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!