Pompe InCommon

High protein-low carb recipe: Mushrooms stuffed with crabmeat

Ingredients:
12 ounces of fresh stuffing mushrooms
1 6-ounce can of crabmeat
3 scallions, sliced thin
1/4 tsp of dried oregano
1/4 tsp of dried thyme
1/4 tsp of paprika (for the topping)
1/3 cup of low-fat mayonnaise or 1/3 cup of fat-free mayonnaise
1/4 cup of grated Parmesan cheese
3 tbsp of grated Parmesan cheese (for the topping)
Black pepper (to taste)
Olive oil (to brush on mushroom caps)

1. Rinse and drain the crabmeat.
2. Combine the crabmeat in a medium bowl with the scallions, oregano, thyme, mayonnaise, pepper, and ¼ cup of Parmesan cheese. Refrigerate the crabmeat mixture until you are ready to stuff the mushrooms.
3. Preheat oven to 350 degrees.
4. Clean and dry the mushrooms.
5. Remove the stems of the mushrooms and discard. Scoop out the mushroom gills and discard.
6. Fill the mushrooms with the crabmeat mixture.
7. Brush some olive oil on the stuffed mushroom caps and place them on a cookie sheet, jelly roll pan, or a shallow baking dish lined with aluminum foil.
8. Sprinkle the stuffed mushroom caps with the 3 tbsp of grated Parmesan cheese and the paprika.
9. Bake in the oven for 15 minutes and serve.

Yields 6 servings; 9.6 grams of protein and 2.7 grams of carbohydrates per serving.

Adapted from a recipe at food.com

High protein-low carb recipe: Standing rib roast

The secret to this juicy roast is the addition of maple sugar and Dijon mustard.

Ingredients:
1 5-pound rib roast, bone in
¾ cup of maple sugar
⅓ cup of Dijon mustard
2 tbs of fresh rosemary, finely chopped (can you use a food processor to conserve energy)
2 tbs of fresh thyme, finely chopped
2 tbs of fresh black pepper, roughly ground (to combine with herbs)
Salt and pepper, to taste
1 jar of bordelaise sauce

1. Preheat oven to 400 degrees.
2. Place the roast in a roasting pan, bone side up.
3. Use a knife to make incisions in the fat.
4. Cover the entire roast with salt and pepper (to taste).
5. Cover the ends of the bones with pieces of aluminum foil.
6. In a medium bowl, combine the maple sugar with the Dijon mustard and spread over the entire roast.
7. In a smaller bowl, combine the chopped rosemary and thyme with the roughly ground black pepper and spread over the entire roast.
8. Place the roast in the oven for 30 minutes to sear the meat.
9. Reduce the oven to 300 degrees and cook the roast for an additional 2 hours (or longer if you do not prefer meat rare).
10. Remove the roast from the oven and cover with aluminum foil. Allow the roast to rest for 10 minutes.
11. Carve the roast (you can use an electric carving knife to make cutting easier).
12. Serve with bordelaise sauce.

Yields 12 servings; 29.6 grams of protein and 9.5 grams of carbohydrates per serving.

Adapted from a recipe at www.food.com

Weathering a storm

No matter what part of the country you live in, Mother Nature sometimes wreaks havoc. Fortunately, there are some things you can do to help prepare for weather-related emergencies, including long power outages that so many people faced in the wake of Hurricane Sandy.

The American Red Cross offers plan and prepare resources for your home and family for many weather emergencies, including:

• Floods
• Hurricanes
• Earthquakes
• Landslides
• Tornados
• Other disasters

You can also download “Preparing for disaster for people with disabilities and other special needs”, an informative booklet that can help you assess what help you will need and create an action plan — particularly if you use a mobility device — including a checklist of items to do before an emergency strikes.

Power failure

“Do you have backup power?” and “What will you do if the power goes out?” are two Quest Magazine articles that can help you plan for back-up power for ventilator and respirator users, including general tips about back-up batteries and gasoline- and diesel-fueled generators (always check with device manufacturers for the most recent information and device-specific recommendations).

The power outlet in a car may be one short-term power source during an electrical outage. Another option is to check with your local hospital to see if you can utilize the facility’s emergency power if your electricity cannot be quickly restored.

Talk to your utility company, local police, and fire personnel about your needs before an emergency to learn if they will be able to provide special assistance.

Clean up and recovery

If you need to evacuate your home during a weather emergency, the American Red Cross offers guidance about checking utilities and major systems in your home to help determine what you will need to repair to make your home habitable again.

‘Tis the season for holiday safety

Getting ready to decorate your home for the holidays? This article provides important tips from some experts on holiday safety.

