Thursday, May 19, 2011

No Preggers or Children--40+ only please

This Blog is for 40+ years. I do my best to find info having to do with the older PKU adult that's why blog is called elderswpku. No babies, no preggys just us grossly overlooked pku'ers.

This may seem mean but pregnant people and babies-children-teenagers and even occasionally young adults get all the support. It's especially hard if you never or will never have children, Truly do any of us oldsters give a damn about breast-feeding?!

I wanted to start an "elders only" group but don't have a clue of how. That's when I decided to post this blog.

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"Thank you Ray Stevens" just isn't enough graditude

Scripps researcher treks across The Sahara to raise awareness for PKU disease

Professor Raymond Stevens, who directs the structural neurobiology program at Scripps Research Institute, completed a six-day, 156-mile ultra-marathon across the African Sahara Desert in April to raise awareness of a disease called phenylketonuria (PKU). A rare, inherited metabolic disorder, PKU patients are unable to metabolize an essential amino acid called phenylalanine, which puts them at risk for severe neurological complications, including IQ loss, memory loss, concentration problems, mood disorders, and in some cases, severe mental retardation.

Raymond Stevens

Stevens obtained his spot in last fall’s lottery among ultra-marathon running enthusiasts for the opportunity to compete in the 900-person race, which got underway April 3. Called “Marathon des Sables,” it has been described as “the toughest footrace in the world.” The course is not revealed until the day before the race begins, but it weaves over uneven, rocky ground and massive sand dunes in southern Morocco, where daytime temperatures exceed 130°F.

Competitors are required to carry all belongings, including food. The only exceptions are group tents and water. Water is handed out in rationed portions at checkpoints.

“This event for me in part symbolizes PKU drug discovery and all of the researchers, families, foundations in the PKU community that have come together with the common goal of finishing a long drug discovery race,” said Stevens, who wrote about his adventures on the website of the National PKU Alliance, where Stevens serves on the scientific advisory board.
“Running this race is like developing treatments for PKU — three steps forward, one step back, but one just has to keep moving forward and eventually one WILL cross the finish line.” In breakthrough findings, Stevens’ group solved the structure of the liver-produced enzyme phenylalanine hydroxylase (PAH), deficient in people with PKU.

On the first day of the Marathon des Sables, Stevens said he felt as ready as he was going to be. He trained in a 160° sauna. He ran “a lot.” And, with his doctor, he tried to manage the tendinitis that sprung up from overtraining in his left ankle and the shin of his left leg, although neither had fully healed.
In the preceding days, he also packed and repacked his supplies, trying to minimize their weight. On the day of the race, he was carrying 2,000 calories of food per day for seven days (including oatmeal, freeze dried packages, almond peanut butter, and pop-tarts), electrolyte powders, a sleeping bag, headlamp and batteries (for running at night), an emergency space blanket, a whistle, a signal mirror, a compass, a knife, a first aid kit, a lighter, and a scorpion venom pump.

The first day’s course, about 21 miles, included crossing the largest sand dunes in the Sahara, where many of the runners (including Stevens, whose left leg also began to swell) picked up severe blisters. The physicians in the medical tent were busy “fixing a lot of feet.”

A victorious Stevens after the race.

Courtesy

The second day’s course consisted of about 24 miles, followed by a third day of another 24 miles. Stevens said he struggled with increasing nausea from dehydration and vomited twice near the third day’s final checkpoint.
The next day presented the longest stretch — 51 miles — and one of the hottest days of the race. After the first 18 miles, Stevens was so dehydrated he was put on an IV (one IV is allowed per participant; require a second and you are forced to withdraw from the race).

“I had to lay in the sand with a needle in my arm that cost me time,” Stevens wrote, “but after 1 liter of saline solution and 1.5 liters of glucose solution, I felt great being fully hydrated and managed to run 32 miles straight to the long stage finish line, crossing at 3:58 a.m.”

He noted he was especially motivated to complete the stage before the sun came up on the fifth day; rumors had been circulating around camp that temperatures were reaching 131°F.
By the sixth day, the 26-mile marathon stage, Stevens “had his head down, just putting one foot in front of another.” Finally, came day seven, 11 miles … and then the finish line!

