August 2022

Unlike July, which had weeks of no tests, appointments, or forward progress, August quickly became busy. On August 4^th, I went in for a special type of MRI, called an MRI enterography. This type of MRI specifically looks at the intestines and can detect obstructions or inflammation. We weren’t sure if it would show anything, but my GI doctor identified it as a test that could potentially provide an answer for my worsening pain with eating. On the day of the test, once we arrived at the imaging center, I had to begin ingesting the contrast to create a clear picture of my intestines. I did this by pushing the contrast 60 ml at a time through a syringe into my feeding tube. I had to take in over a liter, something that caused severe pain and nausea, even though it was going directly into my stomach. After finishing the contrast, I was put into the MRI machine and given some IV medication (through my PICC line) to slow down my digestion. During the test, I had to follow certain breathing instructions, but besides that, the test itself was easy. While I desperately wanted answers for my pain, I was happy to hear that the results of this test were normal because it meant that my SMAS surgery was still successful in relieving the intestinal obstruction. However, since the test showed no abnormalities, I was still stuck in the same place with no answers.

The next step was emailing my GI doctor to come up with a plan. He decided that the next step would be to do an endoscopy where he would look at my stomach and inject pyloric Botox. The Botox would relax a muscle in my stomach called the pylorus, allowing my stomach to empty more easily, a treatment that had a possibility to help my severe nausea. I also hoped that the endoscopy would provide answers for a new type of pain that I was experiencing. The new pain was a severe burning pain from my sternum to my belly button that woke me up from my sleep nightly and had resulted in an ER visit. Luckily, when my doctor looked at my stomach lining, the pain could be easily explained. I had severe gastritis, or inflammation of my stomach, that had begun to bleed. With a few new medications, this issue could be easily resolved, and my burning attacks have since gone away. I am so thankful to have at least one answer, and one symptom that could be fixed so quickly.

Two days after my endoscopy, it was time for my second celiac plexus block. I had one done in February that confirmed my MALS diagnosis, and this second block would allow us to know if MALS is still contributing to my pain. During this procedure, I was sedated and laying on my stomach, and an interventional radiologist inserted a long needle into my back to inject numbing medication into my celiac ganglion, the bundle of nerves impacted by MALS. As soon as I woke up from the procedure, I could tell that the block had worked! My normal, constant stomach pain was gone, and I could even press on my stomach! I went straight from the hospital to get food and was able to eat almost an entire full meal for the first time since March. It was amazing to remember what it was like to not have pain. However, the block wore off around the time that I got back home and the pain I felt then was severe. This made me feel like I would never get better, but I had to remind myself that the block is a glimpse into what my life will be like once I beat MALS.

The Monday following the block, my mom and I got in the car and started our trip to Baltimore, Maryland, where we would be consulting with a well-known vascular surgeon to find out if I have any more abdominal compressions. When you have one compression, you tend to have many. After an 8-hour drive, we were exhausted and went straight to our hotel room. The next morning, we decided to go to the national aquarium to have a distraction before the big appointment. I had the best time with my mom and getting to see many cool sea creatures (my favorite was the sea horses). As we went into the appointment, my heart was about to burst as I waited to hear about what I might have going on. The doctor said that he could see some compression of my left renal vein and left iliac vein but that he wanted an ultrasound, urinalysis, and blood work to confirm before he made a final diagnosis. The next morning, I came back to his office and had a special ultrasound performed. I had a horrible feeling while the tech did the ultrasound as I could see unusual looking images on her screen, but I just hoped that I was just interpreting the images incorrectly. However, once I got the results of my urinalysis and blood work, that bad feeling worsened. My results lined up with a compression of the left renal vein called Nutcracker Syndrome. That afternoon during our drive home, we got a call from the doctor and he confirmed compression and stretching of my left renal vein and left iliac vein. I immediately felt a lump form in my throat. It was horrible to hear that I had two more severe compressions that would need surgical intervention. And while it in no way surprised me to get these diagnoses because of my symptoms, it still hurt to hear. At least I finally found answers on this trip and got to spend so much time being with my mom.

Exactly one week after our appointment in Baltimore, we had a virtual appointment with another vascular surgeon. He was so kind and explained everything very well. He confirmed that I not only had the two compressions that were found in Baltimore (Nutcracker Syndrome and May-Thurner Syndrome), but he told us that they were severe and that my MALS possibly needed to be addressed again. Additionally, he saw that I also have the fifth and final abdominal vascular compression called Pelvic Congestion Syndrome, which is being caused by my other new compressions. Not only was my left renal vein severely compressed, I have a rare form of it where my duodenum is the structure that is causing the compression. Because of my type of Nutcracker, he explained that I would need a surgery called an auto transplant. In this surgery, my left kidney will be moved to the right side of my pelvis, leaving me with two kidneys on one side. This surgery will likely be performed by a transplant surgeon and will be the first of three surgeries. The next surgery would be a MALS revision if I still have symptoms, and then lastly, I will get a stent placed in my left iliac vein to treat my May-Thurner Syndrome. After fixing NCS and MTS, the Pelvic Congestion Syndrome would resolve itself. Obviously, this was a lot to take in. I was heartbroken to hear that I would need three very major surgeries over the next couple of months, but I was also the slightest bit hopeful after hearing that my symptoms align with my diagnoses and that they can all be treated. It won’t be an easy journey by any means but is one that I am ready to take on.