Crawling Out of that Big Dark Hole

It's been a few weeks since a blog update. I've sat down a few times to write, knowing I should update, but I have had zero motivation or inspiration. I've just felt exhausted. Mentally and sometimes physically.

From the beginning, I said I'd keep it honest here on the blog, and I'll be keeping that promise.

I've been in a mental "big dark hole" for the past few weeks. Not a depression, but my thoughts have definitely been consumed with Tennyson's progress - or lack thereof. Since her surgery, we are dealing with huge regression in terms of her muscle strength. She can no longer bear weight on her legs, her head control is poor, and her reflux is back with a vengeance (which means I get to clean up throw-up daily). In the long run, once we get her strong again, the range of motion in her hips and legs will be wonderful for her, but it's hard to see the light at the end of the tunnel when a years worth of work is out the window in a matter of weeks. We are starting over, and it feels like we have a never-ending mountain to climb.

It didn't help that she had six appointments in the last week and I had to explain over and over again to each one of her doctors where she's at developmentally and why she has regressed. It drained me. She saw Genetics (and we found out that Tennyson is still the only little girl in the world documented with her rare chromosome abnormality), Pediatric Surgery (to get her g-tube changed), Orthopedics (because one of her legs is longer than the other due to "pelvic obliquity" - a topic for another day), Neurology and GI (because she was due to be seen), and she had a weight check with Nutrition (she didn't gain or lose anything -not good, not bad).

I can usually carry on and not let the negative feelings consume me. I think I've developed a pretty thick skin when it comes to the slow progress and accepting the cards we've been dealt. But lately, I've let it get to me. When I start to feel negative, I start to think negatively. Everything feels harder. It's a slippery slope that dumps me into a "big dark hole".

I get irritated with the stares we get in public and the strangers who ask annoying questions. I want to say to them, "she's just a little girl!" or "She can understand what you are saying!" (and other really witty, sarcastic things that I would never actually say...). The kids in her class at school have more manners than half the adults we come in contact with while we are out and about.

I went to pick Tenn up from school one day this week and she was sitting in her teachers lap playing with all the little girls (so cute!). One of them came up to me and showed me a sign that had her name on it. I asked her if she knew the names of the letters in her name and she did! It was one of those moments that made me go, "Holy crap - that's what three year olds can do!? This kid is a genius!".

It made me wish Tennyson could do more. It made me sad that she is so far behind - years behind her peers. When I told my Husband how sad I was about it, he said gently, "It's only going to become more obvious the older she gets." I know he's right. He can say those things and somehow it's okay because we're in this together. He adores Tennyson for Tennyson and doesn't compare her to any other kid. He thinks it's awesome she gets to participate with her peers at school and they work hard to adapt all the activities so she can be part of them. From the beginning, he has seemed to accept it all easier than I ever have.

I can't fix the damage done to Tennyson's brain. I won't deny I wish I could. I wish I could have a two way conversation with my daughter. I wish she could tell me about her day, rather than having to ask her teachers or read it in her communication log from her therapists.

Then I read "The Mom I Would Have Been" by Dana Nieder of the blog Uncommon Sense. Definitely worth taking the time to read! She put into words (better than I ever could have) exactly how I was feeling. Sometimes knowing you aren't the only one feeling the way you do is just enough to make you feel okay again. So, I'll work on acknowledging  the feelings I have and then get myself out of this stupid big dark hole.

Amy

tennsense@gmail.com

What's Not Wrong With Her

“What’s wrong with her?” If you ask me this question, I’m likely going to ask you, “What’s wrong with you?” There is nothing wrong with Tennyson - maybe different, but certainly not wrong. She is my normal. Even if it’s different to you, it feels normal to me. One scenario is typical for us: someone will ask, “how old is your little girl?” and I tell them how old she is, and they just say, “ooohhh.” It usually ends there. They have a sort of dumbstruck look on their face as they try and figure out what’s wrong with my sweet girl – trying to figure out why she isn’t doing what they expect her to be doing for that age. One time on a cross country flight, a flight attendant asked me if I had Tennyson tested for developmental delays. As if I wasn’t aware my child has developmental delays. I should have told her I just hadn’t gotten around to it, just to see her expression. Thanks, lady. I know this is a difficult question for an observer to ask, but there are better ways to approach us so my protective mommy instincts don’t kick in.

