Saturday, November 21, 2015

Pumpkin cookies recipe

Adapted from a variety of recipes online, this was my first successful cookie adaptation that was relatively LPLD safe.  Tell me what you think!

LPLD Pumpkin Cookies
1/4 cup coconut oil (54 grams of fat total, divided by the number of cookies you make, I made 52, so just a little more than a gram per cookie! Plus since there's so many medium chain triglycerides, we count coconut oil to be half the fat that it really is)
2 cups pumpkin puree (canned or pureed yourself)
1 teaspoon almond extract
1 cup granulated sugar
1 cup brown sugar
2 teaspoons powdered egg or 2 egg whites
1/2 teaspoon salt
1 teaspoon baking powder
1 teaspoon baking soda
2 cups whole wheat flour
1 teaspoon ground cinnamon
1/2 teaspoon ground ginger
1/4 teaspoon ground cloves
1/4 teaspoon ground nutmeg

Mix all the wet ingredients (oil, pumpkin, extract, eggs) and sugar together.  Mix all the rest of the ingredients together in a separate bowl.  Stir together into one bowl slowly, combining thoroughly.  Scoop heaping tablespoons onto cookie sheets (either greased, lined with parchment paper, or use stoneware; the latter is our preference).  Bake at 375 degrees for 20 minutes or until just brown around the edges.  Let cool for 5 minutes on the cookie sheet, then transfer to cooling racks.  We like to then 'frost' them by dipping them in powdered sugar, but that is optional.  Makes about 50 cookies.
Monica is good at frosting!

Ingredient Essentials

A Tour Through Our Kitchen

Our kitchen has certainly changed since Monica was diagnosed!  I've always enjoyed some weirder or more complicated aspects of cooking and trying new things in the kitchen.  I remember trying to make after-dinner mints when I was in elementary school and winding up with cookie tray upon cookie tray of mooshy, minty, blue puddles.  Yuck!

The first thing we changed when Monica was diagnosed at around 18 months was her milk.  We gradually switched her from whole milk to skim, a week at a time.  First we started filling her bottles (yep, she was definitely still drinking from bottles at that age) with a quarter of 2% milk, three-quarters of whole, mixed together.  After a week of that, she had a week of half a bottle of 2%, half of whole.  Then a week of a quarter whole, three-quarters 2%.  And so we went from whole to 2% to 1% to skim without much difficulty at all.  It took a long time, but just by this one change we were able to drastically reduce her triglycerides.
We do drink organic milk when we can, but there's nothing wrong with drinking conventional.  With milk, we are a little worried about hormones in the milk affecting our girls.  But if money or groceries ever came down to choosing conventional milk or no milk at all, we would be buying up that milk, no matter what!

The hardest thing for us to do without was cheese.  What I did at first was learn to make my own; we were living in Las Vegas at the time, and I was able to get milk from Trader Joe's that would make a survivable mozzarella.  The hardest part of making milk, in my opinion, is finding milk that will actually turn into cheese instead of, well, mush!  I had many many heartbreaking episodes.  I used recipes and supplies from The Cheese Queen and would make large batches and freeze what we weren't going to use soon.  I made mostly mozzarella and sometimes cheddar.  I rarely ate it myself because it was so precious; we would really only use it on pizza for Monica.  It was crumbly and not particularly flavorful, but it was full of calcium and it melted sufficiently.

Then a miracle occurred.  Look what we found at Whole Foods:
I am still amazed that this exists.  I want to buy stock in their company.  I rarely get to go to Whole Foods anymore, since we live in a much smaller town without one nearby, but when we go to the city or visit family, I literally buy them out of this stuff.  It freezes great.  It's admittedly ridiculously expensive, something like $6.90 for each of these blocks.  But man.  We never let it go bad since we freeze it, and it melts beautifully and Monica loves it.  And, perhaps most importantly, I don't have to deal with the hassle and stress of making (and often failing) at making cheese.  HURRAY!

