It has been nearly two months since my last post. During that time, I've kept saying to myself over and over again, "you really need to update the blog," but truthfully I just haven't been able to get around to doing so. It has been really unreal. Seriously.
A big reason for not updating the blog has been a simple lack of time. May and June were hurricanes, literally, of information and knowledge, twists and turns, and ups and downs in our lives. I relate the past two months to a hurricane for many reasons. The information and everything that has happened in the past two months has felt like torrential rains that have continued to pour down upon us, and in fact we feel for the most part that we are just treading water to keep from drowning. We are just going through the motions to keep our heads above water. At times, the rain was mixed in with other things coming out of nowhere, like swirling hurricane force winds. We were getting hit in all directions with what seemed like no shelter from the storm. Then, at other times, it was calm, and we would have a bit of peace. We cherish those bits of peace, but with hesitation. The eye of the storm is always calm, and it never seems to last long, which is also true for us. After the calm comes the storm, always. Always. The past two months have felt like a perpetual storm to us. A perpetual, epic storm. It has been really unreal.
The other, and slightly more covert, reason for not updating the blog is that with all of this information, all of the events, and at times barely being able to catch our breath for many reasons over the past two months, I simply don't know what to say. As a writer, this is a nightmare. I feel like sometimes I'm constantly writing books in my head. In fact, I keep a little notebook in my purse where I'm always writing down lines, sentences, etc. as I think of them. I don't know when or if I'll ever use them, but I don't want to forget them. That all may seem very weird, but that is just how my brain works. Its how I am wired. Some people think in numbers, and logic and science, and I think in words and phrases. However, the hurricane that has been swirling around in our lives in regards to Elizabeth the past few months has not only left us breathless, but it has left us a bit speechless. Maybe it is simply exhaustion. Maybe it is simply just not knowing what to say, or how to say the things that we know and have learned, and have experienced in the past two months. We need to wrap our heads around things, process it, and in some ways that process is unfinished. When I publish the info, talk about it, it is sometimes like rubbing salt in a fresh wound. Sometimes, it is like a big bandage, though, like a release to get that all out. It has been really unreal.
So, despite the hurricane, it is time for an update. I'll divide them into sections. There is just so much to cover. I won't go into every tiny detail, because it would be boring, and in many ways, I just can't. Much of what has happened and what we have learned since the beginning of May has left us pretty battered in a lot of ways. I personally feel like I have aged 10 years in the past two months. We survived the storm, and will continue to do so. We have survived this storm, and we will survive many others to come our way. Somehow. Anyway, updates:
Vision:
The last post on the blog was about Elizabeth's vision regressing, and this is still a concern. After her brain MRI in October, when her small optic nerves were noted and other forms of brain damage in the region of the brain responsible for vision, and then after a visit to a specialist in the field, Elizabeth was diagnosed with CVI (Cortical Vision Impairment). This essentially means that her brain does not interpret visual stimuli as it should, and so she does not have normal vision, because of the delay and lack of processing of her vision by her brain. Most kids with CVI have normal eye exams, meaning that when they are evaluated by an ophthalmologist, their eyes appear to be functionally normal, and they do not need glasses, so their inability to process visual input is often baffling. However, it is clear that Elizabeth needs her glasses. So, once again, she doesn't exactly fit into a diagnostic peg, per say. She meets the criteria for a CVI diagnosis, but we now know that she is not a true case of CVI. She is not a true case of CVI, because there are clearly structural and other issues with her eyes that prohibit her from having normal vision. She needs her glasses to see. Her vision is regressing, and in May, which was reflected in my last post, her glasses prescription had to be dramatically increased in a short period of time due to the regression of her eyesight. So, we got new glasses. She chewed them terribly, and we ordered another pair.
I'm going to take a minute to give some praise to Dittman Eye care in Butler, PA. We order Elizabeth's glasses through them, and I am also a patient there. They are just wonderful, and I have nothing but excellent things to say about the staff and their service there. I call them, and they just order a new pair of glasses for Elizabeth, no questions asked, and they are always so kind and helpful. I really appreciate it, and I highly recommend them!
