The Rarest of Guest Bloggers: SMA Syndrome

My name is Danielle and I am 26 years old. Growing up I suffered with ongoing stomach issues and doctors just couldn’t seem to figure out a cause. These symptoms would come and go in waves and there were even periods of time where I would begin thinking I was doing okay! Sadly, those times were short lived and when my senior year of college came about, things took a turn for the worst.

In 2015, I became very ill. It began with my endometriosis creeping back in and that was followed with my gallbladder needing to be removed, and despite those things, I continued to progressively get worse – especially stomach wise. I was having severe abdominal pain when I ate, nausea, vomiting, early satiety (I would take a few bites of food and feel overly full instantly), bloating and weight loss. I was only 105 lbs to begin with so I didn’t have any weight to lose so things became critical rather quickly.

In February of 2016 I was hospitalized because at this point I was not able to keep anything down, not even water. I had dropped to 90 lbs rather quickly and we were very scared. My doctor had run so many tests but could not figure out what was wrong with me, until he happened to be in the right place at the right time. He was at clinic where he overheard a nurse, who isn’t typically at that clinic, talking about another girl who had just been diagnosed with this rare condition. He immediately thought it sounded very similar to me so as soon as he was done at clinic, he came directly to the hospital and went to the radiologist. He told the radiologist to relook at one of my CT scans but from a different angle.

They immediately saw the problem and he came up to my room where he finally looked at me and said “I figured out what is wrong with you. You have SMA Syndrome,” otherwise known as Superior Mesenteric Artery Syndrome.

This condition, SMAS, I’ve never heard of it, I don’t know what is hitting me and how it is going to change things, what my life will look like now, all I know is it is rare, and though it has terrible symptoms, it can be very hard to detect. I had numerous CT scans but until they specifically looked for the compression itself, they were missing it on my scans for the longest time even though it was right there. The compression can be seen on CT scans, angiograms and upper GI studies with barium.

SMAS is an extremely rare and potentially life threatening stomach condition in which the third portion of your small intestine (duodenum) becomes compressed between your abdominal aorta and Superior Mesenteric Artery.

In other words, part of your small intestine becomes crushed and food is not able to pass through, creating a blockage.

This can lead to severe malnutrition, sometimes resulting in death. The mortality rate for SMA Syndrome is so high (1 in 3) because it is so rare and often times there is a delay in diagnosis. As you can see from my story, I was extremely lucky that he found it when he did or otherwise I might not have been here typing this today.

So how is SMAS treated? There is no cure.

There are also 2 types of this condition. One being acute onset, caused by extreme/sudden weight loss often following something like scoliosis surgery. The other being chronic, meaning it develops over the course of their lifetime. Surgery can be done to relieve the compression, or sometimes gaining weight (usually via feeding tubes) can also relieve the compression and allow food to start passing through again. However the damage done before it is found cannot be reversed and often times the symptoms can still remain even after surgery or weight gain, which is why there is technically no cure. In acute cases, the prognosis is better and oftentimes weight gain is enough to correct it and relieve the symptoms.

Chronic cases are a bit different, these cases are where the symptoms can still remain even after medical intervention, they aren’t easily treatable and there is absolutely no cure. That was the case for me. As soon as I was diagnosed I was immediately put on TPN (total parenteral nutrition) to help get me stable enough and I had surgery within 3 weeks. Since then, I have continued to have an avalanche of problems and my symptoms have remained. I continue to have pain with eating, nausea, vomiting, bloating and severe motility issues. The damage done to my body from SMA Syndrome has caused the entire rest of my GI tract to slow down and not function properly, so I have developed other chronic motility issues from it as well (such as Gastroparesis and intestinal dysmotility), which sadly is often the case for many people diagnosed with SMAS.