Lighting up the night

The International Association of Certified Home Inspectors (InterNACHI) recommends that, whenever possible, you purchase new lights made with flame-resistant, flame-retardant, and non-combustible materials. If you are using old lights, check for any damage before plugging them in or consider replacing them altogether. Problems to look for include:

• Frayed wires
• Broken sockets
• Loose connections

Safe extensions

If you need to use extension cords, do not overload them. The United States Consumer Product Safety Commission (CPSC) suggests using no more than three standard-size sets of lights per extension cord. Also check that they are rated for outdoor use for any outdoor lighting. InterNACHI recommends that you keep both lights and cords away from young children and pets.

How you mount your lights is important too. InterNACHI advises stringing them through hooks or insulated staples without using nails or tacks. Handle lights carefully; never pull or tug them. Before hanging lights outside the home, check the label on the box to ensure that they are certified for outdoor use. Only plug lights into circuits with ground-fault circuit interrupters to prevent electrical shocks.

Another important warning: never use electric lights on a metallic tree due to risk of electrocution. When you are heading to bed or out for the night, always remember to shut off all holiday lights for safety. A timer may help you save on your electric bill and effort spent turning lights on and off.

Also make sure that holiday lights, extension cords, and tree wraps will not interfere with mobility devices.

Decorating do’s and don’ts

CPSC recommends using only non-combustible and flame-resistant decorations wherever possible. Avoid tinsel or artificial icicles made from leaded materials, which are hazardous if swallowed by children. It also recommends not using sharp-edged and breakable decorations or those with small removable pieces. If you have small children, do not use decorations that look like candy or food. They are dangerous if swallowed. Another great tip: wear gloves while decorating with spun glass “angel hair,” which may be irritating to the eyes and skin.

Real vs. artificial trees

These days, many artificial trees are fire-resistant. You can even use pine-scented air fresheners or diffusers to help create a real-tree feeling.

If your heart is set on a real tree, CPSC offers these tips for choosing a fresh tree:

• A fresh tree has needles that are hard to pull off the branches
• Fresh needles should not break when they are bent between your fingers
• A fresh tree trunk should be sticky with resin
• If a shower of needles falls off the tree when you bounce it on the ground, it is too dry

Keep it cool

To maintain freshness, keep your tree away from all heat sources, including fireplaces, radiators, and portable heaters. CPSC advises that trees can dry out fast in heated rooms, which can be a fire hazard. It also recommends trimming about two inches from the tree trunk before placing it in a sturdy stand that is filled with water. Check the stand often and refill with water as needed.

Two more safety tips: never use real, lighted candles on a tree or close to a tree, and test your smoke alarms to make sure they are working properly.

Special deliveries

If you have ordered gifts online or from catalogues — or are anticipating holiday deliveries — leave a note on your front door instructing that packages must not block the doorway, or that you may need extra time to answer the door to sign for packages.
You can also ask family members and friends for their assistance in:

• Transporting packages in and out of your home
• Maintaining a clear path in high traffic areas
• Wrapping heavy or cumbersome gifts

High protein-low carb recipe: Pecan pie

Crust ingredients: 
1½ cups of almond flour
4 tbsp of butter, melted

Pie filling ingredients:
3 eggs, beaten
1 cup of granular sugar substitute suitable for baking
1 tsp of vanilla extract
2 tbsp of butter, melted
1 cup of sugar-free maple syrup
1½ cups of pecans (chopped or halved)

1. To make the crust, mix together the almond flour and the melted butter. Press into an 8- or 9-inch pie plate and put in the refrigerator for at least one hour (you can also make it the night before and refrigerate it overnight).
2. Beat the eggs and the sugar substitute together in a large bowl.
3. Add the vanilla extract, butter, and syrup. Then add the pecans.
4. Pour the contents of the bowl into the crust and bake at 350 degrees for about 45 minutes.

Yields 6 servings; 5.7 grams of protein and 4 grams of carbohydrates per serving.

Adapted from a recipe at food.com.

Start your search engine for caregiving

The Internet makes it easy to locate numerous online resources for caregivers. Fortunately, we have done much of that work for you by highlighting different resources that may help meet a variety of needs.*

The Muscular Dystrophy Association (MDA) understands the unique challenges of people who are caring for someone with a neuromuscular condition like Pompe. Its MDA Salutes Caregivers website provides helpful resources, including videos, magazine articles, links to other MDA resources, and caregiver organizations. You can also “cyber meet” other caregivers by reading their personal stories online.

General caregiving websites

Many organizations and websites are not specific to those who are caring for someone with a neuromuscular disease, however, they can still provide a wealth of information and support services.

The Family Caregiver Alliance provides fact sheets and publications and newsletters, and hosts a movement disorder discussion group. It also has a Family
Care NavigatorSM†, which is a tool for finding state-by-state help and locating possible funding resources.

