Remember when I said that I was going to try and blog more?   I lied.  I have not tried.

Apparently I have a tendency to bite off more than I can chew.  Who knew that raising littles, one with medical needs, finishing grad school round two, starting the kids in school, studying for boards & starting a new job while trying not to let your life completely fall apart at the seams (and feeding your family EVERY DAY) is a lot? Well, we survived and came out the other side. All of that is done now.  (Except the having to keep feeding my children.  That never stops or so I’m told).  I may have time to write now, and I like that idea.  So maybe, just maybe, I’ll do more updates and tell some of the fun stories I have been filing in my brain.

At least this time, I can say that “No news is good news.”

I’ll start with an update on the Beanster, since that’s who most of you care about anyway.  A close friend of mine asked me a few weeks ago, “She is really living her best life right now, isn’t she?”  I responded, “Yes!”  To start, she:

1. Hasn’t been in the hospital since May, and it was for a planned surgery.

Her surgery was for a g-button, a surgically placed feeding tube.  It has allowed us to increase the amount of formula she takes every day as well as “drain” her stomach contents when she is ill and cannot tolerate food. (Read:  Stop her from puking and make her belly feel better and less distended).  It’s consensus that having this tube is one of the main reasons we have been able to avoid admissions.  (Now please go knock on everything wood, pray if you are the type, light a candle and do a happy dance, hoping I didn’t jinx us).

2.  Went swimming, a lot, this summer.

This is one of those things we were told that Bean would not be able to do.  She has to keep her Broviac (central line IV in her chest) dry at all times, so submerging in water is tricky.  Well, thanks to adaptive medical equipment and some cleverness on my (and other TPN kiddo mamas who share ideas on the interwebs), sister spent time in the pool and LOVED IT!

3. Started FULL TIME PRESCHOOL with her brother and academic peers.

This is a big one.  Finding a school that we could get into without an act of God, could accommodate her medical needs, that her brother could also attend and didn’t want to place her in a special education program (that she doesn’t need) was a challenge.  Honestly, we kind of cheated and I enrolled her first and then told the school about her needs, but it all came together.  Bean & Buster are in the same class and we are all very happy.  They love school and are learning so much.  Their teachers have been wonderful and the school (with some strongly worded “suggestions” on my part) has been willing and able to do what needs to be done to keep her safe and take care of her needs while she is there.  The school nurse is one of her biggest cheerleaders. The classroom teachers have stated they  “sometimes forget” she has had such a complex medical history, and she is quickly willing over the hearts of school administration. After a week of school, one of her teachers pulled me aside to express how wonderfully she was doing. She

seemed almost surprised.  I replied “I was never worried about her.  She is going to love school.  I was worried about you all.”

I will say that the process of setting up medical accommodations for your child at school is a TRIP.  Our experience navigating this is probably its own post.  It’s November and we still don’t have everything tied up with a bow.  Maybe when it’s done, I’ll indulge you, but I make no promises given my track record.

4. Started dance class.

She would tell you this should have been first on the list.  The idea of putting on a leotard and dance shoes (tap, then ballet) every Wednesday is the highlight of her week.  Thanks to some awesome recommendations by friends, I found an amazing, low-key studio that is perfect for her and an instructor that is committed to developing a comfortable, nurturing environment.  Seeing her do something “extra” that doesn’t involve a hospital, and having the time and ability to do it, makes my mama heart so happy.  Now, getting food on the table, her to dance and both kids in bed on time every Wednesday is a challenge, but it’s worth it.  (To add to the chaos, Buster is also in Soccer on another night.  We only do “all in” at this house).

So, yeah. Overall, things have been crazy, but oh so good in so many ways.

I also have a handful of really good stories.  Maybe I’ll tell them.

 

 

I’ve mentioned previously that food for our family can be difficult and I often get asked if Bean can eat. The answer to that is, yes, er…most of the time. It depends. I mean… it varies, and she has a pretty…complicated diet.

Beanie is dependent on total parental nutrition (TPN), meaning that she gets nutrition in an intravenous (IV) form. She is hooked up to an infusion through her Broviac line between 12-18 hours/day and that infusion contains carbohydrates, protein, vitamins and some of the time, fats. When she is doing well, her TPN calories are minimal and she eats/drinks a decent amount. Other times, she gets 100% of her calories in her TPN and eats nothing or very minimal. Every time she gets sick and is in the hospital for awhile, she always seems to end up back on full TPN and doesn’t eat much (if anything) for the first week or so home. If she is doing well and hasn’t been ill, she is likely getting less calories in her TPN. Also, even when she is doing well, she’ll occasionally have a random day or two where her belly is more irritable than usual and we won’t let her eat anything to give her gut some rest. (See, what I mean with that super straightforward answer?)

