Article No. 418
9 February 2021
Our family has been through life-threatening episodes of atypical HUS before, so you’d think that we’d be somewhat well equipped to deal with an aHUS relapse. In some ways we were but a few things caught us by surprise. As you may expect, there are many things beyond one’s control when aHUS recurs. Especially when symptoms of aHUS relapse appear on a weekend. On a Saturday, which also happens to be Christmas day. During the COVID-19 pandemic.
Although my teenager’s last period of any measurable aHUS activity was over a decade ago, his genetic predisposition (factor H) indicates a need for lifelong awareness of potential for aHUS relapse. While it had been many years since our family was involved with his aHUS management and treatment, one doctor left us with this lingering message ‘It’s not IF atypical HUS will recur, it’s a matter of when.’ While some might be chilled by that concept, we took it as intended – be vigilant for any signs of atypical HUS activity. Perhaps it will be helpful to other aHUS families to know what we encountered during an aHUS relapse, and what issues we found to be different from our family’s initial aHUS experience.
Every case of atypical HUS is unique, but here’s a look at one family’s experience of during an aHUS relapse.
The Concept of aHUS Preparedness
Atypical HUS can strike without warning and with few symptoms, so though aHUS was moved to the background of a sunnier life we knew those clouds might form again. Have you thought about what ‘being prepared’ for an aHUS relapse means to you and your family? Feeling prepared can’t happen by following someone’s set checklist, as it’s more about individualized needs and specific information tailored to your situation. Preparedness is unique to circumstances, so as years pass that likely means plans must adapt as families move through various stages and changes within their lives and to their lifestyle. My views about ‘being prepared’ evolved as my son grew, and as science and aHUS treatment evolved. Atypical HUS affects not just the patient but the entire family, to include relationships and responsibilities, so planning viewpoints differ greatly if your role is an adult patient versus a family caregiver. Here’s what our family thought about, perhaps it will provide food for thought for others:
What’s your plan at home, if you think a relapse might be starting? Do you keep on hand some basic ‘aHUS essentials’ such as hand sanitizer, disinfecting wipes, blood pressure monitor, urine dip test strips, and an up-to-date Care Notebook? (more on this later).
Are there arrangements in place for other family members and responsibilities at home (including pet care), should you need to be away from home (caregiver) or admitted to the hospital (patient)?
What safeguards and supports have you built into your life as backups in case of illness, and what options are available to sustain yourself (physically, emotionally, spiritually, financially) should a relapse occur?
What lifestyle shifts might be necessary during a relapse? Are some easier to implement or manage, for short versus long term planning? What assistance might be available?
Have you created plans in the event an extended absence is needed (school or work)? (True for aHUS pediatric or family caregivers, too!)
Since atypical HUS activity may cause fatigue or difficulty with focus/memory, it’s possible that you may need accommodations regarding tasks or productivity at work/home/school. What materials, links, or physician documentation have you gathered to share with colleagues, teachers, and others which might explain the effects of aHUS? (Such as Know aHUS: Know Us, the Clinical Tracker tool, Learning at Work or School, or Atypical HUS 1.0)
Figure out in advance what issues and gaps you’d have in your daily life should an aHUS relapse occur. Make a general plan to address those, and keep modifying them as circumstances change. Be prepared at home, as well as at work or school, so that you can stay focused on what really matters during an aHUS relapse – finding the path to wellness. Here are the steps we had in place prior to the relapse.
Preparation at Home & School
Even before the COVID-19 pandemic ramped up, our family maintained a robust supply of items such as hand sanitizer and antiseptic hand towelettes. Over the last 10 years we’ve worked annually with the school nurse to create a grade-specific Individual Health Plan which included limiting viral load utilizing simple steps (such as avoiding shared class materials like scissors or glue). These yearly meetings helped to inform teachers about aHUS in general, and to plan out-of-school options should an infectious outbreak occur (such as measles or flu). Strangely, in 2020 most of the world moved to a similar ‘keep safe’ model due to the pandemic, which meant that my child’s school system offered a ‘remote only’ learning option. When store shelves were bare during pandemic buying frenzies, my family still had enough hand sanitizer, wipes, and disinfectant cleaning supplies. After contacting the school nurse and high school guidance counselor, my child moved to ‘remote only’ learning sessions (and some reduced assignment load) for less than a month before returning to our public high school’s hybrid model.
