Tag: Birth Defects

We Finally Get A Diagnosis

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Previously on “Momma Swears A Lot”:  Upon learning of Baby Girl’s lack of auditory nerves, genetics got involved again to hopefully find out why they never formed. Since her early birth, doctors have been trying to determine whether her various conditions are due to errors in her DNA, or consequences of prematurity.  Prior to the Craniofacial panel meeting, I spoke on the phone with our geneticist. After reviewing Baby Girl’s medical record, the geneticist believed she may have a condition called 3C syndrome. She had blood taken and we waited. Three weeks later the results were back, and like most tests done on Baby Girl, the results were inconclusive:

“The testing we ordered looked at a panel of of 16 genes: AHI1, ARL13B, B9D1, CC2D2A, CEP290, INPP5E, KIAA0196, MKS1, NPHP1, OFD1, RPGRIP1L, TCTN1, TCTN2, TMEM216, TMEM67, TTC21B. The ones most associated with 3C syndrome are KIAA0196 and CC2D2A.
We received the testing result and it was negative – No sequence abnormality was detected. In other words – no spelling error was detected in any of these genes.”

Her geneticist was surprised and the decision was made to do an entire genetic testing of Baby Girl. Four months, and 20,000+ genes later, we had an answer.

♦

Four months prior we entered our geneticists office to discuss what full genetic testing would entail.  Along with Baby Girl’s blood, they would also need samples from each of us, her parents. They would not be doing genetic testing on her father and I but rather using our sample for comparison to track the line of inheritance if an error was found in her sample.  Like asking a fortune teller how you’re going to die, we signed form after form agreeing to wanting to know whatever the results may be. We also had to decide whether we wanted to know any secondary results that may be found, such as if she carried abnormal BRCA1 or BRCA2 genes, the genes typically thought to be responsible for hereditary breast cancer.  This choice also included possible medical and insurance consequences of knowing such information ahead of time.  Since the initial seed was planted in my mind that she had suffered a mutation during development the thought of childhood-cancer has also sprung up. We only wanted to do this once, we wanted to know everything.

After an excruciating 4 month wait, and calling multiple times to check on the results, I finally got the call.  The call came from the geneticist that had been with us since the beginning, since the ultrasound that initially revealed her cleft lip and palate. They had an answer.  She told me that they had found a single mutation that accounted for all her conditions and they wanted us to come in to talk about the findings – in a week and a half… Ten more days of waiting.  I made the appointment but I nearly begged her to give me any morsel of information she could.

“They found the gene responsibility for a condition called CHARGE Syndrome, like you ‘charge’ your cell phone.  As you know I always advise not to search the internet but I know you probably will.”

She went on to tell me one more thing, that the cause of her genetic mutation was “heterozygous/de novo,” meaning it did not come from me or her father but rather was a new, random mutation that likely occurred just after fertilization. In other words, before I even knew I was pregnant.  This small piece of information took so much weight off my soul.  Not just that we finally had a diagnosis but that I hadn’t done this to my daughter.  I had worried that the genetic testing would again come back with no answers and she would end up in the medical books as a new, never before seen case: “Alien Mystery Baby Syndrome.” But more importantly to me was that there was nothing we could have done to cause or prevent this.

After hanging up the phone, I sat back in my dining room chair and stared out the window. I searched for how this diagnosis made me feel.  We had desperately wanted answers for so long and they had evaded us at every turn.  Now that we had a cause, a condition, with a name, and a prognosis, the pencil sketching of what our future might look like began to look more permanent.  Immediately I consciously acknowledged that I would have to let go of the thought that all her conditions were coincidental and she would one day outgrow them and be “normal.”

Initial routine pregnancy testing had said we had a very low chance of having offspring with a genetic disorder. And later when I underwent a double amniocentesis, the chromosomal results for both twins had come back normal.  Our geneticist had warned us then that it didn’t rule out genetic disorders, but in our naive minds it did.  Since her birth the common consensus was that the remedy for most of her conditions would be time.

“Once she gains weight she’ll be fine.”

“Once she gets a little older and bigger she’ll be fine.”

“Once they send her home she’ll start catching up.”

“By the age of two she’ll be caught up and be a normal kid.”

“When she finally gets past all this stuff she’ll be just like everyone else.”

The geneticist was right, I would Google “charge syndrome”, for hours in fact.  I needed to wrap my head around what it meant before I called my husband to give him the news.  He had been my rock through everything but I knew he would need me to be his on this one.

The first thing I saw was the faces of children with CHARGE syndrome. I was terrified looking at their malformed faces.  At every turn our geneticist had warned us about looking online because you are likely to find the most severe cases.  However, looking from those pictures to my girl, she didn’t look like them. She shared some of the facial features but her’s were softer, less profound.  I remember thinking how glad I was that we hadn’t received this diagnosis during pregnancy because these photos would have been even more terrifying. In Baby Girl’s case, she didn’t have every characteristic of CHARGE Syndrome, but CHARGE Syndrome explained all of her unique characteristics.

“CHARGE syndrome is a disorder that affects many areas of the body. CHARGE stands for:
Coloboma of the eye
Heart defect
Atresia choanae (also known as choanal atresia)
Retarded growth and development
Genital abnormality
Ear abnormality
The pattern of malformations varies among individuals with this disorder, and infants often have multiple life-threatening medical conditions.”
–https://ghr.nlm.nih.gov/condition/charge-syndrome

*For additional information I have included a full chart of characteristic, descriptions, frequencies, and links to pictures at the bottom of this post.

The day finally came in late October 2015 to meet with our genetic team and discuss the findings.  By then I had visited countless websites, read hours worth of articles, and started to digested her diagnosis.

When our genetic team greeted us I sensed they were trying to be very gentle with us. We showed them that was not necessary.  We commented that we must be the only parents to be happy and laughing about the news of their child having a genetic disorder.  Of all the countless tests our daughter had endured, this one finally gave us an answer, a solid spot to push off from,  a game plan. Not to mention to keep her Medi-Cal coverage we financially needed a diagnosis.  They reiterated what they had told me over the phone about her condition and went over the defining characteristics.

Coloboma of the eye: clefting in the eye, she does not have this characteristic. She has since been prescribed glasses for farsightedness, but otherwise her eyes are normal. 80-90% of “CHARGErs” (people with CHARGE Syndrome) have significant vision loss or blindness.

Heart defect: she was born with a threatening VSD which defied the doctors by closing on its own in months rather than in years, as expected. She still has a PSO heart defect – typically this hole closes at birth.  She receives bi-annual echocardiograms to monitor her condition. At this time she is stable and is not in need of invasive intervention.  Continuous oxygen is required to keep her blood properly oxygenated.

Atresia choanae (also known as choanal atresia): a blockage of the nasal passage(s), usually by abnormal bony or soft tissue.  Typically this is discovered at birth as the child is unable to inhale through their nose. She does not have this condition; however, her cleft palate has been left open for the purpose of keeping an enlarged airway.

Retarded growth and development: normal birth weight is typically seen and then followed by inadequate growth.  This is most likely due to feeding/swallowing issues in infancy, which is most likely the case with Baby Girl small stature. She is currently proportionate in weight-to-height, and her weight is now good for her age but she is markedly smaller than her twin brother. He outweighs her by 8 pounds and is 5 inches taller. It is likely she will forever be smaller than her peers due to her stunted growth in infancy.
Developmental delays are especially common as CHARGErs typically have low muscle tone (hypotonia), and multiple life threatening condition at birth requiring prolonged hospitalizations. Brain and skeletal anomalies compound these developmental delays.

Genital abnormality: visible abnormalities are more common in males, Baby Girl does not have this characteristic.  However, they may experience delays in puberty, or need hormonal therapy to initiate puberty. Kidney anomalies are also common.

Ear abnormality: anomalous ear lobes, cochlear abnormalities, balance problems, hearing loss. Kind of, yes, yes, and yes.

Since the designation of the CHARGE nomenclature, additional common characteristics have been attributed to the syndrome:
– Choanal atresia or stenosis: decreased/absent sense of smell
– Cranial nerve problems: facial palsy, hearing loss, undeveloped auditory nerve(s), swallowing problems, reflux, sinus problems
– Cleft lip and/or palate
– Dental problems
– Behavioral concerns
– Brain anomalies: Dandy-Walker Variant in Baby Girl’s case
– Increased pain threshold: potential superhero

And after the geneticists explained all of that, they gave us the Secondary Findings: None.

