Something Big.

Quoting one of my favorite movies, Wayne's World, "What I'd really like to do is something extraordinary. Something big. Something mega. Something copious. Something capacious. Something cajunda!"

I'm not sure if this is it, but it's something at least.

I created a facebook page for Martha. It's called "The Life Transparent."

You can follow this link and "like" it. You don't even have to be my friend in real life, or my friend on the internet (I have lot of  "friends from the internet"). So, no stress, no worries, no creepy hot-mess-mommy-stalker-blogger.

Maybe I'll blog here, or maybe I won't. I'm decisive like that, and not nearly this sarcastic in real life, which is a pity for my "friends on the internet."

Happy Thanksgiving, everyone! Our family will be attending Mass in the morning, cooking up some sweet candied sweet potatoes, and spending the rest of the day with our family.

Today I made a Bailey's Mousse pie, which I hope will set (it was pretty jiggly), and my famous culinary specialty, Ritz crackers filled with peanut butter and coated in almond bark. I'm practically the next Julia Child with these knockouts.

The wine is calling....

Love!

Accomplishments and Outings.

You know the creative juices just aren't flowing when your blog posts are only a recap, or a reminder of everything you have going on. Phew! Bear with me.

On Monday Martha had a Cranio-Cap appointment, which also included Cranio-Cap graduation. Hooray! I have a box of cupcakes to make to celebrate the accomplishment, but life has not allowed baking from a box yet this week. Graduating from the Cranio-Cap means so much to me. You see, it is the FIRST thing we started, and then finished with Martha. It had an end. The first and only thing we've done to this point that I can say that about. There are a few other things I pray that she can graduate from, but for now, the Cranio-Cap is a big accomplishment.

I'm very much looking forward to seeing her dark, curly hair grow.

Remember Paul's curls? Oh my goodness, swoon!

(This is Paul when he's about 16 months)













On Monday afternoon I brought Martha to the doctor. She has had a respiratory infection for about three weeks now, which we were managing at home, and is more or less typical even though it is very tiring. But there was something else "off" too. She was in serious pain, and I couldn't get her to sleep or to stop crying...for the whole day. She had had a low fever for days, but my mind was trying to think of what logically could be going on. The last time I couldn't get her to stop crying, she ended up in an ambulance, so I was worried. Anyway, she has her first ear infection, and is now being treated and will hopefully get the respiratory junk taken care of along with it.

On Tuesday I took Paul and Michael to Mass, which is like... a little bit of a disaster. I always need an outfit and new deodorant after daily Mass on my own with the kids. Wow, they keep me in a sweat while I'm trying to hush them and keep eyes stares at a minimum.

Wednesday (that's today), I had another great outing with the boys, except this time was the library story hour. No sweating involved here. The boys got out of the house for something other than Mass or the grocery store. Check for me.

Thursday is Early Intervention therapy. More on this later. The therapists come to the house. It's a circus in here.

Friday is MORE therapy, except this time we leave the house B&E and drive 40 minutes to a private therapy business. The purpose of this visit is to have Martha fitted for hand splints to de-rotate her hands. They will look something like this.

This will be the second orthosis she receives (the helmet being the first). The splint should help her play, and reach for objects and toys. Mark says it the best when he says that her arms were put on backwards ;-) .

This picture was just taken of her on Monday, and it gives an idea of her natural hand position. When she is reaching for something, they rotate worse. I hope these splints aren't too invasive and can train Martha's muscles to move more correctly.

On a pleasant note, Martha's rosy, high cheek bones remind me of my family of origin. I know she will look a little different because of her syndrome, but lately I have seen a resemblance in her face that reminds me that she is here with me...that I am part of her. It is a small sort of comfort.

I know I'm missing something...or a few things, but I'm wrapping it up for now while I contemplate a Diet Coke.

Giant...Gaping...Holes.

I think the biggest challenge in caring for a child with a rare syndrome is the lack of information available. In the age of technology, where you can learn anything about everything with a google search, having no search results becomes isolating.

In addition, the scope of a syndrome can be so great, that you feel like you must become an expert on everything in order to fully understand what is happening. This is where I am at with my daughter. I have a working knowledge of her gastric system, food pump, and swallowing disorder. I understand the way her brain differs from mine, and to a certain extent, how it will impact her development. Cardiology doesn't hold my natural interest, so I'm happy that all I need to know is that Martha will likely not need heart valve surgery as a child.

Beyond that, things get more complicated. After Martha was diagnosed last March, on the drive home I told Mark, "You know, I wished I would have just become a doctor." I had a fleeting thought about doing just that during college, thinking I had the drive to work hard enough to do it.

No, I realize, if I want the answers to the questions I'm seeking, I would have needed to be so much more than just a doctor. I would need to be a geneticist, and neurologist, a nutritionist, hepatologist, physical, occupational, and speech therapist, with a radiologist thrown in for fun so I can switch out my own GJ tubes.

Martha may be the only one out there like her, but I'm certain that I'm not the first mother to walk this trail. Still, I am a member of these support groups that absolutely drive me bonkers. I recently asked a support group if there was any information available on atypical development. There must be a resource out there that lays out the implications for missing certain skills, the importance of other skills, and how their delay or absence impacts the realm of lifelong development. Someone out their MUST know that information, or we wouldn't be doing all of this work trying to get Martha to "midline."

Instead of information from the support group, I get answers like, "Timmy rolled at 11 months, and stood at 24 months," explaining what each other's child did at what times. Or, "Janie walked around two, you just need to be patient," and the super ambiguous, "Each of our children are so unique, they all do things at a different pace."

!!!!!!!!!!!!!!!!!!!!!!!!!Ahhhh!!!!!!!!!!!!!!!!!!!!!!!