These are sold at our local hippie shop and I couldn't believe the ingredients.  No, really, I asked the shop owner to call her supplier to confirm (another reason I love this store).  Can you read the ingredients?  I made it extra big to try... just flour and salt!  That's it!  So lovely!  Admittedly, we will often lightly fry these in olive oil as a special treat for those of us without LPL, but being able to serve the same food to everyone in our family feels so good.  My next project might be to try to get good at making these myself at home, since we won't be living in this town forever.  Nothing like having a backup for my first few attempts that might fail right when dinner requires their success!

Sauces are key.  At least for toddlers.  Look at all these vinegars that we have on hand all the time for different flavors!  White wine, balsamic, red wine, and rice; lemon juice can really kick flavor up, too.  My husband has become quite the sauce-maker!

Sometimes a from-scratch dip or sauce is too much to ask for.  Ketchup, barbecue sauce, fat free salad dressings, even pickle relish and sauerkraut all can save the day.

Our girls seem to adore anything with tomato sauce.  We make a giant batch about once a month and freeze some of the jars, but we will use a store bought sauce, instead, in a pinch (canned or glass both work great, check the nutrition facts on jarred sauce though!).  Soy sauce is another key item that Monica will eat as a dip or a sauce.  Molasses is a nice sweetener for my girls because it packs some extra iron for nutrition (apparently girls who don't get enough iron end up having more trouble in school, interesting!).  Maple syrup and honey are handy for turning a regular snack into something special without any fat, in addition to a fat free topping for pancakes and waffles.

There's the honey!  Yum.  Jellies and jams are fat free.  We like to have a jar with a lid poked with holes, filled with a cinnamon/sugar mix for putting on top of toast or snacks that could use a little extra something.  Like sliced apples.  Delicious!

Greek yogurt and fat free cream cheese are for the times when you need a little creaminess, or a creamy dip!  I'll even add these to peanut butter, to make a peanut buttery dip that's lower in fat and higher in calcium.  Win-win!  The yogurt pictured is a container of full fat yogurt that I have filled (see the label on top in marker? ha) with yogurt that I made myself with a perpetual culture that I bought from Cultures for Health almost a year ago.  It's lasted that long!  It might be cheaper to make organic yogurt than to buy organic yogurt, but I'm not positive.  It certainly uses less packaging, and I find it easy and enjoyable.
The blurry cultures that we use the most - sourdough (for our pizza crusts) and Greek yogurt.

Another way to add creaminess to a dessert or sweet recipe; fat free sweetened condensed milk.  Apple sauce is the classic baking substitute for butter; moist baked goods without the fat!

I bought this years ago for a biscuit mix for backpacking, but I'm starting to use it in Monica's baked goods.  One teaspoon is supposed to replace one egg, but I'm finding it's a little too strong at that ratio!  It contributes to the 'gumminess' of baked goods, the 'stick-together-ness,' if you will.

What about desserts?  Angel Food Cake is a staple; I once found a box that listed a recipe for how to make angel food cupcakes that I cut out and will save forever.  Elementary aged kids just HAVE to have cupcakes sometimes, it seems.  I'll top it with an egg-white-and-sugar frosting, or with sliced fruit or a sugary fruit sauce.  We keep a few, pre-frosted, in the freezer for emergency cupcake needs (what, you've never heard of those?  They happen.  Mostly when parents at school don't give us a heads up that they're bringing treats for their kids birthday.  Monica has been the kid in the corner trying to look cheerful sipping water as the kids around her demolish their brightly colored sugary treats one too many times for this mom...).

This is our favorite brownie mix ever, an awesome find at Trader Joe's, that I believe they have discontinued.  The best part was that they had a single serving size recipe that has helped us immensely in tight, last minute, fixes.  Like before Thanksgiving (What?!?  No pie that she can eat?!??) or other kids birthday parties for which I didn't plan ahead.  You mix two tablespoons of the mix with one tablespoon fat free greek yogurt, stir it up real well in a mug, microwave it for a minute, et voila. Add marshmallows or sprinkles for extra gold stars.  Anyway, I'm currently using my last box to compare my experimental mixes with, to try to make something myself from scratch in case I never find it at Trader Joe's ever again.

If you have a sorbet or cupcake that is less-than-exciting (definitely happens with baked goods are this house, often), sprinkles are a great way to save the day.  The bigger granule types can sometimes have small amounts of fat.  The tiny crystal types are basically colored sugar and are totally fat free.  Or put some chocolate syrup on top, also fat free.  Or both!

