Pamela and I met with Dr Simon Ling, Sasha’s GI clinical lead. The meeting was a long time coming as we were conflicted by the role of the GI service in the management of Sasha's Alagille. When Sasha vomited blood in the summer about six months before her second cardiac surgery it was attributed to a 'posterior nosebleed gone awry'. There was mention of a possibility it indicated bleeding varices and early or developing portal hypertension but this was not investigated. An investigation would have required endoscopy (defined as a minimally invasive scope that requires some degree of sedation and entails some risk of perforation). Recognizing developing portal hypertension could have been a flag to reassess the advisability and risk of a second palliative cardiac surgery. One of Dr Ling's primary areas of interest is in the early diagnosis of portal hypertension.
Dr Ling is a clinical specialist rather than research specialist however he provided by way of overview that there exists a large data set for adult liver decease but a much smaller data set for children and very little for Alagille kids with serious cardiac defects. He sees the discipline as having tried to apply the adult approach to see if similar but he suggested it appears adult treatment outcomes are not similar with 1-2 year olds.
As mentioned, one of his personal interests is in the early diagnosis of varices and portal hypertension, improving non-invasive measures beyond ultra sound and blood work to help clinicians with this diagnosis and then treating paediatric portal hypertension to prevent the bleeding. The issue among doctors, as he sees it, is: "Why do we look at varices if we cannot treat them." The clinic is creating a simple questionaire to ask patients if they wish to have an endoscopy to confirm varices, considering the risks. Retrospectively Dr Ling confirms that with her liver, “Things were a lot worse than we thought it was with Sasha.” As for family centred care and interprofessional practise: “I am interested in how we manage care across multiple teams. The issue of inconsistent communication comes up with surprising regularity.”
For Dr Ling, Sasha was unique. He was dealing with probabilities and didn't see definite indications the liver was struggling. Even if varices were present, and speaking to whether this was a flag to halt the surgery, he confirmed the liver can tolerate heart surgery with varices. With Sasha, he sees care as getting into a cycle: if we do this step, it solves this problem, then there is another and that becomes the horizon and so on. Especially with multiple care teams and complex issues, "It becomes difficult to step back and reassess."
We left the conversation with Dr Ling with several followup opportunities: his connecting with Bonnie and Margaret about the family story underway as part of interprofessional and family centred care education; us all thinking about research opportunities (which could be in the $55,000 dollar range); and consideration for expanded normalization of the palliative conversation from small bowel cases (referred automatically to palliative care) to multiple organ cases like Sasha.
Showing posts with label GI bleed. Show all posts
Showing posts with label GI bleed. Show all posts
Return of the clots
When we got back from the walk her bag was leaking so we out her in the bath and replaced it, she was not charmed at all. But she managed a few smiles. Pam's brother Kenny dropped by and we walked some nice alleys off Wychwood, so peaceful they are. We bumped into Michelle and Pierre and their two gals. Mimi talked to her sweetly for some time but she stared ahead, in her own little world. Mimi is about 7 and old beyond her years. Will she see her sister, she asked me. I don't know sweetie, we'll see, was all I could muster.
After her bath we went out again a bit later on and she managed a happy grunt when she saw the stroller. This time she lay most of the time, the wind picking up her golden freshly cleaned curls. She looked like an angel asleep. Marcia dropped by and walked out to carry on the stroll however her bag was leaking again so we took her in. The clots keep getting bigger and pushing off the bag. So we replaced it in the bath after her first walk and had to replace it again at 7pm and then patch it again an hour later. Henry dropped by. Reina came over and we made some barbecued salami, steak, sausages and had it with a Greek cucumber salad and roasted fries. All she was interested in was a few bits of a freezie.
She is getting crankier as her lips get bluer and so Dr Goldman suggested we try Tylenol suppositories before dipping into the morphine. We just put her to sleep to Grisman and Garcia's bluegrassy and twangy Not Just for Kids : Freight train, freight train run so fast, Freight train, freight train run so fast, Please dont tell them what train I'm on, They wont know what route I'm gone... Lets hope she sleeps.
Fathers Day, everyday
I got my best card ever yesterday, a pink baby elephant sitting on a bigger purple elephant from Sasha. I never knew that a little card could make me so happy. Sasha loved the picture as well, I guess she forgot what she chose. Being a father has been a new life, the only thing I can compare it to is leaving South Africa and arriving in the whole new world of Canada. As someone famously said, The days are long but the years are short. I cant believe it has only been two years!
