Sickle Cell Patients Face Life-Threatening ER Waits

By Sara Hutchinson, KFF Health News

Heather Avant always dresses up when she goes to the emergency room.

“I’ve been conditioned to act and behave in a very specific way,” said Avant. “I try to do my hair. I make sure I shower, have nice clothes. Sometimes I put on my University of Michigan shirt.”

HEATHER AVANT

It’s a strategy to combat discrimination the 42-year-old photographer in Mesquite, Texas, has developed over a lifetime of managing her sickle cell disease, a rare blood disorder that affects an estimated 100,000 Americans.

The hereditary condition can affect a person of any race or ethnicity, but Black patients, like Avant, make up the majority of those afflicted in the U.S.

For people living with the disease, a sickle cell crisis can happen at any time. When it does, their rigid, sickle-shaped red blood cells become stuck in their blood vessels, blocking flow and causing extreme pain or breathing difficulties.

A crisis can escalate into life-threatening complications such as strokes, seizures, and sepsis.

When a pain crisis can’t be managed at home, patients head to the ER to get the high dosage of opioids they need, in addition to IVs to help with dehydration or even blood transfusions. Yet staffers in emergency departments — already overextended and grappling with nursing shortages — don’t always have experience in treating the rare disease. And many doctors, amid a still-raging opioid crisis, remain resistant to prescribing the painkillers necessary to treat sickle cell crises.

As a result, patients with sickle cell disease often face long delays before receiving essential ER care, plus discrimination and suspicion that they are seeking drugs to get high.

“I have to look like I’m not coming in here off the street looking for medication,” said Avant. “I have to put on an entire show to get you to believe that I need care.”

Long Waits

Years of research have documented the delays. A study published in 2013 found that patients seeking care from 2003 through 2008 at an ER for their sickle cell crises waited 50% longer than patients who arrived with broken legs or arms.

Another study published in 2021 found that 50% of sickle cell patients reported having to wait at least two hours before their pain was treated, despite medical guidelines recommending such patients in crisis receive their first dose of pain medication no more than 60 minutes after arriving at the ER.

Medical associations such as the American Society of Hematology, the National Heart, Lung, and Blood Institute, and the Emergency Nurses Association have established guidelines for emergency department-based care of sickle cell pain. And, in 2021, the Emergency Department Sickle Cell Care Coalition, a national collaboration of hematologists, pharmacists, and nurses, helped launch a point-of-care tool to help medical professionals manage the disease in the ER.

But patients and sickle cell experts said those best practices haven’t been widely adopted. A 2020 survey of nearly 250 emergency medicine providers found that 75% of them were unaware of the NHLBI’s recommendations, first published in 2014, yet 98% felt confident in their ability to treat patients with sickle cell disease.

Still, ER horror stories abound among adults with sickle cell disease. For Lesly Chavez, 29, a Houston hairstylist, her worst experience occurred a few years ago. She said she spent four hours in a waiting room before getting seen.

“And when they finally got to me, they told me they could help with ‘my addiction,’ but they decided that there was nothing that they could do for me,” Chavez said. “They just flat-out said no and sent me home while I was in crisis.”

Chavez said she has since avoided that hospital even though it’s 10 minutes from her home. Now she drives to an ER 30 minutes away.

Chavez, who is Hispanic, said she confronts “doubt everywhere I go” because sickle cell disease primarily affects Black Americans. (Those who are Hispanic can be of any race.)

Paula Tanabe, a professor of nursing at Duke University who has spent decades researching ways to improve care for sickle cell patients, said a confluence of factors adds to the racial bias patients may face.

“Emergency rooms are incredibly overcrowded, at rates that we have never seen before, and that’s for everyone,” said Tanabe.

‘People Are Going Uncounted’

Legislators are trying to help. A federal bill introduced in June would allocate $8.2 million annually for five years to a program that trains doctors on best practices for caring for sickle cell patients. Another, introduced this spring, would provide funding for community organizations working to spread awareness about the condition and give student loan relief to medical providers who commit to working on the disease. Some state legislatures have established sickle cell task forces to improve physician education and care coordination.

