UPDATE: We’ll be posting posting status updates (i.e. visiting hours at the hospital, meal train info, etc.) at https://www.caringbridge.org/visit/rachaeljohns.
If you’ve navigated here, you’re probably curious about what’s going on with my health as briefly outlined in my recent FB post (or, potentially, you’re a stranger from the internet who stumbled upon my blog… either way, welcome). Feel free to read on for an in-depth look at what has unfolded over the past couple of months.
My surgery is currently scheduled for March 29th at Swedish First Hill. I’ll be providing updates as I’m able. Texts, phone calls, emails, etc. are totally welcome but please be aware that the next week is going to be very busy; I’ll do my best to respond as soon as possible but if you don’t hear back, please know it’s nothing personal!
Please keep in mind that I wrote this post primarily for myself so if it rambles or doesn’t make perfect sense, remember that I wrote it for my own sanity and processing .
“Feel this… do you have that?”
In mid-January while in Southern California for the Disneyland half marathon, I felt a protrusion in my left abdomen. I turned to Aaron and said “feel this… do you have that?” The answer was “no” but I didn’t think much of it (and WebMD did not scare me with its hyperbolic internet diagnoses). I was, however, worried that I had a stress fracture in my foot following the race so I went to the doctor and decided to have her look at my abdomen while she was at it. No stress fracture but my doc ordered an ultrasound to check out my spleen which she thought could be enlarged. The ultrasound revealed a mass which she suspected was a cyst so scheduled me for a follow-up CT scan.
Learning that a same-day follow up is rarely good news…
I completed the abdominal CT scan the morning of January 26th. It was pretty straightforward (though if you’ve never had a scan done with contrast, let me tell you I wouldn’t exactly describe the sensation as pleasant) and I was back at the office around 11:30AM. Around 2PM, I got a call from my doctor. Spoiler alert, you never want to get a call from your doctor the same day as your test because it’s rarely good news. My doctor told me I had a large mass in my abdomen and used words like “concerned” and “I’m sorry.” She finished by saying she had made an appointment for me to see an oncologist the next afternoon (she made it clear I should reschedule any conflicting appointments… to my friends at NOLA hair salon, this is why I canceled my haircut at the last minute.)
I’d like to say I handled everything with grace and stoicism… but that would be a big, fat lie. My awesome teammates hung in there with me while I cried like a baby at my desk, called my dad and cried on the phone, called my mom and cried on the phone, called my boyfriend and… you get it.
My mom went with me to the first appointment with the oncologist. The office was in the same building as my primary care physician but everyone (the front desk staff, the nurses, etc.) were much nicer than normal. They were the kind of friendly I imagine you’d be to someone you know is battling cancer. For me who, until less than 24 hours prior, had thought the diagnosis was “minor cyst”, this was a little disconcerting.
If you have to pick someone to tell you it’s likely you have cancer, I’d highly recommend my oncologist at the Polyclinic. She explained what we knew (not a lot) in detail: I had a large — approximately 10 cm — tumor in my abdomen sitting near my pancreas at the level of my kidneys. The tumor was vascular (taking in a lot of blood) and had central necrosis (the inside of the tumor was dead cells). She let me know that the next step was a needle biopsy to confirm the type of tumor and develop a treatment plan based on the results.
Interestingly, my father had a large abdominal tumor at exactly the same age as me. He was told it was likely an aggressive form of cancer but it turned out to be something quite different. More on that soon.
The oncologist was able to schedule the needle biopsy for the afternoon of Monday, January 30th (because it was such short notice, this was the only available time of day). Since I was going under sedation, I wasn’t allowed to eat or drink anything 8 hours prior to the procedure which meant I woke up at 5AM to sneak in some breakfast before the cut-off. I’ll remind everyone at this point that I work in the land of free meals and snacks constantly within arm’s reach, so this was a rough day for me on many levels. In retrospect it probably would have been better to take a sick day.
The procedure involved sliding in and out of the CT scanner to guide a very large needle into the tumor to collect a tiny specimen for evaluation. The nurse explained that I would be sedated and that they would be restraining my hands above my head during the procedure (apparently it’s common for patients to forget what’s happening and start flopping their arms around). They also put a washcloth over my eyes to “keep me from getting dizzy.” Translation: “shield your eyes from seeing the giant needle we’re about to jam into your abdomen”. The meds kicked in quickly so participating in what sounded like a 50 Shades of Grey/Grey’s Anatomy crossover episode didn’t phase me at all.
Near the end of the procedure I started feeling a burning pain in my abdomen that got worse once I was in recovery. It was bad enough that I ended up having to be briefly admitted to the hospital so they could give me some meds to get it under control. Afterwards, I got to ride a wheelchair down to the hospital entrance where my boyfriend was waiting with flowers so maybe the whole episode was more of a rom-com after all.
