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Living with primary immunodeficiency (PI) as a senior adult, aged 65 and older, is becoming more common as awareness of PI grows, treatments advance, and people with PI live longer. As a senior with PI, you face unique challenges. Not only do you manage your PI, but you may also navigate other health issues and hurdles related to mental health and health insurance.
If you are a senior with PI, knowing what to expect will help you better manage your condition and overall health. It’s important to be proactive, stay organized, communicate with your healthcare providers, learn as much as you can about your condition, and ask for help when you need it.
As in other age groups, common variable immune deficiency (CVID), an antibody deficiency, is the most common diagnosed PI in senior adults [1]. Some seniors have had the diagnosis for decades, but in other cases, people get diagnosed in their 60s and beyond. In these cases, their symptoms are either new or were overlooked when they were younger [2].
People are rarely diagnosed with other types of PI as senior adults, like chronic granulomatous disease (CGD), Wiskott-Aldrich syndrome (WAS), or CD40L deficiency. Typically, people with these PIs are diagnosed and receive treatment with bone marrow transplant (BMT), gene therapy, or other specialized medicine as children or young adults. Healthcare providers continue to monitor these individuals as they get older. Currently, there is not much known about how people with these other PIs age, in some cases, because they have only recently begun living into senior adulthood. Their conditions are more complex than antibody deficiencies and may have more complications.
Diagnosing PI in seniors can be challenging. Many healthcare providers are unfamiliar with PI, consider it rare, and hold the misconception that PI only happens in infants and children.
In seniors, PI may not be as obvious as in younger individuals. The immune system weakens naturally as people age, even if they don’t have PI. Repeated use of antibiotics or surgical procedures, such as sinus, ear, and lung surgery, can mask symptoms. Seniors may also have existing autoimmune, chronic inflammation, or cancer diagnoses that are being treated with medications that suppress the immune system (immunosuppressants). If healthcare providers do not test a person’s immune system before starting these medications, they may attribute infections or immune system problems to the medications instead of considering PI.
Delays in diagnosis can result in recurrent health problems that become more difficult to reverse as a person ages. Some seniors with PI stay active and have a good quality of life, but others develop health complications.
If PI goes undiagnosed or undertreated, infections, autoimmunity, or chronic inflammation can lead to permanent organ damage. Even after diagnosis, some health issues may continue to get worse. Be aware of health conditions that are common complications of PI and work with your healthcare provider to maintain treatment.
Common lung issues include [3,4]:
Early diagnosis and treatment can slow these conditions, but some may eventually require surgery or use of supplemental oxygen. Make sure your healthcare provider monitors you for these conditions and tell your provider about any new respiratory symptoms.
Gastrointestinal (GI) issues, or problems with your digestive tract, are also common [5]. These infections can cause:
Cancer in general is more common with age. Some cancers are also associated with certain types of PI, especially CVID and WAS [6]. Make sure your healthcare provider monitors you for blood cancers like lymphoma, myeloma, and leukemia and for GI cancers if you have had certain GI infections or conditions.
Cancers or blood disorders may lower antibody levels, which can cause secondary immunodeficiency. Chemotherapy and other cancer treatments can further weaken immunity. It is important to check your IgG levels before cancer treatment to understand whether low Ig levels are due to PI, cancer, or treatment.
Discover common respiratory conditions in PI, treatment options, and everyday ways to support long-term lung health.
View webinarWhile you can’t prevent all complications, follow these recommendations to take control of your health:
Your healthcare providers should do the following health monitoring regularly to help catch any problems early and maintain your quality of life:
Also be aware of other chronic conditions that are common in seniors but are not necessarily related to PI, including [10]:
The organization’s Clinician Finder service provides you with a personalized list of specialists suited to your needs.
Find a specialistHealth problems that come with age, like diabetes, chronic kidney or liver disease, nerve damage, dementia, arthritis, and high blood pressure, can affect Ig replacement therapy. Providers must consider Ig dose, how often it’s given, how it’s given, which product to use, and where treatment will happen (ex., at home or at an infusion center).
The two main options for infusing Ig are intravenous immunoglobulin (IVIG) replacement therapy (through a vein) and subcutaneous immunoglobulin (SCIG) replacement therapy (under the skin). With both IVIG and SCIG, seniors generally should receive lower doses of Ig at a slower infusion rate. Beginning with a lower dose minimizes rare but serious side effects like kidney failure and blood clots, particularly for those with other health problems. Healthcare providers can then increase the dose if needed.
Some senior adults choose IVIG because infusions are less frequent, and they do not have to self-infuse. Going to an infusion center for IVIG also lessens social isolation for some senior adults.
However, the supervising healthcare provider and infusion center or home nurse need to have experience with IVIG infusions in senior adults. Large volumes of Ig, if infused rapidly, could have dangerous side effects. IVIG can also increase blood thickness (viscosity), causing clotting and poor kidney function. Challenges with IVIG in senior adults include:
Those with kidney disease are at particular risk of serious side effects. However, all senior adults have an increased risk for blood clots, including inflammation of surface veins (thrombophlebitis), heart attack, stroke, and clots in the lungs (pulmonary embolism). The risk is higher in individuals with other blood-clotting risks or previous clotting events, particularly those with limited mobility. Healthcare providers should measure Ig serum levels, check kidney function, and adjust Ig therapy if needed.
