My five-year old son punched me in the nose this morning.
He was tired and frustrated and I was trying to rush him out the door. He gets his temper from me. None of this excuses what he did, but at least I can understand.
I thought, Wow, being punched in the face is a great way to start the day. This will obviously be a shit day.
I had no idea.
That afternoon, I got a call from the doctor’s office. My culture came back.
Two weeks ago I had sex for the first time in years, since getting divorced from my son’s father.
After the sex, I got a Urinary Tract Infection. I was uncomfortable, in pain, and I couldn’t sleep. It’s been two weeks, and I still haven’t slept much.
Now I get a call from the doctor’s office, and it turns out I have herpes.
I want to die.
Instead, I am sitting at work in tears.
He says he didn’t know he had it. We used a condom, but he performed oral sex on me. That’s the only way this could have happened.
I like him. At least, I think I do. He’s sweet and nice and he’s been treating me the way I wish my ex-husband had.
Do I still?
I don’t know. I feel like I can’t know.
Tears are rolling down my cheeks.
He’s out of the state for work for the next week. He sent me a text message a bit ago. I told him to fuck off. He called me. I told him the culture came back positive, but I couldn’t say what it was positive for out loud. I’m at work. I’m embarrassed.
Forty-five years ago, I came into this world after the expected length of time with all 10 fingers and all 10 toes. New-baby plump, pink, and hollering out my displeasure at being squeezed out of my warm, comfy spot. I was the fourth child in my family; the first two girls were fine, the third did not survive past his premature birth.
The fifth, my brother, arrived some three and a half years after I did, hale and hearty.
I was born broken.
Only no one could see it: it was on the inside. Within hours it became apparent something was wrong. I was not keeping formula down, I couldn’t tolerate my mother’s breast milk, formula, formula substitutes of any kind. I did not have a single bowel movement.
Tests were run, x-rays were taken.
Turns out I had the congenital defect, “Hirschsprung’s Disease.” A major part of my intestine, to put it simply, did not have nerves and therefore didn’t work.
When I was two weeks old I had my first surgery. Between a third and a half of my large intestine was removed and a colostomy was performed. There are a multitude of photos of me with both a diaper applied the standard way and one wrapped around my tummy over the colostomy opening. My mother became very adept at doing massive amounts of laundry. This is before the era of disposable diapers.
Over the course of the next several months, I had multiple abdominal surgeries.
At the ripe old age of 7 months, it was time to put me “back together.” They operated on me once again and did a re-anastomosis. A re-anastomosis is a surgery that re-connects the remaining colon to the portion of the colon near the rectum. A re-anastomosis was performed so that the colostomy can be closed and I could begin to have regular bowel movements.
My first memory is from when I was seven months old. I have shared this memory with my mother, with whom I haven’t really talked much about things other than the above. I remember lying in a crib, my legs spread and ankles tied to the crib bars, and my wrists tied as well. I remember laying there just looking at my mother through the bars of the crib.
Not upset, not crying, just looking at her.
She’s told me that this exact moment did, in fact, take place. That I was calm and accepting of whatever happened. She explained that I had to be kept perfectly still after the re-anastomosis surgery because, back then, they had to apply clamps to the “seam” where the intestine was put back together. These clamps were inserted through the anus and could NOT be removed or dislodged or the seam would come apart. The clamps would fall off when the seam was sufficiently sealed and the tissue was not as swollen anymore.
Thankfully, this is no longer a necessary part of re-anastomosis surgery.
For a few years after this, I would continue to have follow up surgeries for scar repair, another infection and incisional hernia. And it took years – well into elementary school (which is another story) – for me to learn how to control my bowels.
I don’t know what it’s like to not have a roadmap of scars on my belly. For one surgery they even opened me from the back, putting the incision in the crack of my bum. This is just my reality.
I’ve had additional surgeries, mostly related to the massive amount of scar tissue from my surgeries as I was an infant. I ended up having a complete hysterectomy (I was only 24 years old – I’m honestly still dealing with that loss).
But I’m alive. My parents told me the doctors said it’d be a miracle if I lived past 10 years of age. They didn’t tell me this until after I was well past 10 years old… and now I’m 45. I guess it all worked.
To top it all off, I was born in southern New Mexico to a dirt farmer and his wife in the 60’s. Imagine the empty rattling when one shook the coffers. My family reached out to the March of Dimes, and they were wonderful to us. There wasn’t a children’s hospital in southern New Mexico so all these surgeries and medical care took place at the nearest one – in Denver, CO! The March of Dimes covered many costs for my mother and I to fly back and forth, the costs at the hospital and the surgeries.