Then, there is the more serious side of things that are hiding in the corner. I feel like we're in a big lottery pool, and our name might be drawn on some prizes, and missed on others. Martha didn't draw the ticket for a cleft palate, and her heart ticket wasn't the jackpot, either. If her feeding and swallowing dysfunction were a lottery ticket, she'd win the powerball. But there are still tickets out there for epilepsy, mitochondrial disease, different dystrophies and degenerative muscle and neurological diseases. I keep looking at the way Martha's arms and legs are moving and wonder how many years she can get by without surgery.

But they all keep me in the dark. Because her doctors don't know when or if Martha will develop any of it. They can't rule any of it out, so they watch her closely. I ask about surgery, and they say, "We'll try to avoid it." It's then that my knowledge and education fail me. They know what's out there, what to look for, the tests to run, and when to run them. I don't. They know the implications for a child who will only use one side of her body at a time, and I don't. But in the midst of these specialties are giant, gaping holes where I'm not confident that a specialist will see the big picture, trapped in their pathology.

Several months ago a doctor remarked that maybe there was a place for me in the middle of this discovery, a way to trail blaze this condition that has only, and likely will ever only affect one person. My daughter. I can't tell you how much I wish that role had already been taken by someone else before me.

I want to read the book, not write the book.

Until the next things pops up...

The Plan.

Tomorrow Martha is going to get a new GJ Mic.Key button. The procedure is scheduled in the morning at the St. Paul Children's hospital.

This works out well for us because later that day Martha has a second appointment with a pediatric ENT to hopefully diagnose a possible mouth birth defect and/or cyst-like tissue in her mouth.

In the meantime, Martha's J-port of her tube is completely blocked, and we are running her pump through her gastric port at a much lower rate to keep her nourished and hydrated at a less than optimal level. When liquid is put directly into her stomach, it causes added secretions/mucus, which especially on top of a respiratory infection, are difficult for her to manage. She's doing very well with it thus far, but it will be very nice to get her new button and her pump running full speed again.

Martha has another appointment on Wednesday (a bit more local--only 30 minutes away), that has a tendency to take HOURS. But, this specialty is incredible, and really, REALLY understands Martha and the way she moves, her tone, her strengths and weaknesses. I have a feeling that as soon as Martha loses her Cranio-Cap that this specialty will be bracing her arms and legs. More on that later.

Add all of that to occupational therapy, physical therapy, a Cranio-cap adjustment, an order to medical supplies for bags, syringes, and extension sets, plus a cortical vision assessment, and hearing test to schedule, and there you have our week in a nutshell.

I'm on my second cup of coffee and have a meal plan for the week ready to go.

Just breathe.

Nothing To See Here.

Martha has a respiratory infection. Her first serious one in almost two months, maxed out on breathing treatments and steroids. I don't want to get into details over how Martha's URI's affect her and the rest of the household, but they are most definitely dramatic. High fever, wet cough, dry cough, vomiting, choking, or whatever other symptom pops up, both Mark and I are in a constant state of trying to reassure one another out of our panic.

"She's okay, right?"
"Are we still in the yellow zone? Or have we moved to red?"
"Can we make it through the night?"
"If we brought her to the ER, what would they be doing differently than what we can do here?"
"Fingernails look okay? Lips look pink? Toes look good?"


Respiratory infections are by and large just exhausting. We don't sleep because she can't sleep. The added secretions mean a lot more linen and outfit changes. Keeping on top of the breathing treatments means taking the time to sit and do them...around the clock.


The particular URI that presented itself yesterday came with a little surprise. Martha's pump started malfunctioning. After using my whole bag of tricks, I realized it wasn't the pump, but the actual button inside of her was clogged.

This is incredibly complicated. Holy friggin smokes.

Martha's formula is bypassed to her small intestine. She has separate port on her Mic.Key button that goes into her stomach, but it's strictly for meds. If the content in her stomach is too great (like...more than a few teaspoons), it all comes up, she aspirates and winds up with pneumonia.

In theory I could and DID use this port when I absolutely had to, but it runs a huge respiratory risk, especially when Martha is already up coughing and gagging all night anyway. To route the formula, even at a rate half as fast, makes her cough exponentially worse. She doesn't eat enough to sustain herself, and when she's sick she can't eat at all. The clock is ticking.

Using my googling power, Mark and I spent hours last night suctioning with syringes, pumping hot water, flushing and re-flushing the night away. There was a short-term remedy, but today we're back at square one. A call to Martha's gastro specialist gave us another idea before ditching our Trick-or-Treating plans for a road trip to the Children's hospital for a replacement procedure.

They suggested a little acid to dissolve the blockage. You know, just pump the baby's tube with a bit of Coke. I took the bait, and that's my current strategy, and as negligent as it sounds to send Coca-Cola meandering through my daughter's intestinal walls, if it means I can pump hydration and nutrients back in (we are terribly behind...), then it's worth a shot. My very last resort is a very small amount of Drano, I mean "Clog-Zapper" left at my disposal to pump in Martha's tube. Although my guard is on with this product with the label telling me to use gloves and eye protection when using. Like whoa.

My plans for today, tomorrow, and indeed the entire weekend don't include five hours in the car for a five minute replacement procedure. While I wait for the Coke to erode the minute french size of my daughter's tube, I really hope our efforts produce some results. Round one was a loss, but I'll try and try again.

As I remarked to Mark at a painful hour last night whilst surrounded by medical supplies and prescription drugs, "I can't believe they're letting us do this."

Considerations.

As always, life is wild. I've been blogging, but not posting my blogs, if that makes any sense. I've had this habit recently when I've written a thought-out post in the evening, only to chicken out in the morning and never posting.

I worry about the way our life is portrayed by me on this blog and if I'm giving an accurate picture. Sometimes, someone who has read this blog but isn't intimately involved in our lives will see us in person and be pleasantly surprised that it wasn't as severe as they had imagined. With others it's the opposite affect, and they don't understand the situation until they're in the thick of it, and realized they underestimated.

