Like so many PI patients, I was diagnosed later in life, at age 49. I received a diagnosis of common variable immune deficiency (CVID) after years of dealing with the hallmark symptoms of PI: recurrent ear, sinus, respiratory, skin and urinary tract infections, and systemic inflammation. I then developed recurring inflammatory breast disease. My surgeons were perplexed and giving up hope. In one terrible year, I underwent three surgeries and two hospitalizations. Although I did not have cancer, I required a mastectomy and, as a result, I had to stop working because my body was so taxed.
The explanation for my infection and fatigue finally came when I was working as a clinical director of operations for a home care company that subcontracted intravenous immunoglobulin (IVIg) nurses for specialty pharmacies in South Florida. I realized that the clinical paths of many of the patients I encountered had a similar history to my own. I immediately contacted a prominent clinical immunologist who, after seeing me, initiated the three-month diagnostic process which confirmed the primary immunodeficiency: CVID. Confirmation of my suspicion was still a shock, but I was relieved, because finally there was validation of the root cause and available treatment for all that stuff that was going on in my body. Thus began my PI treatment journey.
My first treatment order was for IVIg infusion administered in my home. Unfortunately, my insurance company did not authorize this treatment, and I was referred instead to a hospital for treatment. There were several problems with this, from my perspective. The particular hospital was chosen because that was where my physician had hospital privileges. It happened to be a 45-minute drive from my home, under normal conditions. But I had to be there at 7 am, which meant traveling during rush hour. I would check into the hospital first thing in the morning, then have to check in again at the infusion center. Then they would have to order my product, so I would have to wait another half hour before actually beginning to infuse. I was in the chair for 7-8 hours. I generally did not check out of the hospital until 5 pm, then had to drive home, again during rush hour. It was just a very long ordeal that took the entire day, and for me, a horrible experience. I did this for 8 months. Then, because I was scheduled for neck surgery, I was allowed to transition my treatment to my home.
When patients are deciding about therapeutic options, I think it is important to understand how infusion centers typically operate. First of all, when working with an infusion center, you have to live with their policies and schedules – even with their physical layout. Some have private areas for treatment, but most are set up in a small room with as many chairs as will fit. You can be packed in with many other patients. It can be very loud; it can be difficult to get food. In addition, many infusion centers are set up to serve oncology patients and may not have much infusion experience relative to IVIg. This is important because many reactions to IVIg infusions are rate dependent. Without IVIg experience, staff may be infusing too much product at too rapid a rate. Their goal is turnover – the faster they can infuse, the faster they can get someone else into that chair. In my case, the infusions were very lengthy. Because I have particular sensitivities, I often had reactions, for which the staff was not adequately prepared. It was all far less than ideal.
For me, getting infused at home was also not without its challenges. Basically, you are letting a complete stranger into your home. This is especially troubling for many during a pandemic. Where has this person been? Does he or she wear a mask? Should you wear a mask? Beyond issues related to COVID-19, is this person respectful of your needs, your time, and your home? You may be lucky enough to develop a good personal relationship with your nurse, but that is not always the case. You may get a different nurse every time, so there may be limited consistency in practice.
In my experience, many nurses were not adequately trained for IgG infusion. For example, I went to sleep during one infusion, and the nurse turned up the rate to complete the infusion more quickly. I had a near anaphylactic reaction as a result. The nurse panicked and called 911. Two other times I had aseptic meningitis resulting from infusions. At that point, I “fired” the nurse. Bottom line: I felt like I had to watch what the nurses were doing during every infusion. I don’t think patients should be put in that situation. You should be able to count on standard practice and quality of care.
I think it is critical that patients have an open discussion with their providers about what options are available to them and speak up about what they would prefer – home or infusion center administration. It is important to understand all the decision criteria in evaluating the success of a treatment — what metrics will determine dose and frequency? How often will this be evaluated? At what point should changes be made? You have to be able to figure out exactly what works for you – both physically and in terms of lifestyle. Ultimately this will increase compliance and ensure the best outcome for you.
Because of my particular physical make up and insurance situation, I have tried many different IVIg products. When I look back over that journey, I see that some of my decisions to change product were motivated by my body and how it responded to particular product formulations – stabilizers used, concentration, and so forth. Some changes were motivated by rate and volume of administration required and what my body could absorb. Some were motivated by insurance coverage. And some were motivated by nurse error and avoidable adverse reactions.
We are all different. With IVIg, or any infusion product, it’s important to find a formulation you can tolerate. You need a doctor who will allow you to explore – who is willing to ask an insurer for an authorization, or who is willing to make product or dose changes. It can be very labor intensive for the provider, so it is ideal to find a “willing partner”.
Finally, if you are working or you have a life, you have a right to convenience. Why should your treatment take such a huge amount of time? Is it centered around what is convenient for the nurses rather than for you? You have to be very assertive. It is your life, health, and schedule that is being impacted.
I’ll just come right out and say it: SCIg is my preferred therapeutic option.
Doing subcutaneous therapy, you have real power and control. You are in the driver’s seat. It makes the whole infusion process less of a big thing. You can infuse where and when it is good for you. You are not dependent on anyone else. Want to infuse after work? Fine. Need to schedule around an event? Yes, make the adjustments you need. Got a trip planned? No problem. The flexibility of SCIg lets you have as much of a normal life as possible. You are in charge.
Of course, you will still need to work closely with your doctor to find the right product formulation for your needs and work together to make any adjustments required. But to my mind, managing the whole process with SCIg is just so much easier.
I know that change can be very difficult. It can require a tremendous amount of time and energy. As the old adage goes, “the devil you know may be better than the one you don’t.” Sometimes it can just feel easier to leave things as they are. Added to that, people with PI, or any chronic condition for that matter, can easily feel powerless. I am here to tell you that you have the right to actively manage your care, to understand and explore options, to push for medications you think best for your needs, to set your own schedules, to ask for a new nurse or doctor, to expect consistency of practice and quality of care, and to make requests of your insurance provider.
I have a few recommendations:
This material represents individual patient experience living with a primary immunodeficiency. This content is not intended to be a substitute for professional medical advice, diagnosis or treatment.
The page contains general medical information that cannot safely be applied to any individual case. Medical knowledge and practice can change rapidly. Therefore, this page should not be used as a substitute for professional medical advice.