Waiting for answers…
There’s a study by Harvard researchers that examined the stress hormone (cortisol) levels in women waiting for breast cancer biopsy results. The study concluded that women who were waiting for their diagnosis had stress levels very similar to those of women who were told they had cancer. I don’t doubt this for a second. The days I spent waiting for the results of the needle biopsy were one of the worst weeks of my life.
The nurse at the hospital following the biopsy informed me I should have the results back in a few days and that sometimes they got them within about 48 hours. I lasted two days before emailing my oncologist’s office for an update. The nurse in her office responded letting me know, very nicely, that the results weren’t back and that we would discuss the results at my follow-up appointment on Friday.
I spent four days pouring over my lab results and researching the abnormalities via Dr. Google and the results were not pretty. I was basically crawling the walls by the time my appointment finally arrived. My mom and Aaron came with me. It’s funny to see how different people react to stress: My mom kept talking about random stuff. Aaron was very quiet. I felt like I was under water; everything was moving in slow motion and it took much longer than usual for me to process what anyone was saying.
When my doctor came in she, blessedly, cut right to the chase: the biopsy indicated that the tumor was not lymphoma (what she had suspected) but a paraganglioma which is a rare, neuroendocrine tumor.
The pathologist who evaluated my biopsy results made the diagnosis locally but also sent my results to a specialist at the University of Wisconsin to confirm. My oncologist had only just received the confirmation back about an hour before my appointment. The recommended treatment was surgery to remove the tumor, the good news being that once removed it is considered “cured” and no further treatment is required.
I needed several more tests and scans prior to moving forward with a surgeon which included:
- PET CT: This nuclear medicine scan involves an injection of radioactive glucose, followed by a scan. Cancer cells take in the glucose and light up on the scan. The scan indicates if the tumor has metastasized anywhere else in the body.
- 24 Hour Urine Test: Evaluating hormone levels in the urine over a 24 hour period indicates whether the tumor is “functioning” i.e. whether the tumor is pumping additional catecholamines (stress hormones) into the blood
Interestingly enough, my dad was also diagnosed with a paraganglioma at the same age as me (29), shortly after I was born. Because these tumors are quire rare (2 in a million) the doctor suspected a genetic component and genetic mapping/testing was also suggested.
They drew blood for some additional blood tests (including testing for the hormones they would be looking for in the urine test) and sent me on my way with a large, orange medical jug to collect my pee for 24 hours beginning Sunday morning. On Monday, I would return it to the lab for analysis. Thanks to Aaron for being such a champ through what topped the list as “most inconvenient lab test ever”.
Positron emission tomography–computed tomography
I left the oncologist with the promise that they would follow up to schedule the PET-CT scan. Because the scan costs about $5K, their policy was not to order the scan until they received pre-approval from the insurance company which can take a few days. My mom, upon hearing this, wondered aloud how long it would be before an insurance company was sued for practicing medicine without a license (since they are making decisions about what tests are medically necessary for their customers), but I digress.
A few days later their office received the insurance approval and the scan was scheduled for the following Thursday, February 9th. It was, again, in the afternoon, meaning I spent another day (at home this time) becoming a cranky mess after fasting leading up to the test, this time to make sure my glucose levels were low enough for the test to work.
At Seattle Radiology, the tech prepared me for the scan and went to draw my blood and insert an IV. Seeing the bruises on my left arm, he says “Oh, looks like you just had a blood draw here, I’ll use the other arm so we’re not sticking that vein again” before turning to my right arm. Upon seeing my black-and-blue right arm from other previous blood draws: “Oh, oops, well I guess it doesn’t really matter.”
With the IV in, the preparation for the scan involved laying down in a dark, warm, quiet room (I learned later that inflammation in the body can show a false positive on the scan and resting prior to the test helps counteract this). The tech came back in with a metal box which he opened revealing a needle, also encased in metal, which he proceeded to inject into the IV. Another 30 min or so, laying down in the dark room so my body could absorb the glucose, then off to the scan.
Overall a pretty boring test though, since Aaron and I were on a fairly strict ketogenic diet at the time, I did wonder, laying in the scanner, if the injection of glucose had just kicked me out of ketosis.
Becoming one of the 0.2%
The follow-up oncology appointment went over the test results. The PET CT scans were pretty fascinating and slightly terrifying: everything was black and gray with vague shadows of red then, suddenly, the scan reached the tumor which lit up like a bright yellow/white light bulb in the middle of my body. It was also huge.