Ok, back to her vision... so at her appointment in May, it was recommended that we see a low vision specialist by her Ophthalmologist due to the rapid loss of vision. So, I made Elizabeth an appointment with Dr. Joseph Paviglianiti (Dr. Pav), who was highly recommended by Elizabeth's Teacher of the Visually Impaired and her doctors. At the appointment, Dr. Pav evaluated Elizabeth, and he agreed that she clearly needs her glasses. She has a combination of strabismus, amblyopia, and nystagmus, which are all issues that involve the eye muscles that impair vision. She also is very farsighted. All of these issues combined require her to wear glasses so that her eyes can work as a team, and so that she can have functional vision. Dr. Pav also said that he does not find Elizabeth to be a clear cut case of CVI, but he could see qualities of the diagnosis within her vision. He agreed to write a letter stating all of this information, so that it would help us to ensure that we would be able to help her to continue to receive high quality vision interventions for Elizabeth. Dr. Pav agreed that she clearly needs to continue to receive visual support services. He also agreed that her recent prescription in her glasses was the correct one for Elizabeth. Dr. Pav felt that her vision issues were related to some type of genetic/ ophthalmologic issue, and he recommended we get her into see one of two leading experts in Genetic Ophthalmology, one of which is at Children's in Pittsburgh, to have Elizabeth evaluated by one of them in regards to her vision. We ended up being able to get an appointment with Dr. Nischal at CHP with a little help from Elizabeth's Diagnostic Referral Team.
The last thing to mention about her vision is that we did have an evaluation at the Western PA School for Blind Children. It was an amazing experience, and we did receive a lot of information that we passed on to her Early Intervention Team. This is our number one choice to send Elizabeth to for at least preschool, and she can stay there for longer if it continues to meet her educational needs. It would be a great fit for her, and we will be taking an official tour soon. She would start in their preschool program upon her graduating from Early Intervention when she turns 3.
G.I. Appointment:
The next big piece to May was Elizabeth's follow-up appointment with her GI doctor regarding her G-Tube surgery and her weight gain. Elizabeth gained very little weight with her G-Tube. It was supposed to have the opposite effect, to say the least. In fact, May brought about a downward spiral of weight for Elizabeth. She actually was losing weight, lots of it, and it sparked lots of phone calls between her doctors and I. I could see her ribs. She was becoming lethargic. It was terrible to watch. Terrible. We have waited and prayed for so long for her to just thrive and grow, and despite of trying numerous ways to get her to gain weight, she was literally wasting away in front of us. We knew there had to be something we were missing, something more we could do, but we just didn't know what that would need to be.
At the GI appointment, Dr. Lindblad was also concerned about her weight loss. He agreed that with the formula and caloric intake she was currently on that she should have gained pounds by now. So, we decided to do some scoping, of both ends, to see if we were missing some type of disease or anomaly of the digestive tract that could be impacting her absorption of nutrients. He also said that if she did not show improvement in weight gain soon, and if the scoping showed nothing out of the ordinary, that he was going to recommend a G/J tube be placed in Elizabeth. Dr. Lindblad said that some kids just don't respond to G Tubes and need to be fed directly into their intestines with a J tube. So, we made a plan. He wanted to see her in a few months to reevaluate and at that point would make the decision on the G/J tube. Unfortunately, the scoping couldn't be scheduled until the end of June, and her follow-up appointment could not be scheduled until the end of August. That seemed unreasonable to me, especially with her recent downward trend, and so I started making phone calls.
Weight Checks, Phone Calls, and a Plan:
I just couldn't stand by and watch her lose any more weight. It was clear the G-Tube wasn't working. Why? Well, no one was quite sure. I just knew we were missing something. That nagging and relentless "mom" instinct just would not let go of the fact that something was just not right with my baby girl. I refused to let her wait until the end of June for scoping and the end of August for a determination on the G/J tube. By that time, if the weight loss continued at her current rate, she could weigh next to nothing. She basically already did weigh next to nothing. She was hovering at 18 pounds. So, I called the pediatrician, and we agreed to check her weight over the next week, and if she did not gain that we should admit her to get to the bottom of the weight loss and lack of weight gain. I also called her Diagnostic Referral Team, and they agreed with that plan, and they also filled GI in. Everyone was on board. Elizabeth lost a pound and a half over the next week, and at my phone check in the day after Memorial Day, Dr. Brown made the plan to admit her the next day. We were going to get to the bottom of why she wasn't gaining weight. The current plan was not working and could just not continue.
