Tuesday, December 31, 2019


I have been thinking about this post for the last couple of days, thinking how different this one feels to write compared to the previous four year in review posts.  While it has been just another year filled with too many doctors visits to count, approximately 15 or more ER visits, multiple procedures, two potential sepsis' due to central line hospital admissions, PEMU stays, routine meetings, nursing challenges, out of state hospital adventures, and the usual major decision making, it has also been a year that has provided some more specific directions for how we continue our care for Sonzee.

This year we were able to find a seizure tracking device that is actually able to detect Sonzee's bigger seizure types due to a company that was willing to give us a company laptop and the go-ahead to keep it until we figured out the best algorithm for it.  We are so appreciative for this peace of mind we have been given.  2019 was by far the worst year of seizures for her, and despite my firm stance on not trialing another medication, I gave in and it sadly turned out the way I anticipated.  She has had twice the amount of seizures than she did in 2016 and over 160 more than last year.  We end the year with her on 2 high doses of anti-epileptic medications, a rapid cycling VNS, and still no seizure freedom in view. 

2019 was the first entire year of her life she spent with a central line and one that was accessed 269 days of the year.  Having a port afforded her the opportunity of adequate nutrition, the ability to avoid multiple hospital admissions due to the ability to treat her with fluids at home, 1.5 times her hydration needs, and to still spend approximately 60 days in the pool getting her swim on; however, it resulted in at least 52 needle insertions and allergic or adverse reactions to numbing creams and antimicrobial patches.  Via her port Sonzee received Total Parenteral Nutrition (TPN) and Lipids in addition to her intestinal feeds and gained 4.2 pounds and approximately 7 centimeters.  However, nothing is ever without a cost, and so we also learned this year that she is anemic and for the majority of the year was Fatty acid deficient.  She is currently on or has had treatments to ensure neither becomes a problem in the future.

In 2019 it was confirmed her bone density is due solely to her CDKL5 mutation as her other genetic panels revealed no other genetic mutations.  Throughout 2019 she only suffered two new spinal fractures and one tibia fracture in January, but as of her March scans all bones were healing appropriately and she has (thankfully) not suffered ANY fractures that we are aware of.  In 2019 we learned her DEXA score is -11.8, which places her in the severe osteoporosis category (anything -2.5 and beyond is osteoporosis) and she is her endocrinologist's all-time 2nd worse DEXA scoring patient (yay?!)  During 2019 Sonzee traveled for the 3rd time to the CDKL5 Clinic of Excellence in Denver Colorado, gained a new interventional radiologist at Columbia Presbyterian in NYC, and added another orthopedic doctor here in Phoenix.

During 2019 Sonzee gained a new home health nurse and then 8 months later lost that same nurse so she can could further her career, but her favorite and first nurse since she started with home health nursing became her main and only nurse.  We also sadly lost our favorite clinical nursing supervisor with our agency due to scheduling, and also had yet another change with her DDD supervisor, but we are learning to adapt to these types of changes better.

In 2019 we learned that Sonzee's CVI score drastically fell from a 5 or 6 down to a 1 or 2 despite the fact that CVI scores do not typically worsen (unless there are extenuating circumstances), but she has managed to thankfully be a solid 2 out of 10 based on her December Opthalmology visit.  This, unfortunately, has left her with limited desire/ability to utilize her Tobii eye gaze device and so it sadly sits collecting dust in her room.  During her recent eye visit, we were also given instructions to begin patching again to try and help her better utilize what sight she has with fewer distractions.

The most challenging part of 2019 was watching Sonzee begin a steady decline in all areas of her health and visible zest for life.  2019 led Sam and I to many difficult tear-filled discussions and arguments with multiple professionals in order to determine what is really the best for Sonzee.  We signed a DNR and made the decision to treat her at home and avoid the hospital at all costs.  While the decisions were not easy to make and put a lump in my throat, we know this is for the best for her.  2019 was the year we fully began to fully embrace palliative care on a different level.  It was the year we refused treatments based on our confidence in knowing Sonzee best and with no regret, but with a tinge of sadness lingering in the air over the situation itself.

It is hard to say whether 2019 was Sonzee's worst year, she has had so many rough times during each of her years, I cannot say one full year was actually the worst, but I can say this year was certainly not her best.  I can say with assurance that as we close out this year, it is the one that leaves me feeling the saddest about where we currently stand, and extremely hesitant for what will come.  I feel like 2019 took a lot from our little bear, and along with it a lot of my faith, hope, and what limited positive outlook I might have been hanging on to.  2019 is another chapter I am glad to be turning the page on, but if I am honest, scared to be doing at the same time.  We have enough years under our belts to know better than to ask for calmness or for CDKL5 to be kinder to us, so for 2020, I will ask that whatever happens, I am able to see and truly believe happened for the best.

The Mighty Contributor

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