There is no beating about the bush: My daughter fits many of the risk factors for developing the intractable ‘Hypothalamic Obesity’ over the next 6 months. She had a higher body mass index when she was diagnosed with her brain tumour, the Craniopharyngioma. The tumour was so large that it caused Obstructive Hydrocephalous (”water on the brain”) and she had an ‘aggressive resection’ (i.e the whole tumour was successfully removed) which puts us in a much better position in terms of reoccurrence but simultaneously increased her chances of having some hypothalamic damage during surgery.
When Hana was diagnosed, and before we’d selected the neurosurgeon, I’d asked one surgeon whether or not her quality of life would improve after the operation considering she’d be on full hormone replacement. He hesitated and said, “Maybe not her obesity.” I remember being quite put out, thinking; Obesity? What obesity? She’s just chubby!
Shortly after the surgery, whilst still in Intensive Care Hana was clearly ‘hyperphagic,’ asking for food constantly. We are now 5 weeks post op and this problem has continued to haunt her… and us. But although this constant desire for food is a risk factor for the development of obesity, there are other really twisted things that may also have happened to my daughters brain because of this tumour and all lumped together, they potentially put her at a high risk of becoming obese within the next 5 months. I want to share them with you now.
Why Hana is at risk of developing Obesity:
When damaged from a tumour, surgery or an accident, the Hypothalamus can go totally crazy and for some really, REALLY cruel reason seems to do all it can to make that person morbidly obese. 75% of children become morbidly obese within the first six months after their craniopharyngioma tumour is removed.
Firstly, the appetite control centre messes up. People, kids, like my daughter, stop receiving messages of ‘fullness’ after eating. In addition, hormonal signals such as ‘leptin’ from fat tissue stores also get ignored, these signals would usually inform the brain that the body doesn’t need to eat. The result is a brain that thinks the body is physically starving. Now, we all know how hard it is to concentrate on anything when we’re really hungry. A brain that thinks the body is starving will only be able to think about things other than food for short periods of time. My daughter asks for food every few minutes of the day and, when one meal is over, will immediately start planning for and asking for the next meal.
It is tragic. It is desperately, desperately heartbreaking to witness. And it is extremely difficult to deal with. Now I think I deal with the hyperphagia pretty well, Hana eats three extremely healthy and well balanced meals a day. I give her healthy low energy snacks such as frozen pear, lettuce leaves and peas as snacks. Remember that I also have to deal with her multiple food chemical sensitivities, I wish I could give her cucumber and carrot sticks as low calories but she’ll react! Something else that really stinks about our situation. SO, if hyperphagia was the main cause of hypothalamic obesity we’d be living in hell, but our daughter would not become obese. But as I’ve mentioned this is not the whole story…
Secondly, as part of the insult, there can be changes to the body’s metabolism, a real slowing down of processes. Basically the Sympathetic Nervous System (Fight/Flight – adrenalin, noradrenalin; Catecholamines) slows down and the Parasympathetic Neural Pathways (rest/digest) become enhanced. This makes an individual feel lethargic and de-energised. This decrease in Sympathetic Nervous System ‘tone’ prevents the patient, from wanting to get up and move around. If this manifests in my daughter she will ultimately feel exhausted and will not get the ‘high’ from running around the playground like her friends. Less activity of course, means more fat deposition.
Thirdly, to add cream to the Hypothalamically Obese Cake, the body starts churning out more insulin than it has before. Kids with Hypothalamic Obesity are found to have ‘hyperinsulinemia,’ higher insulin levels make you more hungry. But, in order to further spite us, as a result of churning out higher insulin levels, insulin resistance then occurs. This means that the body’s receptors for insulin get ‘down regulated ‘ (basically sick and tired of being exposed to it) and then stop ‘listening’. Cells that don’t listen to insulin then don’t get to swallow up the glucose the insulin is carrying which means that sugars get caught up in the blood stream and.. can you see where I’m going? Yes, Type ii Diabetes – ultimately.
Fourthly, children are at greater risk of developing ‘hypothalamic obesity’ if they have endocrine (hormonal) deficiencies as a result of their brain tumour. My daughter’s tumour grew out of her pituitary stalk and had to be cut out completely, she now has no communication between her hypothalamus and pituitary which means that ALL her hormones have to be given to her in the form of medication. Getting the balances right with these isn’t easy. All you have to do is look at consumer groups of people who only deal with ONE of her multiple pituitary dysfunctions to see how hard they find it just to manage their own issue.
Fifthly: GROWTH HORMONE. Hana has no growth hormone now at all, considering she has no pituitary stalk this is to be expected. However, it seems that children are not entitled to apply for Growth Hormone medication over here until 12 months has passed post op. Growth Hormone is not only crucial for growth (by the way, she has the skeletal age of a 3.5 year old, her friends are now turning 7, if we wait a year, her bone age will be stuck at 3.5 and her friends will be turning 8) but also crucial for muscle deposition (over fat, which in turn facilitates and promotes physical activity, which enhances the metabolism). GH also helps other metabolic issues such as: helping thyroid hormone absorption into bodily (for the medics: it helps t4 become t3). SHE NEEDS GROWTH HORMONE. When will we be able to get it? I don’t know, our endocrinologist is going to try and get for it for us much sooner than the 12 month wait. Meanwhile, every month that she doesn’t get GH, her risk of becoming more overweight continues.
Before the surgery I hoped that we would be one of the lucky ones, the remaining 25% that won’t have to watch their child gain weight with the threat of morbid obesity on the horizon. Although her symptoms are milder than many with hyperphagia (i.e she dosen’t eat from the rubbish bin, won’t eat anyone else’s food, and won’t eat overnight) I must say I’m not that optimistic that we are in that category. We think the best thing to do is to assume we aren’t and to begin to take action now to prevent morbid obesity from occurring just in case her weight begins to spiral beyond our control. She has already gained two kgs over the last five weeks. We have five out of the six months left.
Medicine really has very few solutions, if you spend a few hours researching PubMed you’ll find a depressing array of papers that outline few, mostly unsuccessful treatment modalities. We already know that Hypothalamic Obesity is intractable and won’t respond to strict diet and lifestyle changes, most medications don’t work very well, they also have side effects. What many scientific papers are proposing now is bariatric surgery as one of the best treatment modalities. If you look to consumer education (note: there is practically none available), you will advised to ‘manage’ the condition yourself by locking kitchen cupboards and withholding food.
NONE of these are acceptable to me. I have no interest in watching my daughter become obese with intent to put her through bariatric surgery when she is old enough. Nor do I wish to simply ‘manage’ her eating at home with restriction and minimal food access. I do not want my daughter to live a life that is obsessed with and ruled by food. I do not want a treatment approach that simply manages her condition when this hunger demon controls her thoughts. I want her brain to heal.
So, three weeks ago, starting with this premise I began to do some research. We have developed a little action plan on things we want to do in addition to some things we have already started doing. I have no idea if we will be successful in preventing Hana from becoming obese, and if we succeed we may not even know how we succeeded because we will be applying multipe treatment approaches. In my next blog post I will share our first steps with you.