In My World

In My World

In my world when I hear the word flush I think of a syringe, not a toilet.

When I hear the word tank I think of oxygen, not gas.

When I hear a cough it’s time to get out a burp cloth, sometimes two, maybe even three. The tongue curls and watch out, I hope it doesn’t get on me!

When I say I need an extension I know what you think, I’m trying to make my hair long again and be 23. No, that’s not what I’m saying. I’m saying Emma needs to eat.

Other moms want their babies to keep stuff out of their mouths. I am constantly hoping that mine will put something in.

If the electricity goes out you might think, “oh, this will be fun.” I think “crap, I hope Emma’s pump is charged” and “it’s a good thing we’ve got extra tanks”.

A trip to the store requires lots of things: a tank, a button, sometimes a pump, a handful of burp cloths, and oh yah, don’t forget the toys (remember, the ones we want her to put in her mouth).

The one you might think is oddest of all is when I say I have to “plug her in”. I’m not sure what you think that means, but that means Emma has to eat.

If you come to my house and you hear a beeping noise, please don’t be alarmed.

It’s not the smoke detector, it’s Emma’s pump. One beep means she’s done eating. If it’s two, it means she’s out of food. What if it’s three? I don’t know, don’t ask me. If it’s three let’s go get out her owners manual and we’ll see.

In my world when I hear the word button I’m thinking about Emma’s feeding tube, not your jeans.

When you see a onesy you think “oh, isn’t that cute”. I think, “yah those don’t normally work out”.

When I hear a baby crying I know Emma is tired, because she is normally very happy, the happiest baby you’ll see.

When I see a baby crawling or walking I think “When?”

When I see a baby eating I think “When?”

When I see a baby being carried with no tubes attached I think “When?”

But more importantly,

When I see another baby I think “She’s not as cute as mine”

When I see another baby I think “She’s not as strong as mine”, and

When I see another baby I think “She’s not as happy as mine”.

NEHI Diagnosis

On April 28, 2011 I brought Emma to the emergency room because she wasn't feeling well and was getting pale. Within the hour Emma was put on oxygen and airlifted to DHMC. Even after 13 days of tests there, the doctors still didn't know what caused her respiratory distress. The picture to the left was taken during this hospitalization.

A lung biopsy was conducted in June 2011. The biopsy has been sent to at least two hospitals across the country for analysis. The initial results from Emma’s doctor indicated that Emma has “NEHI pathology”. When asked whether she has NEHI the answer is “she has NEHI pathology”. I think a simple yes/no answer is not given in the event that there are other yet to be known condition(s) unrelated to the NEHI that would complicate a prognosis for her.

NEHI is short for Neuroendocrine Hyperplasia of Infancy. I have no medical background and the following may not be 100% accurate, but the following is a brief explanation of NEHI as I understand it. Everyone is born with neuroendocrine cells. It is my understanding that kids with NEHI either have too many neuroendocrine cells in the lungs airway, the cells reproduce producing too many of these cells, or they were born with the appropriate number of cells and the cells never decrease.  Generally, the number of these cells decrease naturally. The cause of NEHI is unknown. In general, most kids with NEHI outgrow their need for oxygen at some point.  

There is little research about NEHI, its’ cause(s), prognosis, etc. The chILD Foundation was established to "to provide support, education and hope to families affected with a pediatric interstitial lung disease and to advocate and raise funds for scientific research." Click Here for a brochure they have prepared on NEHI.

There is not a lot of research on NEHI, prognoses, causes, etc. More important than knowing the cause of NEHI is the hope that one day Emma will be off oxygen. The hope that one day I can carry Emma upstairs to bed without holding Emma in one arm and the oxygen regulator in another, one day not having to hook up her pulse oximeter while she's sleeping, one day being able to take her for a trip without her oxygen tank, and one day being able to go for an outing without being asked "what's wrong with your baby". 

The photo to the right is the Emma we all know and love. She adores her big sister. She is sweet. Everyone always comments on her hair, which we all think is going to be red. She is weary of strangers. And,she is a happy baby who rarely fusses (except when she's tired, someone she doesn't know well tries to get too close to her, and at doctors visits).

