We went to Ottawa to meet with the hepathologist at CHEO on Thursday. One word to sum it up: wow. What an amazing doctor, what an amazing liver team with the dietician and the contact nurse. We feel so well taken care of, once again. Those who complain about the health system in Ontario must not need it as fast as they think they need it. The girls are really sick and we feel we are getting the care they need in a very good time frame.
We sat with the hepathologist before she examined the girls and the biggest issue is the itchiness (caused by the bile acids), which is the most annoying symptom of Alagille syndrome. No word of a lie, the degree of itchiness seems to be going up by the day. The doctor examined the girls and the first thing she noticed is their spider veins on their abdomen, a sign of cirrhosis. Also, the girls' livers are very big and hard, and so are their spleens. She is worried about internal bleeding and wants to make sure the girls are placed on the transplant list earlier than later. In other words, she wants to make sure they are on the list before it becomes an emergency. Blood tests were done on Phuoc. They tried on Binh but were not able to draw any blood. We received a phone call on Friday with Phuoc's preliminary results showing high levels of AST, ALT, Bilirubin and Conjugated Bilirubin. Binh will get another shot at blood tests tomorrow Monday when we see our pediatrician here in Kingston. The doctor also wants a scope done as soon as possible, here in Kingston or at our next visit in Ottawa.
Once we have all of our numbers and data, it looks like we would then be referred to the liver transplant assessment team at Sick Kids in Toronto. The assessment takes 3 days of multiple testings and you end up with a score to place you on the list. If everything stays the same, no complication, it could take 2 years to get an organ. If things get worse and the girls end up in the hospital, their score changes and they go up higher on the priority list. Anyway, this is the way we understood it works.
For sure, a liver transplant will not cure Alagille in the girls. Alagille is there to stay. However, we can hope that with a new and healthy liver, they would do better, particularly with absorption of nutrients. With a transplant starts a whole new game, the rejection issue.
Sunday, February 24, 2013
Tuesday, February 19, 2013
G-Tubes, Here We Are!
Good news, we finally got a surgery date for the girls' g-tubes: March 18 it will be. We are happy since it seems like it will be easier on everyone, starting with the girls. No more tube in the nose, no more tape to hold everything in place, no more checking placement for mom and dad. Each girl has an enlarged liver and spleen which complicates the procedure a wee bit, but we are confident that they will do fine because they are little fighters. They will have a piece of tube hanging out for about 6 weeks until the surgeon installs the Mic-Key button to cover. Something else to learn about. They both gained a lot of weight, each having finally reached 7kg.
We are heading to Ottawa on Thursday to meet with the hepathologist. Michael and I are hoping that the girls will be referred to the transplant team in Toronto for assessment. The itching has increased considerably and it is hard as parents to see them distressed and dependent on meds in order to get a bit of relief. I have heard of biliary diversion (internal and external) to treat pruritus and I would like to hear what the specialist has to say. Also, both Michael and I are willing to see if we could be living liver donors. It will be a very interesting appointment and we are looking forward to it.
The last month has been busy for the Wagner family. We celebrated Tet (Vietnamese New Year), eating yummy food and decorating our home with red lanterns, and everyone has gone back to their usual activities after the Christmas break. Fiona enjoys her tap and karate, Grace her ballet, Rose her Pathfinders and karate, Noah his karate, and the last 3 little boys are busy with just about anything. Liam and Logan go to school half time, and I started to take Toan to a little kids club 2 mornings a week. I officially started my marathon training a month ago and Michael does a lot of his runs with me: it is our little special time. My marathon is scheduled for the end of May, providing that I can remain injury free until then. Since this will be my first one, I don't feel any pressure to beat a personal best, which makes the whole thing fun.
We are already starting to think about what next summer will bring and we have pretty fun ideas for the kids in mind. All in simplicity. Isn't it the best way to go???
I will write another post later on this week after the specialist in Ottawa.
We are heading to Ottawa on Thursday to meet with the hepathologist. Michael and I are hoping that the girls will be referred to the transplant team in Toronto for assessment. The itching has increased considerably and it is hard as parents to see them distressed and dependent on meds in order to get a bit of relief. I have heard of biliary diversion (internal and external) to treat pruritus and I would like to hear what the specialist has to say. Also, both Michael and I are willing to see if we could be living liver donors. It will be a very interesting appointment and we are looking forward to it.