Thankfully, my level of Hirschsprung’s was a fairly mild one. I was lucky. Most babies with this disease have much more severe issues and more extensive damage/surgical needs.
Some babies with Hirschsprung’s even face mortality at birth or soon after.
I’m still broken in other ways, but in this, I am a survivor.
I’m not sure if I deserve a pat on the back or a really good nap, but either way I’m proud of me.
Since last Thursday, things seem to have just started to topple over completely within my family, and I’ve managed to keep it together and make sure that not only are those who need to be okay are okay, but that I am, too.
Yes, this is going to take a hot minute or months to take care of, but I didn’t lose it and I didn’t break! I’m proud of that.
Now if I could only sleep.
*************************************
I wrote the above about 2.5 weeks ago after my youngest got another medical diagnosis and something major happened that I can’t talk about yet. (Nothing to do with medical diagnosis.)
Then, once I got done what needed to be done, it seemed like the shit show appeared with spring break for the kids. I ended up in the ER for a severe migraine that met my IIH (Idiopathic Intracranial Hypertension, which is high blood pressure on your brain, with brain and spinal fluid).
Aunt Becky’s daughter, Amelia, is the sole reason that The Band exists. Amelia, like so many of our children, was born with a birth defect called an encephalocele. She’s gone on to vigorously beat neurosurgery and the odds stacked firmly against her.
So it’s time to Band Back Together for Birth Defects. Share your stories. Tell your tales. We need to learn about the birth defects that have touched YOUR life. Let’s rock out and tell the world OUR stories.
It’s YOUR turn, The Band!
She was born in September. It was hot. And in 12 weeks, we would be speaking a new language.
My baby was just 2 days old when we learned that our “perfect” world was not to be. My whole life I dreamed of motherhood. It’s what I wanted to do, more than anything. Be a good mom. Raise a family. Teach a little person about my faith.
Rewind to my college days – I was one of those students that changed majors more than once. First I was going to be a teacher; I majored in elementary education. Then after two summers directing a summer camp, I learned that wasn’t really for me. I wasn’t a fan of the parental drama.
Then I was an accounting major. I was going to be a CPA. Then I realized I needed more and ended up an accounting minor with a business administration major in both finance and marketing.
But none of that told me what I wanted to do with my life.
All I cared about was that someday I was going to get to be a mom.
I married my high school sweetheart. We’ve been together since 1992. We got married. Got jobs and got ready to have the “perfectly perfect normal life” that we’d always planned. God has a funny sense of humor sometimes.
And then almost 3 years after we got married, 11 years after we first met, we had Natalie.
And then in 2 days, our world was rocked.
Her bilirubin level of 22 just would not go down.
Our family doctor made the decision to send her to a bigger hospital in a bigger city. He told us that his ego was small enough that he knew that she could get better care and more answers there. The ambulance drove away, and we felt terribly alone. She was whisked away to a NICU and our baptism into a world of medicine was begun.
Our baptism was truly begun with a Baptism.
Natalie was taken to a NICU in a town an hour away from where she was born.
My baby sister Bridget was taken from the same hospital to an ICU just 21 years before, after she was born. She never returned home.
I know that was on my dad’s mind.
My baby sister was born with Transposition of the Great Vessels. She lived 7 days. She was operated on by a fantastic surgeon, who just happened to be the very same surgeon who would perform our daughter Natalie’s very first surgery, a cholangiogram.
The surgeon did his fellowship at Children’s Memorial in Chicago. This same place where Natalie would someday have her liver transplant.
So many coincidences…
Our NICU surgeon made the comment to us that is the title of this entry, “We think it’s Biliary Atresia, but that’s really bad so hopefully that’s not it…”
I don’t hold a single ounce of ill will toward the man. Natalie’s case confused everyone.
She was born with a gallbladder. Albeit a shriveled, ugly, non-working gallbladder. But a gallbladder, nonetheless. And that’s just not common in Biliary Atresia.
In “classic” biliary atresia, by the time most kids are born, their bile duct structure (gallbladder included) has shriveled up and is not working. But Natalie was born 5 weeks early, and it’s a progressive disease – meaning it gets worse as time goes by.
Back to my dad. He’d seen things end badly for his child. I know he had his grandbaby’s soul in mind when he told me that we needed to baptize her.
