Do-Re-Mi-Fa-Sol-La-Ti

We all know what they say about the “very beginning”: It’s a very good place to start! Or maybe just Fräulein Maria says that? I don’t know, but I believed her and I’ve always felt it was sound judgment. I’m going to have to be a rebel today, though, and start right in the middle. Trust me, no matter how much time you have, you don’t have enough time to read the whole story from the very beginning. I keep promising I’m going to give you all of the details about everything that has happened since October. My life has been rather unconventional lately, though. I’ve encountered a whole lot of punches, and I have had to roll with every one of them. It’s all such a jumble, as a matter of fact, that chronology does not seem to have all that much meaning any longer. So let’s just get going, right in the middle, shall we?

Last time you heard from me, I was off to the hospital for an MRI. Since my last big HLH flare, I’ve been having very serious hypoglycemia. While I was inpatient this past December, we were surprised to discover that my cortisol was at a critically low level. I practically begged the doctors to allow me to return home to my beloved Christmas tree, but it was simply too dangerous for me to be anywhere but the hospital until this had stabilized with treatment. In the meantime I was well taken care of — I had my darling Doodle service dog Alice Eloise, and one of my favorite nurses, Erin. What I didn’t have was a hairbrush. But did it really matter? My puppy and my nurse certainly didn’t mind my wild hair! Especially in the hospital, a little bit of kindness goes a long, long way.

Serology testing sent off during that hospital stay later revealed that my pituitary gland is not working properly, necessitating an MRI for further information.

The good news is, there is no brain tumor pressing on my pituitary gland! So what does this mean for me? We don’t have a definitive answer to why my cortisol levels dropped so much, so suddenly; but our best hypothesis is that my HLH damaged my pituitary gland. I continue to require my TPN (total parenteral nutrition) infusing 24/7 in order to keep my blood sugar stable, and the endocrinologist thinks I will likely require treatment for the rest of my life. Of course this isn’t exactly pleasant, but even so, I am thankful these interventions are available to save my life.

The bad news is, the entire MRI procedure (or, more accurately, the anesthesia involved in the matter) was a complete disaster. The day before we had been assured that all the arrangements had been made for my magic anesthesia protocol. We were told that there was even a room in the PICU with my name on it so that all of my unique needs could be met in the night following.

So when the anesthesiologist walked into my prep room and asked, “How little do we have to do to get you through this MRI?”, I felt quite shaken. You see, it takes rather a lot to get me through any procedure. But this guy just wasn’t going to have any of that. A quick call to my palliative care specialist later, the anesthesiologist agreed to start out with my protocol, and then see how it goes. This was more than a little worrisome for me… I mean, I wasn’t going to be awake to “see how it goes”. That was on him. But what was I to do? As much as I may have liked to, I simply could not demand to be assigned a new doctor right then, right there… Could I?

When at last I did awake, I was not even slightly surprised to find that the anesthesiologist abandoned the protocol entirely. He reportedly told my parents that I would be out of there by 5 o’clock that evening, an assumption he made before I was even conscious. Soon enough, however, it became quite clear that he was mistaken. At this point in time, he felt it appropriate to ask my parents “how and when they would know that I was ready to go home”… After all, wasn’t I inpatient for days following a simple surgery in December? You know, the hospital stay when I was begging to be released so I could go home to my Christmas tree?! Was he, perchance, insinuating that I had already outstayed my welcome at the hospital? Why yes, I do believe he was. Ugh! Anyway, I needn’t bore you with all of the details. But my body was beyond angry. It is so truly unfortunate; things did not have to be that way. It could have gone much more smoothly for everyone involved, if only he would have listened.

The cherry on top of the whole fiasco was served up the next morning when I was informed that the pediatric anesthesia department wanted nothing more to do with me ever again. And given my age of 23 years old, they are allowed to pass me along, no questions asked. Thankfully Dr. Chismarich, who is my palliative care specialist and one of my foremost superheroes, stepped in, trying to make things right. She has been working so diligently to firmly document every detail of my anesthesia protocol, and to establish a team including an anesthesiologist and a surgeon who would be willing to take on my special case any time I may need them. Because, as you likely have noticed, surgical procedures are always an imminent occurrence in my life.

