So. Are you psyched? Because guess what… I am TOTALLY psyched! Yesterday was a party over here at the Frey household. Isn’t it always a party, though? On this day in particular, however, we celebrated three exciting occasions. April 17th is one of my favorite days ever, purely because it is my big sister Krista’s birthday! She is a lovely girl after all, one of the supreme blessings in my life!
In 1991, the 17th of April was almost certain to be doubly special, as yours truly was expected to enter the scene on that very date. But it will come as no surprise to you that even then I had a mind of my own. To build suspense, I opted to be “fashionably late” and refrained from making my grand premiere until April the 25th. Additionally I am a pretty fair sharer now that I have had time to mature a bit, but honestly, it was probably best for all involved that I had my own special day when I was a tiny moppet.
When I launched my blog on my birthday last year, Krista shared a grouping of photographs taken over a span of many years. This panorama makes it clear to see that growing up there was no place I’d rather be than snuggled up next to my big sissy.
There may be four years and eight days between Krista and me, but we have always been as close as peanut butter and jelly! I’ve known Krista for just shy of 23 years now, and here we are, forever the best of friends!
PS Did you spot the darlingest, Doodlest photobomber?
As awesome as the celebration of us may have been, it was far and away outshone by the third honoree of the party. I am, of course, speaking of our Savior, Jesus Christ!
Christ’s miraculous resurrection that we celebrate on Easter is the culmination of God’s promise to love and provide for us always. Jesus took on the sin of the world to ensure that everyone who believes in Him may spend eternity in heaven by His side! Especially right now, my life is full of pain and uncertainties. Despite that, there is always one unshakeable certainty that serves as my anchor: No matter what the circumstances of the moment, I am a child of the One who has the power to perform miracles! And that, my friends, is something to be psyched about! So while I am absolutely praying that God may one day heal me, I am also saying a prayer of thanks… Thank you Jesus for the miracle of eternal life and for making Your place in my heart so that I may always have faith, hope, and peace!
“He Himself bore our sins in His body on the tree, so that we might die to sins and live for righteousness; by His wounds you have been healed.”
– 1 Peter 2:24
Have you been itching with anticipation to learn the details of all that transpired during my appointments with the specialists in Chicago? Here’s the scoop! The 4+ hour road trip to Chicago was extremely unpleasant for me. My full-body pain, migraines, and belly problems make even the shortest of car rides miserable. But here I am to tell the tale, so as you might have presumed, I did survive!
From about the time I was 15 to the time I was 19, my mama and I could cram all of life’s necessities (and there are an awful lot of necessities, you know) lickity-split into our teeny Volkswagen Bug and hit the road for Chicago. In those days I was doing high-pressure hyperbaric oxygen treatments at a clinic in one of the suburbs. Hyperbarics did provide relief, but for me it required frequent rounds of treatments, meaning Mom and I spent a lot of quality time together as roomies in Chicago. “The Ronald McDonald House near Loyola University Medical Center” became just like home for Mom and me. After all, we spent just about as much time there as we did at our real home.
In the community kitchen I even claimed a table as my own special spot. In a fit of silliness inspired by a particularly amusing episode of “I Love Lucy”, I deemed this table my unofficial kingdom, which I christened “Franistan”. I was the reigning royal figurehead, the “Maharincess”, if you will. I had no qualms about walking around “my House” in my jammies, with a towel wrapped in a turban atop my freshly shampooed head. And why should I have? I was the Maharincess, after all!
Her Serene Royal Highness, Maharincess Sarah Kathryn, circa 2006
Behold, my inspiration and predecessor in goofdom, Maharincess Lucy, circa 1952
Golly, in so many ways it feels as if my days at the Ronald McDonald House were a lifetime ago. Yet when my parents and I once again walked into the House a couple of weeks ago, it felt like a time warp. Was it possible that I had really been away for four years? Not everything was the same. The people there were different. And in the kitchen… What’s this? A remodel?! To where hath mine beloved Franistan departed? Alas, my kingdom had been infringed upon by foreign furniture! And oh, for the love of Lucy, it smelled like sauerkraut! Nevertheless, this zonked Maharincess was delighted to crash in a bed that was much improved in comparison to the former model of yesteryear. Until someone, who shall remain nameless, started snoring. Ahem.
