Our one chance at life changing surgery abroad.

Please help me raise money for my younger sister, Kira.

Kira has been suffering with 2 rare illnesses for over 9 years now.

These conditions are –

• Median Arcuate Ligament Syndrome (MALS)

- A major artery of the aorta that delivers blood to the stomach, liver, and other organs and nerves in the area is being compressed.

https://rarediseases.org/rare-diseases/median-arcuate-ligament-syndrome/

• Nutcracker syndrome (NCS)

- Left renal (kidney) vein is being compressed.

https://radiopaedia.org/articles/nutcracker-syndrome?lang=gb

Both these conditions cause unbearable symptoms and pain. Diagnosis and treatment are often delayed and not well established because it is so rare which is what has happened to Kira.

If the surgery is not done correctly Kira would need more surgery which could end up being stents or a bypass. If the compression is left untreated for too long it can also lead to other conditions due to the lack of blood flow which are irreversible.

(When Kira was a toddler, she was chubby and enjoyed her food. As she started to get older, eating food started to become painful which has now become so severe she has been hospitalized on many occasions while having numerous tests and procedures to figure out what was causing her pain)

As of now Kira has constant stomach and chest pain with debilitating gastro issues. This gets worse with eating and exercise and she now takes tramadol daily to cope. She has lost 2 stone in the space of a year and is always exhausted. If she eats too much she is doubled over in pain and having to increase her medication. She has now become too scared to eat or go too far from her house in case her pain becomes too much. This has had a huge impact on not only her health but her social life which has become less and less over the years as she has tried to hide her illness and how much it affects her from those outside of her family. She can never be spontaneous and has missed out on many events and holidays. It has come to the point now wherever she goes she has to plan what she eats or not eat at all, know there is a toilet within a short distance and medication always at the ready in her bag. These are just a few of the things many of us don’t have to think about in our daily life yet for Kira it has now become second nature. It would be a dream for Kira to be able to eat a full meal with her family without pain, without us feeling guilty for eating in front of her and to be able to enjoy life once again.

When it all started

In 2012 Kira started to become very ill and in 2013 an investigative laparoscopy was undertaken which ended with her being rushed into hospital two days later with SEPSIS and C.DIFF.

Kira was unable to eat and had to be tube fed, this was touch and go whether Kira would actually pull through. She was on multiple antibiotics, steroids, painkillers and morphine which altogether resulted in Kira being in hospital for 1 month until she was discharged with a liquid diet to take home.

By this time, Kira was still very ill and suffering with severe stomach pains and was referred to the gastro team and pain management where they tried her on a range of different medications. She has had many invasive and painful tests with some even being lost by the hospital, again delaying help and diagnosis.

Over the next few years with going back and forth to her GP, A&E and consultants, Kira was first diagnosed with Crohns disease. With a dietitian in place Kira’s diet was limited and this past year has now come to the point where she can only eat fish, rice and potato which still cause severe pain even when taking tramadol.

Kira went back to her doctor’s multiple times and was constantly dismissed being told that it was no longer Crohns disease but was now IBS, stress related and all in her head. Taking matters in to our own hands, we started researching and paid for private tests and appointments with specialists. A Doppler Ultrasound showed MALS and Nutcracker Syndrome and due to NHS waiting times and lack of support and information, we paid to travel to Germany to see a well-known specialist in these compressions named Prof Scholbach.

This image taken from the scan in Germany shows how compressed Kira’s vein is. The arrow pointing to the black space shows the open part of her vein with blood flow, whereas the circle shows how narrow and compressed the vein has become. Her report quotes “very often no blood flow at all crosses the compression site can be found thus pointing to a frequently complete obstruction.”

He also states “very often no blood flow at all within the tightly compressed origin of the coeliac trunk can be found.”