Dream whip is powdered whipped cream that you can mix up with some fat free milk for a last minute dessert topping.  I know they sell 'fat free' whipped topping at stores, but my general rule is that if I can choose between a food that will go bad, and a food that seems to never go bad, I choose the food that can go bad.  Preservatives?  Real-foodiness?  I'm not sure.  I don't know if I'm supported by science in this, but that frozen whipped topping weirds me out.  Dream whip, less so, but it's still not something we eat on a weekly, or even monthly, basis.  The Jell-O pictured I've had for at least 5 years... but the real gelatin that we use isn't nearly so pretty.  It's off-white and in a giant bag since I use it a lot.  I use recipes from Kitchen Stewardship that consist of just gelatin (from Azure Standard) and juice to make jigglers that the girls love.  Not too much sugar + fun shapes and colors = dessert!

The few other LPLD folks I've ever met swear by these, so I searched far and wide to find them.  They are fat free and have chocolate and marshmallow and are so tasty that even people withOUT LPLD love them.  I buy boxes and boxes whenever we find them.  It is so hard to find chocolate things that Monica can eat (pretty much this, chocolate syrup, chocolate brownies that I've already mentioned... that's it.  LPLD is rough on chocolate-lovers).

For snacks, a fruit leather is perfect for all my girls.  Well, not very filling, but it's something that everyone can have, at least, and can live in my purse forever in case of emergencies.  These are my favorite, sold by Azure Standard.  They are thicker than you would expect any fruit leather could possibly be and are all delicious.  And I can buy them in bulk!

Finally, for the small amount of fat that my lovely LPLDers DO get to eat, how do we make sure it's the best, with lots of fat-soluble vitamins (A, D, E, and K!) and essential fatty acids?  I'm currently figuring out how to make breastmilk work; they make fat free formula for babies, but in my opinion, if your baby hasn't had pancreatitis yet, then the benefits of breastmilk outweighs the scary high triglyceride levels!  I'll keep you posted on how things go with Teresa, our newly diagnosed 6 month old, and the milk separator that I have ordered (from the Ukraine.  Huh.)  Our geneticist recommended that Monica get 1 mL of walnut oil every day when she was first diagnosed.  It is chock full of lots of good things, but how do you get a kid to swallow straight oil?  For about the next two years, I would get an ice cube tray and put 1 mL of walnut oil in each spot, followed by two small spoonfuls of sugar and one small spoonful of cocoa powder.  I would mix each cube spot meticulously, and then Monica would get to eat a scoopful of her very own 'chocolate' every day!  We kept it in the fridge and it worked well, but now I use the walnut oil in muffins for her, and hope it adds up to approximately the right amount of good stuff, even if she doesn't eat a muffin every day.  She is also older now and eats a greater variety of food in general, as opposed to as a toddler, when a day's intake might include three noodles and an apple slice, and that's about it.  A toddler will ALWAYS eat a spoonful of sugary, gritty, oily chocolate, it turns out.

That's maybe the hardest part of raising a child with LPLD; balancing enough fat for brain development without getting close to pancreatitis.  So we do try to make sure she gets some fat every day, and try to make it the best possible fat.  Coconut oil is a great one.  Eggs aren't pictured but we use frequently (5 g of fat each!), both whole and separated for their whites (mom and dad get more cream brulee and home made ice cream with lots of egg yolks, darn!).  We can get free range eggs from our hippy shop that are delicious with bright yolks.  I've rendered my own lard from local grass-raised pork that I sometimes cook with.  Olive oil is the go-to fat for our whole family in cooking and sauteeing.  I try to use grass-fed butter on the rare occasions that Monica gets any.  Finally, a frequent and popular snack is a few (10?) almonds or pecans.

So that's a basic run down of how we take care of Monica in the kitchen.  I hope this helps encourage anyone with a new diagnosis that there really is a TON that you can still eat!  Please comment with what you wish to know more about - how I do something, why I do something.

Vegetable Lo Mein

One of our favorite LPLD dinners, fresh and delicious.