The last month has been busy for the Wagner family. We celebrated Tet (Vietnamese New Year), eating yummy food and decorating our home with red lanterns, and everyone has gone back to their usual activities after the Christmas break. Fiona enjoys her tap and karate, Grace her ballet, Rose her Pathfinders and karate, Noah his karate, and the last 3 little boys are busy with just about anything. Liam and Logan go to school half time, and I started to take Toan to a little kids club 2 mornings a week. I officially started my marathon training a month ago and Michael does a lot of his runs with me: it is our little special time. My marathon is scheduled for the end of May, providing that I can remain injury free until then. Since this will be my first one, I don't feel any pressure to beat a personal best, which makes the whole thing fun.
We are already starting to think about what next summer will bring and we have pretty fun ideas for the kids in mind. All in simplicity. Isn't it the best way to go???
I will write another post later on this week after the specialist in Ottawa.
Monday, February 18, 2013
Wednesday, January 30, 2013
A Dedication to Great Fighters
When I was younger, selfish and immature - and please, don't think I am being hard on myself as I really think at some point I was all of that (I BTW much prefer the person I see in the mirror now than the one I was seeing then) - so I was saying, I remember making a very shallow comment to my then husband-to-be in regards to cancer treatments: I told him that I would never seek treatment, that I would prefer dying with dignity, with my hair on my head. Then he said something about our children - if we were to have any someday - and I think I sort of made a face meaning ''tough luck'' or something equally lacking of any substance. Not one of my most glorifying moments, may I say.
Years went on, children arrived one by one. And maturity set in. I was preggo with number 4 and we were in Montreal. Michael was working on finishing the basement while on leave after an Afghanistan tour. I was pregnant up to my ears, barely able to move and we were listening to the radio. There was a Shave for the Cure event at one children hospital in Montreal. Then the light came on as if I got struck by lightning: I decided that I would do that when I ''grew up''. Six years have gone by without doing anything. My father-in-law I love and respect so much fought cancer 2 years ago but his treatments allowed him to keep his hair. He won a very honourable fight against ''the bad guy''.
2012 and the beginning of 2013 have been defining so far: sickness touching people I love and respect around me and myself being able to thank Life everyday for being lucky enough to be healthy. My daughters are fighting a daily battle against Alagille, my dad has health issues, and very recently, someone in our life has been diagnosed with cancer.
My Friend, I dedicate my new ''hairdo'' to you, to your fight. Please accept this as a token of my respect and support. I am only Joey and I can't do much about your enemy, but I can remind people on a daily basis that it is there, that it is a real battle for far too many. I had a choice, you don't. Let me support you morally, and all the other ones who are fighting on a daily basis, including my two little Vietnamese pearls.
Joey xxx
Years went on, children arrived one by one. And maturity set in. I was preggo with number 4 and we were in Montreal. Michael was working on finishing the basement while on leave after an Afghanistan tour. I was pregnant up to my ears, barely able to move and we were listening to the radio. There was a Shave for the Cure event at one children hospital in Montreal. Then the light came on as if I got struck by lightning: I decided that I would do that when I ''grew up''. Six years have gone by without doing anything. My father-in-law I love and respect so much fought cancer 2 years ago but his treatments allowed him to keep his hair. He won a very honourable fight against ''the bad guy''.
2012 and the beginning of 2013 have been defining so far: sickness touching people I love and respect around me and myself being able to thank Life everyday for being lucky enough to be healthy. My daughters are fighting a daily battle against Alagille, my dad has health issues, and very recently, someone in our life has been diagnosed with cancer.
My Friend, I dedicate my new ''hairdo'' to you, to your fight. Please accept this as a token of my respect and support. I am only Joey and I can't do much about your enemy, but I can remind people on a daily basis that it is there, that it is a real battle for far too many. I had a choice, you don't. Let me support you morally, and all the other ones who are fighting on a daily basis, including my two little Vietnamese pearls.
Joey xxx
Friday, January 25, 2013
Medical Update
It seems like a while since I have given any medical update on the Girlies. Yesterday was a busy day with 3 medical appointments with different specialists here in Kingston.
Genetics:
Results of the genetic testing are back. It is now confirmed 100% that the girls have Alagille Syndrome with JAG1 being the faulty gene. There is a 50 % chance that they may transmit Alagille to their children. Alagille occurs in about 1 in every 70,000 births and is equally common in boys and girls. The geneticist also made a point of warning us that the girls have small blood vessels and that any bump on the head should result in a trip to emerg to make sure there is no bleeding in the brain. Follow-up in a year.
