Portal Vein Stenosis: aka The Stent Clot of 2019

If there’s one thing I’ve learned in my 31 years, it’s that we are not given tomorrow. I know we hear that and think it doesn’t apply to us. We close our eyes at night not even slightly doubting that we will ever wake up. We know God has numbered our days, but we assume that the number most definitely won’t run out any time soon.

As many of you know, I grew up with liver disease. I was pretty stable, and a transplant – or even death for that matter – seemed like it was meant for other people. Definitely not me.

Until it was.

Until it was meant for me.

A routine scan in May, 2010 revealed a random tumor, and just like that, I needed a transplant.

One day, my life seemingly hung in the balance when it was everything was normal just the day before. Two days before, I was having fun on vacation in Disney World with my family.  Little did we know.

I completed a dozen tests in a matter of days, and within two weeks, I was listed for transplant.

There were procedures. There was chemo. There was waiting. Yet somehow, my anxious heart found peace.

Within three months, I received a transplant. A teenage boy died so I could live. It was a surreal feeling that has never been lost on me. I still can’t fully grasp it. My parents’ daughter, my husband’s wife, my sister’s sister, my children’s mother… I’m alive because another parents’ son and siblings’ brother is forever gone. 

I fought through an 8 hour surgery, the ICU, remembering how to walk, breathe, talk, understand. I endured indescribable pain and somehow produced strength that I didn’t even know I had. My need to love drove my need to live, and I took life one day at a time. My family and friends held me up when I was too weak to stand. They built a hedge around me with their prayers, presence, and support, and I’m forever indebted to them.

As I healed, my body turned into an unrecognizable skeleton. Pounds fell off of me like it was nothing. My incision, 50 staples in three directions, reopened and didn’t completely close for eight weeks. Home care nurses and my mom worked hard to keep it clean and dressed.

I slowly improved. I regained a lot of my life, yet I wouldn’t feel completely normal for at least a year.

Within months of my transplant, my immunocompromised body caught Epstein Barr virus, and I struggled once again. Not too long after, we realized my spleen was absorbing all of my platelets and white blood cells. After a few months of painful procedures to rule out cancer, we scheduled a splenectomy.

My spleen was bigger than a football (huge) and once gone, my blood counts improved. Again, I healed for a week in the hospital. This time, I received all of my nutrients through a feeding tube. My transplant incision had been mostly re-opened, and I was thankful that it healed quicker this time.

While removing my spleen, my surgeon noticed my portal vein had been compromised. A month after the surgery, I had a stent put in to revive the portal vein. It was a simple procedure, and after one night in the hospital, I was home again and doing well. No pain.

Over the next two years, I had sharp pains all over my abdomen, sometimes mild, and other times, enough to drive me to go to the ER. We finally realized that I had a lot of surgical adhesions – nerves trapped in scar tissue.

The only fix for this would be another surgeon and a reconstructive surgery. My transplant surgeon sent me to a plastic surgeon who performed a reverse abdominoplasty with special care to remove any adhesions. The risk of the surgery would be more adhesions, but thankfully I’ve been free of adhesion pain ever since.

That was August, 2013.

The next several years were very uneventful. I had my tonsils removed, I had (and lost) ear tubes. I got allergy shots.  There were several hospitalizations for viruses with my weak immune system, several rounds of antibiotics for many different bacterial infections… All of this was my new normal. But nothing crazy or super shocking until April, 2019.

I woke up one morning a few weeks ago – one day after Easter and two days before Scott and I were to go to NYC on a fun getaway – sure I had appendicitis. I was in too much pain to drive. Scott was already at work, so I called my mom and asked if she could drive me to the ER. She came quickly, and it didn’t take long to see a compassionate doctor in the ER. He evaluated my symptoms and was in agreement that I definitely had a case of appendicitis. He ordered fluids and m0rphine, and I was a lot more comfortable. He suggested that we run a CT scan to evaluate exactly what was going on. Normally I try to avoid CT scans due to unnecessary radiation, but I was in agreement. Awhile after the scan, he came back to my room and said, “Well, your appendix is fine.” My mom and I looked at each other, confused. Then the word “but” left the doctor’s mouth, and my heart immediately sank. “There appears to be some sort of blood clot in your liver.” Time stopped. He said we needed to get me to the main campus of the hospital ASAP where my transplant team could handle whatever was going on.

