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.
When I was young, I always hated gym. I was never good at it. The running and the throwing of things was just never my bag. I was always short of breath and sometimes even came close to fainting. I always chalked it up to being a fat little kid who was grossly unathletic.
I lived for ninth grade because I only had to take a semester of gym and then it would be over forever, or at least until I got to college. I finished 9th grade and gladly went on my merry gym-free way.
Fast forward to 2007.
I had been out of high school and was in my third year of college. I began experiencing back pain, the likes of which I had never felt. I finally went to the doctor, and while the Nurse Practitioner was listening to my heart, she looked up and asked “You have a heart murmur, don’t you?”
I had never heard such a thing.
After tests, including one where they sedated me, shoved a “probe” down my throat, and looked at my heart (after which I cried for want of a cheeseburger), I was diagnosed with a regular heart murmur with Mitral Valve Regurgitation (which I described to people as “my heart pukes”) and Sub-Aortic Stenosis.
Some time later (2010-ish) my dad, who has CHD, began having Atrial Fibrilation episodes.
He eventually got in to see the doctor his brothers go to, and we loaded up to see if he could fix my dad’s bum ticker.
The next phase of the story I wrote about earlier in this post. During this time I was re-diagnosed with a condition called Hypertrophic Cardiomyopathy or HCM for short.
All of the things that I had experienced as a kid, all of the shortness of breath while running, all of the blacking out in PE episodes – they all made sense now (granted, they still don’t account for my athletic abilities or lack thereof).
After my diagnosis, I was scared. I began taking my drugs like a good little girl and prepared myself. I scheduled an implantable cardioverter-defibrillator (ICD) implantation for July, the month after my dad got his. I recovered just in time to return to work for the school year, and I am feeling better than ever.
After all of that worry and fear I have come to realize some things.
Not every 25 (almost 26) year old can say that she is battery operated.
Before any of this happened, I used to tell my students that I had a plastic heart; now I (sort of) have a metal one.
I tell people that I am part computer, or even bionic.
I make sick jokes about being shocked all the time.
My dad and I even joke about starting a doo-wop group with 2 of his brothers who have ICDs called The Pacers.
I never knew that having HCM and an ICD could be such a source of amusement. I even love to freak people out by letting them touch it under my skin. There is nothing better than the slightly horrified look on someone’s face when they touch the hard, metal rectangle on my chest.
In the end, the laughter and the jokes far outweigh the fear and uncertainty that I had before I got the surgery.
Plus, I hope that in the 6-10 years that it will take for me to need a replacement, they will have integrated an MP3 player into the device.
I guess I could say that I got married the second time because it seemed worth betting that the first time was due to *him…” so a different guy could fix that, right?
But the problem – if you want to call it a problem – is almost certainly me.
My mom said it brilliantly in her recent email to me:
I guess it’s truth out time, and I’m about to be a bad mom.
Truth – Dad wanted to hogtie you and send you to Tijuana before you married Steve*, but I talked him out of it. He was really upset, but I thought he had Steve pegged wrong.
Truth – after living with Steve for two months, I agreed with your father. I wanted to bitch-slap Steve so hard his head would fall off.
Or worse.
Truth – he lied to you about stuff (mostly little things), but I never said a word because I felt it wasn’t my place. But one of those lies cost you 5,000 dollars. You have no idea how furious I was or how much I kicked myself.
I’m sorry.
This really isn’t the time to be landing this stuff on you, but Dad and I both are feeling very responsible for our little girl getting hurt (again) when maybe if we had just opened our mouths, we could have prevented all this. Of course, to be realistic, it may not have made any difference, but these thoughts cross your mind when you’re a parent.
We both agreed when you were little that whoever married you would have to be one hell of a special kind of guy. (In Dad’s words, “God help him”). But I always pictured you either a) single, and blazing through the world in a cloud of glory or, b) married to a guy who was your equal – smart, confident, strong-willed, motivated and out to make his mark on the universe, but at home would have to know just when to push and when not. NOT easy to find!
However this turns out, we’ll always be here – doors and arms open. Remember that. And don’t worry, next time, we’ll speak our piece – welcome or not – and hogtie if necessary!
By the way, your brother wants to kick Steve’s butt for hurting his sister. That’s his way of saying he’s there for you, too. And if you want me there, just call. I can grab a flight and work be hanged! I love you very much – we all do.
