A Hopeful Time for Kids With Sickle Cell Disease: A Q&A With Dr. M.A. Bender and Alix Dassler, ARNP
March 2, 2022
Dr. M.A. Bender, MD-PhD (goes by Bender) is director of the Sickle Cell Program at Odessa Brown Children’s Clinic (OBCC). Alix Dassler, ARNP, is a board-certified family nurse practitioner with Seattle Children’s Sickle Cell Program and Cancer and Blood Disorders Center.
Sickle Cell Facts
- Approximately 100,000 people in the United States have sickle cell disease.
- Most people in the United States with sickle cell disease are of African or Hispanic heritage. But the disease can affect anyone, especially people of southern European, Middle Eastern or Asian Indian heritage.
- SCD occurs in 1 in 365 Black or African-American births in the United States.
- SCD occurs in about 1 out of every 16,300 Hispanic-American births.
- About 1 in 13 Black or African-American babies in the United States is born with sickle cell trait.
Q: Has treatment for sickle cell improved much in recent years?
Yes, it’s a really hopeful time for sickle cell. There’s a common misperception that not much is available for sickle cell. But interventions and medications have improved dramatically over the last 20 years. We used to have just one drug to offer our pediatric patients. Now we have four. They do everything from preventing pain and boosting hemoglobin levels to preventing some of the more severe complications of sickle cell. These newer treatment options are better than ever at helping our patients live full, healthy, active lives.
Q: Is sickle cell curable?
Yes, for some patients. One way to cure sickle cell is with a bone marrow transplant. We also have genetic therapies that involve removing some of a patient’s blood stem cells and modify them so sickling does not occur. They can even correct the defect. However, it’s not without risks and not the right choice for every child.
Q: Are all kids candidates for curative therapies?
If they have a brother or sister with an exact bone marrow match (which is a 1 in 4 chance) then we recommend the family consider a transplant because the results are so good. Virtually all insurance covers it because the risks are low and the success rate is so high.
A bone marrow match with a parent is only half a match. That’s a much harder transplant and not as likely to succeed, but results keep improving and there are a lot more patients in this category.
Genetic therapies do not require any matching, as they use the child’s own marrow stem cells. Thus, eventually, in theory, every child could be cured.
It can be a very tough decision for families because the risks that come with trying to cure the disease are much higher than continuing to use the available treatment options, which are good. Families can also choose to wait and see if better therapies or safer cures come down the pipeline. It’s very personal. We try to give them the best information possible so they can make a choice that’s right for them. We will hold long conferences for families about transplants. Their responses vary widely. Some are very eager to try a transplant, and others think it’s crazy to even suggest a treatment that could lead to their child’s death.
Q: What is the role of the PCP in caring for a child with sickle cell?
Ideally our patients will have a PCP to manage their everyday health concerns like rashes, acne, etc., and do things like well-child checks and immunizations. For medically complex kids, we’ve found it can be hard to convince the family to keep up with their PCP if they are already seeing our team and subspecialists (like pulmonary/neurology/blood transfusion). We work hard to convince families to keep receiving primary care. Both Alix and Patrice Laster, ARNP, DNP, on our team can also provide expert primary care.
Q: What should providers with sickle cell patients be on the lookout for?
Great question. It’s really important to keep an eye out for several things:
- Any pain, fever of 101.6 degrees or higher or breathing problems need immediate attention. We have to take these symptoms seriously every time, in every setting — from the PCP office, to urgent care, to the Emergency Department — and never dismiss them. If a child with sickle cell comes to a provider with any of these symptoms, we want that provider to page our team and talk to us in real time. Fever and breathing problems can be particularly deceptive, as children may look well, but decline and die rapidly.
- The risk of stroke is very high in sickle cell. Any concern of a central nervous system problem requires immediate evaluation and intervention.
- COVID-19 vaccination is critical for sickle cell patients. Sickle cell disease puts kids at higher risk for more severe COVID-19. Likewise, COVID-19 can exacerbate sickle cell and can be particularly hard on the lungs. Patients with sickle cell qualify for the three-dose series of COVID-19 vaccine vs. the two doses plus booster. They may also benefit from an additional fourth dose. There’s also a lower threshold to give them some of the specific treatments for COVID-19 that other kids might not get, such as oral antivirals.
- Surgery and anesthesia bring extra risks. Our team should be involved any time a patient with sickle cell disease will be sedated, whether it’s a major surgery or ENT procedure, complex dental visit or something else. Please contact us by phone at 206-987-7263 or email us at sicklecellclinic@seattlechildrens.org; it doesn’t need to be an emergency page.
