Monday, April 25, 2016

Dirty Little Secret

My daughter eats like she has LPLD.  Because it's what keeps her healthy.  Because she has LPLD.

But me?  I don't.

For some reason, I imagine that in other families with a child with LPLD, the whole family eats like they have LPLD.  But we don't.
Foreground: LPL safe cake.  Background: fat-filled cake.  And a small child's hand going for the frosting that I did not notice while taking the picture.  Ha!

We sometimes all eat the same thing, like pasta primavera, or a chicken stir fry, or my husband's amazing orange chicken (once I nail down how he makes it, I'll post it!).

But most meals?  Not so much.  We'll start cooking all the same way, but then at the part where we add cream or butter, we'll pull Monica's portion out (or enough for Monica's meal plus leftovers for her), and do something slightly different with it.

So the recipes I include on this blog are what I do with Monica's portion, but it's not what I always eat myself.  I feel guilty admitting this to you, dear readers, especially, because for the most part I try to be really upbeat about this diagnosis - it's not so hard!  Look at all the things you CAN eat!  And it's controllable by diet, without weird medications with crazy side effects, how lovely!  And so I feel like I'm cheating you, since it might not be ALL bad, but it's certainly hard enough that I don't personally eat an LPLD diet.  Sometimes I'll even add fat to the rest of the family's food more than what it really needs, especially Mary's, since she doesn't have LPLD and because fat is a necessary part of nutrition, and I worry Mary doesn't always get enough of it.

Even with Monica sometimes I feel like the goal of cooking is to put as much fat as she can handle into her food (which isn't much), and to make sure it's as tastey and nutritious as physically possible so she get's it all!  That's why we do grass-fed meat, wild caught fish, expensive butter, and coconut oil; it's not entirely that I'm a food snob!  When Monica DOES get to eat fat, it's going to have all the essential fatty acids and fat-soluble vitamins that I can find!

As for my husband, Mary, and me, we eat a pretty normal-fat diet.  I don't think Monica feels left out or separate from us, since our food generally looks similar, but it does cross my mind that if I were the best possible mother, I would eat exactly what she does.  But that's not realistic.  Monica is different, and she will be eating differently for the rest of the life.  Pretending her diet isn't different will make me feel better now, but it won't develop the coping skills that she needs to have for the rest of her life.  It sure hurts momma to see your baby developing coping skills, though!

In addition, I have been breastfeeding and/or pregnant every day of Monica's life, and I need fat for my babies.  It's not good for me to be on a drastically low fat diet just for me to feel better about Monica not feeling left out, as that would hurt my other children.  (I will always be curious as to whether a baby with LPLD that is in utero can somehow process and obtain fats in a way that the baby can't after that umbilical cord is cut... I'll let you know if I find an answer to that!)

So, that's my confession.  All the recipes that I post here are pretty tastey and awesome, but they are not what I eat myself all the time.  I don't post the fatty things that I eat because I want anyone with LPLD to feel welcome here, and not exposed to references to tastey things they are trying to avoid.  So, bring on the angel food cake!  Yum!!

Monday, April 18, 2016

Huevos Rancheros!

We went camping over the weekend (just plop camping, out of our car, still fun!) for a night, and this is what we had for breakfast.  So good! Monica devoured hers!
Can't get it in her mouth fast enough!
Dash olive oil
Minced red onion
1 tablespoon sugar
Dash salt
Dash pepper
Dash garlic powder
Canned chopped tomatoes
Canned fat free refried beans OR canned black beans (or both!)
Corn tortillas (or other fat free tortilla of your choice)
Fat free cheddar cheese
Egg white OR egg beaters OR whole egg (5 g of fat per egg)

Optional: Diced avocado, fat free sour cream, minced fresh cilantro, jarred sliced pickled jalepeno

Heat olive oil over medium heat in a saucepan until hot; add onion, sugar, salt, pepper, and garlic powder.  Sautee until soft.  Add one can chopped tomatoes.  Cook until heated through.  Set aside in separate bowl.  Use hot greased pan to cook eggs to your liking (over easy, scrambled, whatever).

Assemble by smearing beans over tortilla, topping with the egg, and then tomatoes, cheese, and whatever optional toppings you like!

Our family doesn't even both to use forks.  Scoop it up with both hands and prepare to make a (delicious) mess!

Monday, April 11, 2016

Why I'm Not Thrilled About Glybera

Have you heard about the cure for LPLD?  It's been making news for a few years now, but just in case you don't listen to the same news I do, here's the deal:

What:  Glybera is the brand name (like Tylenol) for alipogene tiparvovec (like acetaminophen), and it is a therapy with the goal of replacing the lipoprotein lipase enzyme in large muscles of the body in patients with LPLD, to make the symptoms and the disease go away.  In order to make the body make LPLD, the drug has to get a gene sequence for the LPL gene into the DNA of the patient.  Gene therapy!   It is undergoing testing in Europe.  From what I've heard, it was denied the chance to do testing in human subjects in the US at this point.

Who: Glybera is manufactured by the company uniQure.  I am always wary of pharmaceutical companies because they stand to make a lot of money off of patients. 

How:  After receiving an epidural-type numbing medication (or being sedated), patients receive up to  60 injections into the large muscles of their legs to introduce the DNA that will join up with their own DNA and start to manufacture the lipoprotein lipase enzyme.

