Once again crazy life has detained me so it's been awhile since my last update. Many new things and many things remain unchanged. The good news is, Audrey has only been inpatient once since my last blog! She went 90 days to the date! That's the longest she's been out of the hospital since January 2012. This is definitely a trend I hope will continue.
Medically, she's not doing better. We have been able to do more at home to keep her here rather than in the hospital. It's a lot more work for me but so worth it to keep our little family together!Audrey's doctors are, as usual, pushing the envelope of treatment for her. Her amazing genetics doctor is challenging himself to find ways to help her and other patients with her genetic mutations. For the moment we have stalled because we have tried everything, even the "in theory this may help" but I know the science will come up with something soon.
Audrey's endocrinologist has also been hard working. She has thought "outside-the-box" for Audrey and she is now using a insulin pump to deliver continuous hydrocortisone to treat her adrenal insufficiency. We found out through labs that Audrey metabolizes cortisol about twice as fast as most people. This made her management very difficult to control. Since starting her pump though, she has been doing much better! We are so happy to be finding these small things which dramatically improve her quality of life.
And lastly, her GI doctor. He works tirelessly for Audrey (and his other patients) trying to manage her GI system. Unfortunately she has complete failure right now and while we hope to improve this, no one has yet seen any of the children with her mutation improve their GI function. This is a very hard pill for me to swallow. I am hopeful that they will find something to help her but I am realistic enough to know they likely will not. That being said, at least the surgeries she underwent last year have kept her out of the hospital. We had hoped she would be able to tolerate more formula after her ileostomy but that is not the case. What her ileostomy has done, is allow her to stay home and prevent the massive abdominal distention and pain she was living with last year. Last year she was inpatient at least once a month, usually for a bowel clean out and now we are able to avoid that. That alone makes the whole surgery worth it. Like her genetic treatment, we are just about at the end of the line for options for treatment of her go failure at her age. As she gets older additional medications may open up (ones that are approved for adult use only) or we may go the route to try some under compassionate use, though the risks and benefits will have to be carefully weighed. If there is one miracle she needs most, this is it.
Speaking of getting older...guess who is turning 5 soon?!?! I can't believe she is one month from being a 5 year old. Everyday that I see her grow and develop and learn new things is such a miracle because I know how precarious her life is. I have loved seeing her push her self the last few months to do what she really wants to do which is ballet. Last year when she started asking to do ballet, there was no way she would have been well enough or out of the hospital long enough to try. Now she takes a toddler class twice a week and while its hard on her body and takes a lot of medical prep work on my part to get her there, she's so amazingly happy I would move heaven and earth to be sure she gets to go. I can't wait to see all that she will continue to accomplish! Keep it up my girl!
Tuesday, August 5, 2014
Sunday, February 23, 2014
February: The month we lost
The title of this post covers many things. Audrey was admitted on the first of February and discharged on the 22nd. Just 6 days shy of the entire month of February. This admission was so different from any other admission she's had. First she was really sick. While she has been really sick before, this was park the crash cart at her door in the ICU sick. Audrey had never been in the ICU before. She had never required vasopressors or rapid infusions of liters of fluid and blood products just to stabilize her. This was the sickest I have ever seen my baby and I have never been so scared for her. Seeing your chronically ill child this sick makes you confront some difficult facts. First, odds are this is not the last time I will see her go through this same scenario. The nature of her illness prevents her from being able to handle stress and infection like others might. Second, one day, this may become too much for her body to handle. This is the hard part of chronic disease. The hard part of a progressive disease like mitochondrial disease. Things are not likely to get better. This reality made this admission much more difficult for me than others.
Back to the title, Audrey lost major ground in her battle against dysautonomia. While at first we thought her vital sign instability was temporary due to her infection, we soon realized her body was actually unable to control itself enough to come off her vasopressor drips. The problem with dysautonomia is that there is very little to treat it and there is only one doctor in California who will see a pediatric patient with dysautonomia. You can imagine how popular he is and what his wait list looks like. The intensivists and all of Audrey's doctors brainstormed and tried multiple ideas and finally had to consult with outside specialists to figure out how to treat her dysautonomia. So she is on a few different medications which have helped control her vitals signs but she is still very dizzy and needs around the clock medication to control her nausea.
In the midst of her ICU drama, we find out Audrey has a new problem. She can now add adrenal insufficiency to her list of diagnosis. While its possible it has been going on for awhile, she was tested last year so its likely that it was the result of her infection. Thats what happens with mito. Every serious infection or stressor can lead to new symptoms and problems. So, for those that don't know, adrenal glands make cortisol which is an essential fight or flight hormone. Without this your body cannot respond appropriately to any stress whether its a hot day or an ear infection or a car accident, your body needs to be able to respond quickly and Audrey can't do this. Sounds familiar to dysautonomia right? It's been very difficult to tease out which symptoms belong to which diagnosis. Adrenal insufficiency in a child like Audrey is, of course, difficult to treat an manage because her body already doesn't handle stress in the best of circumstances. This will be an ongoing ordeal requiring patience from me and her medical team as we learn how to support her in this process.
