Hannah's Hope

Quick update

2008-03-23T20:23:00.006Z

There has been nothing notable to report since my last update, but I thought it was time to stop in and say that no news from me is invariably good news. Hannah is her usual sassy self and there have been no appointments or tests to comment on. So all is good. I can use my favourite words 'normal' and 'stable'.

Ooh, how could I forget?!? Hannah is now officially a cover girl! She appears on the front cover of the Winter 07/08 issue of the CLDF's Delivery Magazine.

Here is a link to the pdf version of the magazine. As usual she was filthy (covered in paint from the creche at the conference and chocolate from a cake she had just eaten) but it's a picture that truly shows her mischievous personality and that spark of vitality she has.

Reminder - every day is a gift

2007-12-04T15:46:00.001Z

It's a while since I updated, but there hasn't really been anything new to report. That's good, I've had a few weeks where it's been easy to pretend that liver disease isn't part of our family life :) Hannah has continued to thrive at nursery - she seems to be friends with everyone and at the parents' meeting her teachers said she listens really well! Why doesn't she listen to me?!? She wants to be able to read and write like Caitlin, so she makes up stories to go with the pictures in books and can do a great 'H' to mark things with her name. It will be time to get the hankies out next week when she does her nativity, and then again for Caitlin's the following week.

Hannah is at clinic at the local hospital on Friday (the one where the doctor asks me how she is doing....) and we had her bloods drawn today. I have also been writing an article about BA; not a personal perspective, but a factual one. As I have been writing, I have been thinking that this disease I am writing about sounds really depressing. And it does. I started to write something about long term outlook and survival and thought, I don't want to write about the fact that there are survival statistics, that some children die. I want to write about the hope that there is, not the worst imaginable. But it would be a lie, and an injustice to the children who have died and their families, especially those who are in my heart and I call friends. I want to write about the hope to protect myself from the reality of the darkest place a parent can go. Up until then, I was writing in a fairly dispassionate way; it was only when I got to that survival part that I thought about those precious children and then CRAP! - my daughter has this, and I want to make it sound like it's all going to be ok.

So I left what I was writing, and came here to write about feelings not facts. As I logged in, I saw how many weeks it was since I updated, and then started off recording that the reason I haven't been here is 'normal life' and a happy, thriving three year old. So what started off as a tight little knot of fear has dissolved into an appreciation of everyday life with Hannah and a reminder that it is something to be celebrated here more regularly.

Three years today since Kasai

2007-10-12T21:39:00.000+01:00

...and there is so much to celebrate and be thankful for. Three years of answered prayers.

A day to appreciate being at home just getting on with life, to enjoy the fact that the children have no idea of the significance of the day.

A day to look at Hannah, to hold her close, and give thanks for my miracle.

First Day at Nursery

2007-09-26T19:55:00.000+01:00

Hannah started at nursery today! She was very excited beforehand as Caitlin has been telling her all about what to expect. She was thrilled to wear a uniform and be just like her big sister. Today's session was only an hour with half the children there as an induction, and the parents stayed too. They build it up for the next two days then the full sessions (2.5 hours) start on Monday.

Hannah had a great time and didn't want to leave at the end! She managed to cram an awful lot of busyness into an hour, and already has plans for what she is going to do tomorrow!

I can't believe how grown up she looks in her uniform...and I feel sad and excited at the same time that I am going to have every weekday morning to myself....my baby is growing up too fast!

Hannah is 3!!!!!

2007-08-26T20:34:00.000+01:00

HAPPY BIRTHDAY HANNAH!!!

When you have a child with a chronic illness, birthdays are a strange entity. A reminder of the miracle that is in front of you, a lesson that nothing in life is to be taken for granted. A time for great joy, but also a time when the fragility of life is markedly obvious.

One year ago today, I posted with the wonderful news that Hannah had made it to two years old, a milestone that she would never have reached without life saving surgery. Not only that, but she reached that milestone by hurtling full on towards it, not by stumbling along. And now, we have had another whole year of amazing and wonderful health. The future will always be uncertain, but there will never be any doubt about the blessings that we have had. Thank you God for Hannah, for everything she has taught me, for the many tears I have cried and for the many times when she has brought me sweet and unbridled joy. Thank you for my miracle, and help me not to take it for granted.

Blood results from Tuesday

2007-08-25T20:11:00.000+01:00

Hannah's blood results have come back and they are pretty stable....some in the right direction and some in the wrong direction, but overall pretty good :)

I phoned up for them so didn't get the full panel, they should come with the letter. Here are the ones I have, with commentary.

Albumin 45 (previous result 40) Albumin is a protein made in the liver, and if it isn't working well enough the albumin will be low. This is actually Hannah's highest EVER albumin, so great news!

Alk Phos 677 (previous result 1057) This can measure bile duct damage or bone damage, but can be elevated in children when their bones are growing. I'm very relieved it has dropped down so much! It's back within BCH's normal range for Hannah's age group (250-850), although still higher than Hannah's usual, so hopefully it has all been down to her growing so much recently and will continue to drop.

ALT 68 (previous result 36) ALT measures liver cell damage. Not so happy with this one, because as you can see it has jumped up since the last test to above normal range (less than 45). It is also the highest Hannah's ALT has been for over 2 years. However, it is still a good value for someone with chronic liver disease. Hoping it was a one off jump and not the start of an upwards trend.

AST 67 (previous result 56) AST also measures liver cell damage but as it is found in other parts of the body it is less specific to the liver than ALT. Again not overjoyed with the upward trend and above normal value, but like the ALT it is really not THAT high.

Bilirubin 7 (previous result 5) Low bili means no jaundice! This has hovered between 5 and 8 from a couple of months post Kasai. Normal is less than 20, so this is my favourite result. Pre Kasai my little yellow baby had a bilirubin of over 200!

Gamma GT 51 (previous result 57) Gamma GT is very specific to bile duct obstruction. Hannah's GGT is very good for someone with a bile duct disorder. The similar results mean I can use one of my favourite words - stable.

PT 11 seconds (previous result 11 seconds) This is the most important indicator of Hannah's ability to clot blood, and it is completely normal. This reaffirms that Hannah's liver is doing a very good job!

APTT 39 seconds (previous result 35 seconds) This is another blood clotting indicator. The last value of 35 was at the upper limit of normal, and the fact it is now prolonged means that her liver probably is not producing quite enough of certain clotting factors. On it's own though, it is not a concern, as long as the PT stays normal.

No varices!!!!

2007-08-22T21:32:00.000+01:00

Yes, that's right, no varices!!!! I am so happy!

Today went really well. Hannah was a little star from start to finish, and viewed everything as a new adventure. She was very impressed by the sticky plastic covering the little blobs of numbing cream on her hands in preparation for the cannula. She held her hands out in front of her for about half an hour and wouldn't use them, and she kept prodding at the squishy bits with the cream underneath. She got through the whole day without shedding a tear, even when the cannula was put in and taken out (although I think it helped a lot that we wouldn't let her look). She recovered very quickly from her anaesthetic too.....after 10 minutes she was drinking juice, after 25 minutes she was scoffing sandwiches, crisps and a chocolate bar, and after and hour she was asking if she could go to the hospital outdoor play area.

It was about an hour and a half afterwards that the doctor came and told us there were NO VARICES!!!! I have had this worry in the back of my mind for so long that it is hard to believe it has been put to rest for now. Praise God!

We are still left with a nagging little worry. The doctor decided to take some biopsy samples of the gastrointestinal tract during the endoscopy. We are not entirely clear on the reasoning for this, whether it is routine or not, and what they may or may not be looking for. I am not good at coming up with questions when I have surprise news, so I didn't say much apart from 'I see' which I don't really. The biopsy results will take a while to come back (possibly a couple of weeks) and Dr McKiernan is away at the moment so I'm not sure when we will be able to find out more. I don't have her blood results (I did ask and it was agreed to, but the doc obviously didn't remember and neither did I until after we left) so I will be ringing tomorrow to get those, and will see if I can find anything out then.

No varices! No varices! No varices!

It's the big day tomorrow!

2007-08-21T19:57:00.000+01:00

I can't believe how fast the last few weeks have gone. At least the waiting is now over and this time tomorrow at least we will know one way or the other.

