"Height: 99.1 cm (91st centile)
Weight 15.0 kg (75th centile)
It was lovely to see Hannah in the outpatient clinic on 10 July 2007. She is full of life and energy and she looks extraordinarily well and is clearly making excellent physical and developmental progress.
Her ultrasound has shown a significant degree of splenomegaly so we discussed surveillance endoscopy. I will arrange an admission for this.
We also discussed what to do if she should have oesophageal varices and I will give the parents some information on a clinical trial of prophylactic banding.
In the normal course of things I would try and stop her Ursodeoxycholic Acid but in view of the itch I think it is reasonable to continue with this.
Investigations:
Haemoglobin = 11.9
White count = 5.7
Neutrophils = 1.7
Platelets = 263
PT = 11 secs
APTT = 35 secs
Fibrinogen = 2.1
Sodium = 139
Potassium = 4.0
Urea = 3.6
Creatinine = 40
Calcium = 2.62
Phosphate = 1.60
Magnesium = 0.84
Bilirubin = 5
Unconjugated bilirubin = 5
Alk. Phos. = 1057
ALT = 36
AST = 56
Gamma GT = 57
Albumin = 40
Total Protein = 70
She will be admitted on 22 August as a day case for endoscopy and will be reviewed here in one year's time or sooner if necessary."
Friday, August 03, 2007
Tuesday, July 31, 2007
More on endoscopy
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.
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.
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.
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