So Audrey is home now. She's sicker than she's ever been. Her new baseline is way below where her sickest baseline was. I can only hope she will be able to get back some of the quality of life she had before becoming ill but no one knows if this is a realistic goal. For now we will continue this impossible home schedule with medications every 1-2 hours from 6am to midnight. With vital signs and urine dips and check ins with her doctors. Everyone is so happy for us to be home but this is definitely the saddest I have ever felt coming home. I feel like we just lost a major battle and I don't know how we will rebuild and continue on. Her health status is so fragile I'm afraid to take my suitcase out of the car because one wrong move will have us speeding back to the emergency room. All I can hope for is some stability to slow this ever progressing, life-stealing disease.
Back to the title, Audrey lost major ground in her battle against dysautonomia. While at first we thought her vital sign instability was temporary due to her infection, we soon realized her body was actually unable to control itself enough to come off her vasopressor drips. The problem with dysautonomia is that there is very little to treat it and there is only one doctor in California who will see a pediatric patient with dysautonomia. You can imagine how popular he is and what his wait list looks like. The intensivists and all of Audrey's doctors brainstormed and tried multiple ideas and finally had to consult with outside specialists to figure out how to treat her dysautonomia. So she is on a few different medications which have helped control her vitals signs but she is still very dizzy and needs around the clock medication to control her nausea.
In the midst of her ICU drama, we find out Audrey has a new problem. She can now add adrenal insufficiency to her list of diagnosis. While its possible it has been going on for awhile, she was tested last year so its likely that it was the result of her infection. Thats what happens with mito. Every serious infection or stressor can lead to new symptoms and problems. So, for those that don't know, adrenal glands make cortisol which is an essential fight or flight hormone. Without this your body cannot respond appropriately to any stress whether its a hot day or an ear infection or a car accident, your body needs to be able to respond quickly and Audrey can't do this. Sounds familiar to dysautonomia right? It's been very difficult to tease out which symptoms belong to which diagnosis. Adrenal insufficiency in a child like Audrey is, of course, difficult to treat an manage because her body already doesn't handle stress in the best of circumstances. This will be an ongoing ordeal requiring patience from me and her medical team as we learn how to support her in this process.
So Audrey is home now. She's sicker than she's ever been. Her new baseline is way below where her sickest baseline was. I can only hope she will be able to get back some of the quality of life she had before becoming ill but no one knows if this is a realistic goal. For now we will continue this impossible home schedule with medications every 1-2 hours from 6am to midnight. With vital signs and urine dips and check ins with her doctors. Everyone is so happy for us to be home but this is definitely the saddest I have ever felt coming home. I feel like we just lost a major battle and I don't know how we will rebuild and continue on. Her health status is so fragile I'm afraid to take my suitcase out of the car because one wrong move will have us speeding back to the emergency room. All I can hope for is some stability to slow this ever progressing, life-stealing disease.
Saturday, January 4, 2014
New year, new hope
It's been nearly 7 weeks since Audrey's surgery. She's been in the hospital twice since her initial discharge on the 25th. We've been struggling with urine infections since early October. With her last admission we added Infectious Disease to her medical team to help us get a handle on her. While they initially thought they would be able to make routine recommendations, they quickly understood the complexity that is Audrey. Her main problem is her dysmotility. She has spent the last 2 years on first erythromycin then augmentin trying to make her intestines move. Thrown in there are times she's been on flagyl or neomycin for small intestinal bacteria overgrowth. Then in March when we started cathing her, we added keflex to the mix. We've spent the last 2 years wiping out all the normal good bacteria from her body so we are left with more serious bacteria which is why she gets ill so quickly. Audrey is unable to use probiotics since she has a central line and unfortunately she's just not tolerating enough feeds to get her GI tract to make normal bacteria. There are a couple ideas we will discuss with her GI and urologist this month to see if we can get a different plan. For now we are holding our breath hoping she does not get sick before then.
Energy wise, Audrey is doing amazing!! When she is out of the hospital and feeling good I feel like I have my little girl back. She is feeling so much better. Even though she's not tolerating much feeding wise, she is so much happier and feels so much better, her surgery was definitely a success!! It may seem drastic to some to put her through an ileostomy and still be completely TPN dependent, however, if you could have seen what she was going through everyday you would understand. She had severe abdominal distention as her baseline, she had an enema every single day just to pass gas, she couldn't tolerate a single medication through her j-tube without extreme pain. That was her life everyday. She was tired and in pain. Now she is happy. Her tummy doesn't hurt, her medication list is cut in half and her body finally has energy to play instead of using it all trying to make her intestines work. It truly has been life changing for her.