Medical Preparedness at Home
Since infection or inflammation has potential to trigger atypical HUS activity, we opted to keep a supply of urine test strips always on hand. Fever? GI bug? Whenever illness struck, it was time to take out our home vial of urine dip strips and see if there was any sign of hemolysis (destruction of red blood cells). Interestingly, a new aHUS research article from Italy (Brambilla et al, Jan 2021) details the value of urine dipsticks to detect hemolysis as an early, key indication of atypical HUS relapse. We utilized a urine dip test strip on Christmas morning, and called our Primary Care Physician’s answering service to report the color change which indicated a mid-range presence of red blood cells (positive for erythrocytes).The urine dip test result gave me something concrete to report to the doctor, since other symptoms were vague other than dark urine which triggered use of the test strip (he felt a bit tired, didn’t eat much the previous day, had mild stomach and headaches). As many parents have experienced, it wasn’t until the physician call ended that another symptom was evident (a single episode of vomiting). We were directed to go to our local hospital’s emergency room, since no urgent care offices were open on a holiday. It wasn’t until the Emergency Room staff checked vital signs that I realized that I had completely forgotten to take an initial blood pressure reading at home.
Our aHUS Care Notebook
While our family enjoyed a 10 year respite from aHUS flares and medication to the fullest, we still kept informed about advancements in the field of aHUS. We maintained a 3-ring binder of documents with my son’s current health history (annual physical, immunization record, etc) which included the results of his aHUS genetic screening and test panels. There’s a ‘library’ of aHUS-specific research as well a clinical network available to ER or other medical staff who’ve never heard of atypical HUS. I’d included that and more in our ‘aHUS Care Notebook’, such as printed copies of the Clinical Tracker. Along with a few assorted pieces of research I’d printed out, and my plan for a ‘key info about my son & the disease’ was kept ready in a notebook to bring with us as we left for our local hospital’s emergency room. (In case you’re wondering, I kept these papers in heavyweight document protectors. It was amazing at how many documents the medical staff asked to remove from the notebook for review or to copy pages.)
Expect the unexpected with atypical HUS
I should have expected that the aHUS genetic test results were seen as documented proof to shortcut urgent care protocol on diagnosis, allowing medical staff to focus on treatment plans. It allowed medical staff to save time and effort since the aHUS diagnosis and genetic mutation was reported thoroughly, allowing doctors to focus on treatment options. Staff had changed within the pediatric nephrology practice, and both new specialists were great at encouraging a ‘care team’ approach to dialogue and new information. Despite keeping updated on aHUS drug candidates in clinical trials, I hadn’t thought through that those contacts are only available during weekdays on business hours. A number of aHUS clinical trials call for ‘treatment naive’ participants, but many aHUS patients cannot wait for treatment and thus get excluded from drug candidates under study. Excluded before we even began to fight aHUS.
For my family’s Christmas day relapse experience, ‘being prepared’ meant quickly identifying the problem to target a treatment plan that included immediate admittance from the ER to the hospital’s children’s floor for a same-day infusion of eculizumab. Our aHUS care notebook apparently indicated our family’s ability to track and report out blood pressure trends, fluid intake/output, and administer medications at home. After three days in the hospital my teen son was discharged with arrangements for our home monitoring reports, followed by mid-week labs until the second of his 5 weekly eculizumab infusions – with infusion #2 on New Year’s Day. He’s doing much better, thank you and we’re changing treatment plans to suit his improved health.
Atypical HUS relapse isn’t an event, it’s a process. As such, describing family preparedness is only the beginning. Just like the disease itself, there were unexpected twists and turns in treatment availability at the outset of relapse that no amount of planning would have helped. Battling a rare disease takes dedication from many, including the doctors, the nurses at our pediatric nephrology practice, the MORL team at U of Iowa, and our OneSource case manager, and we remain grateful for their care and assistance. We ran into issues not described yet, and it’s likely we’ll face other challenges. So that other aHUS families don’t get surprised as well, those will be the subject of the next article about what we encountered during an aHUS relapse.
Fortune favors the prepared. Be ready.
This is the first in our three-part series: Atypical HUS Relapse – A Family View
Fortune Favors the Prepared (Part One, this article)
aHUS Relapse – Expect the Unexpected (Part Two)
The Ties that Bind (Part Three, the final article in our aHUS Relapse Series)