♦

As wonderful as finally having an answer was, please don’t underestimate our grief.  Learning that our daughter had a genetic disorder and would never be “normal” fucking sucked.  It’s terrible to have to ask if your child’s life expectancy will be shortened. (The answer being baring complication due to her existing conditions, her life expectancy is apparently normal.)  It’s fucking unfair. It is so unbelievably fucking unfair. One in every 10,000 births – not the jackpot I ever dreamed of winning.  If she has children someday the odds are 50% her offspring will have CHARGE Syndrome.

I am not a believer of “Everything Happens For A Reason.” It sucks this happened to our family, it’s fucking unfair, and it hurts everyday.  I don’t believe there is a reason for any of it.  And please please please please please PLEASE don’t try to point one out to me. There are just no words to mend some injuries.

The diagnosis changed the way I looked at her. At first it felt like a veil of expectations had been lifted. She was free to be whatever she was going to be. At first I looked at her with pity, this poor little broken baby of mine. However I think it made me love her more. It allowed me to understand her better.  I still have high expectations for her but when she actually accomplishes them it gives me an unbelievable high for days.

The prognosis for every CHARGEr is different and unforeseeable. The verdict is still out for her cognitive ability.  I don’t like to skip ahead but the twins were recently assessed as part of their high-risk status of prematurity – our healthcare provider will closely follow their development till the age of three.  Baby Boy scored average across the board, and above average in fine and gross motor skills. Proud mama bear over here! They admit their test does not accommodate for hearing loss, and therefore Baby Girl scored in the low average range – which is encouraging.  They expect that as her communication skills improve through acquisition of sign language, as well as fine and gross motor skills/strength, that this score will increase.  We will meet with a communication specialist in the near future to better assess her skills.   They will be tested again in a year, before their third birthday.

I included this information to stress that my daughter is not “retarded.”  Yes, she has a major, complex genetic disorder. Yes, she is extremely medically fragile. Yes, she has developmental delays.  No, she can’t hear you. But she is watching, and she is learning, and she is much more intelligent than most people give her credit for. As I have mentioned before, I am hyper-aware of how people explicitly and unconsciously act around her.  Most outings make me want to scream because people either won’t look at her, walk in a big circle around her, frown at her, or say something along the lines of “poor baby.” UGH!!!!  I know children (and adults for that matter)  with special-needs make most people uncomfortable, and my daughters medical conditions can make her especially scary at times, but she’s so fucking awesome too. My mommy wish as she gets older is for more people to be able to see that.  Like every child, some days they drive you crazy, push you to the breaking point of your mental and emotional sanity, but at the end of the day you couldn’t imagine loving a tiny human any more than you love them.

 

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I tried to keep the genetic information as laymen as possible. Please know, like most genetic conditions, it is extensively complex and cannot be completely, and fairly, explained in this one article.

♦

This post is dedicated to a tiny family in our lives going through a similar experience. It flipping sucks and it’s not fair.  You may feel lonely but you are not alone.  We each process this news in our own time. We love you! – G, J, A & A

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*Major Features of CHARGE Syndrome (very common in CHARGE and relatively rare in other conditions)

FEATURE INCLUDES FREQUENCY
Coloboma of the eye Coloboma (sort of like a cleft) of the iris, retina, choroid, macula or disc (not the eyelid); microphthalmos (small eye) or anophthalmos (missing eye): CAUSES VISION LOSS
Pictures		 80%-90%
Choanal atresia or stenosis The choanae are the passages that go from the back of the nose to the throat. They can be narrow (stenosis) or blocked (atresia). It can be unilateral (one-sided) or bilateral (both sides), bony or membranous.

Unilateral atresia or stenosis can be difficult to diagnose
Pictures

 50%-60%
Cranial nerve abnormality I – Missing or decreased sense of smell 90-100%
IX/X – Swallowing difficulties, aspiration  – Pictures 70%-90%
VII – Facial palsy (one side or both)  –  Pictures 40%
CHARGE outer ear Short, wide ear with little or no lobe, “snipped off” helix (outer fold), prominent antihelix (inner fold) which is discontinuous with tragus, triangular concha, decreased cartilage (floppy), often stick out, usually asymmetric –  Pictures >50%
CHARGE middle ear Malformed bones of the middle ear (ossicles): CAUSES CONDUCTIVE HEARING LOSS Common
CHARGE inner ear Malformed cochlea (Mondini defect); small or absent semicircular canals: CAUSE HEARING LOSS AND BALANCE PROBLEMS  –  Pictures 90%

Minor Characteristics of CHARGE: Significant, but more difficult to diagnose or less specific to CHARGE

FEATURE INCLUDES FREQUENCY
Heart defects Can be any type, but many are complex, such as tetralogy of Fallot 75%
Cleft lip +/- cleft palate Cleft lip with or without cleft palate, cleft palate, submucous cleft palate  –  Pictures 20%
TE fistula Esophageal atresia, Trancheo-esophageal fistula (TEF), H-shaped TEF 15-20%
Kidney abnormalities Small kidney, missing kidney, misplaced kidney, reflux 40%
Genital abnormalities Males: small penis, undescended testes
Females: small labia, small or missing uterus

Both: lack of puberty without hormone intervention

 50%
25%

90%
Growth deficiency Growth hormone deficiency 15%
Other short stature 70%
Typical CHARGE Face Square face with broad prominent forehead, arched eyebrows, large eyes, occasional ptosis (droopy lids), prominent nasal bridge with square root, thick nostrils, prominent nasal columella (between the nostrils), flat midface, small mouth, occasional small chin, larger chin with age. Facial asymmetry even without facial palsy  –  Pictures
Palm crease Hockey-stick palmar crease  –  Pictures 50%
CHARGE Behavior Perseverative behavior in younger individuals, obsessive compulsive behavior (OCD) in older individuals >50%

Other Features of CHARGE: consistent with CHARGE, possibly medically significant, but less helpful in making a diagnosis

FEATURE INCLUDES FREQUENCY
Chronic ear problems Lots of infections, fluid in the ears, PE tubes until teens 85%
Sloping shoulders Underdeveloped shoulder muscles, small or missing pectoral muscles, short neck  –  Pictures Common
Limb/skeletal Absent thumb, extra fingers, vertebral abnormalities ?
CNS abnormalities Hydrocephalus, seizures, abnormalities seen on MRI or CT Occasional
Thymus or parathyroid abnormality Small or missing thymus, decreased immune system Rare
Omphalocele Omphalocele or umbilical hernia 15%
Nipple anomalies Extra, missing or misplaced nipples Occasional
Hypotonia Low muscle tone  –  Pictures 90%
Scoliosis Usually due to low muscle tone Common

No one feature is required to make a diagnosis of CHARGE. Every feature varies from severe to absent in different children.
-http://www.chargesyndrome.org/about-charge.asp

Helmet Hair Don’t Care

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This is just a short post, kind of a bonus episode if you will, to bridge the gap up to Baby Girl’s genetic testing.

A week after we learned Baby Squirrel was Deaf, we met with her Craniofacial panel of doctors and surgeons. We saw this team for the first time while I was still pregnant, after we found out via ultrasound that she had clefting.  This team included her plastic surgeon, geneticist, ENT/Head and Neck surgeon, psychologist, social worker, speech and language pathologist, orthodontist and pediatric dentist, and maxillofacial (jaw) surgeon.  After they filed in one by one to meet with us, they held a conference to formulate a complete treatment plan.

With her latest diagnosis, audiology and speech and language pathology were stricken from her treatment plan.  She will never hear and consequently will never speak so no medical intervention is necessary, just to keep working with her Early Start Deaf and Hard of Hearing program.

At the time she was 9 months old and didn’t have any teeth yet, so the oral surgeons didn’t have much to go on.  We knew from our initial meeting that they wouldn’t have much of a role until she was older, about 7-8 years old, when her adult teeth begin coming in. To bridge a cleft in the gums, currently the procedure is to take bone from the patient’s hip and implanting it in the upper jaw, grafting tissue over it to complete the jaw. (This means she’ll most likely also have some fancy veneers.)  However, new methods are advancing so there is no way to say what the procedure will be when the time comes.