Let us hope, however, that I can avoid the need for such accommodations for at least the next month or two… There will be plenty going on, even if my body does behave itself, as my mama is having her hip replacement surgery today! She has known since she was a teenager that her hip was a problem. Her doctor had told her that she’d be a crippled adult, but we have been very blessed in that, although she has lived in a fair amount of pain for years, she hasn’t been too terribly limited until these past few months. But then suddenly it was as if a flip switched and she could barely walk. Of course this had to happen when my papa was out of town, and I myself was having surgery a couple of days later. I had a walker overnighted so that we could find a way to not only get around the house, but to get around the hospital as well.

But alas, the walker did not make it in time! At least, it wasn’t supposed to. We have a sweetheart of a UPS driver, though. Every Friday I receive giant, heavy boxes of medical supplies, and our UPS friend, Pete, kindly carries them into the foyer for us. Funnily enough, Pete is one of Alice Eloise’s best pals! As a matter fact, she didn’t used to be so crazy about him… After all, he does drive that scary big truck. But once she gave him a chance, she became entirely smitten. And now Alice Eloise and Pete play fetch every time he swings by our house with a delivery! They’ve been known to play some truly fantastical games of fetch, too, beginning in the foyer and stretching all the way across the house into the family room. And on days when there isn’t a box for us on that no-longer-scary-big truck, Alice will wag her tail by the window as Pete drives past our house, oftentimes honking his horn as if to say, “See ya next time, Alice Eloise!” It’s a friendship for the ages, iconic as Charlie Brown and Snoopy. Anyway, Mom’s walker was not supposed to arrive until the evening after my surgery, which would simply be too late. But Pete so kindly met my mama downtown so she could have her walker for our trip to the hospital. Aunt Lesia and Uncle Terry assembled it for us in a jiffy, and poof! We were out the door right on time with not a moment to spare!

We have had to adapt quite a lot these past months, but, largely in thanks to that handy dandy walker, we have found ways to manage. But, of course, Mom is feeling very ready to have that new hip. Please pray that her surgery goes well and that she is feeling much better in no time at all!

And, if you could, please add one quick request at the end of that prayer for me. Because I cannot use my arms, I am unable to do much of anything for myself; but of course I do require such a lot of care each day, and my mama has been my caregiver since Day 1. It has been quite complicated piecing together all of the arrangements necessary to be sure I will be taken care of during my mom’s recovery. We had a lot of trouble finding an agency whose nurses have the right qualifications to handle my TPN and IV medications, but at last we have everything in place. It is naturally a bit unnerving for me to rely on new people for all of my needs. But I am trying to imagine this adventure as a way to make new friends! One can find a silver lining in nearly any situation, after all!

Even with the help of the agency, there were still quite a handful of time slots left unfilled on the calendar. I am truly blessed to have such kind and caring family who have committed to filling in each of those empty spaces. All of my special helpers know who they are, and I thank them dearly!

Believe it or not, Mama’s stay in the hospital will make for the very first time we have spent a night under two separate roofs in over five years now. I must admit that, although I will most certainly miss her while she is away, I am still psyched to slumber party it up with my big sister Krista! Don’t worry about me, Mama. I will be just fine! I love you!

So there you have it! The latest chapter in the adventure that is my life. And what kind of wild shenanigans will I find myself wrapped up in next time? Hmm… I guess you will just have to tune in to find out! Toodles, friends!

Posted in HLH, paralysis, Prayer Request, surgery, Uncategorized | 21 Comments
Merry Christmas and a Crazy New Year – My First Prayer Request of 2015

I have come to find that all of the leftover craziness of 2014 has somehow followed me into the new year. Such a lot of craziness, in fact, that a journal post to bring everybody up to date has been in the works since October! But the chaos has just piled up on top of itself over and over again, growing to the point that I suspect it may fill a series of blog posts. I simply have so much I need to share with you!

Just not right now…

Rather, today I bring you my first prayer request of 2015. Ever since my HLH flare in October, I have been experiencing extreme hypoglycemia. It has been terribly challenging to manage; even with my TPN (IV nutrition) infusing 24 hours a day, my blood sugar still tends to dip. So when I was inpatient last month, the doctors ordered some testing in an effort to determine the cause of this hypoglycemia, revealing that my cortisol levels were dangerously low. Since then we received the results of more tests that had been pending, and it has become clear that my pituitary gland is not doing its job, explaining the lack of cortisol in my body.

