A Drug-Induced Reflection, So To Speak -

Three days ago, my mom began yet another drug – this one administered through subcutaneous infusion therapy at home. Before getting into the exasperating road that led to this situation, now is a good time to reflect on the overall topic of medication and share some insight.

Along my mom’s Systemic Sclerosis (Scleroderma) journey there have been countless situations that have led to a myriad of drugs being prescribed, whether in an acute hospital setting or as an outpatient. “Myriad” is a gentle way of saying, I can only begin to tell you how many drugs (50, 75, or more), what they were, what the situation was, and what benefits (or harm) they brought to her.

To this day, in each situation, there may or may not be time to thoroughly research the pros and cons of a drug, let alone how it applies to my mom’s overall healthcare. This is especially true when there’s a serious health issue that requires quick decision-making. Complicating the process sometimes are drugs with FDA black box warnings, which communicate potential rare but dangerous side effects, and/or important instructions for safe use.

When drugs are prescribed, like many folks these days, I do a Google search to learn as much as possible. This typically starts by using a website such as Mayo Clinic, Drugs.com, or others that provide extensive information on drugs; description, indications, drug interactions, proper use, dosing, etc. I also check patient forums on sites such as WebMD to see what actual users of the medications have to say. Although each patient’s situation is unique for many reasons and may not apply, there’s a lot to be learned from them and experiential information can help formulate questions for the care team. From there, time and access dependent, getting input from healthcare professionals outside of the care team is beneficial. I’m a big believer in gathering information and perspectives to become more informed.

Of course, to be fully armed with a high level of research, it takes time. In a chronic situation that’s possible where a new drug is being presented, but in a hospital setting there’s rarely (if ever) enough time to obtain a lot of information when decisions need to be made on the fly. Considering my mom’s 17 hospitalizations (100+ days) and countless outpatient experiences, I’ve gained more knowledge than ever imagined about the best ways to gather, understand, and communicate drug information related to specific medical conditions.

Overall, the determination of what drug to use isn’t always simple and straight-forward. This is because each person has their own unique healthcare situation, set of personal circumstances, level of risk tolerance, insurance coverage, financial resources, etc. Those factors notwithstanding, the administration of medication is often an art, as much as there is science behind each drug.

You have a headache –> take a common low-risk drug like Tylenol.

You have Hydralazine-induced vasculitis –> begin infusions of high-risk Rituxan, with a black box warning that includes a slew of potentially dangerous side-effects and outcomes.

The later and everything else I’ve shared above brings me back to my mom’s road to the subcutaneous infusion therapy that began this week. How did she end up here? Well, let’s take a look and follow the Bouncing Rx.

Amidst everything my mom has experienced since June 2018 with Systemic Sclerosis, one of the main challenges at times and especially early on, was regulating her blood pressure. That’s common with the disease and especially in someone like her who initially had associated renal crisis with subsequent challenges of borderline Stage 5 kidney disease.

During the first year, there were various blood pressure medications prescribed. It was a nuanced situation and choices were limited due to her kidney issues. One that was given immediately in June 2018 was Captopril, which continues to this day and has enabled my mom to be alive. It’s indicated for people who have Systemic Sclerosis-induced kidney disease and has caused no complications. That drug alone wasn’t enough during the first two years, in part due to constant hospitalizations that led to recalibration of her system after each discharge. At times she was on three to four (possibly even five) medications specifically to control her blood pressure.

Bouncing Rx.

One of those drugs prescribed and taken is called Hydralazine, which in combination with Captopril and two other medications was working. It was the first “extended” period of time that her blood pressure had been controlled. However, after six to nine months, routine blood work showed a red flag that precipitated further tests. Those results confirmed the doctor’s concern that my mom now had Hydralazine-induced ANCA vasculitis, another autoimmune disease in addition to Systemic Sclerosis. This one can progress rapidly and be fatal, especially in someone with her kidney disease and other co-morbidities.

Bouncing Rx.

The treatment –> Rituxan, a cancer drug that is used off-label for ANCA-associated vasculitis. As with every drug and especially one as powerful as this, there’s a long list of potential side effects and risks. This one has a black box warning that says in capital letters, “FATAL INFUSION REACTIONS, TUMOR LYSIS SYNDROME (TLS), SEVERE MUCOCUTANEOUS REACTIONS, and PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY (PML)” Gulp!

