85) How's your hypothalamic health? (Part 2)

Following up from the last post, I have always known that Sasha sustained significant hypothalamic damage as evidenced by his many functional impairments; it had never been confirmed by any radiology reports, however.  Recently, Sasha's hypothalamic damage was brought to light after Dr. Christian Roth reached out and offered to evaluate Sasha's post-operative MRI scans using his semi-quantitative scoring system: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5029599/

Curious to see what it would reveal, I readily agreed and sent him a couple of scans to evaluate. Not surprisingly, the evaluation and "score" of his two MRIs indicated that he had very high risk of HO (in one scan, he scored a "6" and in the other scan, he had a "7" with "7" corresponding to the highest risk for having HO). As reported by Dr. Roth, "damage includes the anterior medial and posterior hypothalamus, and also mammillary bodies, and also the floor of the third ventricle. His ventricles also look enlarged." Although getting a score as severe as Sasha's is not exactly good news, it IS good news that Sasha has somehow been able to lose weight, maintain it, improve his overall metabolic health, and reduce his hyperphagia behaviors in spite of it all. Although he is just a single case example, it provides some compelling evidence that our experimental treatment with oxytocin (and naltrexone) gives hope to those with even the highest HO risk.

It is also my hope that researchers investigate the effectiveness of OT at varying doses (by itself and in combination with naltrexone) for people with HO.  It would also be interesting to see if and how OT treatment works on HO sufferers with and without hyperphagia. 

What if someday all survivors with posterior pituitary and hypothalamic lesions could have their MRI scans evaluated and scored to assess their HO risks? And what if, one day, that assessment would simply get them directed to an evidence-based treatment? Maybe it might even be oxytocin?

84) Getting used to the "new, new normal"- what are the functions of the hypothalamus anyway?

Ho hum, our lives are kind of boring now without the HO Monster rearing its ugly head.  Not that I miss that "excitement"- I guess I'm saying that that I am getting used to the "new, new normal."  We are continuing the open snack cabinet and things are going well.  Sasha's weight is stable more or less.  He had his testosterone dose almost doubled last week so we will expect some weight gain from possible increased appetite, muscle and bone density, and height growth.  If he continues to do well with his open snack cabinet, the next exposure will be to continue with the snack cabinet and to have the fridge unlocked 24 hours a day (right now, the fridge is unlocked only during the day time).

We finally got our fresh batch of oxytocin in the mail.  Due to our trip in New York and my forgetting to order the refill on time, the OT refill was delayed and then further delayed when the pharmacy neglected to send the order after the weekend.  I believe we were using OT that was expired by one week.  We noticed that toward the last couple of days before we received the fresh batch, Sasha was showing slightly more food interest; talking about food more and showing a little more urgency to eat.   However, it still paled in comparison to how he used to be pre-OT.  In the old days, food seemed to be constantly on his mind (and our minds, by proxy).  If he wasn't actively eating it, he was cooking it, asking for it, talking about it, plotting to sneak it, sneaking it, lying about it, and/or melting down over it.  Now that he's back on the newly compounded drug, he's back to a moderate appetite, no evidence of sneaking food, varied topics of conversation (not limited to food only).  As a result, we have a much more peaceful household and a feeling of normalcy again.

As I may have shared before, certain researchers have taken a keen interest in our experiment and one in particular has asked to see his post-operative MRI scans to see if they might reveal information about the specific areas and extent of his hypothalamic damage.  For more information of hypothalamic anatomy and functions, see website: http://humanbrainfacts.org/hypothalamus.php.

Besides the obvious excessive hunger/appetite/weight gain from his hypothalamic obesity, we are certain that Sasha has suffered extensive damage post-surgery due to the impairment in several homeostatic functions:

1) Poor temperature regulation.  In the past, he was physically unable to sweat no matter how hot he felt and no matter how warm the air temperature.  He also has a hard time warming up in the cold conditions.  Once he suffered a very scary adrenal crisis and became bradycardic (heart rate slowed to 30 beats per minute) and his rectal temperature dipped down 87 F (30.5 C).  Despite this dangerously low body temperature, Sasha did not even shiver!  Interestingly enough, although he is still rather heat intolerant- he prefers the temperature to not exceed 75 F (23.8 C) or he feels uncomfortably hot- he now reports that he sweats a little on his head!  We are not sure why or when this changed but it appears to have been improved in the last year- could this be due to oxytocin?  Who knows?

2) Poor circadian rhythm regulation.  His sleep is often disrupted with difficulties staying asleep (waking in the middle of the night, 3 am is typical).   He also has had day time somnolence.  We try to rectify his irregular sleep with good sleep hygiene (regular bedtime, removal of electronics at night) and low dose melatonin before bedtime and stimulants during the day for wakefulness.