Anywho, shall we move on before I inadvertently embarrass anybody? Personally, I have always been one to save the best for last, so let’s get the disappointing tidbits out of the way upfront. While my meeting with the movement disorder neurologist was far from the worst appointment I have experienced, it could definitely be classified as disappointing. In order to streamline the dialogue of the appointment, we gave the doctor a synopsis of my health history that my mom and I had prepared in advance. At five pages, it wasn’t exactly “brief” per se, but considering that it summarized ten years of my very complicated past, one could certainly call it “abridged”. But honestly, no matter how my story is presented, it has a tendency to be rather intimidating. Right away the doctor was clearly flustered, stating that such a complex patient could never be evaluated in one short hour. He informed us that we were in the wrong kind of clinic. He had never seen anything the likes of my stubbornly fixated arms. Were we aware that we were in a movement disorders clinic? He treated Parkinson’s Disease, Huntington’s Disease, dystonia…
With me, there is a whole lot of guesswork. It is rare for me to fit the epitome of any diagnosis; so sometimes I simply have to settle for the next best thing. My arms do not resemble a typical case of dystonia. But sometimes fixed dystonia can present with my chronic pain condition, Reflex Sympathetic Dystrophy/Complex Regional Pain Syndrome (RSD/CRPS).
Although the appointment had an unfortunate start, I will give the doctor credit. He proceeded to give me my allotted one-hour consultation, listening to our questions and helping us brainstorm. By the end of the appointment he seemed to have grasped just how desperately I need answers, and obviously cared, but regrettably he had nothing to offer.
I always feel a hint of apprehension preceding a big medical trip. What if I make the long, arduous journey and the whole shebang is a bust? This is precisely why I prefer to have multiple appointments scheduled when traveling to see new doctors. If one is a flop, the trip may still be salvaged if the other specialist happens to be a keeper. I am more than a little happy to report that this technique was successful, as my appointment with Dr. Lubenow, the RSD/CRPS specialist, went well. He views me as a challenge. Which is fine… Honesty is far superior to ego! Devising a treatment plan for me is not a simple task. In the past I have exhausted the majority of the treatments for RSD. Additionally, the severity of my illness leaves me in a continually precarious state; oftentimes RSD patients have an intrathecal pump or spinal cord stimulator surgically implanted, but because of my pancytopenia, particularly my low white blood cell count, Dr. Lubenow doesn’t believe that I am a candidate. This opinion has been a common consensus between all of the pain management doctors who have weighed in on my case in years past, and as much as it pains me to scratch these interventions off my potential treatment list, I would have to agree.
At this time there are almost no viable treatment options for me, save one. For a few years now ketamine therapy has held the most promise of offering relief. Ketamine is a dissociative anesthetic that works in the brain to essentially inhibit the mind from receiving sensation of pain in the body. The utilization of ketamine infusions is a rising trend in the treatment of RSD, providing much-needed relief for many sufferers.
So what’s the catch? Hm, you know how well I do with anesthesia! And as I mentioned above, ketamine is a form of anesthesia. But hey, wait a sec… There may be hope yet. As you might remember, my last central line placement gave me reason to believe that my intense pain is triggering my paralysis. My anesthesiologist used a very minimal amount of anesthesia, suspecting that the amount of sedation was directly related to the induction of my paralysis. I was very much aware during this procedure, and my paralysis did not seem to present itself until the analgesic effects were wearing off. It was only when my extreme pain came rushing back that the paralysis hit. When I recounted the incident to Dr. Lubenow, he was inclined to agree with me. He believes it to be highly likely that an aggravation of my RSD is a primary factor contributing to my paralysis.
Tomorrow is a big day, and I am in need of prayer… Unfortunately it is necessary for me to undergo surgery once again for yet another central line placement. I’m beyond disappointed that I was unable to hold onto my current central line for little more than a few weeks, but thank goodness it lasted me through my trip to Chicago. Dr. Lubenow was really helpful in developing an appropriate anesthesia protocol in an effort to avoid exacerbating my RSD any further. He is suggesting that this go-around I receive a higher dose of ketamine plus propofol, resulting in a deeper sedation and a longer emergence, so that if all goes well my body won’t be shocked with such intense pain when I wake up… And if our hypothesis is correct, I might evade paralysis, get home to my Alice Eloise, and catch my breath in time to party like a rockstar (or a Maharincess!) on my birthday a few days later.