Her second source of nutrition is from specialty prescription formula called Pediasure Peptide. It is a complete liquid nutrition source (kind of like Boost that you can buy in the grocery store but fancier). It is partially broken down so her weak intestines can absorb it more easily. We call this her “milk.” It’s pretty calorie-dense and she seems to tolerate it well. However, some days she drinks it super well and other days she won’t touch it. (Aren’t toddler/preschooler opinions awesome?) There may come a day when we consider having a feeding tube placed in her stomach so she can take in more of this in hopes we can reduce the calories in her TPN and possibly have periods of time when she doesn’t need TPN at all.

Her third source of calories are FOOD and sister loves to eat. Food with Beanie has been rocky from the start. Her first hospital admission was at 7 months (when she was officially diagnosed with MMIHS), shortly after we introduced solid foods. Since then we have identified a long list of “trigger” foods that exacerbate her condition one way or another. This has been done with some medical & dietician guidance and some through rocky trial and error.

What can’t she eat?

Things like raw vegetables are a hard no-go. They are difficult to break down and tend to “clog” her up. Many cooked vegetables still cause problems, but we have found a few that are OK. With a food processor and an instant pot, I’ve been able to work in things like carrots, zucchini & squash as well as spinach and peppers as long as it is pureed and cooked well. She primarily eats foods that are low-residue, so things without skin and seeds (again, they clog her up). She does OK with some fruits in small amounts with small seeds, like strawberries and kiwi.

The next no-goes are a doozy.

Elli follows a modified Low FODMAP diet. You probably just thought “What the heck is that?” Well, it’s ridiculous, that’s what it is, but it seems to help her and us parents will do almost anything for our kids, so I’ve let this diet take all of us down a completely trying and many-tears-worthy road of trying to find foods that Beanie can eat. FODMAP is an acronym for all things good. Not really, but some days it seems like it. In reality,FODMAPS are carbohydrates that are poorly absorbed in the small intestine. They are found in loads of common foods such as wheat, barley, rye, apples, pears, mango, onion, garlic, honey & beans. They contribute to gas production. Since bloating/distention is a huge issue for Beanie (Yes, I know it’s ironic that I call her Bean and she CANT EAT BEANS), we considerably limit the amount of FODMAPS in her diet. If you want to learn more about the diet in detail and would ever want to try your hand at Low FODMAP cooking, check out this blog. (Yes, I ripped the acronym illustration off from the author). Here is a good one-page reference that goes into the science behind the diet a bit. The diet is primarily designed for patients with IBS, but the reasons why it works for those are also many of the same reasons why it seems to limit some of her symptoms that spur from her poor gut motility.

Through trial and error, we have learned that some FODMAPS irritate her more than others, so we don’t follow the diet to a strict rule, but the basics for feeding Beanie include avoid:

• Wheat (Anything with regular flour in it is on the “no” list).
• Onion (These really seem to set her off)
• Beans/Legumes/Lentils (Hello…they make you gassy. Of course they wreck havoc on her)
• Lactose (We do lactose-free dairy products and there are a couple things she does OK with in small portions).
• Soy
• High fructose corn syrup
• Garlic chunks (She does OK with a little bit of garlic powder, but no garlic-press for me anymore!)
• Apples, pears, peaches (we are SLOWLY testing out some fruits)
• Peas (including pea protein and pea flour)
• Broccoli & cauliflower (back to things that make you toot).
• Inulin (this is an additive in a lot of processed “healthier” foods…especially gluten free foods, which is a major bummer because we are avoiding wheat, so I shop a lot in the GF aisle).
• Coconut (not a big deal until you realize that coconut flour is often used in place of wheat flour)

Yep. That’s a stupid list. The first time I met with a dietician about this diet I cried legit tears. For a while I felt like there was NOTHING she could eat. Some days, I still feel like that. We have attended multiple gatherings (Family events, holiday/birthday parties, Church dinners) were literally the only things she could eat where tortilla chips and rice…and sometimes the rice is tainted. I always try to pack something she can have so she doesn’t sit and watch people eat around her. The few times I forgot have really made me feel like an awful parent as I tell Bean that she can’t eat because everything we have will make her tummy upset. This usually results in us both going and crying somewhere there is no food around.

So, what DOES she eat?

• Protein is her friend (All FODMAPS are carbs). We do a lot of eggs, chicken, turkey, fish, beef. Skip the breading (unless you’ve done your homework), garlic & onion and learn about different spices to use.
• Rice-based carb stuff. We buy a lot of “gluten free” pasta, bread, cereal, etc. However…read the stupid label because there is a 50/50 shot inulin or pea flour/coconut flour is hiding in there
• Corn-meal based carbs (cornbread—as long as there is no wheat flour, grits, corn tortillas).
• Potatoes (but fried food makes her yack, so bake the fries, please).
• Lactose-free dairy products. (Fun fact—cheddar is lactose free. Cheese can cause it’s own problem with her, so we don’t let her chow down on cheese, but some cheddar in a soup or casserole is OK).
• Some fruits in moderation such as bananas, cantaloupe, kiwi, peeled grapes (torture!), mandarine oranges.
• Cheerios (and a handful of other wheat-free toddler staple foods).  I found a substitute for goldfish crackers recently and felt like I won the lottery.