The blood and urine analyses showed that the tumor was indeed functional, meaning it was secreting large amounts of hormones into the blood (the tests measured normetanephrine — normal range is 0.00 – 0.89 nmol/L; my result was 17.25 nmol/L). These hormones, along with fun side effects like headache and flushing, cause hypertension (high blood pressure). For background, I have suffered from hypertension since I was about 13 years old. The doctors did several full work-ups and were totally stumped as to what was causing my blood pressure to be elevated. As it turns out, I am one of the 0.2% of people whose high blood pressure is caused by a paraganglioma or pheochromocytoma. I probably would have preferred to win the lottery but I guess I at least get something special and rare…
Next steps were new medication and an initial consult with a surgeon. The consult was scheduled for after I returned from a (MUCH needed) trip to Mexico to celebrate my BFF’s dirty thirty birthday on a Carnival Cruise.
“Oh we don’t carry that… That medication is very expensive”
Because the latest round of tests showed that my high blood pressure was being caused by elevated hormones levels, I was prescribed a new medication called phenoxybenzamine (fi-nok-see-ben–zuh-meen…try saying that 3 times fast) to better treat my blood pressure.
My regular pharmacy called me to let me know that the medication was on backorder and wouldn’t be available until Monday afternoon. I told them that that, unfortunately, wouldn’t work for me since I would be in Southern California beginning Monday. Thus began almost an hour making ~15 phone calls to local pharmacies trying to find the medication (the closest my pharmacy was able to suggest was in Bremerton which was an approximately 4 hour round trip drive from my house).
The answers from the pharmacies were all various iterations of “sorry we don’t have it” though my personal favorite was a woman who kept repeating that the medication was “very expensive” and that they didn’t carry it because it was “VERY expensive!” Finally, after speaking with a very helpful pharmacy employee at the Walgreens in Ballard, I learned that the Walgreens in Shoreline on Aurora (… the one on 175th St because there is more than one Walgreens on Aurora in Shoreline…) had what I was looking for. I got the prescription transferred and was able to pick it up the next day.
My jaw hit the floor when I saw the price tag: the “it’s very expensive” pharmacist was actually right on the money (so to speak) since the receipt indicated my insurance had saved me a total of $15,344 for the 120 pills I was prescribed.
I headed off on the super fun vacation with medication in hand. The side effects were, unfortunately, rather unpleasant and included: blurred vision, chronic fatigue, racing heartbeat, drowsiness/dizziness/grogginess, and (oddly) nasal congestion. I was both glad to not be having to function at work and bummed to feel so out of it while on vacation.
Consulting a Surgeon
In retrospect, I went in for my first appointment with Seattle Surgical Specialists woefully unprepared. For some reason, even despite seeing the size of the tumor on the CT and PET CT scans, I glossed over the step where I had to have major surgery and skipped right to the part where I would be cured after its removal. I went to the appointment alone (my mom called in on speakerphone because she was traveling) without preparing any questions for the doctor.
The surgeon came in and introduce himself and examined my abdomen. He said it was a “good catch” to have felt it myself.
He went on to open my CT scan and explain that the tumor was wrapped around the left renal vein which meant there was the possibility that it would need to be removed. He continued, saying it was also involved with my small bowel which meant it could need to be resected (meaning they would cut out the part connected to the tumor and reconnected the two ends). Finally (and most scarily) he explained that the tumor was touching the aorta, meaning that potentially it could be difficult for them to get clean margins (difficult to get all of the tumor) increasing the possibility that it could grow back.
He talked about how they couldn’t tell from the scans if the tumor is involved with these organs (i.e. invading them) or just touching them or pushing them out of the way. He talked about potentially being able to peel the tumor away from some organs and veins to preserve them. Unfortunately there wasn’t any way to know this until they are in the surgery.
The doctor let me know it would be an open surgery (so my hopes of a quick, laparoscopic procedure went out the window) and that I should expect to be in the hospital for about a week, with another 4 to 6 weeks recovering at home.
At this point I was feeling pretty shocked but managed to ask something about adverse side effects and things that could go wrong. Mostly they were concerned about infection, bleeding, and pain management. There’s also (apparently) a 5% chance of leakage following a bowel resection which can cause an abdominal infection.
My mom’s kid-spidey-sense must have been tingling because she asked on the phone if I was doing OK. The short answer was “no” but I was focused on breathing, since I was getting that painful lump in your throat that makes it hard to speak, and not bursting into tears in front of my very nice doctor.
The next step was for my case to go before the Swedish Hospital tumor board which basically means all of the doctors involved in the case get together to talk and agree on a treatment plan. The tumor board was taking place the following Tuesday (this visit was on a Monday) so the follow-up visit was scheduled for the following Thursday, March 9th.
I should mention that throughout this process I have been using a great resource provided by my employer called Advance Medical, which is a second opinion medical service. They work to gather your medical records and submit them to experts in the field for review and recommendations. I was still waiting for them to get back to me and Aaron’s aunt Teresa (an MD who has been an awesome source of advice throughout this process) urged me to get an in-person second opinion.
