him a lot during the hike. And, basically, for the young people — it’s nice to be able to give some money back to the MMRF so they can do what they do, because it seems to me that there have been more and more young patients with myeloma coming into the fold over the past 10 to 15 years.

What advice do you have for others who are living with multiple myeloma?

(Take) small steps. I came up with small-steps thinking after my bone marrow transplant. Before they would let me out of the hospital, I had to walk the length of the ward and up the stairs to the next level of the hospital, and that took a week just to walk. When I got back from the UK, I learned to start walking again around Dana Point Marina. Plain walking doesn’t sound like much, but it’s a great brain/mind and physical therapy.

Do you have a motto or quote that you live by?

Accomplish something every day. It doesn’t matter how big that something is. For me, if it’s a bigger thing, then that’s great, but if it’s something that takes only an hour, it’s equally as important. I also try not to stress anymore, enjoy life and not procrastinate.

Is there anything else that you would like to add?

As far as the average myeloma patient is concerned, I think going to groups and talking with other people is a great thing. It was of help to me in the beginning. In the early days, Orange County didn’t have a multiple myeloma group. I had to drive monthly to San Diego. Communication and talking with like-minded people who had similar treatment is key so people don’t feel isolated.

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After learning to walk again following a life-changing multiple myeloma diagnosis, Richard Bell takes on Mount Washington.

Small Steps, Big Dreams

Receiving supportive care can help ease symptoms of cancer and its treatment, improving quality of life for patients. But many with multiple myeloma are missing out on aspects of this type of care, according to study findings published in 

Using the Surveillance, Epidemiology and End Results-Medicare database, researchers identified nearly 2,000 adults ages 66 and older who had received a multiple myeloma diagnosis between 2008 and 2013.

Their goal: to see who received guide- line-recommended supportive care. This includes bone-modifying drugs within one year after diagnosis, influenza vaccination in the first season after diagnosis and simultaneous use of prophylactic antivirals with proteasome inhibitors, drugs that block the action of proteasomes, which are large protein complexes that break down proteins and can increase the risk of certain viral infections such as chickenpox and shingles.

The review revealed that 64% of patients received bone-modifying drugs, although non-Hispanic black patients and those with baseline renal impairment had lower odds of getting these agents.

Just over half of patients (52%) got an influenza vaccination. Again, non-Hispanic blacks had lower odds of being vaccinated, as did patients with dual Medicaid enrollment.

Finally, 49% of patients received antiviral prophylaxis, with patients who were treated in the community-based setting least likely to have this preventive measure taken.

Lack of supportive care may lead to unnecessary high symptom burden and poor quality of life, the researchers said, noting the need for targeted interventions for patients.

Research News and Updates

Receiving supportive care can help ease symptoms of cancer and its treatment, improving quality of life for patients. But many with multiple myeloma are missing out on aspects of this type of care, according to study findings published in Cancer.

Patients With Multiple Myeloma Get Shortchanged in Supportive Care

November 2013 marked the start of Sonia Dolinger’s struggle with severe fatigue, headaches and tremendous sinus pain. “I felt like I had mononucleosis,” she says. “I slept for hours, and when I woke up, I felt like I needed to sleep more.”

Dolinger, then 39, had enrolled in a master’s nursing program at the University of Maryland with the aim of caring for patients with cancer. She was a mother three — the youngest just 5 years old &mdash; and had been a full-time caregiver to her mother, who had just died of ovarian cancer. “I tried to attribute the fatigue to grief or stress, but cancer was always in the back of my mind,” she says.

She visited her primary care doctor, who discovered swollen lymph nodes near Dolinger’s ear. Blood tests revealed a high white blood cell count. Desperate to get to the bottom of her symptoms, Dolinger asked an ear, nose and throat specialist to remove and test one of the nodes. The diagnosis: chronic lymphocytic leukemia (CLL), a cancer of the lymphocytes, or B cells, that begins in the bone marrow and migrates to the blood and sometimes to lymph nodes.

CLL strikes more than 20,000 people in the United States each year, according to the American Cancer Society, and affects more men than women. Some studies suggest that people who were exposed to Agent Orange, a toxic herbicide used in the Vietnam War, have a greater risk of developing CLL. Others point to genetic risk factors: People who have a first-degree relative with CLL are two to four times more likely to develop the disease. But for most people with CLL, including Dolinger, the disease stems from being dealt a bad hand.

