One of those days

It's impossible to ignore the fact that today would have been Ella's first day of kindergarten if she were healthy enough to attend. My news feed on Facebook is flooded with back to school photos. For most parents and children, this is one of the biggest milestones in a child's life.

I know, I know, things could be worse. I'm "lucky" to still have her here after everything she has been through, but some days it makes me angry that the "it could be worse" is that she could be gone; that we're that close to the bottom of the barrel, so to speak.

For the most part, social media has been a positive thing for me. I have been able to connect with hundreds of other special needs moms because of it, and made some real and lasting friendships that would have never happened otherwise.

But the downside is that when everyone is putting their lives on display, we tend to compare ourselves to everyone else. And it's impossible to ignore the missed milestones that continue to come over and over again. First steps. First words. First day of school. Etc., etc., etc. The list will continue to go on.

I don't enjoy having these pity parties, but we're all human and sometimes it's easier to focus on what we don't have rather than count our own blessings. And I honestly don't mind if seeing our struggles makes others appreciate what they do have, because most days are fine.

I try not to dwell on how difficult our life can be. Like anything else, I have honestly gotten used to it for the most part - the frequent hospital stays and the headaches that come along with managing her nursing, prescriptions, supplies and equipment. It's just our life. But some things still smack me in the face and stop me in my tracks, and this is just one of those days.

Trach Week

This week is Global Tracheostomy Tube Awareness Week. Ella has had her trach since she was 6 months old. She was born with a small, fragile airway, mostly due to Pierre Robin Sequence, and was unable to be intubated for surgery as a baby. She was also found to have both central and obstructive apnea and was put on C-PAP through a ventilator at that time. This photo below shows her trach tube clearly. It's held on by velcro ties that must be changed out daily along with care to her neck and the stoma (i.e., the "hole" in her neck) to prevent breakdown of the skin and infection.



There are a few different attachments used for the end of the trach tube. In this photo she is wearing a (purple) Passy Muir Valve which lets her breathe in through the trach but not out and allows her to make noise and have a voice. Without the PMV, she cannot make any noise. The other attachment we use is an HME or humidivent which protects the opening of the tube and provides moisture, and we have an attachment to give her oxygen when needed.

She must be suctioned fairly frequently throughout the day in order to keep her airway and lungs clear. At night when she goes on the vent, the circuit attaches directly to her trach tube. Technically she is "always intubated". For someone without a trach, this happens via the mouth in emergency situations. People who need long term support for their airway and lungs receive a trach.

Wherever we go, we must always have a backup (emergency) trach of the same size and one smaller in case it needs to be changed quickly. We must also carry our portable suction machine, suction catheters, saline bullets, oxygen and a pulse ox monitor.

Although Ella's airway has gotten larger and improved over time, her lungs are weak and do not have much reserve so she will always need to be on the ventilator for C-PAP/pressure support at night. She may need additional support (a breath rate) when she becomes very ill. In addition, she would likely still be a difficult intubation in an emergency situation, so she will always have the trach. There are many people who only have them temporarily, but for Ella it will be part of her for life!

Pseudo Mom

I can't talk to you about when to switch your baby to solid foods. I can't hold a conversation with you about the latest kid's movie or toy. I don't shuttle around from school to ballet lessons and play dates. We don't get invited to birthday parties or build sandcastles on the beach on vacation. In fact, you will rarely see me out in public with my daughter at all. But I am a mom.

I know that I won't ever give her advice on boys and friendships, or help with her homework. I won't have to discipline her and determine grounding sentences. And I won't anticipate her going off to college or getting married, which are all common aspects of being a parent.

When I'm around other moms of typical children, I feel just as out of place as the girl who has no children yet. I have about as much to offer as she does: maybe some anecdotal information I heard about a friend's child or my nephew. I have no first-hand experience on the majority of parenthood topics, and that can feel very awkward like I don't really have a child at all.

But I know being a mom is more than that stuff and deep down at the heart of it we're all the same. We all strive to do what is best for our children and to keep them safe and healthy. Though our day-to-day routines and visions of our children's futures may be very different, a mom's love is universal. I know that she knows I am her mom by the way she looks at me. I know she is telling me that she loves me with her ear-to-ear smile. I know that overwhelming, inexplicable feeling of unconditional love that comes with being a mom, and that is what's most important.

