I feel like I know as much as it's possible for me to know about endoscopy, portal hypertension, varices and banding.
The most important thing that I have learned is that there is no real way to predict which patients are at risk of developing varices. The only definitive way to find out is by endoscopy. The clinical signs of portal hypertension do not always correlate with the risk of dangerous varices.
Something else I have discovered is that while collateral circulation can develop almost anywhere in the gastrointestinal system, the only varices which are at risk of spontaneous rupture and massive haemmorhage are the ones which develop in the oesophagus. So it seems to just depend on whereabouts the varices happen to develop as to whether a particular patient might be at risk of bleeding. Patients with portal hypertension will almost inevitably develop some collateral circulation, but it might be that is in a 'safe' place.
So what that means for Hannah is that although she does not have many clinical signs of portal hypertension, the fact she has some means that she has probably developed some collateral circulation. It all depends on where that blood is being diverted to as to whether she is at risk of developing varices that will bleed.
Although I trust the recommendations of Hannah's doctor, I also like to think things through for myself and work out how the doctor arrived at that conclusion. That process helps me to ask any relevant questions and clear up what I don't understand beforehand rather than being faced with having to make a quick decision on something without knowing much about it. I trusted the opinion that Hannah has a small risk of dangerous varices, then found out why that is for myself in my own time. It has also reassured me that there is more to this than just my own peace of mind, which was one of my inital doubts.
If Hannah does have those varices, then I feel I want at least the chance of having them treated, even if it is only 50:50. They don't know yet that it's the best thing for children, but they do know it is the best option in adults, so it is not a complete unknown quantity. So we are probably going to agree to take part in the study if Hannah is eligible. If we qualify but fall into the no treatment group, we will be able to set up a plan in the event of a bleed, and the information we provide will help to decide what is the best treatment for Hannah and other children like her in the future.
I have to admit I am feeling pretty scared about this. The chance of Hannah having dangerous varices is less than the chance of her not, and I am doing my very best to remain optimistic and believe we will be in the majority, but when you already have been hit with a 1 in 15000 disease which is the one everyone hopes it isn't, you know that the bad shit can and does happen to you (as indeed it does to everyone).
So I'm frightened. Frightened that Hannah will have those horrible varices, and we will move into a new phase of our life. One much more medically intensive than we have been used to to undergo an as yet unproven treatment, or one where a life threatening haemorrhage is is a real possibility.
We have been very lucky, and very blessed. We have watched the tiny sick yellow baby become a distant memory and Hannah beat the odds in every way. Around 50% of babies born with BA need a transplant in the first two years of life. Around 60% have one or more episode of cholangitis in the first couple of year post Kasai. Many struggle with weight gain and sometimes development issues. Hannah has not had any of those complications so far. We've been more than lucky, and can hope and pray and imagine it will continue, but can never assume it.
In a discussion with her doctor about Hannah's prognosis last year, he told me that she stood a very good chance of reaching age 10 without needing a transplant, in the region of 80%. The biggest obstacle to overcome to reach that is portal hypertension. So what they find or don't find in this endoscopy will affect that outlook to some degree. I hope and pray that they will find absolutely zilch and tell us she doesn't need another endoscopy for 20 years, but until we have got through it I can't help but worry. I really do like my version of a normal life, and I'm pretty sure Hannah does too.
And, of course I am still anxious about that stupid Alk Phos. I really hope they run that as part of the pre-scope bloods and it has come down and I can stop stressing about it. She really has been growing a lot and it is sure to be down to that but there is a little flashing sign in my head saying 'bile duct damage' about a thousand times a day. Silly woman that I am.
I'm not looking forward to the general anaesthetic either. The last one was for the Kasai. I remember every moment as if it was yesterday and I think those memories will be hard to suppress. In the future a general will not be so closely linked in my mind to the Kasai, but for this time it is.
This just shows how we have been so blessed to have such a non medical life over the past two and a half years. This is all relatively minor compared to what we went through with the Kasai and what other liver kids deal with all the time, but the fact it is something major for me is a good thing. We are not so accustomed to medical procedures under anaesthetic that we see them as routine and thank goodness we are only there for a day instead of three months.
I am trusting in God's plan for Hannah, that whatever it may be we will find peace with it.
Tuesday, July 31, 2007
Monday, July 30, 2007
Pants!
I am extremely happy to announce that Hannah has been in pants during the day for a whole week! She has done really well, with only a few puddles and most times they were on the bathroom floor so the intention was there! We have coped with Asda, church, and other outings without mishap so far, so I am hoping this is it.