Needless to say, I'm trying very hard to be honest in my posts and especially in my feelings, which is difficult, because if I write something brutal and gut-wrenching, our lives might seem a bit grim, when in fact, it was only one post regarding one aspect of an event. Ho hum. I'm trying to iron out the kinks and have the courage to write prudently and accurately.

One thing that I have been thinking about a lot lately is our home. Our house doesn't photograph well with my camera phone, which is why I don't post house pics on our blog. But, I love our house. Love it. It's a century-old Craftsman style, with hardwood throughout, built-ins, beamed ceilings, and three stone fireplaces. It matches my taste and era. The kitchen cabinets may be over 60 years old, and there is only one bathroom, but we've overlooked it because we just like the rest of the house so stinking much.

But we have problems looming ahead. Our 4-square home has all the living space on the main floor, and the bedrooms and bathroom up a mighty stairway. Our nearly 11 month old daughter isn't sitting or even rolling yet. She will soon have adaptive equipment in the house. Her bedroom is upstairs, her things scattered throughout the house.

When we first moved in, we were comfortable with the idea that we would eventually remodel our kitchen once our children successfully destroyed the remaining functioning parts (they have a good start), but now that plan seems short-sighed. The only thing that changes right now is our ideas, and how much money it might cost to implement those plans.

There is no emergency here, as we'll be able to carry Martha for quite awhile, and the truth is that no one knows what next year, or the next year may look like. We only anticipate that her needs will be best met without stairs involved with getting her to the bath, crib, and toys.  

Life, and God work in funny ways. Mark and I had our eyes on this house for the better part of five years. We never once considered that it might not be functional for our family. I found out I was pregnant within a month of us buying the house, and now we have Martha. I won't question why, because too many of these choices were made with earnest prayers.

Another part left to trust, another knot waiting to be untied.

Babies and Dolls.

One of the best way to get Martha to smile is to have her look at faces. If she can't have a smiling face to look at while she's awake, a close second is a baby doll face. She gets SO excited to see a doll face and tries so hard to hold the baby doll and bring it close. 

We now have several very small baby dolls for her to play with, but she also especially loves two dolls kept from my childhood.

Kimmy

And Lisa. 

What I hadn't quite expected when I pulled Kimmy and Lisa out of the dusty box they'd be stored in, was that it would be Paul and Michael who would also lay claim. Paul diligently found diapers, and then outfits for each of them, and then a suitable blanket for the chilly weather. 

The remainder of the rainy morning was spent interchanging the baby dolls from swing, to bouncer, back to the rocking chair, and then to the couch for a quick cuddle. 

I know that boys play dolls, and that it's not an incredibly huge deal, but this is the first time I've seen it in our home, with the boys playing together. My Paul has such a beautiful imagination that it's been a gift to watch him lead his little brother in pretend play. 

Hope you're surviving the rain! We threw in a nice, long lunch at a restaurant, too. Hope I don't run out of ideas for the afternoon!

Wrapping It Up.

1. I received a great phone call today from the pediatric medical supply company that Martha's tubing supplies come from. Her insurance will now begin covering her Neocate. I was able to get a month's supply from WIC on Monday (I was in a hurry before the funds ran out from the shutdown), and now I can rest easily knowing that from now on (fingers crossed), getting Martha's formula will be as simple as a phone call.

2. I am patiently waiting Luke's arrival home from school. It has been a good week, but I'm looking forward to having him in the house all weekend.

3. I'm bidding on a lot of used toys on ebay for Martha. They light up and make noise, which, with my other children, would have been out of the question. But I see their usefulness with Martha, and I'm excited thinking about how well technology is able to help young children communicate. If she can learn the concept of pushing a button to make sound, then we're set.

4. Looking through my closet, I have exactly one pair of jeans I like. It shows my age, but I really like wide-leg, trouser fit jeans. Do you have any idea how hard those are to find? I won't wear skinny jeans, matchstick jeans, or any other style of jeans that tapers at the leg. I just can't do it. Have I mentioned I'm only few months from turning 30? Are wide leg the new Mom Jeans? I pray not, I dearly love to look like I know what I'm doing.

5. In August I went full-blown Paleo. I felt great, lost a few pounds quickly, and had an enormous amount of energy. Mark was in it with me, too, and I had fun making different pureed soups and spaghetti squash; green smoothies. We fell out of it because we both love bread and potato chips. Ho hum. After a week of carbo-loading lamenting my eldest's stint at school, I feel like the big el crappo. I'm going to try Paleo again, or a modified version of it that includes Oreos, and see if I have a little more energy. We might decide to train for something, too.

Have a great weekend!

That One When He Went to School.

My oldest boy started school this week.

You know....school, as in the brick building with all the children inside.

As I've written before, I can't remember a decision that every caused Mark and I more anguish.

But, once made, it is a decision we found both necessary and right. We weighed pros and cons for months. I sought the advice of people I trusted with the honesty of my heart, and perhaps I was waiting for someone to just come out and tell me what to do.

No one forced our hand or tried to convince us of what would be best for our family. That was our conclusion to make. Because, we could have done many things. I could have decided to "unschool" the kids for a year or two, or have a 2-3 day homeschool week, or even enlist more help to delegate Martha's appointments and household work.

I knew I could do it; that it was possible with a certain amount of creativity and sacrifice, but I wasn't sure if that's really what I was supposed to do.

So, we prayed very hard, and very intentionally. My previous post, "Of Miracles and Madness" details that journey.

When it came down to it, I was very tired and pulled in too many directions. I am a huge believer, and supporter of homeschooling, but this wasn't what I wanted it to look like.

It came down to this.

