Humbled.

This is the only word that I can use to describe the feeling that Lindsey and have gotten from the constant outpouring of love and support from our families and friends. I can hear my mom’s voice tell me “this is what family does” when it comes to supporting loved ones during turbulent times. I can hear my father-in-law’s voice tell us with certitude that the he “is not a religious man, but I firmly believe in the power of prayer.” I can both of my grandmothers’ voices, who I know are stricken with such sadness that Lindsey and I are going through this time, time after time, attempt to instill optimism and hope that Myla will be recover and become even stronger following her surgery. This is just our inner circle of family. Friends, colleagues, and voices from our past have also been heard. It is overwhelming at times, to even think about. I know that there is little else others can do to help Lindsey, Myla, and I navigate our way through this journey (unless one of you has a cure for a VSD or ASD that doesn’t involve open heart surgery…I am looking at you Dr. Lesneski). It’s very hard to answer that question or respond to the “let us know what we can do to help” statement. By sending an email, picking up the telephone, or text messaging, you are already doing what we need. To know you are thinking of us strengthens us.

There is nothing remarkable or heroic about what we are doing (and I am not fishing here). Much like the voice of my mother telling us that “this is what family does,” I will echo that sentiment in terms our situation. We love our daughter. It is because of the enormity of this love that we are forced to do what we have done for her. It is because of this love that we will continue to advocate for her, and its partly because of our support system’s thoughts, kind words, prayers, etc that give Lindsey and I the strength to continue to fight for our magical little Bean.

Most Surreal Countdown Ever.

Not much emotional gobblygook this evening; just some details. This Thursday we head to CHOP with our Bean for our consultation with Dr. Spray, the pediatric cardiothoracic surgeon that will be performing Myla's surgery. Following the consultation, Myla will have her pre-admission testing. This will be comprised of a chest X-ray, echocardiogram, and blood work. This is all old news for Myla as she has had multiple X-rays, echocardiograms, and a large quantity of blood taken. Two weeks from Thursday (Thursday December 22) Myla will have her surgery. We wanted to try to avoid being in the hospital for the holidays, but that is now unavoidable. Myla needs the surgery and this was the first date they had. I assume that a Children's hospital will have some festivities for the holidays, but this is in no way how planned on celebrating our daughter's first holiday season. We aren't really sure of any more details at this point. We have questions for the surgeon about the surgery, the recovery, how long we need to quarantine Myla before and after the surgery, and probably a hundred more questions will come up on our drive home from the consultation.

So today we are 16 days away and counting....16 days to love our little lady up, roll around on the ground with her, spin her in circles, and respect that fine line between squeezing her so hard because we love her so much and squeezing so hard doing further damage. We love you Myla June, you amazingly strong girl.

It’s harder because its invisible.

Well, I guess you can see her feeding tube, but you cannot see the fact that her little heart is working so hard to make the rest of her body work. Recently, Lindsey and I have seen what we call a developmental explosion with our little bean. She’s rolling over like crazy, babbling, and interacting with us so much, it seemingly masks any physiological dysfunction that may be occurring. It is this fact that made yesterday’s cardiologist appointment with Dr. Khan so difficult for us to accept. Myla has not gained much weight in the last month. As I had mentioned in my last blog, Myla has been vomiting more volume, so we had kind of expected limited weight gain. With all of her activity, we knew that she was burning a lot of what she was taking in rather than storing in the reserve tank, otherwise know has her thighs. Dr. Khan let us know that it was her opinion that we need to proceed with Myla’s surgery to repair the holes in her sweet little heart. If we continue to wait, we risk possible permanent damage to her lungs, which would lead to newer and even scarier problems. This news was not easy to hear, as our last appointment was a positive one with the news of her ASD closing by 50% and her VSD showing signs of closure. The concern, however, is not the size of the holes, but the amount of blood that is flowing through holes and the impact that the blood may be having on her longs. As Dr. Khan explained this to us, Lindsey cried, I asked questions, and Myla cued, giggled, and played with the paper that lined the doctor’s table. That picture, if it could be snapshot, is a perfect emotional translation of that moment. Mom and dad are reacting in the ways that we react. Lindsey is the most emotionally honest person I know. It is one of the reasons that drew me to her. I sometimes lean on being a dispassionate/logical problem solver when my stress is activated, and Myla is just being Myla; strong and sweet. I took the remainder of the day off of work to be with my wife and daughter and attempt to sort out the emotions that we were feeling. There was fear, anxiety, sadness, and anger. The feelings came out through tears, harsh words, and questions to whatever is out there…God…science….as to why our baby (or babies in general) are afflicted in such ways. In a previous blog, I spoke of doctors that care. I left around 3:00 to see my evening clients after making sure that someone could be with Lindsey when I left (thanks Glen). When I returned home, I found out that Dr. Khan had called Lindsey and spent about 50 minutes on the phone with her answering questions, providing insight and just supporting Lindsey. Dr. Khan gave us many words of wisdom. The words that resonated the most with me were after she had to reschedule our appointment from last week to Tuesday due to a family emergency she said, “Mrs. Roth, this not the doctor telling you this right now. It’s a human being to another human being. What I dealt with over the weekend was not curable. What Myla has is completely curable.” We are terrified, but at least our terror is related to a cure not the disease itself. Dr. Khan, you have demonstrated to us time and time again that you truly care about Myla. Thank you.