I am a Catholic.
New babies = Baptism is second nature for me. But the reality of this was too much to bear. I’d had the story of Bridget’s birth and death memorized. I was 4 when she died. Her death is my very first memory. Her death prepared me for my future role, of that I am now certain. But in that moment, I could not face it.
Here I was 2 days after the birth of my child.
I’d had pre-eclampsia. I was induced just two days before following 35 weeks of pregnancy. I was a swollen, puffy blob, having gained 30 pounds in the last month of my pregnancy alone. My husband said that the moment I gave birth to our daughter, my blood pressure had skyrocketed to 250/204. No joke. I was given magnesium to prevent a stroke or a seizure.
My mind was so fuzzy.
I was still in shock.
I wanted my “normal” life back!
I was in denial.
This wasn’t happening.
Why was my dad suggesting that we baptize my baby? Did he think she was going to die? I dug my heels in. (At least I tried to. I could only fit my fat feet into a chewed up pair of black sandals – that my German Shepherd had gotten ahold of.)
So my dad did the good dad thing. He did the responsible thing. He overruled me.
He called our dear friend, the priest. He had been the priest at our church when Jason and I met (we met at church, have I mentioned that?). In walked my dad and the priest, through the sliding NICU doors and over to Natalie’s bassinet. I watched it in slow motion. I remember it in slow motion. I don’t even think I have any pictures of the moment that is forever etched in my mind.
At the time of Natalie’s first surgeries, we had not created a website for our kid, Facebook was not around, and MySpace was a name I called my bedroom. What I am saying is that I did not document my thoughts and feelings at that time, the way that I do now. Not that you would have wanted me to. You see, for the first 3 weeks of Natalie’s life, we lived in our home and visited our baby in the NICU.
We did not know in the beginning that Biliary Atresia would be Natalie’s final diagnosis. She began her stay in the NICU under the UV lights, like any other jaundice case. We’d work on “normal” things, like trying to get her to eat. The first day it was 15 ml every 3 hours, then 20ml, then we finally worked up to a whopping 30 ml. I was encouraged, cheered on even, to keep breastfeeding. And so I did. It gave me a purpose. A sense of control.
And when you can’t control anything, you’ll control that one thing with everything you’ve got.
I took to it like a champ. Strike that. I took to pumping like a champion dairy cow. I focused on finding ways to increase my milk production levels. I found special teas marketed themselves as “Mother’s Milk” tea. My loving husband was my biggest advocate. We’d walk down a long hall, into some section of the hospital that was no longer in use, except for the rare nursing mother pumping session. There was a room with 3 old chairs, a sink, paper towels and soap (for keeping supplies clean) and outlet. Not much more, except for some posters donated by a La Leche League USA. I’d plug my Medela Pump into the wall, take a seat on the old metal chair that was missing chunks of vinyl on the seat, and watch the milk rise in the bottles, feeling victorious as the ounces would climb higher and higher, knowing this was for my girl. The aches I felt, sitting hunched over, were worth it. This was for Natalie. This was making her stronger, this I could do.
After a few days, they did a full blood work-up. Her GGT level was around 1700 (normal is 5 – 55), letting the doctors know that something else was wrong.
They’d take her for an ultrasound. “Inconclusive.” She was born with a gallbladder, after all, and was stumping them.
After this happened 3 times, she had a HIDA scan, and then an open cholangiogram and also a biopsy, all on the day that she turned 2 weeks old.
That’s when our surgeon met with us and drew on a paper towel what we were looking at.
He had opened Natalie up, injected dye, and then tilted the table to watch the flow of the dye. And that’s when he said that he hoped that this wasn’t Biliary Atresia. He and the Pediatric GI attending to us in the NICU, both referred us to a wonderful team of doctors at Children’s Memorial in Chicago. And in the meantime, the biopsy slides were sent to Mayo clinic.
Mayo’s answer came back “Biliary Atresia.” But all other local doctors disagreed. So we got the slides back and took them with us to Children’s Memorial.
When we arrived in Chicago, it was like no place we’d ever been. There were bright colors everywhere. There were multiple waiting rooms with lively fish swimming in tanks. We were escorted to an examination room on the first floor. We thought that we must have been in the wrong place. There were no plaques or diplomas oh the wall. We met two doctors and told them we’d hear the term “breastfeeding jaundice.” The awesome doc, the head of the pediatric gastroenterology at the hospital, gave me a look like I’d just crawled out of a cave. “That is a myth. We’ve disproved it.” OK, I thought. Not going down THAT road with him. I had just had my first lecture by a genius, and I wasn’t a fan of lectures. But it let me know that we were in the right place. They knew their stuff. And above all else, they forbid me to feel guilty.
