Living With Sickle Cell: ‘I Don’t Know What It Means to Be Without Pain’

It’s World Sickle Cell Day, and we’re taking a look at the chronic pain and regular hospitalizations that are the reality for many suffering from sickle cell disease.

 
Arm of patient with drip

Nikki Peterson, like approximately 100,000 other Americans, was born with sickle cell anemia. The 43-year-old lives in Upper Marlboro, Md., and ends up in the hospital about four times during what she calls a good year.

Once a month, she undergoes a grueling process called hemapheresis. All of the blood is removed from her body, the platelets and plasma are separated out and returned to her, and then Peterson is given 8 to 12 units of packed red blood cells. This helps to mitigate the pain she lives with every day.

“I don’t know what it means to be without pain. I have nothing to compare it to,” Peterson tells The Root from her bed at Doctors Community Hospital in Greenbelt, Md. “I have what I call my normal pain, and my pain where I need to be in the hospital. They always ask what your pain scale is from 1 to 10. I function on a normal person’s 7 to 8. It’s like my 2.”

The monthly process Peterson lives with takes two days. One to have her blood drawn to find a compatible donor and have the level of hemoglobin in her blood tested, and another for the transfusion procedure. This is one method of helping people with sickle cell disease reduce pain crises, the risk of stroke and other complications. People who suffer with the inherited blood disorder have red blood cells that contain mostly hemoglobin S, which sometimes become sickle cell-shaped, become stiff, and have difficulty passing through small blood vessels. That means less blood can get to parts of the body, and the tissues there become damaged. It hurts.

“It’s the only way I know how to live,” says Peterson, who had to leave her beloved job as a special education teacher because of sickle cell. “In 2002, I had a crisis in my back and it cracked four vertebrae and put me on permanent disability. I started having seizures, and I’m a stroke patient.”

Sonja Banks, president of the Sickle Cell Disease Association of America, says more than 6 million people in the world; mostly in sub-Saharan countries throughout Africa, as well as in Turkey, Greece and India, have one of the several types of sickle cell disease. This puts them at risk for everything from pain episodes in their arms, legs, chest and abdomen to damage to most organs, including the spleen, kidneys and liver.

“It’s actually more of a brown disease, though, internationally, there are more non-African Americans,” Banks explains. “In the U.S., the majority of those who have it are African American, and the next would be Hispanics.”

She says beyond the blood transfusions that allow doctors to punch out some of the bad cells and replace them with better cells, and penicillin for babies, for most sickle cell patients the only help is pain medicine. Banks says the only other treatment is the drug hydroxyurea, a cancer medication that has worked in some patients. Though the sickle cell health community is fighting to get more treatment for the disease, Banks says other illnesses, such as cystic fibrosis, get more medication and attention. She thinks some of this is because many who suffer from sickle cell disease are people of color.

“There’s definitely some disparity, and part of it has a lot to do with race … particularly in the U.S.,” Banks says.

The international community, including UNESCO and the World Health Organization, works to raise global awareness of the disease with World Sickle Cell Day, celebrated annually on June 19. That date is also Juneteenth, known in the U.S. as the oldest-known celebration of the ending of slavery. On that date in 1865, Union soldiers landed in Galveston, Texas, with the news that slaves were free, two years after President Abraham Lincoln’s Emancipation Proclamation. Events are scheduled this year from the Republic of Congo to Ghana and Nigeria to Atlanta and Fort Worth, Texas, some combining sickle cell awareness with the commemoration of Juneteenth.

Drug companies such as Pfizer, along with Banks’ organization, are urging more people of color to get involved in clinical drug trials so that a new drug to fight sickle cell disease can be found. Researchers at Pfizer are working on a potential therapy that is in Phase 3 clinical testing, and doctors there are trying to educate diverse populations about the trials because 1 African American in 500, and 1 Hispanic in 36,000 in the U.S. are affected by sickle cell disease.

“Statistics show that most clinical trials … the minority participation has been very low, so most that get into clinical trials are white Americans,” Banks says. “If you look at other diseases such as diabetes, and look how many drugs that are now on the market for those diseases, the majority got to the market because of white Americans. … So now we look at [sickle cell disease] where a majority of those with it are minorities … it’s a huge disparity, and if we as African Americans don’t get into clinical trials, we won’t see drugs come to the market and we won’t get a cure.”

Banks says she realizes many blacks are deterred because of the 1932 Tuskegee experiment, when hundreds of African-American men weren’t told they were part of a study, were exposed to syphilis and were not given treatment to cure the disease. She notes that government regulations on clinical trials have come a long way since then, and that with an informed and educated community, such a thing could never happen again.