After Baby Girl’s cleft lip was repaired, while we were in the PICU recovering from complications from the surgery, her plastic surgeon would routinely come check on her.  He seemed genuinely scared by her development of septic shock and declared that he was taking her cleft palate repair off the table indefinitely.  For a typical child, cleft lips are repaired when the child is 3-4 months and at least ten pounds; palates are typically repaired at 1 year of age. When we met again months later at the Craniofacial panel, he elaborated – we now had more information. Her surgeon didn’t want to close her palate because he didn’t want to risk her getting that dangerously sick again, she didn’t need it closed for speaking, she didn’t eat orally yet, and it actually helped her by giving her a larger airway.  And as an added bonus, waiting to close a palate allows the upper jaw to continue to grow naturally, while closing it can cause thick scar tissue to stunt its growth, requiring more orthodontic procedures later on.

To back up again, while Baby Girl was still in the NICU, I started to notice that her head was getting flat on one side, along with her forehead appearing asymmetrical. The nurses were good about changing her position but one side of her crib had easier access so she tended to face that way. Once she came home I tried positioning and Tortles to correct it but she had no neck control, and her airway was so fragile that limited positions were safe for her.

Fast forward to her Craniofacial panel, I mentioned this flattening of her head to her plastic surgeon and he prescribed her a Cranial Band aka a helmet. Long before marriage and kids and reality, I used to see kids with helmets and feel embarrassed for the baby and the parents. Clearly there is something wrong with the kid.  However by the time Squirrel was 10 months old, we had already experienced so much that a helmet was the least of our worries. Although I had been aware of my own previous prejudices of children with helmets, I was still caught off guard by some assumptions people had for why she had one.  I understood when people guessed if it was for seizures or epilepsy, it does look similar. However, I had adults (plural) ask me if she had it because she was missing the top of her skull… -_-
For once this poor girl had something that wasn’t medical, strictly for aesthetic purposes – I wanted her to look more normal and not have a asymmetrically flat head!

She was measured and fitted and I ordered her a light pink helmet. The makers of the helmet tried to talk me out of it.  Squirrel would have to wear it 23 hours a day, with a one hour break for baths.  And it was summer time, they are hot and get really smelly from sweating.  She was also on the older side so they couldn’t guarantee marked improvement that would be expected with say a 6 month old. But I wanted to give it a shot, she already has so many thing about her appearance that stood out, if a helmet could cross one thing off that list I wanted to try it.  At home I ordered her big bows to attach to it. She was going to rock this thing!

Photomontage time!
During this time she also had a pH probe test, you’ll see it taped to her face, but to write about it would require including a longer backstory. If you really want to hear about it please message me.  Taking Baby Girl out in public makes me extremely anxious because it feels like everyone is staring at us. On top of a baby on oxygen, add a helmet and a pH probe down her nose and taped to her face and all eyes really are on us. Some people would stare as they walked by, some people would politely smile at us, and others would blatantly walk into our path in front of our stroller to stare at her. I wanted to scream at these people.  I felt like I was being a bad mom by not scolding these people. However I realized these people honestly don’t know any better and I needed to give them grace.  I almost went as far as tattooing a reminder on my wrist but I opted for an engraved bracelet instead. The correct answer is smile, smile if you must look. Even better: smile, say hello, and ask questions!!!  I will gladly answer any questions you have about my daughter, just don’t fucking stare.

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Bonus montage of the twins passing out in different places:

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OK, one last one of their first birthday since I don’t feel like blogging about it:

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And after just 2 months, a week and a half after their first birthday, she graduated from her helmet.
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Coming up next:  Upon learning of her lack of auditory nerves, genetics got involved again to hopefully find out why they never formed. Since her early birth, doctors have been trying to determine whether her various conditions are due to errors in her DNA, or consequences of prematurity.  Prior to the Craniofacial panel meeting, I spoke on the phone with our geneticist. After reviewing Baby Girl’s medical record, the geneticist believed she may have a condition called 3C syndrome. She had blood taken and we waited. Three weeks later the results were back, and like most tests done on Baby Girl, the results were inconclusive:

“The testing we ordered looked at a panel of of 16 genes: AHI1, ARL13B, B9D1, CC2D2A, CEP290, INPP5E, KIAA0196, MKS1, NPHP1, OFD1, RPGRIP1L, TCTN1, TCTN2, TMEM216, TMEM67, TTC21B. The ones most associated with 3C syndrome are KIAA0196 and CC2D2A.
We received the testing result and it was negative – No sequence abnormality was detected. In other words – no spelling error was detected in any of these genes.”

Her geneticist was surprised and the decision was made to do an entire genetic testing of Baby Girl. Four months, and 20,000+ genes later, we had an answer. “Next time, on Serial.”

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Home, Surgery, PICU & Death: Part 1

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This post may not be for the squeamish or hypersensitive. I have included unpleasant photographs and videos showing post-surgical incisions and extreme medical treatments. This post also includes an outsider’s account of the death of a child. That being said, the story I am going to share was profoundly traumatic for our family, leaving invisible scars that may never heal.

The day Baby Girl came home, January 14th, 2015, was wonderful and terrifying. For 4 1/2 months we had relied on her monitors to tell us exactly what was going on with her, and if something happened the nurses and doctors were right there to intervene. The nurses had told us from day one not to rely on the monitors but to watch her coloring. We quickly learned “dusty” = bad.  Nothing is more nerve racking than the moment when those monitors go off and her life, that you have seen first-hand be extremely fragile, is thrust into your hands.

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1/14/2015: First night with both babies home

Please notice the copper rod with colorful rubber bands near her head.  What you can’t see is her feeding tube hanging from it. We would jury-rig many ways to feed her over the months to come, thankfully they became more sophisticated than this.  Probably the hardest thing that we had not been prepared for was the witching hour. Or what you would all call 7:00PM. Every single night at this time Baby Squirrel would start crying and sweating uncontrollably for a solid hour.  One of us would have to sit with her and hold and lower her feeding tube as needed.  We tried everything to comfort her but eventually, for our own sanity, settled on wearing headphones till the episode passed.

They had sent us home with an apnea monitor, not a pulse oximeter monitor, meaning we couldn’t see exactly what her oxygenation level was or exactly what her heart rate was. Instead it would just beep at us incessantly if her heart rate got too high or too low or she held her breath for more than 15-20 seconds. I don’t think we ever took her monitor off her that first week.  Two days after she came home she had doctor appointments, and I remember unloading all her equipment from the car into her stroller and thinking, no baby should require all these cords, people are probably staring. That day we met with her plastic surgeon (yes, my daughter has had a plastic surgeon since before she was born, NBD) and he cleared her for the surgery we had been waiting for for nearly 8 months, since I was 4 months pregnant, the surgery to repair her cleft lip and nose. (Insert Clueless quote here.)  She had been the first baby in her NICU to have a special tape placed across her cleft. (The name of the tape is alluding me now as I celebratorily spiked it into the trashcan when she no longer needed it.) Starting shortly after she was born the special tape was placed on one cheek and stretched across and adhered to her other cheek.  The purpose of this tape was to pull her gums together, closing the large opening in her gums due to her cleft palate, and to stretch the lip skin so there would not be excess tension on the incision once her cleft lip was repaired.

Five days later we were back to meet with her anesthesiologist for the surgery and an Ears, Nose, and Throat specialist to discuss placing tubes in her ears during the same surgery. I will explain later the reason for the tubes. While we were in the waiting room waiting for this appointment, Baby Squirrel had a giant blow out! (That’s a poop explosion for any non-parents.) She hated being dirty so she was losing her mind. Her monitor starts shrieking out in the quiet waiting room because her heart rate was over 220! Thats really high, especially for something like a dirty diaper. For the next few weeks, and year really, we would spend an average of 3 whole days a week at the hospital seeing her various specialists (pulmonologist, gastrointestinologist, physical therapist, occupational therapist, ENT, neurology, though ironically rarely her pediatrician because her care was so complex.)