Advocates for sickle cell patients said investment in data collection to track the disease is also important. Although the Centers for Disease Control and Prevention estimates that some 100,000 Americans have it, the true number is unknown. That’s because no national system exists to collect data on sickle cell, unlike other conditions such as diabetes, cancer, and Alzheimer’s.

“I’m 32 and we’ve been saying it’s 100,000 my entire life,” said Quannecia McCruse, who co-founded the Sickle Cell Association of Houston. “I know there’s more. I know people are going uncounted.”

Eleven state-led data collection programs currently exist and, in February, the CDC opened a new grant application for additional states. Improved data would allow funding to be allocated toward the areas with the greatest need, sickle cell patient advocates said.

Texas had an opportunity to join those efforts. This spring, the state legislature passed a bill with broad bipartisan support to create a sickle cell patient registry, but Republican Gov. Greg Abbott vetoed it, saying it would compromise patient privacy.

“That was a bad excuse,” said McCruse. “We have a cancer registry already, and everyone’s information is safe. That registry would have gone a long way to help.”

While progress grinds slowly, patients like McCruse say they’re forced to balance advocating for themselves during bouts of excruciating pain against the need not to irritate or alienate hospital staffers.

“It feels like someone is taking a Taser and shocking the crap out of me. Or when it’s really bad, and it feels like shards of glass are just moving through my veins,” said the mother of two. “It’s very, very painful. And you’re telling somebody whose body is torturing them that it’s not that bad?”

Alexis Thompson, a hematologist who treats sickle cell patients at the Children’s Hospital of Philadelphia, said she works with her pediatric patients to develop self-advocacy skills. But sometimes that backfires.

“The great irony is patients who are well informed and capable of self-advocating are being accused of being manipulative, because they are capable of articulating very clearly what’s effective for them down to the name of the medication or the absolute dose,” Thompson said.

Sickle cell experts recommend that doctors adhere to a patient’s individual pain plan, if available. Thompson said those plans, which document patients’ diagnoses alongside a recommended medication and dosage, can be uploaded to online portals that patients can pull up on their cellphones when visiting an ER to verify what they need.

Patients such as Avant hope such steps can help decrease their ER waits while easing their anxiety about seeking emergency care.

“I don’t fear dying,” said Avant, “but I do fear dying in the hospital.”

KFF Health News is a national newsroom that produces in-depth journalism about health issues.

Hospitals Face Staff Shortages as Covid Surges

By Lauren Weber, Phil Galewitz and Andy Miller, Kaiser Health News

The Cleveland Clinic in Weston, Florida, on Jan. 11 was treating 80 covid-19 patients — a tenfold increase since late December. Nearly half were admitted for other medical reasons.

The surge driven by the extremely infectious omicron variant helped push the South Florida hospital with 206 licensed beds to 250 patients. The rise in cases came as the hospital struggled with severe staff shortages while nurses and other caregivers were out with covid.

The challenge is finding room to safely treat all the covid patients while keeping staffers and the rest of patients safe, said Dr. Scott Ross, chief medical officer.

“It’s not a PPE issue,” he said, referring to personal protective equipment like masks, “nor an oxygen issue, nor a ventilator issue. It’s a volume issue and making sure we have enough beds and caregivers for patients.”

Nationally, covid cases and hospitalizations are at their highest levels since the pandemic began. Yet, unlike previous covid surges, large portions of the patients with covid are coming to the hospital for other reasons. The infections are exacerbating some medical conditions and making it harder to reduce covid’s spread within hospital walls, especially as patients show up at earlier, more infectious stages of the disease.

Although the omicron variant generally produces milder cases, adding the sheer number of these “incidental” hospitalizations to covid-caused hospitalizations could be a tipping point for a health care system that is reeling as the battle against the pandemic continues. Rising rates of covid in the community also translate to rising rates among hospital staffers, causing them to call out sick in record numbers and further stress an overwhelmed system.