In CLL, leukemia cells build up slowly in the bone marrow, eventually crowding out normal white cells, red blood cells and platelets. Because the hallmark of CLL is abnormal bloodwork, most patients discover they have the disease almost by accident during a routine checkup. “The analogy I use with my patients is that leukemia cells are like weeds growing in your garden. If it’s chock-full of weeds, the good stuff can’t grow through,” says Dr. David L. Porter, Jodi Fisher Horowitz Professor of leukemia care excellence at the Perelman School of Medicine and director of cell therapy and transplant at Penn Medicine in Philadelphia.

Historically, doctors considered CLL an incurable disease with limited treatment options, none of which were curative. Patients either responded to initial treatment, sometimes for many years, before ultimately relapsing or their disease worsened within six months of initial therapy (refractory CLL). Now doctors have more tools than ever in their arsenal to combat CLL, including more than half a dozen chemotherapy-free regimens.

“The treatment landscape for CLL has dramatically changed over the past decade, both for people with relapsed disease as well as people who are getting front- line treatment,” says Dr. Nitin Jain, associate professor in the Department of Leukemia at The University of Texas MD Anderson Cancer Center in Houston. “With the development of targeted therapies, including single-agent and combination regimens, patients are achieving more durable remissions and better quality of life.”

Scientists still don’t fully understand what triggers CLL or how to definitively stop it from progressing. They don’t know why CLL often returns after treatment or why some patients don’t respond at all. Many people experience recurrent disease for years and require multiple treatment courses. Fortunately, with novel treatment options and combination therapies, CLL is increasingly shifting from an incurable chronic disease to a once-and-be-done cancer.

When patients like Dolinger receive a CLL diagnosis, they often feel like their future is being erased. “Hearing the words ‘incurable disease’ was like getting a kick in the gut,” Dolinger says.

After scouring the internet for answers, Dolinger learned about a potentially curative treatment called Imbruvica (ibrutinib). “I remember reading about a patient who took Imbruvica while he was on hospice. Within weeks he had his life back,” Dolinger says. “That gave me hope.”

She joined an online support group where veteran patients with CLL all said the same thing: See a CLL specialist. Within two months, Dolinger was sitting in front of Dr. Bruce Cheson, deputy chief of the Division of Hematology/Oncology at Georgetown the Lombardi Comprehensive Cancer Center in Washington D.C. Cheson has been treating patients with CLL for decades, yet during Dolinger’s first visit with him, he told her the best course was to do nothing. He prescribed Adderall, a medication for attention deficit hyperactivity disorder, to alleviate her fatigue and put her on a check-in schedule to monitor her blood levels.

Unlike solid tumors — of, say, the breast, prostate and lung &mdash; in which the focus is early detection and immediate treatment, the first-line approach for CLL is sometimes to watch and wait. Dating back to the 1980s and 1990s, several studies reported no survival benefits from treating CLL upon diagnosis. “If we jump into treatment too quickly, we risk exposing patients to toxicities without any benefit,” says Dr. Tanya Siddiqi, a hematologist-oncologist at City of Hope in Duarte, California.

Doctors base the decision to treat on evidence of disease progression according to guidelines established by the International Workshop on Chronic Lymphocytic Leukemia. Factors include things such as, enlarged lymph nodes, spleen or liver; decreased blood and platelet counts; evidence of autoimmune hemolytic anemia; and debilitating, flu-like symptoms. When

it’s time for treatment, doctors have a slew of medication options, such as:

Chemotherapy, such as Fludara (fludarabine), Bendeka (bendamustine) and Cytoxan (cyclophosphamide).

Targeted therapies — kinase inhibitors to block the growth pathways of cancer cells, such as Imbruvica and Zydelig (idelalisib), and BCL-2 proteins to regulate cell growth and cell death, such as Venclexta (venetoclax).

Monoclonal antibodies that attach to a specific protein (CD20) on the surface of B cells contributing to the disease and kill the cells, such as Rituxan (rituximab), Arzerra (ofatumumab) and Gazyva (obinutuzumab).

