Too Many Obstructions***** Everywhere

Pseudo-obstruction or mechanical obstruction, that is the question

Dear all, 

Merry belated Christmas. I hope you had some relaxing days full of love, joy, laughter and light. May the new year bring (improved) health, bright hours, calmness, beautiful moments and happiness. Never forget our interconnectedness, we are all part of the universe’s flow. Even in the darkest times it can fill us with hope. Hope turned into strength. Strength turned into energy. Energy turned into new hope. 

The following post is from the past months and does not present the past weeks which have been very hard.

Thank you for your cards and presents. It made me very happy and despite the pain, the screaming and crying, the vomiting and nausea it made me smile. Thank you for your encouraging words in the darkest moments. I often feel like a major burden but reading that I have helped people lifts my spirit again!

Thank you!

Please also have a look at my wonderful and strong friend’s words. She desperatly needs financial help for diagnostics to find some treatment options. If you could donate on her gofundme, I would be very grateful.

Christmas is a very sensible time. Whether you also spent it in hospital (due to Covid, visitors are not allowed – I will write about this next time, healing also needs your loved ones) all alone, in pain (physical or mental), isolated or with your family, better or worse than prior Christmas, full of hope or lack of it, … 

Christmas reminds us of our childhood. Failed Christmas cookie dough. Cozy evenings. Pulling out Christmas decorations. Putting up stars standing on the window seat. The magic of togetherness. Little surprises. Sweet melodies. Warming up. The scent of clementines, fresh pine, woody, warming spices. Christkind’s bell softly ringing. The little things. Home. A feeling of comfort and safety – that’s what I have been missing. What are your childhood memories?

I can be so lucky that I had such a wonderful childhood. Christmas awakes in me a child from long ago. It reminds me of the deep gratitude inside me that can light up the darkest hours. And it reminds me of letting go. Letting go of things that won’t ever come back.

I am so sorry for the lack of updates, so much has happened and it is impossible to give a clear picture of what we have been through in the past months. I am in a very bad state. It takes an enormous amount of energy to write this, but I know that people are following my updates and care. The following post has been written since autumn, so it might not make much sense in some parts.

I have experienced so much cruelty in the past months. It has been the most difficult time so far. I have been crying and screaming. Losing hope. Losing perspective. Untreatable nausea, untreatable pain, untreatable vomiting, untreatable ileus states. Every single day. Doctors at a loss. Isolated. Hours that were survived only.

But I have also been filled with deep gratitude and love. Your support, your words, your thoughts mean a lot to me.

In the midst of winter, I found there was, within me, an invincible summer. And that makes me happy. For it says that no matter how hard the world pushes against me, within me, there’s something stronger – something better, pushing right back.

Albert Camus

I have mentioned this quote several times. It’s one to put on your coffee mug. This winter has been very hard. Camus taught us about this feeling of uneasiness. He does not talk about hope, he rejects hope. I can only guess why. There is something inside me, with no hope left, that still is resilient. Something that revolts from within. I don’t know what it is. In winter, life seems to be absent, but She is hiding only. She sleeps to bloom again.

Almond trees* bloom in Winter. They bloom beautifully and purely. 

It is an endless battle. I won’t overcome it, but I will go through it.

It storms outside. The view to the ceiling is empty.

Winter. Recently I read ‘Spellbound’ by Emily Bronte. It perfectly describes the inability to move away from the situation, where you cannot escape from. Accept it. Nature is unpredictable and strong. But beware the last line ‘I will not go’. What holds us?

Summer 2021

Summer 2021. Months of chronic paralytic (sub)ileus states despite all measures. Full neurogastro workup: enteric neuropathy, pseudo-obstruction, myenteric hypoganglionosis in the colon, colonic inertia. Frequent decompressions and endoscopic emptying. Total parenteral nutrition. It was necessary to insert an ileostomy i.e. an opening of the abdomen where the small intestine is diverted outside such that digestive waste can be collected in a bag and does not need to go through the colon (you can read more about it in the last two posts and below in the section on decompression). Due to the pre-surgeries it was again done via the midline incision. Post-op worried us a lot since we had to combat a major (pseudo-)obstruction flare up. Along with all measures we managed it. ‘Colon problem’ solved. Unfortunately, things got continuously worse the following weeks and months. 

First, the small intestine dysmotility has been that bad that the ileostomy did not bring any relief. Ongoing subileus states, nausea and pain, very irregular ostomy output despite meds. Then, things got a lot worse with major pain spikes that did not improve under the usual pain management strategies from the past years.

Autumn 2021

In the following I will paste and copy extracts from November until now.

Autumn 2021

Despite monster pain and serpent nausea [everything is relative…] mum drove us to the forest nearby such that I could smell some fresh air today.

In onion layers to combat my weird temperature sensations and the weather. I had a dance with Franklin as well.

*

I haven’t been doing well. 9/10 pain from the pseudo-obstructions [and mechanical ones as we would find out] and constant nausea that both seem to be unaffected by all kinds of meds put me under massive exhaustion.

Tomorrow I am getting admitted on the palliative ward. We hope to find some kind of pain treatment and we are going to start the immunosuppressive therapy.

In between I was transported for medical diagnostics to Aachen where an autonomic neuropathy and a small fibre neuropathy was diagnosed – further information will follow.

I was diagnosed with a severe form of autonomic neuropathy (that’s the issue for the orthostatic intolerance and fainting, intolerance to heat due to the inability to sweat, temperature insensitivity in the peripheral nerves etc). We are not yet sure whether it is associated with the enteric neuropathy in the intestines tho.