An ambulance came and drove me to the main hospital. My mom followed behind. Another CT scan showed that my portal vein stent was compromised, so my team booked an angiogram for the next day, Tuesday.

With a MAC (anesthesia), the interventional radiologist came in through my groin and went up my blood vessels to the liver. The portal vein was indeed closing – it was almost completely clogged – and other blood vessels nearby appeared to be a little thin.

My team wanted that same interventional radiologist to repair the issues, and he didn’t have an opening until that Friday. They kept me in the hospital and monitored my liver via almost-daily ultrasounds. I kept busy doing puzzles on my iPad, reading, and coloring in the coloring book my mom got me in the gift shop.

Friday came, and surgery was scheduled. Scott came up to support me. I was prepped and ready to go, and the doctor came in. First, he said he didn’t know if he could do the procedure if I still had contrast in me from Tuesday’s procedure. He ordered an X-Ray and thankfully, the contrast was gone so he said he could proceed.

Next, it’s routine to give consent for a procedure.  You have to hear the risks and then sign something. The doctor went over the risks of the procedure – bleeding, etc… all the normal risks. I asked if he had done this procedure before, and he said it was a very rare problem to have, so no, he had not done this exact procedure. He was hand-picked by my transplant team though, so I was okay with that. Nervous, but okay. Then he started detailing some negative outcomes from other similar procedures.

I quickly looked at Scott, and we both had fear in our eyes. Then the doctor said that I didn’t have to have the procedure – I could wait until the stent completely closed off and come in on an emergency basis. He said whoever was on call would perform the procedure.

That sounded much scarier to me. I think he could sense our uneasiness, so he said he would give us a minute to discuss. As soon as he left, Scott immediately started praying aloud. Transplant had let us know that this was a big problem, and I couldn’t imagine letting it go until it was a life or death emergency with 0 blood flow to my precious, transplanted liver. Scott agreed. We felt that God was leading us to proceed, so we told the doctor that we wanted to go through with it.

They wheeled my bed into the OR, and I kissed Scott and told him I loved him. I thought back to how easy Tuesday’s angiogram was, and I knew I’d be seeing him soon.

Regardless, I was so nervous. I asked the anesthesiologists give me something to calm me down. They gave me Versed while, unknown to me, Scott was calling my parents to update them on the severity of the procedure. They headed up to Cleveland while I was asleep on Propofol.

I woke up in the PACU. I was in pain. I had to pee and I was so thirsty. I wanted to see my husband. The nurse told me I had some bleeding, and that’s why they were monitoring me closely rather than sending me back to the floor. I was supposed to lay on my right side to put pressure on the bleed. I didn’t think much of it. They were still giving me m0rphine for pain, but somehow it felt a little worse. I begged the nurse for ice to chew or water with the swab. She kept reminding me that I was NPO because of the bleeding, in case they needed to put me under again. I didn’t care- I felt like I hadn’t had a sip of water in a week. Before long, Scott appeared with my parents. I didn’t even think twice about seeing my parents even though they weren’t there before the surgery. My husband snuck me more ice, and for that I was grateful. I was so happy to have my family.

The nurse came and told me that they were waiting for a bed to open up in the ICU. I was worried – only super sick people go to the ICU. The only other time I was in the ICU was immediately after my transplant!  The nurse told me it was just because of the unexpected bleeding, and it wouldn’t be for long.

Apparently my doctor went in through my side (at my liver) and once done, his tool would insert some type of plug to close off the blood vessel and prevent bleeding, however, it wasn’t working properly and he was completely unable to close it off. Therefore it bled until it clotted. I had a lot of blood free-floating around my abdomen. My liver was very irritated, and fluid started collecting as well, I would soon found out.

The few-hour-long procedure ended up taking 6 hours.

