Endoscopy and study - thoughts so far

I have been doing a great deal of thinking on the subject of the endoscopy and study. No conclusions yet, but lots of random thoughts that need organising.

An endoscopy is being recommended for Hannah because she has portal hypertension. Portal hypertension is elevated blood pressure in the portal vein which carries blood between the liver and the digestive system. In Hannah's case this is caused by restricted blood flow through the liver because it is damaged. Varices form when the blood tries to find other routes back to the heart. It travels through other veins and these can become overloaded with blood meaning they can burst. A variceal bleed is a life threatening medical emergency. Hannah has been diagnosed with portal hypertension because her liver and spleen are both enlarged. At this stage it is considered to be mild, based on how much her spleen is enlarged.

The thought of a bleed from portal hypertension has been my number one fear since Hannah was diagnosed. A decline towards transplant does not happen overnight, and there would be time to take stock and adjust. The thought of finding Hannah covered in her own blood is the stuff of nightmares. I wish someone had told me in the early days that it is uncommon for varices to develop in the first 18 months of life if the Kasai is working and the liver is functioning. My anxiety levels would have at least halved.

My mind has gone to many dark places in the last 2 years or so on the subject of varices and bleeding. For children with BA and successful Kasai a major bleed can come completely out of the blue. It's often made me wish I can see what was going on inside, to know whether I can put that fear to rest for now. I would have thought I would have jumped at the chance of the endoscopy to find out for sure. Now I am having some doubts.

One of the thoughts I am having is that I don't want Hannah to go through an invasive medical procedure solely for my peace (or anxiety!) of mind. While her doctor is recommending it, the ultimate decision as to whether to go ahead lies with us. I really need to think through the possible outcomes of the endoscopy and whether having it done would be beneficial to Hannah.

If treating any risky varices was the point of the endoscopy, I would have no doubts whatsoever. The problem I am having is that a) if Hannah is not part of the study any varices would not be treated anyway b) if she is part of the study she only stands a 50% chance of being selected for treatment. Perhaps this would be a good point to go into the current protocols and the details of the study.

Currently here in the UK children with varices are not usually treated unless they have a bleed. There are some exceptions to this (such as those based long distances from the liver centre). The treatment is to put a rubber band around the varices which is done by endoscopy. There are drugs to treat portal hypertension but they tend not to work so well in children as in adults.

It has already been established through trials that in adults it is desirable to treat varices before a bleed. At the moment it is unclear whether the same is true for children. This is what the study is seeking to establish.

The way the study will work is that when children are recommended for routine endoscopy they will be invited to take part. To ensure there is no bias the decision to take part is made before the routine endoscopy is carried out. Children are eligible to take part in the study if they are found to have large varices which are at risk of bleeding. They are then divided randomly into two groups; those who will have banding treatment and those who will not. For those who have the treatment, the first banding will be carried out immediately, and further banding will take place until the varices are all gone. There will then be further follow up and banding if necessary for the rest of the two years of the study. The other group will not have any varices banded unless they have a bleed. They will also be followed for two years. One of the things I need to know is what is the plan after the two years are up? If that is not included in the information pack from the hospital then I will definitely ask.

There are so many different scenarios to consider. In some of them the endoscopy seems a no-brainer, in others it seems pointless.

To start with, I am finding it hard to justify going through with if we are not going to agree to be involved in the study. I see two end results - the first, and most likely, being that she does not have any large varices, in which case the endoscopy has served no purpose other than to give peace of mind to the adults involved in her care (and of course me!) The second would be that she was found to have large varices at risk of bleeding. The current protocol means the banding procedure would not be available, at least in the short term, so that would mean spending the next however long in a state of perpetual anxiety about a bleed. I suppose there are some benefits to having knowledge of what is going on, whether or not it is going to be acted on, but that needs to be weighed up against putting Hannah through an invasive procedure.