Last week an anesthesiologist at my (awesome new) local hospital spent over two hours with my mom and me. Although upon first thought the deeper sedation seems counterintuitive, after careful consideration the anesthesiologist agreed that Dr. Lubenow’s suggestions did indeed make sense for my odd presentation. He is even going to facilitate ketamine in the recovery room to be sure I have continual pain control.
Friends, please pray that this new approach might finally do the trick! And if I respond well to this, I think we all may be more optimistic about my proceeding with ketamine therapy. I will be returning to Chicago May 14th for an appointment with Dr. Lubenow to discuss my prospects.
My loyal friends who frequently check in here on my blog have probably grown practically numb to the bizarre nature of my illness. You’ve probably learned to sit back and read, simply taking my word regarding all of this chaos. I appreciate that… Because all too often, there are people who think that because they don’t understand what is going on here, there’s no way this monster of a disease could actually be real. How can pain be so intense that it causes paralysis? Why would IVs and central line placements cause someone to permanently lose the function of their arms? This is bonkers! I admit, on the surface it appears to be just that. And in a way, yes, it is entirely crazy. But while we are so very far away from full understanding, slowly we are realizing that there is some sense behind this madness. Allow me to give a basic explanation of just what havoc Reflex Sympathetic Dystrophy is capable of.
In your mind, revisit the most intense pain you have ever experienced. Maybe it was caused by a broken bone, a surgery, cancer, labor pains, or even amputation. Such pain is all-encompassing, it’s exhausting, it’s agonizing, isn’t it? But you survived. The pain faded. Sadly, RSD sufferers cannot say the same. According to the McGill Pain Scale, RSD is the most painful condition known to medicine. And for most patients, the pain is constant and unrelenting.
RSD is typically triggered by a simple injury, such as an ankle sprain, infection, or some other form of stress on the body. But instead of causing pain that lessens with time, the nervous system malfunctions, misfiring pain signals in a persistent cycle. The patient experiences intensifying pain that is far out of proportion to the initial stressor.
True, there are some individuals who receive therapy for a limb affected by RSD and go on to be free of pain and live normally. Still there is a significant demographic of sufferers in whom RSD spreads; some of us are affected by RSD that covers our entire body, even internal organs! The manners in which RSD can affect the body are many and strange. In addition to constant intense pain, symptoms may include but aren’t limited to swelling, extreme sensitivity to touch, skin changes in temperature and color, changes in hair growth, softening and thinning of bone, dystonia, migraines, sensitivity to light and sound, seizures, and paralysis.
It is thought that RSD can even affect the immune system in some patients. This is the key factor that brings some semblance of sense to my bizarre presentation. My doctors speculate that I have always had some genetic predisposition, either mitochondrial disease or some other mutation. A bite I received at camp followed by a staph infection a few months later triggered my immune system to go haywire, leading to RSD and Hemophagocytic Lymphohistiocytosis (HLH).
The extreme pain of RSD is a medical mystery. Every second I feel as though I have electricity racing through my body, and my skin burns as if it was on fire. And conventional pain management interventions have done nothing to quench it! I don’t say this to make you uncomfortable. But I can pull off a really stoic brave face, guys. I refuse to allow my pain to keep me from smiling. But right now, I need you to see through that for a few minutes. I need you to understand how real and intense this pain is.
I have a beautiful friend who is about to help me illustrate this point. From a medical standpoint, she is more like me than anyone else I have ever known.
This is my dear friend Jessica, and her darling doggy Molly!
When Jessica was eleven years old, she developed RSD in her leg after being sick with Mononucleosis. Thankfully with treatment her RSD went into remission for four years. But then she went away to summer camp, where she was bitten by a tick and contracted Lyme disease. Her RSD relapsed, and she became completely incapacitated by pain. She went from being an A+ student who was active in tennis, lacrosse, and gymnastics to being bed-bound, unable to walk, eat, or tolerate lights and sounds because of this systemic, full-body pain. And while she didn’t have HLH as I do, she did suffer from severe hematologic issues.
Nothing offered relief. Doctors had given up on her making a recovery. It would take a miracle for her to achieve remission.