When we were home we tried to make up for missed time. There was already a significant developmental gap between the twins but we tried to put them together as much as possible.  From spending more than 4 months on her back in a bed her head had become asymmetrically flattened on one side, but most discouraging was she hated to be held.  She would throw such a fit she would have respiratory distress if you tried to hold or cuddle with her. I worried perhaps she was autistic but through speaking with other long term NICU parents, I established it’s fairly common among infants that begin their lives not healthy enough to be held. Even at home, her dislike for being held, plus her great need for growth and therefore strict feeding schedule, had her propped up in her crib for many hours of the day yet again.

As you can probably imagine, it was nearly impossible to bond with her under these circumstances. Since they were born we dreamed of bringing them both home and bonding as a family.  However, when Baby Girl came home she became more of my patient than my daughter.  Not only did she not like to be held, but due to her cleft lip she was never able to breastfeed. So my daughter and I never got to have that magical skin-to-skin breastfeeding induced oxytocin love fest. At nearly 5 months she still didn’t smile so there was no positive feedback.  I loved her in the sense that she was my daughter and my flesh and blood, and when bad things happened to her my heart would physically ache, but it came no where close to the love I felt in my bones for her brother. Judge if you must, I don’t like to admit that. It left me feeling extremely guilty and unfair.

1/18/2015 – Stuffed babies love at first sight
1/17/2015 – Sharing the Pack-n-Play
1/18/2015: Both my babies at once, this happens much, much less than you may think
1/24/2015: Watching cartoons together
1/15/2015 – Both babies in their joint bassinet where they belong, her overnight continuous feeding line can be seen
1/25/2015
2/3/2015 – The twins are 5 months!
The reality of twins…

Before either of my children were discharged from the NICU hearing and vision tests were performed as prematurity can greatly effect both areas. Both of their visions were fine, however Baby Girl’s hearing test continually came back inconclusive, “Refer” to be specific.  We weren’t too concerned because we knew she loved her music and always responded to her daddy’s voice.  Upon her discharge from the NICU she was referred to an audiologist to have her hearing rechecked.  This appointment did not go well as it required me to hold her, and hold her still – it wasn’t happening.  The audiologists did not believe the results were accurate so we rescheduled our appointment to repeat the test and if it still was inconclusive a sedated test was an option.

A week later was Baby Girl’s cleft lip repair surgery.

♦

Before I start writing any of my blog posts I do research.  I go through all the photos and videos on my phone from the target time and read through anything I may have written in my calendar or elsewhere. As I mentioned in the beginning, this time period was particularly traumatic, and this is apparent to me because a lot of the photos and videos I have little to no memory of. In addition, many days I have no documentation to refresh my memory at all during this time because there were days I could barely bring myself to look at my daughter.

♦

Tuesday, February 10th, 2015, at 6:30am we arrived at the hospital and Baby Girl was admitted for surgery.  We were able to go back with her to get her changed into a gown and wait for the surgeon to come speak with us.  We laid the twins together and they fell asleep. Still to this day she is calmed by his presence.

2/10/2015 – Surgery day, he keeps her calm
2/10/2015 – Surgery Day

When the time came I took one last photo of her being wheeled to surgery because there was a little voice in the back of my head wondering if this would be the last time I’d see her alive. All surgeries come with the possibility of complications, no matter how routine,  plus I am a mother, the mortality of my children is never far from my mind.

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2/10/2015 – Baby Girl being wheeled away for surgery

I can’t recall exactly how long her surgery took but it was quite a few hours.  Eventually the plastic surgeon came out and reassured us that everything had gone smoothly and he was really happy with the outcome. We were then directed to Recovery where we would be reunited with our daughter.

2/10/2015 – immediately following surgery, in recovery

Saddest lil’ face you’d ever saw, right?!
But it was also the most amazing! The surgery had completely reshaped her face.

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2/10/2015: Her amazing Before and After

Once she was cleared from Recovery we were transferred to the PICU.  This was our first time stepping through the big mysterious metal doors.  We arrived with our daughter the first time but anytime we had to reenter we had to ring a door bell and announce to the nurse behind the camera who we were and who we were visiting.  I was first struck by how much smaller it was than the NICU.  There was a centralized nurses/Doctor station on the right surrounded by 7 private rooms with sliding glass doors on the left and back.

She was in a lot of pain but plastics came to check on her and reassured us again that the surgery went perfectly and she was given Hydrocodone/Hycet for pain.  As our luck would have it the local hockey team the San Jose Sharks were visiting all the children in the pediatric wing that day, something they do often.

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2/10/2015: The San Jose Sharks visit all the children in the Pediatric wing

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2/10/2015: SJ Sharks swag

By that night the doctors felt she was recovering well and transferred her out of the PICU and out to the Pediatric floor. In the PICU the nurses have 1-2 patients. In general Pediatrics the nurses have…I have no idea because we rarely saw a nurse after her move.  So of course that’s when the fun started.  Baby Girl started screaming and crying and didn’t stop.  Her ear-splitting monitors wouldn’t stop going off as her heart rate remained over 220.  When the nurse would come to check on her there was nothing they could do for her unless it was time for more pain medication. We resolved to taking turns pacing the halls as a mental health break. Eventually I took Baby Boy home for the night and my husband stayed with her.  I would return in the morning to bring her home.

When I arrived in the morning Baby Girl looked like hell and her coloring didn’t look right.

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2/11/2015 – waiting for discharge papers, she didn’t look right to me

My husband had left for work by the time I got there but mentioned the crying had lasted all night.  As her discharge paperwork was being compiled, her nurse and I simultaneously noticed her  MIC-KEY button had come out! The balloon filled with water that holds it in place had ruptured. The feeding tube was still pretty new to me, I had never had to change one on my own.  Down the line I would become a super badass g-tube changing pro, but till then I panicked.  I knew this commonly happens but I also knew that if the stoma (the hole in the skin through which the MIC-KEY button enters the stomach) were to close she would require another surgery to replace it. We were able to put it back through the stoma and tape it in place until I could get my brother-in-law to go by my house and bring us a replacement button.  Hours later I finally had my crying miserable baby home.

For the rest of the day she cried and cried and cried. Then magically the next day she seemed OK. She stopped crying and showed interest in her toys and playing. It was a good day.  We still had to keep the arm restraints on her so she wouldn’t touch her lip or nose.  Unfortunately, this was just the calm before the storm.

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2/12/2015 – Arm restraints so she wouldn’t touch her lip and nose. This was actually a good day, she had energy to play and seemed to be feeling better

That night the witching hour struck and struck hard. Except it wasn’t just an hour, it lasted all night.  We kept calling the surgical department and advice nurses and they kept telling us what she was experiencing was normal after a surgery like hers and to continue with the pain medication. She had only been home for 3 weeks before her surgery so we were still learning her but I had a strong feeling something really wasn’t OK. We didn’t have a pulse oximeter and strangely enough they hadn’t taught us how to listen to her lungs.  That’s right, our child had chronic lung disease and pulmonary hypertension and we had not been taught how to listen to her lungs, we didn’t even have a stethoscope at the time.  At that time we were completely unprepared for recognizing respiratory distress.  Paired with the advice nurses telling us that this was normal.

By 3:00AM my husband and I were at our wits end.  Baby Girl was clammy, had a mild fever of 101, eyes rolling around in her head – not a pretty sight.  At the time her oxygen prescription was 1/8 of a liter of 100% oxygen. That’s barely anything. But with our inexperience, and lack of a proper monitor, we didn’t know if she needed more. We called the advise nurse again and said straight up, “something isn’t right, this isn’t just pain.” We were given an appointment with her pediatrician first thing that morning.

For the ride there I turned her oxygen up to 1/4 liter, still not much but double her usual requirements.  She actually appeared more peaceful on the ride but was still lethargic and clammy.  While the nurse was taking her vitals like temperature and oxygenation level, under the florescent light of the pediatric clinic she looked extremely pale. Her oxygenation level was 75%…because she has pulmonary hypertension her oxygenation level really shouldn’t be below 95% for any extended period of time.

