Shortage of life-saving drug leaves immune-deficient patients in 'terrifying' spot: 'no replacement'
After enduring extensive fertilization treatment and several difficult pregnancies, including a miscarriage and a stillbirth, Illinois law professor Sonia Green eventually grew the family she had so yearned for.
But now, as the mother of four sons aged 16 to 20, she’s been thrown yet another medical curveball: watching three of her boys struggle with a rare immune disorder — and facing a devastating shortage of the life-saving drug, called immune globulin (IG), that they and others like them so desperately need.
“When Harrison was diagnosed, we had to wrap our heads around the fact that we have this baby that we've waited for for so long, but he has this condition,” Sonia, of Chicago, tells Yahoo Lifestyle about when she and husband Colby learned of the disorder affecting her oldest boy. “We were able to keep going because we discovered there's this life-saving medicine. It's not casual — it affects our lifestyle, it takes priority. Everything gets scheduled around [their infusions].”
The three affected sons — Harrison, 20, Holden, 16, and Davis, 15 — live with a primary immunodeficiency called X-linked agammaglobulinemia (XLA), a group of inherited immune deficiencies that leave those affected unable to produce antibodies, making them highly vulnerable to infections. Luckily, IG infusions give those with immune deficiencies the cells that their bodies don’t produce naturally.
“Immune globulin provides antibody replacements,” John Boyle, president of the Immune Deficiency Foundation, explains to Yahoo Lifestyle. “If it were not for immune globulin, any pathogen — even the slightest cold — could be fatal or problematic.”
It’s why the brothers are so relieved that the treatment exists. “Without IG, I wouldn’t be able to photograph, or attend school, or do any of the other activities that I love,” Holden an avid photographer, tells Yahoo Lifestyle. “I am grateful to have a treatment for my condition.”
But the current shortage makes her feel like the “the rug could be pulled from under us,” as the Green boys’ ability to have healthy, active lifestyles hinges on getting that monthly infusion of IG — and without it, Sonia says, their lifestyles would have to change drastically to limit their exposure to individuals who could unknowingly pass on a life-threatening infection.
“If they went longer than that— even six weeks or eight weeks — then we're really rolling the dice with their health. It's terrifying to think, but just take one bout of pneumonia could kill them,” Sonia says. “It would be pretty devastating if these shortages continue — even now we're on an unsafe level.”
The U.S. manufactures nearly 70 percent of the world’s IG products, according to Boyle, and has been unable to keep up with the worldwide demands for IG products as the medicine is now used for a growing variety of off-label treatments — something that has contributed to a 66 percent increase in its distribution from 2012 to 2018 across North America and Europe. While the U.S. Food and Drug Administration first reported shortages in October 2018, they have reached such precipitously low levels that the FDA recently issued a warning.
“FDA monitoring confirms that, despite increased supply of immune globulin (IG) products in recent years, the demand for IG product has also increased over that same time and there is an ongoing shortage of Immune Globulin (Intravenous) (IGIV) and Immune Globulin (Subcutaneous) (IGSC) products in the United States. This shortage could impact patient care,” the warning reads.
“In the setting of the increased demand for IG, other factors that ordinarily might not impact availability may have a greater effect. These could include uneven product distribution across different localities, logistics of contractual obligations, production delays, and other factors.”
According to Boyle, thousands of immune deficiency patients have already been affected; while those who use the IG for off-label uses are being forced to turn to alternative therapies or reduce or change the type of IG product they take (depending on the availability), others have been unable to get any at all — and for those using it for its intended immune-deficiency treatment, it’s a dire situation that could still get worse.
“This is not a classic drug shortage. If anything goes wrong with one plant, there’s a massive ripple effect, because it’s a very fragile supply chain,” Boyle explains. “If you don't have this therapy, as far as antibody deficiencies go, there is no replacement — there is no pill, there is no other therapy. We need antibodies and there's just nothing around it.”
Manufacturing IG is a complex medical process that is dependent on individuals donating their plasma at specialized centers. While the process is similar to that of donating blood, there are more obstacles for potential donors, including additional medical screenings and tests.
While the FDA has made recommendations on how to handle the shortage, the department “does not have legal authority to require a manufacturer to begin producing, continue producing, or increase their production of drugs,” its website statement reads. It adds that when faced with dwindling or limited supplies, healthcare providers “may be required to decide which patients will receive priority treatment.”
Sonia says they’ve been “kind of lucky” that the boys’ IG dosage has not yet been impacted by the shortage — but that it feels imminent every time she picks up their medicine at the infusion pharmacy.
“We’re told that due to the shortage, ‘your medicine will come in a different-sized bottle.’ We’re given a warning that ‘we may not have enough to send you.’”
If the IG shortages worsen, she says she’d be forced to pull Holden and Davis from their public schools and be forced to avoid places where they can’t monitor their contact with people carrying unknown pathogens. While she would let Harrison decide whether or not to take leave from college, she feels that he would “make the right decision to protect his health.”
Beyond “halting them in their tracks,” Sonia fears the shortages of IG could have lifelong consequences for her sons, even impacting any future careers.
“For my sons, we would have to rethink college plans, rethink career choices even, because if this continued through their lives, they will be really limited in what they can do,” says Sonia. “They would need to look at professions where they're not really interacting with people.”
The Greens and the Immune Deficiency Foundation have been working to encourage more people to donate their plasma, one of the few things they can do in the wake of the IG shortages. For as many as 55,000 primary immunodeficiency patients whose lives rely on IG, there’s a “very legitimate fear,” says Sonia.
“We’ve been told we can’t get the medicine, and people are scared,” she says. “If the shortages reach a critical level, I think this is really the only thing that I would be thinking about. How do we keep them safe? How do we keep them from dying? Not to be too dramatic, but I think that's about how stark it would be.”
Read more from Yahoo Lifestyle:
How families of children with cancer cope with shortages of life-saving drugs: ‘It’s terrifying’
Schools nationwide are offering mental health days to address growing issues among teens
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