Currently, we are waiting for further analyses, but it seems that the enteric neuropathy is indeed congenital and the autonomic dysfunction rather acquired.

*

For the latter the planned therapy might be able to help, but for the pseudo-obstructions there are no treatments left anymore other than treating the acute states with decompression, TPN, meds and symptom management which leaves me in a very poor state.

[little did I know…but there is indeed an option].

*

We will see how things go. I am very grateful to be on the ward tomorrow. I don’t need to manage everything on my own anymore [little did I know!] and hopefully, there will be some kind of improvement in symptoms soon. They are all very nice and I will have my own room. Definitely need some rest! And my mum needs rest, too. So win-win.  

*

I apologise for the lack of replies. I have been writing the following in steps in the very few times I have been able to.

moving towards early December

*

Things have developed quite quickly and severely.

In the past months, both the pseudo-obstructions and the pain have increased. We assumed that the pain is due to the abnormal contractions/enteric neuropathy. Sometimes it can become very difficult to distinguish pseudo-obstructions from mechanically caused problems.

Two weeks ago I was admitted to palliative care. We started looking for a pain management treatment that might reduce the pain. From an epidural, to all kinds of meds, a morphine pump and so on, the pain developed untouched to acute episodes – probably the worst kind of pain I have ever had. We know that there is something wrong with the drug absorption i.e. some drugs don’t have an effect at all and others cause very weird reactions. At the same time we have to make sure not to trigger the small intestine too much. After a lot of chaos that needed further treatment, in the end, only sedation and for now strong pain medication every few hours helps to reduce the pain for a bit.

*

At the same time we were looking for the cause for such a severe development and it turned out that there is a major mechanical problem in the small intestine (aside of the pseudo-obstruction) which needs surgical intervention. Moreover, I developed a fistula between the small and large intestines [turned out I didn’t, the mystery still hasn’t been solved]. The reason for the ileostomy was to circumfer the need for decompression and emptying the (mega)colon. Well, this hasn’t worked out. During the surgery they will need to go along the whole small intestine to find the narrowing/twisting and whatever surprises my intestine has produced/developed due to the pre surgeries and enteric neuropathy. They need to be dealt with.

Further, they might need to insert a new ileostomy.

In total, this will be another (risky) surgery with several probable complications during and afterwards.

Unsurprisingly, my body is fully exhausted from the severe pain that seems to be untouched, nausea and vomiting.

*

Another three weeks inpatient stay where doctors and nurses are totally overwhelmed with my body. It reacts so differently than a normal patient does. Today they tried inserting a duodenal tube and despite careful application clipping, my small intestine managed it to push it out within a short time. Hence, they had to cancel the hydro MRI which would have given us some more answers aside from the real time x-ray imaging and two CTs. But no day goes as planned. Sometimes, I have the feeling that some type of Pandora’s box was opened in 2016 when symptoms started and the interventions, especially the first major surgery, has caused an outflow of monstrous problems since then (aside of some apparent congenital issue that has developed so quietly until it bursted out a year ago).

*

I don’t have a choice, I will need to undergo the surgery since we need to have a look inside what’s going on.

I look forward to getting rid of the current acute pain from the (hopefully!) mechanical issue, but I am very well aware of the complications and risks involved, both short- and long-term. And the possibility that they might not find a mechanical issue sinde it may be imitated by the pseudo-obstruction only. Abdominal surgeries in pseudo-obstruction should be kept to a minimum [yes, they do!]. We don’t know what the rest of my small intestine will do after the surgery. I am scared [should have even been more scared than that], first as we can’t properly plan what to do prior the surgery, the surgeons have to cut open and ‘take a walk’ along the whole intestine i.e. also nearby the double transposed and patched SMA and through an abdominal area in which we had 6 major surgeries and many small ones and an intestine which has been in constant (sub)ileus states for the past year and went through two major ileus in the past years. I am especially scared since I don’t feel ready at all. [as were the doctors and nurses]

I am so exhausted and tired from the past weeks lacking rest, perspective (both personal and health). Despite TPN I am still severely underweight and haven’t gained any weight.

I am scared.

*

Unfortunately, due to this development I am not able to continue my studies. It makes me very sad looking back at the past weeks where I almost had no energy or concentration to do any maths or physics or do anything at all. And it breaks my heart that I had to make this decision. [And now it breaks my heart even more as this was not a temporary measurement.]

It hurts to see my mum watching me  surviving, suffering and crying through each day.

I am very grateful for the support on the palliative care ward, but as the doctors and nurses made it clear, their hands are tied. It hurts to listen when the nurses admit that they are overwhelmed and hopeless with the situation as well since they cannot do anything to help.

Will it ever get easier?

I hope things go well and that I get some rest afterwards. I would like to spend Christmas at home, I miss Bo’s cuddles and my legs don’t have space left for more morning bruises.

(little did I know…)

Thank you so much to the people that try to emphasise and help. I really appreciate it and I am very touched by your words.

*

a lot of crying and screaming – a lot of vomiting (bile and digestive content, your intestine always produces it and in obstructions the small intestine cannot move it forward i.e. it collects until there is so much pressure that it finally is vomited) – pain – pain meds – reactions – good luck teddy – more reactions – beautiful scar cut open the ??? time

Surgery Surprise

*

Unfortunately, things were more complicated and severe than we thought. They went in laparoscopically and immediately decided to open up the whole scar again with two other incisions. The surgeons had to remove a tonne of adhesions of the small intestine from the SMA area to the lower pelvis (my megacolon has shifted my small intestine into the pelvis). Especially here it was tangled and knotted up, one bowel loop was twisted at 180° under adhesions and basically stenosed. The whole intestine didn’t move at all, but after the interventions it moved a tiny bit [for the last time so far]. They also had to insert a new/higher ileostomy.