We went over to Birmingham for the bloods today. It seemed a bit weird turning up with no forms for the bloods; I found myself thinking we would get there and they would say "Hannah WHO???" Of course the forms were waiting in phlebotomy. Amazingly we went almost straight in (number 10 was in there and we were number 11) which I think was a lot to do with my choice of going at lunchtime when there weren't any clinics running.

Hannah has bloods frequently enough that she knows what to expect, but not frequently enough to make them a matter of course. It was so much easier when she was a baby and just angry that they were sticking a needle in her and then it was forgotten. She told me in no uncertain terms beforehand that she doesn't like getting her arm checked. She was fine until we got in there and then she started struggling and saying "I don't want you to do it". We just got on with it as quickly as possible, and while the needle was in her arm she kept saying "PLEASE take it out now, PLEASE" through floods of tears. As soon as it was over and she was introduced to a singing lobster and got her stickers she was all smiles again.

We stayed at the hospital for lunch and spent some time after at the outdoor play area. Hannah has the view that the hospital is really quite a fun place (apart from the arm checking) and I want to promote that as much as possible. She doesn't remember all that she went through as a baby of course and things have been so stable since that she has not had the opportunity to build up any fear.

Everything is ready for the extraordinarily early start tomorrow. We have read 'Katie goes to the hospital' (for an ear operation) several times and Hannah understands that she won't be allowed to eat or drink and that she will be put to sleep while the doctors take pictures of the inside of her tummy with a camera. She asked if I would leave her on her own at the hospital and I said I would be with her all the time, except I wouldn't be allowed in the room while they take the pictures. She is also prepared that she may have to stay overnight (hopefully not, but nothing is a given). I've packed for an overnight stay as if I didn't that would guarantee we would have to. And I checked out the location of the medical day care centre so we know exactly where we are going.

So, we're as ready as we will ever be. Hopefully the next time I update I will be able to say Hannah's labs are great and most importantly that she has no varices.

One week to go

2007-08-14T21:58:00.000+01:00

This time next week Hannah will have had her bloods done and we will be gearing up for the endoscopy. I will be so thankful to get it over with and know one way or the other. I so want everything to be ok and to return to our normal sense of denial for another year (barring scary unexplained fevers, but I have learned to live with those). A flu jab and the pointless check up at the local hospital will not do too much damage to that.

I have only admitted this to one person before, but ever since Hannah's kasai I have had a horrible irrational feeling that portal hypertension will be her downfall, if you like, that her liver will keep on coping (I feel sure Hannah's liver has exactly the same fighting spirit that she has herself) but the PH will be what causes her complications or sends her to transplant. Nothing at all to back that up, so depending on what happens in the future that feeling will either be proven to be an incredible case of mum's intuition, or more likely a ridiculous paranoia. I really want it to be debunked for at least the immediate future.

Clinic letter and full list of bloods

2007-08-03T20:08:00.000+01:00

"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."

More on endoscopy

2007-07-31T20:14:00.000+01:00

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.

Pants!

2007-07-30T21:30:00.000+01:00

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.

Endoscopy date - already!

2007-07-19T22:26:00.001+01:00

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.

Blood test results

2007-07-13T12:51:00.001+01:00

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.

Endoscopy and study - thoughts so far

2007-07-12T19:47:00.000+01:00

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.

Birmingham - the news so far

2007-07-11T16:21:00.000+01:00

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.

Working through the worry

2007-07-09T16:45:00.000+01:00

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.

Date through for Birmingham clinic

2007-06-04T21:50:00.000+01:00

The letter came through today for Hannah's annual check up at Birmingham. It is the morning of Tuesday 10th July. She is not scheduled for an ultrasound or anything, but she is due for bloods. I am going to get them done there which means we won't have them on the day, but I'd rather that as I want to make sure her vitamins and the bile acid test get done. I can just imagine the fun (!) of trying to sort out getting those tests done round here, and I'd prefer for all tests to be done at once.

I hope we get to see Dr McKiernan as based on previous experience I will learn an awful lot more than if we see a registrar. I have a few little niggles I want to ask about. She seems to get tired more easily than she used to, but I don't know if that might be 'normal' rather than 'liver' as she is starting to grow out of her daytime naps. I also think she is starting to get some small spider veins on her face, and I want to ask if there is any plan if her labs this time have trended upwards again (there was a very slight upwards trend on the last lot but they were done because she was unwell so that could be why).

Well, with 5 weeks to go it looks like I better start work on my lists of questions. When we only go there once a year and the local checkups involve the doctor asking me how she is doing, then I make the most of it! I will have two - a registrar one and a Dr McKiernan one!

Quick update

2007-05-23T21:18:00.000+01:00

There isn't too much to report. Hannah is still enjoying pre-school. Health wise she has not had any appointments or check ups, although she wasn't well with a virus at the beginning of last week and since then has been tired and extra itchy. Yesterday I noticed the skin is peeling off all her fingertips. I haven't decided yet if it is something I need to worry about or not.

Hannah will be three in August and I am wondering if I will ever have the strength to get her dummy off her. She has a thousand times as much willpower as me. She is not allowed to take it out of the house but it is a constant fixture in her mouth when she is at home. When we get back from our holiday in June I will have to tackle that and give her a bit more of a push towards potty training.

Hannah is due for her yearly check up in Birmingham in July. She will have bloods done and I think her vitamin levels and bile acids checked.

After a long battle, I have finally won disability living allowance care component for Hannah. This has been awarded on the ground of monitoring her medical condition for life threatening changes and also the treatments she needs for her itching.

I must make an effort to add more pictures of Hannah to this blog, and to finish off her medical history.

Nursery Place and the scar

2007-03-29T21:31:00.000+01:00

We had the letter in the post this morning telling us that Hannah has been accepted for a morning place at nursery for next September - hurrah! Even better, so have her friends Caitlin (why does she have a friend with the same name as her sister?), Alfie and Aoife.

Hannah has been enjoying pre-school. Every time she goes she tells me she has "made lots of new friends" so she is obviously a social butterfly!

Today Hannah noticed her scar for the first time - she has been completely oblivious to it up until now. She was complaining of an itchy tummy and suddenly spotted it as we were rubbing her Vitamin E cream in. "What's that?" she asked. "Is it a band?"

"No, that's your scar." I said. "The doctors had to make a hole in your tummy when you were a baby to fix it."

"Why did the doctors have to fix my tummy?"

"Because it didn't work properly"

"Why didn't it work properly?"

I struggled with putting that one in her terms. Eventually I settled with

"Because you were yellow"

"But why did they have to fix it?"

"Because otherwise you would have got more and more poorly. That's why the doctors check your tummy and your arm [Hannah calls a blood test 'checking her arm'], to make sure you are not getting poorly again"

"I know THAT!"

End of conversation for now. I knew I would have to deal with questions about this one day, but hoped I could put it off as long as possible. It's only going to get harder as she gets older and more articulate. I've always tried to be very honest with my kids, particularly about things which are unpleasant. Not brutal, but honest. If they are getting a vaccination, I tell them. Not days in advance, so they can work themselves into a frenzy, but a couple of hours, so they have time to discuss the forthcoming unpleasant event and prepare themselves a little. Far preferable in my book to someone grabbing them and sticking a needle in them without warning. I don't tell them it won't hurt either. I tell them it will hurt a little bit, and they might cry, but it will be over quickly and then they can have a sweet. So when Hannah starts to ask more about her illness, I will have answer her honestly within the bounds of her age. And Caitlin too, and that breaks my heart just as much. The girls fight a great deal but also have a wonderful bond and love each other very openly and very dearly.

Hannah's grandad (my dad)is in hospital at the moment with an undiagnosed heart problem. Also my dear friend Amanda is undergoing life saving surgery today. And some of our other friends at Liver Families are also having very difficult times at the moment with children in serious or critical condition. They are all very much at the front of my mind today.

(Perhaps the last paragraph is an indication that I am ready to start back on my own blog..)

A scary moment

2007-03-22T11:45:00.000Z

Hannah gave me a big scare (fortunately a brief one) on Monday evening. Just after 10pm, she started crying inconsolably in bed. Andy tried to settle her, but she just kept crying, and I went up to her. She was hot again (39.3C), saying her tummy hurt (ow, ow) and shaking. I really did not like the look of her, and with the fact that she had a pale poo that morning there was far too much going on that could be cholangitis. I had given her 5 ml of Calpol but did not want to hang around and see if it took effect.

