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Hello, My Name Is Anna And I Am A 26

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Posted on Wed, 17 Feb 2021
Question: Hello, my name is Anna and I am a 26 year old female living in Australia. I am writing with a bit of a complex condition, so I'll do my best to explain it. I do have an interest in medical research and will likely pursue this, hence why I'm using the wording I am.
I have severe Gastroparesis, this being year 9 since diagnosis. I have always had major issues with constipation and was recently diagnosed with Intestinal Dysmotility by specialists. This is where it gets complicated. I've had some pretty bad treatment from previous doctors who refused to acknowledge the dysmotility despite doing a scan that showed 100% of the tracer remaining after 2 weeks and reverse movement in the large bowel. The current team acknowledges the dysmotility, but this is where it's a little tricky. I have had a PEGJ feeding tube since October 2019, though I could never get the rate up to full, and it's progressively gotten worse in terms of rate and symptoms.  Running the jejunal feeds in 2020 caused a lot of vomiting and pain. I ended up on TPN with the previous team who managed it very badly, and also insisted I do oral food to prove I wasn't avoiding it. This of course, caused more vomiting and frequent Hypokalemia. My potassium has been so bad for the last 12 months and rarely was above 2.5 despite supplementation. I'll get back to that. I also strongly suspected dysautonomia as I often get very dizzy and have heart palpitations on standing, stairs etc. Forward to recently, and am now in the process of being diagnosed with POTS/symptoms of. The cardiologist has put me on Propranolol for managing that and the symptoms are considerably better. I've also been highly suspicious of having a connective tissue disorder as I very much meet the diagnostic criteria for hypermobile Ehlers Danlos Syndrome. I saw a Rheumatologist for this and he is getting me to do an MRI to check for Chairi and CFS leaks. He said I definitely have joint instability and even a heart murmur as a result of some connective tissue problems. I also have severe reactions to many medications, and vomiting reactions to foods, and he suggested Mast Cell Activation Syndrome as a likely cause. I had also been trying to discuss this with the previous team. I've also had a previous diagnosis of Cyclic Vomiting Syndrome which was disregarded, however I do have specific food triggers for vomiting and it did settle with some sedative medications.So, these are my problems:
. My current team want me to get off TPN and get the jejunal feeds working again. I'm on board with that, but. I don't tolerate standard prokinetics like Domperidone, Metaclopramide etc as they cause major reactions, don't change the emptying, and make the POTS much worse. I am on a very small dose of Prucalopride as it does the same. (1/4mg). Every time I get a PEGJ inserted, the jejunal tube flips back into my stomach - literally this week, the night of the surgery. I've asked for separate Jejunal and Gastric tubes but the team wasn't keen initially. I'm still waiting to hear back about the recent flip. I suspect the reverse motility might be affecting this, as well as the vomiting.. The vomiting is an ongoing issue. I can't