The colon is dead (from apoptosis, so no single movement at all anymore), so they cleaned it in surgery to remove the skybala manually (sorry for the details). 

Then they flushed the whole intestine, there is still blood and fluids coming out, but the drainage should be removed in 5 days or so.

Say hi to Pupsi number 2, he looks far better than before! I thought to share how it looks as people don’t necessarily understand what an ostomy is (it is indeed part of your intestine having some fresh air)

*

The post op pain has been a major difficulty, vomiting and circulatory issues as expected and manageable. But the burning, hot, deep pain in the whole abdomen hasn’t been manageable. There are a lot of meds I can’t take because of the pseudo-obstruction, because of heavy reactions or they don’t show any effect, so it is difficult. In the end, I am just laying and curling up in pain screaming and moaning and praying for help. I hope those post op days end soon and my mum and me will finally have some rest. There are sufficiently other problems left.

Thank you for your support, your words and thoughts, they help us through this time.

*

Sympathicolysis via an epidural after surgery has two advantages, first it reduces the pain in the abdominal area and secondly, it can improve intestinal motility. It works similar to the already mentioned drugs that increase the work of the parasympathicus as it temporarily fully blocks sympathetic innervation. Depending on its spinal position it numbs different abdominal areas.

It can also be done long-term via denervation, elimination of ganglions or ganglion blocks (e.g. celiac plexus block) under CT guidance.

Another indication for a long-term sympathicolysis aside of motility and pain reduction (also as last option in severe CRPS) is circulatory improvement (the sympathicus narrows vessels, so blocking it, will dilate them) of the peripheral blood system.

However, such interventions have major risks and shouldn’t be done long-term without clear indication. Classically, it is often done is terminal cancer patients with therapy resitant pain. The temporary plexus block is also used as diagnostic tool in MALS.

*

I am just so exhausted. No sleep at all, pain from surgery and additional issues that have arisen, non-stop vomiting, nausea …

The pseudo-obstruction is causing most of the problems. I might not have the acute pain from before the surgery due to the stenosed loop, but motility hasn’t improved. Tonight I was at the edge of another ileus. And by now it has become so difficult to distinguish between mechanical and paralytic ones.

Reactions (to whatever) have gotten worse and transformed me into some kind of red Christmas Monster with snowballs all over the body. Fire in the inside and fire on the outside.

I have prepared a lot of small things for everyone who supports us so much, I hope you understand that things will arrive in the new year [or summer]. I appreciate your thoughts and wishes so much.

Last night I broke down in pain (during ileus there is just not much one could do than waiting though it), kept screaming and passing out from pain. I think it is only natural, but I heard myself whispering that I cannot go on anymore, that I do not want to go anymore, I begged for help. I am a very rationally thinking person , I am not sure to whom I am talking to in those hours. If only one could push life on pause , just for SOME relief.

I can be so lucky that I have always been a very disciplined person that understood the beauty of endurance.

*

One week post op: pure horror, no notes.

My body doesn’t want to give up, that’s why I am still alive. Am I alive? I am surviving.

Crying. Screaming for my life.

Vomiting stool. Untreatable nausea.

*

Transport to Freiburg for stabilising.

I was transported with the same ambulance car that also drove me down to Bavaria for the neurogastro diagnostics and they were totally shocked to see me in such a state. The issue is with a paralytic small intestine ileus you feel like shit, literally! Nausea builds up enourmsly due to the digestive fluids and stool production in the intestine that sits there for hours. As much as it cannot transport it forward the phases of amotility and for- and backward movements make it equally difficult to vomit. Hence, hours are spent in severest nausea until 1l+ are vomited, deep down from the small intestine.

In Freiburg we started with mission motility stimulation (which would seem impossible due to the weeks of ileus ahead) and proper (!) pain management. It is a proven fact that any surgery makes the intestinal pseudo-obstruction worse (or any other motility disorder). Along with the progressing of the condition itself, my GI system has reacted more and longer after each surgery and went into a full paralytic ileus. I will give a summary of stimulation options soon. We also tried a cortisone pulse therapy which, unfortunately, was not succesfull. I explained previously, that aside of the a/hypo/dysganglionosis (i.e. complete nerve cell loss, reduced number of nerve cells and ‘ill’ nerve cells) and the loss of innervation of the cells left that are in different stages of apoptosis both causing enteric neuropathy; the atrophy of smooth muscle cells causing myopathic components as well as other structural problems, there is also a ganglionitis in the enteric nervous system. A ganglionitis is an inflammation of the nerve cells/nerve ganglion (also outside the ENS, for example zoster where the inflamed ganglion spreads towards the skin) most often due to viral infections. The ganglionitis can be primary, causing the nerve cell loss (or accelerating it), most often due to some autoimmune component (often you can check for certain antibodies e.g. onconeural ones which I will elaborate on in the future as well) or secondary as an infiltration due to the damages of the ENS. Cortisone should help in the first case to reduce symptoms and slow down nerve cell loss. Unfortunately, it did not help in improving motility or with symptoms for me. The pathologists did come to the right conclusion that my ganglionitis is a secondary infiltration.

However, the cortisone treatment did help with the dysautonomia symptoms such as dizziness from the orthostatic hypotension. I will go more into detail about the ANS, dysautonomia, its causes and treatment options in the future, too.

