First of all: We found a new flat! It was quite difficult to find something which we could finance and Bo would accept and where Bo would be accepted. We are very happy and excited now. I am laying in my bed in my new room which is packed with books and notes, clothes and stuff and things everywhere. I would love to organise everything, but my body doesn’t allow it. Wouldn’t it be great if Mary Poppin’s spells would work in real world?
Herzlich lieben Dank an alle, die beim Tragen und Aufbauen geholfen haben! Ihr seid einfach die besten!
A very good friend of mine whom I haven’t seen for ages (except on stage) visited me yesterday. That made me very happy. Lately, I am having so much pain and nausea that I cannot sleep at all and keep mum and Bo awake all night. Hopefully that will get a bit better in the next days.
In the past week nothing exciting happened except that I got the official diagnosis of POTS* and a follow-up appointment with a waiting time of more than two hours for a short consultation where nothing was planned. Bo is helping me to gain some weight.
As I have already mentioned a couple of weeks ago the LRV transposition won’t happen (I am lucky about that as it wouldn’t have solved my problem). The surgeons here in England and in Germany can’t help any further at the moment (except with some pain pain management, which hasn’t work until now either). Any kind of surgery would be too risky for them. However, I won’t be able to tolerate the pain level that much longer and I also can’t take strong opioids for the next years. Thus, I will need a renal autotransplantation. In the US there are several centres that conduct research on it and have collected a lot of experience in ATs in the last years, but especially for patients from abroad you need to pay a lot of money. That’s why I am trying to organise most of the tests here in Germany, such that the costs for the evaluation won’t be that expensive, but the surgery needs still to be paid ($109272 with 30% self-pay discount). I will have another CT (with 3D reconstruction) soon. If this one shows (and later the xray abroad) a clear compression I might not even need the lidocaine testing. Otherwise I was able to find a transplant surgeon who cooperates with the urologists who could do the test here in Germany. I spent several days on calling and emailing university clinics and urology clinics. In the end I luckily got a contact from another patient who is currently at UW Health. Vascular Compression Syndromes are quite complicated and I wanted to explain what exactly this miraculous autotransplantation actually is and why it would help with my symptoms.
An autotransplantation is a process where an organ or a tissue is transplanted from one part to another in the same body. Patients suffering from LPHS** due to Nutcracker Syndrome, or in general people who have severe, long-term kidney pain, benefit from a renal autotransplantation since it breaks the direct connection between the kidney and the nerves and thus stops the pain. Moreover, the body still has two functioning kidneys. That’s why it is preferred to a nephrectomy (removing the affected kidney). Especially people suffering from LPHS can develop bilateral pain and one doesn’t want that they end up in dialysis. The surgeon can decide whether to do it laparoscopically (small incision, with the help of a camera) or via a large midline open surgery. The affected kidney is removed, stored in a special cool solution and then replaced next to the bladder (most often on the right side). The affected ureter is transplanted as well (clue: that is important for the questions in the end!). The left renal vein and artery are kept as long as possible and then anastomosed to the iliac and external iliac veins. The surgery lasts about 5 hours. During the surgery the surgeon will insert a stent in the ureter to make sure that that it stays open after it, but will be removed some weeks later. Recovery will take some time. Many patients fight with the post-op pain, bloating and constipation, nothing compared to the pain before. Follow-ups are important to make sure that everything has worked well. The centre where I would go to wants the patients to be near the hospital for at least a months, follow-ups are after one, three, six and twelve months and then yearly.
There are of course other methods to treat NCS: stenting, transpositions, bypasses etc ., but except the LRV transposition (and also not always!) they don’t have a long-term effect. Any kind of rewiring, elongating or whatever crazy stuff some surgeons do would be too risky for me due to the previous surgeries. That’s why I need to get the AT.
Whether the autotransplantation is the right surgery for the patient must be decided by a multidisciplinary team of transplant surgeons, radiologists, urologists, for NCS vascular surgeons. A lidocaine test can prove this prior the surgery. The LPHS-UW test works as follows. A balloon catheter is inserted in the ureter of the affected side and 0.5% bupivacaine (lidocaine) is injected. It is kept in there for 5 minutes. If the patient is pain free for a few hours it is proven that he would benefit from an AT. Many patients have no pain after the test for several hours up to two days.
Like every surgery it does have some risk, especially if patients underwent other surgeries before. It is a complex surgery. It may be the case that they can’t transplant the kidney back and one has to live with only one kidney. Other organs can be injured, there is the risk of blood clots, infections, stroke and heart attach, bleeding, a blocked bowel afterwards etc.
LPHS pain has been described as the worst pain in medicine. Many patients can’t live a normal life at all, are bed-bound and dependent on strong medications including strong opioids. In fact, they are often labelled as drug addicts.