While we have a long way to go for her, I am finally feeling hopeful. This journey is so hard. Everytime there is an improvement somewhere, either something else fails or the improvement is short lived. But I have hope for Audrey. We are living the motto "Carpe Diem" right now. We are taking full advantage of her good days and making them amazing days, knowing she will likely need a day or two to recover. We are rejoicing that she is even having good days since it has been months without them. We are hoping to spend less time in the hospital this year. Last year she spent 77 days in the hospital. Fingers crossed it is much lower this year!! Audrey has also been matched with a runner in the Irunformichael program so we are so excited to participate with that. Over all we are looking forward to this new year and all the new chances it will bring.
Energy wise, Audrey is doing amazing!! When she is out of the hospital and feeling good I feel like I have my little girl back. She is feeling so much better. Even though she's not tolerating much feeding wise, she is so much happier and feels so much better, her surgery was definitely a success!! It may seem drastic to some to put her through an ileostomy and still be completely TPN dependent, however, if you could have seen what she was going through everyday you would understand. She had severe abdominal distention as her baseline, she had an enema every single day just to pass gas, she couldn't tolerate a single medication through her j-tube without extreme pain. That was her life everyday. She was tired and in pain. Now she is happy. Her tummy doesn't hurt, her medication list is cut in half and her body finally has energy to play instead of using it all trying to make her intestines work. It truly has been life changing for her.
While we have a long way to go for her, I am finally feeling hopeful. This journey is so hard. Everytime there is an improvement somewhere, either something else fails or the improvement is short lived. But I have hope for Audrey. We are living the motto "Carpe Diem" right now. We are taking full advantage of her good days and making them amazing days, knowing she will likely need a day or two to recover. We are rejoicing that she is even having good days since it has been months without them. We are hoping to spend less time in the hospital this year. Last year she spent 77 days in the hospital. Fingers crossed it is much lower this year!! Audrey has also been matched with a runner in the Irunformichael program so we are so excited to participate with that. Over all we are looking forward to this new year and all the new chances it will bring.
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| Best ostomy belt ever! |
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| First time swinging in over a year! |
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| She loves her princesses! |
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| Best big brother ever! |
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| She was deliriously happy (literally) |
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| What keeps her going! |
Monday, November 25, 2013
The BIG surgery
After a week of discussion, planning and testing, it was Monday, surgery day. We knew she was an add on for the afternoon which meant she could be called to OR anytime from 12 to 10 at night. The suspense was hard to deal with. Audrey's Opa came to she the stress and worry with me. Audrey was blissfully ignorant of what was going to happen. She had had teaching on Friday where they made a doll with an ostomy and a bladder catheter in the belly button and we told her where everything was going to come out. She seemed indifferent to the teaching but played with the doll all weekend and was able to tell others all about the ostomy and bag. So Finally at 3 it was time to go!! We went to pre-op and spoke to a dizzying number of people. Going over the conditional plan, allergies and mitochondrial requirements with the OR nurse, the pediatric surgeon, the urologist, the anesthesia team and the pain team. Everyone seemed well read on Audrey and I was as comfortable as I could be handing over my daughter. Surgery was supposed to be 2.5-3 hours.
At hour 2.5, I was called and notified she was stable and surgery was progressing fine. At 3.25 they called say urology was done so the surgeon was closing, finishing the ostomy and then she'd be done. Finally after 4 hours of surgery she was done and going to recovery. Both teams of doctors reported a smooth surgery with no complications. Audrey was very sleepy in recovery but since she was inpatient she didn't need to be fully awake. She was responding to questions and asking for only her princess blanket (she refuses to use hospital blankets) so we knew she was ok. The pain team had placed an epidural so her pain seemed well managed which was a huge relief!! We were back to her room by 10. She had a busy night and her vital signs were a little off but she rested well! Let the healing begin!
Post Op Day 1-
The days after surgery are often the most difficult. Swelling peaks at day 3-5 as does pain and discomfort. With mitochondrial disease, the body has to recover from this huge strain placed on it which has depleted any extra energy it may have had. This was complicated by Audrey's primary team of docs not knowing her well. Of all the days for her to have new doctors this was not a good one. So Audrey slept ALOT! Her body was frantically trying to make enough energy. Her heart rate was high, her blood pressure was low. She had a low grade fever and needed her oxygen. She needed extra fluid. Her pain was mostly controlled unless we had to move her but she was very itchy from the epidural. It was a difficult day for her. Some good things happened though. First her new stroller came! Yay! It had been ordered for her 2 months ago so it was much anticipated, and it was pink! Next, after major surgery, patients have to do the dreaded incentive spirometry. This forces big deep breathes to help open the lungs and get rid of anesthetics. This being a Children's hospital this mean...blowing bubbles!! Same action but much more fun! Audrey willing participated and finally her oxygen started going up and temperature went down. Surgically everything was going well. Her
mitrofanoff was draining well. Her ostomy was pink and happy though still sleeping. We did her first
bag change with the ostomy nurse. By the end of the day, we got our first smile!