Happy Birthday, Ella Grace

On the eve of Ella's 5th birthday, I am thinking about where we were at this exact moment five years ago. I went into labor around 8:30 in the evening and we went to the hospital. We were already aware that she would be born with some type of genetic issue or syndrome based on the findings of an ultrasound I had at 26 weeks, but we didn't know exactly what it was or how she would be when she was born. Since I had spent the past 10 weeks Googling every syndrome known to man, I had had a good amount of time to get used to the idea of having a special child, as much as that is possible anyway. I think the pain of labor did a good job of keeping my mind occupied, and at that point we were just ready to "know".

It wasn't until 4 o'clock the following afternoon that the doctors announced she would need to be delivered via C-section and less than an hour later she was finally here. I remember immediately asking John, "How does she look?" but can't for the life of me remember what he said. I got a quick glance at her in my drug-induced stupor and she was whisked away to the NICU while I was banished to my hospital bed unable to get up due to the surgery.

Now, I understand that doctors often err on the side of "worst case scenario". I guess it's at least in part so they don't imply false hope and to cover their own behinds, but our neonatologist did not have many good things to say when he finally came to my room and delivered the news of her diagnosis. I was handed computer printouts about Cornelia de Lange Syndrome (www.cdlsusa.org) and given a laundry list of the medical problems they had already noted and more they anticipated. All I can say is thank goodness for that little button that gave me more pain medicine when I pushed it. Well, that's until some time in the middle of the night when my epidural slipped out...but that's another story for another day.

It wasn't until the next morning that I really got to meet her for the first time. They wheeled me into the NICU and I saw my tiny, hairy, beautiful baby with a gazillion wires and tubes coming out. At 5 lbs. 1 oz. she was actually a decent size considering her diagnosis and the fact she was a few weeks premature. Despite everything they said was "wrong" with her, she was beautiful. And she was mine. I knew she was going to be okay and that was all that mattered.

Birthdays are always an emotional time filled with a lot of conflicting emotions. I'm so honored to be her mother. She has been through more medically than most people will experience in a lifetime but you would not know it by looking at her. She has a smile that can light up a stadium. She brings so much joy to everyone who knows her, and even to many who have never met her in person. She is honestly the happiest human being that I have ever seen and I could not be more thankful for that. She is resilient beyond belief. She has bounced back from the verge of death on more than one occasion. She endures constant hospitalizations with people poking and prodding and examining her. She smiles at them all. I know that I am biased, but there is just something about Ella. She leaves an indelible mark on everyone she meets and people do not forget her.

But if I'm going to be completely honest about this life with her, I have to talk about the conflicting emotions that come along with having a special child with exceptional needs. Every year that passes, the gap between Ella and her typical peers widens. As a parent it is hard to watch. Not for her, but for me. I know that it is purely selfish because SHE is happy. She doesn't understand all of the things that she doesn't get to do. I really try to not linger there, but I would be lying if I said those tinges of jealousy don't pop up every now and then.

Last week I went looking for a gift to give to her at the party we held this past weekend. The truth is, I could wrap up a toy she has had for years and she would smile just as big when we gave it to her. But I want to be able to get her things even though she doesn't need them or even understand the concept of getting a gift. So I found myself browsing the infant toy aisle for the fifth year in a row and I had a moment. It was obvious upon checking out that I was buying a birthday gift and I was dreading the cashier asking me whose birthday it was and how old she was going to be and imagining the look on her face if I answered honestly that she was turning five. Thankfully that didn't happen.

The occasional pity party is inevitable I think. The trick is to not linger there too long. Accept reality and move on, and above all COUNT YOUR BLESSINGS. I even get angry with myself for going there when I'm fully aware that she almost didn't make it to her 5th birthday and what a gift it is that she did. I know far too many parents who have lost their child and I have to keep it in perspective and cherish every moment. Tomorrow isn't guaranteed for any of us.

I have always loved celebrating any occasion, it's my nature. Anyone who knows me knows that I love a party! So for Ella I try and go all out and make it a true celebration with lots of family and friends because it really is a big deal. It's also a chance to thank those around us for their support of Ella and our family, and we are lucky to have so many people to thank.

