Another one.

I had a couple of good chats with Shierry's physical and speech therapists last week. We're beginning to think about the accommodations she'll need once she starts kindergarten in the fall of 2018 (?!?!). Her physical therapist confirmed concerns regarding the banding around her right ankle. The same amniotic bands that slashed her face also wrapped around her ankle in a not-quite-death grip. She's lucky to have a right foot, basically. The band impairs her movement and flexibility. When she jumps on the trampoline, she always lands on the ball of her foot, never flat. She favors her left foot as she traverses the obstacle course. Even with regular massaging, the banding is still very tight, especially in the back. She'll need surgery to loosen the scar tissue that makes up the band. It will take two surgeries. Ideally, both surgeries will occur before she starts kindergarten. 

There's also this tricky piece of tissue (muscle?) that is protruding a bit on the left side of her upper lip. It is becoming more and more prominent, and according to Shierry, sensitive to the touch. As her surgeon pulled down the skin that is now her upper lip from god knows where on her born face, we're not sure what this bit of annoying even is or where it comes from. As it's a surgical creation, I fear it has only a surgical solution. 

Plus, gah, her eyes, y'all. The prosthetic is not staying in place. Even when it is in, all sorts of irritants get in behind it, become trapped, and her immune system kicks in against the intruders, causing an unseemly amount of mucus and gunk to consistently seep out. It's a problem. She notices. Other kids notice. On top of that, we cannot get goggles to fit the unique topography of her face. I've tried an expensive pair of prescription goggles, nope. I've tried an expensive pair of superhero, comfortable foam goggles, nope. Water gets in, it bothers her eye that she cannot close, it freaks her out, she refuses to put her head under water, rinse, repeat. I'm about to give up on swimming this summer and just deal with it next year. 

Oh, and, today the Senate Republicans don't know what's in their health bill or what it would do. They're voting anyway.

This is absolute madness. What are their goals? What problems are they trying to fix and how is their bill (BILLS) going to address those? I've written before about my concerns. None of which have been addressed by my senators. John Cornyn says that they don't have the luxury of knowing which bill they're voting on.  That is bullshit. This is entirely their process, they own it. I usually try to be polite, but I am furious. This is my daughter they're talking about.  My other senator, Ted Cruz, has proposed an amendment that I guess sounds nice, but really exacerbates the problems already inherent in the original version of the BRCA. To the point where the INSURERS themselves are ripping it apart. The absurd bill to repeal the ACA but not replace it with anything would cause 32 million people to lose their insurance. People like us. 

They don't know what bill they're voting on, they've held no hearings. They've literally never had any answers when I've called, nor have they replied to my letters. 

Maybe your senator will actually listen to your concerns. Please, please, please call them this morning. We need your help. You can reach any of them at 202-224-3121. 

Princess Super Hero.

I told you, Princess Super Hero. 

Oh, Poodle's swell. She just turned 4. She says things like, "Mama, sometimes...when I think of you...I think of rainbows" and "Could be a girl driving that truck. Could be a boy. Who knows." The two most important fake people in her life are Rapunzel and Wonder Woman. She loves Woofy, but does not like her. She is brave. She's the best. 

She is now wearing a prosthetic left eye. It doesn't always stay put. We meet with the occuloplastic surgeon in August to discuss if there's anything to be done about that, possibly surgery. Also on the surgical docket: releasing the banding around her right ankle, caused by the same amniotic bands that clefted her face. Her physical therapist is keeping an eye on it, but it does now appear that the banding is affecting her flexibility and balance. It will require two separate surgeries. We'll figure that business out in November when we meet with her craniofacial plastic surgeon. 

For right now, I am focused on another looming threat to Poodle's health and quality of life. The American Health Care Act (AHCA) is a bill rejiggering the Affordable Care Act, also known as Obamacare. There are many, many issues with it. Please indulge me while I work out, in blog-form (truly, the American way), my thinking on the two most critical issues to our family: bans on annual and lifetime limits and meaningful coverage for pre-existing conditions.