The results confirmed MALS and Nutcracker syndrome as well as;

• Pelvic Congestion Syndrome

- Since the blood from Kira’s renal vein cannot get through properly the blood finds a different pathway leading to the blood pooling in her pelvis causing chronic pelvic pain as well as other symptoms. https://www.cedars-sinai.org/health-library/diseases-and-conditions/p/pelvic-congestion-syndrome.html

• May Thurner Syndrome

- This is where the left iliac vein in your pelvis is narrowed because of pressure from the right iliac artery.

https://www.webmd.com/dvt/may-thurner-syndrome

Even though the imaging from the same scan in the UK show these same compressions they are still dismissing Kira’s symptoms and are not taking any action. A recent hospital phone call again showed they do not understand the compression syndromes and do not listen to Kira as they are still trying to label her with IBS.

The UK doctors have even confirmed that she has made a new vein as her body is trying to make a new pathway for her blood to pass through. This is now taking its toll on Kira. We are glad that we now know the cause after so many years of unexplained symptoms and pain, however the NHS in the UK does not want to move forward with any surgery or even confirm the diagnosis even when imaging and Kira’s symptoms say different. With Kira’s symptoms getting worse day by day and her having lost two stone in less than a year, we feel that it is time to take matters into our own hands yet again.

Through support groups and research, we found a specialist in America named Dr Hsu who has undertaken over 500 surgeries for MALS which has a 90% success rate. Kira sent her scans to Dr Hsu and had a video consultation with him to which he also confirmed the compressions. He sent a referral letter for Kira to have a Celiac Plexus Block and if she was pain free after the procedure it would confirm MALS even further and that surgery would help. Kira gave this letter to her doctors, yet they refused the procedure. Yet again we had to source a pain management doctor of our own and pay out nearly £2,000 to have the block. It involves 2 long needles going through the back and into the compressed artery, numbing the nerves. To our relief the block worked for one whole week. She was able to eat things she hasn’t eaten in years without pain or any of her other debilitating symptoms. We set up another video appointment with Dr Hsu who has been more helpful and understanding than any of the past doctors Kira has had and understands her pain is real.

He has offered Kira MALS surgery to which we have accepted with a November date to be scheduled if all goes to plan. However, the cost of the operation as well as hospital fees, flights, accommodation, etc will come to over £40,000. With the help of any donations Kira would be able to get the life changing surgery in America to treat her MALS.

A news story showing a patient who was treated by Dr Hsu who had the life changing surgery.

https://www.youtube.com/watch?v=IqvA7qkX8tk

For Nutcracker Syndrome, Kira is looking into kidney auto transplant or donating her kidney, which would also help change someone else’s life who has a life-threatening condition. These 2 options seem to be the best treatment moving forward and with the best success rate. Once Nutcracker syndrome is treated this should also aid the Pelvic Congestion and May Thurner Syndrome. On top of this Kira also has severe pectus excavatum, which has now been taken off the NHS in England as it is seen as a cosmetic procedure yet is still available in Wales, Scotland and Northern Ireland.

Her consultant Dr. Kolvekar is trying to get this treatment back on the NHS and has told Kira it is affecting her health and surgery would help her. Please see a link to the petition below.

https://petition.parliament.uk/petitions/329161

This surgery needed is called the NUSS procedure which involves having a metal bar placed in the chest to push out the compression. https://kolvekarpractice.co.uk/chest-wall-surgery-and-pectus-deformity/

Scans have shown that the compression in Kira’s chest is only 4 cm away from her spine causing pressure on her heart and lungs explaining her chest pain and breathlessness. At school Kira use to be very active and sporty yet now she gets out of breath from climbing a flight of stairs. She has also developed long lungs from the pressure as she cannot exhale enough air on every breath. Yet again, this means that we will have to self-fund to have this done.

We feel Kira has been let down by the health system and her doctors by dismissing her for over 9 years suggesting it is all in her head and to get on with it. In reality they should have listened and taken the time to discover she has 2 rare and painful conditions.

All through these years of physical pain and emotional distress Kira has tried to remain positive and completed college and passed her University degree with a First often dragging herself dosed up on medication to make tutorials, lectures and at times completing work from her hospital bed.

Each day Kira is getting worse and we are worried and concerned that if she does not get the necessary surgery soon her health will continue to deteriorate and lead to more health issues and poorer quality of life.

Any money donated would be very much appreciated and help in a life changing surgery for Kira where we hope she can get her life back. We will keep you updated with any new updates and developments,