I’ve became a master label-reader. In this post, I’ve only covered about the top 25% of the whole FODMAP containing food list, so in no way is this inclusive. One of the tricky things is learning which brands of foods are OK.  Pantry “staple” foods such as ketchup, chicken broth, tomato sauce, peanut butter & jelly all vary A LOT as far as ingredients go.  So, what is OK in one brand may not be in another.  If you ever decide you want to cook for Bean and the recipe says to use the Progresso low-sodium Chicken Stock in the soup, that means something, because the regular Progresso Chicken Stock or Progresso Chicken Broth both have onion in them.

Also, portion-size matters. There are a lot of foods that have small amounts of FODMAPS in them, so we can do up to a certain amount. Yes, I have an app for this. It was $8 and worth every penny, and I hate paying for apps.

Oh yeah, we also try to increase, but not overdo, her oral intake of essential fatty acids when she is not getting fats in her TPN. The fat in her TPN, or “Lipids,” come with the highest risk of complications, so we try to avoid using them as much as we can. So, we try to get her to eat the needed amount of fats every day. Pro tip–put margarine on everything of hers that you can. Yes, margarine, not butter, because, again…that matters. (For the food geeks reading this–margarine has a greater concentration of essential fatty acids than butter does…more bang for your buck).

It’s a lot and it took me MONTHS to figure out a lot of the kinks.  There have been weeks where the only thing she has ate were scrambled eggs & cheerios (with lactose free milk in both).  However, it has gotten easier.  I’ve found “safe” cupcakes for birthday parties, modified some of our standard rotating meals to work and joined a couple of super helpful Facebook groups that are moderated by dietitians who can answer questions about recipes and portion sizes.  Finding foods for her to eat have been worth it and I would do it all again.  Sister loves food and she gets it from her mama.  Now, hopefully, you’ll understand a bit more why a dinner or party invitation stresses me out a little more than the average mama bear.

Is your head spinning yet?  Can we come over to your house for dinner tomorrow night? I’m tired of cooking. I can give you the recipe with brand-specific shopping list. 😉

A friend recently apologized to me because she hadn’t read my blog in a few months.  I laughed and told her she was off the hook because I haven’t posted anything since September.  So, in the name of getting one more post in before 2019, here’s an update. Hopefully I can post few good stories about more of our misadventures in life in the coming weeks.

While I would LOVE to say that “no news is good news” as far as the blog goes and that our lives have been so exceptionally boring that I have had nothing to write about. Unfortunately, that hasn’t been the case.  Fall and winter have so far been pretty rough and the lack of posting has more to do with my inability to hold my eyes open for any additional minutes at the end of the night and/or trouble putting together coherent thoughts if I could keep my eyes open.  Part of the business is self-inflicted and more is rotten luck.

As far as self-inflected struggles go, I (much to my better judgment) have found myself back in graduate school, working on a post-master’s certificate to become an Acute Care Pediatric Nurse Practitioner.  So on top of our already busy lives, with my husbands (reluctant?) support, I added to the chaos.  Even though I usually fall into the “good student” category, the adjustment hasn’t been easy on us.  It’s created a bit of financil strain, the parent-load responsibilities haven’t lightened up and the hubs is also super busy with work.  Hopefully when I finish the program in August, I’ll have more opportunities for a career that can allow me to continue to be there for all of Bean’s necessary “stuff” like clinic and therapy appointments and still work in a field that I love (with a more attractive paycheck).  Until then, please send wine, caffeine and laundry services my way.

Some people seem surprised that having a chronically-ill child hasn’t shied me away from taking care of sick kids.  On the contrary, I feel more called to it than I ever have.  My family’s experiences have shown me in ways I wish I never fully understood how crucial it is to have people who are passionate about what they do taking care of children and their families during their most difficult days.  I pray I can make the difference in my patients’ lives that some people have in ours.  I’ve always loved what I did and where I work.  Consider me doubled down and in for the long haul.  Hopefully one day I’ll give academia a rest, though.