The best approach someone had with Tennyson was a nice compliment and then they simply asked, “May I ask what her diagnosis is?” At least she gave me more credit than the flight attendant did. She was just being curious and wanted to know more.

You won’t have to ask me, though, because her diagnoses are what I want to share with you today. Since one of the main reasons I started this whole blogging adventure is to educate people who want to know about her, it seems appropriate, rather than leaving you wondering. I have to admit, before I had Tennyson, I had no clue what any of these conditions were.

I guess I’ll start in order of appearance:

Severe IUGR:

Tennyson was diagnosed with this almost immediately after her birth. IUGR stands for Inter Uterine Growth Restriction (or Retardation, but I don’t prefer that word). It refers to the poor growth of a baby in the mother’s womb. The most common cause is a problem with the placenta. It can also be caused by infection, high blood pressure, smoking, drinking alcohol, abusing drugs, heart or kidney disease, or if you are having multiples (twins, triplets…etc.).

Tennyson on her birthday (to show you just how small she was!)

Hypoxic Ischemic Encephalopathy:

I talked about this diagnosis at the end of Tennyson’s birth story. She was diagnosed with this at 2 weeks of age. Broken down it means: Hypoxic: lack of oxygen, Ischemic: lack of blood, Encephalopathy: a specific brain injury. In other words, a brain injury caused by lack of oxygen and blood flow. When a baby’s brain has suffered from a lack of oxygen, it’s common for the baby to have seizures, have low APGAR scores, have varying muscle tone and reflexes (all of which Tennyson had). There are 3 degrees of HIE: mild, moderate, and severe. Tennyson was classified as having mild/moderate HIE.

Microcephaly:

Microcephaly is when the brain does not grow at a normal rate. Head circumference is measured from the forehead to the farthest point in the back of the head and is plotted on a growth chart. Tennyson was diagnosed with this at 4 months of age. There were concerns about her head circumference from about 2 months of age. Her head circumference at birth was 28 centimeters. Her head circumference now is 39 centimeters. A typical growth rate for a baby’s head is 1.5 to 2.5 centimeters a month. Her head size right now is the size that a typical baby’s head would be at 3 months of age. Tennyson is 28 months old.

GERD:

GERD stands for Gastroesophageal Reflux Disease. GERD is when the contents of the stomach go backwards into the esophagus. Tennyson was diagnosed “officially” at around 15 months…but we have been dealing with the effects of acid reflux since she was about 3 months old. She was put on her first reflux medicine, zantac, then prevacid, now she is on prilosec (among other meds). She has constantly had issues with spit-up and throw-up. There have been some scary throw-ups when she stopped breathing and it was so violent it came out her nose. Thank God for bulb syringes in those moments. They can save lives. Seriously. We worry about aspiration and because we know she does aspirate (when her food goes into her airway, rather than her esophagus) she has had multiple swallow studies.

Intermittent Esophoria:

This diagnosis came unexpectedly when Tennyson was 7 months old. Since Tenn was born early, her eyes were monitored for vision problems. Premature babies in general are more susceptible to vision problems since their eyes have not finished developing. It was determined early on, at 2 months of age, that her vision was good, a follow up at 3 months showed the same. Sometimes we noticed one of her eyes turned in when she was tired. When I asked the Ophthalmologist about this he did a really thorough exam and was able to see it. Intermittent Esophoria is when both eyes are open and can focus on a visual target, but occasionally one eye turns inward. This is improving as her eyes get stronger. She has prescription eyeglasses for farsightedness and astigmatism.

Tennyson - 4 months (example of intermittent esophoria)

Cerebral Palsy:

This is the diagnosis I knew in the back of my mind that was coming, but feared the most. Tennyson was diagnosed the day before her first birthday. Some children are bound to a wheelchair while others are much more functioning, but CP doesn’t necessarily mean the child isn’t smart or able to communicate.

CP occurs in about 1 out of every 1,000 births. Cerebral palsy is not progressive (it doesn’t get worse over time). You can’t catch it from anyone; you can’t test for it before your baby is born (I’ve been asked that one before). There is no cure - only management of the symptoms.  Cerebral Palsy is caused by an injury to the motor control areas of the brain. This can be anything from a lack of oxygen, a stroke, a brain bleed, a head injury, or an infection. CP prevents the brain from communicating with the muscles properly. I always think of it as the brain speaking English, but the muscles only understand Spanish. So any command the brain sends to the muscles, they don’t respond properly. There are different degrees of CP and different types. Cognitive ability is sometimes affected, but not in every case.