3) Non-existent thirst mechanism.  Yet another missing homeostatic function that is missing with Sasha is his thirst instinct.  He doesn't have one and this makes his Diabetes Insipidus extremely challenging to manage.  While most people with intact thirst can manage DI by drinking when they are thirsty to prevent dehydration, Sasha relies on an extremely high dose of DDAVP (0.2 x 20 pills per day), frequent weighings, forced drinking (although the Kitchen Bitch has mostly retired, the Water Witch is alive and well, alas!), and weekly lab draws to check his electrolytes (sodium).  While he has had a history of needing to be controlled from eating to excess, we have the exact opposite problem with his drinking since he has NO DESIRE whatsoever to drink any fluids.  His sodium levels have been in the high 160s (normal is between 135 and 145) and he still has no inkling of thirst even at these dangerously high Na levels. This missing homeostatic function is a very grave one, indeed, and we often wonder how long Sasha would be able to live independently without dessiccating to death if we weren't around to nag and bribe him to drink water.  Although oxytocin is supposed to also have antidiuretic properties, we have not noticed much extra water retention with his oxytocin.  Perhaps this is due to the fact that he takes only a relatively low dose of OT?

4) Impaired social motivation.  I've documented frequently in this blog how Sasha lacked any interest in forming friendships with his peers. Now that he is taking OT, he has made and maintained a friendship with a boy his age.  Although I would say he has only this one close friend, he has been demonstrating increased friendliness and sociability at school with his classmates and hanging out with kids during lunch time every day (rather than hanging out exclusively with his para-educator aide). His demonstrated improvements have been enough to meet his IEP goal in this topic area.

5) Excessive hunger, food seeking, and weight gain.  This area does not require much elucidation since it is the main topic of this blog.  To our relief, OT has provided great improvements in all of this areas related to his excessive hunger, food seeking problems as well as improvement in his metabolic health.

6)  Compulsive behaviors.  Although not well known in the medical literature, it has been anecdotally observed by caregivers and craniopharyngioma patients that there is a prevalence of compulsive behaviors in association with hypothalamic obesity.  As I've mentioned, Sasha had a history of acquiring items for a collection and many of his cranio peers have also had similar tendencies or have demonstrated other types of stereotypic, OCD-like behaviors.  This article sums up how is also related to a hypothalamic function: http://news.yale.edu/2015/03/06/multitasking-hunger-neurons-also-control-compulsive-behaviors.  Since being on oxytocin, Sasha has since stopped his compulsive collecting behaviors.

Functionally, I think it is obvious that Sasha has suffered a significant amount of hypothalamic damage.  However, from what I understand, it is quite difficult to ascertain damage to the hypothalamus from examining scans alone... we will see how much information can be gleaned from his scans, to be determined.

83) Another candy-intensive holiday: Easter

We returned from our trip to New York and were very glad to have had a wonderful trip together as a family and that we all stayed healthy and in good spirits for the trip.  Sasha did very well throughout in regards to his food issues.  As I reported in my last post, we took advantage of the great food in New York and relaxed our normal lower carb regime quite a bit.  Sasha gained a little bit of weight (2/3 of a pound or .3 kg)  during the trip... I'm sure I gained much more, LOL! Because we were away, I was late in re-ordering the refill for oxytocin so we are now using slightly expired (on 4/13) medicine.  I'm not too worried about it though because despite its very short half-life, it has been shown in the literature to have lasting effects for up to 7-10 days even after complete cessation of the drug.  It will be interesting to see if any of his food intensity returns in these last two days before his new batch arrives. In any case, a fresh supply will arrive on Tuesday.

If you recall, we had quite a bit of trouble with Sasha when he got into his sister's Halloween candy supply last October (despite it being locked up) which is what prompted my getting him to start Naltrexone (he remains on 100 mg of this opiate antagonist drug).  Another candy-intensive holiday is before us today with Easter.  For today I agreed to let the kids have an egg hunt (with those plastic eggs you fill with candy).  I went out and bought some M&Ms to fill and his sister was in charge of hiding them for Sasha and his friend (who was over).  She hid the candy-filled eggs in our backyard and ended up stashing the remaining candy it in her room, unbeknownst to me.  Sasha and his buddy were occupied with a computer game.

No more than one hour after Sasha's sister hid the eggs and the remaining candy, she told me that the bag of remaining candy was missing!  She swore that she hid the candy in a safe place in her room, that she kept her bedroom door closed when she was in another room of the house, and that when she returned to her room, the door was open and the candy was gone.  I took Sasha aside and asked him about it and he denied taking the candy.  His friend even vouched for him saying that Sasha had not left his side in the last hour.  Ugh... here we go again, I thought.  His sister kept insisting that the candy was hidden away and that it was now missing.  He kept saying that he did not take the candy.  I decided to drop it for the time being since he had a friend over and to look for it in his room later.  When he and his friend were playing in the living room, I had a chance to check his room and I found no candy.

Hours later when I was out for the evening, my husband texted me and told me that his sister found her candy in another hiding spot in her room!  She apologized right away to her brother and he graciously accepted her apology and moved on.

In the past, it was always Sasha accused (rightfully so) of stealing his sister's stash and Sasha melting down while his sister experienced her own mix of emotions: anger for the unfairness of losing her treat to her brother, frustration at her brother for his behavior, sorrow for herself, compassion and understanding for his inability to control himself, and finally, submission to her role as the sibling of a brain tumor survivor with HO.  She has not told me with these words per se, but has expressed all of these feelings throughout the time we have had to deal with his issues as a family.  When I asked her later how she felt about finding her stash of candy, she said that she felt "really bad."  I was slightly annoyed at her for the wrongful accusation but I understood why she did it; he started quite a precedent for her accusations, after all.