We did have one decision to make. We could opt for an exploratory catheter procedure to attempt to measure the amount of blood flowing to the lungs; however, the doctor believed the end result would still be surgery. So we have decided to forgo the catheter procedure and given the okay for Dr. Khan to set up a surgical consultation with Dr. Spray, the chief cardiothoracic surgeon at CHOP, and also schedule the surgery. We believe it will occur by Christmas. Our cardiologist suggested a pseudo-medical quarantine so to speak for the two weeks prior to make sure that Myla is not exposed to anything that would the push the surgery further back. So if you want a taste of the little Bean prior to surgery, email or call us soon because we don’t know how quickly things will move. I will do my best to continue to update this blog as it relates to our precious, magical Bean, our family, and this amazing, terrifying, loving, sad, and hopeful journey that we have embarked on. Myla, these writings will be a documentary of your strength and when you are old enough to ask mommy and daddy why you have a scar on your chest, we will read you your story.

We are just laypeople

I was planning on writing this post about all of the wrong decisions that we have made along this journey that my family has taken. Every time we tell Myla’s story, whether it is to a new professional we come across or a colleague or a friend, we rehash our wrong turns. I do not feel guilty for making these wrong decisions. Alas, we are just laypeople wandering around this quagmire with only the professionals that we entrust as our compass. We try to take the best data we are given, add it to our own expert knowledge of our daughter, and what we get is a foggy amalgam that typically leads us in multiple directions. Recently, this led us to a new feeding therapist, Nancy. Nancy seems very knowledgeable and has had a good deal of experience with “Tube Dependent” children. I just wish we had found her in July, prior to placing Myla’s NG tube. Perhaps we could have avoided that wrong turn.

Myla’s smile still leaves an indelible mark on my heart every time it shines, but recently, we have noticed a little less smiling. There is a little more whimpering, a little more whining, and a little more crying. This specifically occurs during her feedings (both tube feedings, and when we do our trials with solids). She has also been vomiting more volume, which is always deflating. We aren’t’ sure if its just a phase she is going through. One of my fears is that our smart little Bean is beginning to recognize the unpleasantness that she has to experience daily and my heart hurts thinking of that.

I recall a time when we were in the hospital over the summertime. Our family was there as were some close friends. We were celebrating the fact that Myla had just taken 3.5 ounces by bottle. This was not a frequent occurrence. Our jubilation quickly turned to despair as Myla coughed and vomited almost the entire feeding. Now, it’s more of a time and energy investment lost rather than the emotional investment back then. Her feedings, in general take about 45-60 minutes, followed by at least 30 minutes of keeping her upright. This means, even if she dirties her diaper 10 minutes into the feed, we really can’t change it for an hour and twenty minutes or so. If we violate the 30-minute rule, there is a big chance that our little Bean will throw up. We have to do this every 3 hours during the day. Recently, she has been vomiting a greater amount in terms of volume. Sometimes it seems as though it’s an entire feeding. We will see this week at her GI appointment if she continued to gain weight. If so, it’s both an inconvenience and a let down when she vomits. If she hasn’t gained much weight, then its an inconvenience, let down, and large waste of time.

Tomorrow night will be a first for us. Lindsey and I will leave Myla with her Gram overnight as we travel to New York State for my cousin Lindsay’s “A-Y’s” wedding. Though she has been left in the care of loved ones before, we have never left her overnight. We know Gram will take great care of her, but we will miss her and be anxious parents anyway. Myla does overnight very well to say the least. She was blessed as an amazing sleeper (who knows if its fatigue due to her heart or her McLelland genetics but either way, we are happy). Myla, don’t pull out your tube for Gram!

Been meaning to post this

I wrote this a couple of weeks ago, but never got around to posting...enjoy.

Did I Ever Stare?

I probably have stared, but I just don’t recall a specific time. My assumption is that most of you have stared before. Staring now has a different meaning to me. I am both sensitive to it and aggravated by it. At the same time, I understand it. Myla is 5 months old now and has a yellow tube coming out of her nostril that is taped down to her chunky little cheek, and the remaining 12 inches or so dangles behind her. The waiter at a restaurant, out of genuine concern, asked if she was okay while pointing to her nose. The older woman with a thick British accident informed us that our baby had something in her nose. I am happy to educate these curious bystanders as to the function of Myla’s Nasogastric tube. In fact, I much rather answer questions than catch the eyes from across the room. It is those who stared at the diner while we fed her that I am sensitive too. Part of this may be some distorted thinking on my part. Myla is a cute baby and people like to look at cute babies. But those who look away when I meet their eyes cause me to be skeptical.