For the rotten luck part, fall and winter has not been kind to Bean.  She had rhino/enterovirus (AKA the common cold), RSV and a viral gastroenteritis a 4-week span, all requiring hospitalization.  The rhino/entero and RSV weren’t bad admissions and she was home within 48 hours both times.  However, with her (hopefully) finale virus, nothing was held back.  We were visiting my parents 5 hours away the weekend before Thanksgiving and after being at their house for about 12 hours, it became apparent that I needed to take her to the ER.  Twelve hours after that, she was admitted to the Peds ICU in hypovolemic shock at a hospital away from home. Seventeen days later, I finally signed discharge papers and we made the drive home across the state.  She spent Thanksgiving and her (and Buster’s) 3^rd Birthday in the hospital and her momma cried a lot of tears.  She was pretty miserable for most of the hospital stay and being away from our “home” hospital brought a lot of struggles.  Trusting “strangers” in the care of my child was not something that came easy to me.  I realized how comfortable I have become with her care team when I was so very uncomfortable with a new one.  So, if you are one of those people who take care of my daughter in the hospital or outpatient world, take this as me saying THANK YOU for loving my girl the way you do, learning about her and her disease, how to take care of her, and know our family appreciates you so much.

[Also, consider this another perfect exacmple of why “normal” kid illnesses send our family running for the hills.  Keep your sick selves away from our children and while I’m at it– get a flu shot.]

Another noteworthy thing to mention is that Ronald McDonald House Charities are amazing. We were lucky enough to stay there for the last 15 days of her hospital stay.  We were housed, fed, showered and as rested as we could be while there.  Our family was allowed to be together for most of Bean’s admission with the exception of a few days when Daddy had to go home for important meetings at work.  Buster had a place to play and be a “kid” away from the hospital. Without these things, a very rough situation would have been so much worse.  I’m planning on organizing a meal or two this spring for our local RMD House to give back.  If any friends and family want to help out, just let me know.

Bean is still not completely back to her “normal” since coming home from her long hospital stay, but she has made great progress. She is back to being her silly and stubborn self most of the time. We were planning on traveling to see my parents again for Christmas. After an almost panic attack while preparing and packing to go, I made a last-minute cancellation of plans and then, Christmas was AWESOME (besides a broken washing machine, but hey, we don’t do easy around here).

We went to Church on Christmas Eve, because taking two three-year-olds to an evening candlelight service is a perfectly sane way to spend a night.  (No, actually—it’s not, but I couldn’t have been more grateful to be there).  Then we had dinner and unwrapped presents with “Mudders” and Papa with a visit to see Great Grandpa Pop.  We were very thankful that Gigi, Grandpa and uncles were able to travel to us instead of us coming to them. So, on Christmas Day, the kids got presents x2 and ZERO illness or trips to the ER. We spent the entirety of Christmas Day in our pajamas and the kids did their three-year-old due diligence and destroyed the house at least two times over.  Our family has had a considerable amount of holiday and other big days disrupted with unexpected and prolonged hospitalizations, including past Christmases and birthdays.  A mostly uneventful, dare I say “normal” one, celebrating the birth of our Savior and enjoying our family was exactly what our tired souls needed.

Happy 2019, folks.  I hope this is the year I learn to exhale.

One of the worst things about having a kid with a rare disease is how lonely it can be. Knowing no one who can relate or understands what you are going through is an isolating experience. For almost the first two years of Bean’s life, this was the situation or family was in. Up until very recently, she has been the only child seen at her hospital with the disease. We knew there were other families out there, but didn’t know how to find them or where they might be.

A little over six months ago, I randomly visited the MMIHS Foundation web page. I had looked at it several months prior, but the foundation was VERY new at the time, and there wasn’t much posted. (The organization is younger than Bean!) This time when I visited the page, a link was posted that wasn’t there before inviting families to join a support group! I was immediately hit with excitement! There are other families out there! I can talk to them! Maybe I can meet another mom who knows what this road is like! Could we hang out in person? Could Beanie meet other kids like her and see that she isn’t alone?

The excitement was then taken over by panic. What if I don’t like what I learn? What if the road leads to a place I don’t want to go? What if this “support” group isn’t very supportive? Is connecting with other families a rabbit hole I really want to go down when I have no idea what is in that hole? Is there light at the end of that hole or is it full of darkness? I hesitated. I didn’t click the link. I cried. I prayed. At the time we were still very quiet about Bean’s disease and I knew that jumping down the “hole” would likely change that to some extent. Was I ready for this?

I thought about it for several days. I think a combination of the Lord knowing I couldn’t stay on the lonely path much longer and my own curiosity getting the best of me took over. I clicked the link and requested to join the support group.

I can only think of TWO times in relation to Beanie’s disease when I have actually been given immediate gratification in knowing we made the right call and I didn’t fret praying we had made the right decision. The first time was a couple hours after her (very dramatic) colon removal in the PICU (eternal thanks to a brave surgeon who wasn’t afraid to have a very difficult conversation with me because he knew it was the right thing to do). Shortly after surgery everything about her status started looking better and my “PICU nurse gut” from my years working there told me that she was going to make a recovery. For the first time in two weeks, I exhaled.

The second time I let out a long exhale was the first time I “entered” the online support group. I was given a warm welcome from SO MANY families telling me about their children. Kids were thriving, going to school and not letting their disease limit who they were. THERE WERE ADULTS WITH MMIHS in the group! We weren’t alone. The rabbit hole was where I needed to be, and there was light in there.

