May 18, 2015

Explaining the PKU Diet

Growing up following the PKU diet, you get a lot of questions. You drink a special formula, say ‘no’ to many foods, and often eat different food – and people notice.

me chillin with my formula

Me and my formula

It can be hard to know how to explain PKU, because it is so rare and it’s unlike any food issue most people have ever heard of. After doing this for over 20 years, here are some thoughts I have. Hope they can help!

Note to parents: I believe it is very important to give your child many changes to explain PKU and their special diet themselves, starting at a young age. It may be easier for you to step in and do it for them, but this deprives them of a valuable opportunity to gain confidence and develop ownership over their diet. That independence will help them to be more consistent with managing the diet on their own, which is what you want!

A good rule of thumb is to keep it short. Then if they want to know more, expound.

You could start with, “I have a rare genetic disorder called PKU and I can’t metabolize protein, so I have to eat a really low-protein diet.”

Sometimes that is enough. Often though, people want to know more about what foods you can and can’t eat, what happens if you eat too much, and how you survive without eating protein.

This is where you might have explain that it’s actually just one amino acid in protein (but it’s in pretty much all food protein) that you can’t process. If you were to eat normal amounts of protein, this amino acid (Phenylalanine) would build up in your blood and cause brain damage. Here is a graphic I made to explain that concept:

Click to englarge and view in orginal post

Click to enlarge and view in original post

This leads in to answering questions about the formula. Many people know enough about nutrition to know that protein is essential to staying alive, and they wonder how you survive with so little protein.

You can tell them, “I drink a special formula every day that has all the amino acids, except Phenylalanine, plus other vitamins and minerals I need.”

For the most par I think your answer will just totally depend on the person asking and their interest level and background.

Other than that, be confident and enjoy being interesting! :-)

How do you explain PKU?

April 28, 2015

Interview with Alissa Doerner: A Classic PKU Story

Alissa is a special education teacher, a 5k runner, and a world traveler. She also has classic PKU. Here is her story.

Alissa on her recent trip to London, England

Alissa on her recent trip to London, England

What was it like growing up with Classic PKU?

Growing up with Classic PKU was just like anyone else growing up, I just ate differently. Classic PKU, my special diet, my formula, and all that came with having PKU was all I knew, so I just accepted it. From a young age, I knew and felt that I was different and needed different things than most. I was always on diet, but drinking formula was not my favorite thing to do as it wasn’t as tasty when I was very young. I knew it was what I needed to grow and to feel good so I stayed on diet and drank my formula every day. I stayed healthy and still am. PKU was and still is just a part of me.

What was your Phe tolerance as a child? What is it now that you are on kuvan?

My phe tolerance as a child was pretty low. I ate low protein food products and was limited in my daily protein intake. My formula was always my priority to get enough protein and vitamins. After finding out I was a responder to Kuvan, my phe tolerance has gone up. It is so nice to be able to have a little more freedom with my diet. There are some days when I’m hungrier and it’s nice to know that I’m able to eat some more. Whether it be having more potatoes, a piece of chocolate, or more dark green veggies, it’s been great to have more flexibility. My levels have been primarily in the 2-4 range since taking Kuvan.

What were/are some of your favorite foods to eat?

Artichokes, shitake mushrooms, and Brussel sprouts are my favorite vegetables. I could eat avocado, veggie sushi, veggie Pad Thai, low protein gnocchi, French fries or potatoes (regular and sweet) any day. I love just about any fruit especially strawberries, watermelon, pineapple, and papaya. I love low protein crepes, bread, cookies, pasta, bagels, and pretzels. There are so many more products for us nowadays, so I’ve definitely been taking advantage of that trying new things and ordering them and cooking and baking with them regularly.

Did you ever struggle to follow your diet?

When I was younger I went through phases where it was harder to accept and sometimes discouraged by the many things I couldn’t eat. It was hard to fully comprehend the effects of my diet when I was really young. I had times where I would eat half a bagel or a slice of cake, but found out that higher protein things weren’t really worth the phe when there were alternatives. My parents were always the best chefs and my heroes.

What was hard about dealing with PKU?