I was told recently that our lives will likely take about three years to slow down; stabilize. That in three years, we should have a better idea of our daughter's health and development, that we should see things coming. Her growth will slow, she'll be stronger. We'll know that much more.

But in three years my sweet second son will be 7. My baby son will be 5. If I stay the course for three years, try to tough it out, I will miss it all....them. When I think of how well-prepared my oldest was for homeschool work, how he shows leadership and kindness, and all the time I've invested in his care and formation, I think, "How can I not do the same for these two sweet boys?" "Will I forget them in the mix of trying to do everything I can for my oldest and youngest?" How can I keep delaying their desire to read stories and build block towers, to sit on my lap and snuggle for no reason at all?

I only get this one chance with them. I'm not willing to let it pass.

My views on homeschooling are indeed very strong. But they aren't as strong as my beliefs about motherhood, and my role to each of the children and what I owe them right now, in this family.

What my children need from me in this moment is to be their mom. For however long...with all that entails. I'll give up one thing to be better at the other thing. They deserve that from me, they deserve the best I'm able to give. And I'm bold enough to say that I am doing the right thing here.

Putting my children into school  never once entered my plans before. I refuse to think of this as a defeat, however. We are deciding, with God's grace, to take this decision in stride. The second I start to see our situation as something to grieve, I'm toast. I won't go there, I refuse to go there.

Instead, I'll say "Let it be done," and echo my somewhat reluctant "Fiat" and trust with confidence that the Lord will continue to carry us through.

With Joy.

Of Miracles and Madness.

I was sitting in the doctor's office with Martha on Tuesday. We were following up with her Genetics doctor, and the Genetic Counselor. They asked me how I was doing.

"I'm doing well," I said honestly, "But I wish you would have told me, when I was sitting in this very place six months ago, that our lives were going to explode."

They nodded their heads in agreement. "We like to let parents discover that on their own."

What a discovery that has been for my family. Did they know by looking at me? Something was happening with my heart.

The heart of motherhood.

I had not been at peace. I'd walked around with tears in my eyes, couldn't sleep, and began to seek what in my life was causing this incredible heaviness that I could not lift. I wasn't depressed. It was much different than that. Things just weren't right. 

I thought about the people I knew who took care of themselves, and made time for exercise and entertainment; hobbies. People who breathed and sighed, who were carefree and enjoying their families and children. How would it feel to take delight? To not be so...so....overcome? I envied them in a way far different than jealousy. It wasn't so much that I wanted that, but that God wanted it for me. God desired my peace.

And this is where it gets tricky. Because I believe in God, Jesus the Christ. I believe every moment of my life is ordained by God's will. So I prayed. I prayed, and prayed, and prayed. I asked others to pray for me.

"I'll do what you want, Lord," I would pray, "But you MUST tell me what to do." 

"Lord, you have put our family here, and  I have to, I must, I insist on believing that You have not abandoned us. Lord, I'm doing something wrong here, I know that, but you must tell me that if we follow your plans that You will stay with us, that You will carry us through. I need to know that Your Will for our family is bigger than my own aspirations. Make me believe it, Lord. Force me to believe it."

I prayed and waited, and surrendered.

St. Therese is dear to many Catholics, including myself. I don't say this lightly, but this Saint has worked miracles in my life. Her feast day was last Tuesday, the same day I sat in the Genetics Clinic wearing the face of a mother who knew too well her own limits.

What nobody knew on Tuesday, is that I had been praying for a miracle to occur on that day. I prayed for a miracle. I prayed...for Roses.

I had a decision on my heart that I did not want to make. I knew what I wanted, I knew my goals, my dreams for my family. If God had something else in mind, He'd better make it so clear to me that I could not deny it any longer. (I'm a stubborn daughter like that)

So, you could imagine my utter shock, after spending the whole day away from my family and four hours in the car, when I walked into my house at dinner time, and the first thing I saw was a bouquet of SIX PINK ROSES, sitting on my kitchen table in a vase.

I was trembling as I opened the card, believing that the logical inscription on the card would say "Go for it. I'm taking care of all of you. Love, God."

But it didn't say that. Instead, halfway across the country a friend had been praying for my family's intentions. She didn't know of my wager with God and St. Therese, but felt her heart move to send a rose for each member of my family along with her prayers on the Feast Day of St. Therese.

I can only describe to you the peace that entered my heart at that moment. To know, after so much questioning, that God was indeed watching my over family, that He has NOT abandoned us, and that He knows, more than my best intentions, what is best for my family. Despite my fatigue from the day, I was invigorated. We're going to do what God wants, I told my family. Because I have to trust.

Why did God answer my prayer?  I am not deserving of such a miracle, but I humbly learned that God rewarded my desire to be faithful, and my total reliance on Him when I no longer knew what to do. Indeed, God works in my life the best when I am the most desperate.

Today brings peace. All of those prayers.........thank you from the sincerest depths of my heart.
+FIAT+

Positioning.

The term "positioning" is a new one for me. It's new, but  important. Positioning is the way a person supports their body. This is important for a whole slew of health reasons, probably more than I'm even aware of.

In our situation, positioning is vital for Martha's entire digestive tract and aspiration. As she grows longer and heavier but continues to be delayed in her milestones, positioning becomes necessary to prevent scoliosis and the specific way Martha moves her body. She is followed closely by a doctor who specializes in movement, and who is constantly aware of the unique ways in which she moves her body.

So, in June, we were very happy that our PM&R doctor (Physical Medicine and Rehabilitation), referred us for a seating evaluation for an adaptive chair that would position Martha correctly with her spine and head aligned, and her arms and pelvis supported. How wonderful!

The process quickly turned into a disaster. I wasn't getting any communication from Medical Supply, we waited weeks and weeks to see a demonstration chair (which is required for insurance reasons), and my phone calls went unanswered. Finally, after nearly 10 weeks of waiting, we had a short demonstration in our home with an adaptive chair.