Officials and staff at 13 hospital systems around the country said that caring for infected patients who need other medical services is challenging and sometimes requires different protocols.

Dr. Robert Jansen, chief medical officer at Grady Health System in Atlanta, said the infection rate in his community was unprecedented. Grady Memorial Hospital went from 18 covid patients on Dec. 1 to 259 last week.

Roughly 80% to 90% of those patients either have covid as their primary diagnosis or have a health condition — such as sickle cell disease or heart failure — that has been exacerbated by covid, Jansen said.

Although fewer of their patients have developed pneumonia caused by covid than during the major spikes early last year, Grady’s leaders are grappling with high numbers of health care workers out with covid. At one point last week, Jansen said, 100 nurses and as many as 50 other staff members were out.

In one of New Jersey’s largest hospital systems, Atlantic Health System, where about half the covid patients came in for other reasons, not all of those with incidental covid can be shifted into the covid wards, CEO Brian Gragnolati said. They need specialized services for their other conditions, so hospital staffers take special precautions, such as wearing higher-level PPE when treating covid patients in places like a cardiac wing.

At Miami’s Jackson Memorial Hospital, where about half the covid patients are there primarily for other health reasons, all patients admitted for covid — whether they have symptoms or not — are treated in a part of the hospital reserved for covid patients, said Dr. Hany Atallah, chief medical officer.

Regardless of whether patients are admitted for or with covid, the patients still tax the hospital’s ability to operate, said Dr. Alex Garza, incident commander of the St. Louis Metropolitan Pandemic Task Force, a collaboration of the area’s largest health care systems. He estimated that 80% to 90% of patients in the region’s hospitals are there because of covid.

In Weston, Florida, the Cleveland Clinic is also having a hard time discharging covid patients to nursing homes or rehabilitation facilities because many places aren’t able to handle more covid patients, Ross said. The hospital is also having difficulty sending patients home, out of concern they would put those they live with at risk.

Hospital Infections

All this means there’s a reason that hospitals are telling people to stay away from the ER unless it’s truly an emergency, said Dr. Jeremy Faust, an emergency medicine physician at Brigham and Women’s Hospital in Boston.

The sheer number of patients who are showing up and don’t know they have covid during this surge is frightening, Faust said. As more incidental cases pour into hospitals, they pose a greater risk to staffers and other hospital patients because they are typically at a more contagious stage of the disease — before symptoms begin, Faust said. In previous covid waves, people were being hospitalized in the middle and later phases of the illness.

In Faust’s analysis of federal data, Jan. 7 showed the second-highest number of “hospital onset” covid cases since the pandemic began, behind only an October 2020 outlier, he said. But this data accounts for only people who were in the hospital for 14 days before testing positive for covid, Faust said, so it’s likely an undercount.

A KHN investigative series revealed multiple gaps in government oversight in holding hospitals accountable for high rates of covid patients who didn’t have the diagnosis when they were admitted, including that federal reporting systems don’t publicly note covid caught in individual hospitals.

“People in the hospital are vulnerable for many reasons,” said Dr. Manoj Jain, an infectious disease specialist in Memphis, Tennessee. “All of their existing underlying illnesses with multiple medical conditions — all of that puts them at much greater risk.”

The ER in particular is a potential danger zone amid the current crush of cases, Garza said. He recommended that patients wear high-quality masks, like a KN95, or an N95 respirator. According to The Washington Post, the Centers for Disease Control and Prevention is weighing whether to recommend that all Americans upgrade their masks during the omicron surge.

“It’s physics and math,” Garza said. “If you’ve got a lot of people concentrated in one area and a high viral load, the probability of you being exposed to something like that if you’re not wearing adequate protection are much higher.”

If patients can’t tolerate an N95 for an entire day, Faust urges them to wear upgraded masks whenever they come into contact with hospital staffers, visitors or other patients.

