When patients and families with sickle cell disease (SCD) call Seattle Children’s to schedule appointments, they are greeted by the friendly voice of Enjoli Harris, who is a skilled member of the SCD patient scheduling team and a “sickle cell mom.”
Harris’ youngest child, Nehemiah, was diagnosed with SCD at birth almost 12 years ago and has been receiving care at Seattle Children’s, including at the Odessa Brown Children’s Clinic (OBCC), ever since. The frequent visits have helped Harris not only empathize with the concerns of families, but also better navigate their scheduling needs.
SCD is a group of blood conditions that affect hemoglobin, the part of red blood cells that delivers oxygen to cells in the body. In the United States, approximately 100,000 Americans are affected by SCD, most of whom are of African or Hispanic heritage, however the disease can affect anyone, especially people of southern European, Middle Eastern or Asian Indian heritage.
Children with SCD go to a lot of appointments. At any given time, Harris juggles the schedules of more than 140 SCD families who need to arrange care visits for transcranial doppler ultrasounds, blood draws, pulmonary function tests, ophthalmology exams, nutritional support, physical therapy, speech therapy, psychological visits, cognitive screenings or pain management. The SCD team’s goal is to coordinate multiple appointments into one visit, so families don’t have to make numerous trips or take extra time off work.
Adding Harris to the SCD team is an important part of Seattle Children’s plan to expand its inpatient and outpatient care for patients with SCD in an integrated manner and across a child’s lifespan from birth to adulthood. Part of the plan involves increasing the number and diversity of SCD care team members, as well as the number of days and hours they are available to SCD patients. The expanded team will include providers from both the Odessa Brown Children’s Clinic (OBCC) and within the internationally recognized Cancer and Blood Disorders Center (CBDC) who have direct experience working with kids with SCD, as well as a wider group of key providers at Seattle Children’s.
Improvements in pain management
One of the most pressing issues is bringing new care team members up to speed on the disease’s unique and complex issues, such as the acute pain that many people with SCD suffer from.
“Addressing the pain of SCD is a big focus of our work,” said Dr. Katie Carlberg, a pediatric hematologist and interim director of Seattle Children’s Sickle Cell Disease Program. “Sickle cell pain is different from many other kinds of pain and if caregivers don’t have experience with the disease, they may not understand the urgency of the situation. There are no biomarkers or objective data that care team members can look at and say, yes, I see that your pain is severe and we need to do something about it quickly.”
The SCD team, along with colleagues from the pain management and emergency medicine teams, recently developed and introduced a pain management protocol for providers in the Emergency Department (ED) to quickly address and escalate pain management appropriately for patients with SCD. Since SCD pain differs in each child, one of the key goals of the protocol is to have a pain management care plan in place for all SCD patients. The pain management protocol also outlines a standard for shared decision-making with the patient and their family on how to administer pain relief – orally, via an IV or via intranasal route.
The goal is that each patient’s pain plan will be routinely updated and placed in the electronic medical records system where it will be visible and accessible by providers at Seattle Children’s and elsewhere.
Expanding patient navigation services as well as psychosocial and neurological support
Dr. Carlberg is also excited about the creative ongoing work that seeks to utilize the myriad of resources that Seattle Children’s has to offer to serve our patients more seamlessly across clinical settings. These include social work, patient navigation, the Center for Diversity and Health Equity, child life specialists, Patient and Family Relations, behavioral support and more.
Patient navigation has historically been a tremendous resource to the patients cared for at Seattle Children’s. “Recently, this program has grown and with this new capacity, we see opportunity,” said Dr. Carlberg. “We hope to leverage their expertise and experience as we seek to understand and change the culture of our system to help create more positive experiences for our families within our institution.”
Dr. Chris McCormick is another recent addition to the SCD team. A clinical psychologist, Dr. McCormick joined Seattle Children’s from Children’s Minnesota, where he became passionate about helping kids with SCD manage the sometimes overwhelming symptoms and issues of the disease. Dr. McCormick works with children, teens and young adults who are experiencing sadness, anxiety and anger. He teaches them basic skills to manage the pain that is such a large part of SCD, and helps them deal with needle phobias, pills that are too big to swallow or medicine that tastes bad.
“This disease is challenging, and there is data that suggests that individuals with sickle cell disease along with a diagnosed mental health concern tend to have longer and more frequent hospitalizations. Being able to address those concerns at various life points helps promote healthy development and wellbeing at every stage of a child’s life,” said Dr. McCormick.
Later this year, Dr. McCormick will offer neurocognitive testing in partnership with Seattle Children’s Neuropsychology Department to help recognize when a child has potential deficits in cognitive skill areas. “The earlier we can recognize those things, the earlier we can start to put interventions in place and begin to build skills that have important implications for social, emotional, behavioral and academic success.”
Dr. McCormick is delighted to have specific dedicated time to work with families affected by SCD. “I am really fortunate to play a small part in their overall care and development. It’s work that I really enjoy and love.”
Interim director Dr. Carlberg is excited about expanding the team with knowledgeable and dedicated people like Harris and Dr. McCormick, as well as community care coordinator Chyna Lockhart and care coordinator Hoyan Wong, RN. Seattle Children’s also plans to continue expanding its social work team to ensure patients with SCD have access to social work services 24/7. As Dr. Carlberg said, “the more access our patients have to care, the better.”
Jacob McCullough has also recently joined the team as program manager of the SCD Program. He is responsible for the operations of the program, collaborates to eliminate barriers of care, and ensures short term and long-term goals are met. He said, “as an Indigenous Person, I have personally experienced the inequity that underserved populations have endured in healthcare and research institutions. I have since made it my mission to create opportunity for marginalized populations to receive individualized healthcare, and those living with sickle cell disease have been underserved and neglected for far too long. I’m grateful to be given a chance to make space at the table for our sickle cell warriors and their families, and prioritizing their care on an institutional level at Seattle Children’s.”
Continuing work to improve care for patients with SCD
A variety of other improvements are in progress to improve care for patients with SCD. Of note, the nursing team is receiving specialized training in equity, diversity, inclusion and anti-racism to best serve the unique needs of patients with SCD and their families. This training requirement correlates to a commitment made by Seattle Children’s in its Health Equity and Anti-Racism Action Plan to invest in mandatory, recurring organization-wide training programs focused on equity, diversity, inclusion and anti-racism.
OBCC has added new services to its patient care offerings including ultrasound and x-rays. This means patients with SCD who may need these specialized services as a part of their routine care can get everything in one place instead of traveling to a separate facility.
The team also aspires to offer a broader range of clinical trial opportunities to patients with SCD and families in the near future.
“I want parents to know that the changes being made are very much parent and patient-focused,” said Harris. “Being here, I see an enormous level of understanding and empathy and I really want our community, the sickle cell community, to experience the best of the best. As a parent, this is what I hope for.”
Learn more about SCD in these videos:
For all questions related to sickle cell disease
Call 206-987-2106 during business hours (8 a.m. to 5 p.m., Monday through Friday) and ask for “the sickle cell team.”
Call 206-987-2000 outside of business hours (on weekends, holidays and between 5 p.m. and 8 a.m. on weekdays). Ask for “the sickle cell team.”
For health concerns not related to sickle cell disease
Call your child’s primary care provider. If your child’s primary care provider is through OBCC, call the OBCC nurse line at 206-987-7291.
If you need an interpreter
Call 1-866-583-1527, choose your language and ask to speak to the department caring for your child at Seattle Children’s. This may be the sickle cell team, OBCC, the Cancer and Blood Disorders Center or another team.