Now look sharp, this is where you come in! Today I will be having an MRI to check out this faulty pituitary gland. And… Why exactly is this a big deal, you ask? Well, quite simply it would not be anything but a minor annoyance for most people. But due to my body’s “unique” way of dealing with pain and sensory input, this procedure requires general anesthesia for me. Thankfully I am immensely blessed to have the most sensational palliative care team in the entire world; they have pulled together the arrangements for my magic anesthesia protocol, which is an incredible relief. We all know that I have a way of being unpredictable, though, so I really need your help, too! I would be ever so appreciative if you could please remember to keep me in your prayers today. We are talking all sweet sugar plum dreams, and absolutely, positively, no paralysis!!!

Keep an eye out for a non-emergent blog post sometime soon! Let’s see if life can slow down just enough for me to share the jolly, albeit chaotic, details of my favorite time of year! Alice Eloise and I hope that all of our dear friends had a most blessed and magical Christmas, and a happy New Year, too.

And now, I am off to count some sheep! Or perhaps I should count Doodles instead?

One… Two…

A Very Doodle Christmas

Posted in inpatient, ketamine, Pain, Prayer Request, Uncategorized | 13 Comments
An Update from Mama

After a longer-than-expected inpatient stay, Sarah Kate was released from the hospital yesterday afternoon. I apologize for not providing an update sooner but exhaustion and craziness have gotten in the way.

Sarah’s dad and I were extremely relieved when we received word from the recovery room that Sarah was not paralyzed following her central line placement, but sadly we would soon come to realize that things were not going well for her. When they called out they had told us that she was in a lot of pain and that once they got that under control they would be transferring her to a room in the pediatric inpatient unit. Upon her arrival upstairs, her pain made for a very traumatic introduction to the floor nurses; but thankfully they knew she was in trouble and acted accordingly. Although it was nearly 11:00 p.m., Katie, a member of Sarah’s palliative care team, made an emergency trip to the hospital. It was decided that Sarah needed to be transferred to the PICU where she could receive ketamine and be monitored closely in an effort to lessen her pain. It was an overwhelming night … terrible hallucinations (from the drugs) and a scary incident when she stopped breathing; but we could not be more thankful for the amazing efforts of the doctors and nurses.

Prior to the procedure, the anesthesiologist shared that he would be tweaking Sarah’s special protocol from what had been done when all went so very well in April. He believed that Sarah did not need so much ketamine and said that he would compensate with more pain meds. Given that he was still planning to administer more than 8 times the ketamine he would typically use, we were reassured that it would be alright. He was a fine doctor but hindsight now tells us that approach was a mistake.

Bright and early the next morning, Larry and I made a quick visit to the orthopedic surgeon. It has been decided that I will be having hip replacement surgery just as soon things can be arranged. I absolutely never leave Sarah when she is inpatient, but I was relieved to know that she was in good hands. Brother-in-law Tim came to Sarah’s rescue… She has told me SEVERAL times just how well Tim took care of her while I was gone. Tim also shared what an appreciative patient Sarah is… She never fails to say “thank you” for even the simplest acts of kindness.

Grandpa brought Alice Eloise to see us at the hospital on Saturday. Her love is such a blessing for all of us… There is really nothing better than puppy love.

Since Sarah’s HLH flare in October, she has been having serious problems with hypoglycemia. It was thought that her liver needed to restore its reserve of glycogen, but weeks later it has still been a problem despite keeping her hooked up to her TPN 24/7. So while she was inpatient following her surgery, an endocrinologist was consulted. Testing has revealed that Sarah’s cortisol levels were dangerously low. It will be a few weeks before we know if this is primarily caused by her adrenal glands or her pituitary gland. Sarah was apprehensive about starting the medication as our family has a scary history associated with steroid use; but her doctors explained that there is really no decision to be made as Sarah would not survive if she didn’t begin treatment.

It is so very nice to be home once again. I am sure that you will be hearing from Sarah Kate sometime soon, as she always has her own unique perspective on life. Right now she is struggling immensely in ways we wouldn’t have anticipated. Please pray that we may soon find the cause of these new symptoms so that we can develop a plan to help our typically spunky little Christmas elf feel like her merry self once again.

God hears our prayers and those of so many of you who are praying for Sarah. We are forever hopeful that He may have a miracle in store for her. Christmas brought us all God’s greatest gift and miracle when our Savior Jesus Christ was born… And it is His love that continues to supply us the strength, faith, and hope we need.