Incidentally, Jessica just so happens to know somebody in the miracle business. One day Jessica very nearly went to her Father in heaven when she had an extreme reaction during a diagnostic test. But God turned the situation around and orchestrated a miracle. When Jessica was in the intensive care unit she had a young attending physician who was from Germany, studying in the United States to complete his specialty degree. When he saw Jessica, he said, “Oh my goodness, We have cured systemic RSD back home in Germany! At my hospital, we have been doing studies using ketamine to treat this disease!” Years later when Jessica and her family attempted to contact and thank the physician who initially led them to the treatment that would ultimately save her life, mysteriously the hospital had no record of that doctor having ever been there.
You know what they say: “God works in mysterious ways.” But even more so, He works in miraculous ways. Jessica was at the beginning of a mysterious, miraculous journey.
As a last effort, Jessica and her family pursued ketamine therapy. There are currently three forms of ketamine treatments. Two of these approaches are legal in the United States, and many patients have found relief through these types of ketamine infusions. However there is a more extreme method that has been studied in Germany and Mexico, but has never been legalized in this country. Jessica was told that her only hope of recovery was in Mexico where she could undergo a ketamine-induced coma.
Really? Of all places… Mexico? Doesn’t that sound kind of scary? I’m sure that it was very scary for Jessica to think of traveling to a foreign country in order to undergo this high-risk experimental treatment, especially when she was in such a fragile state. But what was even more scary was the prospect of living the rest of her life in this miserable condition.
So off to Mexico she went. Jessica and her mom spent over two years in Mexico while Jessica underwent multiple comas. No one thought that she would actually survive. But she surprised everybody when she woke up from a coma on her own, singing! She was in remission! The coma wasn’t a magical cure; Jessica faced a rehabilitation period that was wrought with challenges. But with the help of her superb care team in Mexico, she regained her health. Now she is home with her family in Florida, studying to become a physician’s assistant!
My old friends may remember Jessica, as I briefly shared her story a couple of years ago when I too was told that my best hope was to go to Mexico for the ketamine coma. Since then Jessica and I have been in contact, and although we have never met face-to-face, she has become one of my very favorite friends. Jessica still travels to Mexico on a regular basis for follow-up appointments with her doctors; we are hopeful that someday we will finally meet, maybe even in Mexico.
Don’t freak out on me, guys. It has yet to be determined if I am a candidate for the coma. And before moving forward with such a drastic procedure, I would certainly trial the lower-dose ketamine infusions available in this country. But I have spoken with Jessica’s doctor. He is a very special man… And we all know that I need very special doctors. Coma studies are not currently underway, but this doctor has offered to see me in Mexico to better evaluate my case.
Many common threads tie Jessica’s life to my own. But even more personal than our bond surrounding our complicated battles with RSD is our sisterhood in Christ. Jessica is a remarkable young lady in many ways, but I admire her most for her faith in the Lord. She will be honest and say that through her most painful days, it was disheartening to know that God was capable of taking her pain away, yet He continued to let her suffer. But she persisted in trusting Him. Jessica’s favorite Bible verse is Jeremiah 29:11:
“For I know the plans I have for you,” declares the Lord, “plans to prosper you and not to harm you, plans to give you hope and a future.”
Jessica never could have envisioned the incredible, prosperous plans that God had in store for her. He had a miracle of healing arranged for His very special daughter. Sometimes we beg and plead for God to carry us through our trials, only to emerge from those difficulties and forget to thank Him. Jessica, however, is shining for the Lord, allowing her miracle to share Christ’s boundless love with others. Last year she had the incredible opportunity to give her testimony before 27,000 people at the 2013 “Live Loved” Lutheran National Christian Youth Gathering! Jessica is sharing her unique journey to glorify the Lord and to further His kingdom in an awesome way. Just see for yourself!
You can read more of Jessica’s story on her blog, Jessica’s Journey.
It may seem as though a series of lucky (and not so lucky!) coincidences brought Jessica and me together. But the first time I spoke with Jessica she shared a saying with me that she learned during her time in Mexico:
“No existen las coincidencias, existe solo las Diosidencias!” — “There are no coincidences, only God-incidences!”
God works in magnificent ways, and oftentimes He does His work through people. Every single relationship is arranged by God. He knows the needs of His children, and works accordingly. I am so grateful that the Lord has blessed me with Jessica’s friendship. No one could so deeply relate to what I am experiencing. She continually inspires and encourages me, reminding me that, with God’s strength to support me, I can make it through even my most painful days! She knows… She has been there! Jessica is living proof that our God of love can and does perform miracles.Sarah Kathryn Frey