When 71-year-old Terry Evans received a diagnosis of CLL in 2000, the most effective treatment options were chemoimmunotherapy (a three-drug cocktail of Fludara, Cytoxan and Rituxan, more commonly known as FCR) and a bone marrow transplant. A computer technology manager for the city of Long Beach, California, Evans sailed by for years with almost no symptoms.

“I assumed I was part of the lucky 20% to 30% of patients who don’t need treatment,” he says. Evans retired in 2005. Then, in 2007, his white blood cell count doubled within a month. It doubled again the following month. He began experiencing night sweats, fatigue and muscle weakness and lost weight. “I was so weak that I had to sit down after a shower just to dry off,” he says.

That’s when he decided to see a specialist. Turns out Evans had developed autoimmune hemolytic anemia, a complication of CLL in which the immune system attacks its own red blood cells. “The doctor looked at my blood under the microscope and admitted me to the hospital,” Evans says. “If I hadn’t come in, the doctor said, I would have been dead within 48 hours.”

Once his autoimmune condition was under control, Evans joined a three- drug clinical trial investigating a BCL-2 inhibitor called navitoclax (formerly ABT-263), Bendeka and Rituxan. His blood levels all returned to normal during treatment, but the regimen wreaked havoc on his liver enzymes, forcing him to drop out of the trial.

Two years later, he enrolled in another trial comparing Imbruvica with Arzerra. He participated in both groups of the trial, then relapsed again. In May 2017, Evans joined a third trial, in which he stayed on Imbruvica and added Venclexta. “Within one month on the combined regimen, my blood numbers were the lowest they’d been in eight years,” Evans says. “My doctor did a test for minimal residual disease (MRD), testing one in 100,000 cells, and couldn’t find any CLL in my body.”

This sort of treatment merry-go-round is the norm for patients with CLL, but evidence of MRD among treated patients is also becoming increasingly common. By the time Dolinger dipped her toes in the treatment pool in November 2016, chemotherapy regimens for CLL were falling out of favor and novel agents like Evans’ latest drug cocktail were becoming standard therapy. Several studies reported that these agents produced better outcomes with fewer toxicity concerns.

There’s evidence that targeted therapy, such as Imbruvica, can reduce the rate of infections over time and may help reconstitute patients’ immune systems. Experts like Dr. John Allan, assistant professor of medicine in the Division of Hematology and Medical Oncology at New York-Presbyterian and Weill Cornell Medicine in New York City, argue that Imbruvica, in particular, is so effective at altering CLL’s natural history that treated patients may approach the life expectancy of the general population.

In July, a trial that included more than 500 patients with newly diagnosed CLL showed that the combination of Imbruvica and Rituxan stopped the progression of leukemia in 89.4% of participants about three years later compared with 72.9% of those who received the traditional chemotherapy combination — FCR. In addition, at three years, 98.8% of people who received the combination were compared with 91.5% of those who received traditional treatment.

Currently, researchers are investigating a combination therapy pairing Imbruvica with Venclexta, the same regimen Evans has been taking. In a 2019 New England Journal of Medicine study, researchers reported that after 12 cycles of the combined medications, 88% of participants had no signs of cancer and 61% had remission with undetectable MRD. Researchers are also testing whether a three-drug therapy — Imbruvica, Venclexta and Gazyva &mdash; can further enhance responses.

“The downside is that many of these options are lifelong therapies, and every option introduces some level of toxicity,” Porter says. “That’s why ‘watch and wait’ is the preferred first-line strategy.” Because there are two types of CLL — fast growing and slow growing &mdash; watching closely is key. Both types of patients look alike in the clinic but have different blood, genetic and protein markers.

These markers help doctors identify not only which patients are most likely to progress but also which form of therapy might work best. “Some of these risk features change over time and in response to different agents,” Siddiqi says. “So before a patient starts treatment, switches drugs or goes on a new treatment, they should get prognostic markers checked again.”

When the waiting is over, the best initial treatment depends on the patient’s age, medical issues, reason for treatment and personal preferences. Some patients prefer an oral drug over infusions, others want to avoid lifelong therapy, and still others have a low tolerance for side effects. “So we can pick and choose and tailor treatments given these different options,” Allan says.