Once they took me to the ICU, my family went and ate dinner in nearby Little Italy because the ICU wouldn’t allow them to see me again until they got me transferred and assessed. I was highly annoyed. The nurse was redressing some of my lines, and I’m not sure what else she was doing but I just felt like screaming, “LEAVE ME ALONE.” Thankfully, I tried to be nice but I was tired of being looked at, touched, tested, talked to. Thankfully I didn’t remember that the plan was for me to go home the next day because that had obviously changed.

Again, I was so frustrated to be in the ICU. There were bright lights all over. I had 2 IVs, an arterial line, telemetry leads all over my chest, a urinary catheter, oxygen, and a pulse ox probe wrapped around my finger. Was I really in that bad of shape? Once they took my vitals, the doctor updated my med orders, etc., I was worn out. The ICU was pretty firm on only having two visitors at a time. My family returned and I said goodbye to my parents and my husband. I wanted Scott to stay with me so badly, but he was so spent already, and the ICU doesn’t allow overnight visitors.  Everyone promised to come back the next day, and somehow in all of my fear, I fell asleep out of sheer exhaustion. 

The next day, the nurse was gung-ho about the order to remove my urinary catheter. I told her it was a bad idea and that it took me forever to be able to pee after anesthesia. She persisted, and I proved to her that I could not pee on my own. I kept telling her I needed the catheter back, and she did a quick bladder scan on me probably half a dozen times until it showed enough urine in my bladder that I would most definitely have to go to the bathroom. She brought in a fancy “bedside commode,” and I proved to her that I indeed could not go. I won that battle, and she had the next shift’s nurse put the catheter back in.

I have nothing against young nurses. Heck, I used to be one. My next nurse seemed like a baby in comparison, but she was nice, and I didn’t care. Until she tried to put the catheter back in. She successfully placed it – twice – IN MY V@GINA! If you don’t understand a urinary catheter, it goes in the urethra, which is an entirely different place than a v@gina. If you don’t understand that, take an anatomy class. Catheters aren’t the most comfortable things to be placed, and even on m0rphine I asked her to find another nurse who had done this before to come handle it. I asked the first nurse if she had successfully placed a urinary catheter before, and she told me she had, possibly around 20 times. I assumed she probably meant on the fake person in her school’s simulation lab. The other nurse saved the day and they let me keep my beloved catheter until the next day, Sunday, when they finally allowed me to go back to the transplant floor.

It was funny because I had the exact same room as before the surgery. I had somehow gained a room mate, but I didn’t really care. I was just happy to be back on a regular floor and out of the ICU! No catheter, no arterial line, no large bore IV. I could walk, and I somehow was just more alert.

And, unfortunately, more cognoscent of the pain.

The hospital is a horrible place for sleep. The PCNA comes in all night (okay, every 4 hours) to get your vitals. Nurses come in to hang your fluids or antibiotics at midnight if needed. Lab comes in between 5-6 to draw blood. A resident or two comes in around 6 to see how you are and gather any new info to tell the other doctors. Food services brings breakfast in around 8, and between 9–10, the entire team of doctors – the attending, residents, physicians assistants, even your regular nurse – parade into your room to ask you questions. This is your one chance to request any changes in meds or tell them anything that might help them help you. Every single day I told them how bad the pain was and asked, “Are you sure the bleeding should be THIS painful?” And everyday, they reassured me, “Internal bleeding is one of the most painful things there is.” I told them how I felt cut wide open like when I was transplanted, and they reassured me every single day that the pain was normal for what I had going on. I still can’t understand it. I was really in agony. Thank God for m0rphine. But most narcotics make me itchy, so every 4 hours I got 4mg IV m0rphine with an IV dose of Benadryl. What a poor sight I was.

So Sunday, once back on the unit, I was really wanting to take a shower. I had been laying in a gross hospital gown for 2 entire days. My nurse explained that due to the bleeding, my hematocrit was really low (7), and it was too low to let me shower. If I fell or got hurt, it would be very dangerous. My mom suggested that she could wash my hair in the sink if only we had a chair that fit under the sink. My nurse saved the day and found the perfect chair! So I got a washcloth bath courtesy of myself and a blowout courtesy of my dear mama. I felt like a new person!

