Seniors with primary immunodeficiency require extra care

Seniors face unique challenges as they navigate a complicated diagnosis like primary immunodeficiency, according to Dr. Roger Kobayashi, a retired allergist-immunologist from Allergy, Asthma and Immunology Associates in Omaha, Nebraska, and a clinical professor at the University of California Los Angeles School of Medicine.

“Older patients tend to have complications and co-existing disease, which impacts and complicates PI,” said Kobayashi.

In his IDF presentation, Managing PI During the Senior Years, Kobayashi said seniors with PI face myriad barriers to maintaining their health as they:

• Cope with an aging body and mind

• Manage PI symptoms

• Coordinate treatment

• Keep up with primary care provider and specialist appointments

• Unravel complicated health insurance parameters

• Struggle to afford medical bills

• Live in isolation

• Navigate unfamiliar technology

“They are an emerging subgroup, little recognized and with formidable health problems,” said Kobayashi. “The enormous complexity of the healthcare system combined with complex illnesses and complicated healthcare reimbursement is difficult for the young to navigate, much less for the elderly.”

Aging takes a toll on a person’s physical health, explained Kobayashi. Typical health problems associated with getting older include high blood pressure, heart disease, diabetes, and arthritis, as well as mobility issues, poor eyesight, and loss of hearing. The immune system also ages, he said, resulting in a decrease in the number and function of T and B cells, increased infections like flu and pneumonia, increased risk of cancer, and increased autoimmune disease.

“With older people, their immune system is worn down. It doesn’t function as well, and there is an increased susceptibility to infections, poor response to vaccines, poor inflammation, and decreased tumor surveillance,” said Kobayashi.

A person’s mental capacity may also diminish as they age. A weakened memory and social isolation can cause an elderly person to forget medical appointments or not follow through with taking medication or treatments. They tend to rely more on caregivers for help.

“The elderly’s ability to self-manage is overestimated,” said Kobayashi.

An important factor preventing an elderly person from connecting with the care they need is technology. While it’s helpful for most of the population, for seniors, it can be confusing. Since most medical care and communications are internet-based, tasks like reviewing lab reports or reading doctor messages are difficult for older people.

“The elderly are in peril because they can’t navigate,” said Kobayashi.

Kobayashi said in 2015, he treated a 78-year-old woman who had a long history of severe lung infections, chronic bronchitis, bronchiectasis, and sinus disease and most likely had an antibody deficiency for over 20 years.

“This case bothered me, and I began to wonder, what’s going on? How many elderly patients are we following? How many were managing themselves? Was someone caring for them? Did they have other diseases, and how was that impacting the PID? What was their overall health status? Turns out, no one knew,” said Kobayashi.

Kobayashi joined a consortium of 22 private immunology practices nationwide that created a PI patient registry, which has 32,000 patients. In the registry, forty percent of those receiving immunoglobulin (Ig) replacement therapy were over 60 years old, and only 11 percent were 20 years old or less. Furthermore, in his own clinic in 2022, Kobayashi had 147 out of 283 patients on Ig therapy over age 65.

“This is mind-boggling,” Kobayashi.

Few studies exist on PI in the geriatric population, but that doesn’t make the condition rare in seniors, he said. It might be that all older people have an immune deficiency because their immune system deteriorates over time or that autoimmune diseases like inflammatory bowel disease or lymphomas may co-exist with PI, thus obscuring diagnosis, or that older patients tend to have complications from co-existing diseases that impact and complicate PI, said Kobayashi.

Future studies need to focus on factors such as:

• What is the incidence of PI in the elderly?

• What types of PI do they have?

• How many are receiving Ig therapy?

• How many are getting intravenous immunoglobulin therapy (IVIG) or subcutaneous immunoglobulin therapy (SCIG), and what are the risks of both?

• Is there a caregiver?

• Are they administering Ig therapy properly?

•  Are diseases like heart disease, diabetes, kidney failure, high blood pressure, and visual and mental impairment affecting their PI?

• How does the aging process impact PI?

Kobayashi said the long-term prognosis for seniors living with PI depends on the type of PI, the person’s illnesses, and most importantly, the presence of non-infectious diseases. If a person has an antibody deficiency alone and infections, the prognosis is good. If the person with PI has an autoimmune or immune dysregulation, then the outcome is not as favorable.

Ig therapy is critical to good health for a person with a PI who has antibody deficiency, but seniors must get past the roadblocks of health insurance coverage and the administration, monitoring, and distribution of the treatment. Healthcare providers should communicate with their senior patients to ensure treatment compliance, coordinate care between pharmacy, nursing, and infusion services, and monitor patients closely with the help of patient navigators and caregivers.

“We need to recognize that geriatric patients are a unique and growing group with special issues,” said Kobayashi.

Watch the presentation.