By Cecilia Chan
GSN Managing Editor

A

shortage in plasma-derived drugs is causing anxiety among people like Gilbert resident Heather White, who depends on the blood protein to keep infections at bay.

The 41-year-old mother undergoes intravenous immunoglobulin therapy or IVIG, where once a week she gets an infusion of antibodies via her chest port at Ironwood Cancer & Research Center in Chandler. She was recently told by nurses there she had one dose of Gammagard, a sterilized solution made from human plasma, left before she is switched to a new brand.

“There are only two brands left that they can get and there is no guarantee that these meds will be consistently available for the foreseeable future,” White said. “I’m terrified. I’m absolutely terrified.”

Representatives for Takeda Pharmaceutical Co., the manufacturer of Gammagard, did not respond to a request for comment. The drug company was stepping up production and last year boosted its plasma collection but noted the medicine will still be in short supply for 2019.

There is no telling, however, if White will be able to take the two remaining brands.

“The different brands are formulated differently,” she said. “Some I can’t tolerate at all.”

Even so, Gammagard has to be divvied into four smaller weekly doses so White’s body can handle it. Typically people receive intravenous immunoglobulin therapy every three to four weeks.

Without treatment, White said, “I could end up with pneumonia and my body will succumb from not fighting off the illness.”

Intravenous immunoglobulin or IVIG is used for the treatment of other diseases, including Kawasaki disease, Guillain-Barre syndrome and lupus.

Gammagard and two other immune-globulin intravenous products are available but patient shipping schedules were being affected by high demand that was exceeding production, according to the U.S. Food and Drug Administration. An FDA spokeswoman could not explain why the increase demand was happening and said all the information she had was what was on the agency’s website.

According to the American Society of Health-System Pharmacists, nine brands are affected by the shortage of immunoglobulin. The five companies producing those brands all blame the shortage to an increased demand, the professional organization reported. 

Particular products may temporarily be in short supply but that doesn’t mean the immunoglobulin market is in peril, according to John Boyle, president and CEO of the Immune Deficiency Foundation.

“From what we are seeing, we’ve been dealing with this for a while,” Boyle said.

He said last year the shortage was first seen impacting specialty pharmacies and the patients who were taking vials of immune globulin in their homes.

“I think that has stabilized to a point,” Boyle said. “But in the last several weeks I’ve got several calls that it’s hospitals with out-patient or in-patient use of immunoglobulin for on-label patients like primary immunodeficiency and common variable immune deficiency (that are seeing the shortage).

“It seems to be shifting to these hospitals now grappling with this, which brings a whole new patient population being told the product we need is not on hand.”

Boyle attributed the shortage to a number of factors, including more people being diagnosed with disorders where immunoglobulin is the prescribed treatment and the medical community prescribing it off-label or without specific approval from the FDA.

“Immunoglobulin and other plasma products are being used more and more for off-label and investigatory reasons,” Boyle said. “You may have noticed Selma Blair posting about the condition she has, which immunoglobulin is sometime used. It’s not on-label but being explored so plasma products such as immunoglobulin are incredibly impactful for a whole host of conditions.”

The actress Blair was taking IVIG treatments to treat her multiple sclerosis, which reportedly are used in multiple sclerosis patients but is an expensive treatment and not always a preferred route.

“Unlike blood donations where, ‘OK, we have a blood donation center (and) we get some people to donate,’” Boyle said. “You can’t do it with immunoglobulin.”

Drugs derived from human plasma take nearly a year to produce, not like a pill where a company can add an extra shift of workers and churn out product, according to Boyle.

In the past the supply and demand curves have worked, but with the slight increase in demand, everything is being thrown off, he said.

The bottom line is product is tight but there is a supply out there, according to Boyle.

For instance, he said Hospital A may have plenty of immunoglobulin but Hospital B may not and there’s no communication between them.

So what happens to people such as White, who could be told later there’s no more IVIG available?

“That’s the million-dollar question,” Boyle said. “I am a patient myself. We use it for antibody replacement. We don’t make antibodies so we get immunoglobulin. We are going to get sick, we are going to get an infection a normal person can fight off with their antibodies. We require that protective level of immunoglobulin to stay alive.”

He said patients being told there is no more supply, need to contact his organization and to be their own advocate and work with their insurance company to switch to another hospital that may have IVIG. 

“System A might not have it but System B probably does,” he said.

He said every company beside Takeda are ramping up production.

“Every one of these companies has been adding new (plasma) collection centers and Takeda opened a new plant in Georgia to increase (production) capacity,” Boyle said. “Every gram they put out is getting absorbed somewhere into the distribution system.”

Boyle added U.S. donors supplied the bulk of the plasma donations in the country, most of Europe and a big chunk of the rest of the world.

He said the shortage can last into the foreseeable future and that it was incumbent on the hospital system to be better stewards of immunoglobulin and dispense it for on-label uses only.

Patients who depend on the drug can’t afford for them to do otherwise.

“Everyone in the medical community has to be very thoughtful and they may not have realized the supply was so tight but now that they are dealing with that, it can’t be business as usual,” he said. “They have to think about people who need it the most. “

Lately, White has been asking people to donate plasma via social media.

“I’m not usually the one to reach out to ask for help for stuff,” she said. “For the last 10 days I’ve literally been talking nonstop bout plasma donation and how important it is. Already four people have gone in to donate plasma.”

White’s reliance on IVIG began more than a decade ago.

In 2004 while on her honeymoon in Europe she got sick and was diagnosed with pneumonia when she got home. She was given antibiotics and told to come back in two weeks for a check.

“There was scaring in my lungs and they sent me to a pulmonologist,” White said. “I got bronchitis four times in six weeks so I had more in-depth blood work.”

A diagnosis came back: she had common variable immunodeficiency or CVID, which means White has low levels of the proteins that help fight infections.

White suspected she’s had the disorder since childhood as she recalled fighting off bronchitis and pneumonia every single time she had a cold.

In most cases, the diagnosis of CVID is not made until the third to fourth decade of life with permanent damage to the respiratory tract sometimes occurring from severe and repeated infections, according to the American Academy of Allergy, Asthma and Immunology.

The exact cause of CVID is unknown for the most part but it is the most common form of primary immunodeficiency disease and increases a person’s risk for certain cancers such as lymphoid and gastrointestinal, the professional membership organization reported.

Because of her disorder, White is receiving disability benefits.

“I can’t work because I can’t be around large groups of people for an extended bit of time,” she explained. “I carry a mask and hand sanitizer with me and I try to limit my exposure as much as I can.”

She said her 18-year-old daughter has grown up with a mother who was sick all the time.

An estimated 1 in 25,000 to 1 in 50,000 people worldwide has CVID but experts believe the numbers are much higher because they say the disorder is often misdiagnosed.

There is no cure, instead patients like White are treated with immunoglobulin replacement therapy, which most often relieves symptoms. The therapy must be given regularly and life-long.

“All we can do is encourage people to donate plasma,” White said. “It’s scary to think a baby with Kawasaki disease can’t get it and needs it to save his life.”