Getting a Diagnosis Part III of III

Days 49-51: February 21st – 23rd, 2017 

Location: Johns Hopkins University Hospital – Baltimore, MD

 

This blog post is the third of a three-part series in which I received my diagnosis.

You can read Part I, here.

You can read part II, here.

 

Rise and shine. Well, not exactly shine as the sun on Tuesday February 21, 2017 had yet to rise in Charm City. It was still plenty dark outside once the Hopkins staff woke me up to transport me from my bed in the neurology department to begin several tests for the day in advance of meeting the doctors sometime around 11:00am.

 

First on my test docket was a pair of x-rays. Dr. Ivan wrote for a hand x-ray as well as a chest x-ray. The hand x-ray was to help make sure there were no broken bones or something otherwise troublesome in terms of the hand’s structure. The chest x-ray was to examine the bones in my brachial plexus for all of the shaking my chest was doing.

 

When placing my hand on the x-ray table, it hurt so bad to attempt to twist, turn, and hold the x-ray pose for the images to be taken. Even just the burning sensation of having my fire engine red skin touching the table was awful. The simple touch burned. The process brought me to tears. I knew I had to grin and bear it because it was a step towards getting well. It sucked and maybe one day there will be a better way for CRPS patients. The chest x-ray wasn’t painful in anyway. I simply struggled to stay still enough so they could take their pictures. The shaking was completely involuntary so it took a few attempts. Once finished, it was time for breakfast before going for 2.5-3 hours of three additional imaging in the form of two MRIs (one of my head and one of my brachial plexus) and one MRV (of my head).

The hospital transport staff wheeled me through the bowels of the hospital in order to take me to the MRI facility. I remember being wheeled through in quite a daze. I was operating on a few hours sleep with intermittent tramadol since about midnight. While going through in the wheel chair, I remember feeling quite anxious. The length in the MRI machine coupled with pain still seeping through the tramadol and I was fearful of a rough experience. The one thing that helped me remain calm, I kept thinking to myself that I should be able to see my wife shortly after the test was done. I should be able to hold her hand again with hope we would get answers.

 

When it was my turn to stand up and get onto the MRI table, I felt so weak I could barely get out of the chair. A nurse, exam tech, and transport staff member had to lift me up and out. I was pretty much completely debilitated by this point. Once on the table part of the machine, I noticed how cold the room was. My chest was constantly shaking almost like I was shivering anyway so why not throw in my whole body at this point. Thankfully, they had some blankets for my lower half to at least keep my legs and feet warm.

 

Remaining still enough for the exam was difficult, though not impossible. I wasn’t trained in mindful meditation at the time so what did I do to put my mind at ease? I thought about our recent honeymoon split between Napa Valley and Riviera Maya. I thought of my wife’s beautiful smile, gorgeous eyes, and how relaxed we were in both serene places. Eventually, my thoughts carried me into a light sleep – enough to block out the loud noises from the machine. The memories warmed my body too.

 

After the exams were over, I was placed in a waiting area until someone from transport could bring me back to my hotel room. While there, an infant was brought in for an MRI of some sort. My heart and mind were weakened when seeing such a small child in such a vulnerable spot. It also gave me courage that a baby might be challenged with far worse and that I could control my part of whatever and whenever recovery would begin for me.

 

While I was undergoing the exams, my wife and my parents arrived back at Hopkins. A special guest arrived too – one of the Hopkins board members who came to make sure the accommodations were working well for us and that the level of care was up to par. We learned that he came at the request of Mark Futrovsky, my cousin Todd Forster’s partner at the law firm, Futrovsky Forster Scherr LLC. I actually interned for Mark and my cousin way back in the day. The strings we pulled in order to get beyond the ER much more quickly.

 

Awaiting me in my hospital room were my wife and my parents. Seeing each of their faces for the first time that day warmed my spirits. I felt awake for the first time all day. That said, I also was greatly looking forward to resting at some point later on. The neurologists were in shortly thereafter.

 

Johns Hopkins neurology is ranked #2 in the world only trailing the Mayo Clinic. My neurology team at Hopkins was lead by doctor and professor of neurology, Dr. Nick Maragakis. Dr. Maragakis came into the room with an army of residents and med students. I couldn’t tell the latter two types from one another though for the first time in my life, doctors younger than me surrounded me. Not to worry, all of them seemed infinitely smarter than me too.

