Hana’s Hypothalamic Obesity…
Hana hit 51.4 KG this week. So she’s gone from 21.4 KG January 2014, to 51.4 KG in just over 12months. Can you imagine what that must be doing to her body on the insides? It makes me feel sick to think about her liver, her heart and the threat of Type 2 Diabetes looming on the horizon. She had an oral glucose tolerance test a few months ago and this showed that her body churns out enormous levels of Insulin – both fasting and with glucose stimulation. What this means is that her brain is stimulating her Vagus Nerve – which connects to her Pancreas – to constantly pump insulin. This contributes to her sensation of constant hunger but high levels of insulin can also cause weight gain, brain fog and irritability. This is known as ‘Hyperinsulinemia’ and it is a frequent fall out of the Craniopharyngioma brain tumour so I wasn’t surprised to see her levels, the numbers themselves were the serology evidence to what we observe every day: She has brain damage. But this constant churning out of insulin will ultimately lead to fatigue in the beta-islet (insulin producing) cells of her pancreas and she will develop diabetes, it is inevitable, especially when combined with her obesity – I wouldn’t be surprised if that happens this year. You wouldn’t believe that she lives on salad, lentil soup, vegetables and cashew nuts, her diet is so clean and healthy yet she is punished for every mouthful she takes by further, completely unreasonable, weight gain.
She’s so outrageously beautiful…but her tiny body is just morphing beyond recognition. I forced myself to look through family photos of our last holiday together three months before she was diagnosed last year: It’s a different child. She even has a different voice. Then I dragged my eyes over more recent shots of her: wired up at her sleep study, sitting in her wheelchair tormented with an all-consuming hunger and in so much pain – it all showed in her eyes, that deep suffering she experiences EVERY DAY. Actually – the emotion that I got from her eyes in one particular photo of her at her sleep study was betrayal. She was suffering so much she was emanating a sense of betrayal. How could my husband and I let this happen to her? And then flowed guilt (more guilt) that this should happen to my daughter – and not to me. That it is her childhood and her life that is being snatched from her and all I do is stand by and watch from the sturdy viewpoint of my own healthy, strong body. It is a something like a cruel joke that I can move about so easily with my athletic, triathlon trained body, run 12KM without even thinking about it, leap around the lounge with my skinny, leggy four year old who doesn’t walk but skips every where like a gazelle or mountain goat. And Hana looks on at us with eyes full of pain, suffering and betrayal. It is just so unbearably unjust that her one chance at life is being screwed and ruined by this condition that modern medicine has no fricken idea how to treat let alone cure.
And now, more so than ever, she gets stared at in her wheelchair wherever we go. We’ve had the wheelchair for some time and this made people ‘look’ but now she is becoming so much bigger…people stare more now. They don’t mean anything nasty by looking – she just catches people’s eyes now more than before. Kids look at her confused at her ‘different’ she looks and they tug the arms of their parents ‘Why is that girl in that?’ I just switch off and disconnect myself from everything and everyone around me and try to insulate myself in my own world, pretend that I can’t see or notice the looks around me. Sometimes I have to disconnect myself with my own thoughts and just pretend that this isn’t real.
Can this really be happening? Is this really real? I ask myself that every day as she gets off the weighing scales or whispers that she can’t stop thinking of food. Again. ‘I hate thinking of food.’ I would do anything for that feeling of hunger to leave her alone, once and for all.
The worst times are late at night, or if I wake up in the middle of the night – the darkness makes my fears so much worse, it’s like that shadow that hangs over us in the day is there full bodied at night – terrorising me about my daughter’s fate. What the hell is going to happen to her? And can I stop it? I won’t stop trying of course, I won’t stop trying until I die.
We tried the injection drug Octreotide on two occasions – both times it paradoxically increased her appetite rather than subduing it, and it did not facilitate any weight loss during those periods. However, we will need to try it again to rule it out for once and for all. It’s not the nicest drug on the planet as there are other side effects too, and at a whopping $1,400 for a weeks supply, it isn’t kind to the bank balance either (Octreotide isn’t available on the PBS here for Hypothalamic Obesity…yep, lucky us).
Then there is Ritalin, we’ve had some stints on that but have had to come off it a couple of times as we’ve been adjusting Hana’s thyroid meds – she went a little ‘hyper-thyroid’ over Christmas which made her heart rate very elevated. Ritalin isn’t good for Hana’s overall mood and sense of well-being, it makes her emotionally labile and moody. She doesn’t need it for energy either as her energy levels and ability to concentrate are very good – she frequently does mental maths ‘just for fun.’ The main use is to see if it elevates her metabolism a little – but certainly at the lower dose it had no impact on her constant sensation of hunger.
These drugs are not miracle solutions – we never thought they would be (and no one said they would be) but we’d hoped for a more positive impact that the one we’ve observed so far.
We still don’t know whether anyone will conduct a bariatric procedure on Hana. The plan was to try the Vagal Bloc and we had found someone willing to consider this – which was amazing. However, we can’t get around the MRI issue (the Vagal Bloc is contraindicated in MRIs) which Hana still needs them once or twice a year. We are also aware that some studies on bariatric procedures often show total weight gain after a while – for this reason we’d prefer to consider a reversible procedure such as a lap band…if the effects of a bariatric procedure will ultimately ‘wear off’ at least the device could be removed and then we’d be back to square one again, of course.
I’m not sure yet how this year will pan out – once we’ve fully tried out the drugs and either seen success (hmmm) or finally ruled them out, if the weight gain continues to not stabilise then we will definitely pursue a reversible bariatric procedure such as a lap band. If only she could just stabilise. Whatever weight her brain has now hard-wired her body to reach is too high for her to sustain, and it is getting more deadly with each kilo she gains. Speaking of which: I need to organise a liver scan and echocardiogram to check out what’s happening on the inside. Of course there are some other things we are still trying – including homeopathy – but we don’t appear to be close to finding an acceptable solution yet, neither in the form of a cure nor an acceptable long term ‘treatment’ for her condition.
On the bright side…
Hana is much happier in herself these days. She has energy, she swims laps twice a day (before and after school), she plays with her sister, sings and enjoys playing with me again to0. She is spending less and less time with her pens and colouring and more time with the rest of the family. We have got a little more of ‘Hana’ back recently and I keeping holding on to that. Plus, even though the weight is still piling on she is mobilising quite well – thanks to some excellent Occupational Therapy. Her ankle and knee pain has also decreased with a new homeopathic remedy ‘Phos Ac’ at the 200c potency and it’s been awesome to see her get in to the swimming pool without crying with pain as she goes down the steps.
I’ll keep you updated as things progress – hoping to hear back about the Oxytocin case study soon too. In case it’s your first time here, you can read more about my journey as a Nurse turned Consumer after my little girl’s diagnosis with a Brain Tumour here. And just so you know, I usually update my Facebook Page daily, I’d love to ‘see’ you there.