"For everything created by God is good, and nothing is to be rejected if it is received with thanksgiving."
1 Timothy 4:4
But I'm getting ahead of myself. It's been a couple of months since I have written a "what's going on in my life" journal update to share with you, and as you might have guessed, life is very seldom what one would consider humdrum around here! So let's get started and bring you up to speed, shall we?
Very soon after my last journal entry I received a much-needed blood transfusion. My blood counts had been on a gradual decline for quite a while, leaving me feeling very sick and fatigued. But thanks to the two units of blood I was given, naturally my hemoglobin perked right up. I am so grateful for the most amazing medical innovation of blood transfusions and for the donors who give this life-saving gift!
But as is always the case, due to my lagging bone marrow my blood counts all too quickly settled back into the low range. Thankfully my hemoglobin has not reached a critical point requiring intervention, but I recently had a clinic visit with my hematologist/oncologist, Dr. Wilson, and he is working with us to determine the best way to manage this issue.
My iron levels have been a bit on the low side as of late. The tricky thing is, low iron can cause low hemoglobin, BUT conversely low hemoglobin can also cause low iron! The primary solutions are administering either iron infusions or blood transfusions. Sometimes mitochondrial disease patients can have catastrophic reactions to iron supplementation, so as a precaution we are avoiding this treatment for now. We are planning to go forward with blood transfusions as needed, and of course we are hoping that "as needed" won't prove to be a frequent occurrence!
When I went to the hospital, I had a little surprise in tow for Dr. Wilson. Everyone's favorite Hungarian jamstress (that is to say yours truly, Sabrina Giselle, of course) arrived bearing triple berry jam. But I do believe that, as delicious as the jam may have been, it was outdone on the happy meter by another unanticipated treat that was awaiting Dr. Wilson on the other side of the exam room door.
Dr. Wilson gave a delighted "Oh!" upon entering the room to find a fluffy red bundle of pure joy sitting politely with her chin in her mama's lap. That's right, my newly accredited service dog Alice Eloise made her premiere outing to St. Louis Children's Hospital!
A couple of years ago I was inpatient with an HLH flare precisely when Alice Eloise was supposed to come to her new home to live with me. I was very driven to be released from that hospital pronto, as I was just itching to be with the darling puppy whose picture was on display in my hospital room, as if to incite my doctors to work with exceptional diligence to help me feel better. Fast forward to my clinic visit two years later, and Dr. Wilson couldn't believe that my little Doodle pup had already blossomed into a service dog. Now tell me, how do babies grow up so quickly?
Due to the nature of work of some service dogs, their handlers don't like for people to pet them while on duty. But I love it when people stop to talk with Alice Eloise and me! And with the number of doctors, patients, and nurses who took the time to admire Alice Eloise, my sweet service dog concluded that she is perfectly delighted with her new career indeed!
Service dog Alice Eloise had the opportunity to try her hand (or paw, as the case may be) at therapy dogship. Alice, Mom, and I had the pleasure of swinging by 9 East that day to visit my new friend Sam who was inpatient and undergoing chemotherapy. I know from experience how much a person can grow lonesome for their furry friend at home during a hospital stay. Alice Eloise brought Sam a little bit of puppy love that I'm sure he was needing. We hope it added some sunshine to his day!
My parents, Alice Eloise, and I were supposed to be traveling to Cincinnati Children's Hospital next month to see both my mitochondrial disease specialist and my palliative care doctor. But Mom called the genetic counselor this past week to inquire about the status of my pending exome sequencing, and we were given the regrettable news that it appears as though my results will not have returned by our intended appointment date. So now we are postponing the trip until these results are in. This was disappointing for me not only because I am more than ready to have the answers this testing may provide, but also because I was eagerly anticipating the splendiferous time that Alice Eloise and I would have on a gorgeous autumnal excursion to the Cincinnati Zoo! Ah well, c'est la vie.
It's difficult to sit patiently and just wait for things to happen. So while my exome sequencing takes it's jolly time to be completed, my family and I are feeling the need to do some additional research ourselves, continuing the hunt for answers concerning aspects of my illness that will not be provided through my exome testing.
As I have mentioned in the past, I am hypermobile. This means that my body is very flexible, allowing me to easily bend in such a way that appears to be quite unnatural to most people, much akin to Gumby and Pokey of claymation fame. So I was the kid who always won the limbo, who could jump rope with her arms, and who had mastered a number of "stupid human tricks", spectacles that garnered plenty of "oohs" and "aahs" and "Sarah don't do thats". Hypermobility is typically a benign trait, but recently we have become more aware that in some individuals it can come along with a slew of serious health complications. Hypermobility is a signature symptom of a group of connective tissue disorders collectively referred to as Ehlers-Danlos. I was given a clinical diagnosis of Ehlers-Danlos many years ago. But currently there is only genetic testing available for the life-threatening form of vascular Ehlers-Danlos, and once it was determined that I do not have this form we assumed that there wasn't much need for concern.