I think the joy of nappyless daytimes is probably worthy of a post on my own blog, after almost five years without a break!
I'm not going to spoil the ecstatic theme of this post by talking medical stuff, so that will have to wait.
I think the joy of nappyless daytimes is probably worthy of a post on my own blog, after almost five years without a break!
I'm not going to spoil the ecstatic theme of this post by talking medical stuff, so that will have to wait.
Thursday, July 19, 2007
Endoscopy date - already!
The letter came this morning and it is booked for 22nd August. I somehow didn't expect it to be quite so SOON. Crap.
I've been doing some homework and thinking a lot since my post weighing it up. More soon.
I've been doing some homework and thinking a lot since my post weighing it up. More soon.
Friday, July 13, 2007
Blood test results
The news is good! Almost everything was normal or close to it.
Total bilirubin 5 (normal). This measure whether she is getting jaundiced.
ALT 36 (normal)
AST 56 (normal/slightly elevated)
GGT 57 (not sure on the definitive range for this one...some give this value as normal, others as slightly elevated). These three are all markers of liver cell death.
Albumin 40 (normal). This is produced in the liver and measures how well the liver is functioning.
PT 11 (normal). Measures how well blood is clotting.
Platelets 263 (normal). Low values cause easy bruising and bleeding.
Haemoglobin 11.9 (normal). A low value indicated anaemia.
White Cell Count 5.7 (normal). Low value could be due to liver disease, high value commonly due to infection.
The only one that was concerning was her Alk Phos at 1057. This can indicate bile duct blockage or bone damage. That value is pretty high for Hannah - previously it has been 403 (Jan 07), 274 (Oct 06), 275 (May 06). I know (and the nurse reminded me) that it can be elevated in children due to growth, and Hannah has been doing plenty of that lately! The nurse also told me that Alk Phos can be elevated with vitamin deficiency, particularly Vit D. Her vitamin levels are not back yet so we will have to wait and see with that one.
Total bilirubin 5 (normal). This measure whether she is getting jaundiced.
ALT 36 (normal)
AST 56 (normal/slightly elevated)
GGT 57 (not sure on the definitive range for this one...some give this value as normal, others as slightly elevated). These three are all markers of liver cell death.
Albumin 40 (normal). This is produced in the liver and measures how well the liver is functioning.
PT 11 (normal). Measures how well blood is clotting.
Platelets 263 (normal). Low values cause easy bruising and bleeding.
Haemoglobin 11.9 (normal). A low value indicated anaemia.
White Cell Count 5.7 (normal). Low value could be due to liver disease, high value commonly due to infection.
The only one that was concerning was her Alk Phos at 1057. This can indicate bile duct blockage or bone damage. That value is pretty high for Hannah - previously it has been 403 (Jan 07), 274 (Oct 06), 275 (May 06). I know (and the nurse reminded me) that it can be elevated in children due to growth, and Hannah has been doing plenty of that lately! The nurse also told me that Alk Phos can be elevated with vitamin deficiency, particularly Vit D. Her vitamin levels are not back yet so we will have to wait and see with that one.
Thursday, July 12, 2007
Endoscopy and study - thoughts so far
I have been doing a great deal of thinking on the subject of the endoscopy and study. No conclusions yet, but lots of random thoughts that need organising.
An endoscopy is being recommended for Hannah because she has portal hypertension. Portal hypertension is elevated blood pressure in the portal vein which carries blood between the liver and the digestive system. In Hannah's case this is caused by restricted blood flow through the liver because it is damaged. Varices form when the blood tries to find other routes back to the heart. It travels through other veins and these can become overloaded with blood meaning they can burst. A variceal bleed is a life threatening medical emergency. Hannah has been diagnosed with portal hypertension because her liver and spleen are both enlarged. At this stage it is considered to be mild, based on how much her spleen is enlarged.
The thought of a bleed from portal hypertension has been my number one fear since Hannah was diagnosed. A decline towards transplant does not happen overnight, and there would be time to take stock and adjust. The thought of finding Hannah covered in her own blood is the stuff of nightmares. I wish someone had told me in the early days that it is uncommon for varices to develop in the first 18 months of life if the Kasai is working and the liver is functioning. My anxiety levels would have at least halved.
My mind has gone to many dark places in the last 2 years or so on the subject of varices and bleeding. For children with BA and successful Kasai a major bleed can come completely out of the blue. It's often made me wish I can see what was going on inside, to know whether I can put that fear to rest for now. I would have thought I would have jumped at the chance of the endoscopy to find out for sure. Now I am having some doubts.
