Has anyone here ever heard of Superior mesenteric artery syndrome?
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).
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.
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
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.
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.
all these illnesses are rare diseases.
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.
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
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
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
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.
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.
Do you people actually think there's no cure ?