Hannah and I headed off to the hospital. By the time we got there I was beginning to feel like the foolish paranoid parent as it was clear that Hannah was making a remarkable improvement. So we waited for an hour to see the triage nurse, then another hour to see the doctor. By this time she was singing, looking at books and colouring (we have a nice pink Darth Vader now). I wish I'd given up and gone home; when we eventually saw the doctor and he asked what the problem was with Hannah, my answer was not a whole lot and he was in complete agreement. So thankfully we went back home without bloods or further investigation, and Hannah has been perfectly fine ever since.

With two mini cholangitis alerts in the last couple of weeks I am hoping this is not a case of things coming in threes. I am very thankful that these episodes have turned out to be just normal childhood stuff, but they have reminded me that our 'normal life' will always be precarious.

Pre-school and a virus

2007-03-16T10:10:00.000Z

Hannah started at pre-school on 28th February, and she has settled in really well! I was worried she might be clingy and not want me to go but she has been absolutely fine. She is going on Wednesday and Friday mornings from 8:40 - 11:40 (see me getting chance to update her blog on a Friday morning!)

Hannah gave me a scare on Monday of this week. After seeming fine first thing in the morning she came over all tired and lethargic, and her temperature was 40C! With no other symptoms I was on alert for ascending cholangitis (a bacterial infection of the bile ducts, which Hannah is at risk of due to her reconstructed biliary anatomy). Fortunately the fever did have some response to Calpol and I kept a close eye on her until Tuesday morning, when it started to come down; as usual at the point where I was just thinking of packing a bag for the hospital. It is highly unlikely a fever caused by cholangitis would begin to come down of its own accord, so I started to breathe a little easier. Then, on Tuesday evening, Hannah began to develop a rash of fine red spots. By Wednesday morning she was covered in them! Although the rash passed the meningitis pressure test (ie it faded under pressure) I thought it best to get her down to the GP for it to be checked out. Fortunately he confirmed that it was a harmless virus. Although she was much better yesterday there were still traces of the rash and her temperature was still very slightly up, so we stayed away from toddler group. Today she is completely recovered, so to our mutual delight she has gone off to pre-school this morning.

Hannah's next big event is hearing whether she gets in to nursery next September (she should do, as they give priority to children with siblings already attending the school), whether she has been given mornings (preferred) or afternoons, and what session her friends have been given too!

Less itchy, apricots, and pre-school soon

2007-02-10T19:28:00.000Z

Since I last posted Hannah's itching has gradually improved back to the normal level and her poos are also less icky. That is, apart from the one she did last week that consisted of pure apricots! That gave me quite a shock. Hannah picked up on my excitement and kept asking for more apricots so she could do 'another apricot poo'. Good heavens.

It is only just over two weeks until my baby girl starts at pre-school! We have talked it through....mummy is going to stay for a little bit and then go home and come and pick her up later. She will go on Wednesday and Friday mornings from 8:40 to 11:40. I have also put her application in for nursery next September. It is very scary how quickly it comes round to Hannah's turn to doing things that Caitlin has only just finished.

Hannah is still very two and very terrible, but full of life and joy, and I have discovered that nothing improves her behaviour more than removing her dummy and teddy (oh cruel mother). She loves singing (or songing as she calls it) and dancing. When we have song time at toddler group she jumps up after each one has finished and shouts 'Again, again!'. I'm sure she is destined for a career in showbusiness as she loves nothing more than to perform to an audience!

Lab Results Back

2007-01-12T11:10:00.000Z

Nothing showed up, and the bloods are fine (although trying not to think about slight increase in Alk Phos (403 from 274) and ALT (40 from 20)), so no conclusion apart from random minor virus. I'll be keeping an eye on Hannah though. A few people from Liver Families have mentioned that their child's itching is worse in the winter, so maybe it's a climate thing.

Normal ranges for our lab in brackets.

FBC
Haemoglobin 11.6 (11.5-13.5)
MCHC 32.0 (31.5-34.5)
RBC count 4.36 (4.00-5.20)
MCV 83.0 (75.0-87.0)
Total white cell count 9.16 (5.00-17.00)
Platelets 294 (140-400)
Eosinophil 0.23 (0.10-1.00)
Monocyte 0.61 (0.20-1.00)
Lymphocyte 4.30 (6.00-9.00)
Basophil 0.07 (0.01-0.10)
Neutrophil 3.95 (1.50-8.50)
Haematocrit 0.360 (0.340-0.400)

Urea and electrolytes
Sodium 140 (135-143)
Potassium 4.1 (3.7-5.0)
Urea 5.5 (2.6-6.6)
Creatinine 53 (50-105)

LFT
Total Protein 69 (64-79)
Albumin 39 (34-48)
Total Bili 6 (4-20)
Alk Phos 403 (not given)
ALT 40 (5-38)

Hannah not well

2007-01-09T11:32:00.000Z

I've been worried about Hannah for the past few days. Although she still has plenty of energy and is eating normally, she is significantly more itchy than normal, is pale with dark circles under her eyes, and her poos are foul smelling with mucus in. I took her to the doctor yesterday, and he thinks she probably has a virus as her glands are swollen in her neck, but he sent her for bloods and we have taken a stool sample in today to check for anything yucky. Bloods should be back Thursday. This was the first time that Hannah was really negative about having her blood taken (I don't want to be brave....it made my heart ache) and that she has cried significantly. Probably because she is finally getting old enough to remember what happened last time, as we have been lucky enough for her bloods to be fairly infrequent.

On a more positive note, I meant to update back in December that Hannah excelled at her 2.5 yr development check. The health visitor could not believe that this was the same child whose file she read before the appointment. When Hannah showed she can talk in sentences and could name the colours of the blocks as well as stack them, she said we may as well move straight to the 3 year check! Hannah is 90th centile for height and 50th for weight, so physical development is going great too.

Also, Hannah is starting two mornings of pre-school at the end of February...I'm hoping she takes to it as she can be a bit clingy. I think she will enjoy it if she can get over the fact that mummy is going to leave her there.....

Will update later this week when we have some results back.

Clinic and bloods fine

2006-11-04T20:49:00.000Z

The check up yesterday went very well. No massive spleen (it was never that likely)and bloods normal (Bilirubin 4, ALT 20, Alk Phos 274). We successfully negotiated the new hospital, including the bus. I was very impressed that we got a bleeper at the pharmacy for when the prescription was ready (he prescribed an antibiotic ointment for a sore she has developed on her chin), and that there was somewhere decent to get a snack and a coffee.

I have been holding my girl extra close today. One of our friends (and I do consider them friends) from Liver Families lost her baby boy last night. He has been very ill for several weeks and got too sick to be transplanted.

Having a child who is ill, and knowing people whose children are even more ill, or have died, makes you see your own children in a new light. You still get cross with them, but you also appreciate the little things in a way no one who hasn't been there could understand. I will be tucking Hannah up in bed at night, and I say a prayer of thanks that it is her own bed and not a hospital bed. Today I am just thankful that my children are here for me to hold.

Brave Girl

2006-10-27T21:11:00.000+01:00

Hannah had her six-monthly blood test today. I was so proud of my brave girl, who despite flinching a few times did not make a sound or shed a single tear. Once it was over ("I can have my sweeties now?")the production of a lollipop and a Winnie the Pooh 'braveness' certificate was enough to make the whole thing worthwhile for her.

We shall get the results back when we go to Dr Coad's clinic in a week.

Mainly Portal Hypertension

2006-10-11T20:01:00.000+01:00

I emailed Dr McKiernan last night to clear up the questions I had following Hannah's ultrasound. I had hoped to catch him at the CLDF Conference last Saturday but didn't get aorund to it. What I did get chance to do was to thank Professor Millar personally for the clearly excellent job he did with Hannah's Kasai, and to show him just how lively and full of beans she is.

Back to Dr McKiernan....kudos to him for emailing me back straight away first thing this morning! Sadly, Hannah's enlarged spleen does mean she has some degree of portal hypertension. The fact that the spleen cannot be felt means that it is not too severe. He doesn't think there is a need to rush to check her platelets unless she is bruising more (an enlarged spleen can cause low platelets....the spleen sort of eats them up and the bigger it is the more it eats up....and low platelets can mean easy bruising and bleeding) and an endoscopy to check for oesophageal varices is not really indicated at this point....if her spleen gets large enough to be felt then that will be the plan.