The National Alliance for Caregiving offers informative booklets and web resources, and caregiver health self-assessment questionnaires. If you feel led to raise your voice to help support public policies and government guidelines, it provides a sample letter and a link to help you locate your state’s senators and representatives.

The National Family Caregivers Association has a caregiver community action network with more than 50 volunteer members who can reach out to family caregivers, providing education and support, and at times, teaching caregivers to be advocates for themselves and their loved ones. They also have a family caregiver forum where you can post questions, receive support, and communicate with other caregivers.

For those who are caring for a spouse, The Well Spouse Association offers respite weekends, national conferences, and workshops, support groups that are listed state-by-state, blogs, and a forum.

More helpful websites include:

• Caregiving.com: Publishes blogs targeted to those who care for their spouses, partners, children, parents, grandparents, and siblings. They also have forums, groups, videos and more.

• Next Step in Care: This United Hospital Fund website offers easy-to-use guides to help family caregivers, definitions of caregiving terms, and numerous links and resources.

• If you are caring from a faraway city or state, Caring From a Distance provides service directories and helplines, as well as a library of resources about topics that range from consumer issues to financial and legal concerns. You will also find a list of caregiving links and resources at USA.gov that address topics including government benefits, legal matters, long-distance caregiving, and more.

• Caring for aging parents or aging adults. There are additional challenges and contingencies to plan for when caring for aging parents or older adults. The Eldercare Locator is a public service of the U.S. Administration on Aging to help you connect to services for older adults and their families.

Keeping everyone in the loop

An easy way to keep family members and friends informed all at one time is by using tools that are available through websites like Caring Bridge, which offers personal and protected websites that can be used to post both health-related and social updates. You can also take advantage of SupportPlanner, a tool that allows you to post when you need help with household tasks like meal preparation and cleaning, transportation, and respite care.

Two other websites that may be used to recruit help from family members and friends are Lotsa Helping Hands and CareCentral.

*All of the websites and resources listed above are subject to change.
†Family Care Navigator is a service mark of the Family Caregiver Alliance.

Celebrating caregivers

November is National Family Caregivers Month, a month-long celebration to thank, support, educate, and empower family caregivers.

Day after day, more than 65 million family caregivers in the United States play a vital role in the lives of people with chronic conditions like Pompe. National Family Caregivers (NFC) Month is an opportunity to applaud all the caregivers who are very important partners to people living with Pompe.

Launched in 1994 by the National Family Caregiver Association, NFC Month was initially a one- week event, but was soon expanded to a full month. Since 1997, every U.S. president has issued an annual proclamation recognizing family caregivers across the nation.

In addition to celebrating the efforts of family caregivers, NFC Month helps raise awareness of family caregiver issues, educates family caregivers about self-identification, and increases support for family caregivers, according to the National Family Caregivers Association.

Join in If you visit the MDA Salutes Caregivers website in November, you can send an e-card to let your caregiver know how much you care. They also invite you to post a thank-you message to the caregiver(s) in your life.

For more fun ideas on how to thank a caregiver, look for the upcoming Pompe InCommon publication (Here’s to the caregivers).

To learn more about NFC Month, visit the National Family Caregiver Association website. You can also take advantage of its free membership, which includes a quarterly newsletter, Take Care, monthly e-letters, access to its educational library, and more.* Everyone who joins from now through NFC Month will also receive a free family caregiver toolkit and planner.

Give yourself a break

Taking an hour or two to get away and do something fun and relaxing is a way to help prevent caregiver stress and burnout. If you do not have family members or friends who can take over for you, there are some organizations that may be able to provide assistance to help you get a needed break, which is known as respite care.

The ARCH National Respite Network and Resource Center can help you locate respite and crisis care services in your community, as well as possible funding sources.

The Caregiver Relief Fund provides vouchers for at-home respite care for eligible caregivers, such as those who have been caring for someone with a chronic condition for more than 12 months and do not have an annual income that exceeds $80,000.

Support for teens and children in between

Some young siblings of children and young adults with Pompe may also be involved in caregiving. Caregiving.com
hosts a private chat group where teens and tweens can interact with other young caregivers.

The American Association of Caregiving Youth is an organization that is dedicated to supporting young students who are also family caregivers. Its Florida-based Caregiving Youth Project publishes Treasure Talk, a free monthly e-newsletter that includes caregiving tips and resources.

*NFC Month offerings and activities are subject to change.

Understanding some of the changes under the Affordable Health Care Act

The Compilation of Patient Protection and Affordable Care Act provides information about the healthcare changes that took effect on March 23, 2010, including reforms that will roll out over the next four years and beyond.

This article will help you understand how some of those changes may affect you right now. You can also click on "healthcare news" in the categories section to your right to read previous posts about other changes.