When Dolinger’s time arrived, she was working full time as a senior campaign manager for the Leukemia & Lymphoma Society. She started researching clinical trials and, with her doctor’s guidance, enrolled in a three-drug trial at The Ohio State University investigating Gazyva, Imbruvica and Venclexta. “It was one of the first clinical trials with a definitive stop point: 16 months,” Dolinger says. “I told the children I was going to get treatment in a different state, and I would come back and get better — and that’s what I really believed.”

But her foray into the treatment space was harrowing. Like most patients, Dolinger had an infusion reaction after her first dose of Gazyva. As cancerous cells die off in droves, patients often experience nausea, flu-like symptoms and tightness in the chest. The night of her first treatment, Dolinger woke up in her hotel room drenched in sweat, feverish and hurting all over. “I headed straight to the ER,” she says. “I had significant liver damage from the treatment, so they held me for observation. Then I became septic and one of my lungs collapsed.”

Dolinger recovered from the reaction, discovered her white blood cell count had dropped to the normal range and continued the trial. Six months after her first treatment, a bone marrow biopsy showed no evidence of disease in her system — 90% of her cells had been CLL positive at the start of the trial. She completed the 16-month trial. She maintained her MRD-negative status after the trial but experienced severe side effects ranging from infections to adrenal insufficiency that lasted a full year. “I’m just now starting to feel normal again,” she says.

Every patient with CLL has a different disease trajectory. Some patients watch and wait for decades. Others receive an initial treatment with chemotherapy or a targeted treatment and go into remission indefinitely. But the typical CLL course involves periods of disease-free remission interspersed with treatment.

No matter which camp patients fall into, data on long-term outcomes is lacking. “Some of these new agents like Imbruvica have only been around since 2010, so we don’t know what the effects of long-term, continual treatment are,” Allan says. “Plus, many of these therapies are used indefinitely, as long as they’re effective. At a cost of more than $100,000 each year, that’s tremendously expensive.”

Even when treatment works, patients often relapse. When that happens, chimeric antigen receptor (CAR)-T cell therapy may be an option. With this therapy, doctors remove a person’s

T cells, re-engineer them to recognize a CLL-specific protein called CD19 and place them back into the patient to help fight cancer cells. But unlike lifelong targeted therapy, CAR-T cell therapy is a one-time treatment — one that will likely continue to be tested and pushed farther up the treatment chain.

“There have been astounding results in highly refractory patients who have no other options. Some patients are many years out, with no detectable disease in their bodies,” Allan says. The biggest issue: Just 25% to 35% of patients achieve durable remissions, or no signs of cancer for a reasonable amount of time.

Even those figures are starting to shift. At City of Hope, Siddiqi’s team followed 23 patients with refractory CLL for a median of nine months and discovered that more than 80% of them achieved overall remission with about 45% complete remissions and about 70% had MRD-negative results in their blood and bone marrow biopsies within a month of receiving CAR-T cells. These results were durable at three-, six- and nine-month follow-ups and three patients have now been followed for more than one year without relapse. “Now we’re investigating whether combining CAR-T cell therapy with Imbruvica can produce even better responses,” Siddiqi says.

In the meantime, people like Dolinger and Evans feel a bit like they’re playing Russian roulette. Dolinger’s trial wrapped up in February 2018, and she has focused on bolstering her immune system ever since. Evans has been on the Imbruvica/Venclexta regimen for almost 14 months, and his blood markers remain in the normal range. “But I’m always looking one step ahead, just in case I relapse again,” he says.

Scientists increasingly believe there’s reason for hope. Although these newer, more powerful agents are not yet known to be curative, the number of patients with refractory CLL is beginning to shrink. “There are still patients that develop resistance even to the newer agents, but they make up a smaller and smaller percentage of CLL patients,” Porter says.

With the introduction of new and better therapies, CLL is slowly transforming from a chronic disease requiring lifelong treatment to a cancer that doctors can eradicate. “We don’t know if we’re there yet, but I do think we can start thinking about potentially curing it,” Porter says.

