Superior Mesenteric Artery Syndrome

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Superior Mesenteric Artery Syndrome
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    Surgery Saved My Life, But I Still Live With Chronic Illness

    In the life of someone with Ehlers-Danlos syndrome (EDS), it’s rare to ever just have one specific treatment or surgery. In my case I actually had multiple surgeries before learning about my EDS diagnosis or what was causing my debilitating abdominal/pelvic pain and digestive issues. Once I learned about the multiple vascular compressions and that they are also linked to my EDS, I knew I was more at risk, especially with organ damage or failure if I didn’t address them with surgery. Unfortunately since MALS (median arcuate ligament syndrome), SMAS (superior mesenteric artery syndrome), NCS (nutcracker compression syndrome), MTS (may-thurner syndrome) and PCS (pelvic congestion syndrome) are even more rare than EDS, finding a surgery or surgeon was another journey in itself. When I continued losing weight from not being able to eat and drink without pain, became bedridden because of my abdominal/pelvic pain, and then started vomiting and having constant kidney stones, I knew we were desperate and had to do something soon. That something was traveling to Germany during COVID to specialists who understand these conditions and the connection to EDS. With the help of crowdfunding for the cost of the trip and surgery (since there was no way insurance would cover without being in the US), not only did I make it there and get answers and diagnoses that took over 20 years to get, but I had surgery at just the right time. My duodenum had actually stopped functioning, I was having trouble urinating and my left kidney was dropping and getting zero blood flow when I would stand because my renal vein and superior mesenteric artery were completely pancaked. It’s been a long and slow recovery, but I’m now about to hit my surgervisery milestone. I’m not just able to eat and drink again, I don’t have the severe and unbearable abdominal/pelvic pain or symptoms that I accepted would be my reality for the rest of my life. Being able to do things like playing with my two young daughters and helping my husband clean the house or fix meals, I’m so incredibly grateful for. But at the same time, others who aren’t familiar with EDS have a hard time understanding that although I had a surgery that saved my life, it wasn’t a surgery that would cure me of chronic illness or pain. EDS affects each individual differently, but those of us that have it, all know connective tissue is in everything throughout your body. I’ve gone to war and won one battle, but it doesn’t mean I don’t have others currently like POTS (postural orthostatic tachycardia syndrome) or MCAS (mast cell activation syndrome), or that I won’t continue having others arise. The difference now is I know the enemy I’m up against. I have confidence in myself that I continued fighting despite disbelief and misdiagnoses for years. I’ve met some wonderful and knowledgeable doctors that are now on my team, so I’m not alone. And most importantly I’m creating awareness, change and a path for my daughters who are starting to go through this journey. I know family and friends may be confused that my surgery was successful when I still need a cane or wheelchair some days, still have to cancel plans last minute or can’t attend a social gathering, still have food intolerances and still talk about my health. Unfortunately EDS is here to stay, but it just gives me the opportunity to share with everyone I know or come across what I and others are experiencing. I’ve learned to adapt and be proactive with my health. I can no longer pretend I’m a “normal,” functioning human being because it was doing me an injustice and also an injustice to everyone who lives with this disease. It’s OK to rest, it’s OK to save up my spoons, it’s OK to admit there are days I will have to stay in bed all day, it’s OK to take care of my body. But the difference is I celebrate every day I’m still here, celebrate that I have answers and proof of my symptoms and pain, celebrate everything I can do and each moment I get to be a part of, and celebrate that we can still have hope and live despite those obstacles.

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    Being Unique and Rare isn't all its cracked up to be

    In 2010, I was diagnosed with my first rare disease. Its called Celiac Artery Compression syndrome and I had to have a surgery called ‘An Articular Patch’ to improve my quality of life. The surgery worked in a way where my quality of life went from 30% to approx 75%. Then in 2015, I began to fall ill again. I was diagnosed with yet another rare disease called ‘Superior Mesenteric Artery Syndrome‘. This also called for a major surgery where I had to get a bypass on my duodenum. This again improved my declining health from 20% to 70%. In early 2018, I again became seriously ill with ‘Gastroparesis‘. Living with these chronic illnesses has ruined my career, debilitated my social life, and rendered me feeling absolutely useless. Im also dealing with a undiagnosed issue where every 10-14 days I have what I call “My Exhaustion Days”. I sleep for 2 1/2 days straight, unable to do anything but get up to use the bathroom. During these 2 1/2 days I am literally rendered useless. I have major brain fog, confusion, the inability to form thoughts and sentences and just simple life functions. If I try to fight it off the “Exhaustion Days” will extend themselves until my body chooses its time to come back to reality. This mysterious chronic illness has been going on for approx 1 1/2yrs now. Defeated doesnt even come close to how I feel. I have been strongly advocating for myself for close to 12yrs and I no longer have the fight in me. I am beaten down beyond belief. I have lost so much I worked so hard to accomplish. I could go on and on with other issues that have arisen because of these chronic illnesses but I feel like those other issues can be addressed at another time. My main goal here was to explain my chronic illnesses to the best of my ability and to not get to technical (just yet).

