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Front doors of hospital Emergency Department


Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the patient`s chest, abdomen, joints and possibly even their bones.

The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain episodes. Others have a dozen or more crises a year. Patients have expressed seemingly violent descriptions of their pain, including feelings like there is broken glass in their body or like their bones are being crushed. Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers and other causes.

If a crisis is severe enough, you might need to be hospitalized. Unfortunately for many sickle cell patients, emergency hospital visits are often the start of another set of problems. When attending emergency rooms for pain crisis treatment and other sickle cell-related complications, patients have reported long wait times, insulting or disbelieving commentary from hospital staff and racial prejudice. Many have complained that if you are African-American and are in the emergency department for pain, you are labelled as drug seeking. One of the largest populations affected by sickle cell disease are those of  African decent. Beyond pain management needs, if patients do not receive adequate quantities of fluids, medication, oxygen, and more, death can occur.

The underlying reasons for these issues are part of a very complex health care system entailing many shortfalls and failures. They include but are not limited to:

  • inadequate training of doctors and nurses. Generalists often treat sickle cell patients who know little about the disease and patients’ desperate need for pain relief;
  • reluctance to prescribe pain pills due to the opioid addiction crisis;
  • only one-quarter of adult patients reportedly receive crisis prevention medication, such as hydroxyurea;
  • a significant number of family physicians are not comfortable treating sickle cell disease;
  • treating blood cancers like leukemia is apparently more lucrative than sickle cell, since the latter’s patients are complicated, hard to care for and time-consuming;
  • treatment guidelines and educational materials are often ignored in hospitals;
  • inaccurate assumptions that a patient’s detailed knowledge of their own pain medication needs means they are simply drug seeking rather than truly in acute pain.

In a recent interview hosted by ReachMD for their special series, Changing the Conversation about Sickle Cell Disease, Dr. Bernard Lopez from Thomas Jefferson University Hospital provided an updated approach to emergency medical care for patients with sickle cell disease.

Dr. Lopez advised that sickle cell disease is a condition that is not commonly encountered by typical emergency doctors, thus they are likely to be unfamiliar with it. The uncommonness may then lead to a “rare disease” stigma applied to patients in addition to the drug-seeking stigma mentioned above. Furthermore, since patients have been living with pain crises from time to time since birth, they are likely used to it and may not show the typical signs or symptoms of pain.  Again, this may lead doctors to doubt the existence of the pain or its severity.

Here, we are reminded that pain is an individual problem. No one else can feel it but the patient. Unfortunately, there is a power imbalance when patients request pain medication including because they are physically weakened by the pain crisis and very dependent upon health care teams’ knowledge and expertise to treat the immediate crisis and the disease.

Dr. Lopez advises that the first step should be to assess the pain, which is considered an emergency.  It is commonly done by a pain score. Dr. Lopez and his staff immediately try to treat the pain, without testing. He agrees that there is no test that can measure how severe the patient’s pain is. Furthermore, studies show that the faster the pain is treated, the faster it will subside and the more likely the treatment will successfully stop the pain.

The second step is that the health team should measure the patient’s vital signs, looking for indications of infection and additional complications that are ongoing. As sickle cell patients are at high risk for infection, it is critical that physicians look for that, as well as acute chest syndrome, which is the leading cause of death in adult sickle cell patients.

Dr. Lopez advises that the patient is admitted to hospital if they report that their pain has not been relieved enough in the emergency department and they still feel too bad, etc. to be able to go home and manage it there.  Again, as there is no objective measure for pain relief, it is important that the patient is believed when they say that they are in pain.

As ongoing long-term care is critical outside of the emergency department, it is important that the physician assure the patient has follow up care.

In general, sickle cell patients require high doses of opioids, doses that are higher than typical emergency department patients.  They also typically need more frequent dosing than the average emergency department patient.  Therefore, emergency department physicians and staff have to be knowledgeable as well as willing to treat these patients’ needs if their pain is to be properly managed and resolved.

In light of all of this, how can patients increase the likelihood that they will be believed and provided with adequate medical attention when in pain crisis or other sickle cell-related emergency? We initially thought that carrying a letter from their primary physician, as well as their blood records as proof of their disease would help alleviate the problem. Other patients and doctors also recommended this.  However, research indicates that many patients who do this are still being maltreated, ignored or set aside to wait in emergency rooms.  We also considered that wearing a medical ID bracelet may facilitate the intake and treatment process.

While taking these proactive steps may result in some benefit to patients, the systemic problems are such that strong advocacy is still needed to make significant impact for the vast majority of these patients, including at organizational and government levels.  Our FREE ebook, “Achieving Health Victory Through Patient Advocacy!” contains excellent information and resources as to how family, friends and/or professional patient advocates can assist patients to receive optimal health care and results from medical service providers working in different arenas.


To explore some of the options that have helped Sickle Cell Anemia patients, including natural remedies and alternatives, as well as traditional medical treatments, purchase

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