Portal hypertension is very common in patients with biliary atresia (at the CLDF conference we were given a figure of 70-80%) and not all patients with portal hypertension bleed from varices...I know of a number of people who have had portal hypertension for a long time with no complications. I had also suspected that Hannah might have it, as she has some visible veins on her abdomen. Nevertheless I still feel pretty gutted. I did hope that Hannah would be one of those who didn't develop portal hypertension.....it blemishes our illusion of normality a little.

It doesn't really change anything though...as things stand, there is not any need to change the way her condition is managed, unless things get worse....still one day at a time, and something specific to pray about. I spoke to Father Tim yesterday about whether Hannah should remain on the 'prayers for the sick' list at church, when she is climbing around everywhere of a Sunday morning (my thoughts, not his). I said that although she is doing so well, I feel strongly that the prayers of our church community are contributing towards that. So we decided to leave her on for the time being....and I'm very glad of that now.

Other stuff....Hannah has had her flu jab for this year, and is due to have her adult pneumococcal in the next few weeks. Her next bloods are due at the end of the month, and it is not long now until we see Dr Coad - our first visit to outpatients at the brand spanking new University Hospital. I am going to attempt the bus to get there....wish me luck!

The CLDF Conference by the way was excellent....watch my blog for notes on the biliary atresia talk!

A new word for hope!

2006-09-27T20:34:00.000+01:00

http://www.timesonline.co.uk/article/0,,2087-2372358.html

The Sunday Times September 24, 2006

Drug may heal livers of chronic alcoholics
Sarah-Kate Templeton, Health Correspondent

BRITISH scientists have discovered a drug that could cure liver disease, even in alcoholics who continue drinking.

The medicine, found by a team of doctors and scientists at Newcastle University, could become a potential alternative to liver transplants.

Until now cirrhosis of the liver, caused by alcohol, obesity or the hepatitis C virus, was considered incurable in all but the rarest of cases. The only option for patients in the final stages of liver disease was to wait for a liver transplant. However, because of organ shortages many die while on the waiting list.

Clinical trials of the drug Sulphasalazine are expected to begin in Britain next year. If these prove successful, the drug could be used to treat heavy drinkers, whose plight was recently illustrated by George Best, the former Manchester United footballer who died from liver disease last year.

Sulphasalazine, which already has a licence to treat arthritis and inflammatory bowel disease, acts by preventing scarring from developing on the liver.

Tests carried out in the laboratory and on animals have shown that the medication can even reverse damage already inflicted on the liver.

The drug will initially be given to heavy drinkers who have given up alcohol, but too late for their liver to recover naturally. If this proves successful, the medicine will also be prescribed to alcoholics who continue to drink but show a determination to fight their addiction by reducing their intake.

Professor Christopher Day, who heads Britain’s biggest team of liver specialists at Newcastle University, said: “If you stop a drinker with cirrhosis of the liver from drinking, the cirrhosis will still be there. Even though we remove the cause of the liver scarring, by this stage that is not enough.

“The prospect is that you may be able to continue drinking. If the drug is not too expensive, I may say, of course we have to give these patients advice about drinking, but who are we to say, ‘Just because you are still drinking, we are not going to give you this drug’? I would be of the view that it should be tried in patients who are making an effort.

“I would not give it to someone who continues to drink heavily every day, but if someone had cut down to three pints a night and was really trying, why not give him this drug that might help his liver recover?”

Sulphasalazine may also relieve the ethical dilemmas of distributing scarce donated livers to the most needy and deserving. The decision to give Best a liver transplant was controversial because the late footballer continued drinking. Critics argued that the organ should have been given to someone whose illness was not self-inflicted.

If the drug is not prohibitively expensive, it could be given to all liver disease patients, regardless of whether the damage had been caused by a congenital disorder or years of alcohol abuse.

“This drug is not a finite resource, you are not stealing it from someone else — which is always a worry in public opinion. People are dying on the transplant list,” Day added.

After years of heavy drinking or obesity, so many scars appear on the liver that it can no longer carry out its normal tasks such as storing essential proteins and vitamins while cleaning up toxic substances.

The new use for the drug followed the discovery by Professor Derek Mann, a member of the team at Newcastle University, who identified the cells and proteins that may move the liver disease into reverse.

Mann found that when the liver is injured by a substance such as alcohol, proteins called nuclear factor kappa B encourage particular cells to multiply, which scars the organ. By switching off the nuclear factor kappa B protein, the scarring cells die and stop damaging the liver.

There are 45,000 deaths every year in Europe from cirrhosis of the liver but the number is decreasing.

In Britain, by contrast, where more than 4,000 die every year, the number of deaths from cirrhosis is increasing amid growing concern about the country’s so-called binge-drinking culture.

Doctors report that increasing numbers of young people, including women, are succumbing to liver disease caused by heavy drinking and poor diet.

Between 5% and 10% of people in the UK are unaware of having liver disease, which can be diagnosed by simple blood tests. Help is often sought when the symptoms become severe, which is too late for treatment.

Reseach spend per life lost for liver disease is amongst the lowest for all conditions — only respiratory medicine ranks lower.

Copyright 2006 Times Newspapers Ltd.
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It's here!

2006-09-19T20:14:00.000+01:00

At last – the clinic letter from Birmingham has arrived! It was worth the wait just for the sentence “Hannah is lovely [not very medical, but who cares] and is really very well compensated.” Compensated, in terms of liver disease, means that although the liver is damaged, it is functioning normally. Decompensation is when the liver begins to lose its function.

So, here is the long awaited ultrasound report, described by Dr McKiernan as “fine”, which will do very nicely, thank you.

Comparison has been made with the last scan dated 11.10.04. The liver is now slightly coarse in echotexture. No biliary dilatation. The PV measures 7.1 mm at the liver edge and has a flow of 31.6 cm/s into the liver. The HVs, IVC and SV are patent with normal directional flow. The hepatic artery RI = 0.69. The spleen is enlarged measuring 10.7 cm in length (previously 7.0 cm). No obvious spleno-renal shunt noted. The midline structures are obscured by overlying bowel gas. Both kidneys are normal. Right renal length = 7.3 cm (55th centile), previously 5.6 cm. Left renal length = 7.2 cm (55th centile), previously 5.9 cm. Normal urinary bladder. No free fluid.

Right, so overall this is good, and here is my attempt at interpretation:

The liver is now slightly coarse in echotexture Not great, this is indicative of a damaged liver, but at least it is only slightly.

No biliary dilatation I think this is good, and assume it means her bile ducts are not dilated.

The PV measures 7.1 mm at the liver edge and has a flow of 31.6 cm/s into the liver PV = Portal Vein. I haven’t found any normal values for this, but I think this is pretty good. If the portal vein flow is too slow, this is what causes portal hypertension.

The HVs, IVC and SV are patent with normal directional flow This is obviously good as it contains the words patent and normal. I’m guessing that this is about blood flow, and thinking that HVs = Hepatic Veins, IVC = Inferior Vena Cava, SV = Superior Vena [Cava].

The hepatic artery RI = 0.69 RI = resistance index. I think that this is good, although have not managed to find out much about it apart from above 1.0 or below 0.5 is bad.

The spleen is enlarged measuring 10.7 cm in length Not great, as this is the spleen size for a 10 year old child.

No obvious spleno-renal shunt noted Good; new pathways are not forming to cope with restricted blood flow.

The midline structures are obscured by overlying bowel gas The last US said this too, and I still have no clue what it means.

Both kidneys are normal. Right renal length = 7.3 cm (55th centile), previously 5.6 cm. Left renal length = 7.2 cm (55th centile), previously 5.9 cm. Normal urinary bladder Obviously good.

No free fluid No ascites, so good.

So, no nasty surprises lurking inside then! It feels very good to look at Hannah and know that what I can see on the outside is not too far off what is happening inside. I am very thankful that there is no diagnosis of portal hypertension.