8 oz angel hair or spaghetti pasta (we use whole wheat)
3/4 cup chicken broth
1/4 cup soy sauce
1 tablespoon corn starch
2 cups chopped celery
2 cups fresh mushrooms, sliced
2 cups sliced red peppers
1 sliced onion (we prefer red onion for more flavor)
2 cups bean sprouts
2 cups snow peas

Bring a pot of water to a boil, add the pasta and cook for as long as the box says to; drain.

In a small bowl, whisk together chicken broth, soy sauce, and cornstarch.

Heat a wok or large saucepan over medium-high heat.  Add a tiny dash of olive oil, if desired, or use the vegetables own juices to cook them, as you add celery, mushrooms, peppers, and onion.  Cook about 3 minutes, then add the broth mixture, bean sprouts, and snow peas.  Continue to cook and stir about 5 minutes, until vegetables are tender but still crisp.

In a large bowl, mix the cooked pasta and vegetable mixture, and serve.

Adapted from

Forgot to get a picture until we ate it all and put away the leftovers!

Tuesday, November 17, 2015

Toddler eating + LPLD = HELP!

Part 1: When mac and cheese is not an option

Feeding a toddler is hard enough.  When you can’t rely on delicious fatty standbys that it seems every kid loves (macaroni and cheese, hot dogs, chicken nuggets, and pizza come to mind), what’s a parent to do on those desperate days?!?

Max out on dips and sauces

Sometimes all it takes is a dip that she gets to carefully place on each and every bite.  Just be sure you ask where on the plate she wants it, or you might end up with a tantrum, right?!?  Some key dips that we always have on hand:

Maple syrup and/or honey
Barbecue sauce
Asian orange sauce (great on vegies!)

Sometimes I’ll mix up some nonfat yogurt with a little honey for a sweeter dip for fruit or breakfast.  Or add a little mustard to the honey and yogurt, for a honey mustard sauce.  And then steal some for myself.  Yum!

Eat the same thing – and set a good example yourself
Bad news: a lot of bad eating habits come from kids watching us parents.
Good news: correcting our own bad habits is good for us AND benefits our kids, LPLD or not!
Some bad habits that we’ve had to correct over the years:

  • Avoiding unhealthy snacks between meals, and avoiding all snacks an hour or less before meals – if we are very hungry, choosing a piece of fruit, some air popped popcorn, or some carrot, celery, or bell pepper sticks is tough but worth it.  This helps address my own emotional eating, as well!
  • Sitting down to eat meals – especially when I can SEE all the dirty dishes that need to be washed, and I finished my meal 30 minutes ago, it is SO HARD to stay seated while my kids finish up; but no one likes to eat alone, and it’s a great time for your child to talk with you about whatever is on their mind
  • Eating my own vegetables and showing enjoyment – if the vegetables you are eating aren’t that great, maybe you need to find a new way of preparing them, for both your sake and your kids!  I’ve had luck with trying something new from my cookbooks, as well as using my friendly Google search engine to find ways to cook the vegetables I have on hand in a way that other people in the internet have enjoyed, too.  I didn’t know I was overcooking my asparagus – when it’s still crisp, it’s so much more enjoyable, and then a little broth and lemon juice make it just fabulous!
This kid needs energy to stay active!

What bad habits have you broken (or do you need to break, ha!) for your kids, and then benefitted from yourself, too?

Monday, November 16, 2015

What is LPLD?

What do you tell your friends?  Family?  New teacher at school?  Restaurant server?

Here's a run down of my basic responses:
30 second answerfor waiters, ice cream scoopers, the mom giving me a funny look when she overhears me telling my daughter that, no, she can’t have the cheese everyone else is having for snack – but I’m peeling this delicious orange for her!
My daughter has a sort of allergy to fat – she can eat very small amounts of it, but any more than that will make her very sick and put her in the hospital.  So it's important to know what she eats.
Short answer – for the slightly more than casual acquaintance
Monica has a rare disease, one in a million chance of getting it, where she is missing an enzyme that supposed to be in every tissue of her body that lets her body use fat for energy.  So instead, the fat builds up in her blood and can make her pancreas very sick and put her in the hospital.  This hasn't happened yet, but we are very careful that she eats no more than about 10 grams of fat per day.
Teachers and parent friends - here’s the actual letter we gave to Monica’s teacher this year, both for her edification and to pass out to other parents in class.  So far, we’ve gotten nothing but positive feedback.  I know I sometimes feel like a burden, unnecessarily complicating people’s lives by asking them to provide foods that my daughter can eat, but I am constantly surprised by how much people DO want to help even a single little girl to feel included!

