I was on the phone around 10 pm talking with our main doc, monitoring Lucy’s heart rates and respirations, trying to figure out a plan to stop the nonstop clusters of seizures that have been plaguing Lucy all day, all the while attempting to get blood flow back on her blue lumen (once again stuck), when I realized that our bed was made, clean sheets and all. I looked over at Drew and whispered to him during all of this…you made the bed, thanks!
All too often I find that I only have time to write about the big changes in our lives, the big appointments, hospitalizations, milestones, etc.… While important, I really desire to write about the everyday stuff of life too, you know the details that tell the story behind the story.
For example last Wednesday, when Lucy and I headed over to the DuPont ED at 10:30 pm, it was not because that was the most opportune time for us to go, oh no no, that was when I arrived home with Jack who was competing in our county’s Reading Olympics competition. Are you kidding, I hate going to the ED after 10 pm unless absolutely necessary, that’s experience talking! At best we’ll be there a minimum of four hours, but in our world it usually means that we’ll get admitted sometime in the middle of the night/early morning. In this case it was around 4 am when we were transferred to our regular floor, which means no sleep for the mama as the admissions process is long and rounds start around 7 am. My options were little to none however. Phone calls were made to our infusion nurse, to our on call infusion pharmacist to find out which of Lucy’s new seizure meds were TPN compatible(FYI none of them, nor are they compatible with dextrose!), and finally to Dr. R who informed me that she needed to come in. I very rarely travel more than a thirty minute radius from our house for obvious reasons, except for last Wednesday when I was about an hour away. Call it Murphy’s law, bad luck, or a twist of fait but sometime around 7 pm my phone started vibrating in my pocket with our home number showing up on the caller ID. I had that sinking feeling when I saw the number and hurdled over several desks and maneuvered around several students to take the call out in the hallway . I knew that Lucy’s line was having difficulties, but I was secretly hoping that it was not as bad as I knew it was. Lucy was left in very capable hands, both Drew and Helen were with her, but a clotted off line is a serious problem for our girl whose life depends on central access. Even though it was serious, Drew and I decided that it was something that could wait until Jack’s competition was over. Which meant that we would be awake all night, and that her seizure med schedule would be off by a few hours lowering her seizure threshold even further. I am the mama to four kiddos, all of whom need their mama and are in some respects emotionally fragile, I also happen to have one who is medically fragile too. Weighing decisions like a clotted off CVL and staying to support my son in what will most likely be the only Olympics competition he will medal in are the types of decisions that we face in our life. These are the details I am talking about, the behind the scenes stuff of life that make up our life stories.
I know one day I will look back on this time in our lives and wonder just how we did it. I already do, often saying to Drew, “How did we manage to do that?”. Carving out time to do anything other than the absolutely necessary stuff of life these days takes careful planning and organization. Which I admit that I am good at doing, but at the same time I will also admit that I am exhausted from having to do. We have uncommon obstacles that we need to plan around, Lucy’s daily schedule is just not that flexible. Changes to that schedule induce stress which lowers her seizure threshold, among other things, and make for one unhappy little girl and stressed out mama. Knowing this, it makes going places…doing things… planning anything… all that more exciting, how’s that for choice wording. It is these sorts of details that I wish to capture and write about too.
Romans 5:3-4
Not only so, but we also rejoice in our sufferings, because we know that suffering produces perseverance; perseverance, character; and character, hope.
Last Year…
This year…
I recently met someone who asked me if I was Lucy Marlett’s mom.
“Why…yes, I am, I replied with pride.”
To which the person responded, “wasn’t she dying last year?”
“Umm…yes, I suppose you could say that.”
Not really sure how to respond, I then said politely, “aren’t we are all dying, some of us sooner rather than later.”
Lucy just happened to be born with mitochondrial disease, a disease for which there is no cure. She lives a life filled with extraordinary circumstances for which there are no guarantees, but then again whose life does?
This week we find ourselves reflecting more on the past year than usual, it was at this time last year that we were faced with the unthinkable, the possibility that Lucy may not survive. Her life and ours changed and have never been the same since. Although she survived what was feared she might not, she is by no means “better”, but any long time reader of this blog knows that. It is our daily challenge to live life in conjunction with these circumstances.
Her perseverance is inspiring, she is a fighter, determined to live life, not just exist, but live it! We fight so hard to live life because she does!