Hidden Blessings, Evacuating our Home from Hurricane Irene

written September 16, 2011



On August 27, 2011 my family packed up a couple suitcases, some of Emma's medical supplies, and a tote of "emergency supplies" and headed over to my husband's parents house. There was a hurricane going up the east coast that was supposed to make its way through New England. They were predicting heavy winds and rain in our area. We live in a very rural area, and we thought it was likely that we would lose power for a while. We put away stuff that was outside, anticipating, at most, garbage cans and loose toys getting blown around. If it weren't for Emma's need for electricity because of her being on oxygen and her feeding pump, we probably would have stuck it out at home.

VT107 between Stockbridge and Bethel.

On the afternoon of Sunday, August 28, the rain was pretty steady at my in-laws, but nothing out of the ordinary. "Maybe we'll just go home this afternoon", Brian said at one point. It wasn't until late afternoon that we started hearing about the flooding going on in our area on the other side of the mountain.







Over the next couple days we started hearing more and more devestating stories of flooding in our area. Worst of all, we had no idea how our house was doing. We heard that in our little village it was "awful down there", "just terrible", "houses were knocked off their pillars", and "water up to the counters". We were hoping for the best, but bracing ourselves for the worst. At this point, the news channels were not even covering a fraction of the damage across the state. Later we would realize that this was simply because people couldn't get to these places. There were thirteen Towns literally cut off from any access outside of their "island". One of these towns was my town, Stockbridge.

One of the houses in our Village that got lifted, moved,

      and set back down.

Wednesday, August 31, there was finally a route to access our house. What would normally be a 45 minute trip home took 2 hours. I cannot even put in words what it felt like waiting for Brian to call to tell me what the house looked like. The girls and I were out for a walk when we got a voice message from him saying, "You won't believe it, but the house is dry!" He was right about one thing, I didn't believe it. I wasn't going to believe it until he came back and told me in person. I was just dreading the phrase after this sentence... "The house is dry, but..." When he got back he reiterated that the house is dry, but the shed and camper have mud inside. The boat also floated from the back yard to the front yard and into the neighbor's truck. It really is amazing. The houses next to and behind ours had water inside and their carpets were wet, but ours did not. A hot tub at the end of the road was blown off the porch and carried by floodwaters at least 150 yards, but Catie's little red wagon was still on our porch. There were houses on the river side of the road that were lifted off their sonotubes, moved, and set back down 20-30 feet away, but ours was dry. Our garden was matted down, the fence was offset at the corner post, there was debris around all sides of our white picket fence in the back yard, but the house was fine. As I write this three weeks later I am still truly shocked that our house is dry.

Initially, there were three factors we needed to wait on in order to go back home: (1) electricity, (2) water, and (3) access to/from the house. Electricity was the first to get restored. Many in our village did not get electricity back as soon as we did. For any house that sustained water damage, the electricity was turned off until an electrician inspected the house to ensure it was safe to turn it back on. Our water comes from a spring down the road where it is pumped to our Village. The pump house was destroyed, as well as some pipes of this system. Water was restored a couple weeks after the storm. Three weeks later, there is now access to/from the house.

The main road in our Village.

I have been back to the house once in the last three weeks. We only went straight to our house and back to my in-laws. I say "straight to our house" but, really, it wasn't really a "straight" route. The trip still took two hours. We didn't go outside of our little village to look at the other damage as there were lots of people out working on the roads and it didn't feel right to be "site seeing" the destruction.

It is now almost three weeks after the storm and there has been a lot of progress made to the roads in the area. The major east-west route across Vermont, Route 4, was initially closed to through traffic from Woodstock to Rutland. This is a distance of 30 miles. This may not sound like a long distance, but this is approximately 1/2 of the east-west dimension across the middle of the state. The major route in/our of my town of Stockbridge is VT100. This road was closed. To the north of VT100 is VT107. This road was closed. About a week and a half after the storm they were allowing 1 hour of traffic in the morning and evening through sections of Route 4. As an engineer I do understand that it takes a while to build a road, but, as a homeowner, I am still anxiously awaiting being able to get home in 45 minute.