Feel free to copy this and use it yourself!

Befriend your restaurant employees!

Step 2 in eating at a restaurant with LPLD
(Step 1 can be found here)

Whether you cook every meal from scratch at home, or are a take-out connoisseur, things will have to change with the diagnosis of LPLD.  I plan to have many posts on recipes on how to cook at home, but what about eating out?  Is it even possible to maintain your diet with LPLD, and also eat out at a restaurant??

Yes!  We hope the following guide will help you make good choices at restaurants, whether a 5-star Vegas bistro, or the mom-and-pop joint across the street.

First, talk with the cashier, waiter, manager, even the Chef!  Ask about healthy menu options or a separate menu for lean choices.  Be careful: low carb and low calorie do NOT necessarily mean low fat!   Fast food restaurants often have  a list of nutrition facts posted; look for food options that are low fat.  Your daily fat intake goal depends on your doctor’s or nutritionist’s recommendation, but we usually look for items less than 10 g of fat, and then think about how much fat was eaten over the rest of the day. Don’t even look at the other options that are too fatty! Don’t tempt yourself!
All right, the colorful plate gives this away as homemade... but these mushrooms, curried cauliflower, and shrimp would be delicious any place you can get them!

Specific ideas:

  • For breakfast, oatmeal, a smoothie, or yogurt with fruit often work
  • For a salad dressing, or to dip French bread into, try just balsamic vinegar without the oil; it’s got kick and a lot of delicious flavor!  
  • Ask your waiter to have the cook staff hold any cheese, butter, or added oils
  • Ask your waiter to double or triple the vegetables, offer to pay extra; often they won’t charge you, and you’ll have something to really fill you up!
  • Try not to be worried about making so many demands and requests; you are the one who will have to pay for the consequences, through your health and hospital bills, if they make a mistake.  So be polite, but speak up!
Sauteed vegetables in just a touch of olive oil are delicious and beautiful!
It is often harder to find good food choices at fast food places than ‘real’ food restaurants.  Sometimes a grilled chicken filet, salad without dressing, or chicken wrap with a fruit salad is an option, but often not.  Look at the kids’ menu, too; sometimes a kid sized burger can satisfy a craving without putting your fat grams through the roof!

If a restaurant treats you well, then tip the staff well, and become a regular customer so that you build a relationship of trust with them!  They’ll be more likely to continue to offer foods that are good for you, and maybe add to their menu with you in mind!

Saturday, November 14, 2015

Keywords on Restaurant Menus

Step 1 in eating at a restaurant with LPLD

Don’t arrive starving at a new restaurant!  Eat an apple in the car before you go in to make sure you take your time to make good choices.

Certain keywords can tip you off right away to good choices or meals to stay away from.  But also talk with your server about how it’s prepared to make sure you understand the menu correctly:

Friday, November 13, 2015

What can I order at a restaurant?

Reliable standbys available at most any restaurant

Sometimes I just don’t feel like dealing with the hassle of explaining our diagnosis and specific treatment and management of the diagnosis to our server as well as trying to decipher the ingredients on the menu.  Our favorites that can be ordered almost anywhere with minimal fuss?

  • Grilled chicken with steamed vegetables
  • Pasta with marinara sauce (and no cheese, oil, or butter)
  • Pizza without cheese (but plenty of vegetables!) (some crusts have more fats than others; for us, it's just another reason to go to local pizza places instead of chains)
  • Shrimp with cocktail sauce and brown or white (just not fried) rice

Be sure to ask to have any sauces or dressings that you’re not sure about put on the side so you can limit how much you eat.

What are your faithful standby meals that you can order almost anywhere with minimal fuss?

Thursday, November 12, 2015

A Letter to Monica

Monica was a toddler when she was diagnosed, but first we went through multiple doctors who thought she had cancer. It was a difficult few months, but life is back to being wonderful as a family again.