The first time I saw Ella in the NICU

Ella Grace at her 5th birthday party this past weekend

Please Don't Tell Me How To Feel

Having a child with special needs is a process. We grieve and struggle with acceptance, though it does not in any way mean we aren't thankful for our children. It simply means that we must grieve the loss of the expectations we had of a life with a typical child, and learn to navigate the struggles that come with having a special one.

No one expects to have a child with severe disabilities or medical issues, so when it happens to you, you feel like your world has been turned upside down. Adjusting to your new world takes time; how much time depends on the individual. For many, it's a lifetime process.

Grief has been described as coming in waves. At first they come fast and furious, but in time they usually begin to slow down though you still never know when the next one is going to hit. So as grief does, never completely leaving you alone.

The five stages of grief have been described as denial and isolation, anger, bargaining, depression, and acceptance. They say we don't necessarily enter each stage in that order, and often move in between the stages over time. I know for me personally, this is true. Even once you accept what has happened, you still fall back into the other stages from time to time.

This is why I ask that you never tell me how I'm supposed to feel. And I don't mean it to sound harsh. I know people just want to help, and are maybe even trying to help themselves make sense of our situation, but it doesn't help us. The process is far too personal and so inconstant, it just isn't possible to know how someone *should* feel. And if we're feeling far from what you say we should be feeling, it can only make us feel worse.

We don't have control over this process and there is no single prescription for dealing with it. We are going to be sad at times. We are going to be angry at times. We are going to be happy at times. But we cannot predict it. All we need is an ear to listen when we feel like talking, a shoulder to cry on when we feel like losing it, and a voice to help us cheer when we feel like celebrating.

What's With The Name?

When I found out we were having a girl, I was over the moon. I really, really wanted a little girl. My mom and I immediately began shopping like fiends. We couldn't resist all of the precious clothes and hair bows and frilly things that you could buy for a little girl.

Even though Ella spent a considerable amount of time in the hospital as a baby, we didn't let that stop us from dolling her up. Every day we were excited to pick out a new outfit and take pictures of her. It quickly became the thing that helped us retain some sense of normalcy in a very abnormal, and often scary, situation.

It wasn't long before the hospital staff took notice of our obsession with decking Ella out. Her nurses were often excited to join in the fun, and people began stopping by daily to see her and check out her outfit for the day. Because of her extreme medical issues, she has always been hospitalized in an ICU (NICU, PICU, TICU) and at some point, she became known as "the best dressed baby in the ICU".

I believe one of the great things about having a special child is that it allows you to have a different perspective and maybe not take for granted the little things that others sometimes might. When we learned that Ella was going to be born "different", we had to readjust a lot of our expectations, but that did not include how we could dress her! It is something "normal" we can continue to do for her when most days are far from typical.

At almost 5, I'm glad to say that Ella still upholds her reputation at the hospital. People still come by to see what she is wearing and what color bow she has in. I think it makes them happy too since they spend their days around children who are sick and injured and not getting to just be children. It adds a little bit of balance and fun to a sometimes gloomy environment.

Our little fashionista in the ICU at six months old.

The "R" Word

I have been mulling over this post for probably a couple of years now. Being tied closely to other special needs families via the internet and social networking sites, I often see blog posts and articles on this subject. Last night I came across another which got me thinking about it once again.

Mental retardation is a clinical term. It was developed to classify individuals with cognitive deficits, historically those with an IQ below 70. Basically, it describes those born with intellectual and developmental disabilities which may or may not be related to a syndrome.

I have always felt that when I did finally post about this topic I would need to be completely forthcoming and admit that prior to the birth of my daughter I, too, had used the term "retarded" in a non-clinical, derogatory manner. I believe there are only two reasons why someone would use the word: 1) they are ignorant about how and why it is hurtful or 2) they are flippant and don't care that it is hurtful.