Dr. Maragakis ran through the initial set neurological tests with me including following my vision and my speech, testing motor function, and testing my strength. He then had one of the residents begin testing skin sensitivity. The particular resident was a bit of a light-hearted jokester who helped keep this loose considering the touch of his fingers on my hand or wrist sent my pain through the roof. He eventually began taking a safety pin and using the sharp end, he poked my skin tracing nerves from the fingers all the way up through my head, neck, and chest. Some spots triggered more reaction and pain than others. After a few more minutes of a room full of people observing me and taking notes, they began walking me through the next steps of their evaluation and treatment plan.

 

I learned I would likely be at the hospital for most or all of their week. They had the following goals: 1) identify what was plaguing me 2) keep me comfortable 3) get me up and walking a little each day 4) begin occupational therapy while in-patient 5) come up with a treatment plan and 6) get me home within a week. We discussed my neurologist, Dr. Sethi, thought I might be exhibiting Complex Regional Pain Syndrome though Dr. Maragakis and his team were not entirely convinced. They told me they read all of Dr. Sethi’s reports and concluded it was a “million dollar workup and amazing work.” They simply said CRPS should not be as advanced and progressive as it was in just 6 weeks. The team did ask if I had any traumatic injury or perhaps a noxious event that would’ve affected my left arm within the past 12 months. The only thing I could think of was a piece of glass that cut my finger and got stuck about 6 months prior to that window. Dr. Maragakis and his team said that would typically be too far outside of the realm of possibility to trigger CRPS. They didn’t want to take guesses as to what else I could have been plagued with so they went more in-depth with what they were going to do to evaluate me.

 

Dr. Maragakis and his team developed a plan that included a new round of EMG & ENG tests; blood work for over 150 tests including cancer and several other big named diseases; a rheumatology evaluation, a pain management evaluation, and a the aforementioned occupational therapy. I’ll be brutally honest, hearing the intent to test for cancer freaked me out. Thankfully, I was done with imaging and they had all of the pictures they wanted. Depending upon what they found, that would of course affect my treatment plan. My schedule for the remainder of the day would include my first appointment with pain management and my first appointment with occupational therapy.

 

Pain management came to my room not too long after the neurology team had left. I managed to sneak in about 30 minutes of sleep while my family surrounded me. Dr. Tina Doshi represented Johns Hopkins pain management. She is a pain management specialist and a professor. She brought a resident fellow with her as they now ran a similar examination as Dr. Maragakis and his team. They also didn’t want to discuss much at this point in time though they thought CRPS was potentially within the realm of possibility. I remember they increased the dosage of tramadol to help me get more rest. I wouldn’t see the pair again until the next day, Wednesday, after the collective group had more test results.

 

Regardless of what my diagnosis would eventually turn out to be, I was going to require re-learning how to use my left hand, fingers, wrist, and shoulder. What I didn’t know was just how long of a process that it is to do so. I had no idea that a year later I would still be working to regain strength. I guess I just thought it would be relatively similar to physical therapy work. Boy, was I wrong.

 

 

At my first in-patient occupational therapy appointment, the doctor of physical therapy got me and up and walking to start out. We walked a few laps around the neurology wing as my wife and father walked with us. Then, we went into the occupational therapy nook of the neurology department to test some measurements and try some very basic exercises with a squishy sponge. In order to take mobility measurements with basic motor functions like moving my fingers or my wrist, I was literally using every ounce of strength I had in my body to get them to move or wiggle. There was some very minor movement through all of the swelling. When we moved onward to start gripping and squeezing a soft yellow sponge, I was asked to squeeze it for two sets of ten. It was a soft sponge, it cannot be too hard to grip can it? Wow, I was wrong. Struggling to do this just like I was with movement, I ended up getting faint, light-headed, and nauseous from trying to squeeze the sponge. I nearly passed out. The DPT had to lay me down and cool me off to get me to snap out of it. I fainted in front of my wife and father. I’m not sure if I was more embarrassed or concerned that I scared two of the most integral people in my life. While I walked to OT room, I ended up taking a wheelchair back to the hospital bed. I was done for the day. It was time to rest.

 

The lone bright spot of a rough week in the hospital is the fact that Johns Hopkins is close to the best Italian deli in Baltimore, DiPasquale’s. While I slept, Crissy and my parents grew hungry and nobody really wanted to heat the hospital food. The hospital food was far from great though I imagined it to be worse. Nonetheless, when I awoke to my family discussing DiPasquale’s for dinner before my parent would head home for the evening (Crissy was now staying in the room with me), I definitely perked up a little bit. Their porketta sandwich (Italian roast pork) with broccoli rabe, onions, and sharp provolone cheese is to die for. It is literally one of the best sandwiches in the region. Amazing how certain foods have a way of cheering people. I’m no better than anyone else in that regard, waist line be damned, I eat with my emotions from time-to-time too. Little did any of us know, we would be going back to Hopkins for months and a pit stop at DiPasquale’s would become an expected part of the Hopkins trip routine.

