Kramers’ mission against PWS is in 7th year

Posted June 13, 2013 at 5:00 am

By Steve Peterson

It’s now a June tradition in Hawarden, the Kramer Coed Slow-Pitch Softball Tournament & Auction, will take place June 21 and 22 in Hawarden.

The effort is in its seventh year to raise awareness for Prader-Willi Syndrome (PWS), which affects Hawarden resident Anneke Kramer.

This year, there will be a golf outing, “Driving for a Cure,” at 9:00 a.m. June 21, a shotgun start at Hawarden Golf Course. The softball tournament, “Strike Out PWS” and silent auction will be from 9:00 a.m. to 6:00 p.m. at Hawarden South Softball Complex, off First Street and Iowa Highway 12 on June 22 (via the K-18 north detour if coming from the south).

Proceeds from all events benefit PWSA and its Iowa and USA chapters.

“In six years we have raised more than $60,000. We’ll have a lot of vacation packages, airline tickets and Iowa Hawkeyes tickets. All the family gets involved and they know their roles,” said Ryan Kramer.

Anneke Kramer is the daughter of Ryan and Stacy Kramer. She will be a West Sioux first grader this fall. She is active as she likes T-ball and soccer in team sports and playing at home.

“She is doing well with regular checkups. The biggest thing is her calorie intake is extremely limited. It is only 1,200 calories per day,” said Ryan Kramer.

There is a state chapter of PWSA in Des Moines and nationally the effort is based in Gainesville, Fla.

“We have had tremendous support from the community over the years,” said Ryan.

“Discovery of the disease varies a lot. Some diagnosis are made at birth due to lack of thriving or it may be genetics, from ages 2 to 10,” said Ryan. “Early detection is the key.”

According to Stacy Kramer: “PWS is a lifelong condition with no cure. It is the No. 1 cause of genetic-related obesity in the human race. PWS occurs in approximately 1:12,000 to 15,000 live births, equally in both sexes and all races.

The major characteristics of PWS are hypotonia (very low muscle tone); hypogonadism (lack of or incomplete sexual development); hyperphagia (unctrollerable hunger and/or insatiable appetite), cognitive impairment (learning disabilities), and difficult behaviors. Two major medical concerns are morbid obesity, resulting for continual overeating or lack of a controlled diet; and stomach perforation resulting from an eating binge (because the brain doesn’t “Tell” the person when it is full) and causing death.

Because the biggest medical concerns of PWS are related to food and an insatiable appetite, people with PWS have to follow strict dietary guidelines in order to maintain a healthy weight. People suffering cannot metabolize the same way “normal” people do and therefore have to follow strict calorie intakes. It seems that with constant monitoring, the newer generation of people with PWS are better at controlling their hunger urges because they have been raised to know why they feel the way they do. However, it still isn’t necessarily safe to leave food out and un-monitored with a person who has PWS. People with PWS cannot afford to waste calories on something considered as a treat as they need to make sure they are getting proper nutrition through the calories they ingest. For example, people with PWS who are on Growth Hormone (GH) treatment can generally eat a few hundred more calories than those who are not on GH treatment, but that still only means they can eat about 1,200 to 1,400 calories per day (those not on can eat 900 to 1,000 calories per day. That’s not really very much when you consider a regular cheeseburger, small french fry and a milk at McDonald’s is 631 calories (replacing the small fry with the apple wedges and caramel is only 510 calories.) That could be half of a person’s daily diet in one “little” meal by our standards.

The importance of food monitoring with people who have PWS cannot be stressed enough.

“Most cases of PWS are attributed to a spontaneous error that occurs at or near the time of conception for unknown reasons. In a very small percentage of cases, 2 percent or less, a genetic mutation that does not affect the parent is passed on to the child and in these families, more than one child may be affected. A PWS like disorder can also be acquired after birth if the hypothalamic portion of the brain is damage through injury or surgey.”

For more information, visit www.pwsausa.org. Contact people for the effort to strike out PWS events are: Stacy or Ryan Kramer at 712-552-3052.

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