The first issue is the precarious state of Obamacare's ban on annual and lifetime caps. This ban prohibits all insurers, including issuers of large group employer-provided plans like the one Poodle and I have, from establishing any lifetime limit on the dollar amount of essential health benefits for any individual. While large group plans are not required to cover essential health benefits, if they do provide those benefits (and most do), they are prohibited from capping them. Make sense?

I'd heard a lot of hullabaloo about essential health benefits, but until this weekend, hadn't examined them too closely. What I found astonished me. Our family relies or has relied on every single one. 

• Ambulatory patient services. CHECK. Regular exams by two surgeons, an ENT, ophthalmologist, speech therapist, audiologist, occularist, etc. Bazillions of examinations and pre-op and post-op care covered.
• Emergency services. CHECK. One time Poodle ripped out the sutures holding her left eye closed, back when we didn't know the fundamental health of the eye and surrounding bone and tissue. We were terrified that exposing the eye would cause irreparable damage and we rushed to the ER, where they had to facetime with her surgeon because he was in Dallas. We also had to go to the ER one Saturday when we were all puking obscene amounts of nothing and also that one night I chopped off part of my finger with an immersion blender, all of which were covered.
• Hospitalizations. (like surgery and overnight stays). Uh, mother freakin CHECK and, with minimal gripes, covered.
• Pregnancy, maternity, and newborn care. CHECK. Where to even begin with this. The entire tragic sojurn to Houston was covered. The equally stressful but slightly more hopeful trip to Houston was also covered. Constant sonograms, induction, NICU, all covered. The only thing, other than co-pays, I even remember paying was for the fetal MRI, which was, I will never forget, $600. This represented our 20% contribution to the total and the most I had ever, up to that point, paid for a single healthcare service. 
• Mental health. I've alluded to it before, but I struggled with severe postpartum anxiety and depression and continue to see a therapist and take medicine, which are covered. This makes me a functional person and allows me to be a good mom. It's just a fact of our lives. I have bad hips, anxiety, and I love Playing House. 
• Prescription drugs. CHECK. See above, also all of Poodle's post-surgery meds covered.
• Rehabilitative services and devices. CHECK. Poodle sees her physical therapist and speech therapist weekly and that's covered too. Her prosthesis might even fall under this category.
• Laboratory services. CHECK. One time when I was in the midst of my postpartum nightmare, I had to take a 6ish-week old Poodle to the ENT and, two hours later across town, to the hospital for a CT scan. This was on a day where I had begged Dan not to go to work because I literally could not fathom how I was going to get through the next minute, let alone the rest of the day, without him, and so my wonderful mother-in-law was babysitting me (giving my parents and my sister a day off). The CT scan was covered. And I got better.
• Preventive and wellness services. CHECK. Vaccines, various screenings, etc, all covered. Mom, baby, and community healthier.
• Pediatric services. CHECK. Poodle is a kid and sees more pediatric specialists and subspecialists than I ever knew existed and that's covered.

So, because my healthcare plan covered all of these benefits, they could not limit the dollar amount they would pay to cover those benefits. I've had difficulty pinpointing a typical pre-Obamacare annual limit, but little Poodle-doodle has accumulated between $100,000 and $250,000 worth of medical expenses every year of her life. That apparently puts us in the top 1% of healthcare spending!  I've read in multiple sources that a typical lifetime limit was between $1 million and $2 million. Folks, she just turned 4 and we're at about $740,000. We've been spared so many horrible things, we've never had to decide between a medical procedure and bankruptcy. But, we're close to hitting those traditional limits. 

This keeps me up at night because the House version of AHCA would, in effect, allow those annual and lifetime caps to creep back into our lives. The Senate looks to be in favor of maintaining this horrifying particle of the House bill. But, but, but, they say they're keeping the ban on the caps. Which, true, but more importantly, meaningless. Currently, a state's slate of essential health benefits must meet Obamacare's coverage standards. The AHCA removes this mandate and would allow states to seek a waiver from those standards. Meaning, some states, like, say, Texas, could so weaken the meaning of "essential health benefit" as to render the term utterly hollow. As a result, the specific benefits that insurers of large group, employer coverage cannot cap could drastically decrease. Dan and I both make good, steady salaries. We thought we were shielded by our employer-provided coverage. We were naive. We cannot afford to pay $100,000 a year out of pocket. We cannot afford to cover a surgery all on our own. If a surgery no longer falls within the meaning of an essential health benefit, an insurer could institute a lifetime cap on related covered benefits. We're two to three years from hitting such a cap, putting her at about 6 or 7. She's scheduled for a couple of bone graft surgeries at around age 9. What will we do? 