In addition to the State roads, many Town roads are in a state of despair as well. The road to my girls daycare was demolished. I know this sounds like a strong word and exaggeration but I assure you, there are sections that have literally been demolished.

Prior to August 28, if I saw the pictures that I've seen in the last three weeks but under the picture said North Carolina or Louisiana, I would have thought, "oh, that's too bad". Now, seeing those pictures with the caption of Stockbridge, Vermont or other towns that are close to my heart gives me such a stronger compassion for what this means to a person that lives in a community where their town was broken. I cannot pretend that I know what it would feel like to have been one of those not as fortunate as we were. I cannot even phathom what is going through their heads.

I see the pictures over and over in my head. Pictures from local news, national news, Facebook, and images of my road and neighbors houses. I don't enjoy seeing these images, but I cannot stop thinking about it. I always thought Vermont was located such that we miss all the major natural disasters. Usually the worst weather we have are massive snow storms. Once in a while there will be a tornado warning somewhere in the state, but I have never encountered anything like this.

They are comparing this event to the 1927 flooding. I have not seen any official comparisons between the two. It really doesn't matter which one is officially "worse". This is still the storm we will never forget. Although the house remained dry, we are lucky we got out when we did. We heard from our neighbor that they were evacuating people in our village in the middle of the storm.

I would obviously prefer that Emma did not need to be on oxygen and a feeding tube. But, if she didn't have these needs we would have stuck out the storm at home.  It is a hidden blessing that we left because of Emma's medical condition. If we hadn't left we would have ended up leaving during the midst of the storm. Where would we have gone then? Would we have made it to Fair Haven? Where would we have been stuck for days with a five year old and thirteen month old? How would this story be rewritten if we hadn't left?

Tomorrow we will be going home again, three weeks after Hurricane Irene. Our lives will not be back to normal, but it will be one step closer. My route to work will be much longer for a while. The route I usually take is one of the hardest hit roads. Other roads will not be the same for a while either. Eventually, our lives will get back to what I consider to be "normal".

Emma's First Time Camping: Could the "To Bring" List Possibly Be Any Longer

As we prepare to go camping I can't help but wonder is the "to bring" list could possibly be any longer"? It always seemed that the preparation for going camping took much longer than it should. But then again, as one that is notorious for making lists, I always bring way too much and then tell myself that next time I'm not going to bring so much. Next time around, the cycle continues and I still bring way too much.

This year we have a lot to add to out list of things to bring. Also, this time a camp site with electricity is needed. It seems ironic that we will need electricity at a time when you are supposed to be "roughing it". However, in this day and age, don't most campgrounds have electricity? Don't most campers have a TV or portable DVD player? The campground we are going to even has WiFi. What was once synonymous with "roughing it" is no longer the case, and I'm completely good with that.

Anyone with kids understands that the camping list automatically doubles once they are on board. Emma has her own list, and I thought I would share this list. Hopefully others will come to peace with their list after reading this and think, "I guess my list isn't so long after all".

Here is Emma's camping list:
- oxygen concentrator
- spare oxygen tanks (both large and small... you never know if the power will go out and you need to use the large tank)
- extra cannulas
- special tape to put under canulla
- tape to put over canulla
- scissors
- pulse oximeter
- spare sensors
- plug adaptor (you never know if you will need)
- pump
- large syringes
- extra extension
- extra button
- special syringe to check/fill button
- bottles
- formula
- corn syrup (we have to add to her formula)
- water
- bottle brush
- dish detergent
- baby food (let's hope she will actually try some)
- baby spoons
- baby crackers (maybe we can get her to put it in her mouth, we can
hope, can't we)
- medications w/ syringes
- diapers
- wipes
- clothes
- pacifiers
- burp cloths
- quarters (we will definitely need to do laundry while there as she spits up
so much and she'll go through all her burp cloths... and she has a lot)
- toys (last, but certainly not least)

I'm sure I'm forgetting something on this list. If so, I hope we don't forget to bring it with us!