Post Op Day 2-
Audrey woke up bright and early asking to watch Mickey Mouse! I love my girlie! And surprise, surprise, her ostomy was working!! Yay! Today, everyone is cautiously optimistic that we are finally on the right path. It was decided to start a small amount of pedialyte, give her some blood and we were still trouble shooting the annoying itching. Overall it was a good day. We were able to get her up out of bed into a chair and she was awake most of the day. She tolerated the pedialyte well and an advancing plan was made to get us home. Home!!! After 16 days our discussions finally included home! I was feeling so hopeful!
Post Op Day 3-
A day of rest! Audrey finally woke up free of the relentless itching and is still pain free. Since her epidural site looks great the pain team decided to leave it in place and let her have a good day. We had to switch her pedialyte to her jtube from her g because of retching so we did not start formula yet. It was decided her anticipated discharge date will be Monday 11/25, 3 weeks from when she was admitted.
Post Op Day 4-
We are counting the days till we can go home! Audrey woke up again in great spirits and pain and itch free! We were even able to get her to stand on her own to weigh her. The pain team decided to leave her epidural in another day since she's doing so well and the site looks good. Audrey started half strength formula in her jtube! Yay!! Now we have to see what happens with her ileostomy output since it's been high. Urology also came and took out her foley so it's just her mitrofanoff draining urine now.
Post Op Day 5-
It's finally time to remove her epidural. Her dressing is lifting now so it's not safe to leave in. Everyone is nervous but hopeful since it was able to stay in so long she has missed the worst of the post op pain. They started around the clock pain medicine before taking it out so she wouldn't get behind in pain. Her feeds were advanced to full strength!! For the first time in nearly 5 months, Audrey is getting formula!! Her pain ended up being well controlled all day. What a relief! Our biggest problem was getting the ostomy bag to stay on. It's close to the dressing for the mitrofanoff so there isn't a lot of skin to make a good seal. By the 4th bag we both had our break down. She cried that she was scared and so was I. This is one more problem and complication in her life. We are trying so hard to make things better for her, I pray this was the right path.
Post Op Day 6-
Today we are not changing her feeds. This is what she will go home on and we will plan to advance her very slowly. We are going to start capping her mitrofanoff today and draining every 4 hours. This will be the plan for the next 2 weeks so I hope she does well. Her pain medicine is now PRN and she's tolerating that well. It was a restful day in preparation for a busy week!
Post Op Day 7-
Going home today!!
At hour 2.5, I was called and notified she was stable and surgery was progressing fine. At 3.25 they called say urology was done so the surgeon was closing, finishing the ostomy and then she'd be done. Finally after 4 hours of surgery she was done and going to recovery. Both teams of doctors reported a smooth surgery with no complications. Audrey was very sleepy in recovery but since she was inpatient she didn't need to be fully awake. She was responding to questions and asking for only her princess blanket (she refuses to use hospital blankets) so we knew she was ok. The pain team had placed an epidural so her pain seemed well managed which was a huge relief!! We were back to her room by 10. She had a busy night and her vital signs were a little off but she rested well! Let the healing begin!
Back from OR, there's no more room on this tummy!
Post Op Day 1-
The days after surgery are often the most difficult. Swelling peaks at day 3-5 as does pain and discomfort. With mitochondrial disease, the body has to recover from this huge strain placed on it which has depleted any extra energy it may have had. This was complicated by Audrey's primary team of docs not knowing her well. Of all the days for her to have new doctors this was not a good one. So Audrey slept ALOT! Her body was frantically trying to make enough energy. Her heart rate was high, her blood pressure was low. She had a low grade fever and needed her oxygen. She needed extra fluid. Her pain was mostly controlled unless we had to move her but she was very itchy from the epidural. It was a difficult day for her. Some good things happened though. First her new stroller came! Yay! It had been ordered for her 2 months ago so it was much anticipated, and it was pink! Next, after major surgery, patients have to do the dreaded incentive spirometry. This forces big deep breathes to help open the lungs and get rid of anesthetics. This being a Children's hospital this mean...blowing bubbles!! Same action but much more fun! Audrey willing participated and finally her oxygen started going up and temperature went down. Surgically everything was going well. Her
mitrofanoff was draining well. Her ostomy was pink and happy though still sleeping. We did her first
bag change with the ostomy nurse. By the end of the day, we got our first smile!
