TW: The following article references self-harm and suicide.
Q: Tell us about you.
What health conditions do you have and how have they affected your life?
A: My name is Siân, I am 29 years old, and I live in London where I am currently a 3rd-year medical student.
In 2010, when I was just 15 years old, I suddenly developed a severe and constant headache, unlike anything I had ever experienced. From the moment of onset, the pain was 24/7 and it lasted nearly 8 years.
In 2011 I was diagnosed with New Daily Persistent Headache, as well as hypermobile Ehlers-Danlos syndrome (hEDS) and Postural Orthostatic Tachycardia Syndrome (POTS), giving me not only a diagnosis for my constant headache but also explaining the joint pain and fainting I had been suffering with since a young age. I was collapsing multiple times a day by the age of 16 and had a resting heart rate of 120 bpm (for context, 60-100 bpm is considered average. The lower your heart rate the better your heart function).
I spent the next few years trialling different medications, injections, and infusions. Unfortunately, most of these treatments came with pretty severe side effects, and none of them helped my pain.
In 2017, I developed severe gastrointestinal symptoms, including constant nausea, and abdominal and pelvic pain. Eating and drinking would exacerbate both my pain and nausea to the point where I became completely unable to eat. Very quickly I became dangerously underweight and malnourished, which caused me to become bed bound and reliant on a wheelchair to go out and attend my appointments.
Doctors were unable to figure out the cause of these new symptoms despite extensive testing. Whilst bed bound, I decided to take matters into my own hands and began searching through medical literature to try and figure out the cause of my symptoms. After extensive research, I felt I knew what I likely had and suggested it to my doctors. By this time I had been admitted and put on TPN due to the severity of my symptoms and inability to maintain oral intake of both food and water. I am grateful they took me seriously, and soon after they arranged the appropriate tests and I was diagnosed with superior mesenteric artery syndrome, renal nutcracker syndrome and pelvic congestion syndrome.
I went on to have a gastrojejunostomy and left renal vein transposition, with a long saphenous vein graft.
Surgery was a success, and I was able to resume eating 4 days post-op! Miraculously it also fixed my 8-year-long constant headache, before this the medical profession was unaware that renal nutcracker syndrome could cause a constant headache.
The surgery was life-changing and, inspired by my journey and the doctors and surgeons who changed my life, I then went on to do an Access to Medicine course to be able to apply to medical school.
I moved to London and, after passing my access course, started medical school in 2019. However in 2020, after a bike ride, I developed sharp stabbing pains in my abdomen and within 24 hours my symptoms of renal nutcracker syndrome had returned. Scans showed the graft that they had made during my previous surgery had occluded, so I then had a left ovarian vein transposition to bypass this. Unfortunately, this surgery did not relieve my symptoms, so I decided to undergo a nephrectomy in 2022 (kidney removal) to permanently solve the issue.
Due to the nature of renal nutcracker syndrome, I was also able to donate my kidney to someone with kidney failure, essentially solving both my problem and theirs. Surgery for both me and my recipient was successful and I recently returned to medical school to finish my 3rd year.
Hypermobile Ehlers Danlos Syndrome (hEDS)
(Information from NHS UK) Ehlers-Danlos syndromes (EDS) are a group of rare inherited conditions that affect connective tissue. There are 13 types of EDS, most of which are rare. Hypermobile EDS (hEDS) is the most common type. People with hEDS may have: joint hypermobility, loose, unstable joints that dislocate easily joint pain and clicking joints.
Postural Orthostatic Tachcardia Syndrome (POTS)
(Information from NHS UK) Postural tachycardia syndrome, also known as postural orthostatic tachycardia syndrome (PoTS), is a condition that causes an abnormal increase in your heart rate after sitting up or standing up. The most common symptoms are feeling lightheaded or dizzy, palpitations (being aware of your heartbeat) and fatigue.
Superior Mesenteric Artery Syndrome (SMAS)
(Information from National Institutes of Health) Superior mesenteric artery syndrome (SMAS) is a digestive condition that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery).
(Information from National Institutes of Health) Renal nutcracker syndrome (NCS) is a condition that occurs when the left renal vein (the vein that carries blood purified by the left kidney) becomes compressed.
Pelvic Congestion Syndrome (PCS)
(Information from the National Institutes of Health) Pelvic congestion syndrome (PCS) is a common cause of chronic pelvic pain in women of reproductive age. Pain that is intermittent or constant lasting for 3-6 months, present in the pelvic or abdominal region, occurring throughout the menstrual cycle, and without any association with pregnancy is chronic pelvic pain.