Birthday – Christmas – New Year 2021

Spending birthday, Christmas, New Year at the clinic with strict Covid-no visitor-rules after a very traumatic stay in a Wald and Wiesen Krankenhaus, equally traumatic (in fact very similar: post op care without concept, no proper pain management, a surgeon who wasn’t available, discharge with an ileus…) as in 2017, is not the most wonderful thing I would have wished for. However, I am so grateful that Freiburg managed it yet again to stabilise me. I was so happy to be at a safe place. From experience I was prepared and decorated my bed in stars and Christmas lights.

Bodywise, there was no Christmas miracle this time. In the better hours I tried to mobilise (to faint) or read (the same page over and over again) or listen to audiobooks (no idea about what). Origami meditation and wishes to you did help me throughout the day as much as your wishes encouraged me.

I was especially happy when the nurse came with two wonderful bunches of flowers. One with 25 strong shining flowers and a dreamy hidden-meadow-in-the-forest type one, together with a Catweazle-Einstein bear that would keep my company the coming weeks. Qweazle joined us in saying ‘Nothing works!’.

January 2022

The new year continued with extensive trials of intestinal stimulation (my legs wouldn’t recover from the injections). I will get more into detail next time which options there are. Along with NG tube in and out (it never ever drains for me as the content is accumulating in the small intestine i.e. I still vomit whereas NJ tube just never ever stay in due to the intestinal chaotic movements my intestine pushed it out within minutes), prucaloprids (increasing peristalsis) and other crushed pills like pyridostigmine (improving smooth muscle movements) I got many, many injections of neostigmine (which indirectly stimulates nicotinic and muscarinic receptors due to the increase of acetylcholine – you will understand more in the next post; all you need to know is that people with a normal digestive system will have a bowel movement within a short time) and relistor (for opioid induced decrease in motility since I was on some low doses of opioids).

Slowly we got some improvement in the massive pseudo-obstruction flare up meaning I was able to not curl up in screams all day vomiting and crying in pain. Well, the stimulation (when it worked) did cause severe pain spikes since my intestines cannot move properly and the stimulation didn’t ‘tell’ them how to move, it rather increases total peristalsis – but I got through them and my neighbours got through my screams as well.

In my opinion, nausea should be classified as a worse issue than vomiting itself. Especially, in pseudo-obstruction flare ups where the nausea is caused due to the stagnation inside the small intestine it will take many, many hours until the intestine either moves digestive content (even if you don’t eat) a little bit forward or decides to have some reverse movement. So you will have hours in extreme nausea in which you simply cannot do anything whereas the act of vomiting takes a few minutes and the nausea subsides.

Reading ‘The Grass so little has to do’ by Emily Dickinson. Life could be simpler. We should keep moving… where? In the vast foam of uncertainty, in the beautiful mi/acrocosmos.

There are so many reasons why we would like to be a hay. Interestingly, in the poem, she wishes she were ‘a’ hay and not ‘the’ hay. Life can be beautiful and life can be simple. The simpler, the more beautiful. Right? The grass wants to transform. As little as it can do, it can grow. And it can transform. It can follow nature’s flow. In parts at its own pace, in parts according to her heart beats. There is some spark of transcendentalism. 

This reminds me of some things. Heidegger’s transcendental pain about which he talked several times during his lectures in Freiburg. Being understood as transcendence. There is no proper evaluation of him about pain, but here are some thoughts in order. For him pain arises from the fact that we are part of this world, our interaction with the world, the openness. We belong to the world. Pain arises, but here it is not the sensation he is talking about. As the environment forces itself onto us, the impossibility of closeness, pain is caused.

His treatment is partly based on Hegel’s combined Phenomenology of Spirit (development of consciousness) and his Science of Logic (being emerges as essence and essence emerges as concept = thought that consciously reflects on itself, from objective to subjective logic, parousia = the Absolute to itself). He talks about the pain of the Absolute. As long as the Absolute hasn’t reached self-comprehension pain exists (this process of self-realisation might also be Heidegger’s view of the meaning of life). He mentions Trakl’s ‘A Winter’s Evening’ since here the interaction between nature and objects are presented whose root lies in pain. His use of dichotomies might show that everything belongs to something.

Now have a look at Renoir’s ‘Chemin montant dans les hautes herbes’ (1876/77) and ‘Girl Stretched out on the Grass’ (1890) as well as Van Gogh’s ‘Patch of grass’ (1887).

Daydreaming, fond memories, lying in the grass, resting from a long run, time stands still. Beautiful life. Blooming. Wonders. Gratitude.

Whether grass or hay, both spread the same sweetness, both give the same comfort. Both give the same feeling of home. Romanticism.

Some serious medical information follows. 

In the end stage of severe dysmotility such as in a chronic intestinal pseudo-obstruction state there are not many treatment options left. If the small intestine cannot be stimulated with the help of motility increasing measurements and stays in pseudo-obstruction i.e. (sub)ileus states, further surgical options are possible/might be necessary. 

In general, paralytic ileus states are treated conservatively (in some cases, surgery is needed). Aside from the motility increasing meds mentioned in the last two posts, first one can apply stronger meds such as neostigmine which stimulates nicotinic and muscarinic receptors by enabling impulses due to an increase of acetylcholine. Acetylcholine is a neurotransmitter of the skeletal muscular system as well as in parasympathetic (digestion!) and preganglionic sympathetic processes. Obviously, all kind of stimulating and laxatives measures should be applied (see last post for more information) first.

Acute flare ups of pseudo-obstructions (which can last for hours, days or weeks or can become chronic) should be treated just like an acute paralytic ileus.