Very tired mitochondria!
The new PINK stroller!
,
Finally, a smile from my girl!
Post Op Day 2-
Audrey woke up bright and early asking to watch Mickey Mouse! I love my girlie! And surprise, surprise, her ostomy was working!! Yay! Today, everyone is cautiously optimistic that we are finally on the right path. It was decided to start a small amount of pedialyte, give her some blood and we were still trouble shooting the annoying itching. Overall it was a good day. We were able to get her up out of bed into a chair and she was awake most of the day. She tolerated the pedialyte well and an advancing plan was made to get us home. Home!!! After 16 days our discussions finally included home! I was feeling so hopeful!
Happy girl first thing in the morning!
Post Op Day 3-
A day of rest! Audrey finally woke up free of the relentless itching and is still pain free. Since her epidural site looks great the pain team decided to leave it in place and let her have a good day. We had to switch her pedialyte to her jtube from her g because of retching so we did not start formula yet. It was decided her anticipated discharge date will be Monday 11/25, 3 weeks from when she was admitted.
Up in her stroller!
Post Op Day 4-
We are counting the days till we can go home! Audrey woke up again in great spirits and pain and itch free! We were even able to get her to stand on her own to weigh her. The pain team decided to leave her epidural in another day since she's doing so well and the site looks good. Audrey started half strength formula in her jtube! Yay!! Now we have to see what happens with her ileostomy output since it's been high. Urology also came and took out her foley so it's just her mitrofanoff draining urine now.
Post Op Day 5-
It's finally time to remove her epidural. Her dressing is lifting now so it's not safe to leave in. Everyone is nervous but hopeful since it was able to stay in so long she has missed the worst of the post op pain. They started around the clock pain medicine before taking it out so she wouldn't get behind in pain. Her feeds were advanced to full strength!! For the first time in nearly 5 months, Audrey is getting formula!! Her pain ended up being well controlled all day. What a relief! Our biggest problem was getting the ostomy bag to stay on. It's close to the dressing for the mitrofanoff so there isn't a lot of skin to make a good seal. By the 4th bag we both had our break down. She cried that she was scared and so was I. This is one more problem and complication in her life. We are trying so hard to make things better for her, I pray this was the right path.
A big leaky mess!!
Post Op Day 6-
Today we are not changing her feeds. This is what she will go home on and we will plan to advance her very slowly. We are going to start capping her mitrofanoff today and draining every 4 hours. This will be the plan for the next 2 weeks so I hope she does well. Her pain medicine is now PRN and she's tolerating that well. It was a restful day in preparation for a busy week!
She can sit up alone now (to play princesses of course)!!
Post Op Day 7-
Going home today!!
Monday, November 18, 2013
Surgery
I'm terrified. It's 630am but I've been up since 4 and was tossing and turning the hours before that. I'm filled with doubts about the choices that have been made and where we are going with her medical care. With each step we've taken, it's been the one I said I wouldn't do to her. Today will be a life changing day for Audrey and I hope with all of my heart it will be for the better.
So Audrey was supposed to have a cecostomy tube placed last Monday. This tube would have enabled us to flush her colon from the beginning rather than torturing her with enemas to clean her out from the end. However, thanks to the brilliance of her surgeon, a contrast enema showed the valve between her small intestine and large intestine didn't work. I was very disappointed since this really limited our options but I was thankful we found this out ahead of time because it could have meant months of trying to make something work that was never going to work to begin with. After discussions between her team it was decided that an ileostomy would be her best choice. This is where the end of her small intestine is brought to her abdominal wall and the poop drains into a bag. This would bypass her dysfunctional colon completely and hopefully allow her to tolerate feedings. The decision took a few days for me to come to grips with. It was ultimately the surgical fellow who reminded me that this doesn't have to be permanent. This surgery can be reversed. That was a great relief to my mind. I'm terrified of making the wrong decisions, especially ones we can't undo. My only requirement for this surgery is that we also place her mitrofanoff at the same time. A mitrofanoff is where they take her appendix and run it from her bladder to her belly button. Then when it's time to cath her and drain her bladder, we can do it from her belly button. This is much easier for her, not something anybody can see and will allow her to cath herself easily. But this is a major surgery. Both of these procedures are and a mito kid does not handle big procedures. So if we are going to do one to her, we are doing them both so she doesn't have to do this next year.