People like Evans and Dolinger are championing the cause, participating in clinical trials, increasing awareness about novel therapeutics and even raising money to advance research toward a cure. Since starting treatment in 2008, Evans has been helping patients with newly diagnosed and refractory disease in the support group setting. Through his work with the CLL Society, Evans helped establish more than 31 CLL-specific support group chapters across the U.S. and two in Canada. “Training support group facilitators has become like a second career for me,” he says.

Through her work at the Leukemia & Lymphoma Society, Dolinger, too, has a career in the CLL world. For her, it’s about taking action rather than sitting on the sidelines — and it has become a family affair. “My sons have collectively raised $60,000 for CLL research,” she says. “It’s their way of doing something to help Mom.”

In what was once an incurable cancer &mdash; chronic lymphocytic leukemia &mdash; scientists are seeing remissions that could last for decades.

A Fortunate Turn of Events in Chronic Lymphocytic Leukemia

In the seven years since Jeremy Sheldon received a chronic lymphocytic leukemia diagnosis, he has had his fair share of blood transfusions. Six months of chemotherapy initially cleared his disease, but then he developed shingles in 2017 and needed eight infusions of gamma globulin from donated plasma to replenish his declining levels of immunoglobulins — antibody proteins that are essential for maintaining immune function.

Then, in 2018, he developed myelodysplastic syndrome, another type of blood cancer that disrupts the production of blood cells, and needed a stem cell transplant. After that, he received seven transfusions of red blood cells, plus a few infusions of platelets.

“It can be scary to see this big bag of blood coming in,” says Sheldon, 55, who lives in Phoenix. He experienced some shaking and chills from the gamma globulin infusions at first, he says, but was ultimately able to tolerate most of the blood products he received and now feels confident he’s on the road to recovery. “Every day is a good day,” he says.

Blood transfusions are routine in the treatment of patients with blood cancers. In fact, an estimated 15% of the 14 million or so blood units collected in the United States every year go to hematology and oncology patients. Although the safety of donated blood was a concern in the past, improvements in both the collection and testing of blood and its administration to patients have lessened the risks considerably. Among all patients treated with blood transfusions in 2017, the most recent year reported by the Food and Drug Administration (FDA), there were 44 deaths, down from 60 the previous year.

“The blood supply has never been safer,” says Dr. David Chow, medical director of the blood bank at Hackensack Meridian Health in New Jersey.

There are several types of transfusions that patients with blood cancers may need during the course of treatment. Patients who, like Sheldon, receive stem cell transplants are first given high doses of chemotherapy to deplete their own blood cells. That’s why after the transplant, they need transfusions of red blood cells, which carry oxygen; white blood cells to fight infections; and platelets to stem bleeding.

Even patients who don’t need stem cell transplants can develop anemia during chemotherapy treatment. Oncologists can’t prescribe drugs for anemia, such as Epogen (epoetin alfa), to patients with blood cancers because of the nature of those diseases. “Epogen can stimulate the bone marrow to produce more red blood cells, but in patients with leukemia and lymphoma, the bone marrow is not functioning,” says Dr. Qun Lu, a pathologist at Mayo Clinic in Phoenix.

For those patients, blood transfusions are considered supportive care, she adds: Even though they don’t treat the cancer itself, they relieve symptoms of anemia, like extreme exhaustion and shortness of breath, and therefore are essential for helping patients fight the disease.

Some patients with blood cancers also may need infusions of plasma and/or albumin, both of which can be used to treat liver malfunctions that can occur during the course of therapy. Plasma contains clotting factors that help contain or prevent bleeding. Cryoprecipitate is derived from plasma and has specific coagulation factors for patients with specific deficiencies. Albumin, a protein that’s abundant in blood and produced by the liver, is decreased in patients with liver disease or malnutrition and sometimes needs replacement.

Transfusions of red blood cells generally take between two and four hours; plasma and platelet transfusions are generally faster. During the procedure, patients are monitored frequently to make sure there are no changes in vital signs such as temperature, heart rate and blood pressure and that no adverse reactions are occurring.

Some patients who receive transfusions develop reactions that manifest with flu-like symptoms, including chills, nausea and back pain, which can be treated by lowering the rate at which the blood is given and using over-the-counter remedies like Tylenol. However, sometimes these reactions can be severe and even fatal, so all transfusions require close monitoring and preparedness to intervene.