After our demonstration in August, I waited four weeks with hope that insurance pre-authorizations were being run, and that letters of medical necessity were being written.

Finding it odd once again that I hadn't been contacted, I called Medical Supply. I was rerouted to a different "Rep" for the company. I was then told on the phone that the Rep we worked with was no longer with the company and that we needed to start the process over somewhere else.

No progress had been made on my daughter's chair. For three months.

What a waste.

I put a lot of emotion into this adaptive chair, because I knew how much it would impact my daughter's quality of life. Don't they know that? Don't they know that I bring our stroller into the dining room every night so our daughter can sit with our family during dinner? Don't they know that she's 10 months old and the only place I have to set her down is a bouncer seat that my other children outgrew by three months old?

Medical Supply emailed me back all the referral information that I had given them, as well as the line "We're sorry, and thank you for your understanding." I sent a scathing email back thanking them for the apology, but told them in very blunt terms that they absolutely did not have my understanding.

I wasn't unreasonable, however. Because that's where things kinda suck. I can't threaten, and there are no consequences for the person on the other side who let Martha slip through the cracks. I'm at their mercy. If they don't do their job, I can tell them how terribly disappointed I am, but it doesn't change a thing for our predicament.

Yesterday, a week after our fallout, Martha had another seating evaluation in a different location, different vendor and different products. What a difference! I have my fingers crossed that within three months Martha will be sitting in her new chair!

 This is the style of style and brand of Martha's new chair, although this picture is not exactly what hers will look like. Martha's will have a strap across her chest, supports at her pelvis and neck, and be smaller all around.

The very cool thing about this stroller is that the seat detaches and can fit on top of a frame that shifts height. She'll be able to slide up to the table, or as high as a breakfast bar! There is an attachable tray, and a canopy. What a new world for her!

In addition to her positioning system, Martha is also getting this adaptive carseat. I saw this carseat in person, and wow, it's huge. The material is soft and fuzzy, and the model I saw was brown, so it looked just a teddy bear. This system will fit her to 100lbs. We have a pretty big vehicle, but I've never seen anything like this before. Once we have it I'll have to show more pictures so you can actually see how intense this system is.

There are more posts I want to write about this specific situation, because it helped me learn a lot about this process. You, dear reader, will likely never attempt to acquire a pediatric adaptive positioning chair, and it was a first for me. Despite me being a squeaky wheel, it wasn't enough in this case. I will know better next time.

Here's to getting our little one sitting!

What it takes.

Ever wonder what goes into that tube of Martha's?

Martha has been on an elemental formula (Neocate), since she was three months old. Elemental means that the food is already digested and broken down. Why she's on it is still somewhat of a mystery.

Martha went from breastmilk to several kinds of thickened formula. Then something so severe happened with Martha's digestion that it put her in the hospital. She began vomiting after every feeding so that it would block her airway. I couldn't take her out of my sight. I carried a bulb syringe in my pocket to clear her airway when the formula would come up.

Sometimes, if I left the room and came back, I'd see Martha drowning in her own secretions, turning blue. I'd tip her upside down to let the formula drain from her nasal passage. I'd stick the bulb syringe down her throat to get her to gag enough to vomit.

I had the steps to infant CPR taped on my fridge.

Shortly after that, she started having diarrhea with the vomiting and developed an aversion to eating. She lost weight and was diagnosed with Failure to Thrive. This is when she was hospitalized for the second time and put on Neocate. Neocate works, so there hasn't been a lot of investigation into what exactly caused her issues .

The problem with Neocate is that even though Martha's doctors consider it medically necessary, Martha's primary insurance won't cover it yet. We're in the process of appealing that decision. Thankfully, Martha has Medicaid as a secondary insurance because of her chromosomal abnormality. Where this gets tricky is how the funds for Medicaid are distributed. Because Neocate is an infant formula, and because of Martha's age, Medicaid will not cover Neocate either.





Do you know where the funds for infant formula are funneled for Medicaid?

The WIC Program.

Do you understand what that means?

It means that every month I have to go to the Public Health Office at the county, give personal information to the Public Health Office, and get nutritional counseling for my daughter (who does not eat), as well as have her weighed and measured.

It is astonishingly humbling.

I need to be careful now, because I have no intention of complaining about the WIC office. Truthfully, they have been incredibly kind to us. They want to help Martha, they are here to help her, and I'm thankful. The people in this particular office have an uncommon amount of understanding of what it takes to care for a special needs child with a feeding tube and seem to really appreciate the effort we're taking. For that I'm grateful.

Why go through all of this, you might ask? Why go through the extra phone calls to order formula, paper work, blood tests and weight checks? One. More. Thing. To. Do. One. More. Step. To. Manage. 

One...more....appointment............

Is it worth my time? The headache? The humility?

There is a very good reason why our private insurance is trying to milk every month they don't have to pay for Martha's Neocate.

It's because it's incredibly expensive. In fact, it's more money to feed Martha in a month than the entire rest of our family. It's like a mortgage payment. And as she requires more nutritionally, she will require more formula.

(Pantry closet with an extra "Mic.Key Button," box of Neocate, and box of formula bags)

Acquiring Martha's formula is indeed one of the more stressful things I need to do for her. She's only allotted so much per month. If I don't have Martha's formula, Martha can't be nourished. Neocate is hard to supply, and only certain pharmacies and medical suppliers will stock it. If we run out, it is very serious. A baby who is unable to eat and a mother who relies on others to make sure her formula will be available is one of the heavier responsibilities I carry. If I could go out and buy all the inventory of Neocate available, I would, but it just doesn't work that way.

Of course, the natural solution to this is to teach our Cosita to eat. In time. I have some hope.

Kids in the Fall.