Of course, being me, I am still left with questions, and things I would like to know more about. How is Hannah’s spleen so enlarged without being detectable by physical examination? I thought it followed the same rule as the liver, that it should match up with the bottom of the ribcage; and if it can be felt, then it is enlarged and vice versa. I also thought the enlarged spleen was a symptom of portal hypertension, which she does not appear to have. Could she have had PH when her liver was more inflamed when she was younger, which then resolved when the inflammation went down? I’m also keen to properly understand the portal vein flow and hepatic artery numbers; I just can’t help myself. I will be asking those questions of my knowledgeable buddies over at Liver Families.

The follow up at Birmingham will be next summer; no ultrasound scheduled, so there is plenty to smile about.

BIG sigh of relief.

No ultrasound report yet...

2006-09-03T20:53:00.000+01:00

...but hopefully soon! I rang Dr McKiernan's secretary; the poor woman was on holiday for two weeks with no-one to cover, and she seems to have far too much to do anyhow. I wanted to check that it hadn't gone astray, as problems with our post are fairly common occurances, but no, she hadn't typed it up yet. I bet it will arrive and not contain the full US report, and I will have to ring Liver Direct and ask them to send me one out.

I am very proud that I have managed to add a photo strip to the blog (I needed help from Andy with the HTML to position it in the middle) and I love the contrast between the tiny sick yellow baby and the fun loving healthy toddler. Unfortunately the photos are in backwards order and I'm not sure how to fix that, but I'm sure I'll be a multimedia blogging queen in no time.

Hannah has a few things coming up in the next couple of months...flu and pneumococcal jabs, and bloods at the end of October. Then, hopefully, a quiet six months.

Hannah is two!

2006-08-26T21:05:00.000+01:00

This is a very exciting birthday for Hannah. Two years is a very special milestone for a child with biliary atresia, because it is a birthday they could never have reached without successful surgery - the Kasai, or Liver Transplant. Forty years ago, we would have watched her get yellower, and succumb to the complications of liver failure while we stood helplessly by, because neither of these surgeries would have been available. And even in the 21st century, we are among the lucky ones....for now. Babies and young children still die from biliary atresia. Some of them live in countries where the surgeries are not available or where there are very limited medical facilities. Some of their families cannot afford treatment. Even in developed, wealthy countries, the story can still be a very tragic one. Neither transplant nor Kasai have 100% success rates (in fact the Kasai success rate is somewhere around 60% even at the best centres, and often lower) and complications can occur even a long time post transplant.

So the fact that Hannah is even here to be two years old is in itself a miracle. The fact that she has had no problems whatsoever since her Kasai is even more mind blowing. I am grateful for every day that we are at home going about our normal business, and nowhere near a hospital. I never imagined when she was discharged at 7 weeks 5 days that she would reach her second birthday without any inpatient stays, not even false alarms.

Hannah means 'Grace of God' and there is no doubt that she reminds us of his grace every day.

Happy birthday, little sunshine girl.

Clinic, good so far

2006-07-24T21:21:00.000+01:00

Clinic went well on the whole. Weighing and measuring was excellent as usual; weight 12.4 kg and height 88 cm.

Next we saw Dr McKiernan (the man himself this time!) who is very pleased with how Hannah is doing. His examination found what all the others have; liver palpable about 2-3 cm below ribs, spleen not palpable. Based on this, he speculated that the most likely follow up is normal clinic in 12 months, then ultrasound and endoscopy two years from now. However, he did say this depended on the ultrasound.....

I asked some questions about Hannah's prognosis. Medium term, it is very good. In the last study (the good news is that this is soon to be updated, so I shall keep an eye out for that) done in the UK, 85% of successful Kasai patients had their own liver at age 10. The biggest obstacle to that at this time would be portal hypertension. In the long term, her liver is not likely to last all the ups and downs of a normal lifespan, but exactly how long it will last is impossible to say. They would not want to wait until she was ill before transplant, and would prefer that she would be listed when her own liver begins to cause problems.....in other words, why try and keep her going with a deteriorating liver (and risk time running out) when there is a successful therapy out there.

We also had a talk about Hannah starting playgroup after Christmas, and how much medical info to give the staff there. We decided it best not to alarm them with talk of liver transplants and GI bleeds, unless either of those things become likely. Hannah does not need any special treatment or precautions, and as for illnesses, Dr McKiernan shares the same view as I do, that she is better exposed to things now, while she is pre-transplant and good and healthy. So something along the lines of, "Hannah has a serious liver disease, but she has had successful treatment and is now stable. No particular precautions need to be taken with Hannah while she is in the pre-school setting. Like any other child, if Hannah becomes unwell at pre-school then I should be contacted. If Hannah's medical condition should change, I will inform the pre-school staff immediately in writing."

Following the clinic visit was Hannah's ultrasound. It was nice to see Rachael, my best friend's sister, who is a radiographer there and popped out to see us. The sonographer was the same lady who did Hannah's ultrasound the day before the Kasai, and she is lovely. Hannah was much more cooperative this time, not being a starving, screaming six week old baby who needed a team of people to hold her still - not a very pleasant memory. She was angel baby personified today though, so still, holding her dress up out of the way.

I got a little basic feedback from the sonographer, although obviously will have to wait to hear from Dr McKiernan for detailed analysis. Her blood flow is "OK" and I hope this means she doesn't have portal hypertension, but although her spleen has never been palpable, it is nevertheless enlarged, being 10 cm (the size it should be for a 10 year old) rather than 8 cm.
So, A- at the moment I think, and as I suspected, we await the interpretation of the ultrasound for the true picture of where we are now. If follow up is needed quickly, I will be phoned in the next few days, otherwise a couple of weeks until we get the clinic summary letter.

Of course I will update when I get the ultrasound feedback.

***I was also pleased to spot a sticker on Hannah's file during the visit, saying "This patient is a part of the biliary atresia audit". As far as I am concerned the more BA data there is the better.

***Hannah's urso has been upped to 150 mg (3 ml) twice a day, to try and combat her itching and stay in line with her growth, a move I am pleased about as it is the only thing that has made any difference! Fingers crossed......

Birmingham on Monday

2006-07-20T21:57:00.000+01:00

On Monday Hannah has first check up for a year at Birmingham Children's Hospital. We will see Dr McKiernan (hopefully, otherwise a registrar) first, followed by an ultrasound, which will tell us how good her liver is, and whether or not she has portal hypertension.

The signs are at the moment that Hannah will be able to keep her own liver for at least the next several years. I am hoping the ultrasound shows that what is going on inside is just as positive as what we can see from the outside, and what can be gleaned from labs and basic examinations. I shall have a few nerves until the visit is over....and I am going to make the most of our annual visit and turn up with a list of questions.

I shall update with the results on Monday.

Excellent Clinic Visit!

2006-05-08T21:51:00.001+01:00

Hannah's outpatient appointment at the local hospital on Friday went very well indeed! She now weighs 12.2 kg (around 26 lb 12 oz) from the last weight of 10.75 kg which I was worried about back in Feb....so no worries now. Her liver is still enlarged about 2-3 cm below her ribs, but has not got any bigger since November, and her spleen is not palpable. Her blood results were excellent (Bilirubin 8, ALT 25 � woo-hoo!, Alk Phos 275)

So although this was just a basic check up with a general paediatrician, it could not have gone any better, and I am VERY HAPPY! I am going to enjoy these results until we go over to Birmingham for a more detailed check up in the summer.

A good month for Hannah

2006-04-06T21:50:00.000+01:00

Hannah has been much more lively the past month (tummy bug at beginning of March, can't remember if I mentioned it before), although she still has a perpetual runny nose. The molars she was getting are all through, so a teething respite for the moment.

I haven't had her weight rechecked yet, as the anxiety about that was laid to rest the following week when it was found that although she hadn't gained weight, she had grown 5 cm! I plan to have her weighed this week or next.

Eating wise she is still quite faddy and there are not that many things I can get her to eat. She loves bread, crackers, rice cakes, weetabix and other cereal based carbs, but won't eat potato much. Meat is difficult to get into her...I can only manage ham or processed sandwich meat. Pulses and vegetables...big no no. Fruit she does like...anything except white grapes. She is also addicted to bananas..can a child eat too many bananas? I would imagine so, although many tantrums result from the fact I will only allow her one a day. Fortunately she likes most dairy..milk, all kinds of cheese and fromage frais. Anyhow, the main thing is that she is doing pretty well on her own brand of diet :)

Healthwise, we are approaching our local clinic visit at the beginning of May, and bloods just before that, so nerves are starting to creep in. We are not over at Birmingham until the summer. Hannah's tummy looks pretty big to me (but then it always has...lol) and I can see the edge of her liver...don't really like that. I am anxious to see if her spleen has become enlarged...I hope not.