Going out to eat and going to parties growing up was sometimes difficult. My parents always made sure there were options for me or I would bring something I can eat. I didn’t want the attention when I was younger though. I would always bring my own lunch to school. I always drank my formula at home or at school. I wish I had brought it to lunch. I drink my formula every day at work now and love it. Back when I was younger, I thought it smelt funny and did not want to be seen with it.

What helped you stick to your diet?

My family was always there to support me and always made sure there were food options for me at home and when going out to eat. They like to eat a lot of vegetables so at dinner there were always low protein things we all ate and then something that was special for me. Growing up, I liked having my special food that was just for me. I knew that it was good for me and I like to feel good and be healthy and that was usually enough to make me try my hardest every day. Knowing the alternative of not treating PKU and how much harder things could have been if I were born before treatment options always inspired me to try my best. I’ve been given the best treatment and the opportunity and power to do my best and I never want to take that for granted. I am very thankful and feel blessed for all the things that I have and the things I can do.

What role did your family play in your PKU?

My family played the most important role in the outcome of PKU on my life. Their hard work and effort from the day I was diagnoses with PKU paid off as I turned out to be just fine. I don’t know what I would have done without them. I’m very thankful to have parents that care so much and did everything they could to provide me with the best. My family always was there for me in every way growing up with PKU. They are still always there for me. While growing up they cooked, ordered my formula and low protein food, dealt with insurance issues, took me to every clinic appointment, helped me with blood tests, and advocated for me and my diet in every way that they could as parents. They’re the best low protein chefs I know. I never felt left out at any meal at home. They constantly looked for creative and new recipes and things for me to try. They are my number one supporters and fans of me and my successes.

What did you like to do growing up? How did that work with your PKU?

I tried a lot of sports and activities during my school years, PKU never stopped me from trying things. I played soccer, did swim team, gymnastics, rowed crew, did dance, girl scouts, and took art classes. Art has always been my favorite outlet especially painting and taking pictures. With playing sports, I always made sure to drink my formula and get good sleep on my more active days. I’ve always been active at school and outside of school. Now I like to do workout classes and do 5k races once in a while. My next 5k is the MAPKUF Inc. 5k for PKU in Maryland in May. I am very excited to run with my family and friends and to meet other PKUers and their families.

What do you do when you go out to restaurants or eat with friends or in groups?

I always try to plan ahead. Looking at menus ahead of time to see the options helps. Many restaurants are great at accommodating, but you have to speak up. Leaving nuts and cheese off, replacing meat with veggie options, putting salad dressing on the side, there are many ways to cut the phe when eating out. Ordering sides of veggies, a side salad, or a side of fries has always been my fallback and I usually prefer them.

Sometimes before going out, if I know there might not be as many options, I will eat a little something before. I carry my formula everywhere I go and even keep some in my car just in case.

You are currently on Kuvan, how did you come to the decision to try it?

After going to the NPKUA 2014 conference in Salt Lake City, Utah last summer I had the opportunity to network with other PKUers that had tried Kuvan. I heard many positive experiences and more of the research of it while at the conference. I felt that it was a good point in my life to give it a try so I began my trial. What an exciting, life changing experience it was to find out that I was a responder.

Has Kuvan changed your diet? If so, how?

It has changed my life in allowing me to become more flexible with my protein intake. I’m still in the process of finding out its effects and more of what my phe and protein tolerance actually is. It’s been an adjustment, sometimes it even feels odd knowing I can eat some more, but overall it’s been a great one.

What doing in your life currently?

I’m an elementary special education teacher. I’m finishing my second year of teaching soon. I really enjoy teaching my students and helping others. I find my work always interesting, challenging, and rewarding. I’m very happy with my school and career paths.
What are some of your greatest accomplishments?

Graduating with my Bachelor’s degree in Psychology, Cum Laude and my Master’s degree in Special Education, Summa Cum Laude and becoming a special education teacher have been my greatest accomplishments so far.
You just returned from a trip to England. How was it? How do you navigate your diet while traveling?