Once the nurse put us in our room I honestly think she went and grabbed the pediatrician away from another patient because she came in only a moment later. She took one look at her and in her best calm doctor voice, the one they use when you know they are actually really concerned, she asked, “how high does the oxygen flow go on her tank?” “Four,” I replied. “Go ahead and turn her up to four.”  She asked me more questions and before I knew it she was asking a nurse to bring in a wheelchair STAT. (“STAT” is never good. “STAT” was used when the ultrasound found Baby Girl’s heart rate was under 80 leading to their emergent birth. I don’t like “STAT”.)  It literally happened so fast. A wheelchair was brought in, they threw me in the wheelchair, Baby Squirrel in my arms, and that nurse RAN me across the campus to the Emergency Department where they had a room waiting in the super emergency section.  The rooms they leave empty unless their use is truly warranted. (The toothless nipple-popping presumed crack whore complaining about all-encompassing pain while continually asking for more food, whom which we would see on a future ER visit would not get one of these rooms.)  A flood of nurses, doctors, and specialists followed.

While they began looking her over and trying to find veins to start an IV, I tried to reach my husband and tell him to get there. He was over the hill golfing (please don’t judge the guy, he thought, like the advice nurses, that she was just in pain from surgery and it would subside over time.)  I can’t remember if I reached him right away or if we played phone tag, my adrenaline was seriously pumping at this time. Thanks to good ol’ Zoloft I had my head together though.  I wasn’t crying and I was able to converse with the doctors and answer their questions, all using that calm facade voice.  Baby Girl had tiny veins and had always been a hard stick.  I hated to see them pin her down while they fished for veins but it wasn’t the first time.

A coordinator called me out into the hallway to sign standard forms while they continued trying to get an IV started. While trying to call my husband again I saw a nurse with what looked like a power drill and thought in the back of my mind that that was odd but nothing more. As I was putting my phone back in my back pocket a nurse came up behind me and grabbed my shoulders softly and told me that they were going to drill into my daughters leg bones to try to start an IV that way. What the serious fuck?! At that instant I saw through the open glass sliding door the drill touch down on her right leg. I turned away and collapsed to the floor. I exploded into wails with my head on my knees and my fingers clasped behind my head.  The nurses scooped me up and sat me in a chair.  I would later find out the IO wasn’t successful the first time and they had to try again on her other leg.

I don’t remember much after that.
Baby Girl and I were moved back up to the PICU.
My husband arrived 20 minutes later from 30 minutes away.

 

To be continued…

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I Got The PTPPNICUDSD Blues!

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Post Traumatic Postpartum Neonatal Intensive Care Unit Depression Stress Disorder

OK, maybe I made that up.  But I wasn’t just effected by the twins emergency 9 week early arrival, or their 4.5 months in the NICU, or the wild hormonal changes swirling through my brain and body after pregnancy; they all played a nasty game together.

Few people know many details about our 133 day stay in the NICU because it got to a point where it was too painful to continually tell people our daughter wasn’t any better, or she was continually getting worse, or she had received another dire diagnosis. We appreciated everyone caring enough to ask but they most likely received a lie in response. “She’s doing alright,” “she’s still technically premature so she still has a long way to go,” “her lungs just need to get stronger and she’ll be home,” “eh, she’s getting there.”  I rarely to never shared photos of our daughter during this time because, quite frankly, she looked like shit.  I’ll do my best to not make this post horribly depressing but the truth is I am still profoundly effected by our experiences over the last year and a half. And I still struggle with depression every single day.

When the twins were born on September 3rd, 2014, 9 weeks early by emergency C-section, I didn’t get to see them for the first time till the next day.  They were still an abstract idea to me; you know you’re pregnant but you’ve never seen them outside your body before so you don’t really know who you are missing. I even still felt their fantom kicks. I was in so much pain I was switched to Percocet and kept in a sleepy haze.  Most mothers will probably disagree but I loved having a catheter in because it meant I didn’t have to get out of bed.  (I had to pee and then I didn’t anymore. It was fantastic.) My husband had seen our children when he escorted them to the NICU where he fought with the nurses and doctors to get them placed next to each other rather than in two completely separate areas.

While I was in recovery he spent time with the twins and took pictures but he refused to show me until I was able to see them in person. All I knew for almost 24 hours was that my son had dark hair like his daddy and my daughter had strawberry blonde hair like me. And that her face was completely open on her right side due to her cleft lip and palate. We knew she would have the clefts before she was born but we were never able to see if her lip was open all the way up through her nose or not.  Every 2-4 weeks I was given a 3D ultrasound to try to see her face (moms of typical children are probably envious of that), but she always either had her face up against the membrane that separated her from her brother, which made the image look like she had a veil over her face, or her brother’s butt was in the way.

The day after their birth it was my mission to get out of bed and into the wheelchair so I could go see my babies. Holy fuck! You really don’t realize how every move you make uses your core until your core has been sliced open and stapled back together.  It took two people and a lot of crying but I made it into the wheelchair and over to the NICU.  We had always known that with twins it was almost a guarantee that one or both would spend at least some time in the NICU; however, I was extremely naive of how that would actually effect us.

Their first 4 days in the NICU are kind of a blur for me. I was on heavy painkillers and spent my time there fighting the nods while peering through the little door/windows in the side of their isolettes, cupping Baby Girl’s head in my hands, or sitting next to Baby Boy’s isolette holding his tiny fingers sobbing because every breath he took looked like his ribs collapsed to his spine. It would be 5 days before I was able to hold my son for the first time, and 10 days before I could hold my daughter.  You may notice I talk about my son much less than I do my daughter. Once my son’s lungs opened up he was able to breathe on his own.  For the rest of his time in the NICU as long as we let him sleep on his tummy he was happy.

Because they were 9 weeks premature the doctors expected them to remain in the NICU for 4-6 weeks.  Because we knew we were in for the long haul we showered the NICU nurses with Starbucks coffee and giant gourmet cake pops made by a close friend who was a pastry chef.  My charming husband made sure all the nurses and doctors knew us all by name so they would consciously, or at least subconsciously, take the best care of our babies when we couldn’t be there.

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The massive sunflowers hide that these cake pops were huge!

I remained in the hospital for 5 days, before being discharged. I got my wheelchair ride out but my arms were empty.  I sobbed all the way home leaving our babies behind.  I don’t think that crying ever really stopped after that.  I woke up crying, I pumped crying, I fell asleep crying. I relied on my husband for everything. Physical support, emotional support, and he carried it all like the Titan Atlas.

Pumping, pumping, pumping, pumping, pumping, pumping, pumping, pumping. Fuuucckkkk pumping.  Pumping 8-12 times a day, every 2 hours, roughly a 40 minute routine. Do the math with me here, that’s nearly a minimum of 6 hours a day. But not just during the day – around the clock. And the 2 hours starts when you start pumping, so really you only have an hour and change between pumps. I killed myself for that milk and it always made me feel like a complete failure. I could never produce enough for both.  When I would take that sweet liquid gold into the NICU the nurse taking it would always say, “thats all? Are you pumping at least 8 times a day?” I wanted to be a breastfeeding mother, my goal was at least a year. I knew it would be hard but it’s fucking hard!  I tried everything. Supplements, massage, “Power Pumping”, I was spending that 6+ hours a day online researching how to up my supply. My daughter would never be able to breastfeed due to her cleft lip & palate, and my son would end up taking 8 months to figure out milk came out of those things! 8 months! ANYONE that says “don’t cry over spilled milk” has never been a lactating mother!

For the first week home, we would get up and go to the NICU, spend all day there and then come home and do it all over again the next day. The NICU at our hospital was a level III NICU but the babies did not have private rooms. There were 4 main rooms called Pods: Pod A, B, C, and D, and each pod had 6 isolettes/cribs. You could pull a curtain for “privacy.”  The nurses had shift change every day at 7am, 11am, 3pm, 7pm, and 11pm.  At those times all parents had to leave the NICU for 30 minutes before they could come back in; you couldn’t stay with your child around the clock.

After a week I was healed well enough to care for myself and get around on my own. My husband returned to work so he could save his paternity leave for when the twins came home.  I was so scared to be alone. I had become completely dependent on him.  I was constantly crying and sad and he was constantly reassuring me and trying to ease my fears. All the stress surrounding babies that could have torn us apart had brought us exponentially closer.

I delved into a routine in an attempt to keep my sanity. Every morning when my husband left for work I would get up, pump, then head to the hospital to deliver milk. I would stay for 9am rounds where the doctors and staff would come around and talk about all the scary stuff wrong with our children, particularly our daughter. Blood clots in the brain, hydrocephalus, inability to feed, failure to thrive, heart murmurs.  I would stay till about 11:30am then head home. I would drive back to the hospital again at 4:30pm to meet my husband and we would stay till shift change at 7pm then head home and start dinner.

