Crystal Evans lives in constant fear that bacteria will grow inside the silicone tube that connects her windpipe to the ventilator that pumps air into her lungs.
Now, though, those tasks have gotten infinitely harder. A shortage of medical-grade silicone and plastic used for the tubing means she must make do with just one new circuit each month. Since running out of new tracheotomy tubes early last month, Evans boils whatever she has to sterilize it before reusing, takes antibiotics to kill any pathogens that might slip through and hopes for the best.
“You just don’t want to risk infection and end up in the hospital,” she said, where she fears she would be exposed to a coronavirus infection that could prove fatal.
In a very real sense, Evans’s life has become hostage to supply chain disruptions caused by the pandemic and worsened by the demand for these same materials by busy hospitals. Those shortages have created life-or-death challenges for her and millions of chronically ill people, many of whom are already struggling to survive on their own.
Evans’s situation recently went from bad to worse, for instance, when she contracted a potentially life-threatening infection in her trachea, despite all of the precautions she had taken. She is now taking a last-resort antibiotic, which she receives as a powder that must be mixed with sterile water — another supply she struggles to get. “Every single little thing has been this way,” Evans said. “It’s on so many different levels, where everything just kind of eats into our lives.”
Making her plight and that of other chronically ill people more difficult is their desperate desire to stay out of the hospital, where they fear they could be infected with the coronavirus or other pathogens and suffer severe complications. Yet their needs get scant attention, partly because their cloistered lives make them invisible, and partly because they have so little purchasing clout compared with large health-care providers, such as hospitals.
“The way the pandemic’s been handled, so many of us are starting to wonder — do people not care about our lives?” said Kerry Sheehan, of Arlington, Mass., a suburb north of Boston, who has been coping with shortages of the IV nutrition supplements that enable her to live with a connective tissue disease that makes it difficult to absorb nutrients from food.
In the hospital, doctors can often find substitutes for unavailable supplies, including tubing, IV kits, nutrition supplements and drugs such as heparin, a commonly used blood thinner. But for people who manage their care at home, it is often a lengthy struggle to get insurance to cover an alternative supply, and going without can have serious consequences, say disability advocates.
“The big question throughout the pandemic, as covid has ramped up demands on the health-care system, is what happens when there’s not enough of something urgently needed?” said Colin Killick, executive director of Disability Policy Consortium, a civil rights advocacy organization run by people with disabilities in Massachusetts. “The answer, in all kinds of contexts, has been that disabled people go into the void.”
It is hard to know precisely how many people with chronic illnesses or disabilities living on their own, rather than in group settings, might be affected by pandemic-caused supply shortages, but estimates are in the tens of millions. According to the Centers for Disease Control and Prevention, 6 in 10 people in the United States have a chronic disease, and more than 61 million Americans live with a disability of some kind — including impairments in mobility, cognition, hearing, vision or the ability to live independently.
Experts say that medical supplies have been stretched thin because of both supply chain disruptions and increased demand in hospitals overwhelmed by covid-19 patients for months on end in some parts of the country.
David Hargraves, senior vice president of supply chain for Premier, which helps hospitals manage their services, said that there are always shortages of some medical supplies. But the scale of the current disruptions dwarf anything he has experienced before.
“Normally that might be 150 different items in any given week on back order,” Hargraves said. “That number today is north of 1,000.”
ICU Medical, the company that manufactures the tracheotomy tubes that Evans uses, acknowledges that shortages can pose a “significant added burden” for patients who depend on the tubes to breathe. The company said it is working to correct the supply chain problems.
“This situation is exacerbated by an industry-wide shortage of silicone, the primary raw material for producing tracheotomy tubes,” company spokesman Tom McCall said in an email.
“Material shortages in health care are nothing new,” McCall added. “But the stresses caused by the pandemic and current global supply chain and freight challenges have intensified them — both in terms of the number of products and manufacturers impacted and the length of time the shortages have been and will be felt.”
“I think this is a piece of this larger puzzle of people with disabilities being deemed less worthy of resources, less worthy of treatment, less worthy of keeping alive.”— Colin Killick, executive director of Disability Policy Consortium
Killick, who has motor dysgraphia, a condition that causes difficulties with the fine motor skills needed to brush one’s teeth or write by hand, said that in many instances during the pandemic, disabled or chronically ill people had greater difficulty getting supplies and medical care because of the increased public demand for those things. Early on, he recalled how patients with autoimmune conditions struggled to fill their hydroxychloroquine prescriptions because so many others were using the drug to prevent or treat covid, despite a lack of evidence that it would help.
“I think this is a piece of this larger puzzle of people with disabilities being deemed less worthy of resources, less worthy of treatment, less worthy of keeping alive,” Killick said.
Sheehan said she knows what it feels like to be marginalized. For years, the 38-year-old, who identifies as nonbinary and uses the pronouns “she” and “they” interchangeably, struggled to eat and maintain a stable weight as doctors struggled to explain why she was rapidly dropping weight, at one point shrinking to 93 pounds at 5-foot-7.