 

Wednesday the 22nd began with more blood work and a series of EMG and ENG tests performed in the outpatient neurology clinic on the Hopkins campus. I was officially a lab rat getting poked and prodded with needles all day long at that point. Awesome. If you’ve ever had an EMG or ENG nerve conduction study performed on you, then you know what this is like. If you haven’t, then the video below and y description might help. The doctors essentially insert a needle into various nerve and pressure points and test for interferences in the nerve transmission. I tried not to ask questions or even look at the machine because I was fairly doped up on pain meds and I really didn’t want to worry my mind should they tell me something was wrong. Besides, Dr. Sethi just ran these tests and I already knew I have neuropathy issues in my left arm and surprisingly my left leg too. Maybe the latter was a precursor to the back issues I developed from being bedridden so frequently throughout the year.

 

During the transport trips back and forth from in-patient to outpatient, I learned more about the bowels of the hospital and the hopes and dreams the transport department union rep who was wheeling me around. She was an aspiring crab business owner who rattled off crab factoids like Bubba in Forrest Gump could do with shrimp.

It was a welcome distraction from my discomfort and it aided my general lack of appetite. I also realized as a union rep, she essentially knew everyone in the hospital’s less glamorous positions. Being that we were in Baltimore, I look back at the culture and I think of David Simon’s and HBO’s The Wire. The tight knit community has hardworking people trying to make it and rise above circumstance is something I admire. By the way, if you never watched The Wire, I recommend you stream it or get some DVDs.

 

As Wednesday afternoon rolled around and I was back, the neurologists and pain management folks came to visit us all together. Most of the various test results were in and evaluated by radiology and rheumatology. Dr. Maragakis and Dr. Doshi had a pretty strong feeling they reached a diagnosis. I officially have CRPS. Dr Sethi’s intuition was correct. Dr. Maragakis and Dr. Doshi continued to talk to us and educate us about CRPS and the disease that it is. I remember thinking to myself and trying to take the diagnosis in. Now I know what it is. Well, it’s not cancer or anything like that so you have that working in your favor. Don’t tell Crissy, mom, or dad, but I’m scared as hell right now. I just went through all that to confirm my community neurologist’s intuition, and I have a disease that is super rare that even Johns Hopkins doesn’t have a ton of experience with? Great job, Jason, your body picked a real winner diagnosis. At least it was early and treatable. The early diagnosis, 7 weeks from initial symptoms, was a record at Hopkins. They told me they were thrilled to have me a a patient because they could study me as a case for what might be possible with this disease should a person be diagnosed so early. I guess that was supposed to make me feel better. The doctors prescribed gabapentin, a neuropathic pain medication. Gabapentin is a very heavy drug that slows brain synapses. The initial dose level was 300 mg twice a day. I was also prescribed nortriptyline. Nortiptyline is also a neuropathic pain medication with a bit of an anti-anxiety feature as well. I was started out on a 25mg once a day at night dosage. The tramadol was to be used for only a few more days. One key thing to point out, the team also made it sound as if I would get better very quickly and maybe my family and I could put this behind ourselves in a matter of 2-3 months given my age and general fitness level. That was exciting. What was far less exciting, the last diagnostic test to final confirm CRPS and also help reduce symptoms.

 