Gastroenterology:
The buildup of bilirubin in the blood is causing the girls to be very itchy. We are starting Benadryl twice a day to see if it helps. Dr. is making a referral to CHEO with hepatology. Also, g-tube should be installed end of Feb, early March. The girls are gaining very well. From 4.480 kg in Nov, Phuoc is now at 6.28 kg. From 4.985 kg in Nov, Binh is now at 6.65 kg. The girls are on continuous feed at night for 10 hours straight, and also receive bolus feedings at meal times. All that is done with the feeding pumps providing the Peptamen. Anything else, which is *very* minimal, going in the mouth is considered to be only of a therapeutic nature, most likely not absorbed by their system. We are hoping that the Ursodiol will drain bile from the liver and that there will eventually be some absorption. They love the taste of butterscotch pudding and sometimes ask to taste whatever we are eating. However, most of the time, they refuse to put food in their mouth. liver disease tends to make people nauseous. They like soothing on a bottle of Peptamen when they go to sleep at night, while receiving from the tube. We are now working on increasing the feed rate at meals to about 210 ml/hour. They are at 55 ml/hour at night. It is very touchy with them since the slightest increase tends to make them puke everything, including all their meds. It looks like liver functions are still good and damage to the liver is minimal. So no transplant in the near future.
Endocrinology:
Results show that calcium and phosphate levels are up and that the healing process for the rickets has started. However, vit D levels are still undetectable for Phuoc (less than 8) and *barely* detectable for Binh (12). We are now looking at the option of intra-muscular vit D. Dr. needs to get back to us on this. We are continuing with 5000 IU of regular vit D and also with semi-activated vit D (alfacalcidol). We need to test levels of vit A, E, K.
The girls are moving around a lot, sitting up, starting to make a lot of sounds. They are doing very well, considering where they were when we arrived in Canada. The main focus for now is nutrition.
Genetics:
Results of the genetic testing are back. It is now confirmed 100% that the girls have Alagille Syndrome with JAG1 being the faulty gene. There is a 50 % chance that they may transmit Alagille to their children. Alagille occurs in about 1 in every 70,000 births and is equally common in boys and girls. The geneticist also made a point of warning us that the girls have small blood vessels and that any bump on the head should result in a trip to emerg to make sure there is no bleeding in the brain. Follow-up in a year.
Gastroenterology:
The buildup of bilirubin in the blood is causing the girls to be very itchy. We are starting Benadryl twice a day to see if it helps. Dr. is making a referral to CHEO with hepatology. Also, g-tube should be installed end of Feb, early March. The girls are gaining very well. From 4.480 kg in Nov, Phuoc is now at 6.28 kg. From 4.985 kg in Nov, Binh is now at 6.65 kg. The girls are on continuous feed at night for 10 hours straight, and also receive bolus feedings at meal times. All that is done with the feeding pumps providing the Peptamen. Anything else, which is *very* minimal, going in the mouth is considered to be only of a therapeutic nature, most likely not absorbed by their system. We are hoping that the Ursodiol will drain bile from the liver and that there will eventually be some absorption. They love the taste of butterscotch pudding and sometimes ask to taste whatever we are eating. However, most of the time, they refuse to put food in their mouth. liver disease tends to make people nauseous. They like soothing on a bottle of Peptamen when they go to sleep at night, while receiving from the tube. We are now working on increasing the feed rate at meals to about 210 ml/hour. They are at 55 ml/hour at night. It is very touchy with them since the slightest increase tends to make them puke everything, including all their meds. It looks like liver functions are still good and damage to the liver is minimal. So no transplant in the near future.
Endocrinology:
Results show that calcium and phosphate levels are up and that the healing process for the rickets has started. However, vit D levels are still undetectable for Phuoc (less than 8) and *barely* detectable for Binh (12). We are now looking at the option of intra-muscular vit D. Dr. needs to get back to us on this. We are continuing with 5000 IU of regular vit D and also with semi-activated vit D (alfacalcidol). We need to test levels of vit A, E, K.
The girls are moving around a lot, sitting up, starting to make a lot of sounds. They are doing very well, considering where they were when we arrived in Canada. The main focus for now is nutrition.
Thursday, January 24, 2013
A Lesson of Compassion
I believe a more appropriate title for this post would be ''All I Ever Needed to Know, I Learned From A Six Year Old.''
My husband and I have a pretty straight forward approach with our children about the realities of our world. We do not sugar coat it, we present the facts as they are. And it has been the case for a long time. Yes, we live in a world that is not always what we hope it would be. We make it our duty to remind our children that we have it very easy, and that all that matters is to love and be loved, to have a shelter over our heads to protect us, clothing and food on our table. I like to thank Life everyday for what it has brought to us. I remember when I was a little girl, I would watch World Vision on tv and feel a calling: how can I help those poor children? I did not have the courage to follow my instincts in my late teens and early twenties as pressure to be like others was too grand. It's only in my thirties that I found the answer to this question, when Life granted me the privilege of being a mother. Luckily, Michael and I woke up and gradually worked on becoming less and less attached to material stuff: mind you, when your family grows faster than your salary, you sort of have to readjust your way of living. And I am grateful we did. Life has a way, a plan.