Last night Sarah and Betsy visited and Sasha was animated when Betsy turned on the talking Teddy (The wheels on the bus go round and round, Hullo, do you want to play today?) and Sarah discovered her little popup toy, figuring out immediately to push the popups back in. Then it was off to Granny and Gramps to see everyone except Sean and Mark (felled by too much South African Freedom Day sun and sausages). In the car I turned up the tributes to that famous 64 year old and Sash clapped away - but it was not to last, she was so cranky we had to leave after just an hour. Pretty much straight to bed for a quick nap and then the usual game of sit up, bug eyes, pointing NyaNya to the door, napping and getting us running up the stairs at her call. Ah Sash, you have my sleep habits on top of your Alagille itch. We kept monitoring her bag as nothing was coming out, the sign of a big clot getting stuck inside. The big ones can literally push off the ostomy bag.
I cannot lie with her for longer than a few minutes without getting teary, her beautiful golden legs and shoulders are skinny, just a trace of gold fuzz on her arms, as she keeps picking at the gauze covering her PICC and around her belly. She is in #5 diapers because of the size of her tummy but her thighs are smaller so she keeps leaking, Mom says I have had some horrible regression in my kid care skills but same happens to her diaper folds. So we go through all our fitted sheets everyday. Thank goodness for the plastic cover we put over the mattress, not sure why it took us two years to buy one!
Now she sleeps, Mom sleeps down stairs and all is quiet to the drip of the rain outside for the thirsty flowers. That means the red roses on the street will be full and one will be ready for Sasha's headband.
I just remembered, two days ago, I awoke in the morning as her calls seemed just a whee bit more insistent and I found her hanging off the bed, tummy on the edge, feet flailing, fingers gripping the sheet, about to fall down onto the floor. Sash has no interest in standing or walking. She can bear her pink stuffed mini throne chair but the only places she really likes is in our arms and on the bed. And on the bed what I have loved to see over the last few weeks is that she first relearned to roll from side to side and now is pulling her leg over so that she is almost on her belly, her favorite position before the surgery. I got such a kick out of waking up and seeing her sleeping on her tummy with her bum stuck in the air and her legs tightly curled beneath, her arms tight to her sides and her face plastered sideways into the mattress.
Thanks to everyone writing in, it means a great deal as each story is different and it is so important to remember that Alagille ranges very widely in severity and that so many moms and dads have lots to look forward to.
* The beautiful fingers of an Alagille baby....Sasha you are just like my Jordan in so many ways. We love reading about your days and your walks... all our love,
Leisa & Jordan (AGS) - Australia
* My Heart is with you today and I am not real sure ow you get through, but I give you all my strength anf prayers - you are such a great Father and role model for parents of Children with Alagille. Reading Sasha's blog made my heart jump out of my chest - she is so beautiful, she is a true angel.... I am praying that she is content and doing okay along with the rest of your family. I hope that you know how great of a Father you are, you make our family stronger - You are special to us...We hold a little place in our hearts for Sasha and she is included in all my prayers. Love Kristy And family (JIRI AGS)
Thanks, we get strength from our little fighters, our incredible spouses and our supportive family. Pam is amazing, she is now in the waddle stage but still hitting the stairs to deal with the meds I didnt pick up - it has been an evolving feature of our relationship that when one of us is dispirited the other is ready with some sick humour or stay the course support. I am more mobile and able to hold Sash. Pamela is fastidious in keeping Sasha in clean sheets and clothes. We are more of a team than ever before - thanks Sash!
A bloody poignant day
Sasha is bleeding again. We have seen swirls of blood in the ostomy bag and a few small bleeds over the last two weeks but yesterday and today's output with clots again puts her close to how she was in hospital.
