Median Arcuate Ligament Syndrome (MALS)

I remember a young man with Chron’s disease who had major difficulty maintaining weight. Also, after years of not eating regularly, he seemed to lack interest in the act of eating. He received tube feeding through the night. This way he could maintain a decent nutrional status. Maybe hospice care is not aggressive enough to improve his caloric intake. Also, at 73, the uncle could still have decent years ahead. I’m 74 and have spent too many years being miserable. I’m finally ok for now, but it is true that the medical world is less aggressive with older patients. I had to fight for myself.

@kariulrich Thank you for the warm welcome and quick response, I am very touched that you are reaching our personally, this must be a lot for you to manage.

Please be patient with me, I am very new to a lot of these terms, and am playing catch up to understand what stenosis is, or how invasive a TPN might be - I will respond to those points when I have a chance to talk to Lou (he is resting now).

Lou's story is very sad. Tracking back, we determined that his MALS may have started back in 1999, and went misdiagnosed and untreated for so long. The ABQ medical system has really failed him in this regard. Over the years he has seen many internal gastro specialists, but due to health care limitations he did NOT have a Primary Care Physician that was tracking his health deterioration over time.

In 2017, my parents (his older sister) flew him to MA to be seen at Mass General, and he was officially diagnosed with MALs. After that things swung into effect quickly, but keep in mind that he had been living with MALS for almost 20 years(!!!) at this point.

+ May 24, 2017 -- First diagnosed by Dr. Said at Mass. General
+ July 13, 2017 -- Surgery done at University of New Mexico Hospital. Cancer oncologist Dr. Nir and vascular surgeon Dr Rana. - Celiac artery release and cordiozation. Partial removal of celiac nerve ganglia.
+ Aug 4, 2017 -- Follow up complication 2 weeks later, Dr, Rana. Pseudoaneurysm of splenic artery. Surgery- Splenic embolization.
+ May 15 and June 20, 2018 Nerve blocks done at Albuquerque Pain Management Clinic. Anesthesiologist Dr. Whalen.

@racheldmark welcome to our humble group, happy you are advocating on behalf of your uncle. Have they considered giving him tube feedings or TPN to help his nutrional status and help with energy? Does he have any additional diagnoses? I am trying to understand why hospice and not a more conservative approach to nutrition and pain? Small meal, liquid based that are nutrient dense such as ensure is needed if he can tolerate that. MALS should not get to this point, I wonder if there is more going on...if he has stenosis again???

My name is Rachel, and I am writing on behalf of my Uncle Lou, from his home in Albuquerque, NM.

Lou has been fighting a long and difficult battle with MALS. He was finally diagnosed in 2017, after years of struggle and misdiagnosis. In 2018 he underwent the procedure to correct the pinched nerve and ligament, and remove part of the Celiac nerve ganglia. This successfully restored blood flow to his stomach, but did not help ease his pain. Over the last year, he has undergone two additional treatments of nerve blocker injections through the ABQ pain clinic.

Unfortunately none of these treatments helped and the pain has persisted, and even increased over time. He continues to suffer painfully when he eats, from what I understand is classified as severe and therapy‐resistant neuropathic pain.

Like many of you, after surgery the doctors have said that there is nothing else that they can do for him, and the ABQ medical system is very poor.

A few months ago he was briefly hospitalized. The emergency room physician who treated him told him that in her opinion his undernutrition was so severe that he had only months to live.

Currently, Lou is living at home and receiving hospice care, with in home care throughout the day. The services are extensive, and have helped improved his quality of life tremendously, and include many of the things he has been asking his doctors for and not receiving. Better pain and anxiety medications, hospital bed, wheelchair, an on call nurse and 24 hour hospice access, medical alert system, personal grooming, home cleaning services, and on going support.

He is 6'2", 73 years old, and currently weighs 102 lbs. We have him on a regimented diet of Resource 2.0 in addition to one to two small meals throughout the day, but have not been able to get him to consume more than 1,600 calories in a day.

We are very lonely with our struggles to help him, and I have found considerable comfort in reading through this forum over the last few days.

Questions:
* Are there other people out there who are struggling after unsuccessful treatment that we can connect with - even just for solidarity?
* What are your most successful meal plans for maintaining weight?
* Are you on IV fluids, and/or GJ feeding tubes? (These will be hard for us to get right now because he is on hospice, but if there is hope we would like to know)

Dear Mark (@ukmalsman),
I have been reading your posts and have found them so useful. Would you be willing to share what your meal plan looks like for the week? Already I fear I may have been overfeeding Lou sold meals, and want to switch to a liquid diet as you suggested, but I don't know where to start.
Thank you for all that you have already contributed, I am just beginning to dig in to this forum and the MALS PALS on facebook and I feel better equipped than before.