The days came and went. I was thankful to have the same nurse during the day for 3 days and the same nurse during the night for 3 nights. Transplant told me on Monday that I could go home whenever I could get onto oral pain meds. I knew it wouldn’t be that day or the next, and I told them that. I got ultrasounds most days to check the bleeding and fluid. The volume was slowly going down. My hematocrit was still struggling, so I received a unit of blood. That, with a few bags of IV fluid, really perked me up. I was still in pain, but I felt more like myself.

My hematocrit increased. I took a shower on my own on Tuesday. The pain persisted. I really wanted to go home.

I decided Wednesday was a good day to try to go home but I had the attitude of, if the pain was too bad, we’d simply wait a day.

My team didn’t push for oral meds until they abruptly cancelled the IV m0rphine on Tuesday without so much as a warning. I had an order for 2 oxyc0done every 6 hours, so we switched to that. By evening, the pain caught up with me and my nurse had the doctor on call put in a or time order for another dose of m0rphine. I was discouraged, really wanting to be on oral meds so I could go home. By the next day, I was fine on the oral oxyc0done. Uncomfortable, but not in severe pain.  Then the physicians assistant told me the maximum she could legally send me home on would be one oxyc0done every six hours – less than half of what I was taking orally in the hospital. I’m not going to get into my opi0id reform rant right now, but that’s a huge jump. She told me I could alternate it with muscle relaxers and Tylenol once I got home, and that made me feel “safer.” I figured, worst case scenario, I could just get re-admitted if the pain was that bad. (Thank God it wasn’t, and I did well resting at home on the oxyc0done, muscle relaxers, and Tylenol, and I’m very proud that I only took the oxyc0done through the weekend and had 1 extra dose on Monday until I was completely off of it.)

That last day in the hospital though, that Wednesday, was hard. I was so exhausted from not sleeping well. I had been on m0rphine for 8 days. I had started wheezing because of all of the fluid in my abdomen. I was receiving breathing treatments for the wheezing and cough I had. (The cough is a very long story – I had it for about a month due to being sick and then allergies…. I still had it in the hospital and even completely lost my voice.  After the surgery though, it became a new, deep, productive cough… it was just a mess.) I had two panic attacks, and my physicians assistant only let me have half the Ativ@n I take at home until I asked to speak to her, broke down in tears, and she agreed to let me take the other half. My poor husband has never seen me as bad off as I was for those 9 days in the hospital. And on that Wednesday, I just sobbed in his arms. I was so overwhelmed, I feel like I was having a little bit of withdrawal from the IV m0rphine, and I was scared of the pain, a little scared to go home.

They had an ultrasound scheduled for that day, but they were late getting the order in, so my scan didn’t happen until 3pm. I got back to my room around 4, and the scan wasn’t resulted until around 5. It showed the bleeding in a different spot, so the plan was to do a blood test to see if my hematocrit was the same as it was in the morning or if it had dropped. A drop would indicate active, new bleeding. That took an hour to come back, but thankfully, my hematocrit actually increased! So the conclusion was that it was old blood just moving around.

We didn’t get home until around 8ish. I was so excited to see my dogs. Haylie nonchalantly greeted me, and Ruby jumped up on me and squealed in delight. Scott helped me get settled, and I slept until afternoon the next day.

The first few days home, my mom came over while Scott worked. Friday I had an appointment with my PCP and she helped answer some questions and transition me out of being hospitalized. She gave me a prescription for Zofran which I had been taking for nausea, and reassured me that everything was all right.

I rested and rested for the coming days, and I spent the following week (this past week) resting and laying low. My last dose of narc0tic was Monday, and Wednesday was my first day driving again. I had an appointment both Wednesday and Thursday (yesterday) and although I’m pretty sore and weak, I’m only on Tylenol for the pain and am determined to resume my normal life next week.

We’ve been so blessed to receive several meals from people at our church. I don’t have much of an appetite and have been losing weight, but I’m not up to cooking for Scott so it’s been great. We almost have too much food!

Right now I’m still sore and get exhausted by 4pm. I’m slowly getting over the 99* mini-temps and chills I’ve had since the hospital.  I have nausea and an awful appetite and am down just over 10lbs. I think my poor stomach and digestive system are just super confused. My doctors say to give it time.