Other seniors choose SCIG and self-infuse at home because it’s easier, has a lower risk of serious side effects, and is covered by their health insurance. SCIG may be necessary for seniors with kidney disease, severe heart disease, clotting disorders, hypertension, or other conditions. According to research, senior adults, even those on blood thinners (anticoagulants), can tolerate and self-administer SCIG safely [11].
While SCIG may be convenient for some, it may cause problems for seniors with physical limitations. SCIG self-infusion requires several steps, which may be hard for those with arthritis, poor eyesight, muscle weakness, or nerve damage. Often, a caregiver needs to help with the infusions. Note that, unlike IVIG, Medicare generally does not pay for nursing support for SCIG after initial training sessions. Exceptions include facilitated SCIG or when an individual has documented manual dexterity issues.
Besides Ig, other medications might need to be adjusted for seniors because the body’s sensitivity to and ability to break down medications change with age [12]. Healthcare providers should consider chronic conditions besides PI, kidney and liver health, and chronic infections.
Adjusting medication for age is especially important for seniors who take many medications daily, prescribed by different healthcare providers. Even with electronic medical records and pharmacies keeping track, seniors can still be hurt by mistakes with medicine. It’s important for you or your caregiver to know what medicines you are taking and their side effects. Don’t hesitate to ask your healthcare provider or pharmacist questions.
This guide was developed for patients and caregivers to help increase understanding of immunoglobulin (Ig) replacement therapy.
Download or orderThis regularly updated booklet contains detailed information on all Ig products approved to treat PI in the U.S.
Download PDFSeniors living with PI face unique mental health challenges alongside their medical needs.
Depression is common in seniors managing chronic conditions like PI. Someone with depression may feel sadness, anger, hopelessness, or diminished interest in activities. The physical limitations, diminishing independence, and social isolation that many older adults experience can make these symptoms worse.
Another mental health issue seniors may experience is anxiety. Concerns about symptom flare-ups, infection risk, hospitalization, or long-term prognosis can lead to chronic worry. Some older adults may also have emotional trauma from medical experiences or be extra cautious because of their PI.
Seniors can also face cognitive decline and brain fog. These issues make it difficult to concentrate or stay focused, make decisions, or remember details. Chronic illness, pain, and medications can contribute to memory difficulties, slowed thinking, or difficulty paying attention. These changes can be frustrating and may affect daily life.
Loneliness and grief can also be problems for seniors, especially if they live alone. Older adults might limit social engagements to reduce infection risk, but this can end up increasing their isolation and reducing their available emotional support. Senior adults may also feel a sense of loss as they miss their old life, family role, or career and accept that their health and independence have limits.
Even though challenges may seem overwhelming, you can take steps to support your mental health.
It’s a good idea to see a mental health professional if:
Seeking help is a sign of strength, not weakness. Therapists, support groups, and medical providers can work together to create a plan that supports both your mind and body.
If you or someone you know is struggling or in crisis, call or text 988 or chat 988lifeline.org to reach free and confidential support 24/7 from the 988 Suicide & Crisis Lifeline.
Adults 65 and over are eligible for Medicare coverage and should discuss the different plan options with a trusted advisor [13]. Medicare offers many options, and understanding a plan’s coverage is important. Choosing the wrong plan can impact both your health and finances.
Your options are:
-OR-
Based on the experiences of senior adults with PI on Ig or other specialty medications, original Medicare with Part B, Part D, and a Medigap plan (if needed) provides coverage for most medications. Medicare Advantage plans can set their own formularies, so they may not cover all Ig products. People with Medicare Advantage plans have reported that most cover only 70-80% of the costs for Ig replacement therapy. Because those with Medicare Advantage are not eligible for Medigap plans, people on Medicare Advantage plans can end up paying significantly more out of pocket for Ig therapy.
Note that people with original Medicare and Medicare Advantage have reported problems getting Ig replacement therapy during stays in a skilled nursing facility (SNF). SNFs receive a single bundled payment under Part A, which is not enough to cover the cost of Ig therapy. As a result, many SNFs do not accept patients who need Ig therapy or refuse Ig therapy once someone is admitted.
Medicare coverage is complicated. Since Medicare plans vary from state to state and even by counties within a state, you need to research what plans you are eligible for. Contact your State Health Insurance Assistance Program (SHIP) to find trained counselors who can tell you which plans you are eligible for and assist you in finding the answers to your coverage questions [14].
Use this flowchart decision tree to compare your options for Medicare coverage of immunoglobulin (Ig) replacement therapy.
Download flowchartMedicare enrollees face increases in premiums and deductibles as they apply for the federal health insurance program.
View articleThis page contains general medical and/or legal information that cannot be applied safely to any individual case. Medical and/or legal knowledge and practice can change rapidly. Therefore, this page should not be used as a substitute for professional medical and/or legal advice. Additionally, links to other resources and websites are shared for informational purposes only and should not be considered an endorsement by the Immune Deficiency Foundation.
Adapted from the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases, Sixth Edition.
Copyright ©2019 by Immune Deficiency Foundation, USA
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