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Quality Is the Key: An Interview With Drs. Obiageli Nnodu and Wally Smith on ASH’s Sickle Cell Disease Programs

December 12, 2022

The ASH Sickle Cell Disease Initiative is a transformative, multifaceted, patient-centric initiative to improve outcomes for individuals with the disease, both in the United States and globally. As part of this ongoing effort, ASH has brought together stakeholders in the public and private sectors, all committed to significantly improving the state of sickle cell disease (SCD) care worldwide. In addition to the Sickle Cell Disease Coalition and ASH Research Collaborative, ASH’s guidelines, education and training efforts, policy and research priorities, and global initiatives have positioned the Society as a leader in improving access to quality care.

ASH News Daily spoke with two ASH members who have been longtime champions for quality SCD care and who have helped to spearhead some of these efforts over the years — Dr. Obiageli E. Nnodu and Dr. Wally Smith. Dr. Nnodu is a professor of hematology and blood transfusion and director of the Centre of Excellence for Sickle Cell Disease Research and Training at the University of Abuja, and a professor in the Department of Family and Community Medicine at the University of Arizona College of Medicine. Here, she provides an update on the ASH-supported Consortium on Newborn Screening in Africa (CONSA). Dr. Smith is the Florence Neal Cooper Smith Professor of Sickle Cell Disease at Virginia Commonwealth University (VCU) and also serves as associate chair of research for VCU’s Division of General Internal Medicine. In this interview, he offers an overview of current U.S.-based initiatives, including the SCD Centers Workshop, the Sickle Cell Disease Comprehensive Care Act, and more.

How have the newborn screenings in Nigeria gone so far?

Dr. Nnodu: CONSA was launched on October 26, 2020, [and] as you are aware, the program was set up to demonstrate the effectiveness of newborn screening (NBS) and early therapeutic interventions in reducing morbidity and mortality in early childhood for infants with SCD. So far, we have successfully screened a total of 8,144 newborns from 20 sites in four local government areas across the Federal Capital Territory (FCT). Of this number, 83 patients with SCD have been identified, 39 have been enrolled in the clinic, 39 have had their first clinical appointment, 31 have had their second clinical appointment, 27 have had their third clinical appointment, 10 have had their fourth clinical appointment, three have had their fifth and sixth clinical appointments, and two have had their seventh clinical appointment. All the enrolled patients are on medications.

Collaborating with the FCT Primary Health Care Board on the newborn screening program has promoted the expansion, coverage, and acceptability at the primary health care centers (PHCs) across the FCT. The immunization program at these centers has provided the leverage to integrate the NBS program seamlessly. More centers in the FCT have been approached to initiate the NBS program as the project expands to cover areas that are not currently screening newborns at the immunization centers.

While we are excited that the program has recorded some milestones, there are some challenges:

First, with respect to logistics, the locations of the PHCs are spread across the FCT, which requires some funds to initiate/ train, collect samples on weekly basis, and do routine quality checks and supervision for sites and site members. Currently, we are spending more than 110,000 naira each week to sustain this program.

Additionally, some of the workers in PHCs are volunteers who expect to be remunerated for their effort in screening newborns. Some sites have complained about the additional [task involved] without any form of compensation. This has resulted in low motivation and unwillingness to continue the program at these sites.

Lastly, there is also poor awareness and acceptability. We conduct health talks before the commencement of screening on immunization days; however, some parents choose not to participate. Some of the reasons for this refusal include poor awareness about SCD, lack of education, and trust issues, among others. This concern affects the number of newborns screened at each site. We are, however, planning a sensitization campaign on newborn screening in partnership with Rotary International and Novartis. We are also carrying out community entry and sensitization for [site] leaders to provide additional support for the program.

Please discuss how you have supported other countries’ programs in implementing NBS and SCD care.

Dr. Nnodu: Members of the steering committee worked together for two years before CONSA was launched. Being the third country admitted into the consortium meant that we were able to encourage national coordinators in their journey to site readiness. The work with the World Health Organization Regional Office for Africa also helped to set the stage regarding the need to establish NBS programs in several countries. When working as a group, there is opportunity for shared learning as we report on our effort to meet targets; for the stakeholders we mobilized at the institutional, local government, state, and federal level for the launch; and for the way we involve the media in our workshops. We work with the media to not only report our activities, but also to educate them on SCD.

How can national, regional, and international partners support the SCD continuum of diagnosis and care?

Dr. Nnodu: There are six key ways to support:

  1. By providing training for health care workers at participating PHCs involved in the screening and care of SCD, as well as materials for parental education;
  2. by offering transportation to the sites … this has been a major problem; a vehicle that can traverse those terrains would be a great help;
  3. by providing supplementary funds for compensating volunteers at the PHCs, for publicity and other logistics, and for interventions for patients with SCD detected through the program;
  4. by training more staff in genetic counselling to better educate families of carrier newborns;
  5. by providing more training in transcranial Doppler ultrasound technologies to expand the standard of care for children with SCD;
  6. and through the provision of folic acid, antimalaria drugs, oral penicillin, and hydroxyurea.

How is the SCD Centers Workshop helping to increase the number of adult care providers, and how does that effort go about improving the quality of care?

Dr. Smith: Regarding availability, we can say with pretty strong documentation now, that we have increased the number of states that have very dedicated SCD centers for adults. We’ve always had them for pediatrics, but I can give you some examples where there was nothing, and now there is something. The state of Ohio was without a center and now has one again because of work that we’re doing. There are cities in Canada that now have a center. In the state of Illinois, now we have centers in cities outside of Chicago. The state of Mississippi also has broadened their reach. We now receive roughly 30 applicants each year. By the time we’re done, I dare say we’ll have 100 qualified SCD centers focused on adult care.

