PR

34w0d, 8:46 pm. It’s a personal best by one minute and counting.

Every minute from here on out is a sixty-second victory.

IMG_7518.JPG

Advertisements

The Buttonhole: A Picture Story

We’ve had some big developments recently.

IMG_7450

IMG_7449

IMG_7454

I wasn’t sure if we’d see this day for years to come, but L’s weight gain and growth are fantastic, his vitamin levels are hanging in there, and patch vitamins and his unexpected ability to swallow pills mean there’s really no reason to keep g-tube access, which has really been nothing more than a safety net for the last several months. So as of Aug. 18, we are both line- and tube-free for the first time EVER!

There was a strong possibility that we’d have to go in and have L’s buttonhole surgically closed since he’s had his g-tube for four years. But for once the gut gods decided to take it easy on us, and we were down to just a band-aid by the end of the first weekend IMG_7459(L was excited that he didn’t have to keep the apparently-distressing bandage on his belly very long, but disappointed that water didn’t squirt out of his empty buttonhole every time he took a drink….).

There’s still a chance some residual leakage could cause problems, but everything looked stellar at his 1-month follow-up today.

So now we chug onward.

IMG_7447

Follow @ThisGutsyLife on Twitter

Babyface

Growth scan #1: at nearly 26 weeks, Number Two is currently measuring at the 90th percentile and appears to have reached the one-eyed spider doll phase of development:

IMG_7404

Fingers crossed for continued good reports (and that I pass my glucose test next week since it seems her one and only preferred food at this point is ice cream!).

Number Two: The Big Reveal

We’ve recently had our anatomy scan for #2, and apparently there’s this thing people do after anatomy scans that often involves a filled cupcake as the centerpiece of a big reveal. So, here we go, with much fanfare and drumroll and yada yada yada.

Dun da da dunnnnnnnnnn:

IMG_7123

That’s right, that’s a filled cupcake with all the filling on the inside where it’s supposed to be.

That’s what this cupcake thing is about, right? No? Hmmm….

Due to our history, we also had genetic screening at 12 weeks and an ultrasound at 14 weeks to check on abdominal development, all of which looked great, so we went into the 20-week scan feeling pretty good and hoping the keep the streak going.

Of course, we couldn’t make things too easy on ourselves, so our results did come with an “Everything looks great! Except –,” at which point I think I died about 12 times in the span of half a second.

The “except” is that while most umbilical cords have two arteries and one vein, #2’s cord has only one artery and one vein. This is relatively common and happens in about 1% of pregnancies (so naturally it would occur in mine), and it occasionally correlates with some scarier chromosomal or cardiac or renal abnormalities — but thanks to the genetic testing we opted for, we’ve got a green light in the chromosome department, and all other organs checked out at the anatomy scan, so odds are high that this is one of those random things that just happen sometimes, and is unlikely to affect anything else.

It’s like my body is saying, “Heyyyyy, I know last time I effed up and made the umbilical opening too big — that’s my bad. I’mma go ahead and make this one a little extra small to make up for it, m’kay? That makes us even?”

No, body, not exactly. But keep the rest of your shit together this time and I might let it slide.

There is a chance that this two-vessel cord could impair growth as the pregnancy progresses, so we’ll be doing monthly ultrasounds from here on out to check #2’s growth and development. If all goes well, we’ll proceed as usual with the added bonus of a few extra chances to wave hello to our little. If anything worrisome appears on those scans, we may end up inducing a little early – but at the very least we should have a little more than two hours warning this time around!

I’m not going to pretend that I took this completely in stride – I’ve already spent plenty of time imagining all the sinister things that could be lurking beneath this seemingly-simple semi-complication, particularly given the fact that we received a completely clean report after L’s anatomy scan and look where that got us.

But I’ve also had the opportunity recently to spend a lot of time at work thinking and talking about the importance of being in the present; and I think this may be a reminder to me to embrace the present and save my worry for a time when it’s useful. Nothing is ever guaranteed; we were given some breathing room after that early round of testing, but here is a gentle reminder that we will never be completely in the clear because that’s not how life works – I imagine we know that better than most. But things are looking incredibly good for us right now; we know we can handle far more than we used to think we could, if things should take a turn for the worse; and it serves us best to focus on the fact that right now, I am pregnant and my baby is healthy, and to take each month as it comes.

Oh, and in case you’re wondering, the filling is brown. Delicious, glorious fudge. But #2 is a girl.

