Fighting ER Misconceptions About Sickle Cell

Based on an article by Beryl Lieff Benderly

An all too familiar emergency room experience for people with sickle cell disease is humiliating, scary and painful.

Just imagine you are wheeled into the emergency room, every limb on your body feels like large butcher knives are stabbing into your bones. You barely speak except to nod to acknowledge your name and medical record number. A nurse fills out every form, then sends you over to a corner to not disturb others with your cries. You feel like you are dying. Your body hurts, your mind wants to give up and your soul is ready to get out of this pain filled life. This is how it is for many people with sickle cell disease during an emergency room visit.

Last year, Paula Tanabe, an associate professor at the Duke University School of Nursing was commissioned by The National Heart Lung, Blood Institute (NHLBI) to explore this problem.

This issue is complex.

People with sickle cell experience acute and unpredictable pain, severe enough to require large doses of narcotics, is a well-documented feature of sickle cell disease. But Tanabe has found that too often patients in sickle cell agony are not handled properly by doctors and nurses in the emergency department because they erroneously think the patients may be running scams, seeking drugs.

She believes stereotyping is the number one barrier behind that wait: "After being cute little kids with sickle cell that everybody felt bad for when they had pain, now they become this African American 20-year-old asking for high doses of opioid narcotics." 

Ms. Tanabe says better training of health providers and changes in emergency room procedures can improve the care that sickle cell patients receive.

There is hope and help is coming.

Currently, a team has been assembled to update the NHLBI Guideline of Treatment of Sickle Cell Disease. The guideline will be principally aimed at primary care practitioners and their patients to help manage the ongoing processes of the disease. The guideline will also be valuable to hematology specialists and their patients.

There will also be a webinar to discuss these updated guidelines in September 2013 through The Sickle Cell Information Center website.

Personally, I have experienced bad emergency room treatment as well. And, I'd like to thank NHLBI and Ms. Tanabe for their efforts to identify and address this issue.  I appreciate your help in this area.

Source: Kaiser Health News

A New Day Has Come For Children With Sickle Cell Disease

(photos by Denise Truscello/ WireImage)

On January 15, 2012 Play Without Pain: Children’s Hospital Sickle Cell Benefit at The Colosseum in Caesars Palace began with Gary W. Loveman, Chairman, Chief Executive Officer and President of Caesars Entertainment Corporation thanking everyone for supporting this life-changing event. He explained the alarming statistics of children suffering and dying from the complications of sickle cell disease, and the important work being done at Children’s Hospital of Boston to find a cure. “Children’s physician-researchers have done what others have tried to do for decades; they’ve found a way to override the sickle cell defect,” he said. Mr. Loveman then introduced the team of doctors led by Dr. Stuart Orkin and Dr. David A. Williams, who are behind the breakthrough strategies to “flip the switch” on Fetal Hemoglobin F (HbF) and cure sickle cell disease once and for all. “Hope,” he said, “is in sight.”

Enter Celine Dion and Rene Angelil, AEG Live, Children’s Hospital Boston, Southern Wine & Spirits, Angel Management Group, Restaurant Guy Savoy, and countless other sponsors too numerous to name. Only heaven bestows such star power. The rest was history in the making.

As Celine walked onto the stage, a new day dawned for children with sickle cell disease. When she began to sing angels smiled, clouds parted and the rain of hope fell like a beautiful waterfall. With the voice of perfection, accented with flawless wardrobe changes, and make-up sprinkled with tears, Celine’s heart was fully exposed.

While I listened to Celine perform, like only Las Vegas could contain, I looked around the Colosseum in amazement. I was witnessing history. When Celine cried, I cried. God allowed me to live to see the dawning of this new day.
(photo of Celine and A.J. Green, young man with sickle cell)

As Celine sang, my heart sang,

“I see a light in the sky
Oh, it’s almost blinding me
I can’t believe I’ve been touched by an angel
With love

Let the rain come down and wash away my tears
Let it fill my soul
And drown my fears
Let it shatter the walls
For a new sun
A new day has come
A new day has come.”
(song by Aldo Nova & Stephen Moccio)

I may not see a cure for my sickle cell disease; I’m too old for these new procedures. I see, however, the light shining for children with sickle cell disease…………can you see it?

Thank you Melanie, Caesars Palace, Celine and Rene, Children’s Hospital of Boston, and the numerous sponsors of Play Without Pain: Children’s Sickle Cell Benefit. You are all partners in giving the gift of life. Put on your sun glasses, it’s gonna get bright.

(photos by Denise Truscello/WireImage)

An Example for Sickle Cell Treatment at the University of Illinois

The University of Illinois Medical Center at Chicago has housed a sickle cell center for adults for the past 25 years. Recently, in efforts to support patients throughout life and debunk myths about the condition, it has opened a new acute care treatment center to manage uncomplicated pain crisis.

In the past, chronic pain treatment associated with sickle cell disease was coupled with the lack of knowledge, racial discrimination and repeated emergency department visits that contributed to a troubled relationship between patients and nurses.

Boney Johnson MSN, RN at the Center said ‘It wasn't until she began a sickle cell support group and engaged with patients during healthy moments that she realized the degree of misunderstanding that exists when sickle cell disease is mentioned.’ Johnson also noted, “The emergency department was the biggest fear for a patient with sickle cell disease because they have to wait so long to be seen. The patients also feel confronted by unwanted bias in the emergency department.”