Our trips to Vietnam have shaped even more who we are now. Our children love hearing the stories we have to share with them. They are thirsty for experiencing what we have experienced. My 3 older sons are especially touched by the homecoming of their little sisters. They are mature enough to share with us the fact that they felt scared and helpless when they first saw them. They were scared to see them leave us definitely, and they grew attached to them very quickly.
My six year old little boy is a very tender soul, I would even call him a humanist. A couple nights ago, as I was doing my ''rounds'' to kiss everyone and tuck everyone in, I heard him as he was sobbing in his bed. Noah's greatest wish is to see everyone on earth with food, shelter and clothes on. He was consumed that night with the fact that this is not the case. ''What can we do? It hurts me so much inside to know that others like Binh and Phuoc need help.'' It is in our plans to bring all the children to Vietnam to explore those roots that are now part of our family. Noah knows it and is looking forward to that big trip someday. ''Maybe I could have a lemonade stand to collect money for the orphans?'' He made me promise we would go to a store in Vietnam and that we would buy food for the Poor.
What do you tell a little boy in front of all that misery, especially when he feels so helpless? I told him that for now, he was doing a big deed by loving his adopted siblings and welcoming them into the family with such kindness and wide open arms. I told him to follow his heart, always, because he has a good heart who wants to do a lot to help others. I told him not to listen to people who tell him he should do otherwise. I told him to listen to the good voice inside of him, that that voice will guide him into doing great things. And I know that for a fact. My son is a good seed of compassion.
I love you my son, and I hope to be the witness of many walking in your footsteps. You are a grand humanist of your generation.
Many of us, the Grand Adults, should be more like my Little Noah.
My husband and I have a pretty straight forward approach with our children about the realities of our world. We do not sugar coat it, we present the facts as they are. And it has been the case for a long time. Yes, we live in a world that is not always what we hope it would be. We make it our duty to remind our children that we have it very easy, and that all that matters is to love and be loved, to have a shelter over our heads to protect us, clothing and food on our table. I like to thank Life everyday for what it has brought to us. I remember when I was a little girl, I would watch World Vision on tv and feel a calling: how can I help those poor children? I did not have the courage to follow my instincts in my late teens and early twenties as pressure to be like others was too grand. It's only in my thirties that I found the answer to this question, when Life granted me the privilege of being a mother. Luckily, Michael and I woke up and gradually worked on becoming less and less attached to material stuff: mind you, when your family grows faster than your salary, you sort of have to readjust your way of living. And I am grateful we did. Life has a way, a plan.
Our trips to Vietnam have shaped even more who we are now. Our children love hearing the stories we have to share with them. They are thirsty for experiencing what we have experienced. My 3 older sons are especially touched by the homecoming of their little sisters. They are mature enough to share with us the fact that they felt scared and helpless when they first saw them. They were scared to see them leave us definitely, and they grew attached to them very quickly.
My six year old little boy is a very tender soul, I would even call him a humanist. A couple nights ago, as I was doing my ''rounds'' to kiss everyone and tuck everyone in, I heard him as he was sobbing in his bed. Noah's greatest wish is to see everyone on earth with food, shelter and clothes on. He was consumed that night with the fact that this is not the case. ''What can we do? It hurts me so much inside to know that others like Binh and Phuoc need help.'' It is in our plans to bring all the children to Vietnam to explore those roots that are now part of our family. Noah knows it and is looking forward to that big trip someday. ''Maybe I could have a lemonade stand to collect money for the orphans?'' He made me promise we would go to a store in Vietnam and that we would buy food for the Poor.
What do you tell a little boy in front of all that misery, especially when he feels so helpless? I told him that for now, he was doing a big deed by loving his adopted siblings and welcoming them into the family with such kindness and wide open arms. I told him to follow his heart, always, because he has a good heart who wants to do a lot to help others. I told him not to listen to people who tell him he should do otherwise. I told him to listen to the good voice inside of him, that that voice will guide him into doing great things. And I know that for a fact. My son is a good seed of compassion.
I love you my son, and I hope to be the witness of many walking in your footsteps. You are a grand humanist of your generation.
Many of us, the Grand Adults, should be more like my Little Noah.