After numerous attempts to try and get things under control, I had to get a feeding tube placed in my abdomen to help give me the vital nutrition I need to sustain myself. I had a surgical GJ tube placed a year ago. I also am currently on TPN through a central line (port) in my chest due to the severity of my motility issues at the moment and not being able to tolerate my tube feeds right now. So often times feedings tubes are needed even after surgery to help manage the symptoms that remain and to help sustain individuals with SMA Syndrome.

Getting a feeding tube can be very overwhelming. It isn’t easy to process what it is like to have a tube surgically implanted into your body and it is a huge adjustment.

But what made the transition easier for me when I had my surgery was Newbie Tubies. I came across Newbie Tubies on instagram (@newbietubies) and saw that they create packages for people who are getting feeding tubes that are filled with all sorts of awesome things to help someone recover from the procedure and different items for the new tube as well.

The goal is to make the transition easier for someone by sending them a package to brighten their day. You can apply yourself or you can nominate someone to receive a package.

 

When I recieved my package it had things such as a blanket, water bottle, socks, handmade heating pad, tubie pads to decorate and protect the tube, bath bombs and a coloring book. It also included a list of tips for living with a feeding tube for those who are getting their first tube. Also, everything was donut and dog themed to fit my personality, which can be noted on the application to make each package more personalized to the recipient.

I cannot express how much receiving that package meant to me when I returned home from the hospital and how much it lifted my spirits. Newbie Tubies is truly amazing and it is creating an awesome community of fellow “tubies” on social media. It is awesome to be able to connect to others who have feeding tubes as well because you can relate to them and also share tips and tricks with each other to help make living with a feeding tube more manageable.

So if you have a feeding tube, are getting one or know someone with one please go check out Newbie Tubies on instagram. Or even if you just want to help, you can donate money or items to go in the packages sent out. So please check it out!
I share my story and my experiences with SMAS with hope that it will help someone else find their diagnosis and know they aren’t alone in this journey. It can be incredibly hard finding any answers, and I know that if it weren’t for individuals working to spread awareness through social medias and the stories others have shared, I wouldn’t have been diagnosed, I would have continued suffering without answers for much longer.

SMAS is an extremely rare condition, and because of how rare it is it is and due to overlaps with symptoms of many other stomach conditions, it often isn’t thought of. Not a lot of doctors know much about this rare disease, most of the time they have only read about it in textbooks during medical school, but each patient, each case, is unique and complex, not one fitting the case studies or textbooks perfectly.

I had no idea what it was until I was diagnosed, so I hope that reading my story can help someone else out there and just educate more people about this condition. For more information about SMA Syndrome, you can go to https://rarediseases.info.nih.gov/diseases/7712/superior-mesenteric-artery-syndrome.

 

Post by Dani Fantaskey — guest blogger and newbie tubie package recipient


 

Thank you Dani!! Your post is fabulous and I am SO glad your package made such an impact on you – seeing your positive remarks truly inspires me to keep working hard to individualize each package. I love doing it, so I’m glad it doesn’t go unnoticed 🙂 So happy to have you, keep in touch and I’d love to have you back involved with the project anytime! Lots of love!

If anyone has questions or comments for her, comment below or contact me and I will get you in touch with her 🙂

xoxo

Rachel

Tips for Tubies: A Tubie’s Guide To Success Vol. 1

 

