Advocating for Yourself in the Emergency Room

Dr. Simone Eastman Uwan (known to the sickle cell community as Dr. Simone) was diagnosed with sickle cell disease in college, even though she had symptoms all her life. Her diagnosis changed the trajectory of her life and encouraged her to focus on supporting sickle cell patients throughout her career as a medical doctor.

While sickle cell disease caused Dr. Simone to retire early from her clinical practice, her life is vibrant. She educates patients, caregivers, and healthcare providers, and advocates for improved care. Dr. Simone is determined to reshape the conversation about sickle cell disease management with her colleagues in her healthcare community so that her sickle cell community can live longer and dream bigger.

How can Sickle cell patients advocate for themselves in the emergency room?

Sickle cell disease, whenever I think of it, seems like the perfect torture that anyone could invent. It’s invisible to the naked eye and cannot even be adequately evaluated by the existing blood analyses we have available today. There is no imaging that can accurately show the full extent of the devastation the tissues of the body endure.

A person can take a beating from the inside, pummeled black and blue. And yet, that same person can sit calmly, for hours in the emergency room with no tears but simply frustrated for being overlooked.

Challenges

The biggest challenge that I find sickle cell patients have when presenting to the emergency room is that they sit for hours on end, waiting to be seen. And while they sit, they burn in pain because there is ischemia- or a lack of oxygenation leading to tissue damage. This blockage of circulation causes extreme pain, and in sickle cell disease this is referred to as a vaso-occlusive crisis.

With every passing hour that a person sits waiting to be seen without any medical intervention, patients with sickle cell disease are experiencing damage to vital organs. And they are expected to keep quiet until they are seen, whenever that may be.

Like surgery without anesthesia

This would be the equivalent of having surgery started on your body one random day, only for the surgeon to say after starting: “We have no anesthesiologist, so can you do us a favor and just endure whatever pain you feel while we cut into your body?".

What would you say? Would you say “Thank you, no problem, after all, I aim to please?” or would you say “Excuse me kind sir, but would you mind getting another anesthesiologist because I’m really uncomfortable”? What would happen if you waited for an hour into the surgery, and no anesthesiologist came? Would you not cry and scream and demand that someone stop the world right now and give you some anesthesia because the amount of pain you are feeling could stop your heart?

Yes, this is very likely what you would do. Because stopping your pain would not just be for comfort. It would be so that you didn’t overwhelm your heart and cause it to stop beating from all of the pain-induced stress hormones leaking into your blood.

Forced to endure the pain

Patients with sickle cell disease are often made to endure the equivalent of having surgery without anesthesia. If they cry out, they are labeled as disruptors of the peace and are made to look like they came to the emergency room seeking drugs for no apparent reason. On the other hand, I often wondered why some sickle cell advocacy group has not put together a tutorial for patients on how to present to the emergency room in a way that would optimize care for a person living with sickle cell disease. This is what I mean.

Avoiding the emergency room

When a person with sickle cell disease presents to the emergency room, they are often doing so after having suffered for many days at home trying to get the pain under control, usually to no avail. After what seems like forever but could be about five days or so, they will realize that they cannot take care of their pain at home and they must present to the emergency room.

The fact that they have even waited for this long is usually because of prior mistreatment or ineffective care that they received in the past. Experience with emergency room healthcare providers have taught patients living with sickle cell disease that they are not to seek help until they are dying in 10 out of 10 pain. Even then patients will likely endure a level of neglect that has them crying out in agony, “offending” healthcare providers, and sometimes even being kicked out of the emergency room.

ER guidelines

Knowing that the trauma is real and that danger looms if proper steps are not taken, the National Institutes of Health (NIH) and American Society of Hematology at different points have taken steps to establish written guidelines on how a patient should be evaluated in an emergency room. They go as far as saying that a patient with sickle cell disease in an acute vaso-occlusive crisis should be seen and evaluated thirty minutes from triage or one hour from their arrival in order to prevent end-organ damage.

Since most patients don’t know the guidelines, they are not able to use this information to advocate for themselves. Furthermore, although it has been plainly laid out in writing since 2014 by the NIH expert panel report, most emergency room doctors and nurses are not even aware that these guidelines exist!

Self-advocacy

For this reason, patients living with sickle cell disease have to be their own advocates by becoming aware of these guidelines to use at future visits. Furthermore, they should print these guidelines out and highlight the pertinent parts for easy reference when they need to receive emergency care for an acute vaso-occlusive crisis.

Healthcare provider advocacy

Other advocates that patients often don’t use are their primary care physician and their hematologist. Yet again, these doctors are not often aware of the emergency room guidelines either. However, if we can make them aware that those guidelines exist, this creates a potential for advocacy!

Bringing resources and recommendations to the ER

An informed doctor who refers you to the emergency room because your situation has become unstable can easily include the recommendations by the American Society of Hematology, updated in 2020, which says you should be triaged and treatment started within one hour of your arrival to the emergency department. This information coming from another physician, and substantiated by your conveniently available binder of information, highlighted in the right places to show what your doctor’s note or call is referring to, can add a new level to the self-advocacy of patients living with sickle cell disease.

Living with sickle cell

Honestly, it’s about time patients learned how to fight back against the system that often provides them substandard care. Patients living with sickle cell disease are productive members of society, and many have full lives. There should be a way that they can safely return to that beautiful life after dealing with an acute sickle cell crisis in the emergency room. They deserve that much.