During the 2nd week our daughter stabilized enough to be held.  I was terrified. She had so many tubes and wires and cords attached to her and she was just so tiny.  Looking back at photos I can now see how mottled her skin was and why the doctors were so concerned about her coloring. Shortly after she was put in my arms she refluxed and threw up all over me. The bile itself I didn’t mind. It was the vision of it coming out of her mouth and nose because of her clefts. It gushed out of her entire face and then she would choke and turn blue and the monitors would start screaming. Those goddamn monitors. Constantly going off. Today I can read those monitors upside down and backwards, but then I didn’t know what they meant and the sound always scared the hell out of me. Every time one would go off I would jump.  And because the pod was open I could hear all the other babies monitors, and sometimes I couldn’t discern immediately if something was wrong with my babies or someone else’s.  Most premature babies have a feeding tube in their nose down to their stomaches.  When my daughter’s reflux could not be managed and continued to be threatening her doctors opted to move her feeding tube past her stomach and into her intestine with an x-ray guided weighted tube with the goal of reducing the amount of content in her stomach. Like most things with our daughter we would learn, it only helped a little.  I just kept telling myself that someday I’ll get to hear her little voice tell me she’s OK. I’ll explain in a later post why that will never happen.IMG_5763

One doctor noticed that I would always cry when we spoke after rounds, or any time for that matter. She always asked if I was OK and offered to have a social worker come talk with me.  I always said I was fine, I was just scared, and I didn’t want to talk. After a male doctor (let’s call him Dr. K) bluntly told us our daughter had a hole in her heart and would probably require open heart surgery, my husband had privately requested that the doctors speak with more tact around me. However, one day we were told Baby Girl had fluid pooling around her brain causing added pressure.  I remember our primary nurse hugging me for the first time and that I felt things must be much worse than they are letting on in front of me. I asked to speak to the doctor that always checked on me and told her I was ready to talk to someone. I was set up with appointments to speak with a therapist and a psychiatrist.  I didn’t want medication but I was instructed to keep the appointment anyhow. I don’t even like taking Motrin for headaches till I’ve at least tried to drink it away with water.

Three weeks doesn’t sound like a long time now but by their third week I was losing it.  I wasn’t sure if my daughter was going to ever leave the hospital alive, I was only sleeping and hour or 2 tops at a time, I felt nauseous all the time, postpartum morning sickness as I began to call it, and mentally I was a complete wreck. Imagine every negative emotion coursing through you all at the same time constantly for over 20 days straight. Sadness, fear, anxiety, worry, exhaustion, disappointment, regret, loss, inadequacy. And I don’t mean like I was bummed out or feeling blue. My chest ached with deep terrifying sadness and fear. I felt completely useless to my children.  I regretted having them at all.  I showed up to the NICU multiple times a day but I didn’t want to be there.  I pumped nonstop for them but I fucking hated myself when 30 minutes later I only had an ounce or two to take to them.  When doctors would talk to me I could only nod and fight back the tears, I couldn’t contribute to the conversation or to the care of my children. Every time those motherfucking monitors would go off my heart would race and I would go into fight or flight mode. And one day I snapped.  Both of my children’s monitors kept going off, back and forth, loud piercing beeps in the mandated quiet of the NICU. I wanted to get up and run out of there but instead I grabbed my phone and penned a desperate email to my OB.

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My desperate plea to my OB

My OB headed a special prenatal group we attended and we got to know each other very well, much more so than just occasional check ups. So she wasn’t just passing out pills just because I asked.  She prescribed me Zoloft and I picked it up that night. Zoloft is in no way a quick fix. In fact it makes things worse before they ever get better.

I had agreed with my therapist that I would attend a Postpartum Depression support group.  I didn’t think it was the right fit for me but I agreed to check it out.  **In advance, mothers, please forgive me for my thoughts I am about to share from this time. I know any mother can experience PPD, no extraneous circumstances necessary.** The group consisted of about 10 women, all but myself and one other mother had their babies there with them, all singletons under 6 months. We moved clockwise around the group introducing ourselves and sharing what we were struggling with.  The first mom was beautiful and put together, hair done, light makeup, well dressed.  She talked about how she had got to go out with her girlfriends over the weekend and how nice it was but how the baby woke up at 2am that night wanting to be feed and that was stressful for her.  The next mom shared that she couldn’t get any friends or family to come over and help give her a break from her baby, her son nursed while she spoke. Another shared that her son was born 11 days early but did not have to spend any time in the NICU and she was sad all the time, and that her husband had earlier that day watched their son so she could get out and get a pedicure. I wanted to scream at these pathetic women, “You have your healthy child attached to your tit right now, what the fuck are you crying about?! I would give ANYTHING to be in your shoes. My children are fighting a possibly losing battle for their lives right now!”  I didn’t of course. Instead when it was my turn I started hyperventilating/ugly-crying halfway through my name.   I bawled out my situation and vowed to myself I wasn’t coming back.  The poor girl after me said she had the “postpartum blues.”

The first 10-14 days on Zoloft were the worst. I didn’t think it was possible but everything negative intensified, even the nausea. After about two weeks everything finally began to improve.   I met with my psychiatrist and we increased my dose over time to an effective level.  I am not ashamed to admit that I am medicated.  It is the best thing I have ever done for my kids and marriage.  Once the medication took effect I could control my emotions.  I could hold a productive conversation with the doctors and discuss my children’s care without crying.  I could think reasonably and logically.  And best of all I began to see the positive sides of everything; I saw everything as blessings in disguise.  Every time my husband and I had received bad news, we made “positive positive positive” our mantra, and now I could really live it.  I wasn’t manic by any means, I still felt sadness and anxiety but at a manageable level.   My husband recently told me in the past he thought people that needed antidepressants were weak but after our experience he saw that some people really do need them and they can help immensely.

During those long two weeks, after 31 days in the NICU, our son came home. He was technically still only 35 weeks and tiny, just over 6 pounds. Leaving the hospital with our son was even harder than the day I was discharged after their birth.  I felt like we were abandoning Baby Squirrel.  She was still so little and sick and we were going home and being happy with our healthy baby boy. Every joy felt like a betrayal to her.  It was like living in two different worlds. In the NICU everything was solemn, but at home we were making memories and becoming a family.  Our son was growing and developing and thriving and the gap between him and his sister was growing larger.

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Baby Boy’s Homecoming

 When Baby Girl was nearly 40 weeks old they moved her out of the typical isolette and into a crib.  We brought every stimulus we had.  A mobile with colorful birds for her to look at and reach for, pictures of us and high contrast images to tape to the sides, a vibrating chair, and an iPod and speaker full of calm upbeat and relaxing music.  She loved her music. Her nurses kept it on for her quietly 24/7. Every day the nurses would tell us how much they loved all the Beyonce, I accidentally put 3 copies of “Halo” in her mix. And they always commented on how much Baby Girl loved her music, how she perked up when it came on. Six months later on my 30th birthday we would learn how ironic their comments were.

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IMG_6565All progress with Baby Girl was painstakingly slow. Even with the feeding tube down in her intestine she was still refluxing and aspirating.  She was always going up and down on oxygen support.  Due to her prematurity she has chronic lung disease, her lungs are riddled with scar tissue and continually fill with fluid, requiring more and more diuretics to dry them out.  She had regular echocardiograms to watch how her heart and lungs played together.  (My heart is currently pounding in my chest as I try to formulate this next sentence…) At two months old she was diagnosed with Congestive Heart Failure. Without knowing what that meant, it sounded like a death sentence, right?! “Heart Failure.” That super vital organ that keeps you alive, hers was failing.

I think I shut down.  I don’t remember much after hearing that. I can’t remember if I was just numb from the daily stream of bad news or if I was protecting myself for her possible demise. I think that was the universal tipping point; the universe decided we’d earned a miracle.  During a follow up echo a couple days later the cardiologist found her VSD, the large hole in her heart that Dr. K said would require open heart surgery, and that the cardiologist later refuted but said would take years to close, had closed seemingly over night.  Before telling us the cardiologist had other cardiologist confirm it because he did not believe what he was seeing.  Later that day at rounds, Dr. K said, “I’m not supposed to say this but she must have a lot of people praying for her.”