Saturday, January 5, 2013
Two Months Anniversary and GI Update
Today marks our two months adoption anniversary. Looking back at it all, it is quite a blur: so much happened in such a short time, it almost made our heads spin! It is amazing to see how fast we adjusted. The girls are very comfortable and happy in their new environment, the other seven wonders love them, and Michael and I are settling in just fine with our new medical normal. We have ventured out twice so far with the pumps, we just need to fine tune a few little details to make their use easier in public.
Phuoc came right out of her shell. You have to watch her as she rolls all over the house. She can travel quite a distance in a short time. She has a ''silent but deadly'' side in her. Gates and doors became mommy's new obsession: you have to obsess over something...! Phuoc can now even sit up on her own for short periods of time. She loves the ''saucer'' and has the cutest little shy smile. We can work giggles out of her. She seems to be the more independent of the two.
Binh is the little ''needy'' one. She is fine as long as there are people around her. Daddy walks into the room and she claps her hands with a big smile, tongue sticking out: he is her hero! And it makes him feel like a king. She is sturdier sitting up than her sister and loves sitting up in plastic bins.... To each their own. So we have a Binh in a bin. Daddy bought a cute pink one for her. She does not venture out as much as Phuoc yet, but moves around on her back, pushing on her feet. I am thinking I should attach a Swiffer on the back of her shirts. Binh giggles a l-o-t!
In both cases, we are getting chubbier, growing double chins, big thighs and a bum!
On to GI news. The doctor phoned us yesterday to give us more feedback on further results that came back. It looks like damage to both livers is very minimal so far, with no sign of cirrhosis. Very good news. We will be getting g-tubes once the girls a beefier (March???). We are still waiting for genetic testing results to come back to confirm 100% Alagille. GI doctor needs one last blood test to rule out one last suspicion: biopsy showed some indications that they *could* have Alpha-1 antitrypsin deficiency (AAT). We will be meeting with her on January 24 and she will then make a referral to CHEO in Ottawa for a consultation with the hepatologist.
Hubby is going back to work on Monday, children are going back to school as well, mommy will be resuming a normal routine, only with more children now. We loved being together, all 11 of us for the holidays. It will make me sad to see them go on Monday morning! A busy week ahead with dietician home visit on Monday, blood test for the twins and ENT for the younger 3 boys on Tuesday, and dentist with the younger 5 on Friday. Binh and Phuoc will be quite a case for the dentist: a lot of teeth erupted in the last 2 months with the increased nourishment, however, they need a lot of TLC. I have a feeling they will need to be put under for her to do what she needs to do, so I will see if she could do her work when they get their g-tubes installed. More to think about for mommy;)
Phuoc came right out of her shell. You have to watch her as she rolls all over the house. She can travel quite a distance in a short time. She has a ''silent but deadly'' side in her. Gates and doors became mommy's new obsession: you have to obsess over something...! Phuoc can now even sit up on her own for short periods of time. She loves the ''saucer'' and has the cutest little shy smile. We can work giggles out of her. She seems to be the more independent of the two.
Binh is the little ''needy'' one. She is fine as long as there are people around her. Daddy walks into the room and she claps her hands with a big smile, tongue sticking out: he is her hero! And it makes him feel like a king. She is sturdier sitting up than her sister and loves sitting up in plastic bins.... To each their own. So we have a Binh in a bin. Daddy bought a cute pink one for her. She does not venture out as much as Phuoc yet, but moves around on her back, pushing on her feet. I am thinking I should attach a Swiffer on the back of her shirts. Binh giggles a l-o-t!
In both cases, we are getting chubbier, growing double chins, big thighs and a bum!
On to GI news. The doctor phoned us yesterday to give us more feedback on further results that came back. It looks like damage to both livers is very minimal so far, with no sign of cirrhosis. Very good news. We will be getting g-tubes once the girls a beefier (March???). We are still waiting for genetic testing results to come back to confirm 100% Alagille. GI doctor needs one last blood test to rule out one last suspicion: biopsy showed some indications that they *could* have Alpha-1 antitrypsin deficiency (AAT). We will be meeting with her on January 24 and she will then make a referral to CHEO in Ottawa for a consultation with the hepatologist.
Hubby is going back to work on Monday, children are going back to school as well, mommy will be resuming a normal routine, only with more children now. We loved being together, all 11 of us for the holidays. It will make me sad to see them go on Monday morning! A busy week ahead with dietician home visit on Monday, blood test for the twins and ENT for the younger 3 boys on Tuesday, and dentist with the younger 5 on Friday. Binh and Phuoc will be quite a case for the dentist: a lot of teeth erupted in the last 2 months with the increased nourishment, however, they need a lot of TLC. I have a feeling they will need to be put under for her to do what she needs to do, so I will see if she could do her work when they get their g-tubes installed. More to think about for mommy;)
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