Mom
The beautiful part is that they said almost the same thing after my first divorce, although she left out the part about how she always pictured me single.
That would have given me a lot of strength, I think.
I spent my whole life thinking that I was a failure if I wasn’t married – or conversely, that being able to “get guys” to want to commit to me was some kind of major success. I think having a best friend for most of my life who was openly jealous of my relationships probably didn’t help.
But, hey Mom – THIS time I was obviously very hesitant about getting married, and I ASKED you all to tell me if you had any hesitations!
And good grief, Dad, why couldn’t you grow a pair? You knew Steve best, and if you’d said it was a bad idea, wild horses couldn’t have gotten me down that aisle.
Something tells me, though, that I’m not the only person here who bought into this assumption that women simply “should” get married; that getting married is always a victory, even if your (first) husband is half-jokingly gloating that “someone needs to get [you] under control”.
(He did, and so I compensated for that the next time by marrying someone who wanted ME to be responsible for everything. And I was, but it cost me everything I wanted to do for myself!)
I feel like I’m waking up.
Men attacked me when I was a child, so I spent all of my teens obsessed with them, but avoiding any actual contact with them; then I got married as soon as I could; then, divorced and terrified of single motherhood, I got married again as soon as I could; and now here I am fighting my way free again.
It’s been a day and a half since he moved out (temporarily, because things turned violent, though that wasn’t the pattern or anything – but can I add that having the ability to leave immediately if someone breaks that rule with me is something it turns out I REALLY value?), and I’m not in any way looking forward to the next steps, but I do feel like I can see a clear path for the first time in a long while.
Thanks, Mom.
You may have been a little (lot) late, but that helped a lot!
(And you can bet your hiney that when MY little girl wants to get married, I’m making her a laundry list of every reason in the world I can think of not to – if she still does, great, I’ll support her; but she deserves to know what I really think. I guess sometimes the hardest lessons you teach your kids are the ones where you show them how not to do things!)
I can only write this from my perspective, of course. I can’t tell you what my family goes through. I don’t know what my friends experience. I could guess, but that would be it: a guess. But here is what I go through, living with Borderline Personality Disorder.
First is the rage. I can literally see the switch in my head flip from peaceful to ready to explode; I only wish there was a visual clue to those around me. I fill with rage in an instant, and it just explodes out. I’m not violent with it, though that is an impulse I fight every second. My only real hope of it never getting that far is to find the right combination of medications.
From there, impulses. Everyone has basic impulses. Gut reactions. Instincts, even.
The thing about my impulses is that they can be very less than helpful: the impulse to quit a job because of a hard day; the impulse to hurt myself because of a rough week.
I am very lucky that I’m through the job-quitting phase. Every one I’ve left has been for a solid reason. But each time, it was the final straw-impulse that put me there. I’m just lucky my love of current job is stronger than my impulse for self-defense that leads to the “I quit.”
As for the impulse to hurt myself, that started right before I was in the hospital for the first time, and it ended before I got pregnant with my second baby. It lasted less than 6 months, and I don’t plan to do it again. Another impulse that isn’t worth it.
Not all impulses I have with Borderline Personality Disorder are that extreme.
Most of them are standard – not thinking before I speak or act. A lot of it can be brushed away as minor. But words and actions do hurt, and not everyone is so quick to forgive. Worse yet, years of verbal impulses can chip away what patience there is. And I see what I’m doing – I know the pain – but I’m powerless to stop it. I honestly don’t know what I’m saying until it’s out of my mouth.
I know, I know… think before you speak. I’m getting better. I wouldn’t be married otherwise. Here’s the kicker: I can usually convince myself something is harmless or can be explained to be harmless in the two seconds it takes to think before I speak. I’m not usually right, though.
I think splitting is one of the worse parts. Imagine your entire world is black or white, where black is evil and white is godly. Everything is one of the two, no half and half, and NO gray.
That’s splitting. It mostly pertains to people who have Borderline Personality Disorder, but does not have to.
My husband, Pat, has been flip-flopping between the two for years now. He can flip ten times in one day, or he can go days or months before a flip. It has a lot to do with how we are treating each other.
One minute he can be making me dinner and he is white as hell.