Peterson has been involved in two clinical trials. One, at Johns Hopkins, through the National Institutes of Health, ran for a year, which provided everything from hospitalization, to transportation to the hospital around the clock, to a stipend. The 2014 clinical trial also helped Peterson with the thing she needs the most.

“They also provided pain medication. I never understood when I was younger why Grandma didn’t take her pain meds until now, because when it comes to the point where I have to decide ‘Do I pay my rent, buy groceries or pain medication?,’ that becomes a hard question to ask,” Peterson says. “But the greatest thing was that whenever I got sick, I had somewhere to go.”

Peterson says on World Sickle Cell Day, she wants people to focus on the fact that sickle cell disease affects people from all walks of life and on many continents in the world.

“It’s a disease that is a health epidemic, and we do need to find a cure for all patients,” Peterson says, “regardless of what continent, what skin color, what eye color. It’s a world disease.”

Marrow transplant frees sickle cell sufferer from pain

 

http://www.usatoday.com/story/news/nation/2015/01/29/marrow-transplant-frees-sickle-cell-sufferer-from-pain/22557607/
ROCHESTER, N.Y. — With sickle cell anemia, it’s never just one kind of pain.
Sometimes it’s a vicious ambush, fast and pulsating. Sometimes it settles in slowly and stays for days, throbbing deep in the bones.
Sometimes the pain is in one spot. Sometimes it’s in three. Sometimes it’s everywhere, hundreds of knives, jabbing and cutting from head to toe, grinding down the body and foreclosing on any hope for the future.
“It’s the worst feeling in the world,” said Stephanie Ramos of Irondequoit. “It’s like your body is attacking its own self. Beating up on its own self. … It’s a horrible disease. I hate it.”
Ramos knows sickle cell anemia well. She was born with it 25 years ago. Doctors told her mother then that she would be lucky to reach adulthood; each birthday was a celebration and, quietly, a foreboding.
What would the next year bring? How many crises, how many trips to the hospital? How much more to be endured, and for how much longer?
It’s a disease of pain, and three years ago, Ramos was exhausted by it.
Wixon: Sickle cell disease can’t hold back Lincoln’s Tafari Witter through sporting, academic endeavors
http://www.dallasnews.com/sports/high-schools/headlines/20150216-wixon-sickle-cell-disease-can-t-hold-back-lincoln-s-tafari-witter-through-sporting-academic-endeavors.ece

Tafari Witter is near midcourt, bent forward, tugging on the bottom of his shorts, trying to catch his breath. A substitute is headed to the scorer’s table, but Witter looks over to the sidelines and, with a wave of his hand, indicates he’s not leaving the game.
It might be the only time that Lincoln’s starting point guard doesn’t have his priorities straight.
Witter is a razor-sharp student who is on track to be valedictorian of Lincoln’s Class of 2015. He loves Advanced Placement Calculus BC, is part of the Dallas ISD’s Future Leaders Program and in the fall will attend Texas, where he eventually wants to get a master’s degree in business administration.
NBA point guard Derrick Rose is among the people who inspire him, but so is a star from a very different arena: theoretical physicist Stephen Hawking.
Lincoln coach Cedric Patterson smiles when he hears that.
“He’s a unique kid,” Patterson said.
Witter is so unique, and interesting, and engaging, it’s hard to squeeze his sickle cell disease into the conversation. And he likes it that way.

Quality Improvement Sickle Cell Treatment

Am J Med. 2015 May;128(5):541-4. doi: 10.1016/j.amjmed.2014.11.020. Epub 2014 Dec 9.
Quality improvement process in a sickle cell infusion center.
Whiteman LN1, Lanzkron S2, Stewart RW3, Haywood C Jr4, Strouse JJ3, Feldman L3.
Abstract
BACKGROUND:
The American Pain Society recommends that individuals experiencing sickle cell crisis receive parenteral pain medication within 30 minutes of assessment. We examined factors affecting achievement of this standard at the Johns Hopkins Sickle Cell Infusion Center.
METHODS:
Baseline patient care time intervals and data on variables affecting the ability to achieve the American Pain Society goal were measured. Time to first parenteral opiate administration was modeled using simple and multivariable linear regression.
RESULTS:
Mean time from initial assessment to first dose was initially 41 minutes. Increased nurse to patient ratio decreased time to first dose.
CONCLUSIONS:
Of the factors associated with improved times to first dose, only nurse to patient ratio is amenable to process change, suggesting it as a potential target for future interventions.
Copyright © 2015 Elsevier Inc. All rights reserved.

PMID: 25498167 [PubMed – in process]