The foundation support group has been such a wonderful gift to my family and all of the families who enter. It’s a place where we share information about treatments, help people connect with physicians who know about MMIHS. We laugh and cry together.  I have connected with so many wonderful families.  I’ve been able to support others when they need it and had someone to lean on when I needed it.  I have met ANOTHER MMIHS MOM–IN PERSON–and gave her a hug! A year ago I felt so alone and now I know we are not.

The MMIHS Foundation is younger than Bean. It’s a small organization with big goals and even bigger dreams. We want to connect families, physicians and researchers today in hopes that tomorrow, the path is even brighter for the kids we love so much.

The mission on the page reads:

“MMIHS Foundation plans to take full advantage of the present by bettering the lives of those affected by MMIHS through the cultivation of a supportive and resourceful community which promotes advocacy, awareness, education, and research.”

Today is a big day in MMIHS land. It’s the first ever MMIHS day and we are telling the world that we are here, we aren’t afraid to fight and we are better together.

Many people have asked me what they can do to help. Today my answer is click the link below, watch the video, share it on social media and consider donating to this organization.  With your help, maybe those big dreams can become a reality, because Bean’s battle is just getting started.

http://www.mmihs.org/2018mmihsday/

I get a lot of questions about how Bean’s disease affects our day-to-day lives. For the most part, Tom & I work very hard to keep things as “normal” as possible for both kids. We try to do many of the things that other families with small kids enjoy. The kids’ favorite things to do are going to playgrounds, the Botanical Garden, the Zoo—typical toddler stuff. They love play dates and chasing other toddlers around and they destroy my house on the regular. Our lives are just as chaotic as one could imagine in a 2 bedroom, 1200 sq foot house with two, two-year olds can be. Yep, it’s loud.

There are many things that are “normal” or “no big deal” for most families that can be a very big deal for our family, though. I figured sharing more about some of these things could help our friends and family know why we sometimes act sketchy or hesitant when y’all are trying to be social with us.

Food

The biggest and most frustrating thing for me personally is how difficult food and eating in our house can be. At this point, Parental Nutrition (IV Carbohydrates, proteins, vitamins and often fat) makes up most of Bean’s nutrition. She has a central line called a Broviac in her chest and her infusion goes into it. Currently, her infusion goes over 18 hours and she also drinks a specialty formula that is partially broken down (this makes it easier for her finicky belly to handle). She does eat some very small amounts of “real” food as well. She has had periods of time when she has been able to eat more food and other times when she can’t eat or drink anything. This can also vary from day-to-day. When she can eat “real” food, her diet is quite limited. It is a likely that we could attend a dinner or go to a restaurant and there could literally be nothing available that she could eat. (One day I’ll post about her diet that has caused me great strife).

Another complicated layer is that Bean LOVES to eat. She treasures the bites of food she gets and can become very upset when she can’t eat what everyone else has. Our family rarely sits down together for meals anymore. Tom or I are often distracting Bean with something fun while the others eat and then we swap out. I miss our “family dinners” greatly. This also makes social gatherings difficult. So much in our lives revolves around food. Family events, birthday parties, church potlucks, holidays—all of these things are more difficult for our family. Please don’t be offended if we dodge a dinner invite with you. Food is just….hard for us.

Water

I love the water. I love swimming, boating and all activities that involve water. I love pools, rivers, lakes & oceans. I love water. Another major bummer is that Bean’s Broviac CANNOT get wet. If it did, it could pose a massive infection risk that could result in her becoming critically ill. So, at this point in time, you won’t see any family photos of us splashing in the ocean. I do have a few strategies that have allowed Bean to do some limited swimming, sit & build a sandcastle and play in a splash pad. Taking a bath even requires a considerable amount of attention and she gets a lot of “sponge baths.” Participating in these activities is always a calculated risk and we have to be vigilant to make sure we are protecting Bean’s line the entire time. The activities are short lived and if it looks like her Broviac is going to become compromised, it’s an immediate game over. Also, because Bean gets most of her fluids via IV, she doesn’t do super well in the heat because giving her “extra” fluid is a lot more complicated than tossing a bottle of water at her. So, bottom line, please don’t suggest spending a day at the Six Flags water park as a fun activity to do for our family. (There is a company that makes shorty dry suits in tiny person sizes that would keep her torso completely dry. So, maybe one day, we’ll be able to give Beanie that beach vacay where she can splash in the waves to her heart’s content).