Additionally, we as a country have mostly settled on the understanding that people should not be denied health insurance on the basis of a current or former medical condition. Poodle has a congenital birth defect, the OG of preexisting conditions. Obamacare explicitly prohibits a health insurance issuer from denying a person coverage because of a preexisting condition, in both the group and individual markets. AHCA maintains this prohibition. Paul Ryan and others consider the matter closed. However, the confusing and maddening thing about Obamacare is that this prohibition, and all the other prohibitions and mandates, only work if about a million tiny Obamacare parts do their respective jobs. What does this mean for preexisting conditions? It means that the prohibition against preexisting condition exclusions is only effective if the coverage offered is actually affordable. Who cares if you have insurance plans available to you if you can't afford to pay the premiums or deductible, you know?

Which brings us to the community rating rule, the rule that gives the prohibition umph (technical term). The community rating rule prevents health insurers, in the individual and small group markets, from varying premiums within a geographic area based on health status, aka a pre-existing condition. The AHCA guts the protection by allowing states to opt out of the community rating rule. If you'd like more background on pre-existing conditions coverage and medical underwriting, THRILLING, go here. As mentioned, I have large group, employer-provider coverage, so this doesn't affect us at this very moment. But, it will definitely affect her when she has to purchase her own insurance or if something happens to me or this job. It affects many of her friends. Do you know someone with a pre-existing condition who individually purchases their insurance policy on an Obamacare exchange or has small group coverage? If the AHCA becomes law, that person won't technically be denied health insurance, but if they live in a state that opts out of the community rating rule, which, we totally do, the insurance companies can set those premium rates ever higher. A lot of us have trouble paying premiums now. What happens to a person who isn't prohibited from purchasing insurance but who cannot afford to do so? 

I make myself ill attempting to parse what exactly the purpose of this AHCA exercise even is. It doesn't lower costs for those who need it. It doesn't expand or improve coverage. It doesn't make the system more rational or give everyone more freedom, whatever that means. It seems cruel on its face. Poodle is not covered by Medicaid, but the cuts to Medicaid are deeply disturbing. Nearly 3/4 of Medicaid recipients in Texas are CHILDREN. This bill hurts Poodle. It hurts other kids. It hurts poor people. It hurts people with pre-existing conditions. What's its actual mission? Tax cuts. I'd rather my daughter and people like her receive the healthcare they need. 

If you've managed to stay with me this long, my deepest thanks and appreciation. I am also begging you to fight with us. This bill has already passed the House. A handful of senators are drafting the Senate version in closed-door meetings and reports indicate a plan to vote on it next Thursday. If you have a Democratic senator, call them and ask what they're doing to stop this bill. Encourage them to do whatever's necessary to stop it. If you live in a state with a Republican senator who is on the fence, for any reason, call them and encourage them to vote No. This would be folks in West Virginia (Capito), Maine (Collins), Texas (Cruz), Utah (Lee), Alaska (Murkowski), Kentucky (Paul), Nebraska (Sasse), Nevada (Heller), Louisiana (Cassidy), Ohio (Portman), Arizona (Flake). If you have any other Republican senator, call them and ask what the senator's position is on any of these issues and stress your opposition to the bill and the senate's handling of the bill, if applicable. I called Cornyn's office 15 times today before I got through. The polite young woman who answered the phone had zero answers for me, but she registered my concerns. I will call back tomorrow. 

Thank you. Good night. 

Hi my new eh-eh! Woof. Woof. Woof. Woof!

Long time, no talk, internet! How is Poodle, indeed. 