17 Days on the Inside:
We were admitted on Wednesday, May 27th. While I am happy to report that the admission was ultimately productive, there were some scary moments during that time. Also, I try to be pretty positive and pleasant, but there were a few times that I lost my patience with the doctors who were rounding on Elizabeth from the Diagnostic Referral Division. The times when I lost my patience, it was mostly because they kept trying to put Elizabeth into a paradigmatic, typical, normal or usual box. She is none of those things, and no doctor is going to find her on page 387 of their Pediatrics 101 textbook. This isn't just because she is my daughter, and I obviously think that she is special. It is because that she simply does not fit into a box of any kind that is labeled "normally, usually, typically," and she never has ever fit into such a box. Sometimes you need to think outside of the box, especially with kids like Elizabeth, and once we were all on the same page regarding that it all went beautifully, and there was progress.
I stayed the entire time with her. I was there day and night by her bed. Occasionally, I would venture outside of the hospital, mostly because the nurses were telling me just to get out for a few minutes, but mostly I was right there. Whenever she was having a good day, or a good few hours, we would walk to the Healing Garden on the 6th Floor. We also spent some time in the Austin's Playroom and the Sensory Room on the 6th floor with the Child Life team. The Child Life Staff at CHP is amazing. There were several strings of nights, when she had uncontrollable projectile vomiting or terrible coughing spells, that were so scary. They literally took years off of my life. She was choking on her vomit, turning blue. She was coughing so hard that she was wheezing and turning blue. I'm pressing the call button with one hand, and holding her up and trying to help her clear it out to keep her from choking with the other hand. She was so sick. So sick. It is terrible to sit helplessly beside a child that is so sick, especially when it is your child. I missed Celtan, but I needed to be there with her, as her mother and her advocate. I shudder to think what would have happened if I had not been right beside her bed, because sometimes it was minutes before anyone answered the call and came into the room. When she is laying down and starts to choke or vomit, she is not strong enough to sit herself up, nor is she strong enough to effectively clear out her lungs or cough up the vomit, and so she just lays there and gurgles and chokes. If I hadn't have been by her side to react quickly, she definitely would have choked to death. There's no doubt about it.
We were able to get her scoping done as an inpatient, and I'm happy to report that everything looked normal, and all of the biopsies were normal. We also decided as a team to determine whether or not she should eat by mouth at all, which is a point of debate between different members of her medical team. After review of her swallow studies, it was determined that due to her numerous swallowing and anatomical issues in her mouth/ throat, that she should not eat by mouth simply because it is not safe. She also works so hard to swallow that she was burning more calories that she was taking in, which also could explain the weight loss.
It was eventually determined that Elizabeth was going to need a G/J tube. She wasn't gaining weight, and her scoping results were normal. Before we tried the G/J tube, we tried G-Tube feeds only into her G-Tube, but she did not tolerate those at all. She developed a nasty cough, due to reflux and her inability to control the reflux due to her low muscle tone. Her cough was so bad that she had to be put on cough assist, which is unpleasant to say the least. The next step was to take out the G Tube and put in a G/J tube, which would mean that she would now be fed only into her small intestine, instead of into her stomach. So, they put the G/J tube in, and as they increased the rate, she began to have uncontrollable vomiting and diarrhea. Her body, her weak tissues and muscles, simply could not sustain a high rate of feeds. This led to the determination that she could only tolerate a slow rate of feed, which would mean that in order to get her required calories, and sticking with the rate that she tolerated without any nasty side effects, that she would need to be fed 24 hours a day. Obviously, it is our hope to slowly increase the rate of feeds, and maybe get her to a point where she does not need to be fed or attached to a pump 24 hours a day. However, time will tell, and Elizabeth will tell us if that is possible. It is also our hope that one day we may be able to transition to G tube only feeds or oral feedings, but time and Elizabeth will also tell us that. In theory, with the new constant and consistent nutrition and growth, she will become stronger. As she becomes stronger, she should be able to tolerate feeds and manage her reflux more effectively, but no amount of getting stronger is going to correct the numerous anatomical issues in her throat and mouth, which are huge contributors to her swallowing difficulty and safety. Some of those can be corrected by surgery, sure. However, much of her muscle weakness is due to her many neurological issues, and unfortunately, those are not easily, and often not at all able to be, corrected. So, even though our hope would be that one day she can eat like everyone else, and taste all of the delicious foods and you and I enjoy, we also know that it simply may never be a possibility for her. Time will tell. For now, we continue with the current plan, 24/7 feeds, and hope for the best.