Follow @ThisGutsyLife on Twitter

To the ‘pro-life’ senators who support the AHCA:

This is my daughter:

IMG_6630

She doesn’t have a name yet, but her brother is already smitten with her. She appears to be healthy so far, and we’re hoping to meet her around Halloween.

This is my son:

IMG_6702

His name is Lucas. He loves fire trucks, Star Wars, and Neil Diamond. He just turned 4.

If you refuse to protect the life of my son as fervently as you would protect the life of my unborn daughter, you cannot claim to be pro-life.

You see, Lucas has a pre-existing condition. He happened to be born with gastroschisis – a randomly-occurring congenital condition that left a hole in his abdominal wall – and as a result he has short bowel syndrome. It cost more than $3 million to keep him alive in the first few months after he was born, and several hundred thousand more since then, thanks to 11 surgeries, daily at-home intravenous nutrition and tube feeds, home nursing care, frequent visits to medical specialists and various kinds of therapists, and so many hospital admissions that I’ve actually lost count.

You claim to be pro-life, and yet you support a bill that would make it impossible for us to afford coverage for the medical care my son needs to stay alive.

Gastroschisis often results in a C-section in order to protect the baby’s organs during birth. You claim to be pro-life, and yet your so-called “healthcare” bill would make mothers question whether they should have a medically necessary C-section and would threaten the future health of any mother who has had a C-section for any reason, because you’ve classified having had a C-section as a pre-existing condition that will price many families right out of the market.

You dangle the impossibly unrealistic carrot of “freedom of choice” in healthcare as though it were a pinnacle of American values, and yet you would force a woman to carry to term a child who is incompatible with life.

You claim to be pro-life, and yet your bill would make expectant mothers think long and hard at the end of the first trimester, when many congenital conditions are discovered, about whether they can afford to carry to term a life-long pre-existing condition. You would set parents up to make that decision based on their finances rather than their beliefs or prognoses.

You claim to be pro-life, and yet you would throw essential health benefits to the wind, leave 23 million more Americans uninsured, raise premiums for the elderly by as much as 800 percent, and cut $834 billion in Medicaid benefits for low-income Americans in order to give tax cuts to the wealthy. 

You claim to be pro-life, and yet you have thrown the living to the wolves.

If you vote in favor of the AHCA, you are not pro-life.

If you believe that people’s right to life should not depend on their wealth, call your senators today and demand they vote no on the AHCA. 

Follow @ThisGutsyLife on Twitter

Happy Birthday, L

I’ve said before that while many parents think the early years fly by, for us it tends to feel like each year has lasted decades.

In those first hours and days and weeks we didn’t know what L’s future would look like or if he would have one at all, IMG_6713and yet now here he is today “my precious”ing his very own piece of cake (I guess when you’re allowed to have sugar for the first time after four years without any you kind of go Gollum) and laughing raucously as he makes his new Thomas Trackmaster Sky-High Bridge Jump hit him in the junk repeatedly. #miracles

It’s only been four years, but it takes an awfully long time to come as far as he has. His first birthday was really difficult for me, but I’ve come a long way since then too, and each year that difficulty fades a little more.

Happy Birthday, baby boy. IMG_6722

Number Two

I’ve heard a lot of special needs parents say, “I’d do it all again.”

I had a hard time believing them in some of our early months. I knew with every fiber of my being that I would go to the ends of the earth for my son, and I have never once questioned whether L is worth everything we’ve gone through for him, but if I had a choice — would I choose to do it again?

That question certainly complicates the fact that I’ve never imagined myself as the mother of an only child. For the longest time, we had our hands so full keeping our first child alive that we couldn’t even contemplate the added responsibility of a second child. As things gradually grew easier, as we learned to better navigate this medical life, a brother or sister for L began to seem more attainable – but that choice was clouded with what-ifs. In theory, we have no increased risk with a second pregnancy because gastroschisis is spontaneous and randomly occurring; but our eyes have been opened to medical realities we had never imagined before L was born, both our own and those of others, and some far more intense and all-consuming than what our family has experienced. And we know better than to expect we will never have any more bumps in the road with L.

And so, contemplating number two, we found ourselves faced with the real-life version of what has been only a hypothetical before. We knew exactly how difficult it could be if an unexpected complication arose, and we perfectly understood the potential threat to the current balance in our lives — and this time we weighed all those overwhelming negatives with the very real possibility of opting out before we ever got started.

It was never a matter of whether or not we could handle it; we have learned all too sharply over the years that you handle what you are given, because what other choice do you have? No, the real question was: Did we want to?