I fell into the first category. I didn't know any better. I didn't understand why it was wrong to say it. I had never thought about it. I never had a reason to think about it. Then I became pregnant with Ella, and by no fault of her own, she was born with intellectual and physical disabilities. She was what could be clinically described as "mentally retarded". Even though I KNOW it is a legitimate, clinical term, it still hurts me to write that. Why? Because of the stigma associated with the word. Because it's still used so freely and flippantly by so many as an insult to describe something that is dumb, stupid or not worthy. My daughter is the opposite of those things. Her name is not interchangeable with the common perception of the definition of the word "retarded".

I believe a major part of the struggle special needs parents go through, especially in the beginning when they find out their child will be different, is not only grief over the loss of the expectations they had for a typical child, but also grief and worry over how the world will view and treat their child because they are different. We all know that the world can be a cruel place, especially if you possess noticeable differences from what is considered to be the norm or the perfection society makes us believe we should strive for. We see how much more prevalent this behavior is among children and teens, but as we grow into adults it becomes less socially acceptable to treat people this way. We come to know better, probably because we all experienced ridicule at some point growing up and know how awful it feels.

But when it comes to people with disabilities, it is still too socially acceptable to use disrespectful language, and essentially make fun of them, even among adults. Is it because most people have never had a personal relationship with someone who is disabled? It has to be. Because if you have ever known or loved someone who is, you would understand how hurtful it is to hear it. If it's not okay to make fun of people who CAN defend themselves, how is it okay to make fun of people who don't even have the ability to stand up for themselves?

Just like the "N" word, the "R" word has clearly become a pejorative in our society. So why would people purposefully continue to use language that they know is hurtful? Freedom of speech doesn't include hate speech. Originally, freedom of speech was a right given to speak up against the government, to protect and advocate for ourselves. It doesn't imply the right to use dangerous and hurtful language. Just as libel and slander are not allowed to defame someone's character, neither should language that disrespects a population of people who are mostly denied the right to free speech themselves simply due to their disabilities.

People with disabilities were born that way. The same as I happened to be born with brown eyes, it wasn't a choice and it can't be changed. Nor should it. But what can be changed is the way these individuals are viewed and often disrespected in our society - a large part of which is tied to the acceptance and derogatory use of the "R" word.

Technology-dependence

Most people are familiar with the term "special needs" which encompasses anything from food allergies to autism to genetic disorders and the medically fragile. Technology-dependent children, however, are a relatively small population in terms of special needs. It's not often that you see or hear anything about children with these extreme needs that includes things like oxygen dependence/continuous oxygen saturation monitoring and children with tracheostomies who may be either partially or fully ventilator dependent. In short, these children rely on technology to stay healthy and, frankly, to keep them alive.

A month after Ella was born, she was transferred from the NICU at a regular hospital to the children's hospital. At this point, Ella had been diagnosed with a genetic syndrome (CdLS), reflux and feeding difficulty, and relied on a feeding tube to eat which was fairly drastic to us being new to the world of special needs. I remember very clearly looking down the opposing hallway while walking to the NICU and seeing the sign for the "Technology-dependent ICU" (or TICU) and thinking to myself, "My God, those poor children. What has to happen for a child to end up there?" It just sounded bad.

Admittedly, if I were to hear someone utter those words within earshot of me now it would probably upset me because, of course, in our eyes she's great. But just as I was naive back then and had no clue what technology-dependent even meant, I understand this is the truth for the general population.

A short five months later, we found ourselves to be the parents of a child admitted to the TICU. Ella had received a tracheostomy due to a small and fragile airway and an inability to be traditionally intubated. We had also learned that she had both central and obstructive sleep apnea and she was placed on a ventilator at night with some supplemental oxygen needs. It was a scary world to land in with a six month old infant.

But today, nearly five years later, all of this "stuff" seems so normal to us. Ella doesn't go anywhere with out an O2 monitor, suction machine (to help clear her trach), feeding pump, emergency trach bag and oxygen. She is only on the ventilator overnight. Though she has been and continues to go through a lot medically, she is the happiest child you will ever encounter. You see, even we can count our blessings though to a lot of people our situation probably looks frightening and less than enviable. It's all relative, isn't it?

It's not likely that you'll run into a family like ours very often. For one thing, most of our children get sick too easily to be out of the home much, however many do go to school. But if you do happen upon a family like ours, my hope is that you might remember this post and have a better understanding about what it means to have a technology-dependent child. If you look past all the medical equipment, you'll see that we're just a family, like you, doing whatever is necessary for our child to thrive.