Under the Affordable Care Act, insurance providers must now:

1. Provide coverage for people who have pre-existing conditions under the pre-existing condition insurance plan (PCIP). You may be eligible for PCIP if you have been denied health insurance for a pre-existing condition and you have been uninsured for at least six months, are a US citizen, or live in the US legally. PCIP covers benefits that include primary care and specialty care, hospital care, and prescription drugs. Premiums vary depending upon the state you live in and which plan you choose. To learn more about PCIP and get information about plans, premiums, eligibility, and how to apply in your state, visit the PCIP website (PCIP.gov).

2. Eliminate pre-existing condition exclusions for children. Health insurance plans cannot limit or deny benefits to children under 19 because of a pre-existing condition, which is defined as a health problem that developed before the parents of the child applied to join the plan. This rule applies to all job-related health plans and individual health insurance policies. The exception is if your child has a "grandfathered" individual health insurance policy that was purchased on or before March 23, 2010. Learn more here.

3. Limit insurance company barriers to emergency services. If you need to access out-of-network emergency room services, the new rule prevents health plans from requiring higher co-payments or co-insurance (in some cases, you may still be responsible to pay the difference between the amount billed or paid). This applies to all group health plans and individual health insurance policies created or issued after March 23, 2010 ("grandfathered" plans are exempt). Learn more here.

4. Ensure your right to appeal. If your plan denies payment after your appeal, you can ask to have an independent review organization determine if the plan’s decision should be upheld or reversed (usually called an "external review"). This new rule is for health plans created after March 23, 2010. For plan or policy years beginning on or after July 1, 2011, if an insurer denies payment for a treatment or service, it is required to tell you why your claim was denied, inform you of your right to appeal (usually called an "internal review"), and your right for an external review. You will also be notified about the availability of a Consumer Assistance Program, if your state has one. When you request an internal appeal, your plan must inform you of the decision within 72 hours of receiving your request for an appeal after denial of a claim for urgent care, 30 days for denials of non-urgent care you have not yet received, and 60 days of denials of services you have already received. Learn more here.

5. Provide a standardized summary of benefits and coverage. On September 23, 2012, or soon after, all health insurance issuers and health plans must provide you with a standardized and easy-to-understand summary about your health plan benefits and coverage. You will also receive a glossary of terms that are commonly used in health insurance coverage. This is expected to make it easier to compare insurance options from one plan to another. Learn more here.

To learn about other changes, read "Key Features of the Law" and the "Patient’s Bill of Rights" at healthcare.gov.

The official White House website also provides state-by-state factsheets to learn about specific benefits that are available in your state.

If you have any questions, a Genzyme Case Manager can help by reviewing your current health insurance plan. To learn more about the Affordable Care Act, visit healthcare.gov

All information from healthcare.gov is subject to change (and some effective dates may vary based on an individual’s insurance plan and the state an individual lives in). Check with your insurer or Genzyme Case Manager.

NIH Introduces Rare Disease Curriculum for Middle School Teachers

The National Institutes of Health (NIH) has created an effective resource to help facilitate and foster an understanding of orphan diseases. Through its Curriculum Supplements for Middle Schools, the NIH now offers a free, two-week series of lesson plans that cover topics beginning with “What is a Rare Disease?” to “Communicating About Rare Diseases.”

Accessible at http://science.education.nih.gov/customers.nsf/MSDiseases.htm, these downloadable educational tools put into teachers’ hands valuable information on these little-understood medical conditions that may affect students in their classes. Imagine the benefits of having school children learn about rare diseases from a reliable source and within their existing educational environment. Imagine the impact if this resource was circulated to schools through parents and educators…and used in conjunction with Rare Disease Day 2013!

High protein-low carb recipe: Pumpkin bread

Pumpkin bread is great for breakfast, a snack, or even dessert. Perfect to serve warm on a brisk, fall day.

Ingredients:
2 large eggs
4 tsp of ground cinnamon
¼ tsp of ground cloves
½ tsp of ground nutmeg
½ cup of unsweetened applesauce
40 pecans
1 tsp of baking powder
1 tsp of baking soda
¼ cup of old fashioned oats
½ cup of mashed pumpkin (fresh or canned)
¾ cup of sweetener (for baking)
1 tbsp of oat bran hot cereal
3½ scoops of vanilla protein powder
7 tbsp of almond meal (or almond flour)
1 tbsp of milled flaxseed

1. Preheat oven to 325 degrees.
2. Mix all of the dry ingredients in a large bowl.
3. Mix all of the wet ingredients in a smaller second bowl.
4. Grease one loaf pan.
5. Mix the wet ingredients into the dry ingredients. Stir to mix, but do not overmix.
6. Pour into the loaf pan.
7. Bake for 20 minutes (watch for even browning) or until a butter knife comes out clean.

Yields 16 servings; 7.6 grams of protein and 10.6 grams of carbohydrates per serving.

Adapted from a recipe at recipes.sparkpeople.com