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    I got my Hickman line removed today! I have been dreading this since the day I got my line placed but now I’m so happy it’s over!

    So of course, things didn’t go according to plan. I was originally supposed to be sedated for the procedure but instead I was awake. Since my line has been in for a while I had a lot of scar tissue which made it harder for them to get it out. A lot of tugging and three shots of lidocaine (which I hate getting, that stuff burns!)

    I didn’t feel any “pain” throughout any of it BUT I did feel a lot of pressure and almost a heaviness where they were pulling at the line. They had to get some scissors and break up the scar tissue in order for the line to come out. The part I was most anxious for was when they actually pull the line out. With a previous picc line removal it was pulled so fast I felt it coming out inside me, so I was really scared that would happen again.

    The doctor told me when he was ready to pull the line out and asked him to hum while he did. He got in out in literally 2 seconds. That was the fastest part of it all.

    I’m a little sore, nurses said I would be tender for a few days. But I’m proud of myself and what I’ve accomplished.

    Community Voices

    Changes w/ Hickman line removal #ChronicIllness #Anxiety

    I’m getting my Hickman line removed tomorrow and I’ve already been nervous about the procedure itself but I wish the hospital would stop giving me so much different information. First they tell me I need a COVID-19 test, now they say I don’t. Then they say no one will be able to come with me to the procedure, now they say one family member can. They told me I can’t eat or drink 8hrs before the procedure but now they say I can. They told me I don’t have to get blood work before the removal, now they saw I have to. In the outpatient building that has a lot of traffic going in and out. They told me to get there a little after 9, now it’s 8:30 for labs. I’ve also only now been told I have to stay an hour after the line removal.

    It’s frustrating because this is already hard with my anxiety and it feels like it once I’m told one piece of information and I get used to it and form a plan in my head for it they change it and I can’t seem to keep up. I think they need to have better protocols and less contradicting information. #Anxiety #GeneralizedAnxietyDisorder #ChronicIllness #SuperiorMesentericArterySyndrome

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    Rare, is the bond we share

    Each year, across the world, the last

    day of February is marked as #RareDisease Day;

    it is a day set aside to raise

    awareness of over 6,000 rare diseases.

    What

    is the definition of rare? Something not occurring very often, something

    unusually good or remarkable. However, in our home and many homes like ours,

    the definition of rare is dramatically altered.

    My

    daughter, Jayde, lives with #PosturalOrthostaticTachycardiaSyndrome#EhlersDanlosSyndrome
    , #SuperiorMesentericArterySyndrome, and Nutcracker

    syndrome. I have #PosturalOrthostaticTachycardiaSyndrome#EhlersDanlosSyndrome, and Tarlov Cyst Disease. Except for #PosturalOrthostaticTachycardiaSyndrome,

    all these illnesses are rare diseases.

    With

    each diagnosis, the word rare took on an entirely new definition. Rare means

    doctors are dismissive, shying away due to their fear of not wanting to admit

    they do not understand. It is being told your disease is only in your head,

    overcome it, and your disease will magically disappear. Rare is living with the stares and assumptions of others that you are

    anorexic or faking your illness. Rare is being scolded, “Do not label your

    child.” Rare is nails on a chalkboard each time you hear, “Get well soon.” Yes,

    it is a kind sentiment. So why is it annoying? Because your head knows, there is no cure, even though it is your heart’s greatest desire.