She notes, “Our purpose with the acute care center is to provide them with immediate treatment for pain." According to a recent article by Robin Hocevar, “A generation ago, the idea of even having an adult unit for patients with sickle cell disease was unheard of. According to the Sickle Cell Disease Association, the average life expectancy for patients with sickle cell disease is on the increase and currently stands in the mid-40s.”

The University of Chicago has responded to the needs of their community with 85 percent of the state's residents with sickle cell disease that have Cook County address.

Bravo!!!! University of Illinois Medical Center….let others follow your lead.

Check out article. http://nursing.advanceweb.com/Regional-Articles/Features/Sickle-Cell-Disease.aspx?prg=19

Patient Centered Care

I was recently sent an email where I was asked my opinion regarding sickle cell management in the US and abroad, as it relates to improving care for sickle cell disease. The question was posed by suggesting more of a cultural/social model of care rather than a medical one.

I am just a patient with Sickle Cell Disease, and an advocate for people living with the disease. I am not a medical professional, I am not an expert, nor do I profess to know what a “medical model” is verses a “cultural/social” one. All I know is my experience with having Sickle Cell Disease.

As a child, I received treatment by medical professionals within my “cultural” community and the care I received was gentle, compassionate and well trained. It was not until I grew up, began to travel and moved to different communities. More than once I’ve been told by uninformed medical professionals, “What do you have? Sickle What?”

I was disgusted when doctors would tell me, “Oh, you have Sickle Cell, only black people get that disease.” Or, doctors would say, “Oh, we don’t get many black patients here, so we don’t see cases of Sickle Cell.” This calls for more training of medical professionals.

Just for the record, my niece looks white. She is ¾ Anglo & ¼ African American. Because we have a family history of Sickle Cell, she asked her doctor to perform an electrophoresis test to see if she was a carrier for Sickle Cell Disease. He told her, “You’re white, you don’t have that disease.” She insisted, because of her family history (her mother has it). Low and behold, was tested and found to have the Sickle Cell trait. So much for “you’re white, you don’t have THAT disease.” Again, this calls for more training of care providers.

For this very reason, I believe Sickle Cell care should be approached in a patient centered model; (as a disease of humans), not as cultural model (a disease of one racial group). Sickle Cell Disease is creeping silently into all populations, all races (because of mixing) and all cultures. Now, that’s my opinion.

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Calling All People With SC Disease

Calling all people with Sickle Cell Disease to participate in a research study.

The study is sponsored by Ms. Phyllis Bazen, MSN, FNP-C, a Doctoral Candidate at the University of Rochester School of Nursing. Ms Bazen is the Study Coordinator.

The purpose of the study is to explore the most common stressors that adults with SCD cope with and to explore the effects that these stressors have on mood and/or quality of life perceptions.


People with Sickle Cell Disease have many things that bring stress; pain, depression, hopelessness, etc. etc. etc. We have to live on despite these stressors and try to have a meaningful, productive lives...anyway.

The best way to contact Ms. Bazen is the web site contact page, her cell phone, email, or 1-800-464-8668 (in US) See card below for information.

If you decide to participate in this study, you will be speaking with Ms. Bazen who conducts a one-on-one private (confidential) phone interview. In this interview, Ms. Bazen will ask a series of questions related to living with Sickle Cell Disease and related stresses.

The outcome of this study is to gather data related to the stress that Sickle Cell patients endure and find ways to better treat them (us).

Check out the web site at http://www.scdstresstudy.com/

We can all have our voice heard on this one.




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To Those Who Care About Us

I was going to write a separate blog for each wonderful Sickle Cell Center, Hospital, or Clinic that stood out as a beacon of hope for patients with Sickle Cell Disease. What I found was way too many of them to mention separately.

There are so many doctors, advocates, universities, clinics and organizations who are fighting in-behalf of patients with Sickle Cell Disease. They are working to touch the lives of hurting people, researching new drugs to give comfort for their pains, and fighting the uphill battle to find a cure. The more I researched the more I was moved to tears.

There is an army out there who cares about me and all the other patients suffering with Sickle Cell Disease. To this army of health care professionals who see pain and suffering everyday, I say thank you for your compassion. To the advocates who organize walks, conferences and seminars, I say God bless you. And, to those researchers who never get tired of looking into a microscope at sickle cells, looking for a cure, I say DON’T GIVE UP.

There is an army of Sickle Cell patients cheering you on, petitioning God in your behalf, and silently hoping for your success. I tip my hat, I bow my head in respect, I yell “YIPPY!!!”, and I pass on their love for all your hard work.

Shout Out To Clinics

This is the first shout out (say hello) to all those people/clinics who provide comprehensive services for both adults and children and the families of those with Sickle Cell Disease.

The University of Illinois Medical Center has a mission to "improve the quality of life for individuals with sickle cell disease and to reduce the morbidity and mortality of the disease through high quality medical care, patient community education, and research into new therapies.

And, they do just that!

Their encouragement, compassion and quality medical support is greatly appreciated. People with Sickle Cell Disease live better because of your tireless work.

THANK YOU!!!!!!!!!!