Impeccable fashion, around the time of her diagnosis
Dear Monica,
At your 2-year-old well child check with Dr Z, you looked healthy but he was concerned that you weren’t saying 2-word phrases yet, and recommended speech therapy. I wanted to check on whether you were getting enough iron and asked for a CBC to check for anemia, and to see if you had a gluten allergy that was giving you a hard time gaining weight. After we had your blood drawn, I received a phone call from the lab; they told me some of the blood cells had looked funny and we needed to bring you in for a leukemia/lymphoma panel. I was horrified. Dr Z was out, so I rushed to Dr C sobbing; he called the lab and verified what was going on, berating the lab tech for calling your mother instead of your doctor with the information. The lab stayed open late for us to bring you in for another draw, which you were very unhappy about, and your dad and I spent the evening alternating between caring for you and crying on the phone to your grandparents. We were so scared that you had cancer. Dr C called that night with the news that you didn’t have leukemia or lymphoma, but that your blood was so full of fats that the lab had trouble working with it. Your triglycerides were 2700, when they should be around 100, maximum. He had no idea what could be wrong, but he promised to do some research. Your father and I had a tough time sleeping, even with such good news, worried as we were about what was going on.
Dr C had looked into many options for your diagnosis, but none fit perfectly; you were otherwise so healthy! He decided to send us to a variety of specialists, to see what ideas they had. First we went to Dr O, a pediatric cardiologist; he had never seen a triglyceride level so high, and actually laughed at his own confusion, which was a little comforting and a little disconcerting. He did a complete physical exam and ultrasound of your heart (echocardiogram), and couldn’t find anything out of the ordinary. He commented on your diaper rash, and we agreed that it was weird, you’d had it for a few months and it wasn’t responding to anything, even some strong antibiotic ointment we’d been using since seeing Dr Z. Dr O ordered a few more labs, especially a recheck of your cholesterol levels, hoping that it was a lab error and that you were completely healthy. Your triglycerides were 250 this time which confused everyone; it was still high, but not nearly in the thousands; which lab was the correct lab?
Folk music star (with pink blood)
Next we saw a hematologist/oncologist, Dr A. I was very nervous about seeing a cancer doctor. On his exam, he felt that you had an enlarged liver and spleen, and I could feel it, too. He was also confident that your diaper rash had something to do with your illness. He was worried this could be another, more rare and more serious type of cancer, and ordered labs to that effect, but was also confused because you should be so sick as to be in the hospital at this point if you had those cancers. That was reassuring that you didn’t have them, but anytime your child is being tested for cancer is a terrifying time. We talked about what we would do if you needed a bone marrow biopsy, what it would be like. Dr A drew your blood again right in the office, and just looking at its pinkish, creamy color, he was sure that your triglycerides would be in the thousands and that the 250 level was in error (and yep, it was 2700). He made an appointment for us to see him again as well as a geneticist who only came into town a few days every month, Dr L. Dr A saw how nervous we were about finding out if you had cancer and reassured us that he would personally call us as soon as he had an answer.
That night was the worst we had had so far. All I could think of was what I would do if you had cancer; how I would drop out of school for a year to care for you and dad. Wondering whether I would ever want to go back to my career again. Trying desperately to love you even more, just in case this was one of the last few days I would have with you on earth. But also finding myself pulling away from loving you, since part of me was so very afraid of how much it would hurt if you died. I couldn’t sleep well, and woke early with Dad to just sit in the guest room and hug and cry and talk about our fears together. Dr A called soon that morning to tell us that the preliminary test looked negative for cancer; cancer and inflammatory markers were all very low. A few days later he called again to reassure us that you did not have those cancers. We were so relieved, but also so confused.
Shrinking violet; she put up with so many doctors appointments!