stay hydrated on the TPN alone without jej flushes - I only get 1.3Litres fluid in the TPN and an extra 300ml every 2nd night. Any oral water comes back up generally, and I vomit gastric fluids a lot too/or drain.. Then there's oral intake. The new team actually wanted me to keep oral happening, especially without the jej feeds running, to keep the digestion working. I also can't get any stools/laxatives to work without something oral. I've found a cup of tea works the best, ie stays 30 minutes. But oral means vomiting too.. The biggest issue for me is potassium. I feel like I can never get it stable, and it drops so quickly. This current team are better at wanting it to stay above 3.5. This week I had bad diarrhea for 4 days and I feel like it's dropped again (I know my symptoms really well, ie when its 2. 5, 2.2 or lower.) I had a potassium of 1.9 last year and that was bad symptomatically, a little scary. It's tricky because I've had so many mixed messages about the projected outcomes and what I'm supposed to be doing. I also always get blamed for the potassium being low. It's hard to manage. All the signs over the years are telling me my dysmotility hasn't been getting better, it's been getting worse, and drs trying to say I'll be able to eat only food, no feeds/tpn again seems a bit unrealistic. I'm all for trying the jej feeds too but I know they don't work without prokinetics.My team aren't really giving me any clear direction on whether I'm supposed to be eating, how to manage the hydration, how to stop the potassium dropping every 2 weeks and what's going to happen if the jej feeds don't work. I also get low blood sugars so not eating entirely can be difficult too. I don't know what to do, and the potassium stresses me the most. I live alone and have to manage it all. I know I should keep chasing up the potassium but I tend to wait till it's actually low (I'm also very difficult to get bloods from, at all, and they don't use the Hickman line, so they went back to monthly bloods then freaked because it dropped so fast). I get potassium in the TPN but sometimes even that isn't enough. I'm trying to only eat once or twice a day but again, water makes me vomit too. My General Practitioner said if we don't get anywhere in a month or two to let him know and he'll make some things happen.It's just so hard to know what to do. Is it Chronic Intestinal Psuedo Obstruction? A specialist in the US looked at my scans and told me I had the anatomy for MALS and maybe SMA, but that even fixing that wouldn't help because my dysmotility is so bad. Should I even be eating? Will I be TPN dependant? Do these specialists even understand dysmotility fully? The main one has said he doesn't.  Yet everyone is telling me to just eat. Is my body just so depleted from potassium it doesn't take much to drop it? What about draining Gastric fluids? I really try not to but if I don't I vomit them - is that the problem?How do I communicate with my team effectively? They're very hard to catch. And the potassium, what is a better long term plan? If I don't have an emergency stash of potassium tablets for when I know it's bad I can't stop it getting really symptomatic. 
Sorry to be a bother, I'm just trying to get a second opinion on how to best maneuver this. I'm not after any diagnosis, just thoughts. Really appreciate it.
~ Anna