The Fall in MN is too short. Autumn isn't humid, there aren't mosquitoes buzzing about, and it's still not too cold to enjoy hours outdoors. Which is exactly what we've been doing this week. 

(Same shirt, different day. Can you tell it's a favorite?) 

Considering the nice outdoors, I asked Luke to take a picture of Martha Mary and me. 

That never turns out well. 

 Luke, this is how it's done. Let me show you. 

 See how well I took that picture of you and Martha, Luke? You do the same thing as me. 

Your turn, Luke...

Um, I guess it's a little better. 

Luke, could you please get a picture of Martha Mary and me. Where you can see BOTH of us? Multi-step instructions are indeed a challenge for every seven year old boy. 

There 'ya go, buddy!

Martha's really enjoying Flannery O'Connor these days. Never one to let a severed head damper her fall fun.

 We managed to get three walks in yesterday. We'd better get a move on that record today!

Transformation

I was taking pictures of my 4.5 year old, Paul, the other day, and thinking, "Wow, he's really cute. I can't believe how darling he looks!

Can you believe my strapping, handsome young fellow, who was nearly born on the side of road, went from this...

(Paul at 3 months old)

To this...

Paul at 4.5 

Paul's first several years were a challenge, but when he turned four, there was a noticeable change. He learned to talk well and express himself in words before his emotions got away with him. He developed a sensitivity towards others that made him a magnet for other children and making friendships. In the middle of two very concrete and literal boys, Paul is able to teach these boys to play, actually play. A toy which seemed useless suddenly becomes essential once Paul puts a name and a purpose to it. It's a skill, indeed. 

He's as unrecognizable in photos as he is in temperament. 

(Paul at 8.5 months old)

Paul has my heart in a very specific way. It might be because he loves me a lot, or that we're both "middle children." This picture of him falling asleep reading National Geographic with a kickball might explain things, too.

 I try not to project how each of the children will be or what they'll do. I pray they do what God asks of them. But with Paul I have a distinct feeling about him being a natural leader. It's a tall order for our smallest child, but it's been a pleasure to watch his transformation. 

What I Know For Sure: Development

Martha is globally delayed. Every cell of her body is affected by her syndrome; each cell carrying an extra piece of 8th chromosome and a deleted end of 5th chromosome. Every chromosome contains genes that give the body instructions on how to function., and the result of her condition depends on the genes that are deleted and duplicated.

If Martha had a full Trisomy 8, she wouldn't be here today. The genes contained in chromosome 8, when fully duplicated, are terminal and incapable of sustaining life. However, Martha only has a partial duplication. Additionally, the deleted material on 5q is very small. Sadly, the deletion of even very small amounts of genetic material is hugely significant. Despite knowing Martha's chromosome arrangement, the impact is not fully ascertained. I am hopeful that in my lifetime more will be understood about this area of science.

Martha is hypotonic, which means low muscle tone. For those who are hypotonic, it means they have to expend  more effort to achieve the same result. This goes for everything. Martha gets fatigued holding her head or even being held. I often have to lay her on the floor to rest. To hold a small rattle will make Martha's arms quiver with the effort.

The muscles inside her body are hypotonic, too. Her esophagus, tongue and stomach are weaker muscle groups. Every movement and breath expends more effort and is tiring. Her lung muscles are weaker. Martha's inability to eat and her difficulty managing saliva, coughing, and choking are all attributed to her hypotonia.

Doctor Fail #1. Nearly all of our doctors have been incredible, but before her diagnosis, Martha was 3 months old and in the hospital for a second time. I told an attending doctor that I was very concerned about her development. Martha wasn't smiling yet and couldn't hold up her head. She was so weak I felt like I might drop her. I was crying to the doctor. He told me, "She'll probably just be a late walker. These are the kids that don't end up being the best athletes." 

Two weeks later Martha was examined thoroughly by a doctor who later wrote that Martha was "impressively hypotonic and developmentally delayed." Lesson learned: I can only work with people who will listen and take me seriously. My standards are admittedly very high when it comes to my daughter, and I don't have any patience trying to convince anyone of what I already know. This part of my job stinks, because when it comes to my daughter's care, I feel much like Mr. Darcy. "My good opinion once lost, is lost forever." 

At 9 months old, Martha can roll from side to side on the floor. She can hold her head up but tires. I can hold her upright in my arms for a short time.

She cannot lift her head off the floor when on her tummy, or roll from her tummy to back. She isn't able to laugh yet or make many sounds. She just met a HUGE milestone and is now able to reach for toys.

On the opposite spectrum of hypotonia is increased tone. Martha's arms, hands and feet display increased tone. Her hands are fisted and her toes are curled. She moves her extremities in a more fitful way. Often, to have her to hold a toy requires us to pry open her hand. When she drops a toy, it's simply gone for her.

I could go on and on about her development, but to be brief, she is much like a 3 month old, with the exception of the skills on her tummy, which are like a newborn. On a positive note, she is very engaging and aware. Her smile is gorgeous and her temperament is superb. I call her "Mommy's Joy." She might grow up thinking "Joy" is her name.

Written out in such a matter-of-fact way can seem grim. Trust me, it's not. Reading and rereading this myself sounds harsh when presented as fact, but the reality of it isn't bitter. This is just who she is, we aren't bothered by it. If I wrote a synopsis of my own life: "Mallory doesn't work, Mallory can't play golf, Mallory is mildly proficient at piano," it doesn't sound very encouraging. It is what it is. Martha will do a lot of things, but they will be on her timeline...not that it matters.

Babies who develop normally gain skills SO quickly that it is easy to miss their importance. For instance, Martha does not bring her hands together (midline) and clap. I couldn't tell you when or if my other children did this, because in the scheme of skills they were gaining, it wasn't on my radar. I've now learned, these small intermediary skills like clapping, or transferring a toy from one hand to another, play a huge role in brain development. These skills are so vital that Martha is getting therapy to be taught these skills that come naturally for most people. There is a lot to learn, but we take it in pieces.