Tennyson has been diagnosed with Spastic Diplegia Cerebral Palsy. Spastic means muscle tightness. Diplegia CP means that it affects 2 of her limbs (the bottom half of her body). We are anticipating her diagnosis will eventually change to Quadriplegia Cerebral Palsy because she doesn’t move her arms or her legs typically. Quadriplegia CP means all 4 of her limbs are affected. She has the motor skills of a 5 month old. She doesn’t sit unsupported, crawl, bear weight on her legs, stand, or walk…yet. We learned early on that typical developmental timelines don’t apply to Tennyson. She is on her own timeline. But she is smart! She has a good understanding of the words and commands we say to her. She gets it.  

Oral Aversion & Dysphasia:

Imagine never enjoying food. Any experience with your mouth is unpleasant or painful and you never feel good because you are constantly throwing up. As adults, we know food is good. It brings us pleasure and it tastes good. It makes us feel good to fill our stomachs. A baby with oral aversion doesn’t know this because she hasn’t had any positive experiences with food. An adult will find a way to eat, but a baby will simply stop eating. That’s what happened to Tennyson. Oral aversion is a defensive behavior. Dysphasia complicates it by making it difficult to swallow safely and control food and saliva in the mouth. We have gone through phases of having to thicken her milk. She is on an all liquid diet, but we are making a lot of progress with her interest in food since she has had a G-tube placed. A G-tube is short for gastrostomy tube. It is a feeding tube that allows us to pump food directly into Tenn’s stomach and bypass her mouth.

Laryngeal Cleft (Type 2):

I never expected this diagnosis. This condition was found during an airway study last April. When a person swallows, there is a flap that covers the airway so the saliva or food goes into the stomach. That flap prevents food from going into the lungs. Tennyson’s “flap” had a hole in it. The “type 2” designates the size of the cleft. It can range from type 1 to type 4; the smaller the number the smaller the cleft. She had surgery last May to sew the 2 sides of the cleft together. She was one of the first children in the country to have her cleft repaired by a robot. We were told she had quite an audience of doctors and surgeons during her surgery. It healed perfectly and the hope is that she will eventually not aspirate any food or liquids.

Tennyson after her laryngeal cleft repair, May 2011

 Speech Apraxia:

As we neared the end of Tennyson’s first year, it was clear that Tennyson was developmentally delayed, so this was a diagnosis I expected. Speech Apraxia has to do with motor planning. Tennyson has been in speech therapy since 18 months of age (the earliest our insurance would cover it). She get’s speech therapy 2 times a week in our home and she is making progress. It was pretty clear from the start of speech therapy that Tennyson wanted to talk and copy what we were asking her to do. She has difficulty with the motor planning part of it, where her brain tells her muscles what to do to create the sounds she wants to.

Epilepsy:

By definition epilepsy is, “a brain disorder in which a person has repeated seizures over time” (2 or more seizures without an explainable cause). Although Tenny had seizures at birth, they were explained by her brain injury. They were well controlled and she was off her seizure meds by 4 months of age. She had one seizure at 8 months of age due to a high fever (this is a febrile seizure), but I honestly believed she would not have epilepsy. Last July she began having seizures regularly. They have been difficult to control. Her neurologist team has diagnosed them as “tonic spasms”. As a side note, infantile spasms are the worst kind of seizures and her tonic spasms are very similar and if uncontrolled could progress to infantile spasms.

Chromosome Abnormality:

I never expected this one either. We were referred to Genetics by our GI doctor.  Our GI doc thought it would be a good idea to see if there was anything metabolic going on genetically that was preventing her from wanting or being able to eat. Nothing was found that would help us with her feeding issues, but they did find a chromosome abnormality. It’s so rare, that Tennyson is only the 2^nd child in the world to be documented with it. There is another little boy in Finland who has it, but Tennyson is the only (documented) girl in the world with it. On one of her 19^th chromosomes, there is a small section of genes that is duplicated. It is called a micro-duplication. Because of the genes that are duplicated, they have told us that it could put her at a higher risk for heart problems or childhood cancers. After testing, however, Tennyson does not have any major problems like that.

Let me know if you have any questions about anything! I’d be happy to answer them! E-mail me at tennsense@gmail.com. Thanks for reading!

Amy