It was an amazing trip! There’s so much history, neat culture, museums, good shopping and good food in London. I went to Stonehenge and Bath as well, beautiful places. I definitely recommend seeing those sights if you get the chance. While traveling, I always make sure to have a few BetterMilk formula packets in my purse and carry my Kuvan and doctor notes. I also make sure I have a low protein snack or two, just in case I get hungry and am worried about food options. What’s great about formula is that it is very filling and gives you energy. I recommend having it with breakfast at hotels so you can get ice. It’s a great feeling to start the day with cold BetterMilk, but having it on the go works well too. I like to mix it in plastic water bottles to drink on the go and then I don’t have to carry bottle mixers. I was able to find a low protein option in every restaurant I went to. English chips and salads were my favorites.

What would you say to other children, teens or adults living with PKU?

Remember you have formula and food options! Stay on diet and never stop trying. Don’t be afraid to try another low protein food, recipe, or formula. Keep asking questions. PKU is just a part of who you are. It’s up to you how you choose to see it. There are many more options for us nowadays. Reach out to others with PKU, stay in contact with your dietician and doctor. There may be some harder days, but you’ll learn, give it your best shot, and become stronger and smarter. Be proud of your efforts and what you’ve accomplished. Be your own PKU rock star.

What would you say to new parents of a baby diagnosed with PKU?

You will be great parents and your baby will be just fine with your love and support. Formula, diet, and support will be what your baby needs. Reach out to other PKUers, PKU parents, dieticians, and doctors for ideas and support. Whether it be through your clinic, Facebook, email, etc., reaching out to others can give a great sense of connection. You are not alone. There are so many options with low protein food, formula, and treatments like Kuvan nowadays and there will only be more and better quality ones over time.

Anything else you would like to add?

Be an advocate for yourself and PKU. Never stop learning and trying new things (in life and PKU related). Diet for life is key! Make choices that make you happy and healthy.

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March 19, 2015

Stuffed Peppers again

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I thought I’d try making these again. I used zucchini this time. Also, I asked on facebook what people like to put in their stuffed peppers, and it occurred to me that the sauce is maybe what makes the difference. I decided to try an Old El Paso sauce. I chose Roasted Tomato and really like the flavor. The only thing was it was spread pretty thin between all the rice and my husband’s beef. So next time I’ll use two sauce packets, and that way it will be more flavorful and moist.

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This recipe is for two people. For my husband I used beef and I also mixed some brown rice in with it. He got the zucchini and corn in his as well.

Recipe

6 Bell Peppers (I like yellow and orange)

1 ½ cups white rice or low-protein rice, cooked

2 zucchini, diced

2/3 cup frozen corn

1 package of Old El Paso roasted tomato sauce

1 T vegetable oil

For the protein version

1 lb ground beef, browned

½ cup grated Monterey Jack cheese

Cook the rice and brown the beef. Preheat the oven to 375. Cut up the zuchinni, add to the rice. Add corn, oil and stir in the sauce. Grease a pan big enough for 12 pepper halves. Wash peppers and cut through the top to the bottom, so they lie down on their sides. Fill peppers with rice mixture and place on greased pan. (this is where I would add more sauce on top). Cook in the oven for 30 minutes. Sprinkle cheese onto protein versions.

The process

I honestly don’t know how to dice zucchini…so this is what I did. Halved it, sliced the halves, then cut them into smaller pieces.

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Here is all of them before I put the cheese on my husband’s

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January 14, 2015

“Ziti” Bake

Hi everyone! It’s been a while. I guess I got pretty busy with the holidays and traveling.

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Low-protein “ziti” bake (I didn’t have ziti)

This meal was actually inspired by a comment on my page about making ziti bake for protein-eating family with a low-protein version on the side. Sounded good!

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Protein “ziti” bake (using shells)

The protein version I made was inspired by this recipe: Taste of Home Easy Ziti Bake, except I did shells instead of ziti and ground turkey instead of beef. Oh and I just realized I completely forgot to use eggs. Oh well. My husband and daughter liked it.

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Dinner! Nothing better than eating together.

My version went as follows and tasted really good! Nothing like warm, soft pasta in a yummy sauce.

You will need an 8 x 8 inch baking dish. The recipe says to layer the meat sauce and pasta/cheese mixture alternately, but I couldn’t figure out how to do that with the zucchini and I was in a hurry, so I just mixed it all together.