11 months old

Emma is now 11 months old. She still cannot sit up on her own, cannot crawl, cannot stand up on her own, has been on oxygen 24/7 for over a month, and we still don't know any answers. I know this status may sound the same as when I first started this blog. With that said, she has come a long way since our hospital trip in December, six months ago. She has consistently been on the growth chart for a couple months now. She is much more interested in playing, babbling, will say dada, will (rarely) say mama, and is in general a very happy baby.  Others may look at her and see how far behind she is. I look at her and think about how far she has come since December. I use December as a baseline only because that was her first official "hospitalization". I look at her pictures and see a dramatic difference from December to now. I look at her December picture and I first think, how did I let her get to this point? But then I have to change my thinking because I know it wasn't a matter of "letting her" get to this point. It's just, simply put, she wouldn't eat. I have accepted that I couldn't have done anything differently. In regards to her eating aversion, you can make a four year old sit at the table until she eats her carrots; you can't make an infant eat. Even after numerous specialists help, she just wouldn't eat. Now, she has a means of eating. I don't know how many people we've had to tell, "she can eat a full bottle in a reasonable time period, she just doesn't want to".

Over the last few months I have found comfort knowing that, although having a feeding tube isn't typical, she's not alone. I've found a Facebook group focused on people with feeding tubes. I feel like I am now a part of a selective group of people that are focused on sharing questions, frustrations, medical situations, and general topics all related to feeding tubes.

We still don't have any answers for what is going on with Emma. With each test she goes through I hope they will find an answer. It is discomforting to think that you want a test for a rare genetic disease to come back positive, but if we know what condition she has, then we can work to treat (hopefully) whatever it is that she has had. Just yesterday she had a lung biopsy to see if this will give us answers.

The biggest impact to everyday life in recent months has been the fact that Emma is on oxygen 24/7. Wherever we go, we need to bring an oxygen tank and her pulse oximeter. We can now pretty quickly get it hooked up to the stroller. The biggest difficulty is transporting her around the house.

To the left, Emma at 5 months.

To the right, Emma at 11 months.

Emma's 13 Day Visit to the Hospital (9 months old)

We brought Emma to the doctor on April 28 due to some breathing issues over the previous 24 hours. It wasn't long before she was in the emergency room, followed by a helicopter ride to Dartmouth hospital. Initially, they thought the right side of her heart was enlarged, and she was identified as having dilated cardiomyopathy. After three xrays, three echo cardiograms, a catscan, numerous blood work, urine samples, a skin biopsy, heart catheter, broncoscopy, and thirteen days in the hospital, we still do not have an answer. There are still outstanding blood samples and skin biopsy results that have not come back. She has blood as far away as the University of Alabama that is being analyzed. Some of these tests take weeks for results. The skin biopsy, for example, takes twelve weeks for results. Some of the neurological tests are testing for abnormalities with her oligosaccharide levels, enzyme abnormalities, and probably other things as well.

During the length of her stay, Emma's oxygen levels have been low. When we first got to her primary hospital, her level was 67%. The minimum they like to see is 90%. She is currently on oxygen at home, and we have no idea when she will be able to come off of the oxygen. It is a little frustrating that when she had her g-tube and button surgeries she had borderline oxygen levels (upper 80s, low 90s) and this wasn't a concern at the time. The logistics of being on oxygen at home are still awkward, but I'm sure it will become second nature after doing it for a while.

Following this hospitalization, Emma has had blood work to test for surfactant protein deficiency. This is a rare genetic condition that the pulmonologist has only seen about half dozen times in her career, and has not seen any cases of this at the hospital where we were at. The research for this deficiency really only got started about ten years ago, so there is not a lot of information out there about this yet. Therefore, it is hard to make long-term prognoses. If the work-up for this deficiency (which takes 4-6 weeks) comes back normal, the next step is likely to be a lung biopsy. If this test comes back abnormal, we may be closer to an answer. Because she was scheduled for blood work, the neurological metabolic specialist had them take some blood to test for an enzyme disorder.