Dr. Dallas Holladay, an emergency medicine physician for Oregon’s Samaritan Health Services system, said that because of nursing shortages, more patients are being grouped together in hospital rooms. This raises their infection risk.

Dr. Abraar Karan, an infectious diseases fellow at Stanford, believes all health care workers should be mandated to wear N95s for every patient interaction, not just surgical masks, considering the rise in covid-exposure risk.

But in the absence of higher-quality mask mandates for staffers, he recommended that patients ask that their providers wear an N95.

“Why should we be putting the onus on patients to protect themselves from health care workers when health care workers are not even going to be doing that?” he asked. “It’s so backwards.”

Some hospital workers may not know they are getting sick — and infectious. And even if they do know, in some states, including Rhode Island and California, health care workers who are asymptomatic can be called back to work because of staffing shortages.

Faust would like to see an upgrade of testing capacity for health care workers and other staff members.

At Stanford, regular testing is encouraged, Karan said, and tests are readily available for staffers. But that’s an exception to the rule: Jain said some hospitals have resisted routine staff testing — both for the lab resource drain and the possible results.

“Hospitals don’t want to know,” he said. “We just don’t have the staff.”

Kaiser Health News is a national newsroom that produces in-depth journalism about health issues.

Minorities in Oregon Less Likely to Get Emergency Pain Care

By Pat Anson, PNN Editor

Oregon’s treatment of chronic pain patients came under fire this year when state health officials drafted a controversial plan to forcibly taper thousands of Medicaid patients on long-term opioid therapy. The proposal was scaled back after nationwide criticism from patient advocates and pain management experts, who said it would “exacerbate suffering for thousands of patients.”

Minorities in Oregon needing emergency treatment for pain may also be suffering unnecessarily, according to a new study by Portland State University researchers.

"We found evidence that the odds of receiving a lower quality of care from EMS providers are higher among racial minorities in Oregon, when compared to white patients in Oregon, after experiencing traumatic and painful injuries," said Jamie Kennel, a PhD student and lead author of the study published in the journal Medical Care.

PSU researchers analyzed nearly 26,000 health records of patients who received emergency care for traumatic injuries in Oregon between 2015 and 2017.

Only about one in five white patients received opioids or other types of pain medication from EMS responders in Oregon. But the odds were 32% lower for black patients, 24% lower for Asian patients and 21% lower for Latino patients. This was despite the fact that black and Latino patients reported higher average pain scores than white patients.

ODDS OF GETTING EMS PAIN MEDICATION

• White Patients 20.1%

• Latino Patients 17.2%

• Asian Patients 14.2%

• Black Patients 13.9%

"This is very large, concerning and should be motivating for change," said Kennel. "Like most healthcare providers, EMS providers don't desire to provide inequitable healthcare but often have never been exposed to evidence suggesting these disparities are taking place."  

The researchers also found that Asian and Latino patients were less likely to have their pain assessed – a simple procedure in which patients are asked to rate their pain on a scale of zero to 10.

While previous studies have found racial and ethnic disparities in medical care, this was the first to look at both pain assessment and pain medication during emergency care in a large statewide database. Researchers did not look at what caused the inequities in pain treatment, but speculated that racial stereotypes and difficulty in communicating with patients with limited English played a role.

“Although it has been shown conclusively that there are no medically significant biological differences between individuals of different races/ethnicities, there is evidence that medical providers nevertheless believe race/ethnicity to be a medically relevant factor and may be adjusting their clinical actions accordingly,” researchers concluded.  

"We hope that, when exposed to this new evidence, individual EMS providers will work with their agencies to better understand, and take steps to mitigate, this phenomenon in their community." 

A large 2016 study found that black patients who visit hospital emergency rooms are significantly less likely to receive opioid prescriptions than white patients. Opioids were prescribed for blacks at about half the rate for whites with back and abdominal pain.

Another large study of VA patients found that African-Americans on long-term opioid therapy were more likely to be drug tested and significantly more likely to have their opioid prescriptions stopped if an illicit drug was detected.