Have a great weekend, everyone.

Homeschool History.

We learned about nomads today. 

The boys are outside collecting sticks for spears, and digging up tree roots to store for the winter. 

Their clothes are covered in burs from the woods, which they are throwing at squirrels trying to capture to put into their nomad stew. 

This is what learning through play look like. I love every minute of it. 

What I Know For Sure: Health.

Martha nursed just over 3 weeks when she was ambulanced to the St. Paul NICU with pneumonia. She was diagnosed with a feeding dysfunction and put on a thickened formula. She had an echocardiogram done for a murmur and was diagnosed with a Bicuspid Valve (minor issue). The cardiologist mentioned something to me in passing about a link between a bicuspid valve and something called "Turner's Syndrome," even though Martha had no symptoms of Turner's Syndrome. Doctor Triumph #1: The cardiologist was the FIRST doctor to suggest that Martha's eating dysfunction and heart variation were not in isolation. Smart doctor. 

At 3 months old Martha was hospitalized in St. Paul again. She was choking on her thickened bottles, and was refusing to eat. I asked the attending doctor to run a chromosomal analysis to possibly diagnose Turner's Syndrome, citing the cardiologist's comments. The pediatrician agreed to run the test. Doctor Triumph #2: The attending doctor listened to my concerns and wishes for a chromosomal analysis. She acknowledged that I had four children and had troubling instincts about my daughter. Thank you, Doctor. 

The test took longer than the length of admission, and Martha was discharged weaker and weighing less than she had nearly a month earlier. At this point I knew without a doubt something was wrong. I believed I was taking my daughter home to die. 

Martha's chromosomal analysis came back significant. We saw a geneticist, a genetic counselor, had further tests and further visits to receive Martha's diagnosis of an Unbalanced Translocation 5;8. We were told that Martha would fall into the "moderate" category of symptoms. She was noted to have several facial features consistent with chromosomal abnormalities. 

At five months old Martha was placed with a gastric feeding tube extended into her jejunum. She is currently on a continuous, 24-hour a day feed. She has a pump in a backpack we carry around with her all the time. 

~Martha has severe eating difficulties and GERD to the point where she is unable to take anything nutritive by mouth. 

~Martha is sick frequently with serious respiratory infections. It takes a cocktail of breathing treatments, steroids and antibiotics to keep her from being admitted to the hospital. She has averaged 2 illnesses a month.

~Martha had a CT scan and MRI of her brain and spine to rule out hydrocephalus. Her head is abnormally large (macrocephaly) with extra fluid. The extra fluid isn't causing problems at this time. 

~Martha has an abnormal brain. I'm not ready to discuss what that means.

~Martha is "impressively" hypotonic (low muscle strength)

~Martha has a Cranio-Cap to correct her misshapen head. She developed plagiocephaly from her crib positioning and low muscle tone.

Pediatric Specialties Currently Following Martha

Gastroenterology

Pulmonology

Cardiology

Physical Medicine 

Genetics

Ophthalmology

Neurology

Plastic Surgery

Neuro Surgery

+Primary Care and Pediatrics

As time goes on, we are hopeful this list will get reduced. We may need more specialties added, but I'm keeping this post to things I know for sure.

Martha is at risk for several disorders. I have been told that she will require close following by multiple physicians until these disorders can be ruled out, which at this point in her infancy, they can't be.

Here is a little peep of my sweetheart just as she's waking up in the morning. Just look at how beautiful she is! Miss Martha Sunshine! My apologies for the quality of this video, the dimensions aren't right. It's Monday morning and I'm tired. I'll work on it and see if I can improve it later.

i dont do

Let's all clear the air here (in case you didn't already know), that this Mama is not crafty.

In fact, there was a point last year when I threw every last marker in our house away. BANNED! Forever! Paint, clay, and all the rest, you ask?  I laugh. You think I'd let that in my house? The last stash of play-doh the boys got in their Christmas stocking was probably all thrown in the trash by New Year's.

I'm really mean like that. I'm so not even joking.

I have this friend in real life who is ALSO a blog stalker. This is a big deal. She goes by "Karen" on the blog. I'll leave her real identity a secret. She has markers at her house. And pant. And glitter. 

Horrors.

Because she knows how deprived my children are, she generously is sharing her craft plans with me. Baby-crafter over here literally needs someone to photo copy craft plans, arrange the plans and supplies in separate folders, and then hand deliver them to my door. Karen is sweet. She is also a craft enabler.

On Monday the older boys did a craft. Sunday was the Roman Catholic church's celebration of the Virgin Mary's birthday. We made birthday cards for Mary and put them on the mantle.

Because I had a total breakdown on Labor Day and went to the store and bought all the necessary school supplies to put our boys in the public school, I accidentally had markers in the house. They were surprisingly useful.

All jesting aside, it turned out to be a beautiful activity. It was only coloring, cutting and gluing, but a step in the right direction. Pipe cleaners next week? One can hope.

That Hot Mess.

Martha was diagnosed with her syndrome in March, and then hospitalized for a third time in May. We were in the middle of our first year of homeschooling a kindergartener, our middle son was about to turn four, and our youngest boy hadn't even turned two. It was a difficult Spring and we were challenged to question the plans we had so happily made for our family.

One thing we've been forced to consider is moving. We live in a rural area more than 2 hours from the Children's hospitals and specialists. We don't have access to the feeding, nutritional, and positioning experts that a metro area provides. We may have to move. We don't want to, truly. But we've been forced to at least consider it so our family can function.

The second thing we had to take a serious look at is homeschooling. Just like how I love where we live, I love homeschooling, too. But we've had to reevaluate everything we thought we were going to do.