After much soul-searching, lengthy debate, and repeated decisions to delay just a little longer, and a little longer, and a little longer still, we ultimately decided that we did not want to let our decisions be guided by fear.

Number Two is due Nov. 3.

IMG_6630

Gutsy Surgery: Six Months Later

I think medical time moves differently than regular time. It has split personalities, perhaps. When you have young children, the weeks and months seem to fly by somehow (“Can you believe he’s going to be FOUR?! How is he almost four years old?!!!”) but in many ways, even this last big surgery already seems like a lifetime ago.

A friend asked me recently, “So, was the surgery a success?” She’d asked me something similar before surgery – how will you know if it works? – and I had fumbled through an explanation about how there may never be a clear answer to that question, in much the same way that there’s never really a finish line for us; there’s no real “cure” with L, it’s always an ongoing process of management, and varying degrees of better and worse.

Today though, just six months out, I can say without hesitation that our October surgery was a resounding success. Even to write that makes my breath catch just a little – how gutsy of me, to make such a bold proclamation, and surely perhaps a little naive? In our house, we don’t even say “at least bedtime should be easy tonight” because that’s the surest way to guarantee it will take hours; we’ve spent so much time refusing to look directly at progress for fear it will slip away that to voice the words “resounding success” feels downright dangerous. As certain as I am that this surgery was a success, I am equally certain I will live the rest of my life waiting for the other shoe to drop.

And yet – his appetite came in with a roar less than two weeks post-op, and there’s been a hungry lion living inside him ever since. He eats seven or eight times a day, and not just grazing; he’s eating full meals, and my picky eater’s short list of “L-approved” foods has expanded from roughly a dozen fruits, carbs and cheeses (but only in very particular shapes and forms) to an entire world of food groups and variety. Meats! Fish! Nuts! GREEN VEGETABLES!

He’s eating, and tolerating nearly unrestricted liquid intake, and his gut is working so incredibly much better than we ever dared to hope before. He’s having beautiful poops (Look, mama! Let’s take a picture!), and only 2-3 times each day as opposed to the so many we were seeing before. His belly is smaller – it would get horribly swollen before surgery, due to the dilated sections and bacterial overgrowth – and he very clearly just feels better all around. The cause and effect of all these things seems a bit chicken-or-egg, but they all play off each other to keep the rest moving in the right direction.

He was able to come off of TPN (IV nutrition) about six weeks after surgery, at which point we switched to overnight g-tube feeds (that means we’re running a medical version of Pediasure through the feeding tube in his belly, which goes directly into his stomach as if he were drinking it, only in a slow, steady drip that’s more easily absorbed). Over the last three months, we’ve cut the volume of that overnight Pediasure in half, and he’s been able to maintain weight, stay hydrated and stay on his growth curve; we’re hoping to keep forging ahead and sooner or later ditch the overnight feeding pumps altogether.

The biggest remaining hurdle is vitamin deficiencies, which aren’t going away anytime soon and can cause some serious health problems over time if left untreated. But even there, it’s an ongoing process of management, not a discouraging setback – and we have some exciting prospects on the horizon in the form of patches and pills.  

We’ll never be completely free and clear. L’s gut could re-dilate. He could develop a new stricture or obstruction or perforation – I still suppress a dull panic every time he complains of a stomachache. He could experience an illness that unexpectedly throws us into a long downward spiral. And as he grows, he may go through stretches where his body can’t keep up with his nutritional needs, and we may need to supplement in one form or another.

But for now, he is thriving. He is healthy. He is line-free. And we are making up for lost bathtime.

IMG_6242

Follow @ThisGutsyLife on Twitter

It’s an April miracle.

Let it be known that on this day in history, L swallowed a pill.

My not-quite-4-year-old SWALLOWED a motherflipping PILL.

This is huge – not least because about a year and a half ago, he was seeing a speech therapist for feeding issues and receiving occupational therapy for suspected sensory processing disorder. (Admittedly, the driving force behind swallowing the pill was that he loathes me poking the pill and squeezing its innards into his mouth, which doesn’t exactly rule out sensory issues here. Just let me have this for now.)

The potentially bigger reason this is huge, though, is that the particular pill he swallowed is a massive dose of Vitamin D (befitting of the superhero phase we’re currently in, we call it “Super D”). One of his biggest hurdles right now is vitamin absorption; certain parts of the gut absorb certain vitamins, and it’s likely he just doesn’t have the parts of his gut that are responsible for most of his vitamin D absorption. Twice each day, he takes a double dose of a liquid multivitamin designed for kids with malabsorptive disorders, as well as a separate vitamin E, vitamin D and liquid iron supplements.