If we agree to allow Hannah to take part in the study, then there are three end results. Again the most likely is that she is found not to have large varices, and nothing much has been gained apart from peace of mind (not that I am understating how valuable that can be, just weighing it up). If she does have threatening varices, then there is a 50/50 chance that she will not receive any treatment, so again we are back to the major bleed anxiety situation. If she does get picked for the treatment group, then it involves a lot of endoscopies and a medical way of life that we have thankfully become unaccustomed to.

Then, on the other hand, there is the big part of me that says I would move heaven and earth to prevent any possibility of Hannah having a bleed. If that involves some disruption and taking some chances, well, maybe it is worth it. If I were to do nothing, and she had a major bleed, would I forgive myself? I need to do a lot of thinking and praying about this, and Andy and I need to spend some time in discussion.

I know an endoscopy is, when all is said and done, a minor procedure. But I know every parent out there would agree with me in saying that putting your child through any medical procedure is a decision not to be taken lightly.

Birmingham - the news so far

We had a productive and interesting visit to Birmingham yesterday. Dr McKiernan is very pleased with Hannah's growth and development, and her energy levels. She now weighs 15kg (33 lb) and is 99 cm tall! Nothing was flagged up from her abdominal exam, although he said he couldn't be sure of its accuracy as Hannah was so tickly!

We had her bloods done there - the most comprehensive panel for a long time - so no results on those yet. I can either wait several weeks for the clinic letter to come in the post, or I can phone Liver Direct and get the results from there in a day or so. Guess which option I am choosing........expect an update on those very soon.

I didn't get chance to ask any of my questions about possible worsening of portal hypertension. Dr McKiernan threw me for a loop before I had chance by saying he is keen to do an endoscopy to check for varices in the next few months. Apparently age 3-4 is the most common time for varices to develop. I'm still a bit in shock from this as although I knew that a scope was likely in the future, I just didn't think it would be quite this soon. I have quite a few mixed feelings about this. Although an endoscopy is routine it is still an invasive procedure and requires a general anaesthetic in children. If the endoscopy shows large varices, Hannah will be eligible to take part in a European study on prophylactic treatment of varices. We need to make the decision BEFORE the initial procedure. More on this later.

Working through the worry

With Hannah's yearly Birmingham check up coming up tomorrow morning the paranoia is cranking up several notches.

Will her bloods be normal? That is always the first worry. Last time her enzymes which indicate liver cell death were higher than the previous result. I hope that's because she was unwell, and that they are the same or lower than then, putting them back into normal range. The enzymes don't tell us how well her liver is working, but the way I see it the lower they are the happier her liver is. Previously the tests which show how well her liver is working (albumin, bilirubin) have been perfectly normal and there's no evidence they will be otherwise this time. If there was a problem with those she would have noticable symptoms - fluid in her abdomen (ascites) or jaundice. Those are the really important ones and along with clotting factors are the ones where abnormal results would indicate we would be thinking about transplant.

The next thing to worry about is whether there is any indication that her portal hypertension has worsened. This would be the case if her spleen is palpable or if her platelets are low. If those are both OK then we should be good to go till next year before she has an ultrasound and possibly endoscopy. If there is an indication it has worsened (I've now also convinced myself she has spider veins on her face, hoping this will be debunked) then those may take place this summer.

Really, the main worry is whether something will happen this visit to detract from our 'normal' life. Sometimes I feel like we have always got the lucky hand when it comes to liver disease and that one day we will be dealt a really shitty one to make up for it. Like our lion has spent so much time asleep that when he wakes up he is going to be full of energy and ready for a fight. Each time we go for a check up and they are so pleased with Hannah's progress I am walking on air, but at the same time feel like I was given a get out of jail free card. When the next appointment is due I can't help but wonder if one day they might all be used up.

So hopefully this time tomorrow I will have an update that says 'stable'. I've opted to have the bloods done there this time so will have to wait another couple of days for those.

Date through for Birmingham clinic

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

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.