Nasogastric decompression is the next step i.e. with an NG tube one can decompress the stomach to help with the nausea and vomiting (obviously, if it cannot go through the small intestine it moves upwards and vomiting up stool isn’t the yummiest thing). Colon decompression in case of megacolons are done via colonoscopy. Similarly, in Cipo in some patients may benefit from an NG (tube to the stomach via the nose) or NJ (tube to the upper small intestine) or for longer use a PEG or PEJ (percutaneous, see last post for more information) such that one can continuously or intermittently decompress the stomach or the upper small intestine, respectively. One can use them for venting, both for fluids and gas and the application of oral drugs and flushing of the intestines. This may improve upper abdominal distention and pain, too. For frequent treatment it is less invasive and painful to use percutaneous systems. Those can be used at home such that the patients don’t need to stay in the clinics every time such states occur.**

Next, to reduce the amount of waste in the intestines, eating and drinking (if even possible) is stopped. The intestines produce their own digestive fluids and stool (and quite an amount). 

Via the tubes one can also apply contrast agent that can help pushing forward digestive content or one can use them for flushing. 

Antiemetics and pain medication that can both slow down motility should be carefully decided on. Unfortunately, often one can only stand it through.

Unfortunately, the small intestine is quite long and at some point those measurements won’t help anymore.

It might be helpful to reduce the length of the small intestine e.g. by inserting an ileostomy and if already present an ileostomy higher up. There are also cases where parts of the small intestine are resected i.e. the whole length is shortened to reduce the risk of pseudo-obstructions and the transit length for waste to go through. Those kind of surgeries contain quite a lot of long term risks as the small intestine is a very complex system that is important for digestion, absorption (of nutrients, water, electrolytes from the lumen into the blood) and many more processes. Without the colon, fluid and electrolyte losses can already be present, but with a shortened small intestine serious problems can occur. The small intestine can hold up to 10L of fluids (just from GI secretions!). If the small intestine is shortened one tries to minimise the problem of malabsorption by carefully deciding which segments to resect. Ideally, one should try to keep the ileum. One should keep the enterohepatic circulation (see last post). The terminal ileum re-absorbes a good part of the bile into the bloodstream back towards the liver. The duodenum is mainly responsible for the break-down of food without which absorption and resorption is not possible. Pancreatic and bile ducts are given into the duodenum Thus, the surgeons will try to resect ‘something in between’ (i.e. from the jejunum) and the two parts of the small intestine are then anastomosed.

In summary, this may reduce the risk of pseudo-obstructions and with this the pain, nausea and vomiting. However, new problems will arise and patients may stay/become dependent on TPN (definitely for small intestines shorter than 1m). Moreover, patients like me have malabsorption (even if I’d eat I do not absorb it, I also don’t absorb any pills which makes treatment difficult) anyway, so shortening the intestine will increase this problem. If TPN is not possible due to lack of vascular access, correlated liver issues, recurrent sepsis, patients die from malnutrition. It can also cause gallstones (due to lack of reabsorption of bile which decreases the concentration of bilic acid) and kidney stones (due to increased oxalate absorption). Both of which can cause further issues and the need for more surgeries. If the ileocecal valve is removed intestinal bacterial overgrowth can occur which increases the issue of malabsorption even more. If the terminal ileum is gone vitamine B12 cannot be absorbed anymore and needs to be substituted.

Problems include:

• Dehydration 
• Serious electrolyte dysbalances (e.g. loss of potassium with damaging heart issues)
• Diarrhea (not necessarily in dysmotility patients)
• Lack of enzymes, malabsorption, food intolerances; weight loss
• Additional other organ issues
• TPN related issues
• Lack of vitamins

Intestinal failure (IF) = state with insufficient digestion and/or absorption of nutrients, fluids and electrolytes that leads to malnutrition and/or dehydration. Common conditions that lead to IF are:

• Severe dysmotility such as in Cipo or hollow visceral neuropathy and/or myopathy 
• Short bowel syndrome (either congenital or due to surgeries/other underlying conditions such as Crohn’s, ischemia, volvulus, gastroschisis)
• Mucosal illnesses such as microvillous atrophy or epithelial dysplasia
• Fibrosis or severe adhesions
• Tumors (that require the resection of large parts of the small intestine)

Another alternative for irreversible IF is a small intestinal transplant which is very rare (there are about 100 per year worldwide) and complicated surgery only done at a few specialised centres. During this surgery parts or the whole small intestine is transplanted (sometimes also in combination with other organs such as the liver after TPN related problems) to combat irreversible intestinal failure that cannot be treated in another way (e.g. end stage CIPO, short bowel syndrome***, impossibility of TPN and hence weight loss). The difficulty of the surgery, short and long term complications are highly dependent on the underlying illness. On average, if the transplant is successful, 90% survive the first year and 70% survive the three following years. There are official guidelines with indications and contraindications which have to be evaluated appropriately (English and German).

A small intestinal transplant is indicated when the illness is progressive and/or irreversible as well as endangers the life of the patient or significantly reduces the quality of life

and

when the transplant solves/improves the issue. Following Jeppesen prior to surgical interventions for intestinal insufficiency and intestinal failure (for SBS) one should try all available options that could improve intestinal rehabilitation***.