Planning

We have experienced many ups and downs helping Bean battle this MMIHS monster. One of the most anxiety-provoking things about the fight is that things with her can change very quickly. I have put her to bed after an amazing day filled with friends and fun and taken her to the Emergency room the next morning.   We left grandma and grandpa’s house on Christmas morning and headed to the hospital. I have bailed on my coworkers more times than I would ever want to admit because Bean had a sudden change or event and I am suddenly needed at home or the hospital in “mom” capacity. So, when we get invited to events, I dread the RSVP. Please don’t think I’m being a slacker or ghosting on the invite. The truth is, I am always worried that something will happen with Bean and we won’t be able to make it. I also do more than my fair share of making plans with folks and then bailing at the last minute because things changed with Bean. (Consider this an apology to all of those who I have cancelled on recently). We try super hard to live life and plan to do fun things with and without our children, but any time I confirm a reservation or purchase tickets to something, my heart skips a beat.

Germs (Waring–This one contains my biggest soap boxes)

All parents know that sick kids are the worst. Everyone hates to have their kiddo deal with a nasty cold, a GI bug, the flu, etc. Plans get disrupted, parents have to call into work and no one gets a good night’s sleep. Sick kids suck. With Bean, being sick is multiplied to the 10^th degree. Her immune system isn’t compromised, so she isn’t more likely to get sick than any other kid, but if she does become ill, she is considerably more affected by it than other children. “Typical” kid illnesses often have a negative impact on her already poor gut function and her belly can’t hang. She has been hospitalized more than once for a week+ with illnesses that would have been a couple rough days for any other child. Illnesses also seem to set her back on any progress her gut has made and she is often unable to tolerate any amount of food in the weeks following an illness. This makes our family a bit germ-a-phobic. We always have hand sanitizer handy and will unabashedly avoid folks we know to be ill. Please don’t make a play date with us and then say “Oh and Jimmy has had a runny nose for a few days. I think it might be allergies. I hope that’s ok.” Nope, its not ok. The play date is cancelled, better safe than sorry. Even worse, please don’t make plans with our family and then show up with a member of your family visibly ill. It tends to make me curse, then cry, and always ends in an awkward interaction. We don’t do a lot of crowded/busy places during flu season and for the love of all that is good in this world, vaccinate your kids and get a flu shot if you plan on hanging around us. Beanie is depending on it. (Yup, shameless vaccine promotion is happening in the blog, folks. This mama has seen the ugliest versions of vaccine preventable diseases up close and personal and she ain’t messing around).

I pray that after reading that I didn’t scare you into not wanting to hang with our family. My hope is that this bit of insight makes it easier for folks to find ways to enjoy spending time with us. The people who have been accepting and understanding have truly been the difference makers in our lives. We love getting out and about and seeing those we love. Also, are kids are pretty dang awesome and why wouldn’t folks want to spend time with them?  Tom and I are OK as well. 😉

I’ve spent most of my nursing career working in an academic medical center. Full disclaimer—I love it. There is something about working for an institution that is committed to bringing up the next generation of healthcare providers, while conducting research to make care better in the future all while taking excellent care of children and their families that just makes my little nurse nerd heart swoon.

However, it’s not all rainbows and butterflies in academic medical center land. For those of you blissfully unaware of what July means in an academic hospital, Google “July effect” and you’ll see why nurses never want to work July 1. Essentially, it’s physician promotion day. Physicians completing training programs get bumped up a level on the food chain.   Folks that were 4^th year med students in June are intern physicians on July 1—and they are taking care of people everywhere. STAY HEALTHY IN JULY.

So, imagine all of the snarky jokes one could come up with when I tell you my daughter has spent not one, not two, but THREE CONSECUTIVE July 1s admitted to the hospital. This year, we made the conclusion that Bean has an official role in the education of new physicians and she takes it very seriously. Her mom doesn’t go easy on physicians (surprise!), so if you can take care of Beanie in July and live to tell about it, you’re probably going to be a pretty good doc. 😉

Jokes aside (and there are plenty), most of our encounters with new (very eager) physicians have been positive and Bean is so dang cute that she sasses her way into their hearts quickly. Her mom tries to be patient, remembering that everyone who is now amazing at what they do was once new, and we all come out OK. It makes for good fodder, though. It also provides a nice Segway to fill in some of the gaps of her story.

Bean’s first July 1 was the admission that led to her diagnosis. I honestly don’t remember a lot of details about that week beyond being terrified in the emergency room and an amazing physician friend (**shoutout to Jen**) who stayed with me until late in the night to make sure I was ok and that Bean was safely settled into her room. I did make an awesome onesie for Bean that week. The GI Fellow got a good laugh out of it and still enjoys telling the story. (Fellows are physicians who have completed a residency and are training to become a specialist). The admission ended with our family being transferred to the care to the Gastroenterology team. The GI physicians have became like sometimes adored, sometimes despised, slightly dysfunctional (yet brilliant), distant family members, who are permanent fixtures in our lives. Nearly the entire GI department gets a Christmas card from us every year—for good reason.