Despite whatever embarrassment, guilt or remorse any of us may have felt for this mistaken accusation, we all felt great relief and happiness for Sasha's vindication.

Happy Easter, Happy Passover, Happy Spring!

82) Still doing great with higher carbs & minimal food restrictions, even on vacation!

I am writing to you from New York during the kids' spring break holiday.  This vacation is the first vacation we have taken with a greatly decreased "food police patrol" meaning that we have not locked up, gone out of our way to hide food, or restricted snack access from Sasha.  Normally, we have resorted to locking or hiding food from Sasha (we tend to stay in Airbnbs so we have access to a kitchen) but this time we just leave the bagels out and the peanuts around.  We've detected no evidence of excessive eating (can't call it sneaking anymore since the food is out and accessible)!

We are also doing a lot of walking (6 miles a day on average) and despite his flat feet and bad ankles, he is doing remarkably well!  We are relaxing our lower carb food regime on vacation but still eating fewer sweets than most people would on a vacation in New York (so far on our 5th day, we've had frozen yogurt once and shared a double scoop of ice cream between the four of us once- that's it).  Still, we are eating very well and Sasha is keeping up excellent energy in spite of his higher carb diet- gotta eat New York speciality foods like latkes, bagels and pizza while we're here, right?!

Even though he's eating latkes (pictured above), pizza and bagels,
Sasha's excellent energy and lack of hunger
 confirms that he is no longer hypersecreting insulin!

As we have observed on this trip, Sasha's appetite has been moderate and even lower than expected, especially considering all the extra walking we are doing.  It is noticeable that he is NOT complaining of hunger as often as he used to and is not obsessing about his next snack fix.

It is such a pleasure to be here on holiday and not have to stress out about being the food police.  Ahhh...  I could get used to this!  Let's see if he can keep up the good work when we return home.  I anticipate that after another few weeks or so of his open snack cabinet, we will unlock the family snack cabinet next which will mean that the entire kitchen will be unlocked and accessible to Sasha 24 hours a day... wow, won't that be amazing?

81) Oxytocin: making wishes come true! Photos tell the story-

Sasha makes breakfast while I relax!

Extra food left in the pan that Sasha could have had (but didn't take!)

Extra bread left on the counter that Sasha could have had
(but didn't take)

A bite of food left on his plate
(something we would never have seen pre-oxytocin!)

Even for moms!

80) Pinch me, is this for real? Having a "normal" week

It's been a full week with an unlocked snack cabinet for Sasha.  I've strangely nothing special to report, meaning that (dare I say), I have felt like we have lived like a "normal family" this week.  I still can't believe I have just written that sentence.  Let me pause and reconsider what I've just written...

To recap, I supervised Sasha while he stocked his own snack cabinet from Monday to Wednesday evening with an appropriate supply of snacks.  He then restocked his cabinet again from Thursday until Sunday evening.  These snacks were kept in his own cabinet, unlocked 24 hours per day. To our knowledge, Sasha acted very normally with the open snack cabinet with no detectable food intensity during the week.  He appeared to pace himself with his snacks and ate at his normal snack times. We barely supervised him when he made and packed his school lunches over the week.  He ate modest amounts at all of his meals.

Tonight we attended a friend's backyard housewarming party (mingling in the backyard, food laying about on a table at one end of the yard) and Sasha behaved very normally.  He definitely ate the food (nothing sugary but definitely foods high in carbs like bread and tortilla chips) but I also noticed that he was NOT standing around the food table, eating continuously for the duration of the party.  Instead, he helped himself to some food but also took breaks from eating to chat with other guests.  I keep saying he behaved "normally" but I really don't know if that is the right word to describe him since his behaviors from this week are nothing short of EXTRAORDINARY to us.  My husband and I ended up having a really fun and relaxing time at this party and so did Sasha.  There were no meltdowns, no nagging, and very minimal to no stress.  This ability to enjoy ourselves at a party is also extraordinary.

Earlier today, Sasha was invited by his friend, A, to go miniature golfing.  We all ended up going (A, his mother, Sasha, Sasha's sister, and I) and had a great time.  Again, this was a "normal" activity that he did- hang out with a friend on a weekend. And again, although it appears to be a normal for most people, having a friend and a social life is a relatively new phenomenon for Sasha!  Speaking of social life, we learned at his recent IEP meeting that he has successfully met one of his goals about "engaging in appropriate peer relationships with classmates."  Although we tried to push for the inclusion of this goal for next year because of our uncertainty of his ability to maintain the goal on his own, his teachers in attendance assured us that Sasha is quite social with his peers in class and that he participates voluntarily in two different social clubs at school.  Wow.  It appears that Sasha's social skills and motivation have changed for the better as well!

I am so grateful to oxytocin as I'm sure none of this would have been possible in the past without replacing this important neurohormone. Perhaps this week was not "normal" per se, but rather "extraordinarily normal."