The next minute he used instant mac and cheese, not the regular, and he’s suddenly evil. True story. My defense? He knew I wouldn’t eat the instant shit, so why did he bother making it?
Not everyone is one or the other, but this doesn’t mean they are gray. We’ll call them transparent. I don’t think there is a better way to describe it. They are the random people in the world you come upon who leave little impact beyond the few minutes in their presence. A cashier who wasn’t bad or good, just transparent.
And my kids, we’ll call rainbow. It’s like a whole different way of thinking.
As for myself, I’m usually black or transparent.
That’s just how life with Borderline Personality Disorder works.
I have had 10 miscarriages – just saying that is hard for me
For so long I have tried to sweep it under the rug – once my number became larger then three I became numb to it all. I’m not really an emotional person, but this topic always brings up memories as if it all happened yesterday.
I have been through 10 miscarriages in 6 years.
I am 28 years old – I have been pregnant 13 times – and have 3 living children.
I can be a very private person, but I think miscarriage and infertility have enough secrecy surrounding it that I do not want to perpetuate it. The more it is talked about – the more women and families can feel supported and listened to and important – not embarrassed and ashamed like I am struggling to not feel. I am opening up the door to talk about it – so here is my long story:
My first two miscarriages were kind of a blur to me. We were not trying to get pregnant and basically found out we were expecting the same time we realized we were miscarrying. I had always heard that having one miscarriage was ‘normal’ and so I honestly didn’t put too much thought into it. They were still very painful and devastating to me but I thought once we were actively trying everything would be OK – that no one would have more then 2.
My husband and I decided to start trying for a family and we actively began trying to conceive using basal body temping as a guide. We became pregnant again in November 2004 after the first month of trying. I was about 6 weeks pregnant just around Christmas when I miscarried (#3). This time it hit me – hard. I mean I have never heard of someone who has had 3 miscarriages ever – let alone in a row.
Basal Body Temperature Chart using Fertility Friend
I began feverishly doing my research.
With my basal body charts I had noticed that my luteal phase was under 10 days (according to research the shortest it should be for a successful pregnancy) so I began to take vitamins B6 and B12 to lengthen it. I went to the doctor and his thought was that my progesterone was low and that is why I was not able to hold on to the pregnancy past 6 weeks. So a new plan evolved. I would stay on the vitamins and go on a progesterone supplement the moment I found out I was pregnant. This made the basal body temping so important – I needed to know the exact date.
We began another month of trying to conceive (TTC). Thermometer in hand and a plan in mind we became pregnant again in June and I was on the progesterone medication. The plan was to stay on until 12 weeks pregnant and then to slowly wean myself off. When 12 weeks came along we lowered the dose of progesterone but I began to bleed so we quickly went back onto the medication. The baby was doing fine and the new plan was to wean off at 20 weeks. 20 weeks came and I was successfully weaned off with no further complications. I had my first full term baby (Big P) in December 2005 – a healthy boy.
Big P – 8lbs 1oz
My husband and I had always wanted to have our kids close in age, so we starting TTC again relatively quickly. I began the basal body temping again and got pregnant pretty quickly. When I got the positive I went to the doctor to get a prescription for the progesterone and started taking it again. I miscarried #4 shortly after 7 weeks. My doctor and I both thought it was because the progesterone was not started soon enough so I was given a prescription for the next time to begin the day I had a positive test. I got pregnant again and started the progesterone but miscarried #5 at 6 weeks 5 days and I was starting to lose hope. I went back on the vitamins and we began TTC again. Thinking back it probably would have been better to give myself a few months to heal physically and emotionally but I was determined and had the okay from my doctor.
In July 2006 we got pregnant again and everything was going smoothly. I was on the progesterone and we had an ultrasound that showed the heartbeat and the baby was growing. I was on bed rest again for the first 20 weeks and was weaned off the progesterone at 20 weeks. Everything was going smoothly. At 8 months pregnant I awoke with vertigo – fell and cracked my wrist. I was taken to the hospital and without going into too much detail I was diagnosed with possible stroke and they ran a large amount of tests and I was hospitalized.