She's great. She's very bossy. If you ask her, she says that she's not bossy, she's funny. We tell her that it's good to be both. 

She had surgery in June. The goal of the surgery was to fix her right eyelid. It drooped. Distressingly, it still droops. This is the first surgery that didn't do what it was supposed to do. We see her surgeon in a couple of weeks. I hope that my expectations were just too extreme, that actually the surgery was a subtle success. Before the surgery, the only visceral reaction I ever had to her eye was a smile. When you're missing bone and tissue, your eye can move in more ways and convey more specific emotions than a typical eye. But as I massage her scarring every morning and evening, I try to not look too closely. Everything looks the same as it did before surgery, but I can't think that she went through it for nothing, that it's possible that some future surgery might also be for nothing, that maybe there's nothing more to do. I miss a breath. 

But, do you know who is completely unfazed by this and so in the moments when I feel like I need to step away, actually helps to bring me back? Shierry. She is loving going to school every day, even if it means extra long days where she goes to speech and physical therapy while her classmates are napping. She also has a new dog ("eh-eh" in Shierry speak). We were just supposed to foster this dumb dog, but Shierry saw her, spoke in dog to her, and named her "Woofy." To quote Dan, "I guess we have a Woofy now."

Shierry's 18-month checkup was around Christmas. She had only recently recovered from her palate repair and started seeing a new speech therapist. While she could sign as many words as were could teach her, she voiced very few words. She was noticeably agitated that she couldn't communicate to the degree she wanted and I could not teach her signs fast enough to compensate. Her pediatrician suggested that we seriously consider getting her a speech assistive device and I was looking into ASL classes.  Her speech therapist recommended that we give her new palate and more intensive therapy schedule time and re-evaluate in a few months.

Well, as of this morning, Shierry talks nonstop from the second she wakes up until she passes out. "Hi Mama. I'm awake. Baba's working. That's Mama's purple dress. This is my bed. I'm wearing my Mickey shirt. Hi Woofy. Woofy come baaaaaaack. I want to eat a waffle. Go downstairs and eat a waffle…." The other day, she was eating her snack and said, "Mama, I have cheese in my mouth." Which, while accurate…who cares, you know? Even when what she says is boring, I'm still pretty into it. Cognizant at all times that she and we are lucky for her speech. She has to work very hard and will continue to have to practice and do stretches and repetitions that others don't. With every new surgery, her delicate and weakened muscles get disrupted and there is potential for disaster, like, a fistula could happen (just thinking about that gives me the vapors). But, she has an amazing speech therapist who is On. Top. Of. It. She is a licensed speech language pathologist, so obvs, she knows all about speech. But, just as important, she knows Shierry. If something sounds off, she will know if it's just because Shierry is being a pill. Or if we need to do more practice words. Or if we need to do more stretches. Or if some more serious intervention is necessary.

After the most recent surgery, her speech was much more nasal than it had been previously and when she hit certain sounds, she was having some nasal bursts. Mostly, that seems to be working itself out. We see the speech therapist at the craniofacial clinic tomorrow for a checkup. Last time we saw her, she thought Shierry sounded a little hypo-nasal. Who knows. Last week Shierry told me that Baba had given her gummy bears when she was riding her bike. As long as she keeps being a genius narc, I'm good. 

Eyeballs

Stuck. 

It's official. Poodle is getting glasses. Poor kid, between her parents' genes and her clefts, she never stood a chance. She was always going to get glasses eventually to physically protect her eyes, but she also has a pretty awful astigmatism in her right eye that needs immediate attention. Of course. 

I learned a lot about vision at this ophthalmology appointment. One is that I will never in my life be able to spell "ophthalmology" without the assistance of spell check. But mainly that your vision is not just about your eyeballs, it's about how your brain learns to interpret input from your eyes and other senses and when it stops learning. The glasses will correct the input before it gets sent to her brain. Without the glasses, the input would travel to the brain distorted and the path from the eye to the brain would warp and warp until the brain is like, enough, your vision is completely jacked. Which, turns out, is pretty much what happened with her left eye. 

