10 months. 260 days today since Shelby fell ill. 6,240 hours of pain. 374,400 minutes of wondering what is wrong with her abdomen. But who is counting?
Shelby is. I am. We all are. But we are not counting backwards, but looking forward towards a trip to Wisconsin where a team takes on dysautonomia and challenging gut issues daily. We do not know a date but only know that the team has accepted her case.
As we wait anxiously and expectantly that the team will have an answer, a treatment, a plan to let Shelby be pain free and eat again, I have pondered about the journey we have been on. When I started this blog, my intention was to write frequently. But as Shelby’s health declined more and more, it became hard to keep up. Squeezing in a Facebook post to let family and friends know what was going on was a challenge at times. So as we await communication from Wisconsin, I thought I would recap the 10 month journey.
Before we walk the journey of the last 10 months, it is important to know that Shelby is a rock star in managing physical pain. In 2008, she hurt her ankle when jumping off of a trampoline. Reflex Sympathetic Dystrophy (RSD), also known as Chronic Regional Pain Syndrome (CRPS), set into her left foot. In 2009, she went into remission after intensive physical therapy, only to flare in 2012. After numerous epidurals and sympathetic nerve blocks to control the pain, Shelby attended the Mayo Clinic Pediatric Pain Rehab Clinic (PRC) in summer 2012 and learned to live with chronic pain. She went into her freshman year at Sam Houston High School as a confident, strong young woman who served in Texan Corp, as president of Young Life and as an AVID officer, and who participated in drama and yearbook. Shelby was diagnosed with neuro-cardiogenic syncope and Ehlers-Danlos Syndrome type 1 in 2013, but she worked hard to create a life full of living, helping others, and working towards her goal of a career in medicine.
Columbus Day – a day off! Shelby and I were shopping with her friend Olivia and she said her stomach hurt. Nausea followed and by Wednesday, October 15, Shelby could not stand as the pain was so bad. Into the ER we went, expecting a gallstone, a kidney infection, a something easy. Shelby was admitted for 6 days, even participating by Facetime to be inducted into the National Honor Society at Sam Houston High School. Told it was probably just a virus and weird body response, Shelby went home eating a soft, low fat diet.
10 days later and 10 pound lighter, Shelby passed out from the pain. Into the hospital for 17 days – with every diagnosis thrown around that a private, non-research hospital would think of – h pylori, obstruction, gallbladder disease, kidney stones – even porphyria. After every test came back normal, near normal, slightly off but not definitive, a GI doctor looked at me and asked, “Can’t you just call your friends at Mayo and have them figure it out?” I had to reply, “Just because our last name is Mayo does not mean we have a team of doctors at our disposal!”
A second opinion from Children’s Dallas urged us to self-refer to the Mayo Clinic. On November 18, we completed the referral process for the Pediatric Diagnostic Clinic at Mayo to see Shelby. At the end of that week, Shelby was walking like a drunk woman! Tremors filled her body and she acted drunk. We boogied back into the original treating hospital for the 4 worst days ever. Shelby was overdosed on medication by a doctor, we were told all of Shelby’s symptoms were in her head 36 hours after the overdose, and were reported to CPS for medial neglect of Shelby. I am not going into more details at this point, but continue to stand amazed that after 30 days inpatient and an overdose by a doctor led to a CPS report on us. We left the hospital and maintained a daily therapy routine, where Shelby saw specialists, physical therapists, psychiatrists, counselors to keep her alive until we got to the Mayo Clinic.
December 16 brought another dark day, as Shelby was taken with lights and sirens to Children’s Dallas with seizures. No meds worked – and after testing, we learned that the non-epileptic seizures were her body’s response to pain. Pain so bad that Shelby passed out and seized continually. Shelby was sent home on pain medication and we counted the days until we left for Mayo.
Shelby awoke thrashing over the holidays. Her legs were moving all over the place! After our fabulous PT suggested looking at restless leg research, I read that antacids and antihistamines made restless leg worse. Of course, that’s what I had given her prior to bed. Three days later, I weaned Shelby off of Prevacid – and her tremors stopped. Completely. Her pain was still bad and seizures continued, but her tremors were gone. Although the tremors were gone, Shelby had drop attacks, where he legs refused to work, that remained. What was it about Prevacid?
We would find out at Mayo. On the Friday of our first week at Mayo, Dr. Rayna Groethe, probably the smartest woman I have ever met, scoped Shelby. Dr. Groethe, a tall woman, came charging into the waiting room to talk to me after the scopes. She explained that Shelby had stomach ulcers and she did not believe that Shelby could process medicines so she was testing for that. CONFIRMED! A ZEBRA had been found amongst the sea of horses at Mayo! Further testing showed that Shelby does not have the liver enzymes to process 3 major categories of medicines! At least we now knew this information, but the treatment for ulcers is not medicine that Shelby can process. In addition, gall bladder testing, MRI testing, and the scopes all showed that Shelby’s gut was acting in a hyperactive state. Dr. Groethe stated this was the first case of a child diagnosed with a HYPO-motility disorder now demonstrating a HYPER-motility issue. After a week of gut rest with a NJ tube and feeds, testing for mast cell activation disorder, and a lot of physical therapy, Shelby was better and we returned to DFW after 5 weeks of testing and treatment at Mayo.
On the drive home, I attempted to schedule follow up appointments, only to find that the doctors we had in place that sent us to Mayo were not comfortable treating Shelby upon our return. SHELBY IS TRICKY – we heard over and over again. In came our wonderful doctor, Dr. Michele Burzynski, who coordinates Shelby’s care and treatments.
After returning, Shelby attempted school for 3 days, and attempted to eat, and live. Downhill, rapidly, Shelby went. Further follow up testing was done locally and Shelby became unable to eat anything without vomiting. First a PIC line was placed for feeds and then a Groushon Catherter was placed for IV nutrition in April and May. Once nutrition improved, Shelby was able to walk freely! Less use of her wheelchair and less drop attacks with her legs but more constant stomach pain ensued. In late April, Shelby was sent to UT Houston to their dysautonomia neurologist for evaluation. Dr. Butler agreed to take Shelby as a patient and into their research program, and suggested that we see Dr. Van Arsdall, the GI that specializes in dysautonomia at UT Houston. Dr. VanArsdall, a wonderful GI, has completed testing and now referred Shelby to the Chelimsky team at Children’s Hospital of WI – as they do tricky all the time.
Through it all, discussion about Shelby’s stomach focused on mast cell, gall bladder disease, median actuate ligament syndrome (MALS) and sphincter of odi dysfunction. Because Shelby has Ehlers-Danlos Syndrome, which is a connective tissue disease where collagen (glue that holds your body together) is under-produced, and because she has a pain condition where her brain perceives pain differently, SHELBY IS TRICKY.
So now we wait. Patiently. Expectantly. In hopes that Shelby will be able to Eat. Drive. Walk. Stay alert. Sleep. Run. Attend school. Work. Skip. Swim. Go to a restaurant. LIVE AGAIN.
To join us in helping Shelby LIVE AGAIN, we are counting on donors!
1000 give $10 in 10 days! Please consider joining our team!