We have finally just got to start on Rifampicin for Hannah's itching last Saturday. She has started on the lowest possible dose. I think there may be a little improvement, but there are still marks on her neck. She has left her ears alone a bit more, I think...it's so difficult to tell when it is naturally fluctuating anyhow.

Otherwise, she is a mini monkey and loves to climb! She is not deterred in the least by falls and is straight back there. Her speech is progressing all the time, and she can manage to link two words together, as well as having a good vocabulary. Of course she can already say "silly mummy"!

Normal stuff, unhappy Hannah

2006-03-08T22:18:00.000Z

Since about mid-January Hannah seems to have been constantly suffering from some virus or another. She had two heavy colds/coughs in quick succession and then last Thursday she had a sickness and diarrhoea bug which is still not quite 100% cleared up. She also has back teeth coming through all over the place, is pretty worn out and clingy, and in general is not a happy bunny.

In addition to this she was weighed today and hasn't gained ANY weight in the last four months :( She was on the 75th centile before (!), so this means she has only dropped down to just below the 50th, but I still feel a tad anxious about it...especially as I had been thinking she looked thinner, but I am trying to remember that babies are much fatter than toddlers....:)

I know these are all routine things, but coming all at once they have knocked her sideways somewhat, and I am looking forward to getting my usual, swinging-from-the-lampshades Hannah back

Raising awareness!

2006-03-01T20:47:00.000Z

Hannah is featured in an article about childhood liver disease!

New Year Update

2006-01-13T20:22:00.000Z

Far too long since I updated, and Hannah is still doing great! Her clinic visit to the local hospital in November was a slightly mixed bag. We had the first ever completely normal bloods, which was very exciting, and Hannah was on 75th centile for weight and height! Unfortunately her liver was enlarged again, which it hadn't been in the summer (a bit mysterious, but I suppose may be down to how she is growing)but at least that gives some explanation for her big belly.

Shortly after that Hannah took part in a "Diploma of Child Health" exam at our local hospital, where the candidates were invited to examine her abdomen, given a few basic facts, and to name the likely diagnosis. She even got paid! I welcome this sort of opportunity, to help to educate others about paediatric liver disease.

Day to day, Hannah is doing very well indeed. Her development is bang on target, and she still has a very sunny personality, except when she doesn't get her own way! The only problem she has is with fairly mild itching, and I am wondering whether a change in meds may be necessary. She has almost completed a hectic vaccination schedule, the last one is the MMR on Jan 25th, and we also have to hope that her Hep B took, we shall find out about that when she has the MMR. So I am hoping we may have a 'non-medical' stretch following this until her next local appointment in May, and Birmingham in the summer.

One Year Kasai Anniversary

2005-10-12T21:53:00.000+01:00

Hannah is one year post Kasai, and gets to drop 5 medicines! Her vitamins ADEK and the prophylactic antibiotic. I feel a little nervous about the last one, although the use of prophylactic antibiotics in BA is not a proven science.

Hannah had her final Hep B vaccination on September 24th, and has a blood test to check for antibodies in January. She also has two flu jabs, pneumonia booster and MMR coming up in the next few months, and routine bloods in a couple of weeks. Hopefully after January she will have another break from being poked for a few months.

Hannah's next clinic visit is 4th November at the local hospital. We will get her blood results back then.

Hannah continues to do very well. She is now running around confidently and on September 20th we got her first shoes. She hasn't been weighed since 3rd August when she was 20lb 13.5 oz, but she certainly feels and looks like she is growing. She has a great appetite.

I still think her belly is big. I have always thought this and keep being told that it is normal (her liver and spleen have not been enlarged and she has not had ascites) but I keep looking at it and thinking it is not right. I think I measure it for a few days to hopefully put my mind at rest.

Hannah is still experiencing fluctuating itching. This is despite being on urso twice daily and antihistamine once daily. I can increase the antihistamine to twice a day, if this doesn't work we will have to look at something else. The itching seems to be more of a mild irritation to her over most of her body but she does still sometimes scab her ears, but not as badly as before. So the itching has become more generalised, and her ears are still the worst, but she is not damaging them so much.

The Kasai anniversary has mixed emotions for me as the memories of last year are not very pleasant, but I am so thrilled at how well Hannah is doing and has done in the past year. And please God may that continue.

Hannah is walking!!! :-)

2005-09-06T21:50:00.000+01:00

Two weeks ago Hannah first took a couple of shaky, unsupported steps. I can�t believe how fast she has some on since then. She is now walking from one side of the room to the other!! (Although the success rate is not 100%.) She holds her arms up in the air, bent at the elbow, which looks very cute!

Happy Birthday Hannah!

2005-08-26T21:03:00.000+01:00

Hannah is one year old today!

Two clinic visits and Hannah's first holiday

2005-07-05T20:47:00.000+01:00

The last few weeks have been busy for Hannah. She had blood test done on 3rd June (not a whimper, brave girl) and we obtained the results on 10th June at an appointment with Dr Coad. Her LFTs remain stable, in normal range apart from a mildly elevated ALT. Here are the results.
Total bilirubin 5 (UK measure)
ALP 344
ALT 60

I also raised the paediatric assessment unit �issues� at this visit and have since received a copy of a letter Dr Coad has sent to them so hopefully next time there will not be any more problems�.hmm, won�t hold my breath�..

On 18th June Hannah embarked on her first ever holiday, to Dawlish Warren, Devon, SW England. This was much needed and anticipated by all the family. A fantastic time was had by all and we returned on Saturday feeling relaxed and refreshed. Hannah�s favourite aspect was the sand on the beach which she would have eaten by the spoonful if permitted to do so!

Yesterday was our first visit to Liver Clinic since January. Unfortunately we did not get to see Dr McKiernan but did see an excellent registrar who was most informative and receptive. There was plenty of good news. If things continue as they are, and subject to confirmation from Dr McKiernan, then she can stop her vitamins in October (1 year post Kasai), return to Birmingham in a year, and move to 6 monthly blood tests and local hospital visits. Woohoo!!!

She will continue indefinitely on her rotation of antibiotics. In a way this is a relief as I would feel extremely nervous about her stopping them. Her Urso has been increased from 60 mg twice a day to 100 mg twice a day. This is partly as her weight has increased and also to try to relieve the itching she gets in her ears (she scratches them until they bleed). If this fails then we have been advised to try Piriton (an antihistamine) before moving on to any of the more heavy duty itching meds.

More good news � her liver is now a normal size and long may that continue! The veins on her tummy which had been dismissed locally as �insignificant� were discussed as a possible symptom of portal hypertension. The doctor said it was encouraging that her liver and spleen are not really enlarged but reassured us that this would be monitored and we may have an ultrasound at our next visit to see �what�s going on in there�.

So all in all a good visit and our little miracle girl continues to astound us.

All's well!

2005-05-06T20:13:00.000+01:00

Hannah had her 8 month development check up on Wednesday. She now weighs 18lb 13oz and is still above the 50th centile line! She is very tall (perhaps I should say long!) at 73 cm (nearly 29 in).

Her development is completely normal for a baby of her age, which I really knew already but it's nice to have a health professional say it.

The doctor who examined her was asking about biliary atresia as she examined Hannah's tummy. I saw a look of concern come over her face, and she asked if Hannah had any symptoms now. I replied that her liver is enlarged, but stable, and the look of relief on her face was evident! She said she could just feel the edge of it. I can imagine that she just thought for a moment she would have to give me some bad news....

Hannah continues to be a complete livewire - I cannot imagine what a little terror she will be when she gets to Caitlin's age. At the moment she is into absolutely everything including picking up and eating microscopic particles from the floor. She is very difficult to keep amused and gets bored quickly, but overall is a very happy baby.

Coming up next - blood tests early June followed by a clinic appointment with Dr Coad on the 10th, then a much needed family holiday!!

Chubby Baby

2005-04-13T16:18:00.000+01:00

WOW!!!!!
Hannah's weight has moved above the 50th centile for the first time since before her Kasai!!!!!As of today she weighs 8.21 kg or 18 lb 1.5 oz.
Fantastic!!! She has gained 3 lb in 7 weeks!!!!!
And it's all making little chubby arms and legs.