It then says within the black box, “Fatal infusion reactions within 24 hours of RITUXAN infusion occur; approximately 80% of fatal reactions occurred with first infusion.” Double Gulp!!

After reading through the black box and full drug profile, you can imagine there were incredible concerns and lots of questions to understand as much as possible. The long and short of it, and even considering excellent insurance coverage, there was no other medication choice from a medical standpoint. Triple Gulp!!!

Thankfully, her first infusion only caused a very minor side effect of a sore throat. From there, she had no issues with the remainder of the set of four infusions over a month, followed by two infusions 6-7 months later during Covid, and one 6-7 months after that in May 2021.

For over a year, Rituxan was yielding improvement in various aspects of her health, as proven through how she felt and lab results. But, it wasn’t leading to remission of the vasculitis….until lab work this past June showed the ANCA was negative. Yahoooo!! Dr. Donohue (rheumatologist) was thrilled to give us the news and Rituxan was no longer needed. Breathing, figuratively speaking, became a bit easier.

Unfortunately, that joy was short lived. A month later routine lab work ordered by Dr. Eidelman (immunologist) revealed an undetectable level of immunoglobulins (IgG) – the most common type of antibody found in blood circulation, allowing it to control infection of body tissues. By binding many kinds of pathogens such as viruses, bacteria, and fungi, IgG protects the body from infection. With a dearth of immunoglobulins, she now has a susceptibility to countless pathogens.

Why did this situation arise? A known potential result of the Rituxan infusions. It helped wonderfully in ways, but caused this very dangerous condition with her IgG.

When initially learning of this predicament from the immunologist, and the “why” this happened, my family and especially my mom felt disconcerted and disheartened. She had been going along without anything major since her near-death experience in April 2020. There’s never a good time to be diagnosed with undetectable IgG, but during a pandemic it’s much more concerning.

One reason is that in addition to having had little response to the vaccine and therefore having no protection to SARS-CoV-2, now she has no defense to a plethora of other pathogens. Second, a related reason for concern – we were wondering what the treatment options would be, and if it was needed at the clinic or hospital. Not places you want to be during a pandemic and especially in light of this new diagnosis of undetectable IgG.

Bouncing Rx.

The treatment –> Hizentra, another drug with a black box warning noting potentially dangerous side effects, though no capital letters shouting the risk of death like Rituxan.

Hizentra is an immune globulin medication made from human plasma, which contains antibodies to help the body protect itself against infection from various diseases and treat primary immunodeficiency diseases. We’ve been told this “should” successfully address the immune system issues and it will take at least three months, but there’s no guarantee of success or time frame. If it’s working, she will need to continue receiving it weekly for the rest of her life. If it’s not working, there may be another drug option, which means I may be back with another blog post called A Drug-Induced Reflection, So To Speak, Part 2.

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Caregiver/Advocate Takeaways

There are thankfully many drugs that save and extend lives. Despite the benefits that many of them can provide, they may also bring about other serious conditions.
Before prescribing a drug, it’s important a doctor uses both art and science, considering each patient’s holistic situation to determine if the potential benefit is greater than the risk of side effects.
Situations are fluid, especially in acute settings such as the hospital, so it’s not always possible to gather a lot of information and from multiple sources.
In any situation, at minimum, do your best to search the internet for info about drugs. Look for information from patients, caregivers, patient advocates, and healthcare professionals on trustworthy websites that provide insight, so there can be constructive conversation with the doctors.
Each patient and their patient advocate have a right to ask as many questions as necessary to gain an understanding, so an informed decision can be made.
Ultimately, it’s the patient and/or healthcare surrogate’s decision how to move forward…not the healthcare professional.
As always, it’s important to read and understand the American Hospital Association’s Patient’s Bill of Rights.

Thanks for reading A Drug-Induced Reflection, So To Speak. As with other 1 Day 1 Week posts, you’re welcome to share your thoughts below and/or share a story as a full blog post of your own. Whether you’re a caregiver, patient advocate, or involved in caregiving/patient advocacy in some way, the worldwide community of caregivers and patient advocates can benefit individually and collectively from “hearing” what you share about your experiences.