I can tell from my own experience that this is the worst kind of pain I ever had. Since it has been getting worse and worse I won’t say that this is the worst pain in medicine. Pain doesn’t know any limits. It isn’t like a normal tummy ache, not even comparable to the MALS pain I had before the surgeries (which is described as pain similar to pancreatitis), it is gruelling. It is constantly at a level of 5/6 with frequent pain spikes at 9/10 which can last for many hours. Well, it is basically the same pain as if you pass kidney stones all the time.
- What are the symptoms for LPHS?
- Why can NCS only occur on the left side whilst LPHS can be bilateral?
- Why does Cialis help with LPHS?
Solutions will be posted tomorrow. If someone sends me the right answers, (s)he gets a reward of course. All 3 questions right: Snuggle with Bo, walk Bo, chicory coffee with hemp milk (and one or two other hemp products); 2 questions right: Snuggle Bo, walk Bo; 1 question right: Snuggle Bo. For people abroad I can offer a 15/10/5min Skype tutorial on Feynman diagrams with Prof Bo.
I know that the cost for the surgery is a lot and believe me I do feel very uncomfortable to ask for so much money. I am still totally flabbergasted how many people have donated in the past three months. But I would be grateful if you could share my pages to friends and family to spread awareness and ask for some support. Every even smallest donation would mean a lot. The situation is quite complex, also due to the former surgeries, but if I can somehow convince my insurance to cover the cost or parts of it (I would still need to pay it in advance), I would donate the money as I have previously explained.
Apropos the small-things-accumulate-to-quite-a-lot-topic. Recently I had to think about all the time I have basically lost because of this (excuse the word) shit illnesses and calculated some stuff. An average person spends about a week per year on the loo. I have definitely spent more time on it or next to it than that (I estimate it on at least 3 months in the past three years). I have also spent about 9 months in hospitals in the past three years. I have had more than 200 blood tests (i.e. about 1000 trials to get some blood, so 1000 bruises), more than 50 urine samples, about 30 pregnancy tests and thus a lot of X-rays, I drank more than 25l Moviprep, I have spent in total several weeks on getting to and back from appointments, probably months if I calculate the waiting time. I have spent weeks for consultations, tests, laying on tables for examinations. I spent weeks for researching my conditions, days to explain it to people who simply don’t get it. I have several folders of test results, evaluation and documentations. I have boxes of medications and alternative medicine trials. I have spent days for special food preparation. I have written hundreds of emails and letters to doctors and institutions.
Either I am in too much pain or I try to recover from another pain spike or the meds finally kick in and I somewhere else in dinosaur land. Or I am somewhere in between. Sometimes it hurts more to accept that I can’t do anything else than the actual pain.
I have spent almost 3 years in pain, from which probably at least a tenth was so severe that I wasn’t able to do anything, another tenth where I was just laying down unable to move in pain and watched how life was passing by, maybe a tenth where I actually felt so good that I thought that I was able to live a normal life again. All together I have lost a lot of time I could have used for studying, working, exploring the world, socialising, supporting others, running and sleeping :’). I have learnt how precious time is, accepted (I am still learning tbh) that I simply can’t do anything every now and then and don’t waste it for things I could still waste it for, if that makes sense.
Chronic illness is a part-time job. I can be lucky that I am a scientist and know a bit in biology and medicine, that I have a curious nature and want to understand everything in detail. Researching, investigating, organising. It is a job that doesn’t pay but only costs. In the spider web of doctors from vascular and gastro and pain management and all the little side paths in between I have to sort out the missing communication and links in between. For someone who is already affected by an illness this causes more unnecessary pressure. I am so grateful to have my mum regarding that. She is supermum. We often joke about it, but actually it isn’t funny at all. She is my secretary, nurse, ER doctor, dietician, cook, physio, donkey:(. Not because I am too lazy, but because the pain level and weakness doesn’t allow it or I refuse another ER visit as I am fed up by doctors who tell me that they can’t do anything. The health care systems should be more efficient, more communication and transparency are needed. A better understanding of chronic illness/pain in the general public would be very helpful as well. If someone isn’t able to work or study because of a chronic illness that doesn’t mean the person is on holidays. Wirklich, Mama, du bist einfach die beste. Du machst und machst und machst ohne ein Danke abzuwarten, ohne, dass ich überhaupt nach etwas frage. Du versprichst selten etwas, weil du es einfach direkt machst. Es gibt Leute, die versprechen und reden viel, aber nichts passiert, du redest nicht und machst es direkt. Du schläfst ein, wenn ich einschlafe und wachst auf wenn ich aufwache (also nicht sonderlich viel). Wenn ich all die kleinen Details auflisten würde, wäre ich in ein paar Tagen noch am schreiben. Ich hab dich lieb!