Eventually, a geneticist diagnosed her with a rare inherited connective tissue disorder called Ehlers-Danlos syndrome — which was exacerbated by a cervical spine injury after a car accident. After other treatment options failed, her doctor instructed her to get nutrition via intravenous fluids administered at home.
“He got me back to life,” Sheehan said. “I felt like I had my life back — I had energy.”
But with thousands of covid patients in intensive care units, hospitals began reporting shortages of the IV nutrition supplements. As cases skyrocketed this winter, a critical intravenous multivitamin that Sheehan uses every day also became scarce. Instead of getting seven doses weekly, she began receiving just three. Some weeks, she gets only two to stretch across seven days before the next shipment.
“Now I’m sleeping all the time,” she said. “I just don’t have enough energy, and I still don’t feel like I’m rested when I wake up.”
Sheehan said she has started to lose weight, and her muscles are atrophying like they did before she was diagnosed and began getting IV nutrition. “My body is eating itself,” she said.
Her life amid the pandemic has also gotten harder for other reasons. With mask mandates lifted, she is considering skipping the physical therapy that has kept her muscles functioning even with her limited access to nutrition — because of the heightened risk of infection.
“It’s going to make me give up the last few things that I’ve held on to,” she said, tallying the skipped family gatherings and visits to her beloved niece over the past two years. “Zoom only sustains you so much.”
Even before the pandemic, Brandi Polatty, a 41-year-old romance novelist, and her two teenage sons, Noah and Jonah, were often isolated from others in their home in Jefferson, Ga. They suffered from extreme fatigue, struggled to eat enough and sometimes felt too ill to work or go to school full-time because of a genetic mutation that inhibits their cells from making enough energy.
It took years before doctors used muscle biopsies and genetic testing to confirm they had a rare disease called mitochondrial myopathy caused by a gene mutation. After much trial and error, the family found that getting nutrition through feeding tubes and regular IV infusions that contain dextrose, vitamins and other supplements helped clear their brain fog and eased exhaustion.
To keep up with the life-changing treatments at home, between 2011 and 2013 the mother and teen boys each received permanent ports in their chests, sometimes called central lines, which connect a catheter attached to an IV bag from outside the chest to a vein near the heart. The ports make it easier to administer IV fluids at home because the Polattys don’t have to search for hard-to-find veins and push needles into their arms.
With the regular IV infusions, Brandi Polatty said she was able to avoid hospitalizations and support her family by writing romance novels. Jonah, 14, eventually got healthy enough to remove the chest port and his feeding tube; he now relies on oral medications to manage his disease. His older brother, Noah, 16, still needs infusions but felt strong enough to study for the GED, pass and enroll in music school to learn guitar.
But now, some of that progress is threatened by pandemic-caused limits on supplies of saline, IV bags and heparin, which Polatty and Noah use to keep their catheters free of potentially lethal blood clots and to avoid infections.
Normally, Noah would receive 5,500 milliliters of fluids every two weeks, in 1,000-milliliter bags. Because of shortages, the family has sometimes instead been receiving those fluids in much smaller bags, ranging from 250 to 500 milliliters. That means switching them more frequently, increasing the chance of introducing infection.
“It doesn’t seem like too big of a deal, right? We’ll just change your bag,” Brandi Polatty said. “But these fluids are going into a central line, and that blood is going to your heart. If you get an infection in your port, you’re looking at sepsis, usually an ICU stay. That’s what makes central lines so scary.”
Those ports also need to be flushed with saline and heparin.
“Every month, even if I don’t use my port, I have to flush saline and heparin,” Polatty said.
The risk of infection in a central line is a real and serious concern for people on such supportive therapies, said Rebecca Ganetzky, an attending physician at the Mitochondrial Medicine Frontier Program at Children’s Hospital of Philadelphia.
The Polattys are among many patients with mitochondrial disease who have faced tough choices during the pandemic because of shortages of IV bags, tubes and even the formulas that provide their nutrition, she said. Some of those patients cannot go without the hydration and nutrition support.
“If they can’t do that at home, they have to get into the hospital to do it,” she said.
Other supply chain disruptions have left disabled people without replacement parts for wheelchairs and other accommodations that allow them to live on their own.
Evans, the Massachusetts woman who relies on a ventilator, hasn’t left her home for more than four months after the wheelchair-accessible ramp outside her front door rotted beyond repair and had to be torn down in late November. Supply issues drove the price of materials beyond what she can afford on a fixed income, and her insurance offered only limited help.
As she has waited for prices to come down, Evans has had to rely on help from nurses and home health aides. But every time someone enters her home, she fears they could bring the virus in with them — and despite not being able to leave her home, she has been exposed to the virus at least four times by aides who have come to help her.
“The general public has no idea what a lot of us are dealing with through the pandemic, when they want to go out and live their lives,” Evans said. “But then they’re spreading the virus.”