The fellow (also a doctor, so many levels of hierarchy at the hospital) took the slides and reviewed them. He asked the genius doctor to review them. Their first review said that she may have Cystic Fibrosis. They said it just did not present like “typical biliary atresia.” And so, sweat tests were done = “negative” was the answer. Genetic tests were sent away, and those took 6 weeks to get back. 6 long weeks of desperate waiting. The results came back negative.
After we had left the NICU, we waited for the other shoe to drop. We were still waiting. We waited for the bad news that we knew would come. Every inconclusive answer left me feeling more and more frantic. Genius doctor had told us that for a Kasai Procedure, the procedure to treat Biliary Atresia, to be most successful it must be done by the time the child is 12 weeks old.
The clock kept ticking.
Just a few days later we returned to Children’s and during a follow-up exam, Natalie happened to have a dirty diaper. Genius doctor took one look at it and re-diagnosed her with Biliary Atresia.
Who knew the answer was in the poop?
She was admitted and had another biopsy; it was again inconclusive. The kid is consistent. She then had another cholangiogram, this time a percutaneous type, which was then followed by a Kasai Operation on December 19 of that year. She was 11 weeks old. We’d gotten her surgery in by the time she turned 12 weeks.
Her new surgeon (also a genius), called hers a case of “correctable Biliary Atresia.” We’d learn later that things are not always what they seem and rarely are they as simple sounding as something called “correctable.” But, for the moment, we had an answer.
She got to come home on Christmas Eve.
Little would we know that within a year she would be listed for a liver transplant. I could not have guessed at this point that when she turned 17 months old that I would be giving part of my life to her.
His brain stem is deteriorating, a side effect of the chromosome abnormality he has. Twenty-nine surgeries haven’t been enough to save him, though they have bought him more time with us.
We are told that he’s the only child in the world who has his conglomeration of medical conditions (the chromosome abnormality, spina bifida, a connective tissue disorder, chiari malformation, intracranial hypertension, and another half-dozen minor diagnoses).
The amount of pain medication he receives every day is a drug-addict’s dream, is administered around the clock to keep him from experiencing pain. It is so beyond awful that I don’t have words to express my feelings. Watching him decline is the worst thing I’ve ever experienced in my life and that is saying something.
As if that isn’t enough, the two children my family adopted from Ukraine eighteen months ago have a lot more “going on” than we were told about.
My two-and-a-half-year old has Down syndrome, autism, and reactive attachment disorder. She functions at the level of a ten month old.
My four-and-a-half year old has Down syndrome, a heart defect that wasn’t repaired properly, systemic juvenile idiopathic arthritis, atlanto-axial instability, autism, tethered cord, syringomyelia, mild hearing loss, and is considered both medically complex and medically fragile.
We’ve been told repeatedly by numerous specialists, that she isn’t going to have a long life. She functions at the level of an eight month old.
Neither of the girls walks, talks, signs, eats (they’re g-tube dependent, just like my six-year old) or interacts well with people (they interact, but only on their terms).
When we adopted the girls, we knew they had Down syndrome and that the four-year old had a heart defect.
Everything else has been a big ‘ol surprise since we brought them home. Honestly, it feels like discovering new problems with our kids never ends.
We didn’t know our son had this chromosome abnormality and would die soon. If I’d known this, I wouldn’t have adopted him, or at least not when I did.
To top it all off, my marriage is falling apart. I know I should care, but I don’t have the emotional capacity to handle it. I just want him to leave me alone. I don’t want to have to deal with him on top of everything else.
I’m struggling.
I’m struggling in every sense of the word. I don’t know anyone that understand how this feels.
Yes, lots of people have a medically-fragile child.
Yes, lots of people have large families.
Yes, lots of people have multiple children with special needs.
But I don’t know any other people who have a large family with lots of kids with special needs, some who are medically fragile, with one who is terminally ill?
If there are, would someone please point me toward those people? I REALLY could use a friend, someone who’ll say, “This totally sucks!” along with me. I know people don’t know what to say to someone like me, but I still want them to say something – the silence is deafening.
Fourteen and a half years ago, our lives changed forever.