    Rare

    is the isolation you feel after losing the majority of your friends because

    they cannot understand your reality. It is also finding a tribe of people who

    appreciate and accept you for you because they live in the same tormented hell. Rare is the pill

    case with five rows for each day of the week, ensuring you do not forget to take your medication. It is

    becoming more of an expert in your illnesses than most doctors. Rare is

    the mask you don for the world to see, smiling on the outside while your body is searing in pain. Rare is the frustration

    of needing medical supplies, but you cannot purchase those supplies due to a

    virus outbreak causing mass hysteria. Rare is a rolling cart in your living

    room, full of medical supplies, an IV pump, and a sharps container. Rare is

    closing your eyes, waiting for the sweet relief of sleep, waking each morning, praying the new day will be better than the last. Rare is the terror you

    experience when a new symptom rears its head, hoping it is a fluke and will quickly

    vanish. Rare is comprehending, you are considerably different, with the consuming

    desire to be like everyone else. Rare is the heartache that comes from knowing

    you will not graduate with your senior class because your surgeries and illnesses have made it impossible to keep up with school. Rare is

    buying the concert tickets, hoping you are well enough to attend the show six months

    later. It is mourning the life you can no longer live while learning to

    celebrate the mundane and smallest of victories. Rare is discovering

    there are only three doctors who specialize in operating on your spinal

    condition. It is the six-hour flight across the country in excruciating pain

    and no sleep, so your daughter can have her wish granted to photograph the

    California Coast – and knowing you would do it again to see the sparkle in her

    eyes.

    Rare is having your child’s diagnoses

    unlock your own medical mysteries. It is then living with the knowledge and

    guilt that the genes you passed onto them, is what makes them ill. Rare is the indescribable symbiotic bond you share with your daughter from needing to be more

    than her parent. Rare is a multitude of rollercoaster emotions, from fear and hope

    to elation and #Depression.

    Rare means digging deep and finding the hidden reserves of strength and determination

    to press on. Rare is living a life bathed in prayers, hopes, and wishes.

    Rare is my daughter, rare is me – Rare is us

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    Tips on a hickman line #ChronicIllness

    I’m getting my first hickman next week, I’ve had two picc lines before for TPN but my doctor had to pull my second line yesterday. During its original placement it was put to far deep into my chest so it had to be pulled twice, a total of 5 1/2 centimeters. After all that the insertion site was bigger than the line so it couldn’t heal properly. We’ve attempted to place a hickman before but I had a panic attack so this time they’ll put me fully asleep but I’m still really scared. #ChronicIllness #HickmanLine #PiccLine #SuperiorMesentericArterySyndrome #TPN

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    I’m new

    <p>I’m new</p>
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    SMAS? What Kind of Acronym is That?

    For as long as I can remember, I’ve had many health issues such as chronic migraines and #PosturalOrthostaticTachycardiaSyndrome. For as long as I can remember, I’ve missed countless days of school and teenage milestones from these illnesses.

    I experienced my first #Migraine at 2 and POTS my whole life, but I was finally diagnosed with POTS in March of 2016. #SuperiorMesentericArterySyndrome is just another one in the books now.

    On April 2nd, I attended my biannual cardiologist appointment with Dr. Joel Brenner. I went in thinking not much was going to change with my treatment plan, but that I did have something clearly wrong with my body. My parents and I weren’t sure if what I was experiencing had to do with POTS or something else. I was sadly wrong though when I thought it really could just be POTS.

    The first thing I told my cardiologist was that I had been out of school since February 14th. Over those few months I was home with extreme fatigue, migraines, nausea, vomiting, abdominal pain, and a lot more. I was throwing up for eight hours about once a week and barely able to keep up with myself. He told my mother and I that he thought I may have median arcuate ligament syndrome, a syndrome that has basically the same symptoms of SMAS. A few weeks later on April 19th, we were sent for an ultrasound at Johns Hopkins Children’s Hospital that would confirm whether or not I had median arcuate ligament syndrome.

    The wait for these results was short, but dreadful. I had no clue what was about to happen to me or what was to come. On April 24th we received the results of the ultrasound. When we got these back we saw that I did not have , but some rare syndrome called superior mesenteric artery syndrome and another called nutcracker syndrome. I was relieved until I realized I may need surgery for SMAS.

    During the rest of that day, my mother and I did continuous research on what this syndrome was. We found that superior mesenteric artery syndrome is when the duodenum (the first part of the intestine) is compressed by the superior mesenteric artery and abdominal aorta. Symptoms of this includes: nausea, vomiting, getting full quickly, abdominal pain, bloating, burping and weight loss. The angle of the compression is about 38-56° for a normal person, it becomes severe at about 25°. Only .013% to .3% of the world has this #RareDisease and it has a one out of three mortality rate. When we both saw this, it really started to make sense with my past history. I had been dealing with this all of life and had many doctors look past my symptoms. It also started to scare us both as we saw my health decline greatly in the last few months.