default
Follow up: Dr. Olgeta Xhufka (0 minute later)
Hello, my name is Anna and I am a 26 year old female living in Australia. I am writing with a bit of a complex condition, so I'll do my best to explain it. I do have an interest in medical research and will likely pursue this, hence why I'm using the wording I am.
I have severe Gastroparesis, this being year 9 since diagnosis. I have always had major issues with constipation and was recently diagnosed with Intestinal Dysmotility by specialists. This is where it gets complicated. I've had some pretty bad treatment from previous doctors who refused to acknowledge the dysmotility despite doing a scan that showed 100% of the tracer remaining after 2 weeks and reverse movement in the large bowel. The current team acknowledges the dysmotility, but this is where it's a little tricky. I have had a PEGJ feeding tube since October 2019, though I could never get the rate up to full, and it's progressively gotten worse in terms of rate and symptoms.  Running the jejunal feeds in 2020 caused a lot of vomiting and pain. I ended up on TPN with the previous team who managed it very badly, and also insisted I do oral food to prove I wasn't avoiding it. This of course, caused more vomiting and frequent Hypokalemia. My potassium has been so bad for the last 12 months and rarely was above 2.5 despite supplementation. I'll get back to that. I also strongly suspected dysautonomia as I often get very dizzy and have heart palpitations on standing, stairs etc. Forward to recently, and am now in the process of being diagnosed with POTS/symptoms of. The cardiologist has put me on Propranolol for managing that and the symptoms are considerably better. I've also been highly suspicious of having a connective tissue disorder as I very much meet the diagnostic criteria for hypermobile Ehlers Danlos Syndrome. I saw a Rheumatologist for this and he is getting me to do an MRI to check for Chairi and CFS leaks. He said I definitely have joint instability and even a heart murmur as a result of some connective tissue problems. I also have severe reactions to many medications, and vomiting reactions to foods, and he suggested Mast Cell Activation Syndrome as a likely cause. I had also been trying to discuss this with the previous team. I've also had a previous diagnosis of Cyclic Vomiting Syndrome which was disregarded, however I do have specific food triggers for vomiting and it did settle with some sedative medications.So, these are my problems:
. My current team want me to get off TPN and get the jejunal feeds working again. I'm on board with that, but. I don't tolerate standard prokinetics like Domperidone, Metaclopramide etc as they cause major reactions, don't change the emptying, and make the POTS much worse. I am on a very small dose of Prucalopride as it does the same. (1/4mg). Every time I get a PEGJ inserted, the jejunal tube flips back into my stomach - literally this week, the night of the surgery. I've asked for separate Jejunal and Gastric tubes but the team wasn't keen initially. I'm still waiting to hear back about the recent flip. I suspect the reverse motility might be affecting this, as well as the vomiting.. The vomiting is an ongoing issue. I can't stay hydrated on the TPN alone without jej flushes - I only get 1.3Litres fluid in the TPN and an extra 300ml every 2nd night. Any oral water comes back up generally, and I vomit gastric fluids a lot too/or drain.. Then there's oral intake. The new team actually wanted me to keep oral happening, especially without the jej feeds running, to keep the digestion working. I also can't get any stools/laxatives to work without something oral. I've found a cup of tea works the best, ie stays 30 minutes. But oral means vomiting too.. The biggest issue for me is potassium. I feel like I can never get it stable, and it drops so quickly. This current team are better at wanting it to stay above 3.5. This week I had bad diarrhea for 4 days and I feel like it's dropped again (I know my symptoms really well, ie when its 2. 5, 2.2 or lower.) I had a potassium of 1.9 last year and that was bad symptomatically, a little scary. It's tricky because I've had so many mixed messages about the projected outcomes and what I'm supposed to be doing. I also always get blamed for the potassium being low. It's hard to manage. All the signs over the years are telling me my dysmotility hasn't been getting better, it's been getting worse, and drs trying to say I'll be able to eat only food, no feeds/tpn again seems a bit unrealistic. I'm all for trying the jej feeds too but I know they don't work without prokinetics.My team aren't really giving me any clear direction on whether I'm supposed to be eating, how to manage the hydration, how to stop the potassium dropping every 2 weeks and what's going to happen if the jej feeds don't work. I also get low blood sugars so not eating entirely can be difficult too. I don't know what to do, and the potassium stresses me the most. I live alone and have to manage it all. I know I should keep chasing up the potassium but I tend to wait till it's actually low (I'm also very difficult to get bloods from, at all, and they don't use the Hickman line, so they went back to monthly bloods then freaked because it dropped so fast). I get potassium in the TPN but sometimes even that isn't enough. I'm trying to only eat once or twice a day but again, water makes me vomit too. My General Practitioner said if we don't get anywhere in a month or two to let him know and he'll make some things happen.It's just so hard to know what to do. Is it Chronic Intestinal Psuedo Obstruction? A specialist in the US looked at my scans and told me I had the anatomy for MALS and maybe SMA, but that even fixing that wouldn't help because my dysmotility is so bad. Should I even be eating? Will I be TPN dependant? Do these specialists even understand dysmotility fully? The main one has said he doesn't.  Yet everyone is telling me to just eat. Is my body just so depleted from potassium it doesn't take much to drop it? What about draining Gastric fluids? I really try not to but if I don't I vomit them - is that the problem?How do I communicate with my team effectively? They're very hard to catch. And the potassium, what is a better long term plan? If I don't have an emergency stash of potassium tablets for when I know it's bad I can't stop it getting really symptomatic. 
Sorry to be a bother, I'm just trying to get a second opinion on how to best maneuver this. I'm not after any diagnosis, just thoughts. Really appreciate it.
~ Anna