One of the thoughts I am having is that I don't want Hannah to go through an invasive medical procedure solely for my peace (or anxiety!) of mind. While her doctor is recommending it, the ultimate decision as to whether to go ahead lies with us. I really need to think through the possible outcomes of the endoscopy and whether having it done would be beneficial to Hannah.
If treating any risky varices was the point of the endoscopy, I would have no doubts whatsoever. The problem I am having is that a) if Hannah is not part of the study any varices would not be treated anyway b) if she is part of the study she only stands a 50% chance of being selected for treatment. Perhaps this would be a good point to go into the current protocols and the details of the study.
Currently here in the UK children with varices are not usually treated unless they have a bleed. There are some exceptions to this (such as those based long distances from the liver centre). The treatment is to put a rubber band around the varices which is done by endoscopy. There are drugs to treat portal hypertension but they tend not to work so well in children as in adults.
It has already been established through trials that in adults it is desirable to treat varices before a bleed. At the moment it is unclear whether the same is true for children. This is what the study is seeking to establish.
The way the study will work is that when children are recommended for routine endoscopy they will be invited to take part. To ensure there is no bias the decision to take part is made before the routine endoscopy is carried out. Children are eligible to take part in the study if they are found to have large varices which are at risk of bleeding. They are then divided randomly into two groups; those who will have banding treatment and those who will not. For those who have the treatment, the first banding will be carried out immediately, and further banding will take place until the varices are all gone. There will then be further follow up and banding if necessary for the rest of the two years of the study. The other group will not have any varices banded unless they have a bleed. They will also be followed for two years. One of the things I need to know is what is the plan after the two years are up? If that is not included in the information pack from the hospital then I will definitely ask.
There are so many different scenarios to consider. In some of them the endoscopy seems a no-brainer, in others it seems pointless.
To start with, I am finding it hard to justify going through with if we are not going to agree to be involved in the study. I see two end results - the first, and most likely, being that she does not have any large varices, in which case the endoscopy has served no purpose other than to give peace of mind to the adults involved in her care (and of course me!) The second would be that she was found to have large varices at risk of bleeding. The current protocol means the banding procedure would not be available, at least in the short term, so that would mean spending the next however long in a state of perpetual anxiety about a bleed. I suppose there are some benefits to having knowledge of what is going on, whether or not it is going to be acted on, but that needs to be weighed up against putting Hannah through an invasive procedure.
If we agree to allow Hannah to take part in the study, then there are three end results. Again the most likely is that she is found not to have large varices, and nothing much has been gained apart from peace of mind (not that I am understating how valuable that can be, just weighing it up). If she does have threatening varices, then there is a 50/50 chance that she will not receive any treatment, so again we are back to the major bleed anxiety situation. If she does get picked for the treatment group, then it involves a lot of endoscopies and a medical way of life that we have thankfully become unaccustomed to.
Then, on the other hand, there is the big part of me that says I would move heaven and earth to prevent any possibility of Hannah having a bleed. If that involves some disruption and taking some chances, well, maybe it is worth it. If I were to do nothing, and she had a major bleed, would I forgive myself? I need to do a lot of thinking and praying about this, and Andy and I need to spend some time in discussion.
I know an endoscopy is, when all is said and done, a minor procedure. But I know every parent out there would agree with me in saying that putting your child through any medical procedure is a decision not to be taken lightly.
An endoscopy is being recommended for Hannah because she has portal hypertension. Portal hypertension is elevated blood pressure in the portal vein which carries blood between the liver and the digestive system. In Hannah's case this is caused by restricted blood flow through the liver because it is damaged. Varices form when the blood tries to find other routes back to the heart. It travels through other veins and these can become overloaded with blood meaning they can burst. A variceal bleed is a life threatening medical emergency. Hannah has been diagnosed with portal hypertension because her liver and spleen are both enlarged. At this stage it is considered to be mild, based on how much her spleen is enlarged.
The thought of a bleed from portal hypertension has been my number one fear since Hannah was diagnosed. A decline towards transplant does not happen overnight, and there would be time to take stock and adjust. The thought of finding Hannah covered in her own blood is the stuff of nightmares. I wish someone had told me in the early days that it is uncommon for varices to develop in the first 18 months of life if the Kasai is working and the liver is functioning. My anxiety levels would have at least halved.