We are able to say that they’re qualified based on the training that we’re giving. We are teaching people how to negotiate with their hospitals to get the centers funded and staffed. You might think that we would start by trying to work on specific aspects of care, but we very seldom talk about those specifics. We leave that to the individual physicians to come learn. And we’re actually coming up with a training program for individual physicians that want to get, essentially, a fellowship in SCD. There is [currently] no such thing as a fellowship in SCD. So what we started with was quality at the presence level, and now we are going to get quality at the process level. And we are very proud to have somebody in 100 different places almost, saying, ‘I am dedicated to care for sickle cell disease. Not only that, but I have a team and a hospital that is dedicated to caring for patients with SCD.’ It has been a morale lifter for those physicians. Imagine what it’s done for the patients to know that finally, somebody is advocating for them at their hospital to make sure they get the care that they need.

On the Sickle Cell Disease Comprehensive Care Act, how can that function to also help improve access to care and quality of care?

Dr. Smith: Well, if you think about what happened with cancer centers when they were first created, there were something called “comprehensive cancer centers” — federally funded centers designed to do research, to do mentoring and teaching, and to bring high-value, top-tier care to patients with cancer. And they had to be of a certain quality to qualify as comprehensive cancer centers. That is still true. What we’re trying to do is to get the federal government to recognize that SCD is the same. Not all SCD care is good care. And how do you know that good SCD care is being provided? The person providing it is well trained. The institution provides the funds to do it. And we are now saying to the federal government that they also have a responsibility here. In fact, the federal government would be doing itself a favor, because suddenly, some of the unnecessary care from hospitals and emergency departments can be transferred to places like clinics and infusion centers if there is a comprehensive SCD center there. That’s why the Comprehensive Care Act is important — it directs CMS to create a demonstration program in up to 10 states to improve access to comprehensive, high-quality, outpatient care, including recommended clinical, mental health, ancillary, and support services, for individuals with SCD who are enrolled in Medicaid. It’s all working together — participating states would receive a federal medical assistance percentage equal to 100 percent with respect to amounts expended for medical assistance for medically necessary services to treat patients with SCD cared for under the program.

Recognizing that education and improvements in practice are part of this ecosystem along with policy changes and the SCD centers themselves, how are evidence-based guidelines factoring into the landscape of quality SCD care?

Dr. Smith: ASH’s role in SCD began to increase nearly 10 years ago when the Society, along with other SCD stakeholder groups, put out a report card saying that, as a community of organizations, stakeholders, and so on, we were failing on several points. One was in the quality of care. The second one was in the availability of providers. One was in the global nature of our support for SCD. It’s a global disease, and we gave ourselves failing grades. We have now tackled that on each side [in the United States and globally]. One of the strongest and first things ASH did was to ask, ‘What evidence do we have for the right things to do [in treating] patients with SCD?’ We gathered teams of experts from around the country to come up with that evidence, sift through it, grade it, and decide what evidence rose to the level of a guideline.

The guideline is important, not only because it sets best practices… If you think about it, from guidelines emerge quality indicators. Quality indicators do more than simply tell you what to do. They are actually a “Better Homes & Gardens” seal of approval. If you’re doing this, your hospital is number one. We can now take certain quality indicators, and every hospital can know how well it is doing now. I would be in favor of hospital associations like NCQA and the Joint Commission taking some of these quality indicators and applying them to hospitals. Because I don’t think that the quality indicator has teeth until it matters at the level of how you get paid. And some of these quality indicators matter in terms of how hospitals get paid. That’s my goal. I’ve been pushing that for years, along with ASH. We really want to see these initiatives put into the fabric of care. And suddenly, hospitals that might not have been interested in SCD will become interested because they know that they’re giving poor quality care, and the guidelines are exposing them.

As the host of the ASH podcast series “Bringing Sickle Cell Disease to Life,” can you tell us a bit about what it was like to work on that series, and the value that it brings?

Dr. Smith: When we set out to make the Bringing Sickle Cell Disease to Life podcast, we were looking to capture pointed stories. People tend to remember stories better than they do statistics. People tend to remember anecdotes better than they do slide shows and graphs. When you talk about your experience with your doctor, you generally talk about what they did for you, how personable they were, a specific visit, a little pat on the back they gave you, something like that. And you make huge judgments about their general quality based on anecdotes like that. And we thought, “We’re going do that for SCD. We’ve been busy flashing lots of statistics up in front of people for years, showing them to federal [and] state policymakers … looking at the really sad survival data and … morbidity in SCD. But we haven’t brought it to life yet. How do we bring it to life? Let’s tell some stories.”

We told the story of Dr. Clarice Reid ... the first Black female head of the National Sickle Cell Disease Program at the National Heart, Lung, and Blood Institute. We told the story of Dr. Marilyn Hughes Gaston, who directed the Bureau of Primary Health Care. We told the story of Dr. James Eckman, who started the first state-funded transfusion center for patients with SCD ... These were poignant stories. Dr. Gaston told the story about a baby that died in her hands from sepsis due to SCD. Well, guess what, that motivated her to do the prophylactic penicillin study. So, “Bringing SCD to Life” has so many meanings. Ultimately it is about building awareness, and we believe that these stories will save lives indirectly by helping more people understand SCD and to therefore understand the importance of quality clinical care.

Learn more about these and other ASH and ASH Research Collaborative SCD programs at the Advancing Progress in Sickle Cell Disease Update and Reception, taking place Monday, December 12, from 6:00 p.m. to 8:00 p.m. Central time at the Marriott New Orleans Warehouse Arts District, Gravier DEF.

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