    At this point in time, it was just a waiting game to see what needed to happen next. This could include: new doctors, new medications, more testing, and surgery. I can say the only new item off of that list that I did not receive was new medication. While I waited it seemed hopeless and I felt like I would never get better, or at least feel somewhat normal for a while.

    By June we were meeting with the surgeon Dr. Brenner had recommended. He recommended Dr. Eric Jelin, a pediatric surgeon, who also just so happened to be one of the only surgeons in the country who performs the surgery for SMAS. When we first met with Dr. Jelin, we told him all of my symptoms while he looked at my ultrasound results. At first he believed that I did not have it due to the fact that I wasn’t throwing up enough. That was until he heard more about me and did more testing. He realized that since I’ve had this for so long, my body realized I could not take in high amounts of food and I was able to self modify my diet. Dr. Jelin also saw how severe angle of the compression was from the rest of the testing, my compression was at a 12 degree angle. The only option we had was for him to perform a laparoscopic duodenojejunostomy, this was to help make a bypass so I was able to eat and gain weight. Though this wouldn’t fix the compression and abdominal pain, I would have a much better quality of life. My main concern though was making sure that he didn’t turn my innie belly button into an outie from the incision made in my belly button.

    For a while my family and I spent a lot of extra time together and even took a little weekend trip to Williamsburg, trying to get the best out of our summer since we were uncertain of when my surgery would be.

    On June 22nd I had my final pre-op test that would determine when I had my surgery. They did a live x-day of my stomach and intestines with contrast to show how small the opening of my duodenum really was. For a while the contrast sloshed in the area but finally made its way through. When Dr. Jelin saw these results he wanted to do my surgery as soon as possible, but we were unable as many things popped up. Then before I knew it, the surgery was scheduled for August 17th.

    I spent the rest of my summer doing anything fun my body would possibly allow me, spent some time in the hospital trying IV saline therapy to get my fluids, and resting. During this time, my POTS really took a turn and I experienced very minimal symptom days.

    Soon, Friday August 17th made its way around and I had never been so nervous. It was my first surgery and a major one at that. My parents and I woke up around 3am to get ready to be at Johns Hopkins by 5am for my surgery at 7am. The time went by slowly, but my parents, surgeons, and specialists supporting me the whole time. Before I knew it though, I was put under and out of surgery. The surgery went extremely well and the only bad thing they found was that the angle was at 10° instead of 12°. I woke up with an unpleasant NG tube down my throat that drained my stomach, but I did have the best nurses once I made it to my hospital room. I would then spend seven days in the hospital with many family members and friends coming in and out.

    My day and night nurses for the weekend were so wonderful and patient with me, they always came to check on me and make sure I was as comfortable as possible, as well as helping my parents. My mom never left my side and my dad only left when he absolutely had to. Dr. Jelin also made sure to check in and let me know just how well I was doing. They all made me feel like I wasn’t even in the hospital. I started to have hope that the rest of the stay would be just as good.

    The bad day came around though and was the Monday after my surgery. I was extremely tired, in pain, and aggravated. The NG tube kept me from talking and being able to sleep because of the drainage noises and pain. I was told the tube should be coming out that day, but when they did the live x-ray it showed that the surgery was a success but that the tube was two inches too deep. My nurse had to pull it out by two inches and leave it in for another painful day.

    The next day the tube finally came out and I was able to talk, shower, and sleep again. By Wednesday I was on a full liquid diet since my stomach needed to readjust for about four weeks and by Thursday night I was able to leave Johns Hopkins.

    Being back home was an adjustment as I was having trouble getting up, moving, and being on a restricted diet. I became very malnourished and weak from being unable to eat, I also became extremely ‘hangry’. The week after I got home I was finally able to eat softer foods and help build myself back up.

    On September 12th, I had my post-op appointment with Dr. Jelin where I had the strips from my incisions removed and the go ahead to start eating real food again. Though I still have pain and I am still recovering with POTS problems on the side, I can finally eat real food and get on the road to recovery.

    I’ve had my ups and downs since the surgery, whether it be health wise or with the people around me, but I’ve tried to stay positive. I was finally able to start cyber school and continue my schooling on October 2nd, I have never been so motivated.

    I am almost 17 and I have battled issues no teenager should have to battle, but I am strong and a fighter. I am still a normal girl with likes and dislikes, such as loving photojournalism and hating cheese. I knew my journey would never be easy from the beginning, but I knew I could never give up on myself and the people that support me endlessly. I don’t think I could ever thank my family for their support and the doctors enough for giving me the chance to recover.

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