Your eye has to develop in the right place and at the right time in order to have a clear cornea. S does not have a clear cornea; lord knows the journey on which her left eye traveled during fetal development. Also, at some point her eye tissue dried out, resulting in scar tissue covering the front of her eye. So, she has a cloudy cornea that is covered in scar tissue. At two weeks old she had that ultrasound where they determined that at best, she was maybe getting a spec of light into her eye and up to her brain.Your brain develops and learns to utilize most of its visual pathways early in life. If no input comes in, your brain doesn't develop the pathways. And at some point your brain stops even trying. The brain ain't waiting around for a spec of light. So she will never be able to see out of her left eye. Ever. Scientists could develop all sorts of amazing, weird transplants and it wouldn't matter unless they could also reinvent her brain. 

We asked the doctor about any future possibility of vision in her left eye because we are in the process of considering/working out a plan for a prosthetic. We are extremely conflicted about it in general, but at least now we don't have the burden of wondering if we are the ones removing a chance for her to see out of that eye by replacing it with a prosthetic. 

However, I was still emotional when the doctor told she was unequivocally forever blind in her left eye, unless science and reality change. Not because it matters in any actual way, Poodle's gonna poodle. I'm just too sentimental of a person and I don't really like hope being shut down. I was also simultaneously processing that she will now always be wearing glasses. Which, again, doesn't matter, she has little buddies who wear glasses, and it will of course be good for her. But, I don't know, baby glasses take up a lot of face real estate and are not subtle, and it felt a little like I was losing sight of another face. 

Oh well, it's happening. The astigmatism and depth perception issues are definitely contributing to her overall balance problems, so I look forward to seeing the effects of proper vision. 

Bonus ankle band update: we took her in to see the pediatrician on Friday. She called the ortho team at Dell. They said that it's only their thing if the foot is turning inwards, otherwise it goes to plastics. Lucky for us, we already have a plastic surgeon. So, we're going to see him at the beginning of March. We're considering having a PT eval done on her in the meantime. I might wait to see how the glasses affect the walking, but I think having a PT report would help the surgeon know what he's dealing with. We'll see. 

Oh Wow

Poodling. 

There are three main things going on right now: her ankle, her speech, and her eyes. Wait...ankle? Yes. Let's go back.

The fetal diagnosticians' best guess as to what caused the clefts was amniotic band syndrome. I say that like it's a known thing, but, as far as I can tell, it's still a fairly mysterious diagnosis. This is the definition, lifted from Texas Children's Fetal Center, who took AMAZING care of us during the couple of weeks spent awaiting a diagnosis, I can't even get into it without crying. Anyway:

“Amniotic band syndrome (ABS) is a rare congenital disorder that results when the amnion, part of the fluid-filled sac around the baby, is damaged. This produces bands, or fibers, that can wrap around or trap parts of the fetus, decreasing blood flow to those areas. Hands and fingers are the most common body parts to be affected by ABS, but it also may damage the face, legs or toes.”

According to one of our doctors, these bands are sticky, lethally sticky, sometimes cutting off limbs and appendages. With a cleft as severe as Shierry's left cleft, those effing bands probably latched onto her little fetal face like an evil octopus and stole bone and tissue from her. That's pretty much what he said. Except without most of those words. However, never in all of our many, many sonogram or MRI images did either doctor ever see any bands, and as I understood them, they usually do see them floating around in there. So, he couldn't say for sure that is was ABS, but all of our genetic screening came back clean, I was in good health, so ABS was his best guess. I never thought to ask at what point this attack may have occurred, but from googling, it doesn't seem like there's a definitive answer? 

So, she's born, and she has that bonus cleft on the right side. She also has a little ring around her right ankle. My dad was the first one who noticed it. I initially thought it was just a little chub roll. Except, she wasn't a chubby baby. She was tiny, she had no rolls anywhere. So maybe ole Gramps was right. We knew ABS usually affected limbs and what not. Could the same monsters that grabbed her face also have pinched her ankle?

You can see the banding there on her ankle. 