Thank you all

@kellylinn nice to meet you! Let us know how your celiac testing goes! What are you having done today? Welcome to Mayo Connect it is wonderful to have you hear, great photo! I have not gotten very good at all the filter settings! Love!!!

Hello my name is Kelly and a year and a half ago I was diagnosed with a dissection of my SMA and Celiac ligament syndrome. I had bypass surgery for my SMA that took 9hrs & 47min. And this morning I am going back up to OHSU for more testing now to address my Celiac.

@redhead63 No, my surgeries did not fail at all...they were both a success. I need to explain further...I have a vascular disease called Fibromuscular Dysplasia or FMD, on top of my MALS diagnoses. My other diagnoses include Ehlers-Danlos Syndrome, brain aneurysms. My first surgery was a complete success... I had open surgery that involved a bypass of the celiac artery with a Dacron graft... after the ligament was released my velocities stayed elevated so the surgeon did the bypass...it was also determined through intraopertative ultrasound my hepatic/splenic artery had stenosis that was not picked up on imaging so I had a bovine patch angioplasty. This surgery was a complete success, I gained weight, had energy and no pain....for three years. My pain did return, but not because the surgery was not successful. During my second work up all test showed my arteries where good...but I started to loose weight, could not eat due to pain, my quality of life deteriorated to the point it was decided to do an exploratory surgery (It is very rare to have this done now, due to the technology advances in imaging) but we could not find the cause of the pain so my vascular surgeon opted to do it. My vascular disease (FMD) there is little known about it... it can occur in any of layers of the artery, it causes abnormal growth of the artery...and depending on which layer will determine type (Some types of FMD are more aggressive than others) Sure enough...when my vascular surgeon looked directly at my artery with ultrasound there was intimal tissue that had grown inside the graft. Not expected but the source of pain was found, and revision of my graft was done. Had I not had an underlying vascular disease this complication probably would not have happened. After the second surgery I was symptom free again for many years. Unfortunately the pain has returned, but I cannot keep having surgeries to investigate my graft. I am working on managing the pain currently. This will be a chronic condition for me, like others. Each of us are different, and success is in the eyes of the patient. In my eyes, both my surgeries were a complete success and I am appreciative of the compassion my vascular doctor displayed for me. Because of his skills and training I was able to have a many pain free years, and I know I will always have his support and guidance in the future. ❤️

@techchick1025 So happy to hear you got the help you needed, especially in your complicated case. I know it is difficult when you have a diagnosis of fibromyalgia and when you see a doctor it beomes easy to blame all your symptoms on fibro. I had a similar experience in my early twenties when I had symptoms of joint pain, every doctor I saw thought my pain was due to fibromyalgia. When I lost weight I was told they suspected I had an eating disorder, they thought stress and anxiety was why blood pressure was high at a young age... but no one took the time to put all the pieces together. Finally after several decades I went to Mayo and a wonderful cardiologist took a step back and put the peices of the puzzle together, a rare vascular disease. It was too late at that point to cure my hypertension, however I will say Mayo for me has saved my life. Diagnosis in a complex patient is not easy. Once I had my diagnosis it still was not black and white... there where many more diagnoses to add. Healthcare is dynamic and so is the knowledge. I can appreciate what you have gone through, especially the travel for healthcare ..it is expensive, exhaustive and can have a negative impact on over all health. Hopefully in sharing our experiences we can help each other navigate the good and the bad aspects...especially in the MALS community. I believe strongly that more support and care is needed, especially aftercare of complex patients like us. May I ask what did your local doctors do differently that made a difference to you? For MALS, finding a GI doctor that will look at a vascular cause is one area we need more awareness...so many of us are sent to GI,which is needed but when all of our GI work up comes back normal the investigation stops. Vascular disease can be invisible... especially for those under the age of 70-80, not something commonly thought of in young and middle aged adults.

I am trying to catch up on posts, so I really appreciate you letting me know about the Nov 4th post! Look forward to reading it.... love my carbs....guessing that is what you are referring to. My cookie based diet worked wonders for helping maintain some weight...now I am into Red Bull energy drink for lunch, little pain with that.

I made 4 long trips to Mayo and got absolutely no where with any single problem I have and there are many. They focused only in fibromyalgia which I’ve had for 25 years. Suggested 3 week pain management class costing thousands and completely ignored the incidental finding of avascular necrosis or my immune system and connective disease issues. I found all new fairly local DRs that are on the ball now. I look forward to getting the proper treatment and attention from DRs that actually listen and see the big picture.