Fabry Disease

Almost at the end of my first week of my clinical rotation down in Charleston, WV.  It’s technically my 3rd week of a clinical nutrition rotation since I had 2 weeks at Mon General Hospital, but that was about 2 months ago so it feels a bit like I’m being re-oriented. No worries though, I feel like I’ve caught on pretty quickly to the computer program, nutrition care process, and the layout of the hospital (I’ve found if I wander around long enough, I’ll find my way back to the dietitians’ office…haha).

I’ve seen quite a few patients so far, and most have fairly common medical conditions that you’d see in any hospital – Type 2 diabetes mellitus, coronary artery disease, gastroesophageal reflux disease, asthma, anxiety, depression, myocardial infarction, stroke, HIV, cancer, chronic kidney disease, hypertension, hyperlipidemia, nausea/vomiting, …. the list could go on, but that’s what I remember off the top of my head.

I also came across a patient this week who had been diagnosed with Fabry Disease, and I had to look it up because I couldn’t remember what it was. So I thought I’d share what I found through a bit of internet research:

Fabry disease is a lipid storage disorder that is caused when there is an alteration in the structure or function of an enzyme (alpha-galactosidase-A) that metabolizes lipids in the body.  Fabry Disease may be better known as alpha-galactosidase-A deficiency.  When this lipid metabolism process is unable to function properly, it causes a dangerous and unwanted build-up of a particular type of lipid in the body (globotriaosylceramide) that can damage the eyes, kidneys, nervous system, and cardiovascular system. It’s also X-linked, which means it shows up in males, and females with the gene will be carriers or have a milder form of the disease.

Symptoms begin in childhood or early adolescence and as stated above, are related to the eyes, kidney, nervous system, and cardiovascular system.  Patients can develop clouding of the cornea, impaired arterial circulation, increased risk of heart attack or stroke, enlargement of the heart, and kidney disease. Symptoms also include hearing loss, ringing of the ears, GI problems, aches and pain, and dark red spots on the skin.

Patients with Fabry disease can undergo enzyme replacement therapy to reduce the lipid storage, and thereby easing the patient’s negative side effects and improving organ function.  Patients may also require a kidney transplant or dialysis, but premature death is common.

Click for more information:

http://ghr.nlm.nih.gov/condition/fabry-disease

http://www.ninds.nih.gov/disorders/fabrys/fabrys.htm

Smoothies for Breakfast

In my last rotation with WVU dining services, I was able to choose a new food item, recipe, or something of the like to implement and write a report on. I decided to provide a smoothie bar during breakfast hours for those students in Evansdale Cafe.  The project included planning, food costing, advertising, implementing, writing a literature review, and writing a 16-page summary of the quality improvement project.  While this was several weeks ago, I thought I’d pass along some of what I came up with, because it was a fun project to be a part of.  The staff at WVU were extremely helpful in every aspect of the project– letting me take creative reigns, printing off customer counts, invoice orders, ordering the food, helping with set-up, tear-down, and even working next to me during the busy 2 hours, running the second blender. I am very grateful that the staff at WVU are so willing to help – they definitely care about their dietetic interns!

Abstract:

            Fruits are an important part of a healthy diet as they contain vitamins, minerals, and fiber that are beneficial for heart health, digestive health, and more.  However, less than 23% of Americans are consuming the recommended 5 or more servings of fruits and vegetables per day.¹ In fact, a study by Steven E. Shive found that students at a community college have a typical fruit intake of 1.7 servings a day.² By providing students at WVU with a smoothie bar during breakfast hours, it creates the opportunity for students to increase their fruit intake that day by 1-2 servings.  A smoothie bar was planned and was implemented on Friday, September 28th from 7:30-9:00am and served approximately 80 students.  Students were able to design their own smoothie or choose one of the three smoothies shown on signs with nutrition facts attached.  Peaches, bananas, strawberries, blueberries, raspberries, and blackberries could be mixed with vanilla soy milk, 2% milk, orange juice, and yogurt.  Each smoothie took less than a minute to make and the smoothie operation worked well with two employees running the blenders and taking orders.  There were approximately 5.5 labor hours involved and the average food-cost of a smoothie made was $1.165.  Feedback from the students was positive, as many students lined up early, gave positive comments, asked if the smoothie bar was permanent, and were happy to have something different, unique, and nutritious on a weekday morning.  If there is enough staff available to run the smoothie bar, I would recommend keeping it in rotation several times a month.

Signage for Smoothies <– Check out a PDF of the sign I made for the smoothie bar, which explains how to order and shows all the ingredients available.

Smoothie Nutrition Facts <– I analyzed the smoothies for their nutrients and made signs that displayed the nutrition facts for 3 different smoothies — Triple Berry (strawberry, raspberry, blueberry), Peach-Raspberry, and Strawberry-Banana.

Working the smoothie bar on Friday morning. Set-up took about an hour for one employee (me)– thankfully all the food and supplies were organized on carts in the dry storage, walk-in cooler, and walk-in freezer the day before, so set-up in the morning went “smoothly”…haha

Making one of the approximately 80 smoothies we made that day. The first people to get a smoothie at 7:20am were a group of WVU student-athletes who had just finished with an early morning practice.

 

This project also involved taking into account food safety rules and regulations – dairy products were stored in tubs of ice to keep below 45 degrees F; employees wore hair restraints, aprons, gloves, and properly washed their hands; serving utensils were used; and the smoothie bar was located within 25 feet of a hand-washing station.

The most popular smoothie was the strawberry-banana smoothie. We used about 10 pounds of strawberries that morning and about 35 bananas!