I brought this up the at my next oncology appointment the following day and my doctor first emphasized the importance of not delaying treatment and getting surgery as soon as possible, then provided a referral to the Seattle Cancer Care Alliance for a second opinion consultation. Another big thank-you to Teresa for talking me through some questions to ask the surgeon to help me make a final decision.
SCCA was able to get me scheduled the next week on Tuesday, prior to my follow up at Swedish Surgical Specialists. The patient coordinator who scheduled me mentioned that the surgeon was one of her favorites which was encouraging.
My mom came with me for this appointment and I was glad for her company since it was a little overwhelming in an unexpected way: SCCA is a massive place. I was used to being one of only a few people waiting to see the doctor. By contrast, there were probably 70 people waiting to see doctors in this waiting area.
We were called back and checked in then a surgical resident came in for an initial conversation. He examined me, asked some questions about my symptoms and medical history, then a bit later the lead surgeon came in and spoke with us.
Most of what he said was similar to what my first surgeon said: he thought it was likely that they would have to remove a section of the small bowel, identified areas where there were not clear margins between the tumor and the aorta, and mentioned the renal vein, though he seemed more confident that the vein was merely draped over the tumor, rather than involved with it.
This time I was armed with more specific questions and felt generally more confident in the conversation:
- Who would be assisting with the surgery? (A 4th year resident)
- Who was the anesthesiologist? (A resident or NP with an attending supervising)
- Do they have experience managing blood pressure during surgery? (Yes, the attending)
- Will a vein specialist be involved? (No but available if needed)
- How many of these have you performed? (A few but we look at this more like a general, large abdominal tumor which is my focus)
He let me know that a vascular surgeon was always nearby but that he felt confident in his ability to preserve the renal vein. The SCCA surgeon was also a bit more relaxed about the timeline, pointing out that I’ve likely had the tumor growing for years so a few weeks here or there likely wouldn’t make much of a difference.
Speaking with Teresa and my mom (who has been in touch with her various MD friends) it was pretty clear there were positives and negatives to both choices (Swedish and SCCA/University of Washington) and I was pretty terrified that I would make the wrong choice.
I attended my follow up appointment with my surgeon from Swedish prepared with questions to fill in the gaps of information I had missed during my previous visit. My surgeon would be assisted by a vascular surgeon to makes sure they did everything possible to preserve the veins. He let me know that the anesthesiologist who would be working on me was part of a group who specialized in the kind of BP stabilization I would need. He also said I would need to be in the ICU for a few days following the surgery so my blood pressure could be closely monitored.
(Because the tumor is secreting so many hormones, stabilizing the blood pressure during the surgery is very important. Handling the tumor while removing it can cause additional hormones to be dumped into the bloodstream which can cause the BP to spike up and, once it’s removed and the hormones are no longer being secreted, the BP can drop suddenly.)
After the conversation I felt confident in my choice to move forward with scheduling the surgery with Swedish which was a huge relief. I was worried I would be second-guessing my choice if I was unsure.
The surgeon was going to be traveling the next week and my mom pushed him to schedule my surgery in before he left (we were at the appointment on Thursday and the surgery would have been the following Tuesday, March 14th) because I really wanted to just get it done. A few hours later I got a call back letting me know that there wasn’t an OR available on the 14th so they ended up pushing the date back to March 29th.
This ended up working out well because it gave me some time to let my manager determine who would take over my work while I was gone. I also submitted the paperwork to take short term disability and they needed time to process everything.
Finally, and probably most importantly, I’m having an additional nuclear medicine test before the surgery. This test involves an injection of radioactive iodine (rather than glucose) and several scans to see if the tumor takes in the iodine. If it does, this will be a useful tool after the tumor is removed to make sure it isn’t growing back and to identify it early if it is.
Before I left the office I had to sign a release giving the surgeon permission to operate on me. Because they don’t know the extent of the tumor’s involvement before they begin operating, I had to make a decision about whether they should prioritize removing all of the tumor or preserving areas where the tumor is involved. I ended up giving them permission to resect my bowl, remove my kidney, and replace a portion of my aorta with a prosthetic if needed to get the entire tumor. If they tried to preserve these areas and weren’t able to get all of the tumor, there is a risk it could grow back or metastasize to another area of the body. For me, who likes to be in control and know what’s going on, though, the idea that I’ll go under not knowing exactly what will happen is pretty scary.
Overall it’s been a long, stressful couple of months. My mom pointed out that I was looking tired… I asked her if it looked like the kind of tired that comes from being worn down by extreme stress for the past 8 weeks. Apparently, yes, that’s exactly what it looked like.
That aside, I’ve continuously felt incredibly grateful for my friends and family, as well as my job and the insurance and flexibility it provides.
I’ll provide updates as they come and will do my best to respond to texts/email/FB messages. It is going to be a busy week so please bear with me and know that if I don’t respond, it’s not personal!