Ingredients:

  • About 6 oz of low-protein pasta of your choice (I used fusili)
  • 1 Tb olive oil
  • 1 zucchini squash
  • Garlic salt
  • Spaghetti sauce of your choice
  • 2 Tb Alfredo sause

Recipe:

Preheat the oven to 350 degrees F. Cook pasta to al dente. Fry zucchini in olive oil until they are somewhat soft, and salt with garlic salt. Drain pasta, add zucchini and a generous amount of red sauce plus about 2 Tb of alfredo sauce (there isn’t much protein in that amount). Pour into baking dish. Top with a bit more red sauce. Cook in the oven for 10 to 15 minutes.

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Viola!

December 5, 2014

Link to Interview with Recombine

I was interviewed recently about life with PKU for this genetic testing website. They published it for National PKU Awareness day.

Me You and PKU – Recombine

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November 4, 2014

BBQ Pulled Jackfruit!

I have been excited to post about this dish ever since I learned about it from Chef Andy at my recent PKU Event. This recipe is so simple, and so delicious!

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Have you ever even heard of Jack Fruit? I hadn’t. But it turns out it is the perfect substitute for pulled pork. It’s appearance and texture when shredded make you feel like you’re biting into a warm satisfying BBQ sandwich.

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Can you believe that is fruit?

Look for canned Jack fruit in your local Asian Market, or possibly a health food market. My local Sprouts did not carry it, but an Asian Market down the freeway did.

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It is important to buy the Jack fruit that is in just water, and NOT sweetened. 

1 can of this makes about 2 sandwiches. You boil the jack fruit and let is simmer for an hour. Make sure to keep adding water though. The second time I made this I forgot and ended up with charbroiled jack fruit because all the water evaporated.

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Chef Andy’s recipe included a homemade BBQ sauce, but I am all about convenience, and BBQ sauce is actually pretty low in Phe (8 mg/ Tablespoon). Also, I thought it tasted really good on toasted bread with mayonnaise spread on the inside of each slice.

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Ingredients

1 can jack fruit in water

Up to 1 bottle of your favorite BBQ sauce

2 slices of low-protein bread or buns (or whatever bread you can tolerate)

Mayonnaise

Recipe

Empty can of jackfruit with water into a sauce pan. Make sure fruit is covered in water (add water if needed). Bring to a boil. Let simmer for 1 hour, until fork tender, adding water as needed.

Drain water. Pour fruit into a mixing bowl. Pour 3/4 cup BBQ (or to taste). Stir well, breaking up chunks of jack fruit.

Toast bread or warm rolls. Spread with mayonaise. Spread BBQ jack fruit onto bread and enjoy!

October 10, 2014

Protein Cooking

You may be thinking that was a typo. But no, I meant, “protein cooking,” not, “Low-protein cooking.” Because, for many of us, we are not living our PKU life in isolation, we are a part of a family! We often end up cooking protein and low-protein food side by side.

Rice-stuffed peppers next to Turkey-stuffed peppers

Rice-stuffed peppers next to Turkey-stuffed peppers

Growing up I am sure I took it for granted that my mom cooked two meals pretty much every night for dinner. There was the normal or protein dish, and then there was my dish. Then she was always good at serving a fruit and vegetable that we would all eat. Bless her for doing that for me and our family!

Now I have my own family and I have the challenge of cooking two meals a night. Because I am used to my diet, it’s not that resisting the protein foods is hard. It is actually that motivating myself to cook something I can’t eat is difficult at times. But I do my best. Also, I’m not that good at cooking meat! And I don’t have as big of a food repertoire, so I have to really try to get out of the box.

I should add, my husband is very understanding, never complains, and almost always likes what I cook! So I am lucky. :)

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Since protein-cooking has been a struggle for me I imagine it has been hard for others as well. So I would like to share some things that help me.

5 Ways to Navigate Protein-Cooking as a PKUer:

1. Find a recipe you are excited about

Sometimes you may have to make a sacrifice and just cook your family’s favorite meal, even if you can’t eat it. But most of the time I think it is a good idea to think of something that sounds good to you. For me, cooking takes a lot of mental energy. I have a handful of recipes that I’m really comfortable with. But when I go online and try to look for new ones, in order to motivate myself to plan, shop for and cook a new recipe, I have to be excited to eat it myself.

