When the ER Can’t Hear You: Tips and Transition Tools for Families with Sickle Cell Disease

Q&A with Andrea Williams, Founder, Children's Sickle Cell Foundation

January 18, 2014
By Kristina Grifantini

Andrea Williams
Andrea Williams
Andrea Williams had no idea that she and her husband were both carriers for sickle cell trait until their fourth child, Jonathan, was born with the disease in 2000. When two people with the trait have children, each child has a 25 percent chance of having sickle cell disease and a 50 percent chance of carrying sickle cell trait. Sickle cell disease, which affects millions of Americans of all races, often results in extreme pain, chronically low blood count and, in some cases, early mortality. In the disorder, blood cells change shape under stress, leading to blockages in blood vessels and organs.

Williams' experience led her to launch the Pittsburgh-based Children's Sickle Cell Foundation, Inc. (CSCF). The foundation provides children up to age 21 with strategies for ongoing health and well-being practices. Williams also works with NICHQ on the Working to Improve Sickle Cell Healthcare (WISCH) project, which focuses on better coordination of services, newborn screening, transition from pediatrics to adult care and other improvements. We recently spoke with Williams about some of her challenges and inspirations.

What drives your work in improving care for families with sickle cell disease?

In all my work, I draw from my own experiences and other parents' experiences. From tons and tons of surveying we hear similar stories from parents: you're at the emergency room (ER) and things aren't going well so what do you do? Many patients face a lack of understanding and timely care at the ER because practitioners don't understand sickle cell disease or suspect the patient is drug-seeking when they ask for strong pain medication. It boils down to this: What do you do when the nurse can't hear you?

I found myself confronted with this dilemma when Jonathan was having the strangest pain episode I had ever seen: he was literally screaming in pain and then he would fall asleep, before waking up screaming and falling asleep again. As a mother, I was so afraid. I thought he was having a stroke or something else serious.

I took him to the ER and did everything you're supposed to. I called first so they were expecting us. When we got there, I asked the nurse if he can have something for pain and the nurse said there aren't any orders written. I said, "Can someone do something because he's in pain?" She came back 15 minutes later with yet another healthcare practitioner. By then, Jonathan had gone through the process of waking up screaming and falling back asleep three times. I was getting more and more fearful and agitated. I repeated the story to the practitioner and was still denied pain medication.

I thought, what is the right question that will get me the help I need? I approached the nurse again and asked, "Is there anything he can have for pain until the order is written?" After additional checking, she found a note saying he can have morphine until an order is written by the physician. I was livid. I couldn't believe she didn't think to check for a note in addition to the traditional order. We spent more than a half an hour sitting in pain for no reason.

How does that experience inform your work now?

Out of that experience I've written pieces and participated in discussions on what should families do when the people working in the healthcare system can't "hear" them when their needs aren't being met. It's about stepping back, not demanding the care for your child, but changing your tone of voice and asking for help in a way that's not threatening. I get a ton of pushback from people saying it shouldn't be this way and I know that, but it's human nature. If you ask a non-accusatory question you will be more likely to get help rather than a "why aren't you doing something" tone which makes people defensive. I recommend trying an inquisitive tone and ask "Is there something that can be done while we're waiting?" instead.

One of the most important things I try to express to the parents through this organization is to keep hope. Right now, I can't change that children have sickle cell disease, but, we can help patients to be as courageous as they can and empower families with education and support.

How did learning collaborative quality improvement techniques and partnering with NICHQ help your organization?

It gave us an opportunity to look at what we're doing well and explore ways to do it better; to push ourselves out of our comfort zone; and to identify and meet more of the needs of our participants. It has also helped us figure out what will it take to move us from good to great. I think our organization is really the better for it.

We also learned that if you're not looking to make improvements, you'll keep doing things the same way over and over again. Having a mindset of improving helps remind you to celebrate what you're doing well, and at the same time pushing yourself to do better.

What are the changes you're most proud of?

The first is our transition program. For patients over the age of 18 with sickle cell disease, we offer tools for going back to school, finding a job and other support for transitioning to adulthood. We changed our approach from having a large class to scale back, take our time and increase the amount of interaction a week with a small group of patients. We used to meet once a week for 10 weeks; instead we tried meeting twice a week for four weeks with a handful of people. This has allowed us to really focus on the strengths and areas of challenge for the individual, help them set reasonable, attainable goals that they can have for life.

We also created a transition center in our building with four computer stations and instructions. On Tuesdays and Thursdays we cover everything from life skills, computer literacy, social interactions, medical navigation and others. We have a "real-world" component which consists of a scavenger hunt of real-world tasks, like registering for selective service or making an appointment with their primary care physician.

In addition, we now provide free monthly bus passes. We found that participants feel more empowered because it gives them freedom to move around, so when we'd tell them to follow up and look for a job, they had the means and opportunity to do so. It opened their eyes to help them realize that they can do this and take care of things, such as grocery shopping or attending job interviews. It was an amazing addition to the program and we didn't anticipate that.

What have you found to be helpful for parents of newly diagnosed newborns?

We've just launched a newborn initiative for parents of patients under five years old. One component is a parent-friendly resource, something small that moms can carry in their purses with them all the time and keep notes in. We are also considering an electronic version that can integrate with EMR and medical home information. Because the guide is made for parents by parents—and reviewed by a hospital—we hope it's accessible and "back to the basics." When my son was diagnosed, I received a huge manual and it nearly scared me to death. Especially with parents of newborns, you don't want to scare them, you want to empower them.

When Jonathan was diagnosed, the social worker said something that stuck in my head, "The way you deal with this disease is the way your child will deal with it." It is true to this very day. I realized that I needed to figure out how to manage this disease so my son would be well adjusted and have a healthy life. Now, I want to share that realization and empowerment with other parents and caregivers of children with sickle cell disease.

How do you summarize your work in one sentence?

The whole idea is to change the mindset of people who have sickle cell disease so they're not looking forward to dying, but toward living every single day and "living well with sickle cell."