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Overcoming Confusion and Misinformation About a Life-Saving Drug

September 16, 2013
By Kristina Grifantini

Join NICHQ and organizations around the country in celebrating National Sickle Cell Awareness Month in September. We invite you to explore our resources, stories, videos and other materials related to improving care for sickle cell disease, as well as celebration activities our project teams have planned for this month.

For the past seven years, Patricia Kavanagh, MD, who specializes in pediatric care at Boston Medical Center, participated in a camp that catered to children with sickle cell disease and other serious diseases. Initially, children with the genetic blood disorder were physically smaller and had less energy; Kavanagh constantly mistook children for being several years younger than they were. In the last few years, she has noticed a drastic difference: children are appropriate sizes and have more energy. And she and her partners in the Boston Medical Center pediatric hematology team have seen fewer hospital stays and visits from children with sickle cell disease.

One of the reasons for the notable improvement in patients is the more widespread use of a medication called hydroxyurea, originally used as a chemotherapy agent but approved by the FDA to treat adults with sickle cell disease. In sickle cell disease, blood cells morph under certain stressors that make them clog arteries, resulting in debilitating pain and eventually tissue death. Hydroxyurea prompts the body to produce more of a certain blood protein that lessens these pain “crises” and other symptoms in at least two types of the disease.

“Children grow better on hydroxyurea. You can physically see it in the way they look,” says Kavanagh. “It’s not a cure-all; it doesn’t remove all pain, but it’s far reduced. Patients say they feel different on it.”

A micrograph of red blood cells (erythrocytes)
A micrograph of healthy erythrocytes (red blood cells). [Source]

Aside from hydroxyurea, patients may rely on pain medication, transfusions and emergency room visits (where they may or may not get the appropriate care) to manage their disease. But nothing yet has shown to be as effective as hydroxyurea: an extensive, multi-year clinical trial known as “BABY HUG” showed statistically significant lower rates of pain, acute chest syndrome, hospitalizations and transfusions for young children on the drug. Additionally, a recent study in Pediatrics — of which NICHQ’s Suzette Oyeku, MD, MPH, is co-author — shows significant healthcare cost savings associated with use of the drug.

“Hydroxyurea, in addition to other interventions, has the capability of turning sickle cell disease — which frequently caused loss of life at a young age — to a chronic, manageable condition,” says Kavanagh.

Though hydroxyurea can dramatically improve the quality of life for many patients, concerns about potential effects on future fertility, as well as confusion and misinformation about the drug, often prevents both providers and patients from exploring its potential benefits. Teams in NICHQ’s Working to Improve Sickle Cell Healthcare (WISCH) program, funded by the Health Resources and Services Administration (HRSA), are hoping to overcome these barriers through increased tracking and education.

Addressing Misconceptions

Through grant work supported by WISCH and HRSA, the Boston Medical Center is one of several clinics establishing a registry based on electronic medical records to better document and track who is on hydroxyurea, effects, and if conversations around the drug and its side effects have taken place. This helps to ensure that no patients fall through the cracks.

Run chart documenting hydroxyurea use among pediatric SCD patients at Boston Medical Center
Run chart documenting hydroxyurea use among pediatric SCD patients at Boston Medical Center from July 2012 through June 2013.


​The Boston Medical Center already has found several patients that have not had conversations with providers on hydroxyurea, though its guidelines aim to have discussions with families before children have their second birthday. Currently, of its 103 pediatric patients eligible to be on the drug, 73 percent are on it, and the benefits are showing. Kavanagh says they’ve seen “dramatic” drops in the number of visits to the emergency room and hospital stays.

“Not enough children with sickle cell are offered hydroxyurea, and when they are, half of parents are reluctant to initiate hydroxyurea due to feelings of uncertainty and concerns about efficacy, safety and side effects,” says Lori Crosby, PsyD, and researcher on sickle cell disease at Cincinnati Children’s Hospital Medical Center. Through WISCH, the hospital is currently working on conducting a formal assessment and gathering feedback on materials (like a visit decision aid) to spark conversations between patients, parents and providers about hydroxyurea.

“Some adult patients don’t want to take the drug because they heard rumors it causes cancer,” adds Alice Cohen, MD, director of the Division of Hematology and Oncology at the Newark Beth Israel Medical Center. “Another obstacle is that doctors aren’t spending enough time discussing hydroxyurea in appointments, because visits typically focus on pain management rather than chronic care.”

To help spur more provider-patient conversations and dispel myths, the Newark Beth Israel Medical Center—which treats 300 adults and 400 children with sickle cell disease—created a video that followed their own patients’ experiences with hydroxyurea (see below). The center also introduced hydroxyurea into a provider checklist for sickle cell visits that highlights other comprehensive issues to check, such as vaccinations and eye exams. Finally, the center also developed a database of the center’s patients, documenting what type of sickle cell disease patients have, if they were or weren’t taking hydroxyurea, white blood cell count, toxicity, and number of hospital admissions, ED visits, and transfusions.

“We started monitoring patients to give them an individualized approach and make sure everyone is on an appropriate dose,” says Haywood Barnes, a community healthcare worker that works closely with Cohen and others on quality improvement in sickle cell treatment.


Watch patients discuss why they began taking hydroxyurea and what the experience was like for them. Courtesy of Newark Beth Israel Medical Center. [Source]


The video and database have been “big” for the team, says Barnes. The video created not only a tool to educate patients but also helped patients talk to providers and feel more comfortable by discussing the video. Additionally, Barnes has organized in-person community activities like bowling, skating, horse riding and spa days to let patients meet, discuss and ask questions about hydroxyurea and other areas of sickle cell disease support.

“Since we’ve begun an educational approach, we have absolutely seen an increase in how many patients take hydroxyurea,” says Barnes. “Before we were at 30 percent and now we’re at about 58 percent of adult sickle cell patients taking hydroxyurea.”

The teams say that next steps include education of hydroxyurea on a larger scale as well as helping to improve adherence to taking medication, which is a common problem in managing many chronic illnesses. 
 
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