Also, because Elizabeth is a true "failure to thrive" child, and her body simply does not understand hunger, or that it needs to eat, it was producing large amounts of gastric acid, even after she was no longer getting any food fed into her stomach. After 5 nights of vomiting and choking and having a drain attached to her stomach to drain all of her gastric fluid, it was determined that she needed a different cocktail of reflux meds to keep the gastric acid at bay. She is now on a Proton Pump Inhibitor, which basically tells her body to stop, or slow down the rate, at which it produces gastric acid. So far, with the slow rate of feeds, plus the magical cocktail of reflux meds, we do not have vomiting or diarrhea. She sometimes has a little bit of diarrhea, but that is normal considering that she is fed into her intestine. The fight to get the team to agree to this type of med was totally unreal, and their reasoning to me was that they know that it is usually an issue with the insurance company to get the PPI meds covered. My response to them was that they obviously did not understand my relationship with the insurance company, nor did they understand my track record with them. Once they tested the pH of her gastric acid, and the result was a highly acidic pH level, 2 to be exact, they realized that they had no choice. It was no wonder she was pulling at her ears, and her eyes were watering, and she was miserable. A pH of 2 is acidic enough to burn a hole into a piece of wood. Her entire chest and throat probably felt like it was on fire from all of the acid she was producing and throwing up and choking on. The doctors were right that the insurance company denied the drug cocktail, but after a few phone calls and me once again having to turn into basically the Hulk over the phone, they were covered by 2 p.m. the same day I started calling the insurance company. That increased my record of victories against them. I would really love to see what the notes in their computer system say about me and all of the phone calls that I have had to make to them to dispute denied coverage about this and that for Elizabeth.
The other important piece to the hospital visit was that we were able to pull Endocrinology in to consult on Elizabeth. At our team meeting at our admission, we decided that we also wanted to look into her growth hormone levels, because that may be a piece that we are missing in relation to Elizabeth's poor and very slow growth. They tested several different types of growth hormone levels, and there appears to be a small issue at this time with Elizabeth having low levels of growth hormones. They are low, but not super low, and so at this time, they are not going to start growth hormone therapy with her, but Endocrinology does want to follow her closely. The growth hormone piece was also important, because it helped us to receive our first major diagnosis: Septo Optic Dysplasia. This is a diagnosis that has been mentioned several times with Elizabeth, but we now know that she has all three diagnostic criteria of the diagnosis. It explains her growth issues, as well as her vision issues. There are other pieces to Elizabeth that this diagnosis does not explain, and so we wait for other diagnoses to be confirmed to fill in the spaces. In the meantime, Endocrinology is going to follow her every couple of months to check her growth hormone levels, her growth, etc. Even though she does not need growth hormone therapy now, it is likely given her current levels that she will need that in the future, especially as she gets closer to puberty. Here is a link if you'd like to read more about Septo-Optic Dysplasia:
http://ghr.nlm.nih.gov/condition/septo-optic-dysplasia
As per requests from a few of you who do not follow me on Facebook, I will post my status updates from our time in the hospital in a different post. They may make you laugh and will also give some more detailed information about our stay. I wouldn't have thought to post them here, but I had some friends who are not on Facebook who asked if I could, because they heard they were worth reading. So, I will do that. I guess sleep deprivation and stress are great inspirations for writing... who knew?
