A new study

There's always hope for a better treatment! And sometimes, they might come from unusual places. This is really the only thing out there beyond the rapamycin. He's not eligible for the trial though. (trouble w/ font, sorry)

Viagra may help kids with rare, disfiguring disease, study shows

By ERIN ALLDAY, San Francisco Chronicle

Stanford researchers may have discovered a drug for a rare and often untreatable disease that leaves children with massive, and sometimes deadly, growths on their faces, necks and other parts of their bodies.

Here's the twist: The drug is Viagra.

In very early reports, sildenafil -- best known under the brand name Viagra -- reduced the size of growths in three children with lymphatic malformation, a disease that causes spongy cysts to swell and clog up the lymphatic system.

It's too soon to say how effective Viagra could be in treating most cases of lymphatic malformation, but Stanford researchers are starting a clinical trial and are currently seeking patients to test the drug.

"Some of these kids have no other hope," said Dr. Al Lane, a pediatric dermatologist at Packard Children's Hospital at Stanford. "The first child we treated, the malformation was so big and she was in such bad shape that there was nothing we could do for her. We gave her the sildenafil, and we were blown away."

Lane stumbled upon Viagra as a possible treatment for that child, a 5-month-old girl, by accident.

Sildenafil was first developed in the early 1990s to treat high blood pressure, but early clinical trials found that it was more useful in treating erectile dysfunction. Today, sildenafil is sold primarily under the name Viagra for erectile dysfunction, although it's also used under the name Revatio to treat a rare form of high blood pressure involving the lungs.

About a year ago, Lane examined the infant girl, who had severe lymphatic malformation with growths that had swelled up in her chest and wrapped around her aorta. The pressure caused high blood pressure and, eventually, heart failure, so Lane gave her sildenafil.

The drug helped her heart problems but Lane also noticed a massive reduction in the lymphatic malformation. Intrigued, he tried the drug on two other patients with lymphatic malformation and noticed similar results.

Lane wrote up his findings in a short letter published in the New England Journal of Medicine last month. Since then, a fourth patient has been treated and also saw some improvement. In all four cases, the growths started to come back when the children were taken off sildenafil, and the parents opted to resume drug treatment.

Lymphatic malformation affects the lymphatic system, which is part of the circulatory system. The lymphatic system removes excess fluid from tissues and organs, and transports white blood cells. In patients with lymphatic malformation, the vessels that make up the system become clogged, and fluid builds up and creates large cysts or masses of sponge like tissue.

Other physicians who treat lymphatic malformation say Lane's work is intriguing, but not yet cause for major celebration. With only four patients treated so far, it's too early to say just how effective sildenafil is, said Dr. Jonathan Perkins, an expert in vascular anomalies who treats children with lymphatic malformation at Seattle Children's Hospital.

"Honestly, I don't know what to make of this just yet," Perkins said. "Is the drug safe? What does Viagra do to little kids? This is very interesting, and it needs further study."

Erin Allday is a San Francisco Chronicle staff writer. eallday(at)sfchronicle.com. For more stories visit scrippsnews.com

Read more: http://www.abcactionnews.com/dpp/news/health/Viagra-may-help-kids-with-rare-disfiguring-disease-study-shows#ixzz1my2Wrod5

Winter Fun

Very intently decorating cookies for Valentines Day present

He's his mothers child, he doesn't care for finger painting, so we use a brush :)


This has been a habit of his since he was a tiny baby... except now, he's not so tiny... doesn't stop him!

He's doing really well, if you can't tell. Healthy, and we still have about a week and a half til we have to go to Cincy.

Feeding Tube Awareness- Part 3

The current tubie- (and his "belly button" - www.bellybuttonsandbelts.com)
This is a g-tube (short for gastronomy tube). Specifically, it's a mic-key button. Technically, between the NG and this tube, he had a PEG tube, but that's often a temporary tube right after the tube is placed. The PEG is probably 4-6" long, and is a soft silicone tube that hangs from the body. Adults sometimes have PEG's, but most children have "buttons", because they're less likely to be snagged.
This picture was him sleeping, i couldn't get one of him standing still (a surprise, of course).

He's connected to his overnight feeding from about 8:30-5:30. it runs on a little pump at a rate of about an 1.5 oz/hour. The body normally slows down the gut at night, so it can rest. Little Micah doesn't have that luxury. We run it pretty slow for that reason.

One thing i haven't addressed is the "why" of the tube, other than initially (aspiration). He outgrew his aspiration at about 6 months. But, believe it or not, he lost the innate feeding reflexes, not to mention the desire. At about 6 mo, he was cleared for baby food consistency, which is less likely to be aspirated. At 11 months or so, he was cleared for liquid. The long and the short of it is that he's not that interested. His stomach is so small, and his tube feedings so frequent that he has very little idea of hunger (minus the pre-surgery stuff). He also has issues moving food around in his mouth, probably a problem related to the lymphatic malformation or the surgeries to treat it. On top of that, his mouth doesn't open much. Again, probably the LM, surgery, and the fact he's never had to use it. Here's an example of his dilemma. We'll call it, Micah vs. the Dum Dum.


Hopefully tomorrow will be part 4.. We'll see it i get to it... Might be a little late. Hoping to add a little tubie humor, and a little of tubie ettiquette.

Feeding Tube Awareness- Micah's day

Micah has a bit of a rigorous tube feeding schedule (although not what it used to be, at all! Phew!) His stomach for whatever reason is unusually small (could be not having any food for days after birth, his nissen fundo surgery, ???) He gets 4 "boluses" (a large quantity over not much time) and he gets continuously fed overnight. His boluses are given to him about 1/3 or 1/4 at a time, at 15 minute intervals (so his first feeding is given at 8:30, 8:45 and 9:00, for example)
His tubie schedule:
8:30-9:30- 4.5 oz food, 1 oz H20,
11:30-12:30- 6 oz food, 1 oz H20
2:30-3:15- 4.5 oz food, 1 oz H20
5:30-6:30- 6 oz food, 1 oz H20
8:00- Meds and 1 oz H20
8:30pm-5:30 am, 13 oz food, 3 oz H20.

Overnight (and sometimes during the day) his food runs on a pump that gives it to him at a steady rate. He has significant reflux issues which are currently controlled by meds, and one of his biggest vomiting triggers is too much at a time. We've made significant gains in the last few months (one happy mom!).

He currently gets about 1200 calories a day, and 8 oz of extra water. i never would have thought a 2 1/2 year old would eat that much normally.

Sadly, he normally gets pediasure. it's pretty much crap in a can, but it has a lot of advantages (safety, consistency of calories, portability, ease of feeding). Sometimes we do a blenderized diet, where we blend whole foods or baby foods and put it through his tube. My own OCDish tendencies muddle that process, trying to find perfect combo's of foods to get him what he needs. For example (Here's one of many pages of my calculations). He needs 30 calories per ounce of food, which is a little outrageous, really. The only food i can compare it to is full fat full sugar pudding. Ew. On top of that, you have to at least be somewhat close on fat and protein as well.