 

References

1. Brown, B.J., & Hermann, J.R. (2005). Cooking classes increase fruit and vegetable intake and food safety behaviors in youth and adults. Journal of Nutrition Education & Behavior, 37(2), 104-105. Retrieved from http://search.ebscohost.com/login.aspx?direct=true&db=a9h&AN=17041069&site=ehost-live
2. Shive, S. E., & Morris, M. N. (2006). Evaluation of the energize your life! social marketing campaign pilot study to increase fruit intake among community college students. Journal of American College Health, 55(1), 33-39.  Retrieved from http://search.ebscohost.com/login.aspx?direct=true&db=a9h&AN=21558776&site=ehost-live

Ideal Body Weight – Devine Equation and Hamwii Method

In clinical nutrition in order to calculate energy and protein needs for a patient, ideal body weight is typically calculated.  One equation used is the Devine Equation.

For females this equation is:  45.5 kg + 2.3 (__ inches over 60 inches)

For example, a female who is 5’7″ should weigh 45.5 + 2.3 (7) = 61.6 kg

To get to pounds, simply take the kg and multiply by 2.2.

So, 61.6 kg x 2.2 = 135.52 lbs

For males this equation is: 50 kg + 2.3 (__ inches over 60 inches)

For example, a male who is 6’1″ should weigh 50 + 2.3 (13) = 79.9 kg

79.9 kg x 2.2 = 175.78 lbs

There is another formula used to calculate ideal body weight, the Hamwii method. This method is easier to use if you are more familiar with pounds and inches rather than kgs. It also allows for some “wiggle room” by taking into account body frame.

Females:  Add 100 lbs for the first 5 feet of height, and then an extra 5 pounds for each inch over 5 feet. Add 10% for “large” frames or subtract 10% for “small” frames.

For example, a female who is 5’4″ would have an ideal body weight of 100 + 4(5) = 120 lbs. Depending on her body frame, her ideal body weight could be between 108 lbs and 132 lbs (10% of 120 = 12 lbs).

Males: Add 106 lbs for the first 5 feet of height, and then an extra 6 pounds for each inch over 5 feet.

For example, a male who is 5’10” would have an ideal body weight of 106 + 10(6) = 166 lbs. Depending on his body frame, his ideal body weight could be between 149.4 lbs and 182.6 lbs (10% of 166 lbs = 16.6 lbs)

If a person is over 120% of their ideal body weight, then hospitals typically use an “adjusted body weight” which is then used as their “feeding weight” and used to calculate their energy and protein needs.

For example, a patient whose current body weight (CBW) is 189 lbs,  whose ideal body weight is 140 lbs is at 135% ideal body weight (IBW) .Their protein and energy needs will be based off of their adjusted body weight (ABW) which is found by:

ABW = .25(CBW – IBW) + IBW

So the patient in the previous example has an ABW of .25(189 – 140) + 140 = 152.25, or 152 lbs. If the patient’s energy needs are 25 kcal/kg, then you’d use 69.2 kg (152 lbs)  instead of 85.9 kg (189 lbs). This would make the patient’s energy needs 1730 calories rather than 2150.

Where have I been? FNCE!

I haven’t made a post for the past few weeks…I have had 3 weeks off from my regular rotations, and used that time to travel, visit friends, study for my online classes, and attend conferences. In fact, tomorrow morning I’m leaving for Charleston, WV to attend the WV Diabetes Symposium. So the “3 whole weeks off” has definitely been a busy, whirlwind period of time.

So last week I was at FNCE – the annual Food & Nutrition Conference & Expo which was held in Philadelphia, Pennsylvania October 6-9th (Next year it’s in Houston, TX!).  FNCE is held by the Academy of Nutrition and Dietetics, so most of the attendees were dietitans, dietetic interns, and dietetic undergraduate students, but there were also nurses that I ran into that are Academy members. Registration for the event was $210 and includes a multitude of educational sessions to attend each day, culinary demonstrations, and the expo hall.

There were literally about 14 educational sessions going on during each time slot, so it was difficult to pick which one to attend, but I ended up attending:

• Hot Topic: Ancient Grains — What Is Their Future?
• Achieving Six-Figure Careers in Dietetics: How Confident Conversations and Taking Risks can Get You There
• Childhood Obesity Prevention: Building a Sustainable Community Nutrition Network
• Dietitians as Health Coaches: A Call to Lead
• In the Kitchen and Outside the Box: Cooking Lactose-Free
• Leader vs. Manager: Five Strategies to Propel Your Career
• Diabetes in a New Light: Diabetes-Friendly Fare with Flavor

And there’s also the Expo hall where different food companies and nutritional organizations have booths with different information and goodies. I had my body fat tested with an infrared laser at the Subway booth, and also talked to the registered dietitian for Subway on how she got her job.  I have enough granola bars now to last me a month or two, about $80 in coupons, and I also picked up some great recipes.  Although the 3 days went by quickly, it was an enjoyable and educational conference and I hope I’ll be able to attend another FNCE in the future!

Food samples at the expo

Booths at the expo

Mary and I with a giant frosted mini-wheatie

cooking demonstrations

ingredient composition of white chocolate, milk chocolate, and dark chocolate

McDONALDS at a nutrition expo?? Yes. They were giving out oatmeal and coffee. There was also KFC at the expo…yeah…

Paula Deen giving an culinary demonstration on cooking diabetes-friendly fare with flavor

Paula Deen’s spicy shrimp & vegetable gumbo

Enjoying the sites of Philly in the evening