Merry Christmas to each of you. May you, too, be filled with His love.

Posted in inpatient, ketamine, Pain, RSD, surgery | Tagged , , , , , | 12 Comments
Shoutout from the Whirlwind — A Prayer Request

Hi friends! It’s just me, reporting from the twister that swept me away a couple of months ago. Newsflash: That aforementioned twister has not yet been so kind as to set me back down on solid ground, no matter how politely I tap the heels of my ruby slippers! But I must take a moment to shout out from my whirlwind with a prayer request. It appears as though I’ve found myself in a bit of an emergency once again. Surprise, surprise.

Some of you may recall that I have lost two central venous catheters (CVCs) this year, one in February and one in April. It seems that my body is having trouble holding onto central lines. This complication can sometimes come along with Ehlers-Danlos, my connective tissue disorder. Dr. Malik, my surgeon, did beautiful work in placing my current line, stitching it in impeccably so that it could last me as long as possible. However, for a while now we have noticed it slipping out of place, gradually becoming more and more problematic until finally this week it stopped offering blood return. Ruh-roh! This is most certainly a predecessor to even more significant issues that could follow any minute, so I’m just thankful that this line is still infusing my TPN and IV meds. But this is a limit I simply cannot push. Consequently it is necessary that I have a new CVC placed even sooner than pronto!

Because of my unique needs it surely isn’t easy to hastily arrange a surgery. We have run into some complications because Dr. Malik recently left the hospital, leading to the more-difficult-than-anticipated task of tracking down the best doctor for the job. But one of the brightest silver linings of my recent HLH flare was that I had the pleasure of getting better acquainted with my palliative care doctor who was acting as my hospitalist while I was inpatient. Dr. Chismarich is truly a remarkable doctor in every way. Somehow she managed to pull together all of the bits and pieces necessary for my procedure. And take my word, there are A LOT of bits and pieces. Now that all is set in place, I will be having my surgery today at 2:30.

That’s not all. My mom has been a candidate for a hip replacement for quite some time. But as my caregiver, she has pushed back this intervention for as long as she could, waiting for a calmer time when my health might reach a better place. Eight years later, that time still has yet to arrive. But ready or not, this week rolled around and my mama’s hip decided it has just about had enough. Shazbot.

Wait, there’s more. With Mama barely able to walk, we clearly needed some help. But my daddy has been out of town on business. Although he returned to St. Louis late yesterday evening, he had some pretty close exposure to the flu while he was away, meaning he has to keep his distance from me until we know he is all clear. And even my big sister is out of town for work this week, which happens once a year at most! But thank goodness Aunt Lesia and Uncle Terry live just down the street; and honestly, I can say with full certainty that there is no way we could make it through this fiasco without them. They so kindly drove us to St. Louis for a consultation with a prospective surgeon, which incidentally was cancelled by his office just as soon as we pulled up at the hospital’s valet parking pavilion. Alice Eloise and Uncle Terry have had plenty of bonding time as he has been on, um, “doggy duty” duty. And with Aunt Lesia’s help I had a much-needed shower and even some impromptu dental work. It’s been quite the adventure! Or misadventure, perhaps? Either way, we still have some major details to figure out for when my mom has her own surgery; but thanks to the Frey family down the street, by golly, I think we just might survive this week yet!

And hey, you! I could use your help, too! Could you please pray that my surgery goes smoothly today? For those of you who are new here, I have a history of paralysis following surgery, a mystery that took far too long to solve. I wish I could say that a fair bit of sleuthing was all it took to find a solution, but the price tag was quite literally my left arm. In February I received so little anesthesia for a procedure that I was aware throughout. This was a frightening experience to be sure, but because I was awake I had the opportunity to finally learn that my paralysis begins when the analgesic effect of the anesthesia ends, inviting my intense pain to hit full-force. Unfortunately my left arm has yet to awaken from the subsequent paralysis. But this incident led us to the development of my own special, detailed anesthesia protocol that successfully evaded my paralysis when I had my last procedure. Please, please, please pray that this protocol is once again the magic ticket!

Now, I’ve got a big day ahead of me, so I’d better boogie. Toodles!