So after much discussion and planning, today is that day. Sometime this afternoon, my daughter will go to the OR and they will disconnect her small intestine and put it on her tummy and then create an opening in her belly button to her bladder. She will no longer poop from her bottom.. She will no longer need daily enemas and suppositories and buckets of miralax and milk of magnesium, we hope, and pray that this surgery will dramatically change her life so we don't live in the hospital for bowel clean outs and abdominal pain and distension. We will see...
So Audrey was supposed to have a cecostomy tube placed last Monday. This tube would have enabled us to flush her colon from the beginning rather than torturing her with enemas to clean her out from the end. However, thanks to the brilliance of her surgeon, a contrast enema showed the valve between her small intestine and large intestine didn't work. I was very disappointed since this really limited our options but I was thankful we found this out ahead of time because it could have meant months of trying to make something work that was never going to work to begin with. After discussions between her team it was decided that an ileostomy would be her best choice. This is where the end of her small intestine is brought to her abdominal wall and the poop drains into a bag. This would bypass her dysfunctional colon completely and hopefully allow her to tolerate feedings. The decision took a few days for me to come to grips with. It was ultimately the surgical fellow who reminded me that this doesn't have to be permanent. This surgery can be reversed. That was a great relief to my mind. I'm terrified of making the wrong decisions, especially ones we can't undo. My only requirement for this surgery is that we also place her mitrofanoff at the same time. A mitrofanoff is where they take her appendix and run it from her bladder to her belly button. Then when it's time to cath her and drain her bladder, we can do it from her belly button. This is much easier for her, not something anybody can see and will allow her to cath herself easily. But this is a major surgery. Both of these procedures are and a mito kid does not handle big procedures. So if we are going to do one to her, we are doing them both so she doesn't have to do this next year.
So after much discussion and planning, today is that day. Sometime this afternoon, my daughter will go to the OR and they will disconnect her small intestine and put it on her tummy and then create an opening in her belly button to her bladder. She will no longer poop from her bottom.. She will no longer need daily enemas and suppositories and buckets of miralax and milk of magnesium, we hope, and pray that this surgery will dramatically change her life so we don't live in the hospital for bowel clean outs and abdominal pain and distension. We will see...
This is Audrey with her ostomy doll. She learned what it's for and why it's there and how to put the bag on. She was also site marked for the location of her ostomy, where it should work best. X marks the spot!
Friday, November 8, 2013
Motility testing
This week had a big note on my calender for the past few months. This week Audrey's motility test was scheduled. We have been waiting for this since the beginning of April. While at that time I was on the fence, it became more and more obvious that this was something we had to have done. I came to the hospital with mixed emotions. First, I was so nervous that the test would be "normal." Not that I won't something to be wrong with her, but her GI motility is anything but normal. In the mito world we are faced with test after test saying "it's not normal but it's not completely abnormal." Second, I worried the results would be awful. What if her motility was so bad nothing worked? What does that mean for her long term? Finally, I worried about how she would handle the procedure. What 4 year old wants rectal probes in place all day long? Would she be able to tolerate the pain? This was before I knew exactly what was entailed in this testing.
So with all this on my mind, Audrey was admitted on Monday for two days of "cleanout." She could not even tolerate this so it was quickly scrapped. She hasn't been fed in 4 months, how much stool could there be? Wednesday she went to the OR to place the motility probes. They pulled out her GJ tube (which we did not anticipate and it was only 2 weeks old!) and fed a catheter into her jejunum. The catheter has the pressure sensors on then every few cms. They run water through the sensors so as the intestines move, the water is moved and the pressure is measured. Then they placed the same type of catheter (only much longer) into her colon all the way through to where the small intestine and large intestine meet (the cecum for those who know). Each catheter has 8 probes so a total of 16 probes were measuring her activity. She came back to her room and was hooked up to the machine by the GI nurse who stayed with her all day. For 4 hours they just monitor her. This is the "fasting" state. What her intestines do when shes not being fed. At hour 4 she was given IV erythromycin which makes the stomach contract. At hour 5 she was supposed to be fed 8 ounces of formula in 30 minutes in her stomach. This was a huge problem since Audrey has not once in her life consumed 8 ounces at one time, hasn't had feeds at all in 4 months and hasn't tolerated feeds in her stomach for the last 18 months. We comprised to see "as much as she could tolerate." This ended up being 4 ounces in 30 minutes. She was very uncomfortable, nauseous and in pain at this point. At hour 6, a bisacodyl enema was given through the colon probe. We suspected this would work well since this was her home regimen. Sure enough with in 30 minutes her intestines moved. Unfortunately she vomited all of the formula we had given her one hour and a half before and then some. Thankfully at this point the test was over. Now I had to anxiously wait till Thursday to get the results back. Here are some pictures:
This is a picture of the screen reading a very quite tummy.
This is Audrey being entertained
This is the catheter that is in her stomach and jejunum. The little blue caps are attached to each sensor.