  1. The doctors work for YOU. Not the other way around. If a doctor (or a nurse, tech, or anyone else in the medical system) treats you with any less respect or dignity than you deserve, consider finding a new specialist.
  2. No question is a bad question. There are awkward questions and there can be a boatload of questions, but all of them are important. Ask until you’re satisfied, even if the doctor is acting rushed or distracted. Your health and confidence is more important than anything else.
  3. Some surgeons aren’t big talkers – they like to get the job done; make a list of questions and concerns and make sure to ask them the first time you see them pre-op/post-op or during your follow ups, it could be the only time you see them!
  4. Recovery can be even more challenging than surgery itself. Have people who will be around to help you or at least set up some people to come visit and check on you each day. Before surgery, set up a place by your bed or couch where you can keep some essential items so you won’t have to get up and down every time you need something.
  5. Don’t push yourself! There are no “shoulds” with chronic illnesses or tube feeding. If recovery is taking longer than planned, take some time off from school or work if you are able to! Learn that it is okay to say no when your friends want to go out to eat or get drinks late on a Friday night, if you feel cruddy or just don’t want to be around food, it’s okay to stay in or suggest a different plan. No guilt.
  6. Learn to advocate for yourself. It can be hard to really get doctors to understand what you truly feel and then to get what you need to be comfortable. Be persistent and thorough in explaining symptoms and how it affects your life. If you aren’t good at being forward, take a parent, spouse, relative, or friend who can help make sure everything gets covered.

 

These are just a few of the major tips for getting started with “tube life,” but they’re applicable throughout the journey with feeding tubes and really with any chronic illness. Learning to manage your case, advocate for yourself, and stay on top of appointments/doctors, questions, and treatments both past and present can be a big task, but staying organized and figuring out early on what methods work best for you to manage it all is really beneficial in the long run.

Keep your eyes out for more tips, the next round will be more tubie-specific regarding tube care and what to look out for vs what not to get freaked out over! 🙂

Thanks for reading and  I hope it was helpful! If you have questions or suggestions don’t hesitate to comment or message me!

 

 

The Start of Newbie Tubies

During recovery from my second tube surgery just 3 months ago, which I admit was long and pretty brutal pain wise, I realized how lucky I am to have such an incredible family support team working around the clock to help make me as comfortable as possible. I know so many people, of all ages, are walking the same journey but don’t have the support system that I do, and because these conditions are so uncommon, most people have never heard of them or have no idea that anyone can require a feeding tube no matter how old they are or how healthy they have been.

 

IMG_1208
3/2015– In the hospital for a picc line infection and the decision was made to place a surgical feeding tube
IMG_8485.jpg
10/2017– first week of recovery from jtube surgery; the pain was unbearable, my sweet dog by my side 24/7 and constantly alerting me to pain and tanking blood pressures.

Sadly, most of us –myself included– just have to learn from experience, both our own and from online connections to people who are also experienced tubies or caretakers. I’ve had tubes for 3 years now, so I have a lot of first hand experience as well as what I’ve learned from my online community; so, if I can use my experiences and my knowledge to help bridge the gap and make the transition to tube life at all easier, I can’t imagine not doing it.

img_7313
2016– me on a “good” day; a summer day embracing my tube and my central line and not being afraid to let the world see those parts of my body that keep me alive
IMG_9131
3/2017- My post-surgical, bloated tummy with both a gtube and a jtube; sadly my tubes haven’t helped me gain weight, yet!

In December I began looking for small businesses, mostly on Etsy, that would donate tubie products and a few other care items to my cause and throughout the holidays I used my own funds to buy other products that were on sale, some items included were microwave heating pads, cute socks or fuzzy socks, soft throw blankets, journals and motivational books/journals, etc. I have a more detailed list I will share later on 🙂

IMG_9093.jpg
One of my first big donations–  heating pads from DivineComfortRicePks on Etsy; definitely recommend her products 🙂

In January I had enough to begin! I set up an online application and at this point (January 12) I’ve already had 10 applications! I’m so happy to have another way to share my knowledge, advocate, and most importantly, just to help others who are going through the same/similar things I have/am. It is amazing and inspiring and I couldn’t be more excited.

IMG_8947
My first package, it was smaller and more simple than what I send now, but it was for a beautiful friend who was anxious about surgery and it inspired me to continue with this project.

As excited as I am, I just can’t fund it all on my own. I use all of the profits from my paintings (whatever is left after canvas, paint, mediums, etc.) as my main fund, but that’s limited and depends on how much art I can sell. I do have a few other fundraising ideas, but I will also need to find more donors who are willing to help just because they feel that my cause is worth it.