Landline More Important Than Cell Phone for Chronically Ill Patients

By Barby Ingle, PNN Columnist

I cannot stress enough the importance of having a traditional landline when people are turning to cell phones or internet-based phone systems (VOIP). In 2018, over half of American households were "wireless only homes."

People are switching for cost and convenience, but many are unaware of what they’re losing when they drop their landline. This is a topic close to my heart, because my own father would still be here with us if he had a working landline the night he passed away.

We see television shows where people use their cell phones to call 911 to report a fire or emergency. The fact is it doesn’t work that simply.

Major limitations are introduced when you call for help from a cell phone or VIOP system, which can leave a chronically ill person or someone in an emergency situation unable to receive help immediately.

As a pain patient whose husband worked outside of our home for years, this is something to know and take steps to remedy before something happens to you.

There are a variety of reasons to keep your landline. One would be quality. A landline gets a clear, reliable connection with virtually no delays or lag times. When I do a radio or podcast interview, they usually ask that I call on a landline. Audio issues on a radio show typically occur when a guest is calling from a cell phone or computer.

Calling 911 for help from an area with a weak wifi or internet signal can cause problems. If they can’t hear you clearly, that can delay getting help to you.  A landline almost always provides clear coverage because of the telephone network infrastructure in place across the USA. You don’t have to rely on spotty tower connections or a slow internet.

Many home alarm systems also use landlines, because even when the electricity goes out, the phone line won't. If the alarm system is hooked up through VOIP or a cell phone, there is no guarantee that the call will go through or the person will understand you.

During last year’s disastrous wildfire in Paradise, California, many cell phones were useless because phone towers were damaged or lost electricity. Residents without landlines couldn’t be warned about the approaching fire and over half of the cell phone calls to 911 failed.

Home Alone? Then Keep Your Landline

Having a landline to call in emergency situations is worth the expense. At an additional cost of about $10-20 a month you can have a basic landline active in your home so that you can call local numbers and emergency numbers such as 911 or 0.  

As a chronically ill person myself, I am home a lot. My cell phone service is not great at our house due to the rural area we live in. We use the traditional landline even for regular calls with our family, friends and of course in emergency situations.  

The biggest reason to have a traditional landline is safety. With a landline, you don’t even need to speak.

As long as you can get the phone off the hook and dial “O” for an operator or 911 for emergency services, they can listen in -- in case you are being robbed and need to be quiet or unable to speak for any reason.  

Another advantage of a landline is that your street address comes up automatically on a 911 operator’s computer screen. They know precisely where you are calling from.

That is not always possible with a cell phone. They may be able to determine what cell phone tower your call is being routed through, but they won’t know your exact location.

Imagine your child trying to call 911 because you are having a seizure or unconscious. If the child is too young to speak or remember your address in an emergency situation, a cell phone might as well be a toy.  

If you can only afford one phone line, make it a traditional landline if you spend most of your time at home due to chronic illness. Chances are when you’re out and about, others will have a cell phone and be able to call emergency services for you.  But when you are home alone, trust me, having a landline can save you time, money and perhaps even a life. This is a fact that my family found out firsthand with the death of our father.  

Barby Ingle lives with reflex sympathetic dystrophy (RSD), migralepsy and endometriosis. Barby is a chronic pain educator, patient advocate, and president of the International Pain Foundation. She is also a motivational speaker and best-selling author on pain topics. More information about Barby can be found at her website.  

Power of Pain: Making a Hospital Stay Easier

By Barby Ingle, Columnist 

Just as you should take a personal medical history to your doctor visits, being prepared for emergency room visits and hospital stays is also a good idea. A safe, smooth visit is exactly what you need when you are a chronic pain patient who is trying to heal or fight an illness.

I have unfortunately been to the hospital too many times now over the past 18 years. In the beginning, I did not go prepared. Nevertheless, through multiple visits, I have found a few things that allow me to get better treatment from the staff. 