I will admit  that deciding whether or not to homeschool this year was one of the most difficult things I have ever questioned. Trying to make the right choices for your children carries with it such an incredible pressure that it actually weighed my heart down. For the better part of two weeks I was brought to tears thinking about the subject.

The children are home. For now. And it's going quite well. We get up early in the morning, and get started and finished before the distraction of Legos and weapon-building commence.

But there are multiple phone calls interrupting our happy learning. I wake up sleeping babies to run to therapy and appointments. I have to rearrange every day to fit that day's agenda. It's a lot to manage. Some days I am prepared to take the pressure of educating my children off of my shoulders and delegate it like I have needed to with several other things in our life. I want to live my ideal, but at what cost?



If the days that don't work take over the ones that do, the school down the road is ready and waiting to accept them. I will honor that. It might be too much. I can say that. I may need to focus on a different child's needs that only I can provide, and let someone else work on the reading and writing. I don't want to, but I will.

Thankfully our family has an army of supporters at our back, each giving us the encouragement we need. Some are my cheerleaders and support every decision we make because they know we are putting our children first. Others know my heart and can empathize with my blubbering ramblings. There is no judgment. I'm a hot mess when it comes to this schooling business, and I'm always so thankful when a friend or family member let's me pour it all out to them. They're amazing, regardless of the decisions we end up making.

Our family doesn't see putting a child in school setting as a failure, or even less-than-desirable. It's just that...for us, we want the kids home, to have our family function in this particular way. To give that up, well, would be difficult, just like moving would. But we also see educating our children as an incredible responsibility. In fact, as one of our greatest responsibilities. And so, if at any point our wants conflict with our responsibilities, we will choose the latter. When that day will come, I'm not sure. Anytime between now and college, which leaves a lot of years for wavering.

I believe this pull of my heart will never go away and I will constantly wrestle with trying to do the "right" thing for each of the children. This year brought a full house, but also a different deck of cards that I hadn't planned on playing. Every option must be considered, and prayed about ultimately. Our daughter is already taking part in the school system (more on that later), and that impact alone requires constant evaluation with how that impacts the rest of our family. It will change our plans, and maybe even our ideal.

Is This Thing On?

I left, I know. For months.

And so much happened.

Oh my goodness. So much happened that when I look back over the years of this blog, the early mothering, toddlers, adjusting to little sleep, all the petty complaints, moving, moving, and more moving, and decisions over schooling and places to live, so little did I know.

But then a few months ago it all came to me.

That every struggle, fight, and tear all had a purpose. Looking back at each pain from every year of my memory, it was all orchestrated, all put into motion; it was all planned. Not one trial from my childhood onward has been in vain.

It was all for this.

Martha Mary is now 9 months old, and I see it as no accident that I stopped blogging shortly before her birth.

Life now is different. How different?
There once was a certain life. There were our childhoods, our schooling, college, marriage, family, and children. Years passed, and then she came along. Now there is now only before and after; a BC and AD of our reality.

Because when you have a daughter who is diagnosed with an Unbalanced Translocation 5;8, and from all accounts and records, she is the only person on the planet with their chromosomes duplicated and deleted in this way, your life is shaken upside down.

If you know anyone out there with a missing 5q.35.3 with a duplicated 8p21.1-23.2 attached to their 5th chromosome, I would travel the world to find them.

Thankfully, she is our JOY. She is our purpose, she is the gift we never thought to ask for.


But it means I'm a Googling monster. I've mastered the art of PubMed searches, and have read every link and journal associated with "Partial Trisomy 8" "8p Duplication" and "5q deletion". The list of diagnoses  and symptoms is extensive. There's time for that later. I'm only just introducing you.

I'm not sure what this blog can do anymore. Like me, it can't be the same. There is no going back. The edges are raw, the pain is too visible and the fatigue wears like a heavy coat. There are tears over the unknown, and tears yet to be shed over heartaches that have happened months ago.

Is there a place for our new life on this blog? Our life is so happy, but it can be hard. Nothing about this beautiful girl causes us sadness. Nothing. But there is so much extra. There is therapy, teachers, people in our home all the time. There are appointments, more therapy, doctors, doctors, and more doctors with sub-specialties I never knew existed.

Let's not leave out the seating specialists, feeding specialists, nurse-coordinators, orthotists, special education classes, support groups, medical supply, feeding tube clinics, nutritionists, dietitians, and oh goodness, I may have just lost you...

But it's never enough. There are no easy answers, and often there are not any answers. There is nothing but an endless string of appointments, follow-ups, and watchful waiting. And do you know what that means?


I'm frustrated all. the. time.


There's time for all of that later, too.

And then let us not forget my Little Men

I couldn't ask for sweeter, more caring sons. These boys hold me together. They are fun, imaginative, affectionate, and keep me moving and ALIVE.

I shall dub this one Strong Father, Strong Daughter.


What will this blog be about? I'm not totally sure. I'm beyond worrying about recipe disasters and the moral obligation for cloth diapering. Seriously you guys, I'm so far beyond that now.

But I don't want to blog about all about my frustrations either. I don't want to blog about being let down; disappointed with those whom I expected more from. I don't want to be "that" mom who compiles a list of what or what not to say to a mother of child like mine.

Really? Do I have to get into it? "No, your advice is not helping me in this moment, and your concerns and questioning do not help me." Am I really going to be that person? The angry one who keeps getting burned and develops a great tolerance to heat but also avoids the fire altogether?

I may not be able to avoid it. I may just get very, very honest. And maybe a little sharp. A little sassy.

Which is hard for a Minnesotan, ya' know. We just don't come out and say it. 


But I love. I really, really do. And maybe love means being a little more honest...and a little sassy sometimes.

I'll do it for her. I'd do it for all of them.

+FIAT+