I had a follow up with my transplant surgeon on Monday. A new scan showed that more bleeding and fluid had been absorbed. There was discussion of blood thinners while I was inpatient, and for now – thankfully – I just have to take a baby aspirin. My labs looked amazing, and everyone was really happy with my progress. I have a follow up scan next Wednesday, and we plan on doing ultrasounds every 6-12 months to make sure this doesn’t happen again.

So, you wonder what caused the stent to be almost completely closed off? “Low dose” hormones I was put on in October for premature ovarian insufficiency, aka “when your body doesn’t make enough estrogen, putting you at risk of heart disease and osteoporosis.” My surgeon told me they were not low dose as the OBGYN had told me. So I’ll deal with that when I visit her in a month.

I also want to add that in the hospital, my sweet friend visited me a couple times and my sister in law came for a great visit one evening before the surgery. The thing about being in Cleveland is that it’s an hour away from most of my friends and family, so that makes visits even more special.  I’m so blessed to have great friends and family. I received so many cards, texts, messages…. I felt all of the prayers and I thank anyone who prayed for me. 

I know this post was long, but my hope is the same as always: that anyone struggling with something similar can either find answers or courage from what I went through. 

This experience had reminded me again that life is fragile, and we are not guaranteed tomorrow. I was lying in the ICU when I was supposed to be having an amazing time in NYC with my husband. What a difference a day makes! I was so afraid to die, and thank God I didn’t.  I couldn’t imagine leaving my husband or my family and friends. I was seriously so afraid I wasn’t going to make it. Maybe it was the anesthesia or the drugs, but it’s scary when you are in really bad shape and are powerless to change anything. 

I have a renewed joy of life and love of each new day. I thank God for putting that in my heart and for reminding me how special this life really is.

Much love to you all.

Urgent: All Over Again

It was Wednesday.  We had just left the Cleveland Clinic, and I told Mom how excited I was that I wouldn’t have to come back for three whole weeks!!  It had been months since I got a break this long, and with the splenectomy behind me and the fact that it was my one-year transplant anniversary, it felt so good.  I knew the CT scan and ultrasound could come back with abnormal results, and I knew there was an ongoing issue with a vein to my liver narrowing, but I wasn’t worried.  It was a good day.

Then Thursday, I got a phone call.  Funny how one phone call always has the power to change so much.

It was my transplant coordinator.  The ultrasound showed there might be more narrowing, but the radiologists and surgeons discussed my case and decided they’d need a different kind of CT to be sure about the plan of treatment.  I had to come in Friday for an abdominal CT because the matter was urgent.

Phone calls like that have been getting familiar, so the future scenario had definitely crossed my mind.

I went in Friday for the CT with IV contrast – no big deal.  I’d already had a few earlier this month, and dozens more in my lifetime.  But at the end of the day, I got a phone call, again from Molly my transplant coordinator, saying the narrowing has worsened and the portal vein and left renal vein to my liver are too constricted.  There isn’t enough blood getting into my liver, and my surgeon, the head of transplant at the Clinic, and a couple of interventive radiologists have discussed my case.  They all came to the agreement that I need to come in as inpatient Tuesday morning (Monday is Labor Day) to begin a process to open the veins.  Tuesday, they will put me on heparin therapy to thin my newly platelet-loaded blood, and Wednesday morning they will put me under general anesthesia (thank goodness) while they go in through my liver and feed a catheter through to the veins in question.  They will then inflate balloons in the veins and pull out.  If the veins stay open on their own, that’s great.  If not, they will put stents in to keep them wide open.  Knowing how I handle anesthesia, I’ll be in and out for the rest of the day, and then Thursday, I will have an ultrasound to see if the veins are doing well.  If they are, I can go home.  Let’s pray they will be.

So that’s my week.  And there goes next week.  I was supposed to start driving, go back to my nanny family, and basically get on with my life without my spleen, finally feeling good.  But sometimes life has other plans in store for us, so I have no choice but to again, bow low and do what’s best for my body, reluctantly conforming the rest of my world to oblige.

Your prayers are so greatly appreciated.  This is a risky procedure, plus there are so many risks to undergoing anesthesia.  And lately I’ve been completely emotionally drained, and honestly, really struggling. Having a hard time staying afloat right now.