Q: Can PCPs help manage the sickle cell aspects of care?
Absolutely! It is ideal to team up with the family’s trusted and local PCP. We will fully support and partner with any PCP who wants to coordinate sickle cell care for their patient in the primary setting! Get in touch with us any time to discuss your patient.
Q: How Is Seattle Children’s Sickle Cell Program changing?
We are significantly expanding and enriching our services to support patients with sickle cell and their families. Our team, located at Odessa Brown Children’s Clinic (OBCC), includes a dedicated psychologist, social worker and registered dietitian. We work with schools, camps and community groups to support our patients in various ways, including making sure they have access to rent and grocery assistance and other community programs as needed. We have done this for years, but we’ll be able to do it on a larger scale going forward. This approach matches OBCC’s vision for offering families truly integrated care, and it’s part of our Sickle Cell Program’s vision of being a world-class center of excellence.
We are not anticipating an influx of new patients; expanding our program is about better serving the patients we already have. We follow about 140 patients currently.
Q: Why is the sickle cell program located at OBCC?
OBCC (both locations, Yesler and Othello) is more central and accessible to the patient population we serve. OBCC Othello (opening this month) in particular is a robust hub for transit and community services and is closer to where many of our families have moved in recent years as gentrification uprooted Central District residents.
Sickle cell patients still visit the main hospital for certain treatments or services, including transfusions and surgery. We maintain a strong collaboration with Seattle Children’s Cancer and Blood Disorders Center (CBDC), which encompasses hematology, bone marrow transplant and cellular therapies. CBDC is consistently ranked among the nation’s top pediatric cancer programs.
Q: Do you offer care via telehealth?
Absolutely. It’s a very convenient mode of care for our families and is firmly built into our practice.
Q: How do you stay coordinated with PCPs?
Since we adopted EPIC in 2020, communication between PCPs and our team is better than ever because we can easily copy them on all our notes.
PCPs are always welcome to contact the Sickle Cell Program at 206-987-7263 or email us at sicklecellclinic@seattlechildrens.org. For urgent matters, call the Provider-to-Provider Line at 206-987-7777 and ask to speak to the sickle cell team (not hematology in general). You’ll get one of us — Alix, Patrice or Bender — on the phone.
Q: Do kids with sickle cell trait (but not the disease) need to be seen?
Initially, yes, for education and genetic counseling. After that a healthy child with the sickle cell trait (one sickle gene and one normal one) doesn’t need to be seen by us. One in 13 children of African descent in the United States have the trait. The exception is the rare child with the trait who is having red flag problems like pain or respiratory problems. We definitely want to see those kids.
Q: How has the pandemic changed care for patients with sickle cell?
We’re not sure if it’s pandemic-related, but we keep close tabs on our patients, and there are a handful we haven’t seen recently and can’t track down. We don’t know why; if they’ve moved or just stopped getting care with us and/or other providers. We want to get them back in.
Q: Do you recommend genetic counseling?
Yes, we recommend genetic counseling for the parents of anyone with sickle cell and for individuals who have the sickle cell trait and are planning a family. Because genetic counseling for sickle cell can be a lot more complex than many are aware of, it is key to be seen by someone with expertise in sickle cell. Our sickle cell program doesn’t have capacity to do genetic counseling at OBCC. Those referrals should go directly to Hematology/Oncology at the main campus and will typically be seen within several weeks (do not refer to Genetic Counseling, where the wait will be much longer). Patients with Kaiser insurance should be directed to Kaiser.
Q: Do people who have the sickle trait always know it?
Many don’t. In Washington state, every newborn has been tested since 1991, meaning the first kids are now around 30 years old. Their provider can contact the Washington Department of Health (DOH) at 206-418-5410 to request their newborn screening results. DOH will fax the provider a copy of the report to pass along to the patient. Unfortunately, the system is better set up to bring people in and retest them (poke and charge) rather than just check the existing records.
We recommend that everyone check their DOH record to see if they have sickle cell disease or trait (trait is more commonly forgotten and missed over time because it doesn’t cause symptoms). The risk for people of European descent is lower, but just because you are white in appearance doesn’t mean your ancestors weren’t of color.
Q: What if someone wasn’t screened at birth?
We suggest all immigrants be screened for hemoglobin disorders. However, the common screening test for sickle cell is imperfect, the definitive screening test is expensive and the names of the tests vary by lab and hospital. We — Alix, Patrice and Bender — are happy to take calls from PCPs to review their options and help determine the testing that works best for them and their patient.