We rode that high as long as we could.  But Baby Girl’s reflux was not improving.  Dr. K proposed giving her a g-tube and tracheotomy. We refused.  I adamantly did not want my daughter to have a g-tube.  The thought of them cutting open her perfect, soft, smooth belly skin was unbearable.  Just the sheer thought of it made my eyes well up and spill over.

In early/mid November all the nurses went on strike.  Due to the delicate nature of the NICU, the nurses brought in travel nurses and quietly warned all the parents so they could prepare.  For 48 hours my husband stayed with her to make sure there were no set backs due to nurses that were inexperienced with her very particular care instructions. For 48 hours he stayed with her, sleeping in a straight backed chair and leaving only at shift changes.  This ended up being a blessing in disguise as the travel nurse that had our daughter taught us how to take care of our daughter, and further more, she let us. By the end of the two days we felt much more comfortable with her routine care.IMG_6879

The discussion over a g-tube and trach continued for days before my husband made it very clear that the word “tracheotomy” was not to be uttered ever again unless they could guarantee that it would fix all her breathing issues and allow for her to come home, they couldn’t. However, after much back and forth we consented to the g-tube. They assured me that it was just a tiny hole and once she learned how to eat normally they would take the g-tube out and there would only be a small scar. The hope was that if we could get the feeding tube out of her nose and throat she wouldn’t gag and reflux anymore.  Surgery day came and we felt reassured that the performing surgeon had actually performed a major chest surgery on my husband nearly 15 years earlier.  The surgery was quicker than expected and Baby Girl’s pain was manageable.

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Baby Girl’s first g-tube

Unfortunately, the g-tube didn’t stop the reflux. She needed a pediatric Ear, Nose, & Throat specialist but the closest one was nearly an hour away in Oakland, CA. She was transferred via ambulance with her daddy in toe for what was intended to only be a couple days. Once there and settled in the ENT scoped her nose and throat and found she had a hiatal hernia, the upper part of her stomach was pushing up through the diaphragm and into her chest region.  She would need a Nissen fundoplication surgery to repair it.

Nissen fundoplication, also known as laparoscopic fundoplication, is a surgical procedure to treat gastroesophageal reflux disease (GERD) and hiatal hernia. In GERD it is usually performed when medical therapy has failed, but with paraesophageal hiatus hernia, it is the first-line procedure.
-good ol’ Wikipedia

The surgery would be done with scopes through tiny holes in her belly and down her esophagus and was expected to take only a couple hours tops. I told my husband to call and update me the second she got out of surgery since he was there with her and I was at home with our son. When you have a newborn/preemie at home time ceases to follow its typical trajectory and I noticed 4 or 5 hours had passed and I still hadn’t heard from my husband. I called him and jokingly chastised him for not calling me when she got out of surgery and asked how she was doing. He didn’t know.  She was still in surgery and no one was telling him anything.  He even went and looked for her back in the NICU thinking maybe they had taken her straight back up there and forgot to come talk to him.

After 7+ hours a doctor emerged to inform my husband that her Nissen was successful, but they had found that during her previous surgery to place her g-tube the surgeons had perforated her bowel and colon causing fluid to leach into her abdomen. To repair the damage and save her from developing sepsis they had to make a large incision across her stomach above her belly button and remove the damaged sections of her bowels and colon. So much for worrying about a tiny g-tube scar, she would now have a scar to rival my c-section incision.

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Bloated after over 7 hours of surgery, sporting a fancy new g-tube button

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Abdominal scars

It took her three weeks before she was stable enough to be transferred back to our home hospital.  Her daddy stayed with her the whole time and made it back just in time for Christmas.

The Nissen had greatly improved her reflux, as long as she wasn’t fed too quickly, but she still wasn’t tolerating any food by mouth without inhaling it into her lungs. For the next month it was a continual boomerang of oxygen support.  They asked for our consent to start her on a stronger diuretic, one they had warned us about when she was born, Lasix. Lasix is a very effective diuretic but also strips the body of electrolytes and potassium so she would need routine blood work to prevent damage to her heart and kidneys. After only one dose and she was able to come off oxygen. Again she completely surprised the doctors.  She still had pulmonary hypertension and chronic lung disease so after about a week she needed to be put back on oxygen but only a very small dose.  We got to the NICU one day to find an almighty Discharge Checklist next to her crib. We were so excited! Her discharge was finally looming in the near future.  We were ready for the challenge in front of us because at least we wouldn’t have to come to the hospital every. single. day.

In true to Baby Squirrel form, a discharge date would be set and then moved back. A date would be set and then she would randomly experience apnea over night while in the care of a nurse that wasn’t familiar with her, thus restarting the 5 day waiting period before she could go home. Then she had to pass a car seat test proving she could make the 20 minute drive home safely. Again, against our strict orders, a night nurse performed the test and she failed due to the pads being in the incorrect position causing her oxygenation level to drop.  This presented a huge setback. Now the only way they would let her go home was in a “car bed” which would need to be special ordered. Amazon to the rescue!! This thing felt like a freaking death trap the way it precariously attached in my car but on January 14th, 2015 I got my final wheelchair ride.

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The scariest wheelchair ride of my life!

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Saying goodbye to our children’s NICU aunties and Grandma, their primary nurse, was extremely bittersweet. They had been our family for nearly 5 months. They were the only people in the entire world that knew exactly what we were experiencing every day.  On Baby Girl’s final day I left this letter to her team:

Dear [primary nurses’ names] and ALL the nurses that care for, talked to, played with, held, comforted, and loved our daughter:

We can never repay you or thank you enough for the gift you have given us. Thank you for being [Baby Girl’s] mothers while I could not.

We are so overwhelmed with excitement to finally have our family whole and be able to bond with both our children. You have been our family for the last 4 months. You have witnessed the hardest days of our lives. Your kind hearts comforted us when [Baby Girl’s] mounting prognosis felt dire. You are forever in our hearts every time we look at our beautiful children who have taught us how precious and fragile life can be. I cannot come up with the right words to truly express our gratitude.

We will truly miss all their aunties & grandmas.

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Saying goodbyes 

Leaving the NICU my sister took one last picture of Baby Girl’s empty pod…IMG_7877
Still to this day I cannot articulate how morose this photograph makes me feel.

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Baby Squirrel’s Homecoming

In conclusion, over a year later, some days I feel like I am ready to get off Zoloft, I have even reduced my dose a bit.  But then there are times that I’m not sure I could handle caring for all my daughters special needs without it. When Baby Squirrel is happy and smiles or laughs my heart swells with joy unlike anything I’ve ever experienced, it’s so amazing and wonderful and I don’t know how I could love someone so much. But when she is irritable and sick and crying for hours/days on end or shitting and vomiting nonstop I want to shoot myself in the face (metaphorically speaking, I promise.) I don’t want to stay on medication forever but it’s still something I’m struggling with daily.  Currently my daughter is in the middle of another asthma episode.  She wakes up multiple times a night crying inconsolably, coughing and desaturating thus requiring additional oxygen till she can recover.  She doesn’t have a typical cry, due to multiple factors it’s quieter and courser but it burrows into your brain and eats your goddamn soul.  Along with her pulse oximeter and feeding pump we now have to haul her suction machine upstairs every night and back down again the next day.  She requires 30 minutes of multiple nebulizers 4 times a day followed by CPT (chest physical therapy.)  I do all this while also caring for her twin brother, who is now running all over the place and getting into everything, and while trying to keep the house in relative order. I can’t take her anywhere for fear of her catching another cold and landing back in the hospital again (those exciting tales to follow in later installments.)  Which leave me feeling full of regret that my son is missing out on countless experiences because of his sister. This was not intended to be a pity-party or a sob story but rather to paint a picture of my daily reality and why I don’t feel ready to get off my mediation at this time. Maybe I’m weak but our daily life is entirely too overwhelming without a little chemical help.  And on the worst of days bottle-o-wine help. 😉

I know this was an exceptionally long post; thanks for sticking with me as I try to bring our story up to speed.  Below are more mostly unshared photos from our time in the NICU.  Coming up next: the twins at home, Baby Squirrel’s long awaited cleft lip repair, and her very real, very close call with death. Processed with MOLDIV

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Let’s Get Started, Shall We

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Welcome to my first post! Please begin by reading the “About” section.

