Only five weeks after my tethered cord surgery, I was back in the OR. My nurse woke me up at 4:15 AM to prep for surgery and give me a pre-surgery bath. At 6 AM, I was taken back to preop. This time, both of my parents were allowed to go back with me since Covid restrictions had relaxed a bit.
Once again, I decided to go into the OR awake to meet my medical team and see the operating room in action. The surgical team was so kind, they cracked jokes and explained the procedure to comfort me. This time they even let me pick a song to play aloud as the anesthesia was given to me. My selection: We Are the Champions by Queen.
When I woke up, my pain was in control, much different. The new protocol for EDS patients post-surgery was ketamine. This strong non-opioid drug helped much better than the anesthesia and medicine I was given during my previous surgery. I was in the Neuroscience Intermediate Unit, which is the step-down ICU, so I could be closely monitored. Evidently, the guideline was to have the ketamine in a locked box on the medicine pole next to the hospital bed. With this fusion surgery, a three-inch incision was made in the front of my neck, and my esophagus was shifted to access my spine.
The ketamine made my vision hallucinogenic. The clouds out the window seemed to shifting mountains and the ceiling seemed to be moving and at times even crumbling. It was scary to me as I didn’t feel like I had any control. After about a day on ketamine, my eyes started rhythmically vibrating back and forth and it was difficult to see anything at all. This new symptom only accelerated my vertigo and nausea. Apparently, this is called nystagmus and it can be a side effect of ketamine. Even though I was told this could happen and was temporary, it was terrifying to think it might not go away.
My dad’s parents came to town to help hold down the fort during this surgery. They helped run errands, make dinner for my younger three siblings while my parents were attending to me. My grampa sat with me a couple times in the hospital. I know it was difficult for him to see me in this condition.
They weaned me off the ketamine and put me on a hefty dose of opioids. The next day, the pain grew out of control and there was a new shooting, burning, electric shock like pain throughout my body. I took a combination of morphine, dilaudid, oxycodone, fentanyl, muscle relaxers, and nerve pain medications but nothing seemed to help. Ironically, I seemed to be in more pain taking most of those medications. There would be slight relief for the first fifteen minutes and then the pain returned to a greater degree.
My parents took shifts sleeping at the hospital. They ended up bringing an air mattress because sleeping in a chair just didn’t work. They were exhausted and emotional from seeing me in so much pain.
A couple days later, I woke up in the hospital convulsing. I thought I was having a seizure or dying and during that moment no one was there with me. My dad had briefly left my room for a call and my mom was with my other three siblings and wasn’t scheduled to return until after lunch. When I tried to reach for my phone and press the call bell, I could not voluntarily move my arm; it kept convulsing violently with the rest of my body. Screaming, as loud as my lungs would allow, the staff rushed in to help me. All I remember after that is nurses and doctors flooding around me frantically and saying that my vitals weren’t good. I have never been so scared in my life. I thought I was going to die. There were so many things I wanted to do in my life and so many experiences I had yet to have. Although in tremendous pain, I wasn’t ready for my life to end.
The next thing I remember is waking up in the Neuro ICU. my parents explained to me that I had experienced opioid-induced hyperalgesia. This is when taking opioids has the opposite effect and causes more pain receptors, therefore more pain. The possibility of this condition had never been mentioned to us. The medical team transferred me to the neuro ICU and put me on a high dose of ketamine; titrating all the way up to 7.75 mg/kg. All opioids were discontinued. The pain I felt was so excruciating. My neck muscles spasmed, my migraine roared, vertigo persisted, but my gastrointestinal symptoms were the worst. Ironically, the surgical incision was the least painful.
One parent was allowed to sit with me in the ICU, but they were not permitted to sleep. Blow-up mattresses were not allowed in the Neuro ICU. My mom was with me the first night. Despite cameras in the room, she timed the nurse visits and after about 2a laid down on the floor until right before the 6a morning shift. In different ways, we were all just surviving.