So today was precious time together and we started with the kind of email we love from one of the core nursing team at 4D:
"I just wanted to take the opportunity to thank you both so much for creating Sasha's website. It has become a part of my day, to check in a see how everyone is doing and I want to thank you for giving me a way to do that. The photos taken by Heather are absolutely beautiful. Thank you for sharing them. Sasha is in my thoughts everyday. I am so happy that she has been able to spend this time at home with such wonderful, loving parents. My love to all of you...Lauren"
So are we, thank you! Sasha wore her beautiful new red dress from Auntie Jess as we went with Sam for a walk and met Momma Adelle in the alley short cut to St. Clair. Momma is a spry 81 but doesn't look a day over 74 and she came up to me slowly and said, You have the dog, can I take the girl. Momentarily I felt like the father in the Blues Brother scene How much for the girl, the little girl but then the moment passed and Momma and Sasha and Sam and I sashayed down the alley and chatted about her impossible condition. She told me where she lives and beamed when I congratulated her on her rock garden. She has 12 grand children and is recovering from a stroke, just back from rehab. I told her Sasha had two strokes. We all connected on the neighborhood cosmic string of life. She turned to walk home on the next block and turned back and said, Its all in God's hands...
An idylic day, weather-wise, the perfect temp and a smidgin' of cooling breeze. Matt dropped by on his way to tennis with Dad in Barrie. Sasha took a sleep while Mom picked up some pasta. On her return I walked to Urban Fare to get some some tasty treats. Calea Pharmacy guys dropped off 10 more TPN bags and then 6 more metoclopramide IV bags proscribed to curb Sasha's nausea/vomiting and Irene the CCAC nurse came by to do a cap change. Then Granny took Sasha for another walk, we love that Granny and Grampy walk regularly with Sasha as its her favorite thing and we get 25 mins to hang together. We paged Dr Goldman to update him. Pam got the TPN setup by 7pm, we hooked her up first to the meto and then it was sleepy time.
A lovely day except for the peeks under our daughter's beautiful red skirt to see how much of her life energy has drained away.
Pammy is convinced Sasha will pass as she goes into labour. Its her biggest fear. And it is really hard to see how long she can continue growing varices, bursting varices, bleeding... And Pammy's real due date is three weeks away but they think she will be a week or two early. So here we are living and dying at the same time. One way I cope is by talking a lot about it with people. And if Dom or Chris or Paul or mom or dad or Kenny or Lorna arent available I will talk to myself. Its one of those bad habits I picked up while thinking too much. I like to prep my radio interview or academic presentation or client meeting out loud even when there are no radio interviews, you just never know.
I did call in to a radio show a few weeks ago for the first time as the host was casting aspersions about our very good Mayor and I was going to offer my observations that he is a really genuine hard working problem solver who doesnt hug the limelight like our previous embarrassment of a developer's mayor and then I swore in the first sentence and just heard dead air as I was cut off two words later. Left hanging.
So we are similarly waiting, checking under her hood, hoping, our only solace that she is smiling between her whining. We always could say she wasn't in pain, but she was uncomfortable tonight. She woke after a few hours and then again was tossing and turning around 11.30. Another of those firsts I mentioned earlier was that over the last three weeks she has relearned to roll onto her side, hanging her legs out like a weight. Now is rolling 3/4 onto her tummy which is her favorite position with her one leg pulled tightly into her tummy and the bag of browny red fluid sitting between her legs, under the brown summer dress because she doesn't really fit into onesies any more and we want to keep her little fingers away from the raw skin around her bag.
Tonight she played my fingers like an instrument. She has the most amazing fingers which we noticed when at a very young age she started to fan the thin thin paper in the phone book. She loves drums and xylaphones and puts a finger out to be touched. Tonight she ran the edge of her fingers over my fingers, with the touch of a feather. Another new thing over the last few weeks is that she flicks her eyes open and shut like a little game between us.
So today was precious time together and we started with the kind of email we love from one of the core nursing team at 4D:
"I just wanted to take the opportunity to thank you both so much for creating Sasha's website. It has become a part of my day, to check in a see how everyone is doing and I want to thank you for giving me a way to do that. The photos taken by Heather are absolutely beautiful. Thank you for sharing them. Sasha is in my thoughts everyday. I am so happy that she has been able to spend this time at home with such wonderful, loving parents. My love to all of you...Lauren"
So are we, thank you! Sasha wore her beautiful new red dress from Auntie Jess as we went with Sam for a walk and met Momma Adelle in the alley short cut to St. Clair. Momma is a spry 81 but doesn't look a day over 74 and she came up to me slowly and said, You have the dog, can I take the girl. Momentarily I felt like the father in the Blues Brother scene How much for the girl, the little girl but then the moment passed and Momma and Sasha and Sam and I sashayed down the alley and chatted about her impossible condition. She told me where she lives and beamed when I congratulated her on her rock garden. She has 12 grand children and is recovering from a stroke, just back from rehab. I told her Sasha had two strokes. We all connected on the neighborhood cosmic string of life. She turned to walk home on the next block and turned back and said, Its all in God's hands...