Busy baby

2005-04-02T10:24:00.000+01:00

With all the temperature drama I forgot to mention in the last entry that Hannah is on the move!!!!!And sitting up!!!!!! Shows how this thing means you can focus on the negative too much. She is getting around by rolling over and over, and can move round in a circle on the spot to change direction. She is sitting really well but needs plenty of padding around as she has a tendency to fling herself backwards. We have got a Galt playnest which we had for Caitlin and this is invaluable as she can topple in any direction without hurting herself.Another new development - we have seen the first evidence of her understanding words!! If you say 'Caitlin' she very deliberately turns around and looks at her sister.

Just for reference I was confused in my last entry by the holiday weekend. The temperature developed on Monday evening and the trip to the hospital was on Tuesday.

Mini cholangitis scare

2005-03-30T17:37:00.000+01:00

We've had our first 'unexplained fever'. Hannah developed a slightly raised temperature on Sunday evening about 6. Calpol brought it down, but on Monday morning the temperature had begun to climb again and by lunchtime had reached 39.5 degrees (103 in farenheit), with no other symptoms so we decided it was time for a trip to the hospital. The upshot is that the LFTs came back as normal so it was put down to being viral, and we were sent home, but it was a bit like pulling teeth to get there! More of that shortly....

Any BA/Liver parents out there will appreciate this as a brilliant set of LFTs
Total Bilirubin 6 (0.35 in US measure)
Albumin 36
Alk Phos 275
ALT 49
AST 60
Hurrah!

So....the story. As per the 'swollen tummy' incident we were seen first of all by a house officer. I told her that there should be a copy of the shared care guidelines from Birmingham and the letter from Dr Coad on the ward. No, not on the computer. No, we don't keep any files on the ward, they are all in the 'bowels of the hospital'. So I produce Dr Coad's letter. Ah, you need to be seen by a registrar. Wait for the registrar to finish with another patient. Well, Hannah seems fine, I don't want to take blood unless it's really necessary, I'm sure it isn't, I'm sure it's viral but I'll just check with the consultant. Wait for the consultant to finish with another patient AND write up the notes. Oh no, don't assume anything without ringing Birmingham, they're very fussy about their patients. Registrar phones Birmingham. Overheard: 'If it were ascending cholangitis there would be other symptoms wouldn't there? Oh right, not necessarily. And it's not really necessary to do bloods is it. Oh right, it is.' So it took 2 hours to get to the point where they took the bloods, whereas if they had been able to find the guidelines from Birmingham they would have told them to do the bloods straight away. SIGH!

I am going to ask for my own copy of these guidelines to take with me if I have to go there - it will save a lot of time and bother.....Anyway the good news is that today Hannah is 100% well and completely fever free, so not only was it 'just a virus', it was a short lived one too, so we are very thankful.

Vaccinations...getting there

2005-03-22T16:05:00.000Z

Hannah has had a quiet couple of weeks, just the Hep B vaccination last week (she was her usual brave little self, suppose she thinks having needles stuck in her is just something that's part of life). She remains the picture of health, fortunately she seems to have avoided the nasty virus that knocked big sister Caitlin for six. We have the next hep B on April 13th and then no vaccinations for 5 months!!!!!!! (After 11 injections in 8 sessions over 4 months )

Unfortunately there's a bit of a glut of them in the autumn, but after that I think there's just Pneumovax at 2 years and the yearly flu jab.Her next clinic appointment (Dr Coad again) is on 3rd June so she will have bloods taken just before that. So long as Hannah's health continues to be as good as now, we have a much needed family holiday planned just after that.

Caitlin is back to her usual self which is a huge relief, it was not very pleasant to see my normally bubbly little girl so ill and withdrawn. This is the first time she has had anything more than a cold or upset tummy and I don't think she knew what hit her! The docs think she was hit by 2 viruses in quick succession and her little immune system struggled to cope. I am working on an independent website to free up the caringbridge space for someone else, so will post a link to that when it's finished

First clinic visit to local hospital

2005-03-08T06:29:00.000Z

Hannah had her appointment yesterday with Dr Coad, the consultant paediatrician from the local hospital. Everything went brilliantly! He examined her tummy and said he could just feel the edge of her liver but nothing else. Her blood tests from last Weds have come back with "virtually normal" liver function! The numbers are stable from her last bloods on Jan 11th. So at the present time things couldn't be better, thank the Lord! He actually bought up the issue of taking her in to the children's ward and said we should always be seen by preferably a consultant but at least a registrar, and Birmingham's notes should always be referred to. Her weight is now up to 15lb 13oz.

Hannah has unfortunately been exposed to mumps! I am hoping that she'll have a natural immunity from me (I believe this is why they don't do MMR until 13 months) but am going to phone Birmingham to check. Big sister Caitlin is also poorly at the moment (not mumps!) and feeling a bit sorry for herself - seems to be some sort of virus - so hope none of the rest of us get it. Back to Hannah, we see Dr Coad again in 3 months, next bloods just beforehand. Next up is Hepatitis B vaccination next Tuesday.

Just as an aside I faced an inquisition from a pharmacist today as to why Hannah is on a long term low dose of antibiotic. Had to go into the whole biliary atresia/kasai/cholangitis saga in the middle of a crowded shop...ah well.

At the moment Hannah really is our 'miracle baby', we could not have dreamed that she would be doing so well at this stage, please pray with us that she continues to do so.

A frustrating trip to the paediatric assessment unit

2005-03-02T09:47:00.000Z

I've been a bit concerned that Hannah's tummy looks a bit swollen. I took her to the GP 3 weeks ago, he referred us to the local hospital children's ward. She was seen by a house officer (don't they rotate from one ward to the next every 3 months) who examined her for about 5 minutes. He didn't pull up any of the notes and protocols from the liver unit. He tapped her tummy but said he couldn't find any fluid, it was probably just wind. I don't feel that for a baby with a serious and rare condition we were taken seriously especially with the sinister implications of a swollen tummy in this condition - he had to ask me the name of the procedure she's had (in her notes surely). No bloods ordered or anything. Anyway I have spoken to her named consultant at the local hospital, he has been really helpful. We were due to go to his clinic for a routine visit Monday and he has asked for bloods before then (they weren't due for another month) because I'm concerned. So we go for bloods today and see him Monday - will update after that. I'm praying I'm just a paranoid mummy and everything is fine....We are also going to tell him that we are not happy about her being examined without the notes from Birmingham, and if we have to take her in with any possible liver related concerns then we want to see a senior doctor.

First Update

2005-02-14T15:16:00.000Z

Hannah is nearly six months old and weighs just over 15 pounds. She looks great and is a typical bouncing, wiggling baby. She is pink and chubby and looks the picture of health. It is hard to believe when you look at her that she has such a serious disease. Thank God for the fantastic people at Birmingham Children's Hospital Liver Unit. It is hard not to be obsessive about her health, particularly as all the good work could be undone by a few bouts of cholangitis (infection of the bile ducts in the liver caused by bacteria from the intestine - a common complication of the kasai and one that causes liver damage very quickly). However we try to take a day at a time and enjoy this time when she is in such good health.

The first six months

2005-02-12T21:37:00.000Z

Before birth and the first few days

In August 2004, my second daughter Hannah was born, after an uneventful pregnancy. A scan for growth at 32 weeks ended up being very detailed and showed a baby already weighing around 5 lbs, who had no detectable anomalies. Hannah’s birth was very quick, and fairly traumatic, but normal. (link to blogpost about evil midwife). She was a healthy 8 lb 4.5 oz, and by my calculation around 4 days overdue. Hannah passed some meconium, which was a normal blackish green colour, immediately after birth and several times afterwards. She slept continuously for the first twelve hours of her life, having to be roused for all her checks. Unfortunately I was unable to do the same. Hannah showed no interest in feeding until I initiated skin to skin contact. Following the sleepy period, she was awake continuously for the next 24 hours (apart from a brief nap initiated by the car journey home), and screaming unless she was snuggled in someone arms, or feeding.