In one of those tests they discovered I had a blood disorder called Factor V Leiden. Everything was going relatively smoothly with the pregnancy. I was having some weight issues – having only gained 10lbs and was 8 months pregnant they were checking to see if the baby was growing -which she was. I was being induced just over 2 weeks early because of the vertigo and possible stroke. Our healthy baby girl (Princess R) was born in February 2007.
Princess R – 7lbs 14oz
This is where the story starts to get a bit crazy. I had 2 more miscarriages (#6 & 7) due to failed birth control. We were not trying to have an other baby yet – however these losses were still quite painful.
In May 2007 I was diagnosed with Celiac Disease and was on a strict gluten free diet. We had wondered if that was an underlying cause contributing to the miscarriages and we’re hopeful that was the answer. I still had the constant vertigo that started in January 2007 and was seeing a neurologist for possible causes. During one of our meetings she mentioned Factor V Leiden again. That was the first time I had heard of it since back when I was pregnant with Princess R. The neurologist thought that could be the cause of my possible stroke when I was pregnant. I was sent to other specialists for that.
My husband and I were ready to expand our family again. I went off birth control in the beginning December 2007 and we began TTC again. I became pregnant the first month but lost miscarriage # 8 at just over 5 weeks. We didn’t take any breaks between that loss and trying to conceive again and we became pregnant again the next cycle at the beginning of January.
I was back on the progesterone and everything was seemingly going okay – baby was perfect. We had made it past 8 weeks of pregnancy and thought everything was going to go smoothly. We had told extended family and friends and had begun taking daily photos of my growing belly – our kids were excited.
Big P and Princess R telling the family about the growing baby
A phone call came to me a few weeks later that shattered me. The baby (Triton) that had made it to 13 weeks was “no longer viable” and he had passed away (miscarriage # 9). I was confused – I had done everything ‘right’ – I was on the progesterone, was on bed rest – everything. I was scheduled for a D&C because I did not want to deliver at home.
The OB who was going to be doing the surgery turned out to be a lifesaver to me. Another miracle that Triton brought into my life. My OB had read over my chart, talked to me for a long time about my history and pegged that I had been diagnosed with Factor V Leiden, a blood disorder that predisposes me to making blood clots.
The surgery was scheduled for April 24, 2008 and I was able to get the answer I needed. When the pathology came back it showed blood clots caught in the umbilical cord cutting off the supply to Triton. He had given me the answer and we had a new plan and a concrete diagnoses for all my losses – Factor V Leiden.
Recovering from surgery, my husband and I were not trying to conceive yet. I did become pregnant (seriously it’s like he just has to look at me to get me pregnant) the next month but miscarried again (#10) likely because I was not healed up completely from the surgery. We were both ready to start the process of adding to our family and met up with my OB again.
The new plan – because Factor V Leiden predisposes me to throwing blood clots normally and any pregnant woman’s risk of blood clots increase anyway – my chances were pretty high. This is the reason for my miscarriages, my possible stroke at 8 months pregnant – but luckily there was something we could do. I was still going to be on the progesterone for 20 weeks because I did have an issue with low progesterone – it was just not the whole story.
I continued with the basal body temping and this time added low dose aspirin (it’s a blood thinner). Once I got that positive pregnancy test – I went on the progesterone and was put on another medication called Fragmin. This medication is a needle that I inject into my lower abdomen – it is a blood thinner that is safe to take while pregnant. This medication was designed to thin my blood enough to stop me from making clots and putting me and baby at risk for miscarriage or still birth.
I injected myself with this needle every day – I was covered in bruises but everything was working. It became second nature to me. Since it is not safe to go into labor while on blood thinners I was placed on bed rest at 36 weeks because I had begun to dilate. The plan was to induce me again just over 2 weeks early – I had to be off the blood thinner to deliver but could not go over 12 hours without the medication or I would risk another stroke. So, the safest thing to do was a planned early induction.
In February 2009 our third full term baby (Baby E) was born perfect and healthy. I was put back on the Fragmin blood thinners and had to continue giving myself the injections for 8 weeks postpartum.
Baby E – 7lbs 13oz
Now, if you are still with me – thank you. It is hard to condense this story into a few paragraphs. I don’t really have a ‘moral’ or ‘message’ to this story except this is my story. It has been a very difficult and extremely painful journey.
It has taken me a long time (and I am still working on it) to accept what has happened and to begin to digest it all.