An example of this would be my recent discovery of Southwest Salad. My husband liked it in a restaurant so I thought I’d try to make it for him, but it sounded really good to me too.

2. Aim for health, but take it easy some days

I know using as much whole food as possible is ideal. But I need to be realistic for my circumstances and phase of life (a young family trying to get through grad-school with a 1 year old and a budget). Sometimes I have to use canned soups, and even the occasional freezer meal. I have found that most of the time it is pretty doable to plan for about three meals a week that are more fresh or harder to prepare, and the rest of the time I go for more convenience.

Examples: Grilled cheese and tomato soup; fish, tater tots and green beans

3. When it comes to meat, the Crock Pot is your friend

I hope to improve my meat-cooking skills, but in the mean time, the crock pot is a great way to almost guarantee it will turn out well. Casseroles are simple as well. Also, making use of prepared meats like meatballs and sausages is easier than trying to make the perfect fried chicken or grilled steak (…actually I’ve never even tried to make steak). As far as chicken goes, mine often turns our dry. But recently we discovered that chicken with the skin on stays more moist and my husband really likes it.

Examples: Crock pot chili (ground beef, beans, onions, tomatoes, spices),Casserole (pre-cooked ham or sausage, rice , cream of mushroom soup, onions, cheese), Crock pot sweet and sour meatballs (store-bought sauce, meatballs, green peppers, pineapple)

4. Branch Out with Protein Sources

Meat isn’t the only way to get protein (as we all well know!) Try using legumes, chick peas, quinoa, nuts, peanut butter, tofu? (haven’t done that myself) to mix things up. Add on cheese (to almost anything if you’re my husband); add bacon bits to soups and salads. Canned meats such as tuna, salmon and even chicken can be really helpful.

Also, sometimes instead of doing two main dishes, my husband and I share a main dish but I throw in a protein side for him such as edamame if we are having Asian food like this oriental salad or chow-mein dish.

5. Master the art of the low-protein version

My mom was good at this. I have shared a few examples of this on my blog. When the family had casserole she would make “goop” for me. When they had burritos I got low-protein mexi-rice with all the veggies. The idea is to take the flavor of whatever you are making for the family, and apply it to a food that is lower in protein such as low-protein pasta.

Examples:

Beef stroganoff | lo-pro stroganoff

Taco Salad with black beans and cheese | lo-pro taco salad

Pasta or Quinoa salad | Lo-pro Pasta salad 

Southwest salad with black beans and cheese | lo-pro southwest salad

Stuffed peppers with beef | stuffed peppers with rice or low-pro rice

So if you are a PKUer cooking for a family of protein-eaters, I hope this has inspired you or given you some good ideas.

What do you like to cook for your family?

September 22, 2014

Creative Cooking for PKU Families Event

This Saturday I went to an awesome event hosted by my PKU clinic at Phoenix Children’s Hospital, sponsored by BioMarin Pharmeceutical Inc.

It was held in a cooking studio called AndyFood. There were a couple presentations followed by a cooking workshop by Chef Andy Broder. I learned some interesting things, got inspired about cooking and met a bunch of awesome people!

IMG_3125Me and baby! I could not have made it through this event without the help of various wonderful dietitians that held and distracted an over-tired baby girl while I mingled and cooked.

Presentations

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One of my favorite parts was hearing from Christine Brown, the Executive Director of the National PKU Alliance. She got us up to date on the latest goings on in research, education, support and advocacy. So much good stuff! One piece of news I found particularly interesting is that soon they will be launching a website specifically geared toward adults. Topics featured will include weight management, bone health, maternal PKU etc. Here is the statement on NPKUA’s webpage:

Plans are underway to create an adult focused website that will serve as the virtual home of the program and a community base for adults with PKU. Conceptually developed by adults with PKU, and specifically geared to the education and social support of PKU Adults, the website will be the only one of its kind. The hope is that this platform will also serve as an educational center for all aspects of adult PKU with educational webinars, short instructional videos, and social networking capabilities.