Yesterday the motility doctor and her GI doctor came to speak with me about the results. What they found was on its her intestines did very little moving. As you can see in the graph above. Thankfully her stomach did respond to the IV erythromycin and there were a few contractions made into her small bowel from that. Feeding her did not make her intestines do anything at all. The bisacodyl showed the only the very proximal part of her large intestine has movement. The distal part (the descending colon) had no movement ever. Normally the recommendation is for a distal colectomy but Audrey has mitochondrial disease. This is a progressive disease. Cutting out the piece that doesn't work may help right now but in 6 months or 12 months she may need more out and more out. We just don't know. The next option is to do a c-tube. This is where they place a button in the very begining of her large intestine which does have a little movement and through there we can do the enema flushes. This is not a long term option because we know at some point this will stop working. Our last option is to do an ileostomy. This is where we stop using her colon completely and let her stool out from her small intestine. They also found that her g-tube is located too close to the exit of her stomach. This may be contributing to her pain and may be blocking her stomach. The hope is by relieving the "downstream" obstruction in her colon, her stomach and small intestines will function "normally."
This is very hard news to hear. Though I was expecting her to have poor motility. Being told that she will keep losing what little response she has now is heartbreaking. Having to make these decision is impossible. What if it's the wrong decision? What if it only causes her more pain and suffering? With every step we take with Audrey, I am constantly weighing her quality of life. Right now the has spent 3 of the past 4 weeks in the hospital and the one week she was home her stomach was huge and she was getting around the clock pain medicine. So something needs to happen. The plan is now to move forward with the g-tube relocation and to place the c-tube. I am not ready for the colectomy and I don't think she is ready for the ileostomy. Ultimately we may end up doing one or both, I may end up regretting the c-tube. But this is the least invasive we can be with her at this time and I hope that this will buy us time to come up with a better solution for her. I have no illusions that this will fix anything or make her better. But I hope this will make her more comfortable and keep her out of the hospital for longer periods of time.
So with all this on my mind, Audrey was admitted on Monday for two days of "cleanout." She could not even tolerate this so it was quickly scrapped. She hasn't been fed in 4 months, how much stool could there be? Wednesday she went to the OR to place the motility probes. They pulled out her GJ tube (which we did not anticipate and it was only 2 weeks old!) and fed a catheter into her jejunum. The catheter has the pressure sensors on then every few cms. They run water through the sensors so as the intestines move, the water is moved and the pressure is measured. Then they placed the same type of catheter (only much longer) into her colon all the way through to where the small intestine and large intestine meet (the cecum for those who know). Each catheter has 8 probes so a total of 16 probes were measuring her activity. She came back to her room and was hooked up to the machine by the GI nurse who stayed with her all day. For 4 hours they just monitor her. This is the "fasting" state. What her intestines do when shes not being fed. At hour 4 she was given IV erythromycin which makes the stomach contract. At hour 5 she was supposed to be fed 8 ounces of formula in 30 minutes in her stomach. This was a huge problem since Audrey has not once in her life consumed 8 ounces at one time, hasn't had feeds at all in 4 months and hasn't tolerated feeds in her stomach for the last 18 months. We comprised to see "as much as she could tolerate." This ended up being 4 ounces in 30 minutes. She was very uncomfortable, nauseous and in pain at this point. At hour 6, a bisacodyl enema was given through the colon probe. We suspected this would work well since this was her home regimen. Sure enough with in 30 minutes her intestines moved. Unfortunately she vomited all of the formula we had given her one hour and a half before and then some. Thankfully at this point the test was over. Now I had to anxiously wait till Thursday to get the results back. Here are some pictures:
Yesterday the motility doctor and her GI doctor came to speak with me about the results. What they found was on its her intestines did very little moving. As you can see in the graph above. Thankfully her stomach did respond to the IV erythromycin and there were a few contractions made into her small bowel from that. Feeding her did not make her intestines do anything at all. The bisacodyl showed the only the very proximal part of her large intestine has movement. The distal part (the descending colon) had no movement ever. Normally the recommendation is for a distal colectomy but Audrey has mitochondrial disease. This is a progressive disease. Cutting out the piece that doesn't work may help right now but in 6 months or 12 months she may need more out and more out. We just don't know. The next option is to do a c-tube. This is where they place a button in the very begining of her large intestine which does have a little movement and through there we can do the enema flushes. This is not a long term option because we know at some point this will stop working. Our last option is to do an ileostomy. This is where we stop using her colon completely and let her stool out from her small intestine. They also found that her g-tube is located too close to the exit of her stomach. This may be contributing to her pain and may be blocking her stomach. The hope is by relieving the "downstream" obstruction in her colon, her stomach and small intestines will function "normally."