When patients receive regular blood transfusions, they can develop a condition called iron overload. Red blood cells contain iron, so each time a patient receives a red blood cell transfusion they are putting more iron into their body. Since this can be different for each person, a doctor will decide if a patient has iron overload and if treatment, such as iron chelation therapy, is needed. In addition, patients can develop antibodies against substances known as antigens on red blood cells or platelets from donors. That can make it more difficult to find compatible blood. To locate the proper match, before a transfusion, the patient’s blood is tested for both its aBO and Rh type and for the possible presence of antibodies.

Still, it’s important for patients who have developed antibodies against donated blood to alert their physicians of their history before every transfusion — particularly if they’ve moved to a new treatment center. “Once these antibodies form, they’re considered lifelong; therefore, the patient is always at risk of having a reaction,” says Dr. Kaaron Benson, director of the blood bank at Moffitt Cancer Center in Tampa, Florida. “We may not know about it at our hospital if it happened somewhere else.”

Some patients may need to have their blood specifically tested before they even begin their cancer treatments. For example, patients with multiple myeloma, a blood cancer that forms in a plasma cell, are often prescribed Darzalex (daratumumab), a drug that’s effective at treating the cancer but also interferes with testing for blood compatibility. Those patients need to undergo special blood typing before starting Darzalex, in case they need donated blood down the road.

All donated blood components are carefully screened to ensure that donors do not unknowingly pass along viruses like HIV, hepatitis or West Nile. All platelet donations are also tested for bacterial contamination, though that testing isn’t fail-safe. That’s because unlike red blood cell units, which can be refrigerated, platelets are stored at room temperature for up to five days. “When you are storing platelets at room temperature, small amounts of bacteria can multiply into the millions and cause a severe infection,” Lu says.

Last December, a 23-year-old woman being treated for acute lymphoblastic leukemia at The University of Texas MD Anderson Cancer Center in Houston died after receiving platelets that were contaminated with bacteria. The incident prompted the federal Centers for Medicare & Medicaid Services (CMS) to review safety procedures at the hospital. The agency reported that it found a number of safety shortcomings — for example, nurses were not regularly monitoring the patient’s vital signs during the transfusion, nor had they done so for 18 other patients whose records the agency reviewed.

A spokesperson for MD Anderson said the hospital system transfuses 200,000 blood products each year, 75% of which are used in patients with hematological cancers. “We have policies and procedures in place to protect our patients. However, in rare instances, severe reactions occur,” the spokesperson said.

Since the CMS investigation, MD Anderson has reviewed all its safety procedures and retrained the nursing staff to practice more stringent patient monitoring. The hospital’s lab is also establishing a “hemovigilance unit for real-time monitoring of patients at risk for a transfusion reaction,” the spokesperson said.

Some companies are developing technologies to make the blood supply even safer. In 2014, California-based Cerus won FDA approval for its product, Intercept, which combines a chemical compound with UVA to block the ability of viruses, bacteria or parasites in donated blood to replicate. The company has shown that the technology can inactivate more than 25 different pathogens.

More than 40 blood banks have started using Intercept-screened platelets routinely, including Mayo Clinic, even though it makes obtaining blood more costly, Lu says. “It can be hundreds of thousands of dollars more in increased costs per year,” she says. “More than half the platelets we use are pathogen reduced, but we’d like it to be 100%.” Colorado-based Terumo BCT is developing Mirasol, a system that combines vitamin B2 (riboflavin) with ultraviolet light to inactivate pathogens and stray white blood cells in platelets, which can also cause reactions in some patients.

The product is approved in several countries and is being tested in clinical trials in the U.S.

Terumo hopes to apply the same technology to red blood cells, and in 2018, it started U.S. trials in patients to prove it is safe and effective.

Cerus is also working on moving its technology into the treatment of red blood cells. Many physicians welcome the idea of pretreating more than just platelets for pathogen reduction, because even though red blood cells can be refrigerated, there’s a tiny chance that some bacteria can grow in cold temperatures.