Was the race was worth it?
“Absolutely,” he said. “I enjoyed it fully, which is easier to say now the pain has gone away.” He added that his favorite part was “turning off my headlamp in the middle of the night on the 51-mile-long stage and running up the cold dunes in the dark looking at the stars.”
Would he run the race again?
“No way,” he laughed. “It was a once-in-a-lifetime dream.”
More on the Web:
• http://www.scripps.edu/research/faculty/stevens
• PKUHeroes.org

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Friday, April 22, 2011

Comparing low protein food cost

As we all know low protein foods are a lot more expensive than normal foods...at least 3x more. I am fortunate, when the state finally started covering up to $200 of food. My doc, dietician, and metabolic nurse made the arrangements for assistance.

Regular Food: Low Protein Food Cost:

1 doz jumbo eggs $3.34 Egg Replacer 12 oz box $6.99
1 doz Cinnamon Rolls $3.99 Cinnamon Rolls-pkg of 8 $9.95

Hamburger Buns 8 pkg $2.99 Buns 8 pk $16.99

Cheese Slices 16 pk $5.99 Cheese singles 16 pk $16.99

Spaghetti 16 oz pkg $3.99 Spaghetti/pasta 17.6 oz bag $14.98

Well you get the idea. The price of low protein food is insane. Unless you have assistance it's hard to keep these on hand. There are several states that offer the assistance: Texas offers a $200 a month allowance but only on approved foods but No desserts

And if there are others that are disabled and can't spend a lot of time cooking/cleaning there are what I call heat n eat products. They're very convenient and very pricey, but for the most part worth the price. The prices posted do not include shipping. Cold/dry have different costs and minimums I've seen a $50 minimum for cold ship foods-$30 minimum for dry foods. I found as much as $75 minimum on one companies products

Here's a list of some of the low protein foods shops

www.cambrookefoods.com

www.pkuperspectives.com

www.dietforlife.com

www.dietspec.com

www.specialtyfoodshop.com

www.ener-g.com

www.halfhourmeals.com

www.food4celiacs

www.shsna.com

www.nutriciahealth.com

These food companies are what I've found so far I'll post additional companies as I find them

Metabolic Formula Options

Waaay back in the 60's-70's the only metabolic formula that was available was Lofenalac I was a strange child I loved the formula straight with no flavor additions and warm. Nowadays there's a wide variety of metabolic formula. Most I've tried tasted like jock-socks. Of course that's my opinion. The only one that was tolerable is to me was unflavored Periflex only because I tasted similar to Lofenalac but eventually caused lower GI problems.

After 12 years on the Periflex It eveutually ate through my enamel. No one told me that I needed to take extra care of my teeth, I was ignorant. I lost them 2 years ago. So if you still have your teeth, it's not too late, I would suggest the new toothpastes that contains something that strengthens enamel. Or at least rinse your mouth out after you drink your formula.

Applied Nutrition corp http://www.medicalfood.com/
Phenylade essentials

phenylade amino acid blend

NUTRICIA - www.shsna.com\

Periflex Advance-unflavored, orange, chocolate

Phe Maxamum Drink® (orange and berry)

Lophlex™

Milupa PKU 3

CAMBROOKE FOODS www.cambrookefoods.com

Bettermilk

Camino pro®-sorbet stix, sorbet drinks

SOLACE http://www.solacenutrition.com

PreKUnil tablets-

BIOMARIN

KUVAN

ABBOTT NUTRITION www.abbottnutrition.com

Phennex TM

Cambrooke foods came out with probably the most tolerable of all the formulas--BetterMilk. It's low volume, 1/4 cup water per envelope-or to taste. I've found that it has a sweet scent...kind of like angel food cake batter. The only downfall to this formula, is as it sits it gets thick and hard for me to drink/swallow. I add grape gatoraid and kuvan tablets and drink it within 14 minutes and you don't get that "thickening" I was switched to Kuvan, gatoraid & bettermilk combination due to lower GI problems + burned it my throat from fruit juice.

Kitchen Doodads for arthritic elderswpku

Many of us elders, sad to say have one or more areas that are arthritic. Unfortunately I've found a high levels seems to magnify arthritis pain. I thought it would be good to list a few "arthritis devices to make cooking easier especially if you cook in volume for a week's worth of food. Most don't cost a lot and are a huge help.

Easy Opener opens bottlecaps, pull tabs and box tops.

Paring knife

Knives/slicers

Peeling Plate Keeps foods still while peeling

Rolling pins are necessary if you're going to make low protein bread, cinnamon rolls, regular rolls, even tortillas. I had a long time ago a marble rolling pin instead of the traditional wooden. It's heavier weight and dosen't take so much pressure to roll out dough. They are kind of pricey but worth it if you have arthritis hands, shoulder(s) or neck