Where:  Lipoprotein lipase is naturally located in just about every tissue of your body.  The injections are only into a big muscle because the muscle is easy to get to, and your legs use a lot of energy, so they could definitely benefit from the extra energy contained in those pesky triglycerides, taking them out of the blood!

When:  All the individuals involved in the studies have been adults.  No one is even thinking of using this in kids anytime soon.

My LPLD girls!
Why:  That is the question!  Why would this treatment be right for you or your loved one?  I have to admit, I would have to be having a lot of trouble with my LPLD to consider this treatment.  And there are plenty of people out there that are!  As for us, here is my take:

Pros of Glybera:
  • First gene therapy to be approved (in Europe), ever!  How cool is that!  We are entering the age of gene therapy, a topic of sci fi novels for ages!
  • Well designed testing and treatment - I have a little bit of a background in research and with the adeno-associated virus that they use to 'infect' the patient's cells with the new DNA, and I find it all very clever and exciting!
  • Rare diseases like LPLD hardly get any attention from drug companies, it's wonderful that someone is looking into a cure!
Cons of Glybera:
  • Price tag of $1 million.  Yikes!  Admittedly, it might be a fair price given all the research that has gone into it, and the small percentage of the population that actually has LPLD and therefore might benefit from the treatment, but for a disease that can be largely controlled by diet, that's a tough call to make.  For my family, without any episodes of pancreatitis, it's not worth it at this time.
  • First gene therapy to be approved (in Europe), ever!  How scary is that!  We are entering the age of gene therapy, a topical of sci fi novels for ages because there is SO MUCH that we can imagine going wrong when you start to mess with DNA.
  • Effectiveness - from what I've seen, this treatment will not let an individual with LPLD eat like someone without LPLD.  At best, an individual experiences fewer episodes of pancreatitis, while maintaining a very low fat diet, than they did before.  But maybe that's because the only patients trying this out are ones who have many episodes of pancreatitis in their history.  Maybe someone with LPLD who has never had pancreatitis would get to eat macaroni and cheese every day, and still not get pancreatitis.  An interesting thought... but it's a lot of money and pain to go through just for mac and cheese!
  • Longevity - from what I've seen, the effects only last maybe 10 years.  Maybe less.  After that, maybe you spend another $1 million to get retreated?  Or you go back to eating the very low fat diet you were on before?  Hmm.
  • Immune suppression - patients with LPLD may have to take additional medications so that their immune system doesn't fight off the DNA that Glybera is introducing.  This means the individuals may be more susceptible to other infections, since their immune system is kept from working as well as usual.
  • Not an option until my girls are 18, at least, anyway!
In summary: it's best to control this disease with diet.  For those who struggle with dietary control, especially if you were diagnosed later in life or have developed diabetes, and are suffering from numerous bouts of pancreatitis every year, this might bring hope of better control.  This treatment is incredibly expensive and not available in the United States, so, in the meantime, I will continue to do all that I can in my little blogging world of making dietary control a less daunting!

Interested in a more detailed description of the trials Glybera has already gone through?  Try here

Tuesday, April 5, 2016

Bunny Bread!

I wasn't planning on posting this, but my mom liked it a lot over Easter, so maybe you will, too!  It's a fun breakfast bread that we finished every scrap of this year.

Not perfect, but the girls thought it was sufficiently bunny-like
In your breadmaker, combine 4 cups whole wheat flour, 1/4 cup honey, 2 tablespoon yogurt, 3 eggs (or just egg whites), 2 teaspoons grated lemon peel, and 1/4 cup apple sauce.  Start on the 'dough' cycle to mix it well.  Turn off breadmaker once mixed, place 1/4 cup whole wheat flour on top of mixture, with 1 envelope yeast and 2 teaspoons of salt on top of the dry flour.  Allow to sit for 12-24 hours before running the 'dough' cycle fully.

Add 1/2 cup raisins and 1/2 cup dried cherries to the dough as you knead the dough until smooth and elastic.  Placed in a greased bowl, turning once to grease the top.  Cover and let rise for 1 hr or until doubled.

Shape into a bunny; cut off 2/3 of the dough and form it into an egg-shaped ball.  Place onto your baking sheet.  Cut the remaining dough in half, form one into a teardrop shape.  This will be the bunny head and ears.  Roll and pull the thin end longer, then cut the thin end down the middle to be the two ears, attached to the head.  On the narrow part of the egg-shaped body, press down on the edge of the dough to make an indentation where the head will rest and position it.

Cut 1/3 of the dough remaining, make a rope 6 inches long, make a u-shape and slide it until the body and head, leaving loose ends sticking out about 2 inches to make the front paws.

From the last of the remaining dough, make a rope 9 inches long, and make the ends fatter and the middle thinner.  Fold the rope in half and make a ball in the middle to be the tail.  Tuck the thin parts under the bunny body and leave the tail and ends of the back legs sticking out.

Cover your bunny and let it rise about 30 min.  If desired, then use scissors to form rabbit toes, slits for eyes, maybe tiny cuts for eyelashes.  Pinch the nose into place.

Bake at 350 for 40 min.

Serve with bunny salad (made with canned pear halves, cloves for eyes, marshmallows, and a maraschino cherry):