Total Parenteral Nutrition (TPN)
(Information from the National Institutes of Health) Total parenteral nutrition is a medication used to manage and treat malnourishment. Total parenteral nutrition is indicated when there is impaired gastrointestinal function and contraindications to enteral nutrition. Total parenteral nutrition (TPN) is when the IV administered nutrition is the only source of nutrition the patient is receiving.
Q: What has been your biggest challenge, or hardest thing you have had to come to terms with, since your diagnosis?
My challenges have varied throughout the years. For example, before my life-changing surgery in 2017, just getting through each day was a challenge. My headache had a huge impact on my life and eventually, my symptoms became so severe that I was bed bound. In 2012, I became very depressed and started self-harming.
At this time I was in 6th form, but struggling to keep up with school. The pain was so severe and the side effects from the many medications I was on had turned me into a complete zombie. I made 2 suicide attempts in the space of a couple of days, and the second time, I ended up in ICU and very nearly didn’t make it.
To make things more complicated, I was living in Dubai at a time when suicide is illegal (attempts are illegal and if you die there are consequences for your family/sponsor). I narrowly escaped also being arrested. After this, my school would not let me return and I was forced to drop out.
In 2013, I moved back to the UK with my mum. I was still very suicidal and it was felt that it was too risky for me to stay where my care for both my physical and mental health was very poor, as well as the illegality of suicide. My care was transferred to the NHS now that I was permanently living in the UK. But this didn’t change the fact that I had exhausted all of the treatment options that were available for my condition. There was still very little hope of recovery, or even finding some relief for my symptoms.
Moving forward to today - Unfortunately, about a month after my last surgery I developed another constant headache. However, this felt completely different to the one that I had dealt with for 8 years as a result of my nutcracker syndrome. The type of pain, severity and location are all different. I was re-diagnosed with New Daily Persistent Headache (which was what I was misdiagnosed with all those years ago!).
So currently, my biggest challenge is my new headache and continuing to study despite it. Also having PTSD due to everything I have been through, which adds a layer of complexity to navigating medical school as well.
Q: Despite your challenges, what keeps you going and brings you joy on difficult days?
This year I have taken up pottery, which I love! I find it very relaxing and after a busy week at medical school, it’s a great escape for me from the pain and stress.
I love surprising my friends and family with my creations. Recently I gifted a mug I made to my GP, who has been a huge support to me over the last few years, and who is sadly leaving the practice. It is so lovely to see someone be so excited to receive something I made.
I am also a very active person - I love cycling, swimming, and Tae Kwon Do! I also have two dogs, Bailey and Clover, who are my world and when I go back home to visit my mum I love taking them for walks in the forest. I had to stop all of these activities when I became unwell, but after extensive physio to help me get my strength back after all of my surgeries and associated symptoms, I am hopeful to be able to get back to exercising again soon!
Q: What is an achievement you're most proud of since being diagnosed?
My biggest achievement is getting into medical school. Its something I never thought would ever be possible for me, sometimes I still have to pinch myself about it because it feels so surreal!
Nobody in my life ever thought I would be well enough to go back into education or work. I never thought I’d know what it would be like to live without a headache or constant pain, but less than 2 years after my life-changing surgery for I was accepted into medical school in London. All of my hard work paid off.
I am now in my third year and I am so proud of myself for everything I have accomplished.
I was recently asked by one of my doctors, who helped me when I was at my lowest with my health, to give a presentation at one of his multidisciplinary team meetings on interesting cases. I presented my case and patient experience and received really good feedback, with senior doctors saying they had learned a lot and would never forget my case.
It still feels strange to see my doctors, who played such a crucial part in my recovery, see me as a colleague now. It reminds me of how far I have come.
Q5: What are your hopes for the future, especially when you graduate and become a doctor?
I hope that I can graduate from medical school and become a doctor who specialises in treating patients like myself. I hope that my experience of living with several chronic conditions will help me be understanding and supportive to patients, someone that people can rely on when they are desperate because I understand how overwhelmingly difficult it can be.
I know I am very privileged to be this well, and to have had the care that I have had (though it hasn’t always been good), but the conditions I have are often neglected by the medical profession, both in terms of research and the care that patients receive.
I want to help change this.