A New Kind of Normal

IMAGINE TAKING YOUR infant to the pediatrician for a routine mumps vaccine, only to have him contract the very disease you were attempting to avoid. What if your next child inexplicably stopped walking and standing following a polio vaccine? It sounds surreal, but that’s exactly what happened to Joanne Pease when her son Curtis and his younger brother Jeff were just babies. What Joanne didn’t know at the time is that both boys had an undiagnosed immune disorder that made them vulnerable to infection from normally safe live vaccines. Joanne later testified at Senate hearings to have live vaccines discontinued, although legislative changes did little to ease the blow for the Pease family, whose third son, Mitchell, was born with the same immune disorder. Thankfully, Mitchell was diagnosed early and able to avoid any additional health complications. Still, for this resilient clan, learning to live with chronic illness and its subsequent treatments has become a way of life — a new kind of normal.

Transitioning from Hospital to Homecare

The Pease siblings suffer from Xlinked agammaglobulinemia (XLA), a congenital immunodeficiency that occurs in about one in 250,000 males and is inherited in an X-linked recessive pattern. Females carrying the mutation pass it to their children. But, thanks to advances in medicine, many primary immune deficiency diseases like this one can be successfully treated. The mainstay of therapy is intravenous immunoglobulin (IVIG). “When I was young, I was sick all the time and nobody knew why,” recalls Curtis. “Finally, when I was 21 /2, I was sent to Children’s Hospital and accurately diagnosed. Once I started IVIG, my health improved dramatically within a matter of weeks.”

People with primary immune deficiencies like the Pease brothers suffer from chronic infections because their bodies lack certain kinds of antibodies. These important cells, also called immunoglobulins, help the body destroy germs that cause infections. IVIG temporarily replaces the immunoglobulins, or antibodies, that many immune-deficient patients are missing.

Curtis and his brothers depend on IVIG to maintain their health and quality of life. But where, when and how often they infuse has evolved over the years, based on their ages and preferences. Obviously, living with a chronic illness is never easy, and figuring out which treatment protocols are most effective is influenced by many factors, including insurance coverage, access to care and lifestyle of the patients involved. As young children, Curtis and his brothers received one shot of intramuscular IG every 10 days, but as they grew older, they began IVIG treatments that were administered in a hospital setting every three weeks.

The hospital infusions were effective but tiresome for active young boys, and Curtis’ father, Dan, decided to learn how to perform the infusions himself so that they could begin administering the treatments at home. Home infusions, whether performed by a nurse or family member, offer the patient privacy and comfort not available in a hospital setting, as well as the ability to plan infusion times around their own schedule. With three chronically ill boys to care for, the convenience of infusing at home was obviously appealing to the Pease family.

“When Dad first learned to do infusions, the nurse taught him at the hospital and then came out to the house for a week and let him practice on fake arms,” says Curtis. “The nurse supervised him for a while, but he got the hang of it very quickly; today, he’s better at finding a vein than most nurses at the hospital!”

From IVIG to Self-Administered Care

As the oldest of the Pease siblings, Curtis felt the pull of independence first. While he appreciated having his father administer the weekly infusions, he began desiring a greater level of freedom and control over his own healthcare. That’s when he made the decision to try subcutaneous immune globulin (SCIG) infusions.

The SCIG infusion, which is readily self-administered once a patient has been trained, offers an alternative infusion method to IVIG. It is also administered more frequently than IVIG. As a result, trough levels for SCIG patients often stay more even than those of IVIG patients. Curtis’ brothers complete their IVIG infusions in about 45 minutes every two weeks, whereas an SCIG infusion for Curtis takes up to two hours weekly. Curtis feels the autonomy and increased feeling of wellness he gets from this method outweigh the time differential. “The bottom line for me is that I hate needles, and while subcutaneous takes a bit longer, you can’t miss and there are no veins involved. For me, it is the best treatment option,” Curtis says.

Proponents of SCIG say the infusion method is relatively inexpensive to administer and can be performed by people without formal medical training (no need to worry about small air bubbles in a syringe, for example). People can perform SCIG infusions at home as part of homecare for family members, or, as in Curtis’ case, they can administer their own infusions.

Paying for immune globulin treatments can be prohibitive, and the Pease family has been fortunate that a series of job changes has kept them continually insured for the infusions they depend upon, while helping them avoid the insurance cap chronically ill patients dread. “I made it my business to learn as much as possible about insurance and to fight for our rights,” says Joanne. “When we wanted my husband to administer the boys’ infusions at home, for example, we had to request a case manager and convince our insurance company that they would save a lot of money by allowing us to make that change. In the end, everyone benefited.”

Thankful for Every Day

At age 27, Curtis has been living with chronic illness for more than 25 years. Because it’s all he’s known, he says he feels pretty “normal”most of the time. He makes his living as a cabinet refinisher and shares a house with his younger brotherJeff. And like a lot of young men, when he’s not working he enjoys sports and ridingATVs. “The only time I really notice that I’m sick is at the end of a workday when I get very tired,” Curtis says. “I’ve accomplished most of the things I’ve wanted to do because I refuse to let the disease limit me. I just wish I had more energy and stamina. Maybe future treatments will be able to address that issue.”

Although many patients like Curtis find encouragement through various support groups, Curtis says he’s had no need for one; his immediate family provides all the empathy, understanding and support he needs. “I’ve had this disease my whole life and so have my brothers. Jeff has polio on top of XLA, but he’s in remission and doing just fine. We are all fighters, and we don’t let this diagnosis get us down. At the same time, we’ve learned not to take anything for granted. We have a lotto be thankful for.”