In the meantime, we saw a speech therapist for you; after a few appointments, she decided that although you were a little behind, you weren’t behind enough to need her care. You were adding words and phrases to your vocabulary quickly, and she had no worries that you would catch up easily. What a relief!
Next we met with Dr L on July 31, 2012, a geneticist with an Italian accent. He listened to your story carefully, and then told us his diagnosis: familial lipoprotein lipase deficiency. He told us the only real sure way to diagnose this was only done in Germany, as far as we knew, but since the only cure was to cut the fat in your diet to almost nothing, we should try it and see if it helped. We had lots of questions for him and he was a very kind man. It was a relief to talk to him, but also scary to hear of what your life would be like from now on: less than 10 g of fat per day, threats of pancreatitis if you ate too much fat and pancreatitis’ life-threatening potential. It was nice to finally have a name for what was causing your high triglycerides, enlarged liver and spleen, and diaper rash (xanthomas or cholesterol deposits in your skin), but the diagnosis meant big changes for the rest of your life. It was so much better than cancer, but it was still hard to be grateful. He felt your symptoms would resolve and your appetite would greatly improve if we could just get your triglycerides to less than 1000.
Good luck with that sand, Dad

Next we met with a gastroenterologist, just to make sure we were covering all aspects of what you could have. He disagreed with Dr L and dismissed our protests of how well the diagnosis fit; he had another disease, a congenital deformation of your bile ducts, that he felt was the cause, and he wanted a liver ultrasound to verify it. Since we wanted to make sure your liver was OK, anyway, and it was a very non-invasive test, we agreed. Mom lay with you on the bed while they did an ultrasound of your belly, while Dad danced around and did silly things to keep you happy. Thank God, your liver and bile ducts were perfectly normal!
In the meantime, we started your new diet gradually. Up until now, we pushed fats whenever we could to help you gain weight, and you drank whole milk all night. We started with the milk, each week slowly putting in more of a lower fat milk into your bottles, working from whole to 2% to 1% and finally skim. You never seemed to notice or mind. Hurray! We started watching what meats and desserts you ate, cutting out the chocolate chips that you had always loved so much. After a month on this diet, we rechecked your blood. The results were amazing: triglycerides of 529! We were overjoyed.
At our next appointment with Dr L, I just handed a paper with your lab results over, and relished watching his face as he slowly got to the good news: his eyebrows SHOT up. He was just as amazed and excited as we were. We talked about the next steps: adjusting your diet to keep you healthy, pushing iron until your levels were normal, getting in vitamins every day. But mostly we were all relieved and amazed at how quickly your body was recovering from all this with the proper (difficult) diet. We talked more about where this had come from; your father and I both had to have the rare recessive gene, but as far as we could tell, no one in our ancestors had ever had it. Our children all have a 1 in 4 chance of having the disease, and a 50% chance of carrying the recessive gene like we do. Only one in a million people have this disease. But we already knew you were one in a million!
Your dad and I had a few months of real difficulty with your diagnosis. Your dad was very angry at God for doing this to you. I was mostly sad, and kept thinking of all the foods that I love that I could never share with you, could never be part of your childhood. We did our best to find other exciting foods for you; chocolate syrup, meringue cookies, all the lollipops and sodas you wanted. Food allergies were becoming more and more common, and my friends reassured me that as you grew up, this would be just like having an allergy, and your peers wouldn’t ostracize you for it. You would not be an outcast for this diagnosis. Hearing this gives me hope that this won’t be such a burden for you to bear. One of the greatest fears of being a parent is the suffering that we know our children will inevitably have to face; we want to put off the pain of life for you as long as possible. But with this diagnosis, we have to not only have your blood drawn regularly, but have you fast beforehand, which is hard to explain to a toddler. This is a great difficulty, for you more even than for us. But the greater the difficulty, the more God can do with it. We are excited to see what goods God will bring you and the world from this illness. We love you very much.
Monica meeting her new sister for the first time, months after Monica's diagnosis

Wednesday, November 11, 2015

Amount of fat in common fats and oils

I loaded this table into my phone and use it all the time when trying to figure out what recipes would work for my daughter, as well as reminding myself how much fat she’s gotten in the past few days, and whether or not we can afford to splurge a little for a special meal!

What other fat-containing foods would you add to this table?

Living with LPLD is going live!

I've thought about this for a long time, and I'm finally acting on it.  The mission of this blog is to help other patients and parents who have lipoprotein lipase deficiency in their genes.  I want to provide support, recipes, my interpretations of medical research, and discussions for the difficulties of the day-to-day living with such a rare disease.  Maybe I can even help you to communicate with your doctor better!  Remember, I can't give you medical advice, so the bottom line will always be to talk with your doctor.