9 months old

Emma turned nine months old yesterday, and I would like someone to tell me where the time has gone. Today she will get weighed by the visiting nurse. She hasn't been "officially" weighed for about four weeks now. I am looking forward to seeing what this number is that we've been following so closely. I am a technical person and love working with numbers, so it is odd to me that this number, the pounds and ounces of my child, is the number that has influenced so much of my life over the last nine months. I even have a spreadsheet with her weights charted over the last few months. Obsessive? No, I think this is just my inner geek coming out in the form of the need to make a spreadsheet for anything and everything.

She is still eating every three hours. I recently read that 70% of babies sleep through the night by the 9 month mark. Emma would sleep longer if she could, but we have to wake her up every three hours (three hours from start feed time to start feed time). I have no idea how long she would sleep if we let her. A full night's sleep is becoming a distant memory to me. There are times when we do fall behind on her feedings and these usually occur during the night or when go places. I certainly can't say that we've never fell behind on her feedings for the day, but she does get her 8 feedings per day in probably 88% of the time.

I think Emma has been making good progress over the last couple months. I am equally concerned for her weight as I am for her development. She is behind in her developmental age. She cannot sit unsupported, she cannot roll from her back to tummy, etc.

A little summary for those just chiming in and may not have been following since this blogs conception. Emma is classified as a "failure to thrive" baby, and has a mickey button for assistance with eating. She has not been diagnosed with anything, although she has had numerous blood tests, urine and stool tests, quick scan MRI, head ultrasound, full MRI, EEG, EKG, upper GI test, a chromosonal microarray test, and an EMG. I may be forgetting something her, but, to summarize, she's had a lot of tests to try to figure out why she can't gain weight and why she doesn't want to eat. She's been seen by a GI specialist, speech therapists, physical therapists, genetics, nutritionist, metabolic neurologic, neurologists, and others. She is currently on medications for reflux and muscle relaxer.

8 Months Old

As noted in another posting, Emma got her Mickey button at 8 months old. This is more conventient than the tube, but is currently a bit pink/blistery/oozy. After talking to the nurse, it sounds like this is granulation tissue and is quite common. She suggested using triamcinolone creme to help reduce this.

While under sedation for the mickey button surgery, an EMG (electromyogram) test was conducted. This test evaluated the electrical activity in nerves and muscles to determine if there are abnormalities in the way nerves transmit impulses or to the muscles. Preliminary results from this test appear normal.


Her formula has changed to Pregestimil. According to the packaging and on-line information, "the fat blend in Pregestimil Lipil consists mostly of a special type of fat called "MCT oil," a "medium chail triglyceride oil." Enfamil's Pregestimil Lipil's unique formulation is designed for feeding babies with diseases or disorders that make it difficult for them to absorb the fat blend in most other formulas." She seems to be spitting up less and not quite as uncomfortable on this formula.

We have asked numerous times in the past whether Emma should change formulas and the answer was always to "just stay on what she's on". We asked about hypoallergenic formulas recently as we were wondering if she might have a protein sensitivity or a food allergy. The doctors don't seem to think she has a food allergy, but were agreeable to giving the hypoallergenic formula a try. It appears to have decreased her overall volume of spit ups. She does still have one or two big spit ups a day (and additional minor ones). She still sometimes get uncomfortable, but this appears to be less in frequency and intensity. She still has her bad days, but overall we think the formula is more appealling to her sensitive tummy.


It has been two weeks since her last weight check in. At that time she weighed in at 13 pounds, 9 ounces. She had a very good growth rate at her last check in (about an ounce per day). This is a typical growth rate, but atypical for Emma. Her growth rate has typically been much less than this. I am looking forward to see what she will weigh at her next weigh in.

Welcome To Holland by Emily Perl Kingsley

Although Emma has not been diagnosed with a disability, sometimes I feel like I'm in Holland...

