It has been a long day! We had to leave our house by 6:45 to get to the hospital for an 8:00 AM check-in and we’ve been going ever since. I was shocked by how nervous Little was and by how it showed … she didn’t talk! That’s right, SILENCE was the coping mechanism of choice.  I was perfectly calm until she started doing that and then I found myself freaking out a bit.

Then they gave Little Valium to help relax her and all of a sudden her lips were loosened and she felt free to share how nervous she was. The medicine continued to kick in and she started crying too. Then more people started piling into the room … a tech started an echocardiogram, the anesthesiologist explained what would be happening and then asked the tech multiple questions about what was showing up on the echo, the cardiologist came in with two teenagers who were shadowing her today and then the nurse came in to see if we were all ready. Little closed her eyes and shook.

At that point Dr. Miyamoto asked me to join her outside the room and she proceeded to prepare me for the worst. She explained that she was nervous about the procedure and promised to stop at the first sign of a problem. She explained what steps they would take if Little’s heart were to stop beating. Bottom line? She freaked me out. I held it together long enough to dress up in a surgical “bunny suit” (think Stay Puff Marshmallow Man) and be with Little as she fell asleep and then I headed to the waiting area where I closed my eyes and shook.

The one hour procedure took 2.5 hours and then Dr. Miyamoto joined me in the waiting room, her head was shaking as she sat down beside me. So, she was fully expecting to go in and find conditions in the heart that would mean our follow-up conversation would be all about heart transplants; turns out nothing was as bad as she was expecting. It seems Little’s “sinus node” is broken — that’s her God-given pace maker. They put in a lead through her groin and up to her heart, set a healthy heart rate/pace and watched as all her numbers evened out and she began to look … WELL. Recommendation?  A pacemaker to be put in early next week. (As in, potentially Tuesday)

The question remains as to why this is happening. No one is sure if cardiac disease caused the sinus node malfunction or if the sinus node malfunction caused the cardiac weakness. A biopsy of the heart was taken during the catheterization today and will be tested for disease. A genetic panel will be done too to see what shows up on that front. There is one other test they want to do, a special type of EKG, but that will be done at a later date — a pacemaker takes precedence.

We are spending the night at the hospital tonight and there has been discussion about keeping us here until the procedure next week (it is NOT a surgery). There is also discussion about how it is silly to keep us here for that long. Little and I are learning together about having an open hand.

Thank you for praying for us. Little’s heart keeps leaving the medical staff completely flummoxed and I truly believe the anomalies are results of prayer.

During these two months I have often found myself on the verge of freaking out. There have been verses I’ve quoted and Truth I’ve claimed but I want to leave you with one song I keep singing over and over. And then I’m off to close my eyes … and sleep.

“But I know whom I have believèd,
And am persuaded that He is able
To keep that which I’ve committed
Unto Him against that day.”

Daniel Whittle

 

Anyone who knows me will hear the phrase, “I like setting goals” as an understatement. The amount of joy I get from making a list of plans is truly ridiculous. It’s my favorite thing about New Year’s day and a refreshed list is the reason I don’t mind Monday mornings. I’m  not sure when this love started but it’s been going on for a loooong time.

Little’s atypical development first became evident before she was even four months old. She was diagnosed with Agenesis of the Corpus Callosum at a year and setting goals took on a new level of intensity. I would sit down every six months and pull out the book that listed developmental milestones. I would write down the next set, or move forward those that had yet to be met, and think through ways that we could work with Little to move her forward. Initially there were a lot of phone calls to organizations and therapists who were then able to give us clear directions and specific actions that would help our Little
as she grew and developed.

Together with her speech therapist we taught her how to chew. Together with the physical therapist we taught her to crawl, sit, stand, and eventually walk. There was occupational therapy, listening therapy, aqua therapy, music therapy, developmental therapy, and hippo-therapy (horses)! For years there was always another goal to meet, always another milestone to aim for, always a plan to work.

This past January I sat down to do my prayer and planning time and I felt convicted about the lack of effort I had been putting into the development of my children. 2017 felt like such a waste of a year; back pain had kept me laying flat for most of the duration and I felt my family had paid a high price. I prayed. I planned. I wrote pretty goals. . . somewhere.  I can’t for the life of me remember where I put all this work and beauty. I only remember that I told Jeremy, “I really feel like Little has been floating along and I have lowered my expectations for her.  This year needs to be different.  I want to be more purposeful and more present when it comes to Little in 2018.” Good grief.