180º

When we were at CHOP (The Children's Hospital of Philadelphia) last year for Ella's fundoplication revision, I was asked to participate in a study about decision making by parents of seriously ill children. As evidenced by this blog, I have never been shy about sharing my thoughts and experiences going through this life and agreed to take part. Today, four months later, I received a follow up survey which I just completed.

Many of the questions regarding my current emotions and state of mind were the same: to what degree have I felt anxious/
scared/hopeful/angry/guilty/depressed/proud/etc. As I was checking off my answers, it really hit me how much things have changed for the better in a relatively short period of time.

This past summer we experienced one of the darkest times, a close second to a few years ago when she was barely holding on to life on the oscillator after contracting RSV. This time, it wasn't the result of an acute illness but rather the process of her body and systems failing her. Nothing is scarier than watching your child deteriorate and not knowing how to stop it. I had just made the most drastic decision ever concerning her care by getting her transferred to Philadelphia to undergo a risky surgery. The alternatives we were presented with at our home hospital were risky as well, but also indicated that her quality of life would be drastically reduced. Thankfully, the surgery was successful and Ella is thriving.

It all feels like a dream now; like it didn't really happen. Once we headed home, I did my best to not look back - surely a coping mechanism at least in part. I think it's difficult to really gauge the degree of despair when you're in the moment. You know things are bad, but you're also focused on a solution so there isn't a lot of time for wallowing. But shutting out the experience once it was over didn't allow me to realize how far we have bounced back, until today when I did the survey. The difference is night and day. A complete 180.

There are never any guarantees, especially when dealing with a complex and fragile child. I know that won't be the last time we're faced with a tough situation. As things progress, we understand that our choices will become increasingly unappealing and difficult, but we can also rely on the fact that we have come through those dark times before and are stronger for it.

What It's Like

I want to preface this by saying that nothing I post here is meant to invite any kind of pity. I know I have stated that before. I really just want to help others understand what this life is like and realize it's okay to ask questions and not feel sorry for us and to talk to us about our lives and our kids. It's also an outlet for me to talk things out as we move along in this journey and our perspective changes.

The best analogy that I have come up with to describe what it's like to have a child like Ella is it's like having a perpetual newborn. Any parent can remember the days of having an infant when they depended on you for everything. Eventually, most babies grow up and become more and more self sufficient as time goes on. But with a child like Ella, that doesn't happen. Just as when she was a baby, she needs us to do everything for her plus the medical stuff that comes along with her condition. She depends on us to be fed, changed, moved, etc. She can't tell us what she needs or wants or what is wrong. She doesn't sleep normally throughout the night. It's very similar to having a newborn that grows bigger in size.

You hear a lot about things like "baby blues" and postpartum depression which can be hormonal, but is often also situational for new moms due to things like less freedom, lack of sleep, the worry about caring properly for a fragile new child, and changes in social and work relationships. I think it's safe to say that just like our children don't grow out of the newborn stage, us moms often suffer from perpetual "baby blues" because the situation doesn't change much as they age. It's exhausting, challenging and stressful on an ongoing basis.

I'm lucky to be connected to hundreds of other moms in similar positions as mine. It's one of the most important things to my sanity. But it also allows me to know that dealing with extreme stress and mental health issues is rampant among us. Some are able to get by without medication or therapy, some are not. We all have to do what's best for ourselves and our own situations. And I think (hope) the stigma of dealing with these issues is decreasing because no one should feel ashamed about asking for help.

We are lucky to have access to private duty nursing for Ella. She is too fragile for daycare or school. It's difficult having extra people in your house, but you get used to it. I know that I can't physically or mentally do this alone. Just as babies grow up and are able to help take care of themselves, we have to bite the bullet and realize we can't do it all by ourselves forever. It isn't natural. So when the guilt creeps in that I have a nurse at night to sit and watch my child while I'm asleep, or so that my husband and I can take a break and go out for dinner, I have to snap myself back into reality that this life isn't normal and I deserve the reprieve. It's a constant struggle, similar to those I'm sure every other mom in the world goes through. I know that "mom guilt" is universal! This is just how it plays out in our world.