My mind has gone to many dark places in the last 2 years or so on the subject of varices and bleeding. For children with BA and successful Kasai a major bleed can come completely out of the blue. It's often made me wish I can see what was going on inside, to know whether I can put that fear to rest for now. I would have thought I would have jumped at the chance of the endoscopy to find out for sure. Now I am having some doubts.
One of the thoughts I am having is that I don't want Hannah to go through an invasive medical procedure solely for my peace (or anxiety!) of mind. While her doctor is recommending it, the ultimate decision as to whether to go ahead lies with us. I really need to think through the possible outcomes of the endoscopy and whether having it done would be beneficial to Hannah.
If treating any risky varices was the point of the endoscopy, I would have no doubts whatsoever. The problem I am having is that a) if Hannah is not part of the study any varices would not be treated anyway b) if she is part of the study she only stands a 50% chance of being selected for treatment. Perhaps this would be a good point to go into the current protocols and the details of the study.
Currently here in the UK children with varices are not usually treated unless they have a bleed. There are some exceptions to this (such as those based long distances from the liver centre). The treatment is to put a rubber band around the varices which is done by endoscopy. There are drugs to treat portal hypertension but they tend not to work so well in children as in adults.
It has already been established through trials that in adults it is desirable to treat varices before a bleed. At the moment it is unclear whether the same is true for children. This is what the study is seeking to establish.
The way the study will work is that when children are recommended for routine endoscopy they will be invited to take part. To ensure there is no bias the decision to take part is made before the routine endoscopy is carried out. Children are eligible to take part in the study if they are found to have large varices which are at risk of bleeding. They are then divided randomly into two groups; those who will have banding treatment and those who will not. For those who have the treatment, the first banding will be carried out immediately, and further banding will take place until the varices are all gone. There will then be further follow up and banding if necessary for the rest of the two years of the study. The other group will not have any varices banded unless they have a bleed. They will also be followed for two years. One of the things I need to know is what is the plan after the two years are up? If that is not included in the information pack from the hospital then I will definitely ask.
There are so many different scenarios to consider. In some of them the endoscopy seems a no-brainer, in others it seems pointless.
To start with, I am finding it hard to justify going through with if we are not going to agree to be involved in the study. I see two end results - the first, and most likely, being that she does not have any large varices, in which case the endoscopy has served no purpose other than to give peace of mind to the adults involved in her care (and of course me!) The second would be that she was found to have large varices at risk of bleeding. The current protocol means the banding procedure would not be available, at least in the short term, so that would mean spending the next however long in a state of perpetual anxiety about a bleed. I suppose there are some benefits to having knowledge of what is going on, whether or not it is going to be acted on, but that needs to be weighed up against putting Hannah through an invasive procedure.
If we agree to allow Hannah to take part in the study, then there are three end results. Again the most likely is that she is found not to have large varices, and nothing much has been gained apart from peace of mind (not that I am understating how valuable that can be, just weighing it up). If she does have threatening varices, then there is a 50/50 chance that she will not receive any treatment, so again we are back to the major bleed anxiety situation. If she does get picked for the treatment group, then it involves a lot of endoscopies and a medical way of life that we have thankfully become unaccustomed to.
Then, on the other hand, there is the big part of me that says I would move heaven and earth to prevent any possibility of Hannah having a bleed. If that involves some disruption and taking some chances, well, maybe it is worth it. If I were to do nothing, and she had a major bleed, would I forgive myself? I need to do a lot of thinking and praying about this, and Andy and I need to spend some time in discussion.
I know an endoscopy is, when all is said and done, a minor procedure. But I know every parent out there would agree with me in saying that putting your child through any medical procedure is a decision not to be taken lightly.
Wednesday, July 11, 2007
Birmingham - the news so far
We had a productive and interesting visit to Birmingham yesterday. Dr McKiernan is very pleased with Hannah's growth and development, and her energy levels. She now weighs 15kg (33 lb) and is 99 cm tall! Nothing was flagged up from her abdominal exam, although he said he couldn't be sure of its accuracy as Hannah was so tickly!
We had her bloods done there - the most comprehensive panel for a long time - so no results on those yet. I can either wait several weeks for the clinic letter to come in the post, or I can phone Liver Direct and get the results from there in a day or so. Guess which option I am choosing........expect an update on those very soon.
I didn't get chance to ask any of my questions about possible worsening of portal hypertension. Dr McKiernan threw me for a loop before I had chance by saying he is keen to do an endoscopy to check for varices in the next few months. Apparently age 3-4 is the most common time for varices to develop. I'm still a bit in shock from this as although I knew that a scope was likely in the future, I just didn't think it would be quite this soon. I have quite a few mixed feelings about this. Although an endoscopy is routine it is still an invasive procedure and requires a general anaesthetic in children. If the endoscopy shows large varices, Hannah will be eligible to take part in a European study on prophylactic treatment of varices. We need to make the decision BEFORE the initial procedure. More on this later.