The diagnostic test with therapeutic benefit is called a stellate ganglion nerve block. Yes, a mouthful. In layman’s terms, a stellate ganglion nerve block is a procedure in which a pain management specialist injects medicine into the ganglion nerve. The nerve runs through the cervical and thoracic part of the spine with many functions in the wrist and hand. The optimal injection site to reach the stellate ganglion nerve is located between the Carotid artery and the Jugular vein. Did I tell you I keep hitting the lottery with this CRPS stuff or what? An injection into a tiny nerve running between the Carotid and Jugular? That’s the best you have? Really? Wtf?! I didn’t want anyone going near those areas with a needle. What if you miss and I bleed out? Thankfully, my dad was there to ask all of the questions on my behalf. I was too drugged up and also too fearful to coherently ask the questions that mattered. My father gladly took on that role and asked about the safety in the procedure, how they do it, and any other possible options. Surprisingly, the procedure is relatively safe though it comes with a big legal disclaimer. The procedure is performed under fluoroscopy – a live imaging machine that enables the doctors to make sure they’re hitting the proper injection site with a needle. If you’ve ever had an epidural, you likely had it performed under fluoroscopy. The benefit of the procedure is such that most patients feel immediate relief. I didn’t really have many other options so I had to go for it. The only question was could Dr. Doshi fit me in on Thursday, Friday, or perhaps discharge me and have me return the following week. I wouldn’t find out until the next morning. Again, the way it was billed to us was that if successful, this procedure would drastically alter my current state and help me get back on my feet. Dr. Doshi even felt the procedure might put the CRPS symptoms completely away since we caught it so early.

 

Among the details that determine the efficacy of a stellate ganglion nerve block include immediate pain reduction, a droopy eye, a hoarse voice, and increase in mobility. If any two post-procedure effects happened, then the procedure would be determined as a success and it would help to confirm CRPS.

 

Early Thursday morning, I was woken up by my nurse to let me know Dr. Doshi was able to squeeze me into the pain management clinic schedule and perform the procedure around 10:00am. In order to do be ready and waiting, I had to leave the hospital room by 7:30am and have transport bring me over. The pain management clinic where they perform the nerve blocks was very busy. There was a guy moaning and groaning constantly about his back pain. He and about 4 other people were ahead of me that morning. Dr. Doshi administered some light anesthesia around 10:00am to help me take the edge off.

 

My turn in the operating room for the procedure came circa 11:00am. I was wheeled into the room on a stretcher and the room was extremely overwhelming. There was an army of people in the room ranging from doctors to nurses to technicians. Everybody was doing something to help get me set up for the injection. I think I counted 8 people in the room and some big heavy equipment that turned out to be the fluoroscopy machine. This hospital bill is going to be fun. I hope insurance covers it all.

 

Dr. Doshi and her team talked me through every step of the process. It was of course fully recorded and professional yet still immensely overwhelming. The team put a bunch of strange thermometer things on my fingers measuring temperature in both extremities for comparison’s sake. My left fingers were running cooler than my right fingers which confused me given I felt so much burning pain in my left hand. I didn’t bother to ask Dr. Doshi. I simply tried to remain calm throughout all of the things they were doing. I could hear them say things like “there is the Carotid artery” or “that’s the Jugular vein.” For obvious reasons, it makes you a bit uncomfortable the first time. I recall praying the procedure would go as planned while also wishing Dr. Doshi’s team would stop talking. I wished my wife could be at my side. I was genuinely scared.

When it was eventually time for the injection, it isn’t like what I anticipated. I thought it might be a gentle pinch ala a flu injection. It was actually much more forceful. Despite the anesthesia, I could feel the needle create pressure on my throat. I did my best to remain calm though I was definitely uncomfortable. The process of injecting the medicine is monitored via fluoroscopy and I could hear Dr. Doshi telling her team the medicine was spreading “beautifully.” She was happy with their performance and they slowly began to inject more medicine. Apparently, the proper technique for highest efficacy is a slow release of the medication versus a quick one. After about 3 more minutes, they were wheeling me into the recovery area.

Here is a recording of someone undergoing a stellate ganglion nerve block. 

A few minutes into recovery and I noticed the pain that was at about an 8 on a scale to 10 was reduced to a 3 or 4. The medicine was working. Another 5 minutes or so and they were telling me my left eye was droopy. Great, I’ve checked 2 boxes. My voice wasn’t hoarse at all yet. Most importantly, I was able to begin moving my wrist and fingers. I was able to wiggle fingers and slightly bend at the wrist. It was amazing! I could move my fingers without causing excessive amounts of pain for the first time all year. I looked around for my phone, but I left it back in my room with my family. After an hour passed, I was finally being brought back to the hospital room with the hopes I could be released later on. Prior to leaving the pain management clinic, I scheduled a follow up injection. Dr. Doshi made sure to tell me she felt like I might set records for recovering from CRPS and he had a very positive outlook for me.

 

At around 4:00pm I was given my discharge. I was on my home with a diagnosis, a treatment plan, and a positive outlook. My instructions were to continue taking all of the prescribed medication and to begin occupational therapy after a few days of rest at home. I was thrilled and I thought perhaps my battle CRPS might be short lived.

Beat CRPS!

 

Jason

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