An idylic day, weather-wise, the perfect temp and a smidgin' of cooling breeze. Matt dropped by on his way to tennis with Dad in Barrie. Sasha took a sleep while Mom picked up some pasta. On her return I walked to Urban Fare to get some some tasty treats. Calea Pharmacy guys dropped off 10 more TPN bags and then 6 more metoclopramide IV bags proscribed to curb Sasha's nausea/vomiting and Irene the CCAC nurse came by to do a cap change. Then Granny took Sasha for another walk, we love that Granny and Grampy walk regularly with Sasha as its her favorite thing and we get 25 mins to hang together. We paged Dr Goldman to update him. Pam got the TPN setup by 7pm, we hooked her up first to the meto and then it was sleepy time.
A lovely day except for the peeks under our daughter's beautiful red skirt to see how much of her life energy has drained away.
Pammy is convinced Sasha will pass as she goes into labour. Its her biggest fear. And it is really hard to see how long she can continue growing varices, bursting varices, bleeding... And Pammy's real due date is three weeks away but they think she will be a week or two early. So here we are living and dying at the same time. One way I cope is by talking a lot about it with people. And if Dom or Chris or Paul or mom or dad or Kenny or Lorna arent available I will talk to myself. Its one of those bad habits I picked up while thinking too much. I like to prep my radio interview or academic presentation or client meeting out loud even when there are no radio interviews, you just never know.
I did call in to a radio show a few weeks ago for the first time as the host was casting aspersions about our very good Mayor and I was going to offer my observations that he is a really genuine hard working problem solver who doesnt hug the limelight like our previous embarrassment of a developer's mayor and then I swore in the first sentence and just heard dead air as I was cut off two words later. Left hanging.
So we are similarly waiting, checking under her hood, hoping, our only solace that she is smiling between her whining. We always could say she wasn't in pain, but she was uncomfortable tonight. She woke after a few hours and then again was tossing and turning around 11.30. Another of those firsts I mentioned earlier was that over the last three weeks she has relearned to roll onto her side, hanging her legs out like a weight. Now is rolling 3/4 onto her tummy which is her favorite position with her one leg pulled tightly into her tummy and the bag of browny red fluid sitting between her legs, under the brown summer dress because she doesn't really fit into onesies any more and we want to keep her little fingers away from the raw skin around her bag.
Tonight she played my fingers like an instrument. She has the most amazing fingers which we noticed when at a very young age she started to fan the thin thin paper in the phone book. She loves drums and xylaphones and puts a finger out to be touched. Tonight she ran the edge of her fingers over my fingers, with the touch of a feather. Another new thing over the last few weeks is that she flicks her eyes open and shut like a little game between us.
friday on my mind
She awoke uncomfortable and we put her back to sleep. There was no blood in the ostomy bag. I had a mixture of delicious fresh pasta. The food kept arriving and it was good. Sasha slept until about noon.
Jane dropped by baby boy Eli and Reina and Lisa were over and we brought Sasha down to say hi. Meredith dropped by with beautiful Sarah. It was just like old times.
Jeff dropped by to clear up the back. Kenny and Cindy visited. It began to rain again, I walked the plastic troughs I had laid to carry the water away from the house, enjoying the spout of water on the pavement and grass.
She just doesnt feel like sleeping tonight, she is bubbling with new words, moving the milk bottle back and forth between her hands, clasping with one and slapping with the other. Squeeks, exclamations, laughs in her throat as she drank the milk. Put the bottle down, rolled over. Another laugh, pulled at the bottle, repeat. Lorna and Reina are now hanging out while I write.
Jane dropped by baby boy Eli and Reina and Lisa were over and we brought Sasha down to say hi. Meredith dropped by with beautiful Sarah. It was just like old times.
Jeff dropped by to clear up the back. Kenny and Cindy visited. It began to rain again, I walked the plastic troughs I had laid to carry the water away from the house, enjoying the spout of water on the pavement and grass.