July 1 #2 was a big day–Bean was discharged from the hospital after a 38 day stay, including 3 weeks in the Pediatric Intensive Care Unit (PICU). She had an infection in her bloodstream that was the result of bacteria in her weak intestines “leaking” out. It resulted in her having her colon removed (she now has an ileostomy) and ever since then she has relied on Total Parental Nutrition (TPN), aka IV nutrition, for support.   She hasn’t been able to tolerate eating/drinking all of the food she needs to grow. She has a long-term IV catheter called a Broviac and she is hooked up to an infusion for 12-18 hours each day. (It varies based on a lot of different factors).

I’m not exaggerating in the slightest bit to say that her coming home that July 1 was a moment that may have never happened. Sister fought for her life. I don’t want to go into the details of her being in the PICU and I don’t know if I’ll ever be able to write about them. Please just trust me when I tell you that there are amazing folks there who devote their lives to making miracles happen. I worked in the PICU for the first eight years of my nursing career, and having my former colleagues save the life of my daughter has brought me a level of awe and humility that I didn’t know was possible.  So, I guess July 1 #2 is a happy one. Also, she hadnurse practitioner team taking care of her on that July 1, which saved at least one intern from having to deal with her mama.

July 1, #3: This month—I just plain don’t like July. Bean has been admitted three times in thirty days with exacerbations from her disease. Essentially, her intestines will decide that they aren’t going to do their job for a bit and she has to hang out in the hospital until they come around. In the last week she went for an endoscopy procedure and the GI physicians found a “kink” in her plumbing near her ileostomy. She went to the operating room to have it repaired two days later. We are hoping

that this plumbing problem is the reason her disease has been so exacerbated recently, but time will tell. Gut function in MMIHS seems to have a cyclic factor to it. A person can have months with minimal problems followed by months that are a constant problem without a lot of rhyme or reason. Also, if she becomes ill for any other reason (cold, flu, UTI, etc.), it really messes with her gut function. Overall, Bean took the surgery like a champ. She was in the hospital playroom THREE HOURS after surgery, cooking up dinner in the play kitchen like it was any other Friday night. She seriously amazes me. She’s currently still in the hospital, as we are waiting on her intestines to go back to work. Someone with a healthy gut would still need a few days to recover, so it’s not expected that she will take a few days longer.

This July has been a lesson in many things: Patience, grace, humility and humor are probably at the top of the list. Our family is exhausted beyond belief and you can see that Bean is over it. She isn’t sleeping well and simple things are starting to become tantrums for her. I can’t blame her and honestly, I’m a bit jealous—I could go for a tantrum myself. For some reason it’s frowned upon when women in their thirties roll around on the floor screaming.

I have to be grateful, though. This month I have learned how amazing some of the nurses on the GI floor are. They love on my daughter hard and take good care of her mama as well. The GI physicians have been patient with me, answered my questions and entertained my nuances. The surgery team handled me breaking down on them after a rough day and Bean’s surgeon can’t come into the room without giving me a hug. The (brand new!) interns have stepped up nicely into their new roles, listening and learning from Bean. Of course, she sassed her way into their hearts by July 2. Buster, always making life an adventure, thinks coming to the hospital is an exciting way to spend your day. He constantly solicits high fives from all medical staff and provides comic relief for all who encounter him.   Today one of the interns said that he made a rough day for her better. That’s my boy. He sure can bring a smile. My “hospital family,” friends and coworkers from over the years seem to be a steady trickle of visits to make the days go by faster and they bring caffeine to keep me awake and sugar to keep me quiet. As awful as this July has been, it’s also been a great reminder of how lucky we are to have so many people in Bean’s corner.

I also started a blog, and folks seem to be reading it.

**Newbie blog mistake.  I put two posts up at once.  I recommend scrolling down and reading my first post before starting this one.**

 

An ultrasound at 22 weeks into my pregnancy, gave cause for concern for baby “A” (Bean). This resulted in more ultrasounds than I could count for the next ten weeks, but never an official diagnosis–only that she had an enlarged bladder. We were told this could be caused by a number of things, ranging in severity, none of which could be determined until after she was born.   In the NICU she encountered challenges but seemed to work through them and was still discharged home within an appropriate range for her gestational age and with minimal pediatric specialist follow-up. We went home with the thinking that we had dodged a bullet.

When the twins were 7 months old, she was hospitalized for what was initially thought to be a bowel obstruction. Through a course of events, we ultimately learned that the worst-case scenario presented while I was pregnant was our reality. Bean has a genetic ACTG2 mutation that results in a disease called Megacystis Microcolon Hypointestional Peristalsis Syndrome, or MMIHS. It results in weak smooth muscle in a number of her organs. Essentially, she has a large and weak bladder, a small portion of her colon (that has since been removed, but that is a long story for another day) and the muscles in her small intestine do not work properly–things move very slowly and uncoordinated through them. The disease is EXTREMELY rare and there are less than 100 known children that have it. There is no real treatment outside of symptom management and there is no cure. You can visit www.mmihs.org to learn more.