Contraindications are

• Co-existing illnesses that cannot be treated and lead to death
• Severe damages or illnesses of other organs (that cannot be transplanted as well)
• Sever infectional diseases 
• Difficulties that are likely to occur during the surgery e.g. due to pre-surgeries or anatomies

After successful assessment the patients will be put on the transplant list and wait until the organ allocation is successful and the patient is fit for surgery. The patient has to be available at all times, as soon as a small intestine is available the patient will be contacted. It is very important that the patient arrives at the clinic as soon as possible (which does not mean that the transplantation will definitely take place since the surgeons have to evaluate the small intestine first). Any examinations that are needed (and can be done prior) should be done before listing the patient to make the process as smooth as possible.

The transplant itself is complicated as all abdominal vessels need to be reconnected (mainly the SMA, IMA and the celiac artery, but also the tinier vessels). The small intestine needs space. Previous surgeries, scar tissue, abnormal anatomies can make things a bit more complicated. To get more space one might need to intervene further e.g. with a transplantation of the abdominal wall.

In any case an ileostomy will be inserted (whether the patient still has a functioning colon or not, if it works it is temporary only). 

There is no standard protocol since the surgery highly depends on the intestine and the abdominal space of the recipient i.e. pre-surgeries and underlying conditions play an important role. It takes at least 8 hours, often longer. The small intestine has to be transplanted within 6 hours (only if you want to see an open abdomen). 

After the transplant the patient needs close monitoring on the ICU, the nutritional and immunosuppressive therapy is started. Although the organ is correctly allocated (blood group and other factors) the body realises that the organ is new. Immunosuppressive drugs help in a way that the patient’s immune system recognises foreign substances less. Hence, the patient will also be more susceptible to bacteria, infections etc. It is important to find the right balance between isolation and protection from both the rejection reaction and possible problems due to the decreased immune system. 

The main complication is the rejection of the new intestine and related issues to the decreased immune system. In the past years those have been reduced significantly due to immunosuppressive therapy, post op care and surgical techniques themselves (for accurate surgical notes and immunosuppressive therapy have a look here). In the late 1980s when the first intestinal transplants where conducted the 1-year survival was 20% or less!

Patients’ lives suffering from severe functional GI disorders or short bowel syndrome can be saved with the help of such a transplant. About 80% of the TPN dependent patients are able to eat again if the surgery succeeds. Prior the surgery, TPN dependence is a major indication as its survival rates do depend on the underlying illness, the 1 year survival for Crohn’s is above 90% , but below 50% for cystic fibrosis due to TPN complications.

If such a surgery is decided on most of the patients will have undergone many other interventions and have a very poor quality of life and/or are about to die. 

Please think about organ donation, with the right documentation you might be able to save a life (or even more).

Also, if you suffer from severe intestinal insufficiency or from a severe motility disorder and a small intestine transplant might be an option, don’t get refferred to a transplant centre too late. The process of evaluation and allocation is very long (not to mention that there are far more people waitining for a small intestine than transplants available per year – in Germany, there are about 30 people waiting and every year there are 3-6 transplants!) and the patient shouldn’t be in a very unstable state prior the transplant. This decreases the success rate and increases the risk of the need for a multivisceral transplant.

Palliative care is another option, in some cases the only option. More on this in my next post as well.

Endurance is limited. Is it? How to endure? Physical pain. Mental struggle.

Indeed, according to science, human endurance is limited. However, we can improve our endurance with learning, reflecting and exercising. Our body can find ways to reduce expenditure of energy in difficult times. With constant continuous struggle on the body, it can manage to lose increasingly less calories per day. At some point it saturates, studies on marathon runners have shown that it does at about 2.5 times of the runner’s resting metabolic rate.

We can exceed it well above for short times, but obviously not for longer durations. That’s what intensity and time is about. 

The resting metabolic rate is some quite interesting quantity. You won’t be able to calculate it. Take me as an example, despite full TPN and lots of calories I don’t gain any weight, still bobbing around under 40kg. It doesn’t matter whether I am able to mobilise and exercise or whether I am bed-bound, my body does not want to go above it. Doctors don’t know where the nutrition ends up. There are probably small small black holes inside of me. Jokes aside, there are some interesting things to mention.

The resting metabolic rate (RMR) and the basal metabolic rate (BMR) both measure the energy of your body needed to function. The BMR measures the absolute minimum requirements such as circulation, digestion, breathing and temperature stability; the RMR measures the energy needed for minimum activity such as minimal movement and eating based homeostasis. 

Obviously, age, gender, size and genetics (can use the Harris- Benedict equation) play an important role, but so do a lot of other factors that are difficult to quantify.

• Muscle mass since here metabolism takes place
• Body composition in general 
• Changes in body fluids

More accurately is the proper testing in a lab where both can be evaluated under formal conditions and a certain protocol and the use of indirect calorimeters. The patient is isolated in a chamber with a constant supply of ventilation.  Most of the energy expenditure can be calculated from the rate of oxygen utilisation. I think those tests should be done more often for patients suffering from underlying/co metabolic conditions to understand more about their energy needs. A BIA (bioelectrical impedance analysis) measurement might be helpful as well. With the help of a device, which can measure the electric impedance of a current applied through the body, one can non-invasively evaluate the patient’s body composition. Impedance, Z, is nothing else than resistance, R, and reactance, X, (it is a complex number: Z = R + jX) from which parameters can be calculated.

• R -> BCM (body cell mass):  cell mass that metabolises i.e oxygen consumption, carbon dioxide production and energy expenditure -> nutritional status (low in malnutrition)
• X -> ECM (extra cellular mass): extracellular fluids -> nutritional and physical status (high in oedema, for example)
• BCM + ECM = FFM (fat-free mass) & FM (fat mass) = weight – FFM
• Phase angle in deg = X/R * 180 degrees/pi: cell membrane efficiency -> training and nutritional status (low in poor status, the higher the more trained)

This can now all beautifully be put into a neat diagram.