“The development process is behind for red blood cells,” says Dr. Jeffrey McCullough, professor emeritus of laboratory medicine and pathology at the University of Minnesota in Minneapolis and a consultant to both Cerus and Terumo. “But this research is moving ahead, and we expect over the next several years this will become the new paradigm for blood safety.”

To lower the risks, some oncologists infuse just one unit of blood, then wait to see if the patient needs more, rather than ordering two or more units upfront. “We used to think a two unit minimum of red blood cells for adults was important,” Benson says. “Now we have good studies showing we can lower our blood transfusion thresholds and reduce the number of units per transfusion episode, and that further reduces the risk to patients.”

Another potential development on the horizon involves freeze-dried plasma, which was used in the military in World War II but fell out of favor because it raised the risk of transmitting hepatitis B. Now that it’s possible to test donated blood for viruses, freeze-drying plasma is making a comeback. In October 2018, the FDA approved a freeze-dried plasma product made in France for emergency use by the U.S. military.

Clinical trials are underway to determine whether freeze- dried plasma is safe to use in patients with a wide range of disorders, including cancer. The product is designed to be stored up to at least one year and then mixed with sterile water for any patient who needs a transfusion. “This is something we could potentially use, particularly in the emergency setting or in remote areas,” where patients sometimes face a delay waiting for plasma, which is usually frozen, to be prepared, Benson says.

The holy grail of blood transfusion is so-called artificial blood, a blood substitute, which could provide an alternative method for carrying oxygen through the body. Several companies and academic researchers have tried to develop artificial blood but had limited success. One product, Hemopure, was approved in South Africa but rejected for approval in the U.S. after advisers to the agency suggested further studies needed to be done to prove the product’s safety and efficacy. Overall, the human trials that have been done with artificial blood have reported an increased risk of heart attack and death.

So, for the foreseeable future, donated blood is the only option for patients with cancer who need transfusions.

For that reason, oncologists often urge family members and friends of patients to donate blood to help replenish the community supply.

Still, Chow says, it’s not necessary for patients to receive donated blood from someone they know. “Patients often want their relatives to donate blood because they believe that blood is ‘cleaner,’ but that’s not the case. All blood donors are rigorously tested,” Chow says. “We want everyone to go out and donate blood. Even if the blood doesn’t help that particular patient, it will help someone.”

When blood is not needed urgently but might be anticipated after a major surgery, patients who start with normal blood counts can donate their own blood well in advance so that there is time for them to generate more blood, then have it stored and transfused back if needed because of blood loss after surgery.

For Sheldon, who still receives chemotherapy once a month, blood transfusions have become so routine that he no longer worries about the process. His advice to other patients? “Don’t be scared,” he says. “The doctors know what they’re doing, and they’re always testing, so you can have confidence that the blood they’re bringing in is the right match.” Nevertheless, it is important for all patients receiving blood products to be aware of the risks and measures taken to reduce them.

New technologies boost safety for patients with cancer who need donated blood.

Transforming Blood Transfusions in Cancer Treatment

Having a parent, sibling or child with blood cancer raises an individual’s risk of also receiving a blood cancer diagnosis, according to the results of a large European study.

Age at diagnosis and the number of affected first-degree relatives also are major risk factors, according to study findings published in 

Researchers analyzed data from the Swedish Family-Cancer Database, which included 153,115 patients with a blood cancer and 391,131 first-degree relatives. The highest relative risks were seen in certain Hodgkin lymphoma (HL) subtypes, lymphoplasmacytic lymphoma and mantle cell lymphoma. Elevated risk was also observed for myelodysplastic syndrome and polycythemia vera and essential thrombocythemia, two types of myeloproliferative neoplasms.

The increased risk of chronic lymphocytic leukemia (CLL) depended on the age of the affected relative and the number of first-degree relatives, the researchers found. In cases of non-Hodgkin lymphoma, HL and CLL, individuals who had a sibling with the disease were at higher risk.

The familial risk was more pronounced when relatives were diagnosed at younger ages, according to study findings.

Based on the data, lead author Dr. Amit Sud from the Institute of Cancer Research in London hopes to provide information for guidelines on genetic testing and screening. “The results should also encourage conversations among families, clinicians and patients about familial risk,” Sud said in a press release.

Hematology 2 | CURE

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