During our early craniofacial clinic visits, the nurse coordinator would often bring in a wide-range of random pediatric specialists. Both to help us, but also, I think, because Poodle was a bit of a curiosity. Like, seriously, who is this baby, why does she have all these clefts,  and what is there even to do about it. One of these random dudes was a geneticist.

Now, internet, I am hear to tell you that every mother of a special needs child has at some point been blamed by someone for causing  the problem. This is that time I was blamed. There's something in my chart about a certain medication I had taken and the geneticist asked me how long and when I had taken it, but I didn't recognize the name of the medication. I turned to Dan panicked, and froze, I didn't know how to answer him. Was that it? Had I carelessly taken some medication and caused all of this? But Dan was calm, and remembered that the only real medication I had taken the past couple of years was a muscle relaxant for the torn labrums in my hips and he asked the doctor if the drug on the chart was a muscle relaxant. Yes it was, and I had stopped taking it months before I even got pregnant, so that was out. Whew. He then asked if I regularly took prenatal vitamins. I thought about it, and the only days I didn't take them was when I was too busy puking to consume anything. So I answered, "mostly." This, this he was interested in. He said, "mostly? Why do people answer like that. It's either yes or no." I was a bit aghast by his response, and he continued that often clefts are caused by a lack of folic acid. Well, if that's the case, I thought, fuck this guy, because I ate so many fruits and vegetables when I was pregnant, a few days of missing a prenatal vitamin and I was still consuming, on the whole, like, A LOT of folic acid. So, at this point, I was mad,. He was rude and also wrong, Dan and I told him that the other doctors thought it could be ABS. He was basically like, that's cute. We showed him Shierry's right foot. He was like, uhhhhhhhhhhhhh, yep, could definitely be ABS, byeeeeee. He couldn't tell us anything about ABS that we didn't already know, so he left. 

At this point, in our minds, this confirmed it. ABS caused both her clefts and the band around her right ankle. What did this mean for her foot and her development? Who knows. But we knew that a lot of kids loose feet and hands to ABS, so we started calling her right foot her little "miracle foot." We alerted her pediatrician. She told us that she didn't have much, if any, experience with this, but that we would keep an eye on it. As Poodle grew, her legs and feet seemed to be developing normally and in sync. She didn't seem to favor one side or the other. Until she started running. 

She started walking a little late in the game. She can't see out of one eye and also she toddles with abandon so she ran into furniture and walls and the dog a lot. She also fell a lot, sometimes because she tripped over something, but also sometimes, she just toppled down for no apparent reason. Through the last few months, she continued to enjoy walking but not be great at it.

The clinic at which she does speech also does PT and OT. This week, her speech therapist told me that a couple of the physical therapists were concerned about the banding on her ankle and the effect it's having on her walking. Her gait is off. It's very wide and she almost limps a little bit. The PTs think that she's hesitant to put weight on her right foot, she's very sensitive to new floor surfaces (going from tile to carpet), and that the banding could also be contributing to her awful balance. Oh, and, in their experience, kids have to get the banding released surgically or risk it rupturing. 

Shierry has surgery all the time, you'd think I wouldn't be freaked out over one more. But I was and am. I had just sort of gotten a hold on all the craniofacial and speech stuff. And now there's a whole new set of doctors and surgery and possibly therapy to deal with. And rupturing?! What does that even mean?!?! One of my friends told me about the physical medicine doctors in town and I also tracked down (it wasn't in any way difficult to do, everyone's at Dell) the local pediatric ortho team. I'm getting a plan together. I work for the legislature, it's session time aka unpredictable nutty time. I literally have no idea how this is all going to happen, but I guess it will. We have to keep that little miracle foot safe. 

We are lucky that one of my sister's good friends is a PT and that she introduced us to her clinic, at which our girl receives such wonderful care. We are lucky that my friend knows everything and is always there to point me in the right direction. We are lucky our pediatrician respects us and always gets us what we need to get her into see the best doctors. We are lucky that she is such a funny, tough, outgoing goof. Shierry woke up this morning and exclaimed,  "Oh wooooowwwww." Indeed.

This post is much too long already. Speech and eyeballs in next post.