doctor
Answered by Dr. Olgeta Xhufka (1 hour later)
Brief Answer:
Gastric electrical stimulation device may be an option.

Detailed Answer:
Hello dear XXXXXXX welcome to Ask a doctor's service.
I read your query and here is my advice.

I am sorry about this situation you are having. You are having a severe gastroparesis , medical treatments are failing and PEGJ is also failing in previous attampts.
This is causing you difficulty feeding and low potassium levels which is also probably affecting heart function.
I think you can discuss with your specialist
about trying gastric electrical stimulation or pacing. A device inserted on stomach or intestines to stimulate their movement.
Also you may need intravenous therapy in order to keep your electrolytes balance especially potassium.


Hope I have answered the question.
Let me know if I can assist you further.
Above answer was peer-reviewed by : Dr. Kampana
doctor
doctor
Answered by Dr. Olgeta Xhufka (0 minute later)
Brief Answer:
Gastric electrical stimulation device may be an option.

Detailed Answer:
Hello dear XXXXXXX welcome to Ask a doctor's service.
I read your query and here is my advice.

I am sorry about this situation you are having. You are having a severe gastroparesis , medical treatments are failing and PEGJ is also failing in previous attampts.
This is causing you difficulty feeding and low potassium levels which is also probably affecting heart function.
I think you can discuss with your specialist
about trying gastric electrical stimulation or pacing. A device inserted on stomach or intestines to stimulate their movement.
Also you may need intravenous therapy in order to keep your electrolytes balance especially potassium.


Hope I have answered the question.
Let me know if I can assist you further.
Above answer was peer-reviewed by : Dr. Kampana
doctor
default
Follow up: Dr. Olgeta Xhufka (28 minutes later)
Unfortunately I've been told by a previous Gastroenterologist that Pacemakers aren't very effective for idiopathic/non-diabetic Gastroparesis patients. Also in Australia there is a big pricetag and you have to pay for it yourself, it's about $20,000-$40,000.
Regarding the potassium yes, but I'm not sure that helps me work out a better long term plan with my team?
default
Follow up: Dr. Olgeta Xhufka (0 minute later)
Unfortunately I've been told by a previous Gastroenterologist that Pacemakers aren't very effective for idiopathic/non-diabetic Gastroparesis patients. Also in Australia there is a big pricetag and you have to pay for it yourself, it's about $20,000-$40,000.
Regarding the potassium yes, but I'm not sure that helps me work out a better long term plan with my team?
doctor
Answered by Dr. Olgeta Xhufka (33 minutes later)
Brief Answer:
See details below.

Detailed Answer:
Hello dear and thank you for asking again.
It is seen that pacemakers work better on diabetic gastroparesis but it doesn't mean that they don't work on idiopathic gastroparesis.
I am sorry about this high price they have.
Taking count that the medical treatments you are having all failed as well as PEGJ I believe that pacemakers may be the only chance.
Intravenous therapy is necessary in this case till you find a solution for gastroparesis.
You can retry PEGJ before considering the pacemaker.
Above answer was peer-reviewed by : Dr. Yogesh D
doctor
doctor
Answered by Dr. Olgeta Xhufka (0 minute later)
Brief Answer:
See details below.

Detailed Answer:
Hello dear and thank you for asking again.
It is seen that pacemakers work better on diabetic gastroparesis but it doesn't mean that they don't work on idiopathic gastroparesis.
I am sorry about this high price they have.
Taking count that the medical treatments you are having all failed as well as PEGJ I believe that pacemakers may be the only chance.
Intravenous therapy is necessary in this case till you find a solution for gastroparesis.
You can retry PEGJ before considering the pacemaker.
Note: For further follow up on digestive issues share your reports here and Click here.

Above answer was peer-reviewed by : Dr. Yogesh D
doctor
Answered by
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Dr. Olgeta Xhufka

General & Family Physician

Practicing since :2011

Answered : 10125 Questions

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Hello, My Name Is Anna And I Am A 26