 

IMG_9073
11/2017– My first public art sale/”show” at a local elementary fundraiser; I sell my paintings on my blog, on facebook, and just through friendly conversation/inquiry 🙂

Depending on the products I have on hand and the person (based on age and needs of the tubie), each package has a value of $25-$50, and that is without shipping. Shipping can cost a minimum of $14-$15 but can be as high as $30, again depending on weight and size of items and whether or not they will fit in flat rate boxes.

Right now, I’m still working with some donated items to create each package; right now, it is mostly tubie pads, some self care/pampering items, a handful of heating pads, blankets, and some extra little do-dads. But I’ve made a large dent in my supplies, so I am thinking a bit more about how to acquire some new donors and brainstorming some opportunities to find new products either by donation or for major discount!

26653039_10211000341541272_1874031463_o
One of my favorite tubie pad shops, tubie whoobies, donated some incredible tubie pads for us.
DSC_1288
Some of my “do-dads” that just add a little fun, color, or comfort to a package for kids, young adults, or even kids/siblings of the tubie!

I love supporting small businesses and I include a card in every package with the information of each (business) donor so that my newbie tubies will be able to purchase more of any product they find helpful. I also post any business/shops that donate to my instagram, facebook, and blog, which ends up hitting over 3k people, many of whom are facing chronic illnesses and make great customers.

I know I have so many loyal followers who like to support all I do, and I don’t want anyone to feel any pressure to donate when it is not convenient for you; honestly, I’m sure I’ll be posting my amazon lists or shipping needs fairly regularly, so if now is not a good time, please do not feel a need to donate or purchase anything for me!

 

That said, here are a few ways you all can help right now:

  1. I accept re gifting!

**Did you receive some Christmas gifts that you know you aren’t going to use? Maybe too many of one item? I know many spoonies end up with lots of coloring books and fuzzy socks!

Or do you just have some extra stuff you aren’t sure what to do with? If it’s something someone could love, it’s package sized, and its in good shape, don’t throw it out!

Way too many adult or child coloring books? How about gel pens, markers, crayons, or any other drawing/writing instruments??

Small toys for children, stickers, etc? Common one, too many stuffed animals?

An extra planner? Fuzzy socks or small throws?

I guess you can say that I will be your “goodwill.” Let nothing go to waste or sit around unloved, instead, donate it to a good cause!**

See the lists below for more information about helpful products/items; I will take a lot of small items to save for someone who would love them!

 

  1. Purchasing a painting

**My art directly benefits my project! You can order my paintings on the blog or by contacting me directly through email or facebook.

Any profit – what I don’t use for paint/canvas/pouring mediums/etc.—goes straight into shipping and shopping for Newbie Tubies!

If you are local, we can meet in person to avoid shipping costs.

 

  1. Donations

The last option is just making a donation. If you want to make a monetary donation that would likely go towards sponsoring shipping for a box (or two!), you can do so through pay pal, send a check, or if you’re local we can work out a time to meet up for a cash donation if you prefer that.

I am also including my amazon wish list, “Newbie Tubies Wish List,” and my Etsy list, that have items, labeled/listed by priority (amazon), that I update based on what I could use at any given time.

If you are local and would like to donate but would rather pick up an item or two at the store when you go, I am including a list of other items as well. They can be dropped off at multiple locations, so please email me and we can make a plan (or if you work with my parents they will take the items as well). 🙂

If you are a small business, or know others with small businesses, and would like to directly donate products, you can contact me at positivelyrachel101@gmail.com about what product you have and how we can best go about shipping/picking it up!

Thank you so much for reading through all of that, if you made it! Below are each of the lists I have talked about; please, feel no pressure, it is just an option for those who have expressed an interest in helping out 🙂

Click each link below to go to said list:

Amazon list

Etsy list

A detailed list as well as my paypal account can be found in a separate post, here!

 

Thank you all so much!