IMG_6327.JPG

And these doses are large. To put it in perspective: a typical dose of adult vitamin D drops (and remember we’re talking about a 36lb child here) is 4,000 units. His regimen included those 4,000 unit drops twice each day, plus a 50,000 unit “Super D” once a week – and he was still deficient. We bumped up to 50,000 unit Super D twice per week — still deficient. We did recently add in these neat patch vitamins that claim to absorb directly into the bloodstream through the skin, which we’re hopeful might be a turning point for us, and our initial trial in the week before his last round of labs did show his Vitamin D levels moving upward – but by a whopping one point, while still taking his biweekly Super D, so the jury’s still out on whether or not we can switch to more regular-sized doses of patch vitamins (full review to follow after May labs!)

Currently, we put all these vitamins through his g-tube. After his last big surgery, we were able to drop the twice-daily 90 ml of green bean puree that had been part of his vitamin regimen for years (green beans are great for thickening up short gut poop!), and we had started squeezing the Super D into his mouth, which he hated, but it would be a miserable, hopeless battle right now to try to get him to take all the rest of these supplements orally, twice each day. (Have you ever tasted liquid iron?!)

Swallowing a pill, though — that’s new territory. He’ll still need alarmingly large doses of anything that goes through his gut, but we’re slowly weaning off of his overnight tube feeds; vitamins are the last big hurdle. And swallowing pills could open up new doors. Tube-free doors.

To be continued….

Follow @ThisGutsyLife on Twitter

Giving Voice to the Absences

img_6268Each non-Leap Year, Rare Disease Day is observed on February 28. I say it’s observed on February 28 rather than it is on February 28, because RDD is actually February 29. Like Leap Year babies, RDD chooses February 28 as its backup day of observance in all years that cannot be evenly divided by 4 (and also in years that can be divided by both 4 and 100, but not also 400 – have you ever read the actual rules for Leap Day?!).

It’s fitting that February 29 should serve as Rare Disease Day; it is, after all, a rare day. Perhaps even more fitting is its absence, its silence, in three out of every four years. Most years, for most people, February 28 slides on into March 1 as though February 29 were never even a thing. Unless you are one of the rare people born on Leap Day (at around 4 million worldwide, they are a group only 1% the size of the rare disease population), you probably don’t give a second thought to that non-second of time at midnight in which February 29 doesn’t really exist.

Rare diseases often exist in silence, too. They live in the absences: the friend who stops coming to gatherings because the exhaustion from a late night might incapacitate her for days; the coworker who stops coming to the office because the pain is too much; the mother who doesn’t explain why her child is still in the hospital because she doesn’t know where to begin.

Unless you have been directly affected by one of them, it’s likely you’ve never heard of most or all of the nearly 7,000 rare diseases recognized by NORD. I had certainly never heard of gastroschisis or short bowel syndrome until they were quite literally thrust into my lap four years ago. But if you know even a handful of people, odds are high you know someone who has been affected by rare disease. By definition, each rare disease affects fewer than 200,000 people in the United States at any one time, but collectively there are more than 30 million people living with rare disease in the U.S. – that’s about 1 in 10 – and an estimated 350 million people worldwide.

This year’s RDD theme is research, and research is certainly a fundamental necessity for the rare disease community. Fewer than 5% of rare diseases currently have a treatment, and lack of knowledge and quality information can result in devastating delays in diagnosis. Many people remain undiagnosed, sometimes for entire lifetimes. And the need for treatments and cures is pressing; rare diseases are responsible for 35% of deaths in the first year of life, and 30% of children with rare diseases will not live to see their fifth birthday.

Last year’s theme, however, will always resonate most deeply with me: Patient Voice. On a true Leap Day in 2016, RDD made those 350 million voices heard. Research is brought about by advocacy and awareness, and advocacy and awareness begins with voices. The patient voice is the best advocate in a world where even most doctors don’t understand rare diseases, and it is the best avenue for placing faces next to scary, unpronounceable words. It’s voices that fill the absence; voices that inspire more voices; voices that make sure someday, somewhere, a mother will never feel as completely and utterly lost and alone as I felt in 2013.

To hear more voices or to learn more about rare disease, visit rarediseaseday.org or Rare Disease on The Mighty.

Follow @ThisGutsyLife on Twitter