We had some positive news a couple of weeks ago at Myla’s cardiologist appointment. We found out that Myla’s Atrial Septal Defect (the hole in the wall that separates the top two chambers of heart) had closed by about 50% since it was first discovered. This was very surprising and welcomed news. We also saw that that pressure of the blood flow across her Ventricular Sepal Defect (the hole in the wall that separates the bottom two chambers of heart) has steadily increased. This sounds like a bad thing, but is actually a good thing and is also a sign that the hole could be closing. Higher pressure indicates that there is tissue obstructing the blood flow. If there was no pressure, then the blood would be freely flowing from one chamber to the other. Myla’s body is still trying to figure out how to cope with this blood flow, so one of her arteries is mildly enlarged. This is something that we will continue to monitor. We have another echocardiogram scheduled for November, but for now, open-heart surgery is not imminent nor is it in Myla’s immediate future.

Myla is still taking no fluids orally. We continue to attempt Stage 1 baby food with her. For Myla, she still does not associate the idea of putting food in her mouth with getting rid of the feeling of hunger. We will continue this difficult work with her so that she can eventually become an autonomous eater. As always, we appreciate everyone’s continued support and positive thoughts!

On human needs....

Food is one of the most basic human needs. Eminent psychologist, Abraham Maslow, listed the need for sustenance as primary on his hierarchy of human needs. That is what makes Myla's feeding difficulties so perplexing. As many of you know, four days after leaving our July stay at CHOP and 5 days after the dropping the NG tube, Myla simply stopped sucking on her bottle. We were never told that this was a possible side effect of NG tube placement; however, in retrospect, it is more common then one would think. Every time that I reflect on that decision, I feel extreme resentment toward the doctors on the GI floor of CHOP for not going over the possible negative effects of the NG tube. I also feel guilty for not doing my homework, but enough about my emotional responses.

Let's get to an interesting philosophical question that has been sneaking into my synapses these days. We can all agree on the idea that eating is an extremely primal function. We need to eat to live. Interestingly enough, Myla has learned that she does not have to eat to live. Eating for Myla was either uncomfortable or plain old painful when she was eating. Even at 6 weeks, she figured out that she could eat just enough to stay alive and minimize the pain. At three months, she learned that she need not expose herself that pain now because the tube is filling her up. I think that we could also agree upon the fact that this type of learning is rather evolved. The interesting paradox here is that the more higher order thinking in this situation actually is much less adaptive for her than the primal need of eating to sustain life (as a sidebar, as I am engaging in this philosophical soliloquy, I itched my right eye with the hand that just handled extremely hot habanero peppers...not so evolved thinking there).

This leads one to the conclusion, perhaps that since Myla has lost this primal sensibility that all animals are born with, she needs to be retaught this and perhaps the only method of reteaching this is to allow her to feel hunger again. Myla now receives 6 feeds per day (every 3 hours) of highly fortified formula. If she is at all feeling hunger, it is not nearly enough to motivate her to eat. If and when (more likely when), we decide to wean her from her tube, we may have to take a leap of faith that over time, if we allow her to be hungry, that primal need to eat will kick back in, and we can hit the reset button. This process would include significantly reducing what we are giving her through her tube. This is also a very frightening prospect. She needs to gain weight to encourage that stubborn hole in her heart to close. If it doesn't close spontaneously, she needs to gain weight to be fit for surgery and to assist with a speedy recovery, but my lord, with each passing day, my disdain for that tube grows.

"I love my cereal"

I put that in quotes because it is an original lyric to a Sharon Roth classic song entitled "I love my cereal." The song was initially written for Myla's amazing cousin, Sydney (aka Syd Vicious aka Hurricane Sydney). My mom would sing it as she fed Sydney her cereal. My mother, not known for her tonality, actually concocted a very catchy little tune.

"I love my cereal..It is so good for me...I love my cereal...It tastes so heavenly."

This tune has been reprised recently as we have begun the process of introducing purees and cereal to Myla. We are not expecting miracles; however, Myla has been doing pretty well with it. We are going very slowly and following Myla's cues as to not have her develop another aversion. Tonight was amazing. Lindsey, Gram (Lindsey mommy), and I were singing and dancing all over the place, and the little Bean was loving it. She was smiling and singing and actually swallowing a lot (dare I say the majority of the food?) We are not close to being able to take out the feeding tube yet, but we are seeing some signs that we have possibly turned a corner.

Feeding Myla through her tube, on the other hand, is quite onerous. We have slowly gone up from 2.5 ounces every 3 hours during the day (4 times per day) to 4.5 ounces every 3 hours (6 times per day). The actual feeding takes about 1 hour to complete. Then, we must keep her upright for 3o minutes following, for a total of 90 minutes. This is part of our new gastroenterologist's plan to get Myla to feel hunger again. The goal is to totally get her off the overnight feed. Tonight, she will finish her last feed around 11:30pm and not eat again until around 7am.. Let's see how she does.

Myla had her follow up with Dr. Piccione, the CHOP pulmonologist. We like him. She weighed in at 12 lbs 10 ounces there (14th percentile), which must have been cool for Lindsey to see after not such a good weight gain the previous week. My hero wife has been carrying the load so to speak when it comes to the doctor's appointments. I barely have time to breath at work these days so getting out for an hour or two is becoming more and more difficult; however, there are certain appointments that I will never miss. That's it for now. Roth out.