It was a short yet productive stay at the hotel DuPont. We managed to check quite a few things off of our medical “to do list”. We were discharged early on Friday afternoon after deciding to hold off on giving Lucy blood. Her counts are low for Lucy, and in the past we would have transfused her at this point because her heart rates would be indicating that it needed more fluid, but she is currently not acting symptomatic of needing blood, so we wait. The increase in clonidine and the addition of phenobarbital appear to be doing something for Lucy’s autonomic system, we are not seeing the erratic heart rates that we are used to seeing in our girl. Instead of swinging heart rates we are seeing swinging body temperatures…ugh. We have learned that if it’s not one thing, then it’s at the very least something else.
We left with an increase in her anti seizure meds and a new med in our pharmaceutical arsenal, IV valium. We are cautiously optimistic that valium is going to be beneficial for Lucy. She did not have a paradoxical reaction to the med which is amazing in and of itself, and it appears to calm her and make her sleepy, all things we were hoping this med would do for our girl. IV valium is on the nationwide drug shortage list, as is about 75% of the meds that Lucy is currently taking, so supply is an issue but we are able to get a limited amount. For most of you reading this, learning that there is a nationwide drug shortage list is probably news to you. For those of you who are or who have a medically complex kiddo like Lucy then you are most likely affected by this list too. I could go on a rant about how we live in one of the wealthiest countries in the world, how we have access to the best medical facilities, how where we live we are surrounded by pharmaceutical companies, but yet we are unable to get certain life sustaining medicines. If you read the list many of the companies site manufacturing delay as a reason for the shortage. When the drug finally becomes available or there is an alternate drug that can meet the patients needs, the supply is limited or becomes limited and demand increases, as does the price…hum. Like I said, I could go on a rant about this issue, but I think I’ll just leave it alone for now.
Lucy’s central line issues appear to be resolved or rather dissolved(ha ha) for now. We have been able to infuse and draw back blood on both lumens better than we have in a while. Lucy’s central line is her life line, without it she would not be here. Preserving the integrity of her line is essential and we try to be as proactive as we possibly can with it, even so things like this happen. We are so grateful that we have top notch medical care so close to home.
Thank you for all of your thoughts and prayers, love and support.
We’re still here trying to figure everything out. I am going to make this short as I have been awake for almost two days, and I’m not even the manic one, but fear I will be soon if I don’t get some sleep. Lucy’s dye study happened around noon today. Conclusion, the line is most likely clotted off, no real surprise there. Another round of TPA was ordered and IV team was successful in unclogging the stuck lumen, but round two was ordered so that we can get blood return back on the other lumen. We are still in the process of doing this at 11 pm tonight. Our hope in the morning is that we have all of our hardware issues resolved so that we have enough points of access available to us so that we can transfuse blood product. If we are unsuccessful then surgery will have to be consulted. We are hoping for the non surgical option.
We increased Lucy’s Keppra dosing for the fourth time since starting her on the med two months ago. We also started her on IV valium in hopes of having it help with seizure control as well as behavior/sleep issues. Lucy has an awful paradoxical reaction to benzodiazepines of which valium belongs in that category of meds. To say that I was nervous about starting her on this med tonight would be a huge understatement(remember I mentioned that I have been awake for almost two days the last thing needed is a paradoxical med reaction), at the same time I was the one who told our doc that I think we need to try it and see if it works. He was in total agreement, but jokingly said to give her her first dose after 5 pm as he was not going to be in the hospital after that time…ha ha. Much to everyone’s surprise Lucy has yet to show any paradoxical reaction to the valium. In the past these types of reactions happen quickly.
As always we are being well taken care of here at the hotel DuPont, and are relieved that we have been able to resolve so many issues in a short period of time. Thanks for all of your thoughts and prayers, love and support!
Lucy and I came to the ED last night due to technical difficulties with her central line. One of the lumens on her line has stopped working altogether. Lucy’s level of care is too much to not have both lumens fully functioning, we had no other options but to bring her in so that we could get all of her meds into her. The fear is that the line is not just clotted off but that there is a break in the line somewhere internally. It “popped” earlier this week when we were drawing labs from it. A dye study needs to be done to determine the cause of the problem. Of course we need to wait until the morning to have this done, which means we have been admitted. While in the ED the attending was not happy with the way Lucy “looked”, so a CBC was ordered and lo and behold Lucy’s blood counts have dropped significantly over the last week. Something is going on, and so now starts the medical detective work. Lucy has had a bad week of manic episodes and seizures. Conversations have been had with our main doc, neurology, palliative care, and hospice about what we can do for Lucy. Hopefully by being in-patient we will be able to resolve some of these issues sooner.
Today we are celebrating the birthday of a truly beautiful soul!
Happy Birthday Meggie O!
We love you so much!