Anyway, I am very grateful to her amazing team of doctors. I am also full of love and gratitude to the many family members and friends who came to visit us, sent cards and gifts to brighten our day, who brought food for me, and who continuously checked in with me to show their love and support. We are truly blessed. We were on the news several times, because they filmed Elizabeth and I at the birthday party for CHP that took place while she was in the hospital. We also had a lovely surprise and Queen Elsa herself visited Elizabeth in the hospital thanks to the Dittman Family. It was certainly the highlight of our stay, and the pictures are priceless. She kept looking at Elsa, smiling, and Elsa gave her a crown, which she wore quite frequently throughout the rest of her stay. We also came home to many unexpected and very generous donations from so many friends, family and organizations in our community. We were totally overwhelmed by the generosity. We are truly blessed with many amazing people in our lives, and we are so grateful. Seriously, it has been really unreal. The generosity, kindness, and so many other good things keep pouring in for our Elizabeth and for our family. We will never be able to repay all of this, but we will pay it forward. We will never be able to thank you all enough for your love, generosity and support, but we will continue to thank you repeatedly forever and ever. You will never know how truly touched we are by everything.
We were discharged on June 12th. It was great to be home again as a family. I can't even begin to express how much I missed Celtan. It is hard when your heart is torn between two places. I needed to be with her, but I also wanted to be home with him. All of these appointments and hospital stays have negatively impacted him, and he cries when I leave to take her to appointments. He doesn't want me to leave, and he always is convinced that I am not coming back. I can't leave his sight for five minutes without him throwing a huge tantrum. He has clear separation anxiety, and it is no doubt that everything that is going on with Elizabeth is the reason why. It is heartbreaking to see Elizabeth struggle, be so sick, to require so much extra care, but it is equally as heartbreaking to see how this has impacted Celtan. Every time he hears me mention the word "hospital," he asks if I will come back. Since my pregnancy with Elizabeth, he has been subject to being away from me for long periods of time due to hospital stays. First, I was admitted for bed rest and observation for the last month of my pregnancy with Elizabeth. Then, she was in in the NICU for almost 6 weeks. Since that time there have been a handful of hospital stays and admissions for Elizabeth, and I go with her to be by her side while he is left behind. It is terrible. Truly terrible. It is not fair. I get that life is not fair, believe me, I get it. I totally get it. So, now on top of all of Elizabeth's needs, I'm trying to resolve the anxiety and restore the collateral damage to Celtan that all of the events with Elizabeth have caused for him. It can be done, but it will take time.
Anyway, Elizabeth is fed 24 hours a day through a pump. We follow her around with her IV pole, untangle her from lines, give meds, are now conditioned to run by the beeping of the pump, and all of that good stuff. Basically, on top of all of my other degrees, work experience, etc., I am also a full time nurse. I have been training Jason on what to do when the pump beeps and shows this message or that message, what meds to give when, how to give different meds, things to look for, emergency procedures for if she pulls out her feeding tube, etc. I am home with her the most. Even if I decide to go out with my friends or family for a few hours or for an evening, even if Jason is staying home with the kids, I still make sure before I leave that all meds have been given, her bag has been changed, and that she is basically ready to go. When you have a child that is attached to medical equipment 24/ 7, it changes the dynamics of your life, but we are making the best of it. Truthfully, I think Elizabeth enjoys the constant parade that follows her around. Someone always has to have her IV pole or backpack nearby, and I thinks he feels like a princess that has a parade of helpers following her. It is pretty funny to see her smile as we follow her around.
We are getting the hang of going out of the house with the pump in our backpack. It is a team effort, and can be quite the process. I just refuse to be confined in my house. My wandering spirit cannot be confined into one location for long. I get restless. I need to be out and about. Plus, I want both Celtan and Elizabeth to see and experience so many things that await them beyond the doors of our house. Celtan loves to help, and it is good for him. I often feel like he knows and deals with too much for only being 5. However, in other ways, I feel it is good for him to feel like he has a place in all of this.
Elizabeth doesn't seem to be bothered by any of this at all. She is much more energetic. She is definitely interacting more, and it is great to see her finally grow. She is getting chubby cheeks and thighs, and I can barely see her ribs anymore. She is gaining weight, still slowly, but we are definitely on an upward trend. The hospital stay was really unreal, but it has definitely brought about a lot of information and the outcome we were hoping for... a plan for Elizabeth to grow and thrive. We have been waiting for two years to see her grow and thrive, really and truly grow and thrive, and finally we are seeing that. All along it has been clear to me that we were blessed with a miracle. A beautiful miracle that continues to unfold right before our eyes. She has defied the odds numerous times in two years, and she still continues to do so. We could not be more proud of her, and we continue to watch her amaze. It has really been unreal.