In 2009 I began having severe headaches and extreme light sensitivity, maladies that I have dealt with everyday since. I was seen by a neuropthalmologist who was concerned that I may have Chiari malformation, a condition in which brain tissue extends into the spinal canal. However, this hunch was subsequently disregarded based on a previously performed MRI.
But recently a girl named Lissy and her mom, Linda, reached out to me when they stumbled upon my blog. Since then I have had the pleasure of getting to know them, and I am so grateful to have them as my friends! God has a way of bringing incredible people into our lives, blessing us in ways that we could never anticipate.
In addition to being one of the sweetest young ladies I know, Lissy had Chiari herself. She brought it to my attention that a single MRI is not adequate screening for Chiari, but rather a suspected patient should undergo very specific imaging to be evaluated by a Chiari specialist before confirming or dismissing a diagnosis. Unbeknownst to my family and me when we cursorily investigated connective tissue disorders so long ago, Chiari commonly coincides with Ehlers-Danlos. And while it couldn't explain all of my symptoms, oftentimes Chiari patients do present with quite a few of the problems that I myself have.
So taking this knowledge into consideration it would be foolish not to further investigate Chiari as a possible factor in my illness, wouldn't it? There are a handful of medical centers with expertise in Chiari, but when a person is traveling across the country to see a doctor, it's a bit unsettling not knowing what they will be walking into. I have lived through a few too many horror stories involving arrogant doctors; but Lissy has already traveled the Chiari path, so she and her mom were kind enough to suggest the neurosurgeon that they trust and love. So we are working to attain a good referral from one of my current doctors, and if all goes as we are hoping, I will have an evaluation with this Chiari specialist in Bethesda, Maryland, in the not too distant future!
Recently I have been in close contact with my palliative care doctor in Cincinnati, Dr. Meyer. We have had a few conference calls to discuss the options to manage my pain. Dr. Meyer has quickly become one of my very favorite doctors. For years I have battled such intense pain, and although I have some very kind and intelligent doctors who wish they could help, they don't want to step beyond their specialty trying to treat my pain and inadvertently hurt me due to the complexities of my illness. I am so thankful to have Dr. Meyer on my team! My sensitivities to medications and my inability to take medicine orally due to digestive tract paralysis very much limits my treatment options. Dr. Meyer has really stepped up in his attempts to help me.
But my goodness, even with the aid of a fantastic doctor it can be so complicated to attain proper pain control. Dr. Meyer is prescribing a medication that he believes could benefit me in IV form since my body does not absorb or tolerate oral medications. But my current home health care company doesn't supply this medication! So right now we are all jumping through hoops (and I don't mean the fun type of hoop jumps that Alice Eloise does) as we try to decide the ideal way to proceed. We are facing a tough call about transferring my care to a home health company who could take on my case and supply this new medication along with my TPN and other IV medications. This company has a considerable patient base affected by mitochondrial disease, so it really appears as if it could be a nice fit for me.
My only qualm is that I would lose my current nurse, Jenny, and I just love her so much! I don't have the opportunity to see friends nearly as often as I would like, so I have come to delight in my visits with Jenny every Tuesday morning when she comes to change my central line dressing and to draw blood for my lab work. She perfectly indulges my need to engage in silly banter, carrying on many a witty conversation with me. And bonus, she likes my cooking! But we share a special bond in that she cared for two little brothers from St. Louis who passed away a few years back after fighting HLH, the rare hematologic condition that I suffer from. From this experience Jenny gained firsthand insight into this devastating disease in a way that so few people can understand. Simply put, Jenny and I have gotten along swimmingly from our very first meeting.
It's not always easy to find a nurse who clicks with you. I've had some nurses whose lack of knowledge regarding central lines and TPN was downright scary. And I've had nurses who were just... I don't even know. I tend to like weird people in general, but there are certain types of wacky that are just too much for me. For instance, when I spent three and a half months away from home I had a most, er, interesting nurse. She liked to spend her free time at the discotheque. She was perfectly nice, and I wish her nothing but many happy years full of boogie nights, but I'm not so sure I'm groovy enough to be a part of that scene. Anyway, I am sure that everything will work out fine, but I can't help being quite nervous about the whole thing. Really, what are the chances of my having yet another disco diva as my nurse?
Now that's what I get for waiting so long in between journal posts: One stinking long account of my recent medical happenings! I don't mind it so much, but I do apologize to you who are reading this... That is, if you are indeed still reading, dear friend.