She has been hospitalized over 20 times in the last two years, including a 38-day admission last summer with 3 weeks in the Pediatric Intensive Care Unit where we nearly lost her.  She has had over 6 operations. (I know I have a lot of gaps to fill in). She has weekly therapy and home health visits and sees multiple specialists. (She keeps her GI doc the busiest, though). Sometimes between my work and her, I feel like I live at the hospital. Our family has been through the ringer to put it lightly.

We’ve been fairly quiet on social media about her disease and our life surrounding managing it. So, you might be wondering why I’ve decided to be up-front about it now.

I don’t have a great answer and what I have is complex. It has taken Tom and I a long time to process how open we want to be on social media about our lives and Bean’s condition. Her disease is not super easy to explain and there is a lot of inaccurate/incomplete information out there. Truthfully, I don’t have the emotional endurance to answer a lot of questions most days and the idea of having Facebook posts with a long list of comments and questions makes me queasy. On top of that, I feel like one day this will be her story to tell, not mine. I don’t want her to be defined by her disease or known as “that sick kid.” She is my daughter through and through– One day she will have very precise opinions on how she wants her story told and who gets to hear it. (Consider this your disclaimer that if she ever comes to me and says to take this down—it’s gone).

Ultimately, we decided that the benefits of letting folks know more about her disease and our lives around it outweigh our reservations. We also know there are more people who would have helped us along the way had they known what we were going through. Rare diseases don’t get the attention so many other things do. There are no national organizations raising millions of dollars every year to find a cure for MMIHS. There aren’t many physicians that know a lot about MMIHS. Her doctors are learning about it along with us. Her disease comes with many more questions than it does answers. So, in the name of awareness and not letting rare diseases like Bean’s be overlooked, we’re telling our story.

A lot of people likely have questions. I am going to try to work through them by sharing more, but please give me some time. Remember that answering a bunch of questions has been one my biggest deterrents and I’m still struggling with how much to put “out there.” I’ll answer one right away, though.

What can I do to help?

Here are my top 3 answers:

• Please don’t think of our daughter as a disease, but as a little girl. She has a fierce approach to every day. She is two. She loves sheep, dinosaurs, owls and singing songs from her bed at night when she should be going to sleep. She doesn’t like to smile for pictures, but when she does it lights up the room. She lives for the details in life and never hesitates to assert her authority, particularly over her twin brother. Her life is harder than most, but she finds joy in every day and we are blessed to have her in ours.
• I always glad accept coffee that isn’t from a parent’s lounge and food that isn’t from a cafeteria when we set up camp in the hospital. [Bonus points are rewarded for plain lattes and real food with vegetables]. I’m writing this from her hospital room right now, feel free to ask where to send food.  The nurses also seem to have a sweet tooth around here.
• Remember that her name means God answers prayers. We won’t turn any of those away.

 

 

 

[Note: I’m not going to refer to my children by their actual names in this blog in hopes of preventing future embarrassment should they ever search themselves. So, here goes an awkward attempt at writing about their names without actually using them. 😉 Their nicknames have been “Buster” and “Bean” or “Beanie” for awhile, so that’s what we’re going with.]

We struggled to name our children. The permanent-ness of the decision weighed heavily on our minds. If you decide you hate your car seat or crib, you are a trip to Target or a two-day wait on the Amazon Fairy away from something new. If you decide your parenting style isn’t working for you, change it up. With a name, there is no going back (without confusion and a lot of legal fees). So, when the twins arrived at 32 weeks, they were born nameless and remained that way for well over 24 hours (much to the dismay of our NICU nurses).

Buster’s name had been on our “short list” for most of my pregnancy. It worked with “Thomas” (the pre-determined middle name due to a long line of Toms) and we couldn’t think of any nicknames would make us hate the decision later. It means “little warrior.” So, when he entered this world at a whopping 3lbs 7.5oz, flailing all 4 limbs and rolling himself over in his isolette, it seemed to be the perfect fit. Today, it still is. He meets each day ready for adventure.

I knew my daughter’s middle name would be Mary. It was my great-grandmother’s name. Her defining characteristics were a fierce devotion to her family, an insatiable work ethic and a stubborn streak that still sparks stories twenty years after her death. The woman had some serious intestinal fortitude. I adored her.

The Hebrew translation of Beanie’s name means, “My God has answered.” When she entered this world, we knew there was a possibility that her life would come with real challenges. She needed a strong name to stay with her through them. So, she was given a name that would be a constant reminder that God answers our prayers, and a prayer that she inherited the perseverance of her namesake. There is nothing that could suit her better.

Bean has faced more challenges in two years than most encounter in a lifetime.  She meets each day with a level of grit that makes her my biggest hero.  Hang around if you’re interested in adding her to your list of folks to admire.  You can laugh and cry along with me as I describe a different version of parenthood than what I had in mind.