When the seed was planted for this blog, the biggest roadblock, other than coming up with a name, was where to start. Currently my twins, A & A, are 15 months old and so much has happened! How could I begin without explaining where we’ve been, but we’ve been everywhere, including near to death. My daughter, Baby Squirrel, has been through so much that I’ve even forgotten about many of her diagnoses that have been overshadowed by additional diagnoses.

Regret hit me for not starting earlier. Then I recalled I had started earlier. Shortly after the twins were born while they were both still in the Neonatal Intensive Care Unit (NICU), I was in the deep depths of misery. I would spend my time between trips to the hospital scouring the internet for hopeful stories about preemies and VSDs and Dandy-Walker Syndrome, and all the rest of the scary words being thrown at us daily. I remember even googling, “are kids worth it?”  My husband and I had always wanted kids. We had struggled with infertility for a year before having a miscarriage and beginning fertility treatment. I knew I was not OK, I needed help.

The following post I submitted to March of Dimes: Share Your Story:
09/2014
31 Weeker B/G Twins in NICU + Special Needs Child (Cleft lip/palate & Dandy-Walker Syndrome)

Where to begin… My husband and I tried for over a year to get pregnant before seeing a fertility specialist. We had been pregnant once but I had a miscarriage at 8 weeks. Turns out I have PCOS (Polycystic Ovarian Syndrome) and don’t ovulate normally. However, after one round of ovulation therapy (and a warning that we might conceive triplets) I did in fact conceive. At 5 weeks we learned it was twins. The first day we laughed. The next day I cried, all day. I was so scared. One baby is hard enough work on its own. After a few days when it sank in a little we were so excited.

The pregnancy started out well. At our gender ultrasound we didn’t learn the genders but we did learn that Baby A had a cleft lip & probably palate. We were devastated.  At the time it felt like the worst news in the world. Like our world with perfect healthy babies was over. Later we learned Baby A was a girl, I felt so bad for her. I felt like we wouldn’t be able to take newborn pictures of her or want people to see her. Feelings that I feel very guilty about now. After meeting with a geneticist my husband and I decided to get an amniocentesis to find out if either of our twins suffered from any larger chromosomal problems. After the traumatic procedure and 2 very long weeks we learned that our son & daughter were chromosomally  perfect. Over time we met with what would be her craniofacial team and saw how they would help fix her clefts. It was so reassuring and our fears were greatly relieved.  I thought that was the end of our worries.

Unfortunately our baby girls story gets worse. At a later ultrasound we got hit with another huge bombshell – a defect in our baby girl’s brain. They found that our baby had Dandy-Walker syndrome, a congenital birth defect affecting the cerebellum & 4th ventricle. They said it looked mild but Dandy-Walker syndrome can cause physical & intellectual impairment to severe mental retardation. There is no way to see how it will express itself. Again we were devastated. Her brain! No parent ever dreams of having a child with special needs. But every time i worried about her she would give me a kick. Like she was reassuring me she would be fine. Although I was heartbroken I had so much faith in her.

Before pregnancy I always said I was pro-choice and I would never bring a special needs child into the world. But she was already my baby girl. I could feel her moving inside of me. I was shocked when doctors gave us the option for a “reduction” to abort just her. We didn’t consider it for a minute.

My pregnancy continued on. We did research and came to terms with another of her conditions. I had Braxton-Hicks contractions everyday starting in my 2nd trimester. After being evaluated by Labor & Deliver they determined I just had an irritable uterus and lots of contractions were just my thing. Unless they got painful I was OK.

At 31 weeks, 3 weeks into my maternity leave, I started having contractions that felt like period cramps. I tracked them and they became more frequent. I called in and they told me I should go into L&D for observation. At 6pm on 9/3/2014 I was chitchatting with a nurse as she tried to get the heart rate monitors on my belly. She was having a little trouble finding baby A so she went out to get an ultrasound machine. I wasn’t concerned and told my husband to use this time to go move the car and grab my bag in case they wanted to observe me over night. A male nurse came back in and after locating baby A got on his phone and called someone “STAT”. I asked what was wrong but he wouldn’t tell me. A few people entered my room and checked baby A again and called more people in. They told me baby girls heart rate was under 80 and she would have to come out via emergency c section. I was so scared. I tried calling my husband to get him back in the room. They grabbed me and started giving me an IV and telling me about general anesthesia. Right as my husband returned they were rushing me out of the room and down to the OR. Once I was on the table I could feel fast moving hands all over me but couldn’t see anything due to the infamous blue curtain. I was so scared they were going to cut me  open before I was under I kept yelling “I’M STILL AWAKE! I’M STILL AWAKE!”

My babies were born at 6:29pm. Baby girl was 3 pounds 6.3 oz. Baby boy was 4 pounds 5 oz.

I woke up in so much pain. My husband had escorted our babies to the NICU where I wouldn’t see them till the next day.

The next day I was determined to get into a wheelchair so I could see my babies. When I first saw my daughter I was shocked by how big her cleft was. It was completely up and through her nose. That was the least of our worries. Both our babies’ lungs were underdeveloped. It killed me to watch my son fight to breathe. I cried nonstop the first few days every time I saw him. Baby girl had the same condition but didn’t seem to be struggling as much. A few days later when they sent me home they told us to expect them to remain in the NICU for 4-6 weeks. Leaving them was the hardest thing.

During the first week we learned our baby girl ALSO has a hole in her heart. It was presented to us as a very large hole that could require open-heart surgery. I was so scared she was going to die. I ran out of the NICU and collapsed in a chair in the hallway, repeating to my husband “I can’t lose her, I can’t lose her…” Later we met with a cardiologist who explained that although it’s one of the largest VSDs he’s ever seen they usually close on their own in a few years. That he would not operate on it. Due to the hole in her heart her lungs have excess fluid in them making her breathing fast & hard. In order to help with the fluid in her lungs they had to give her diuretics which caused her to lose even more weight. Seeing her so tiny compared to her brother is so difficult.

Now almost 3 weeks later our son is doing great. He is off air and up to 5 pounds. We are working on breast & bottle feeding. Once he learns how to eat he can come home. We all hope that is in the next 2 weeks.

Our daughter has finally started to gain some weight back but she is still a pound and a half smaller than her brother. She struggles to breathe and they change her breathing machines often. It still kills me to see her so skeletal. Holding her is scary due to her small size. Plus she has terrible reflux so her feeding tube had to be pushed down into her intestine. Most days feel like 1 step forward 2 steps back.

As for our daughter’s Dandy-Walker syndrome, her ventricles do not seem to be swelling at this time but the doctors still think she will need a lot of extra help. Its so heartbreaking to hear. I hate having to “wait and see.” I am so overwhelmed with fear of having a child with special needs, let alone having another baby to care for at the same time. I cry most of the days. My husband is so amazing and supportive but I feel like I am drowning in despair.  I have an appointment with a psychiatrist to discuss possible PPD.

I knew our twins would most likely be in the NICU but I never realized how hard it would be. My husband stayed home with me for 2 weeks to help me recover but now is back to work till they come home. My routine is going to 9am rounds by myself. Listening to the doctors talk to each other makes everything sound so scary and I don’t understand most of it. I try breastfeeding my son and then go home and pump every 2 hours. At 4:30pm I meet my husband over at the NICU again. Its so draining. Pumping all the time and going back and forth to the NICU takes up all our time. Our babies aren’t home and I still don’t get time to sleep. I am hoping its easier once they come home.

But I am also terrified of them coming home. I’ve seen them struggle to live. I’ve seen how fragile they can be. I’ve become to rely on their monitors to show me they are OK. I don’t trust myself with my own kids. It might be PPD but I am terrified of being a mom. I’m scared I won’t survive it. I feel like every thing and every thought is overwhelming. I’m terrified of having a child with special needs. But I also don’t want to set up a self-fulfilling prophesy by treating her like she has special needs.

Right now I pray my daughter keeps gaining weight as that will hopefully help many of her health issues.

I just wish I could fast forward in time.

Phew, that was painful to revisit.

In posts to follow I will work on getting you all up to date with all the fun stuff thats happened since their exciting arrival.

Till then,

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PS. Ironically I don’t think I swore at all in this post. I’ll work on that.