Thanks for always being here.  I love you all so much.

Amanda

Another Update

Once again, I’m a little late on an update!

I’m all healed from my massive splenectomy!  My 40 staples are out, my incision is healed, and other than fatigue and sore (cut) abdominal muscles, I think I’m doing pretty well!  This surgery was a lot easier than my transplant, no question.  Homecare is gone, I’m done laying on the couch all day, every day 🙂 and I’m eagerly awaiting the day when I can drive again (mid-September).  

My labs have fabulously improved, and with the platelets and WBCs under control, we’re now working on the red cells.  I’m anemic, and my oncologist is trying iron supplements for a couple months before we look at any other measures.

As far as my surgeon, during the surgery, he discovered a narrowing in one of my main portal (liver) veins.  The narrowing causes decreased flow into my liver which could increase my liver enzymes (bad).  So tomorrow I’ll be at the Clinic for an ultrasound and CT scan to get a better look at that.  And a visit to infectious disease to see why my pneumonia shot isn’t “sticking.” 

That’s really it.  Hoping to start “normal life” next week (minus the driving 😦 

Happy weeks to all of you!

Amanda

In over our heads

Seeing we just returned from Florida on Saturday, May 22, regular testing at the Clinic on Monday and meeting with my long-time pediatric hepatologist on Tuesday was a little stressful.  Then, we found out the testing revealed my liver needs to come out now, and in a matter of a discussion lasting for under an hour, our lives were turned completely inside out.  My doctor and I have a close relationship, and she fought back tears as my mom broke down.  I knew then, that we were forced onto a journey that we didn’t ever believe would happen to “us,” a journey that we ultimately will never forget.

Wednesday was a matter of the transplant coordinators figuring out how to schedule the “cornerstone” appointments of anesthesia, social work, surgeon, and hepatologist around completing testing like EKGs, ABGs, a dozen of physician consults, and meeting everyone from social workers to psychiatrists to anesthesiologists.  In no time, they had me scheduled to spend Thursday and Friday attending a long checklist of appointments and tests, with a much needed long weekend (Memorial Day), followed by intense Tuesday and Wednesday schedules.  And then I’m done.  Or so they say.

Monday’s tests were an abdomen ultrasound and a contrast MRCP (a form of MRI)  The new venous collaterals and mysterious tumor sent up a million red flags, and on Tuesday, my doctor told me how important it was that we expedite the pre-transplant work up and get the liver out ASAP.  As I’ve said, she was excited about the outcome and thinks my life will be something I’ve never known before.  I’ll feel good, have energy, and be able to really live.  Being sick since age 5, I just won’t have any idea what it’s like.  I like the idea of it though!

In between telling our close friends and family, the next few days were just rough.  

Thursday was by far the craziest day of pre-transplant prep – it was painful, it was long, and we had a ton amount of information thrown at us.

5:45am: Leave for Cleveland
7am: Laboratory – about 30 vials of blood pretty much drained my energy
7:40: Adult gastroenterologist consult
8:30 Financial specialist
9am: Radiology and surgeon consult 
10am: Chest X-ray (which got pushed to late afternoon)
10:30am: EKG  (which also got pushed to late afternoon)
11am: Pulmonary function testing
11:30am: Transplant coordinator
12:30pm:  Computerized health questionnaire
1pm: Infectious disease
2:30pm: Arterial blood gas testing (PAIN) 

Friday was a little better…

The day began at 7:30am when I got radioactive dye injected into my vein for later bone imaging.
8:30am: Lisa, social worker
10:30am: Bone scan to check for any movement of the tumor
HOME early!

I chilled like crazy all weekend, as fatigue is my biggest symptom and I don’t bounce back too well from long days.

Next week, I’ll be meeting with a hem-onc, a women’s health CNP, an anesthesia specialist, and there will be a large teaching session where we will be drilled in the As-Zs of a liver transplant.

I’m a little thankful I’m too tired to care much about this.  Otherwise I think I might be a mess.   Plus God is carrying us right now, I can feel it.  Plus this isn’t quite reality yet.

Love to you all.