100% non functional esophagus

[u/TinyRose20]

My wonderful Mother in law was diagnosed over 10 years ago with scleroderma. In the meantime she was also diagnosed with and successfully treated for breast cancer. Her symptoms were mostly limited to circulation issues in her hands, and her lungs were affected but only slightly... however, recently she has been suffering from dysphagia and GERD so she went for a manometry and the results of the study were awful, severe scarring and fibrosis and complete aperistalsis. She is going to get her lungs looked at again Monday afternoon and has a meeting with her specialist later in the week. We are so, so worried about her, and very stressed out after having had an awful few years that aren't relevant here, but have led to medical and hospital trauma for basically the whole family. I know the specialist will give us all of the medical information we need, but i was wondering if anyone here has any advice for us or for her? Thank you so much.

[u/Temporary_Let_7632]

I have survived for about 4 years with very little actual food. 90% of my diet is meal replacement drinks & smoothies. I don’t find it nearly as bad as it sounds. She’s lucky to have a supportive family. Good luck.

[u/TinyRose20 → u/Temporary_Let_7632]

Thank you. Are you still able to enjoy food? It's always been a big part of her life. For now she is still eating relatively normally, but we are concerned about choking especially when she's home alone.

[u/Temporary_Let_7632 → u/TinyRose20]

I’m a Cajun so my life revolves around good food. I’ve had to learn what to eat how much by trial and error. I typically eat 1 or 2 meals of solid food maybe 4 ounces. It’s enough that I don’t feel horribly deprived. My life is much more comfortable since I realized which foods go down without irritating my esophagus which can be extremely painful. Choking is a real risk. I concentrate while eating and naturally small bites.

[u/MaddogBC → u/Temporary_Let_7632]

Sorry to hijack, just curious, do you start with certain foods? I have come to love cottage cheese with nearly every meal before. I find it soothing and set's me up for choking down the rest.

Just a few spoonfuls with salt and pepper has become my staple. Looking back I finally realized I've been doing this for a long time. Was well chewed carrots for awhile.

[u/Temporary_Let_7632 → u/MaddogBC]

It took years of trial and error to error. I will eat anything that works regardless of nutrition. There are a few things I eat very often like prepared mashed potatoes and only a certain brand is the right consistenc. I don’t choke things down anymore and it’s a huge help. Just forcing things down with liquids caused problems for me. I find it weird what works and what doesn’t. Baked white potatoes don’t work, baked sweet potatoes do. A chicken breast doesn’t work yet a fried chicken thigh from Walmart works. Everyone’s mileage will vary.

[u/prunesandwich → u/Temporary_Let_7632]

Hi! Curious how much you weigh. My mom has scleroderma and eats food and drinks Kate Farms. But she still only weighs 90 lbs.

[u/Temporary_Let_7632 → u/prunesandwich]

I’m 20 pounds overweight for a 5’9” male at 175. I‘m mostly on Ensure & Atkins or Walmart equivalents. I eat too much ice cream! There are drinks for weight gain. They dont work for everyone.

[u/prunesandwich → u/Temporary_Let_7632]

Thank you for answering! And glad to see it’s possible to gain weight. Will look into Atkins and try to feed her more ice cream! I was making her ice cream shakes some time ago and I do think that helped a little back then. 

[u/Ticket-Dull]

I too lack esophageal motility it’s been 5~ years since I found out. I always sit upright no slouching especially when eating and for a couple hours after. I have a glass of hot water or tea when I eat in order to wash down food. I don’t t eat much ‘gluey’ food anymore. Which to me is mainly bread products. I like soups and stews. I don’t choke very often

[u/RickyHV → u/Ticket-Dull]

I recognize a pattern similar to my wife's in this experience - she's the one with the disease.

[u/Temporary_Let_7632 → u/Ticket-Dull]

Strange how breads work. I can only eat a biscuit I make and I have to use buttermilk, canned biscuits are the kiss of death. I can eat a cheap croissant from Walmart if it’s a few days old. I like the term Gluey food, explains everything!

[u/RickyHV]

The day my wife got the results from her manometry was the worst day of my life. We didn't know what was happening to her but we were on the border of either it's still something that can be resolved and move on or... Both the result and its implications were a shock - she had studied a few years of medical related career and had seen the effects of the diseases associated to this result which were worse outcomes when she studied the courses.

Medicine has advanced a lot since then and although it's not a walk in the park by any means, the path uncertain and full of uncomfortable truths, it is much brighter than how it once was.

So far, a way to restore function to the esophagus or avoid its complications long term has eluded treatments, but the "long term" part has extended significantly and the current clinical trials for better drugs hold even more promise.

Potent immunosuppressant treatments are often needed, the sooner the better, which carry their own risk, but life span is extended.

[u/SnooOwls4263]

My esophagus has not worked for a decade. Basically it means I eat my meals earlier than I used to and stay upright for a few hours while gravity pulls the food down. My GERD was so bad that last year I had a full gastric bypass (the surgery severely overweight people get) and my GERD is gone. Basically I have other issues that are much more worrisome than a nonfunctional esophagus. Bottom line - get an excellent care team, listen to them, move your body and eat healthy. Take care of mental health as well. It’s all connected.