To be completely honest all of these loose ends and unknowns have been weighing on me the past few weeks. Decision making isn't my forte. Sometimes I am so exhausted I need my mom to decide between "Pippi Longstocking" and "Roman Holiday" for my nightly entertainment! But I am praying that God will guide us to make wise choices that will result in the best plan for my care. He always reveals His perfect will in time!
"Trust in the Lord with all your heart and lean not on your own understanding;
in all your ways submit to Him, and He will make your paths straight..."
At the same time, I'm making an effort to focus more of my prayers on giving thanks. This isn't exactly a novel idea. (Just ask the Apostle Paul!) But whenever we are in the middle of stressful and painful circumstances, it only makes sense that we will feel more joyful if we concentrate on the many blessings granted to us, right? And I am certain that it pleases God when we remember to say "thank you", too!
One of my best friends, Tiffany, once asked me how I manage to keep smiling after a particularly tough day. I quickly responded that on my most difficult days, I usually watch "Winnie the Pooh", as that silly old bear can always give me a giggle. Tiffany was momentarily taken aback by this true but unexpected answer... But then again, Tiffany knows me well enough by now to expect the unexpected! The real secret behind my smiles isn't a secret at all; lasting happiness is something that Winnie the Pooh could never provide, but it comes from simply being grateful for all that God has given us. I admire the integrity of others who can live through truly distressing experiences and not become bitter, but instead share God's boundless saving grace with those around them. I believe God wants to give all of us this perspective if only we would open our hearts to it! A thankful heart is a happy heart.
The stress I am feeling right now is minuscule in comparison to what the Lord has helped me to overcome in the past. And what's more, I know that this stress is just a part of the efforts that will hopefully reduce my pain in the future... For that I am genuinely grateful!
Even with all of this medical hoopla, I've managed to fit a bit of fun into my hectic schedule, as is my custom. I've fallen into a time warp and gotten in touch with my 90's child roots as I've relapsed into my long-forgotten Spice Girls groupie behavior. I thought it would be diverting to revisit the bygone cinematic "masterpiece" of my childhood, "Spice World", you know, just to see how ridiculous it is now that I am not six years old. Blimey! To my utter shock, I found "Spice World" to be prime entertainment; and while I'm being unabashedly honest here, I might as well confess that I've watched it at least five times in the past month. The Spice Girls albums are once again an integral component of my music library. I purchased and am devouring a book entitled "Real Life, Real Spice". And I am seriously contemplating putting my old Spice Girl dolls, which I presume are now highly sought-after collectibles, on display in my bedroom once again. Girl Power!
Now to answer the riveting question on everyone's lips: Who is my favorite Spice Girl? For me, Baby Spice has always been the star! So, who's YOUR favorite Spice?
And One Final Thought...
Before I sign off, I want to take a few moments to bring attention to the fact that August is Digestive Tract Paralysis (DTP) Awareness Month. Writing about the particulars of the digestive tract certainly doesn't make for exquisite prose, but sometimes it simply must be done!
You know that painful "I-feel-so-full-I-could-burst" sensation that you had on Thanksgiving when you decided to go for that fourth piece of pecan pie? Or when you had the stomach flu for a few dreadful days that seemed never-ending? Many DTP patients experience these pains constantly.
My blog gains quite a bit of traffic from search engine queries regarding digestive tract paralysis. This tells me that unfortunately there are many people who are in need of answers to their belly problems. I experienced severe abdominal pain for five years before I knew to request that I be tested for gut dysmotility disorders... And if it's up to me, I would like to prevent other sufferers from waiting in pain for such a long time before receiving a diagnosis.
Eating is such a natural thing that it is usually taken for granted, but severe cases of DTP leave patients completely unable to eat! Such patients require nutritional support through enteral nutrition involving feeding tubes placed in the stomach or in the jejunum. And people who cannot tolerate these types of feeds depend on a riskier alternative called total parenteral nutrition (TPN) in which they receive intravenously administered nutrition through a central venous catheter.
There are not nearly enough effective treatment options available for DTP patients, so raising awareness and promoting education about DTP is essential as a means to support new research!
So to all of my friends who are able to eat, would you do me a favor? Next time you dig into a scrumptious blueberry bagel, a delicacy I reminisce upon most fondly, please remember me and the other DTP patients who so desperately need help. After all, blueberry bagels are a blessing to be very thankful for!
* For more information, check out my page "What is Digestive Tract Paralysis?
", along with these insightful websites:
• Learn about DTP at www.g-pact.org
• Learn about life on enteral and parenteral feeds at www.oley.org
Sarah Kathryn Frey
Have you ever thought of "thankfulness" as a blessing? As much as we wish we could avoid unpleasantness completely, we all have "one of those days" from time to time. And I've had a quite few of "those days" in these past weeks. But God is always faithful in continually giving me little reminders to be thankful, especially lately when I have needed a boost... And a thankful heart does a lot to lift a tired spirit!