I thought I might be experiencing Superior Mesenteric Artery Syndrome (SMAS) again because the stomach pain felt so similar to what I experienced in September and October. I had sharp pains at the very top of my stomach, and the soft part in between my ribs. I also had intense cramping in my lower stomach and it felt like there was a blow torch from my esophagus to my belly button. I had hardly eaten since being admitted for surgery and stopped eating and drinking completely on day five post surgery.
The medical team helping me seemed worried and perplexed. My mom was adamant more tests be run on my stomach. A CT scan with contrast was ordered. It was important for me to drink three bottles of liquid oral contrast to help the radiologist read it. Drinking was so painful and I moaned in pain through it. My mom set up her computer and played a show called ‘Inventing Anna’ in hopes it would distract me. I could hardly see it, as my eyes could not focus, but listening to it helped pass the time a little. The only thing the CT showed was fecal impaction. It was disappointing, as we knew it was more than that, and not knowing was unnerving.
My dad was distraught and quiet, but I knew he was talking to his MUSC colleagues in hopes of helping me. My mom seemed a bit manic-like, hardly slept at the hospital, exploring anything and everything on the internet. My stomach proceeded to get worse and I was unable to eat, drink, or take meds by mouth. For five days, I ate and drank nothing. I was given IV saline with glucose in it. My body was getting thinner, weaker, and my face pale. The medical team became more concerned. We all agreed an NJ tube would need to be placed again, as nutrition is critical.
The medical team, in concert with my parents, decided that it was best to move me from the neuroscience wing to the Children’s Hospital. No doubt, my case was complex, there were so many symptoms that spanned multiple specialties, it seemed to make sense to get to the Children’s Hospital so I would have easier access. Sadly, there is even less knowledge and awareness of connective tissue disorders/EDS in the pediatric setting. It’s as if they don’t study or even discuss it in medical school. Perhaps this is because it takes so long for it to be identified by healthcare professionals that a patient is already an adult once she finally receives the proper diagnosis. I say “she” as almost 90% of the patients who present with symptoms are female.
Other than the transfer from the adult University Hospital to the Children’s Hospital by ambulance, I hadn’t left my hospital bed (used a bedpan). Sometimes my mom would get something in her head and she wouldn’t let it go. This time, it was “we can’t hit the two-week mark without bathing.” She called a proverbial ‘code red’ and two nurses put me in the wheelchair and hosed me down in the shower. Because of the hyperalgesia on my skin, the water felt like hornet stings when it hit me. I sobbed. Two minutes in the shower felt like an eternity. Even though I was wearing my neck brace, sitting upright made me so incredibly dizzy. I was spinning, vision was blurry, head felt intense pressure, like it was going to explode and my skin was on fire. We stopped the shower early and didn’t get to my hair, which hadn’t been brushed in well over two weeks. On the way back to the bed, I got a glimpse of myself in the mirror. My reflection was unrecognizable. In that moment, I realized there was no way to save my hair. The next day, most of it would be cut off.
Millie came to town and helped me cut my hair. It was so dreaded and rubbing against my neck, which hurt. I couldn’t sit upright without the symptoms getting worse, so she and one of my nurses cut off the dreads as best as possible while I was lying down in bed. I went from having long hair down my back to a short jagged bob. Not ideal, but necessary.
My mom was emphatic that more tests should be run. She felt we were missing key pieces of the puzzle. Yet, the hospitalist did not share her urgency. Dr. Patel seemed concerned I might have MALS and suggested testing for this. The hospitalist’s response was that even if I had MALS, I wasn’t strong enough to do the surgery, so why do the testing. And, he said even if the gallbladder was not functioning well, it’s not like I was in a situation to have it removed. The hospitalist stance was unnerving to my mother and disappointing to me. It seemed either lazy or a path a doctor takes when they give up. We wanted to know the “why’ behind the ‘what’. The hospitalist’s solution to my every issue was nutrition. Yes, nutrition is critically important, however, I was receiving it from the NJ tube and felt no better.
Although the pain had not subsided, once my vitals were in a better range, I begged to go home. The Children’s Hospital was not able to help me with pain control. Taking opioids was not an option given my opioid-induced hyperalgesia. There was really no reason to stay. After sixteen very long days, I returned home.