Wednesday, April 01, 2009

A picture to explain our panic.....


This is a graph I (okay, Warren actually - I'm a dunce at Excel) just made of Matthew's weight gain over the past 18 months. Keep in mind that before his diagnoses, his weight also increased. That was one of our only indicators that something was wrong - Matt went from being in the 50th percentile for weight and 75th percentile for height (where he had been since birth) to shooting off the charts in both height and weight. In hindsight, that was a sign of his hypothalamic brain tumor, but at the time we just worried he wasn't getting enough exercise! Anyhow, back to the graph. We've got a pretty good record of Matt's weight since diagnoses because we track his sodium levels along with his weight. I went back and looked at his weights over these past 18 months and made a graph. You can see his gradual climb of 25 lbs. the first year after diagnoses (still extreme), but once you hit his surgery date at the first part of December, you can see the almost unbelievable 25 lb. spike to now!

Meeting with Dr.Lustig was very reassuring and very refreshing - although it did leave us with a ton of questions about which direction to go with Matt's care. Dr.Lustig is *the* person researching hypothalamic obesity, and he reassured us that there is absolutely nothing we could do to stop the weight gain. We have had several people tell us that Matt just *HAS* to be sneaking and hoarding food, and that we needed to lock our cupboards. I have had a really hard time believing that because anyone who knows Matt knows that he is the most honest child on the planet. He has an almost debilitating conscience, is honest to a fault and he says he's not sneaking! Also, I do all the cooking and grocery shopping. I never notice food missing and I am with Matt for the majority of his meals. I spend 90% of my life in my kitchen and I have no evidence or suspicion that Matt is stealing food! Dr.Lustig discussed a study done in the 1970's in which 40 children were confined for one month. They were fed a strict 500 calorie per day diet (that's the equivalent of 2 pieces of bread, a piece of fruit, and a glass of milk!) for that entire time. At the end of that month EVERY SINGLE ONE OF THEM had gained weight!

Dr.Lustig has discovered a connection between the way the hypothalamus is damaged and the messages it is sending to the body in individuals with this disorder. When you eat, and create fat cells, those cells have something called Leptin inside. When you have a lot of Leptin (fat), your Vagus nerve (which goes from your brain to all of your organs, including the stomach) tells your brain that you have a lot of energy. It tells your brain to make you feel full, and it tells your brain that your body is free to USE that energy - for exercise and such, but also for basic functions like sweating, maintaining body temperature, etc.... For some reason, in people with HO, their brains get the message that they have NO Leptin, when in reality they have oodles of it available. For this reason, the body goes into starvation/survival mode. They create excess insulin to help pack away these calories. So, Matt's brain thinks his body is starving and won't allow him to use the calories that he has stored so amply. This also explains why Matt is often cold and clammy, doesn't maintain his body temperature well, and doesn't sweat (truly - no sweat, even when we've gone on a long hike and everyone is hot and sweaty!)

There are very few options for treatment of this extremely rare disorder. The first is a drug called Octeotride. It suppresses the excess insulin production and has been marginally effective. Dr.Lustig has written several papers and done several research studies with this method. He says it can often help to stabilize weight, but most kids don't lose any weight. It is also a drug that is not approved for this purpose, so you have to appeal your insurance company. The drug is also extremely expensive ($1,600 per month) and difficult to administer. It is a shot and is apparently takes some extra training for the nurses who give it. It is not a practical option for long term, but some people have found it very helpful for the 6 months or so they can remain on it.

The second option is even less well researched. It is a surgery where they actually go in and clip that Vagus nerve - where all the Leptin/brain messages are being mixed up. It has been extremely effective in animal studies (scary, huh!) and they are just beginning to use it on people with HO. Dr.Lustig is doing a study right now, but so far he only has 5 children who have done it. The success with those 5 has been great, but it's not exactly a large sample! By cutting the nerve, the insulin production goes down permanently, there is a weakened hunger mechanism, and the brain can no longer send those confused, "you're starving to death" messages. Obviously, our concerns lie in the lack of history, but with such a small population that has the disorder, I'm not sure we'll ever get a study that thousands of children have validated.

So, that was basically our trip! We've got a lot to think about. Warren will take Matt back the first part of May. I'll be 36 1/2 weeks pregnant. I would prefer to not give birth on an airplane, so I think I'll probably stay home....... although it will be hard, as I want to be there! During that next trip, we can talk more about the surgery, as I'm sure we'll have lots more questions by then! We are also meeting with the surgeon just to get a sense of the reality of the surgery, recovery, side effects, etc.... It sounds like it would be a quick outpatient procedure, done laparoscopically. I just wish there were more options! Even more doctors who were researching this would be great! Everything I google comes up with articles this doctor has written, and I'm not sure I'm really getting unbiased information! :-) I guess I should just be thankful that there is *someone* out there working on ways to help.

Oh, and San Francisco was fabulous! The weather was perfect all 3 days. We wandered Fisherman's wharf on Sunday - eating fresh seafood on the patio of a restaurant that afternoon. Monday was spent in the hospital nearly all day long, although when we left we ate the most fabulous Mediterranean meal. Maybe I was just starving (since Matt had to fast, we all did), but everything tasted so wonderful! Then Monday morning we walked down to Union square, had a yummy breakfast and wandered expensive shops for a bit. When we came back to our hotel we checked out, had our luggage stored, and went to the top of our hotel to this amazing restaurant (Top of the Marc) where we had expensive salads and expansive views of San Francisco. It was beautiful - and who knows if we'll ever stay at that hotel again, so it seemed like something we should take advantage of while we were there! It was a fun, but quick trip. I wasn't all that excited to land in SLC, wearing my capris and spring shirt with sandals only to find *more* snow and crazy cold temperatures! Come on Spring!

8 comments:

Scott said...

Sounds like a good trip even though you didn't get any definite answers....

belmomma said...

wow alli, sounds like you've got lots to think about now! I know that you and Warren will be inspired as to what is the best path, and remember, LOTS of people are praying for your family!

Christina said...

I can only imagine how tough it would be to make that kind of decision! What a blessing, though, to meet with someone who knows so much about this.

Katie said...

Whoa. You have a lot going on! I don't believe for a minute that Matt would sneak food. I'm sure those people were trying to help, but that's annoying when people say things like that when they don't know very much about his condition. I'm glad you found such a good doctor. We'll be praying for you!

Jessie said...

Whew - that's a lot of stuff to ponder. I know you guys will make a well-informed, and well-prayed-out decision. Kind of cool to learn more about how our bodies work, though. Really, I mean, how did someone figure out how all that stuff works?
The brain is so amazing.
Hoping for the best, Matt!

Just dandee said...

Wow! Lots to think about. It wonderful that *someone* is trying to figure all this stuff out. Best wishes.

Karen said...

WOW... that is so much information to absorb and yet not many options. I have been thinking about you. Keep me posted and I can't wait to see your new baby GIRL. SOOON!!!

Heather Parry said...

After reading all the other comments I echo them, but my one additional comment is, "Who was in charge of regulating the scientific studies of children in the 1970s?" Seriously, they gave them only 500 calories per day? I can't imagine that. I can't imagine limiting my kids' food intake in any measurable way. On a more serious note, I hope you guys can come to a decision that you all feel comfortable with. I'm sending good vibes your way.