We had her bloods done there - the most comprehensive panel for a long time - so no results on those yet. I can either wait several weeks for the clinic letter to come in the post, or I can phone Liver Direct and get the results from there in a day or so. Guess which option I am choosing........expect an update on those very soon.
I didn't get chance to ask any of my questions about possible worsening of portal hypertension. Dr McKiernan threw me for a loop before I had chance by saying he is keen to do an endoscopy to check for varices in the next few months. Apparently age 3-4 is the most common time for varices to develop. I'm still a bit in shock from this as although I knew that a scope was likely in the future, I just didn't think it would be quite this soon. I have quite a few mixed feelings about this. Although an endoscopy is routine it is still an invasive procedure and requires a general anaesthetic in children. If the endoscopy shows large varices, Hannah will be eligible to take part in a European study on prophylactic treatment of varices. We need to make the decision BEFORE the initial procedure. More on this later.
Monday, July 09, 2007
Working through the worry
With Hannah's yearly Birmingham check up coming up tomorrow morning the paranoia is cranking up several notches.
Will her bloods be normal? That is always the first worry. Last time her enzymes which indicate liver cell death were higher than the previous result. I hope that's because she was unwell, and that they are the same or lower than then, putting them back into normal range. The enzymes don't tell us how well her liver is working, but the way I see it the lower they are the happier her liver is. Previously the tests which show how well her liver is working (albumin, bilirubin) have been perfectly normal and there's no evidence they will be otherwise this time. If there was a problem with those she would have noticable symptoms - fluid in her abdomen (ascites) or jaundice. Those are the really important ones and along with clotting factors are the ones where abnormal results would indicate we would be thinking about transplant.
The next thing to worry about is whether there is any indication that her portal hypertension has worsened. This would be the case if her spleen is palpable or if her platelets are low. If those are both OK then we should be good to go till next year before she has an ultrasound and possibly endoscopy. If there is an indication it has worsened (I've now also convinced myself she has spider veins on her face, hoping this will be debunked) then those may take place this summer.
Really, the main worry is whether something will happen this visit to detract from our 'normal' life. Sometimes I feel like we have always got the lucky hand when it comes to liver disease and that one day we will be dealt a really shitty one to make up for it. Like our lion has spent so much time asleep that when he wakes up he is going to be full of energy and ready for a fight. Each time we go for a check up and they are so pleased with Hannah's progress I am walking on air, but at the same time feel like I was given a get out of jail free card. When the next appointment is due I can't help but wonder if one day they might all be used up.
So hopefully this time tomorrow I will have an update that says 'stable'. I've opted to have the bloods done there this time so will have to wait another couple of days for those.
Will her bloods be normal? That is always the first worry. Last time her enzymes which indicate liver cell death were higher than the previous result. I hope that's because she was unwell, and that they are the same or lower than then, putting them back into normal range. The enzymes don't tell us how well her liver is working, but the way I see it the lower they are the happier her liver is. Previously the tests which show how well her liver is working (albumin, bilirubin) have been perfectly normal and there's no evidence they will be otherwise this time. If there was a problem with those she would have noticable symptoms - fluid in her abdomen (ascites) or jaundice. Those are the really important ones and along with clotting factors are the ones where abnormal results would indicate we would be thinking about transplant.
The next thing to worry about is whether there is any indication that her portal hypertension has worsened. This would be the case if her spleen is palpable or if her platelets are low. If those are both OK then we should be good to go till next year before she has an ultrasound and possibly endoscopy. If there is an indication it has worsened (I've now also convinced myself she has spider veins on her face, hoping this will be debunked) then those may take place this summer.
Really, the main worry is whether something will happen this visit to detract from our 'normal' life. Sometimes I feel like we have always got the lucky hand when it comes to liver disease and that one day we will be dealt a really shitty one to make up for it. Like our lion has spent so much time asleep that when he wakes up he is going to be full of energy and ready for a fight. Each time we go for a check up and they are so pleased with Hannah's progress I am walking on air, but at the same time feel like I was given a get out of jail free card. When the next appointment is due I can't help but wonder if one day they might all be used up.
So hopefully this time tomorrow I will have an update that says 'stable'. I've opted to have the bloods done there this time so will have to wait another couple of days for those.
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