Posted in emergency, Prayer Request, surgery | Tagged , , , , | 14 Comments
Homeward Bound

I know that many are concerned about Sarah Kate so I wanted to provide an update. Her numbers are trending in the right direction… Enough so that the hope is that she will be able to go home today. Sarah received more blood yesterday, and her fevers are now low-grade. She is feeling totally lousy; but as long as she is stable, home is the best place for her to be. I draw Sarah’s labs every Monday, but it is reassuring that for the time being we will be checking them even more frequently to be certain that things are continuing in the right direction. I am confident that quiet cuddle time by the fireplace with Alice Eloise will speed the recuperation process better than anything else ever could.

I have to share a silly Sarah story that tickles me every time that I think about it. Those of you who know Sarah or who have followed her blog are privy to the fact that our girl is usually pretty sharp, as evidenced by her quirky, quick wit and whimsical storytelling. Typically nothing much gets by her, but HLH flares are not typical. In fact, Sarah probably won’t remember much at all about the events of this week after the fact. Her body is so very weak and tired, and that exhaustion affects her mind, too. So the other night while Krista was here visiting, she informed Sarah that the Royals were going to the World Series. Sarah did not respond until the next afternoon, when she giggled to herself and admitted that she had just figured out that Krista was talking about the Kansas City Royals. Instead, she had found herself hoping that the Cardinals would make it to the World Series so that her favorite “royals”, Will and Kate, would be traveling to St. Louis. I guess I should apologize to Sarah Kate for spilling this secret, but such cuteness just had to be shared.

God has seen us through this tough week… Now we are thankful for the blessing of going home.

Posted in HLH, inpatient, Reflections | 7 Comments
We Have an Answer…

Sarah Kate’s labs have confirmed that she is in an HLH (Hemophagocytic Lymphohistiocytosis) flare. She has had constant fevers since Saturday (some in excess of 105); so that, along with dropping blood counts and outrageously high inflammation markers, has left her feeling terribly sick. Sarah received blood today, and her doctors will now monitor her closely as they consider potential treatment options. They will likely start her on a medication that has helped to settle things down in the past. Given Sarah’s history of HLH flares, a bone marrow transplant would be a consideration; however, Sarah is just not strong enough for that to be a viable option. Other HLH treatments like chemotherapy and high-dose steroids are also believed to be too harsh for her, especially considering her chronic pancytopenia. Sarah’s doctors and nurses at Mercy have been incredible… Last night they transferred her to the PICU to keep a closer eye on her. We have been so very blessed by their devotion, kindness, and excellent care.

Before Sarah was transferred to the PICU, Alice Eloise’s Uncle Tim took it upon himself to bring her to the hospital to see her Mommy. It was so very sweet of Tim and absolutely wonderful medicine for Sarah.

 

 

Sarah looks darn good considering all that she has been through the past few days… But as her Mom, I can attest that those sick girl eyes speak volumes.

God provides for our every need, and you, dear friends, are among His very best blessings. Thank you for your continued love, support, and prayers. It is truly comforting to know that so many are supporting us in this fight.

Posted in HLH, inpatient, Prayers, please! | Tagged | 22 Comments
I Left my Creativity at Home, so this Title will Just Have to Do…

So October 3rd was pretty great. The week following? Hmm, not so much. The travel most enthusiastically kicked my booty. You know that saying, “nothing is impossible with God”? It’s true. Somehow I did indeed make it to Rhode Island and back. But I am pretty sure that pigs could fly more easily than I can, even when taking into account my advantage of having an airplane to help me with that endeavor.

Consequently my body has been angry all week, finally culminating with a high fever and a trip to the ER last night. I was admitted so the doctors could keep a close eye on me… And it’s a good thing, too, as my blood counts took a big hit in a matter of hours. We still don’t know what’s up. Could it be a Hemophagocytic Lymphohistiocytosis (HLH) flare? Sure. Kind of looking that way. Could it be sepsis brewing? Mhmm. A possibility but cultures have come back clear so far. Could it be Post Booty Kicking Syndrome (PBKS)? The NIH has yet to release any statistics on PBKS, but I think it is a reasonable addition to the suspect list in this particular case.

I know you’re anxiously awaiting the details of my Rhode Island trip, but for now I am just gonna have to offer an IOU. Capeesh? But in the meantime, could you please pray for me? I know that I can always count on you! Toodles!

“My health may fail, and my spirit may grow weak, but God remains the strength of my heart; He is mine forever.” – Psalm 73:26

 

Posted in emergency, inpatient, Prayers, please! | 16 Comments