This is very hard news to hear. Though I was expecting her to have poor motility. Being told that she will keep losing what little response she has now is heartbreaking. Having to make these decision is impossible. What if it's the wrong decision? What if it only causes her more pain and suffering? With every step we take with Audrey, I am constantly weighing her quality of life. Right now the has spent 3 of the past 4 weeks in the hospital and the one week she was home her stomach was huge and she was getting around the clock pain medicine. So something needs to happen. The plan is now to move forward with the g-tube relocation and to place the c-tube. I am not ready for the colectomy and I don't think she is ready for the ileostomy. Ultimately we may end up doing one or both, I may end up regretting the c-tube. But this is the least invasive we can be with her at this time and I hope that this will buy us time to come up with a better solution for her. I have no illusions that this will fix anything or make her better. But I hope this will make her more comfortable and keep her out of the hospital for longer periods of time.
Wednesday, October 16, 2013
Pain
I thought I'd take a minute to post about chronic pain in children. I know, as my coworkers can attest, children feel pain. Babies feel pain. Whether born 2 weeks after their due date or 4 months before, they all can feel pain. How they express it and how they cope with it is what varies. A premature infant may only need to be swaddled to cope, or may need huge doses of pain medicine. The fact is everyone feels pain differently and everyone copes differently. As adults we assume that when you have pain you are laying there, crying, unwilling to do anything until the pain is managed properly. But what if you had pain everyday of your life? Would you still refuse to do anything until you received pain control? Even if you knew, adequate pain control will never be reached?
This is our experience this past week. Audrey has chronic neuropathic pain. I can't tell you what she feels on a daily basis. I can say that before she was on her current medication her stomach hurt so bad she couldn't touch it. For those that don't know, neuropathic pain is just what it sounds like, pain in the nerves. There may or may not be a reason for it,there often is not a cure for it. Patients learn to "live with it" while trying various pain meds, anti inflammatories and experimental medications like anti-depressants. This chronic neuropathic pain is very common in mitochondrial disease and can be severely debilitating. We are lucky for now as Audrey's medication, an antidepressant, keeps her pain at a level tolerable to her. Again I don't know what that level is or if it would be tolerable to me but whatever it is she rarely mentions it. So when Audrey says she's in pain I listen to her. When she says she's in pain and needs to go to the hospital, we go. She is not laying there screaming in pain. She is not crying or lethargic. She is restless and she says she hurts. That is enough for me. Audrey has learned to live her life with some degree of pain constantly. This means that if she wants to play with her dolls she will even if it hurts her stomach to sit up. Because her stomach ALWAYS hurts. She has learned she can't wait to feel better to play, she may not feel better. Ever. So when the doctors come in to assess her knowing she is receiving two IV pain medications on top of her normal medications and they see her sitting up playing with her toys but still saying her stomach hurts, believe her. She has learned to cope with more in her 4 short years than most of us will in our life. She has figured out away to deal with how bad she feels everyday but still play and develop as she should. I ask her medical staff to ignore their preconceptions of how she "should" behave when in pain and take her words at face value. This is true not only for Audrey but for anyone who suffers from chronic pain.
This is our experience this past week. Audrey has chronic neuropathic pain. I can't tell you what she feels on a daily basis. I can say that before she was on her current medication her stomach hurt so bad she couldn't touch it. For those that don't know, neuropathic pain is just what it sounds like, pain in the nerves. There may or may not be a reason for it,there often is not a cure for it. Patients learn to "live with it" while trying various pain meds, anti inflammatories and experimental medications like anti-depressants. This chronic neuropathic pain is very common in mitochondrial disease and can be severely debilitating. We are lucky for now as Audrey's medication, an antidepressant, keeps her pain at a level tolerable to her. Again I don't know what that level is or if it would be tolerable to me but whatever it is she rarely mentions it. So when Audrey says she's in pain I listen to her. When she says she's in pain and needs to go to the hospital, we go. She is not laying there screaming in pain. She is not crying or lethargic. She is restless and she says she hurts. That is enough for me. Audrey has learned to live her life with some degree of pain constantly. This means that if she wants to play with her dolls she will even if it hurts her stomach to sit up. Because her stomach ALWAYS hurts. She has learned she can't wait to feel better to play, she may not feel better. Ever. So when the doctors come in to assess her knowing she is receiving two IV pain medications on top of her normal medications and they see her sitting up playing with her toys but still saying her stomach hurts, believe her. She has learned to cope with more in her 4 short years than most of us will in our life. She has figured out away to deal with how bad she feels everyday but still play and develop as she should. I ask her medical staff to ignore their preconceptions of how she "should" behave when in pain and take her words at face value. This is true not only for Audrey but for anyone who suffers from chronic pain.
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