Also, a blue whale’s tongue (which has the weight of an elephant) sips down about 7 tonnes of food each day, so about a thirtieth of his weight.

As a former runner endurance –

‘the ability to endure an unpleasant or difficult process or situation without giving way’ (Oxford dictionary) -

might be uncomfortable.

Add a little bit of discipline. And growth begins.

Indeed, both are accumulative. It might give the illusion of a standstill. 

There hasn’t been any improvement in the past days, weeks, (…). But time is relative. Things may improve if we just wait a little bit longer. Provided waiting is still an option. And we can grow the growth with experience, learning and continuous working. They will all add up to the accumulative process.

In any way, it is a process (unpleasant and/or difficult or more) that has some meaning.

We live because we love.

Walk as if you are kissing the Earth with your feet.

Thich Nhat Hanh

Saudade (Autumn 2021)

Just some Autumn thoughts from the past weeks on the short walks through the rain of falling leaves. Autumn may be a reminder of the cold ahead, but with the summer warmth at your back.

The red, copper and amber leaves are just like the green ones. They have done their amazing job. Now they will nourish the earth. 

It is a smooth passing from an apparent end to a new beginning. 

The leaves aren’t afraid of falling. They fall gracefully and peacefully.

Every leaf is the tree itself. We should be aware of the constant and continuous interactions. Take nature as an example. She shows us how letting go can be beautiful. Why are we so attached? Acceptance can be made so easy. 

And we should be aware of the constant change, the only constant. As the seasons come in cycles, our mind and body should flow up the helix of life.

Feel the beauty above and feel the beauty below. Feel the harmony all around you.

And as we keep walking we should mindfully imprint our paths. 

Every thought, every action, every word – every step has to be chosen with care. Each expiration goes radially out, to become one with the wind that carries those leaves and each inspiration will nourish you.

Breathe out your fears and let them turn into (an) energetic inspiration. And peaceful serenity will fill your body.

There should be no grief. The summer has ended, the cold winter is awaiting around the corner. But the warmth of your inner summer sets all grief aside. Allow yourself to be nostalgic and wistful, for the things you have lost. Value them, but let their weight drop until they vanish into the Autumn air. Reminisce. Saudade.

Experience this state of mind.

Weltschmerz.

With each step show your gratitude and Earth will be grateful as well.  

Beginning and end united. 

You will be home. Home mists in the morning and home with an earlier sunset.

Harmony. Calmness. Awareness. 

Also: The Flaming Lips/My Cosmic Autumn Rebellion, Paper Aeroplanes/Winter never comes, The Tallest Man on Earth/Little River, Autumn Rhythm, Autumn by Frederic Edwin Church, Indian Summer by William Trost Richards.

More Thoughts in Between .

Recommendations

Random songs

• Dot Allison e.g. Constellations, Can You Hear Nature Thing, Heart-Shaped Scars
• Skullcrusher e.g. Prefer
• The Idyll Opus (I-VI), Adjy
• Manifold, Dekker
• Wonder, Lomelda
• Brightside, The Lumineers (whole recent album)
• The Wandering Hearts: Dolores, Dreams, Never Too Late, On Our Way; Run
• Lean On Me, Imaginary Future 
• Dragon New Warm Mountain I Believe In You, Big Thief: Sparrow, Change, Time Escaping, The Only Place
• Changes, David Bowie 
• Survivor, Tim Baker
• Constant Tomorrowland and Hug From A Dinosaur, Torres
• The doll, Astro Tough
• Big Moon Light, Vanishing Twin

Next time: book recommendations, from Buddhist philosophy to Rovelli’s relational quantum theory

Best songs and albums for the new year

• The New Year, Death Cab for Cutie
• Auld Lang Syne and Daydreamer, Roo Panes
• New Year’s Resolution, Camera Obscura
• Better Days, Goo Goo Dolls
• Let’s Start The New Year Right, Bing Crosby
• Better Days, Guy Clark
• Funky New Year, The Eagles 
• Next Year, Foo Fighters 
• Better Day, Ocean Colour Scene
• Little Light, Amos Lee
• Things Are Great, Band of Horses
• Give Me The Future, Bastille – No Bad Days
• LP3, Hippo Campus
• A Change Is Gonna Come, Sam Cooke
• Chimacum Rain, Linda Perhacs

Albums to listen to in the next three months

• 11.2 Big Thief, Dragon New Warm Mountain I Believe In You (whole album release) – Carousel
• 18.2 Metronomy, Small World & Beach House, Once Twice Melody
• 22.2 Alt-J, The Dream
• 4.3 Stereophonics, Oochya!
• 25.3 Placebo, Never Let Me Go
• 8.4 Calexico, El Mirador
• 13.5 Bear’s Den, Blue Hours

I am not doing well. Since the last surgery the pseudo-obstructions have worsened a lot. Currently, we are investigating the cause for the severe pain in the lower part of the intestine just behind the new ileostomy. It seems to be mechanically caused . However, it is very difficult to distinguish between Pseudo-obstructions and mechanical obstructions. Especially, if they both cause each other. Surgery is contra-indicated as long as it is not necessary (i.e. life-threatening obstructions, severe ischemia etc). Hence, we need to find a solution that combats both issues and their symptoms meaning severe pain, nausea and vomiting. Both motility stimulating drugs and ‘standard’ pain management are not sufficient, the doctors have to cooperate (whereas my body does not seem to cooperate anymore). Since the oral absorption is basically non-existent we started a full parenteral treatment only (where it is possible, some GI drugs need to get through the intestine and some are going ok in high doses and crushed). The evaluation regarding a small intestine transplant is on-going. Due to the pre-surgeries and the complexity of my conditions it is not easy to evaluate its level of success and possibility. I will update further soon.