But then I ask myself whether this is truly wasted time? Perhaps this is just an episode (which did become a bit longer) in my life, an active part of a (slow) transition. Just like the caterpillar which needs some time to become a butterfly****. At the moment I am still crawling up a very high tree, slipping, falling down, climbing up again. I am just (slowly) preparing to clap my wings. Little by little. From time to time I have wonderful views on the valley of life. Life can be beautiful. Life is beautiful. I have always wondered whether the butterfly still knows that it was a caterpillar before***.
In the end, I am more concerned to lose myself in all of it.
Thanks a lot to Karina who published one of my posts on her website. She is an author, blogger and journalist who has been through quite a journey due to her chronic illnesses. Writing has helped her a lot to cope with everything. She raises awareness and supports other people with chronic conditions. She is very inspiring. I wish her all the best!
Well, I will try to organise my stuff now.
Well, that was exhausting. I think I will never stop struggling to rest as much as I actually should. Bo says that he had a lovely walk with my mum!
*POTS: Postural orthostatic tachycardia syndrome is a condition that affects the blood flow, it is some form of orthostatic intolerance (i.e. when being upright). Its official definition is an increase in heart rate by at least 30 beats per minute without a change in blood pressure (most of the patients have a rather low bp and when standing up it drops down) if the patients stands up within 10min of standing plus accompanied symptoms. The official diagnosis is via a tilt table test. One is moved from a lying position to a 70 degrees angle which provokes the symptoms. Primary symptoms are fainting, uncomfortable rapid increase in heartbeat (in serious cases this can lead to panic attacks), palpitations, lightheadedness, difficulty breathing, dizziness, chest pain, fast exhaustion up to exercise intolerance, headaches, nausea, brain fog etc. There are many symptoms affecting the digestion causing constipation, abdominal pain etc. as well. However, the cause for POTS is most often some kind of autoimmune disorder such as EDS or vascular compressions, such that it is difficult to know whether the symptoms come form POTS or something else. There are certain triggers one should be aware of such as rapid temperature changes, certain medications, stress. It is advised to eat at least 6g salt per day (more than it’s actually healthy), drink lots, eat small but frequent meals, one should avoid long sitting or standing…
**Loin Pain Hematuria Syndrome: severe, long-lasting around the flank area, back, sides, upper abdomen which can be uni- or bilateral. The pain can radiate into the groin. During my pain spikes it radiates even to my left leg, middle thigh. It is described as stabbing, deep, pulsating, burning or throbbing pain. It is worse when punching the area. For me it is worse as soon as I am upright, walking, standing, sitting, driving in a car (especially with the humps in London). It is defined by additional blood in your urine, which can be macroscopic or microscopic. Many patients have blood only during pain spikes and some none at all. It is caused by chronic stress to your kidneys (such as kidney stones) or NCS. For NCS there is pressure inside the veins which cause the walls of the renal vein to break open which can lead to blood in the urine. It isn’t life threatening (most of the time), buy restricts life extremely. It is also difficult to diagnose since most often the kidney function is normal, blood tests come back normal. Patients have been through odysseys of doctor appointments and hospital stays including frequent ER visits. Due to the extreme level of pain many patients develop depressions and doctors associate the pain to their psychology.
***I think it doesn’t. It may remember things it learnt as a caterpillar, but due to its metamorphosis it probably can’t remember its former state. The body tissue is reorganised to produce the butterfly. But I am not sure. If someone knows the answer, please let me know!
****The study of the structural colouration of the wing scales of Papilionidae, swallowtail butterflies, helped in the construction of more efficient LEDs. They use 2D photonic crystals to improve the extraction of light which would be internally trapped as well as multilayer reflectors to control the direction of its emission.
1. What are the symptoms for LPHS?
Uni- or bilateral flank pain, which may radiate to the groin pain, may be a continuous, intermittent or only occasional, hematuria (micro- or macroscopic, sometimes only during pain spikes), spikes are often accompanied with nausea.
2. Why can NCS only occur on the left side whilst LPHS can be bilateral?
The ‘typical’ NCS is caused by the compression of the left renal vein between the abdominal aorta and the SMA, which can only occur on the left side. The right renal vein can’t be compressed like that. LPHS can be caused by NCS, but also other conditions such as kidney stones which can occur on both sides.
3. Why does Cialis help with LPHS?
Cialis relaxes the smooth muscle cells of the urogenital tract. No matter what the cause for LPHS is, research shows that the affected organ is in the end the ureter and the kidney colic like pain is due to spasms in the ureter.
More information can be found here.