She just doesnt feel like sleeping tonight, she is bubbling with new words, moving the milk bottle back and forth between her hands, clasping with one and slapping with the other. Squeeks, exclamations, laughs in her throat as she drank the milk. Put the bottle down, rolled over. Another laugh, pulled at the bottle, repeat. Lorna and Reina are now hanging out while I write.
Today is the day
After five long months at SickKids Hospital in Toronto we are taking Sasha home. Not as we expected and hoped, to continue our lives happily together, but to let Sasha go to sleep in her home, in our bed, surrounded by our love, at the time of her tired body's choosing, in as much comfort as we can provide.
This blog will give us a chance to celebrate her life and record her days at home.
Sasha has a severe form of Alagille Syndrome, born without a pulmonary artery and a very compromised liver that resulted in an early and severe case of portal hyptension.
Despite Sick Kids telling us there was great risk in surgery, her first shunt was a great success and we returned home within two weeks. She developed the severe itchiness that is a mark of Alagille along with an infectious laugh, a love of people and that wonderful baby curiosity. Just as she was starting to walk, we prepared for a second cardiac surgery.
We were bumped a number of times. And the foreboding and fear grew.
Her second surgery on December 14 began like the first, with internal bleeding. However this time the bleeding did not stop after the cardiac surgeon went back in. With the loss of blood to her lower organs, her liver went into failure. After her liver numbers stabilized she started to poo and vomit blood. When surgeons cut her open to see the site of the bleeding, she bled so much they quickly stiched her up. A fistula resulting from opening up her belly and she has been venting tummy fluid and blood for more than 3 months.
After 5 months of hope that some proceedure would right the balance in her little body after her second cardiac surgery, we decided not to go ahead with a TIPPS proceedure that might have stopped her GI bleeds by alleviating the portal hypertension that has developed in hospital (or possibly was lurking before).
When we looked at where she is now, and how far she has come back since the weeks following the surgery, we felt: enough.
We didnt want to put her through another 2-3 proceedures to give her maybe 1 or two more year of life, or not, with a high chance of death on the table, or coma, or confusion where she didnt recognise us for weeks.
Now she knows she is loved, she is not in pain and heartbreakingly she still has smiles and laughter for those around her.
When you enter the world of paliative care, you are experiencing life and death at the same time. We are all living and dying. With Sasha we are trying to live life to the fullest possible even as we know that she likely has only 3-4 days before she will prefer to sleep over waking.
This blog will give us a chance to celebrate her life and record her days at home.
Sasha has a severe form of Alagille Syndrome, born without a pulmonary artery and a very compromised liver that resulted in an early and severe case of portal hyptension.
Despite Sick Kids telling us there was great risk in surgery, her first shunt was a great success and we returned home within two weeks. She developed the severe itchiness that is a mark of Alagille along with an infectious laugh, a love of people and that wonderful baby curiosity. Just as she was starting to walk, we prepared for a second cardiac surgery.
We were bumped a number of times. And the foreboding and fear grew.
Her second surgery on December 14 began like the first, with internal bleeding. However this time the bleeding did not stop after the cardiac surgeon went back in. With the loss of blood to her lower organs, her liver went into failure. After her liver numbers stabilized she started to poo and vomit blood. When surgeons cut her open to see the site of the bleeding, she bled so much they quickly stiched her up. A fistula resulting from opening up her belly and she has been venting tummy fluid and blood for more than 3 months.
After 5 months of hope that some proceedure would right the balance in her little body after her second cardiac surgery, we decided not to go ahead with a TIPPS proceedure that might have stopped her GI bleeds by alleviating the portal hypertension that has developed in hospital (or possibly was lurking before).
When we looked at where she is now, and how far she has come back since the weeks following the surgery, we felt: enough.
We didnt want to put her through another 2-3 proceedures to give her maybe 1 or two more year of life, or not, with a high chance of death on the table, or coma, or confusion where she didnt recognise us for weeks.
Now she knows she is loved, she is not in pain and heartbreakingly she still has smiles and laughter for those around her.
When you enter the world of paliative care, you are experiencing life and death at the same time. We are all living and dying. With Sasha we are trying to live life to the fullest possible even as we know that she likely has only 3-4 days before she will prefer to sleep over waking.
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