I was keen to get out of the hospital, for a number of reasons; the main ones being that I missed Caitlin so much and that I was desperate for a bath (after Caitlin was born I had a nasty infection and feel there was a link to the hospital baths). Hannah had a general check up, and was pronounced perfect. She also passed her hearing test, and had a slightly shallow hip on the hip scan. We knew that would have to be followed up anyhow as we have a family history of CDH (link).

We had to wait for Hannah to have a wee before we could go, and I put cotton wool in her nappy so I could be sure (hard to tell with disposables and little newborn wees). When she eventually produced, I noticed the cotton wool was yellowish. So, when Hannah was around 17 hours old, we went home. Then followed the sleepless night from hell, with the aforementioned baby who would not sleep and HATED the crib. At this point I had slept around 3 hours in the last 48, and had given birth to boot. Insanity was beckoning – then we discovered swaddling. Hannah was a big baby, and I am only little! Obviously she had become very used to her confined space, and was much happier wrapped tightly in her blanket.

The next day we were visited by the community midwife – not Marie, who had looked after me during my pregnancy, but a stand-in. She made no comment, but when she left the notes she had written stated “Hannah has mild facial jaundice”. Around this time she had her first non-meconium bowel movement; it was a pale yellow colour.

Over the next week we were visited a couple of times by Marie, my usual community midwife. Hannah continued to be jaundiced, but not terribly so. Otherwise she was fairly settled and feeding well. Marie asked me on each visit about the colour of Hannah’s poo, to which my answer was that it was yellow.

Aged 10 days to 4 weeks

The usual handover between the community midwife and the health visitor happens when the baby is 10 days old and the midwife makes her final visit. It is usual then for the health visitor to make one or two visits, then you have a weekly baby clinic facility where you can have your child weighed and speak to a health visitor if you wish. The next official check-up is then at 6-8 weeks.

So, Hannah is 10 days old and still jaundiced. Not a problem, as healthy full-term babies are often jaundiced for up to two weeks. When Marie visited, she explained that she would need to return when Hannah was two weeks old to check the jaundice had cleared, otherwise Hannah would need to be sent for a blood test.

My baby might need a blood test – scary stuff! Fortunately at the two week visit Hannah did not appear jaundiced, and Marie discharged us to the health visitor, while expressing surprise that the HV had not contacted me to arrange an appointment.

The health visitor rang and arranged an appointment for the following Tuesday. Over the weekend Andy and I noticed that Hannah was still looking jaundiced. From what Marie had said we were concerned that this could mean a problem. However, Hannah was well and we felt happy that it could wait until the Tuesday appointment…..when the health visitor did not turn up. Of course I rang, and left a message, but no-one got back to me until the HV arrived unannounced on the Thursday afternoon, fortunately 5 mins after we had arrived home. I immediately raised my concerns with her regarding Hannah’s continued jaundice. She was at best dismissive, and at my insistence said she would contact the GP to seek his opinion, and call me back if I needed to take action. It is also worth noting that at this visit, she observed Hannah’s wee (bright yellow) and poo (pastel yellow), and made no comment other than “Oh, I see her wee is quite yellow.” At this point Hannah is three weeks old, and I would like to mention this (link) UK protocol, which clearly Marie was aware of…..

The days passed. Hannah got yellower. Her wee got darker. There was no word from the health visitor. Aged 4 weeks, I took her to the baby clinic, and demanded that she be sent for the blood test. The health visitor eventually agreed to ring the paediatric assessment unit at the hospital to ask their opinion. They wanted the blood test done ASAP. I said I would take her the next day.

Thinking they would draw some blood and send us on our way, I took Caitlin with me. Actually, they also wanted Hannah to wee in a pot. It is not easy to try and get a four week old baby to wee in a pot while you also supervise a two year old in a room full of medical supplies, especially when the pot only holds about 4 teaspoons. The first wee came out while the doctor was examining her tummy. The second wee came out in the pot, but so did a poo…no good. The third wee…success. Well, there was some in the pot, and lots on my trousers. Only three hours to get to that point, and I opted to go home before the blood results.

The blood test was horrible too. Her blood was squeezed out a drop at a time from her hand into seemingly hundreds of little tiny tubes, while she screamed constantly. Not really what I was expecting. Her little hands were bruised for weeks.

They also explained to me what might be causing Hannah’s jaundice. I remember them mentioning thyroid problems and breastmilk jaundice….but not liver disease.

That afternoon, I had a very non-committal call from the hospital. Hannah’s blood tests had shown that she did not have breastmilk jaundice, and that she needed some more blood tests to investigate further. Being Friday, I should not worry over the weekend, particularly as Hannah was well in herself, but I should bring her back to the hospital at 9 am on Monday morning.

When Monday arrived, Hannah and I set off for the hospital. The doctor sat us down and explained that Hannah had a high conjugated bilirubin. This indicated that her jaundice was due to a blockage of bile from her liver. She needed to have an ultrasound to find out what was causing the blockage.

The first attempt at the ultrasound was a miserable failure as Hannah simply screamed and thrashed around as she was hungry. We took a break and I went off to feed her. The second attempt was more successful, although had I been more experienced it would have raised a few red flags that the ultrasound was being done by a consultant radiologist rather than a radiography technician. He told me immediately that he could see a problem; Hannah had a cyst on her bile duct. He told me this was “rare, but well documented”, and we were dispatched back to the ward with a brown envelope.

There, the doctor told me that Hannah would require surgery for the cyst, and this would be done at Birmingham Children’s Hospital. This is our local children’s hospital, so it seemed routine to me that a four week old baby requiring surgery would be referred there. I didn’t realise at the time that she was actually being referred to one of only three paediatric liver centres in the UK. He also expressed his relief that Hannah had the cyst. “We were all very worried that it was something much worse, where the bile duct is missing or damaged….that would have been a million times worse.” I obtained my first in my collection of doctors’ drawings in biro of the liver and biliary system.

Hannah would need to stay in hospital until Birmingham made a decision on when they would do the surgery; however, we would be allowed to go home overnight. I can remember phoning my mum from outside the hospital and being so relieved that Hannah wouldn’t be “one of those little yellow children you see on the news who need a liver transplant”.

Andy, meanwhile, had been researching online furiously at work, and provided me with a medical term (the doctors at our local hospital don’t seem to like doing this) in the form of choledochal cyst (link). He brought a number of printouts home, and we read through them that evening after we were allowed home.

We found that choledochal cyst was a very rare congenital abnormality, with figures of around 1 in 100,000 births. However, of all the things it could have been, it was pretty OK. Hannah would be ‘fixed’ after her surgery, which would involve replacing the damaged section of bile duct with part of her own intestine. There was a small risk of complications developing later on, but in most cases, the surgery would completely fix the problem.

After another two days of sitting around the hospital (no further tests) they decided Hannah could go home. This was a horrible time because although I knew that the next step would be to go to Birmingham, I had no information on when that would be happening. The second day we were sitting in the hospital a paramedic asked me if I was waiting for an ambulance to Birmingham. “I don’t know!” was my answer. We were sent home on the Wednesday evening without Hannah’s medicines, because the pharmacy couldn’t get them organised. It was the next evening before they were available and Andy went up to collect them. I was pretty shocked to see three large bags, but fortunately most of the space was taken up with the Vitamin K supplement, which was in little glass vials, with a tiny amount in each. She was prescribed Vitamins A, D, E and K, and ursodeoxycholic acid.

I didn’t expect to have so much learning to do with Hannah. I knew how to look after a baby. I’d done it before without major mishap. Here she was, five weeks old, and I’d already had to deal with blood tests, ultrasound, weeing in pots, and facing up to an operation. Now I had to learn giving your tiny baby half a dozen different medicines with a syringe. You don’t find these things in the baby books.

So, we tried to stay calm and wait for the call from Birmingham, not knowing if it would be that week or in a few months. There wasn’t long to wait. The following Monday I had a call from the liver unit admissions secretary, saying they would like to admit Hannah that coming Sunday for tests and possible surgery on the Tuesday. I said that the local hospital had given us very minimal information, and the secretary said that one of the surgeons would call me back and give me some more information.

I learnt that Hannah would be in hospital for 7-10 days, and that the surgery needed to be done soon to minimise the risk of complications. I also got given some actual medical names for things, which was a first.

In patient at Birmingham Children’s Hospital

This section is still in progress

Post Kasai – the first three months

This section is still in progress