Hello, my name is Anna and I am a 26 year old female living in Australia. I am writing with a bit of a complex condition, so I'll do my best to explain it. I do have an interest in medical research and will likely pursue this, hence why I'm using the wording I am. I have severe Gastroparesis, this being year 9 since diagnosis. I have always had major issues with constipation and was recently diagnosed with Intestinal Dysmotility by specialists. This is where it gets complicated. I've had some pretty bad treatment from previous doctors who refused to acknowledge the dysmotility despite doing a scan that showed 100% of the tracer remaining after 2 weeks and reverse movement in the large bowel. The current team acknowledges the dysmotility, but this is where it's a little tricky. I have had a PEGJ feeding tube since October 2019, though I could never get the rate up to full, and it's progressively gotten worse in terms of rate and symptoms.  Running the jejunal feeds in 2020 caused a lot of vomiting and pain. I ended up on TPN with the previous team who managed it very badly, and also insisted I do oral food to prove I wasn't avoiding it. This of course, caused more vomiting and frequent Hypokalemia. My potassium has been so bad for the last 12 months and rarely was above 2.5 despite supplementation. I'll get back to that. I also strongly suspected dysautonomia as I often get very dizzy and have heart palpitations on standing, stairs etc. Forward to recently, and am now in the process of being diagnosed with POTS/symptoms of. The cardiologist has put me on Propranolol for managing that and the symptoms are considerably better. I've also been highly suspicious of having a connective tissue disorder as I very much meet the diagnostic criteria for hypermobile Ehlers Danlos Syndrome. I saw a Rheumatologist for this and he is getting me to do an MRI to check for Chairi and CFS leaks. He said I definitely have joint instability and even a heart murmur as a result of some connective tissue problems. I also have severe reactions to many medications, and vomiting reactions to foods, and he suggested Mast Cell Activation Syndrome as a likely cause. I had also been trying to discuss this with the previous team. I've also had a previous diagnosis of Cyclic Vomiting Syndrome which was disregarded, however I do have specific food triggers for vomiting and it did settle with some sedative medications.So, these are my problems: . My current team want me to get off TPN and get the jejunal feeds working again. I'm on board with that, but. I don't tolerate standard prokinetics like Domperidone, Metaclopramide etc as they cause major reactions, don't change the emptying, and make the POTS much worse. I am on a very small dose of Prucalopride as it does the same. (1/4mg). Every time I get a PEGJ inserted, the jejunal tube flips back into my stomach - literally this week, the night of the surgery. I've asked for separate Jejunal and Gastric tubes but the team wasn't keen initially. I'm still waiting to hear back about the recent flip. I suspect the reverse motility might be affecting this, as well as the vomiting.. The vomiting is an ongoing issue. I can't stay hydrated on the TPN alone without jej flushes - I only get 1.3Litres fluid in the TPN and an extra 300ml every 2nd night. Any oral water comes back up generally, and I vomit gastric fluids a lot too/or drain.. Then there's oral intake. The new team actually wanted me to keep oral happening, especially without the jej feeds running, to keep the digestion working. I also can't get any stools/laxatives to work without something oral. I've found a cup of tea works the best, ie stays 30 minutes. But oral means vomiting too.. The biggest issue for me is potassium. I feel like I can never get it stable, and it drops so quickly. This current team are better at wanting it to stay above 3.5. This week I had bad diarrhea for 4 days and I feel like it's dropped again (I know my symptoms really well, ie when its 2. 5, 2.2 or lower.) I had a potassium of 1.9 last year and that was bad symptomatically, a little scary. It's tricky because I've had so many mixed messages about the projected outcomes and what I'm supposed to be doing. I also always get blamed for the potassium being low. It's hard to manage. All the signs over the years are telling me my dysmotility hasn't been getting better, it's been getting worse, and drs trying to say I'll be able to eat only food, no feeds/tpn again seems a bit unrealistic. I'm all for trying the jej feeds too but I know they don't work without prokinetics.My team aren't really giving me any clear direction on whether I'm supposed to be eating, how to manage the hydration, how to stop the potassium dropping every 2 weeks and what's going to happen if the jej feeds don't work. I also get low blood sugars so not eating entirely can be difficult too. I don't know what to do, and the potassium stresses me the most. I live alone and have to manage it all. I know I should keep chasing up the potassium but I tend to wait till it's actually low (I'm also very difficult to get bloods from, at all, and they don't use the Hickman line, so they went back to monthly bloods then freaked because it dropped so fast). I get potassium in the TPN but sometimes even that isn't enough. I'm trying to only eat once or twice a day but again, water makes me vomit too. My General Practitioner said if we don't get anywhere in a month or two to let him know and he'll make some things happen.It's just so hard to know what to do. Is it Chronic Intestinal Psuedo Obstruction? A specialist in the US looked at my scans and told me I had the anatomy for MALS and maybe SMA, but that even fixing that wouldn't help because my dysmotility is so bad. Should I even be eating? Will I be TPN dependant? Do these specialists even understand dysmotility fully? The main one has said he doesn't.  Yet everyone is telling me to just eat. Is my body just so depleted from potassium it doesn't take much to drop it? What about draining Gastric fluids? I really try not to but if I don't I vomit them - is that the problem?How do I communicate with my team effectively? They're very hard to catch. And the potassium, what is a better long term plan? If I don't have an emergency stash of potassium tablets for when I know it's bad I can't stop it getting really symptomatic.  Sorry to be a bother, I'm just trying to get a second opinion on how to best maneuver this. I'm not after any diagnosis, just thoughts. Really appreciate it. ~ Anna