As one doctor recently said it is such a rare condition (plus the accumulation of other conditions and many major interventions in the past years), that it is impossible to find any clinic or any studies that would give us help in taking the right decisions. Or another doctor that calls it ‘tragic’ and leaves…

*Also one of Camus’ essays I recommend reading. There is only the sword and the mind, the latter wins. Almond trees survive the hardest winters and so can humans survive as well. ‘Such is the character that in the winter of the world will prepare the fruit.’ He talks from his own experience from Algier. ‘Before the vastness of the undertaking, let no one forget strength of character.’

**5.4 The Good Doctor episode shows a young girl with visceral myopathy and just before her whole colon and small intestine are resected they turn to a PEG for venting and meds for motility which obviously is far less invasive.

***Here I mean short bowel syndrome and functional intestinal failure due to the lack of absorption and/or dysmotility. Short bowel syndrome can be congenital or due to trauma, mesenteric ischemia, cancer, CEDs, multiple surgeries, volvulus etc.

Here you can find the S3 guidelines regarding intestinal failure in Germany by the DGEM which mainly focuses on SBS.

Here you can find a keylecture of chronic intestinal failure and the resolution via transplantation (in German).

****Intestinal rehabilitation is basically a multidisciplinary program (surgeons, gastroenterologists, in motility issues also neurogastro specialists/neurologists, specialised nurses, dieticians, psychologists for emotional support and specialists for all co-occuring conditions e.g. interventional radiologists, pain specialists, immunologists…) that should help to ‘rehab’ the digestive function in long-term and often complex GI issues and/or to improve quality of life and longer survival. It is very important that those patients are closely followed. There should be good communication between the departments and competent support. Unfortunately, there are not many centres and often their capacity is quite low. Here you can find out more information from a pioneer clinic in the UK.

*****Obstruction from obstruere – blocking, building a barrier, from ob (in front of, in the way of) and struere (building). When there are obstructions in front of us, we try to find ways around them. Sometimes we are able to go through them, to combat them. Sometimes we find alternative ways. Sometimes it turns out that tha apparent obstructions are new possibilities. But please tell me sir, what do you do if there are chronic obstructions within you, if you are obstructed yourself? That’s Cipo.

My babies

And for Shakespeare lovers:

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I would never give up. Freiburg is the place I feel comfort and safety. I know that the doctors and nurses know me, my history and my conditions. They provide good pain management and intensive care. I am very grateful that they stabilised me yet again.

As usual I managed it to convince my mum for a short hospital escape in between injections and infusions. It is always a risk and in the end it is always a bad decision, but I need nature, I need my little adventures (I think this won’t ever change). Due to non-visitor Covid rules my mum and Bo drove to Freiburg (which, unfortunately, is not nearby) and stayed in a hotel nearby to see me downstairs. One day we had a trip to ‘Schauinsland‘ and on another day we walked a bit of the angels’ path. I want to share some thoughts I had in those hours.

The angels reminded us of the very important things in life. Patience, gratitude, honesty, love, trust, peace, mindfulness and togetherness. Stay tuned for some philosophy song recommendations with angels floating around next time. My mum is my angel.

Passing by

Sei du selbst die Veränderung, die du dir wünschst für die Welt. (Be the change you wish to see in the world.)

Schauinsland is a mountain with an elevation of 1284m in Freiburg (about a 30min car ride from the centre). It means ‘look into the country’*, you can see the other Schwarzwald (Black Forest) mountains and its Dreisamtal, perhaps your view may reach towards the Swiss Alps and the Vosges**. We were very lucky that day and indeed had a wonderful view in all directions. The sun was warming my face and the cold ground that was partly covered in snow. And it also warmed my heart, sweet memories of exploring and getting lost in the woods were flowing through my whole body. There is also a cable car and if you are fit enough many hiking trails (both for nature, sports and culture lovers). In the Middle Ages the area produced a lot of silver and lead. Anyway, there is a figure called ‘Windbohrer’ made out of an oak trunk standing in the strong wind. It says ‘als Hommage an die Kraft der Natur und als Warnung vor deren sinnloser Verschwendung’ (as a tribute to nature and as a warning of her senseless waste).

In Buddhism wind is a symbol for mind since it is also in constant flow. Flow means balancing out and illness causes (and sometimes is caused by) imbalance. Wind can be a soft and warm summer breeze, it can be refreshing, but it can also turn into a dangerous and devastating beast. Our bodies are wonderful, beautifully strong and strongly beautiful, but in us we can grow equally strong dangers. We should nourish our bodies with awareness and gratitude. We should not waste our health. It truly is a gift. Wind should and cannot be stopped, the constant flow between our thoughts and our bodies, the constant effects of past experiences on today’s decisions, cannot be turned off either. We might not be aware of it, but it is always present. More about Japanese wind philosophy here.

What this quote and figure in front of the wonderful view reminded me of was that everything can be transformed and we are constantly changed by our interactions (more on this in my next post). Life is interconnected and impermanent. That’s why happiness can be found. In fact, the acceptance and right use might be the source of all happiness. Nothing is free from this change. 

Life is dynamic and so is chronic illness and the intersection of life with chronic illness. 

Change means possibilities. Change means opportunities. 

Pain is impermanent. And so is suffering.

It gave me a deep breath of hope.

Short escape to the hotel to cuddle Bo