Median Arcuate Ligament Syndrome (MALS) – the exhausting fight to find out the rare cause of your pain when your doctors tell you it's “...all in your head”
by Sarah Clark, 39, Southampton
Twitter: @sarah_clark_84
If you’re told enough times by doctors that “there is nothing physically wrong with you...", where do you draw the line between believing what your doctors tell you, or trusting your own “gut instinct” to find out the real cause of your pain?
It took over 15 years of not being believed to finally get my Hypermobile Ehlers-Danlos Syndrome (hEDS) diagnosis in February 2020 - aged 35 - followed by autism in 2022.
I was previously under a mental health team for 15 years, who misdiagnosed; Emotionally Unstable Personality Disorder (EUPD), and this made my physical problems even less “credible” to doctors for so many years - I simply was not believed, as mental disorders took precedence.
From 2021 I went through various diagnostic tests for “Median Arcuate Ligament Syndrome” (MALS). The median arcuate ligament sits just below the diaphragm and compresses the celiac artery and celiac plexus nerves.
This can cause severe pain after eating, vomiting, diarrhoea, followed by severe constipation and significant weight loss. I stopped eating solid food altogether because my pain after eating was so severe it was better for me not to eat at all.
I lost nine and a half stone in four years.
There are actually overlaps between MALS and the eating disorder Avoidant Restrictive Food Intake Disorder (ARFID) which is characterised by a person avoiding certain food types or limiting the amount of food intake and considered an eating disorder.
Dr Carolina Baeza-Velasco and colleagues, found that a higher proportion of patients with eating disorders meet the criteria for Joint Hypermobility Syndrome or EDS, beyond the prevalence of eating disorders within the general population. However, due to symptom overlap and diagnostic overshadowing, eating disorders can be misdiagnosed in those with EDS.
Dr Gillian Harris, from ARFID Awareness UK, points out ‘Given the reported co-morbidity between ARFID and autism and EDS (as well as comorbidities such as MALS), perhaps the usual presenting symptoms of ARFID should be described here. ARFID can be seen in young children from the age of 2 years and at this age, food is usually refused on sight; the food will not even reach the mouth. Few foods are always accepted and new foods are always refused. This leads to a very limited-range diet and is always linked to sensory processing difficulties. However, a slightly different presentation involves the ‘fear of aversive consequences’ when eating foods; usually of vomiting or choking. This presentation might sometimes include discomfort on eating, as does any eating disorder where food intake has been limited; but not extreme pain. There will always be an underlying trigger for this latter type of ARFID, e.g. extreme anxiety on moving schools, with some induced sensory reactivity. Some foods or liquids can be taken, however, without any discomfort once a feeding regime has been established, and this is where AFRID is distinguishable from the food avoidance seen in MALS’.
In MALS food avoidance is caused by the severe pain after eating.
The MALS prevalence rate is 2 in every 100,000 people, and can be very challenging to diagnose because most healthcare professionals simply lack knowledge of this condition. I was left using a bowel irrigation device 3-5 hours per day for over three years and was told my gut motility issues were linked to my childhood trauma so I needed to get the mental health team to help me.
Disbelief
The combination of not being believed that my pain was real, the severity of the pain itself, and the trauma of the daily trans-anal irrigation left me fighting for my life in a coma, in intensive care, after being resuscitated because I tried to end my life in January 2021.
However, If I had been believed that my stomach pains were real, and more importantly - helped, this near-death experience could certainly have been avoided.
This iatrogenic harm was caused by my doctors not believing me and refusing to write the referrals I asked them to write for tests I was prepared to pay for privately – I was told I didn’t need the tests as they told me nothing was wrong
The diagnostic celiac plexus nerve block I had in March this year, gave me such significant pain relief that I could eat 3 food meals a day again for a few months. But the nerve block has worn off now, so I have been back on a liquid diet for almost five months. It’s a very long, hard fight to get any further treatment, and at times I’m too unwell to chase up all the hospital teams I'm under, so I am left ‘managing’ the best I can until I find the strength to persevere to get any further tests or treatment.
I’m very proud to say however that I despite so many challenges, I recently got a distinction for my MSc dissertation researching “What are the daily experiences of autistic people living with hEDS?”.
The important message here is my determination, and how proud I am of what I'm managing to achieve despite severe challenges and I'm glad I've found the brilliant support and information from SEDSConnective.
My overall message – is that sometimes you need to be persistent, believe in yourself, and believe in your “gut instinct” and never give up hope.
References:
National Associate of Rare Diseases (accessed online August 2023) ‘Median arcuate ligament syndrome’
ARFID AWARENESS UK (Accessed online August 2023) ‘What is ARFID?’ URL:
https://www.arfidawarenessuk.org/what-is-arfid
Baeza-Velasco, C., Seneque, M., Courtet, P., Olié, É., Chatenet, C., Espinoza, P., Dorard, G., & Guillaume, S. (2022). Joint Hypermobility and Clinical Correlates in a Group of Patients With Eating Disorders. Frontiers in psychiatry, 12, 803614. https://doi.org/10.3389/fpsyt.2021.80
Professor Thomas Scholbach (Accessed online August 2023) “Weakness of gastric and intestinal peristalsis” URL: https://scholbach.de/weakness-of-gastric-and-intestinal-peristalsis