We had already suspected that something was not quite right with Anna. She had stopped nursing after a few days. I assumed that it was my fault, and since I was afraid that she was hungry, switched to formula and bottle feeding. She did well with that for a few days, but then it seemed like a challenge just to get her to drink an ounce. We called the nurses on the maternity floor where she was born and were reassured that she probably just had gas and an upset stomach because of the switch and not to worry.
I was still worried, but we had our first postnatal pediatrician appointment the next day (July 25), so I knew we would get answers then.
In the meantime, I received a phone call from someone from the state of Michigan. I’m not sure – my memories of that are vague. I remember that she told me that Anna had potentially tested positive for one of the disorders in the newborn screening test, and we should go to the hospital to get blood drawn to confirm it.
I was concerned, but still focused on our doctor’s visit.
When we went to see Dr. Simms, her pediatrician at the time, as soon as we were called back to the exam room, she greeted me, looked Anna over, then excused herself to get one of the other doctors.
I guess she wasn’t sure how I would react to what she would tell me next.
When they returned, she explained that Anna had tested positive for maple syrup urine disease (MSUD), it was very serious, and she was very sick. T
he only specialist in the state who saw patients was located at University of Michigan: we were expected down there and we needed to leave as soon as possible. I remember that her southern accent was soothing and the other doctor gently held my shoulders and guided me to a chair, but my mind was in a fog. I didn’t understand… Anna was born healthy. Both of her Apgar scores were 9. That meant we were supposed to live happily ever after.
Right?
Right???
At the time, Lance was working at Hafer Hardware, and it was lunch time. Dr. Simms allowed me to use the phone in her office to call him. I called the store, but he had just left for lunch. And of course, this was before we had cell phones. I’m sure it was only a few minutes before he finally called back, but it seemed like an eternity.
After reaching him, Anna and I headed home to pack before we headed to Ann Arbor.
On the drive to U of M I poured over my “What to Expect” book. MSUD wasn’t in there. I had heard of it, but only as an ailment of a serial killer in a crime novel I had read, not as a real disease.
When we got to the ER at U of M, Dr. Allen, a neurologist who treated patients with MSUD, and a gaggle of med students crowded into the small room. I remember that he removed Anna’s diaper and passed it around for the students to smell. He then took a swab of her earwax for them to smell. We were completely flabbergasted… what did he think he was doing?
We didn’t realize he was doing it to show the students that both smelled like they had been smothered in Aunt Jemima’s.
You see, there were signs that Anna had MSUD, but we never picked up on them.
She had a very high-pitched, shrill cry, but we joked that she’d sing opera someday. By the time she was 3 days old, she tensed her muscles so tightly she rolled herself over. We bragged that we had a wonder baby who was months ahead developmentally. Her diaper smelled sweet and syrupy.
We chalked it up to being new parents, and being so in love we thought her dirty diapers smelled good.And what we thought was fussiness with the bottle and me not being confident in nursing was actually because her brain had swelled so much that she lost the suck and swallow reflex.
By the time we were in PICU, she had an IV in her scalp because she was so dehydrated that it was the only blood vessel they could use. She had an NG tube giving her nutrition. She was hooked up to heart monitors and pulse-ox monitors. She looked so tiny and helpless there in the bed. We’d never felt so helpless as parents as we did then.
That day is one of the few times I’ve seen my husband break down and sob.
What information we were given about MSUD over the past few days was overwhelming. Most of the official definitions included two very frightening likely outcomes: mental retardation and death.
The prospect of having a child with a restricted low-protein diet was daunting as well. Would we need to become vegetarians, too? If we wanted to eat meat, would we need to hide it from her, making late night drive-thru runs to satisfy our cravings? Those fears seem so trivial now, but they were so real to us then.
Over the next two weeks, Anna got stronger.
After she got out of the PICU, she was moved to a regular room. After she regained some weight and was able to take feedings by bottle, we were able to come home.
She was sent home with the NG tube, as she was still taking about a quarter of her formula that way. Shortly after we got home from the hospital, she grabbed the NG tube and pulled it out, flinging stomach acid all over me. I should have known then that it would serve as foreshadowing for how the next thirteen years would go!
Thankfully, we were able to remove the tube after a week.
As I think back to those days, I also look ahead to what faces us.
When it comes time for her liver transplant, I know that we will face long days in the hospital full of tears, hope, fear, and prayers. The big difference will be that this time, we will be filled with hope.
Hope that although we will be entering a new chapter full of uncertainty, we will be free of the fear that MSUD has caused over the past 14 years.