Welcome To Holland by Emily Perl Kingsley

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting. After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland." "Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

Mickey Button

On Thursday Emma got her mickey button. The procedure was relatively short. We had to wait almost an hour after the sedation wore off because as part of their protocol they check her oxygen levels afterwards and hers kept dipping below what they like to see for departure (90). This occured during her g-tube placement, so the nurse said that this may be "Emma's normal level". The number was dipping under 90, but she had normal coloring and wasn't discontent.



It has only been a short time since this surgery and all is going well. The biggest advantage of the mickey button over the peg tube is that we no longer have to deal with the ~10" tube that would get tucked into her diaper. She was starting to realize it was there and hold onto it during diaper changes, etc. There was always the fear of getting it yanked out.

A Day in the Life of Little e (7 months old)

I currently eat 8 times a day. I was eating 4 ounces per feeding but it just got increased to 4.5 ounces. My arching is better than when I was in the hospital in December, but it hasn't gone away. I get uncomfortable when I eat I sometimes get in the arching position. I also get into this position at other times in the day.

My development is behind. I can hold my head up for a little bit, but after a while it starts to flop backwards. I cannot sit up on my own. I can reach for toys but I do not pass them from one hand to the other. I can roll from my stomach to my side. I am content on my back and do not roll. I also do not crawl or creep. I do like to talk and am making different noises. I try to play as much as I can to work on my development, but I have to spend so much time eating and settling down after I eat. This really takes away from play time. I'm not sleeping through the night because my mom keeps waking me up to eat. Every three hours I have to eat. Sometimes I will get off schedule but then mom creeps up the following feedings (2 hours 45 minutes) to get me back on schedule.

Big C, little e

As you've probably figured out by now, the "little e" in the title of this blog is Emma.  She is classified as a failure to thrive baby, although I prefer to think of her as simply a baby who has difficulty gaining weight. So what, or whom, is "Big C". That would be Emma's big sister, Catie. Catie is four years older than Emma. The two were born around the same size, Catie weighing in at 7lb 8 oz and Emma at 7lb 6oz. As a baby, Catie was always well within the growth charts. She never had any trouble eating, and to this day is still a great eater.

G Tube Placement (5 months old)

The scheduled surgery for Emma's g-tube is slated for 9am on December 23. Emma is 5 months old. Emma cannot eat anything prior for a number of hours. On the positive side, this means a little sleep for mom. Unfortunately, she is unable to sleep, thinking about the day to come. Mom gets woken up at 8am by the nurse saying there is an 8am slot for Emma. No time to shower, mom changes out of her pajamas and off we go.

Self Conscious for Baby

Emma is too little to know, think, or care about what others think of her. So, as her mother, I take on that role. The few weeks after Emma was born and we were out in public, I used to relish in the comments from strangers, "oh, look at the baby... she's so little... ". A few months later, these comments start to get old. I remember when Emma was four, five, even six months old and people thinking she's a newborn. It was cute

A Little Background

We found out while I was in labor that Emma was breech. She had turned during the last week. When she was born, it literally looked like Emma was folded in half, with her feet up near her ears. This was a natural position for Emma for the first few weeks of her life. As I look back, I wish I had taken a picture of this for future scrapbooking. Gradually, her feet would come down to her toes.

Emma has never been a good eater. It took her about 3-4 weeks to get back up to her birth weight. She has always spit up frequently and gets uncomfortable while eating. She has never wanted to eat what she is supposed to.

Why a Blog?

I never thought I would be one to write a blog, but here I am. The goal of this blog is to, hopefully, reach out to others who have babies who are classified as "failure to thrive". I know there are others out there going through this, but sometimes it can feel like you are the only one. The sleepless nights, wondering when she's going to catch up to the other babies, the lack of answers after all the testing, and the day to day life of caring for a baby going through this can be stressful.

I hope to find others going through this or have gone through this. There are so many causes of failure to thrive and it is frustrating to not know why your baby can't eat what she should to gain the weight that other babies their age are gaining.