Cooking workshop

Andy demonstrated how to make several low-protein dishes and then let us jump in and help in the preparation.

Andy and his Sous-Chef

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The most inspiring parts for me were learning of two new ideas for meat subsitutes:

Jicama

He used this in ministrone soup as well in a stroganoff — shake n’ bake style.

Jack Fruit

This stuff sort of shreds like pulled pork! So it tasted great in his BBQ pulled jackfruit sliders recipe. I hope to try these myself so look for upcoming blog posts!

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Pictures

Potatoes au gratin with sweet potatoes, low-protein rice, and vegan cheese

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Everyone helping out

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Grant (resident young PKU super-star) making biscuits!

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An awesome couple I met who has an 8-month old baby with PKU. They were smart and got a baby-sitter. :) They are gonna rock low-protein cooking for their son!

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Minestrone soup with jicama, other veggies and low-protein rice

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Elena loved everything! And she does not have PKU, so that means it was really good :)
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August 20, 2014

Southwest Salad

This is another restaurant inspiration. I’ll admit, when we were first married, my cooking repertoire was sort of small. When I was single I was ok repeating spaghetti, pasta, tomato soup, etc. But Now that I have a family I like to create variety. I have gradually been collecting more and more recipes that are simple and reasonably healthy. There are plenty of recipes out there of course, but it has taken me time to find the ones that work well for me.

Recently my husband had southwest salad at a buffet we went to, and loved it. So I went home to look up recipes. I soon realized this is one of those recipes that could be really complicated, but doesn’t need to be. Also, it was one that can be easily adapted for my diet, so the work for one meal is making two. That is always nice when you are cooking for both PKU and normal eaters!

So here is my southwest salad recipe:

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Iceberg lettuce

Onion (could be green, red or white…I had white on hand this time)

Corn (canned or frozen, thawed)

Tomatoes, diced

Avocado, cubed

Cilantro

Tortilla strips

Online I found lots of recipes for different dressings, but I preferred to just go with the quick and easy way of simply mixing BBQ sauce and ranch. It tastes delicious! I would guess I did about 2 parts ranch, 1 part BBQ sauce. (you know me, I’m an estimator)

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For your information, for the PROTEIN VERSION I add black beans, possibly canned chicken, and shredded cheese. That’s it! Two versions of one easy, tasty meal.

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August 12, 2014

Homemade Raspberry Jam

This is the first time I’ve done anything like this, so I am excited about it! This was freezer jam so it was really easy. Turned out delicious! And there’s nothing better than knowing you made it yourself!

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To make raspberry freezer jam, I simply followed the recipe that came with the pectin packet

I bought 4 lbs of raspberries in bulk. I actually mashed them up and put them in the freezer for a couple weeks until I had all the tools and time to make the jam.

To make freezer jam you need:

  • Fruit
  • Pectin (found in the jell-o section)
  • Caning jars
  • Sugar
  • Sauce pan, bowls, spoon/spatulas
  • A funnel would be helpful

There are recipes for jam online, but I simply followed the instructions and recipe that came in the pectin packet. For anyone interested I will walk you through my process.

First I had to thaw the raspberries. I put them in the fridge the day before making the jam. Then I just had to stir them a bit once I put them in this bowl.

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Washed the jars and lids

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The recipe says you can strain half of the berries to get out some seeds. I strained less than half because I didn’t want to run out of raspberries. You have to use the exact amount the recipe calls for. Little PKU shout out– I used my formula can to balance the strainer! :)

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Measured out the sugar and stirred it into the raspberries.

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Next I had to boil the pectin according to the instructions, and stir that into the raspberry/sugar mixture. I didn’t have a funnel so I used a measuring cup with a pour spout to pour the jam into the jars.

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I had to let the jam set for 24 hours. Then I put one can in the fridge and the other three in the freezer.

That’s it! No fancy equipment required.

Oh, and this sweet girl woke up in the middle of the process. She was such a good girl and had some lunch in her high chair while I finished the final step of pouring the jam into the jars.

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My beautiful baby girl is over 1 year old! She likes bananas with the peel on

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