For a better hospital stay, I now ask for a room in a quiet part of the hospital, as sharp and sudden noises exacerbate my pain. Most hospital rooms now have their own thermostat, so you can control your own temperature. If your room does not have its own, you can ask the nursing aide to assist with making you more comfortable, such as getting warm blankets.

When I am assigned a roommate, I ask for my bed to be farthest from the door so that their visitors don’t accidentally bump into me, and I can have less interruption with my resting. When possible, before their guests arrive, my husband or I inform them of my condition and how noise raises my pain levels. It is best to explain it to your roommate prior to his or her guest’s arrival so that he or she may explain it to them for better cooperation.

I have also learned to bring blankets and pillows from home. They are typically softer and my quilts are more comforting, both in warmth and as a reminder of home.

Most of the hospitals I have stayed in now offer an air mattress that can be used to adjust the bed to your preference. Comfort should be a big consideration so that you can heal faster.

It is almost unavoidable to not get poked with a needle as a patient in the hospital. They typically check blood at least once a day and use IV fluids to keep you hydrated. Medications are also administered with needles or through your IV. When they are drawing blood or putting in IV needles, ask for pediatric needles because any new trauma can cause RSD to spread to a new site.

If a person takes my blood and I find them to be supportive and cooperative, I have asked that they be the one to check my blood every time during that visit. I even had a nurse who agreed to come in to take my blood, even though she was off duty for one of the days I was in the hospital. I now have a “portacath” – a small catheter connected to a vein. I ask them to take blood draws from it for less needle poking.

While you are asking for better ways to get through a blood draw, have a nurse place a sign above your bed designating your affected limb(s). I had a nurse at the last hospital who put a red bracelet on my unaffected limb and a red sticker on my chart. This served as a good reminder to the nurse and aides as they walked into the room. They see multiple patients on your floor, and as patients come and go often, you want to stay on top of your care. Be your own “chief of staff” and employ the same practices at the hospital that you do with your regular doctors.            

I also bring to the ER and hospital a list of medications. Sometimes I have had to have my own brought in. I think it is good to have my own supply there so I can control when I take them. Otherwise, have the nurse check with the hospital pharmacy to see if they carry all of your medications. Nurses can’t always be there at the appropriate time to administer medications or help with other needs due to an overload of patients. Therefore, if you have your meds available, you can stay on schedule.

I also have found that the hospital has charged me for taking my own medications, even when I brought them from home, although the cost will be less than having them providing you the medication. This can also save you from mix-ups in medications by their pharmacist. When you are on pain medication at the hospital, make sure to not wait until it is worn off before asking for more. Hospital employees often times are taught to order your medications 30 minutes after you ask for them, so it could be 45 minutes or more before they actually arrive to your room from the time you ask for them. Keeping pain low is easier then lowering pain after it has skyrocketed again.                       

Something I do at home is keep items on the bedside table for easy reach and use. In the hospital, I use my tray table to serve the same purpose.  I have it placed in a position so I do not bump into it when resting, but it is close enough to utilize it for my things.  Also, if a nurse moves it to assist me or take blood pressure, I am sure to ask her to move it back into position when she is finished.

Healthcare institutions that are accredited to assess and treat your pain have been mandated to treat pain as the fifth vital sign.  You have the right to be taken seriously, believed and demand pain control. If you feel that your needs are being overlooked or intentionally ignored, ask to speak with hospital administration as soon as possible. Remember to be calm when complaining or they may not take you seriously. 

It never hurts to ask for things that can make your stay more enjoyable and comfortable.

Barby Ingle suffers from Reflex Sympathetic Dystrophy (RSD) and endometriosis. Barby is a chronic pain educator, patient advocate, and president of the Power of Pain Foundation. She is also a motivational speaker and best-selling author on pain topics.

More information about Barby can be found at her website.

The information in this column should not be considered as professional medical advice, diagnosis or treatment. It is for informational purposes only and represents the author’s opinions alone. It does not inherently express or reflect the views, opinions and/or positions of Pain News Network.