The irony that I can’t find my plan for 2018 is not lost on me; suffice it to say that the reality looks nothing like the dream anyway. Each week I find myself sitting with my planner and a list of phone calls that need to be made; calls to people who can give us clear directions and specific actions that will help our Little in this new stage of growth and development. I find myself needing to be very purposeful and remarkably present! Where there were once therapists of all shapes and sizes, the calls now include Cardiologists, Orthopedists, Dentists, and Medical Supply companies! And just as the Lord put people the right people for the right time into our lives in all those early years, He is leading us to His right choices once again.

Little had her MRI on April 10 and the Anesthesiologist/Cardiologist was unbelievable.  He spelled out all the risks for me and spoke with compassion and transparency.  It was amazing to hear what he would be doing over the course of the “study” (stopping Little’s lungs so that clear images could be taken of her heart and then restarting her lungs to allow a couple of minutes of breathing before stopping them again, etc.). Hearing this had the potential to be terrifying but he calmly assured me that my daughter would be under his constant watch and care. The “study” was supposed to take 1.5 hours but ended up taking three and he made sure to keep me updated and then came out personally to fill me in on her condition and how everything had gone. I had been watching and that had NOT been happening for others in the waiting room.  Dr. Nichols was HIS right choice for us.

The Director of the Cardiomyopathy Program was on call when Little was first admitted to Children’s back in February and so she has been on Little’s case ever since.  During the MRI, Dr. Miyamoto came to check on Little twice and even sat with me for a bit to talk about how I was doing and how I was feeling about where we are in Little’s treatment.  She is HIS wonderful right choice for us and you can “meet” her here:

We had our check-up with Dr. Miyamoto this past Wednesday and discussed the results of Little’s MRI.  The brain scan showed that she still has no corpus callosum *wink*. The scan of Little’s big heart confirmed that the left ventricle is operating at 25% and the right ventricle is operating at 30%. The characteristics that they were looking for to determine if she had a specific heart disease were not present so… she does not have Right Ventricular Hypertrophy and we are glad for that. This does mean that we still don’t have any more understanding of what and why this is happening and during this visit the doctors became more concerned about Little’s lungs.

The phone calls I have been making this week have included calls to the scheduler at Children’s so that we could schedule a Heart Catheter. There have been calls to several PA’s in the heart clinic and we have been working with Little’s meds to try to get excess fluid out of her lungs and get her ready to be put to sleep again this Friday. They will insert the catheter through an artery in her hip and follow it up to her heart.  The purpose is to measure the pressure in her heart and her lungs. There are risks that come with this procedure, the anesthesia being the greatest one. Please pray that Little’s heart will maintain a steady beat and that the doctor will be able to get a quick and accurate reading. (We just found out today that Dr. Miyamoto will be doing the procedure and my heart rejoiced.)

We are taking a bag with us on Friday. The doctor says that the procedure could be quick and the results could be encouraging which would end in us going home. Depending on the pressure readings, we could end up staying at the hospital as they consider treatment and next steps. Either way, the doctor believes that this “heart cath” will enable us to have a better picture of Little’s prognosis and help determine clear directions and specific actions as we continue to walk through 2018.

More calls have been made and more will be made tomorrow but this post has gotten exorbitantly long! Remember to pray for Little when you brush your teeth… and in that vein come back to read a great story about a phone call with HIS right dentist.

SaveSave

It’s spring break here in Colorado and I am so thankful for the slow pace and days to adjust. I feel that the weather has been an expression of His mercy: the sun has been out all week and the sky has been clear. Little and I returned to Children’s yesterday for a check-up; my hope being that what we would learn would share the same clarity. Here’s what we learned:

1. It quickly became clear that 30 min. of extra driving time is NOT enough to compensate for morning traffic in Denver.
2. When dealing with doctors, there is a noticeable difference in one’s confidence level if you have just woken up after a night on the hospital couch or if you get ready at home and come into the hospital dressed like a human and wearing makeup.
3. In the past three weeks Little has lost 14 pounds. Clearly it was time for that to stop; medications were adjusted.
4.  We met with the head of the Cardiomyopathy department and it was so helpful for understanding what is happening inside Little. The doctor I talked with yesterday is the same doctor I met when Little was first admitted at Children’s and the things she said yesterday were the same things she said Feb. 26. It is much easier to understand a doctor when you have slept and aren’t in shock over a diagnosis. Here’s what I now know… The typical presentation of Dilated Cardiomyopathy shows a heart with an enlarged left ventricle that has decreased function; the ventricle fills with blood but the “squeeze” isn’t strong enough to pump everything back out.                                             The thing that makes Little’s situation extra “interesting” is that her left ventricle is shaped just like it should be but has decreased function; it’s hear right ventricle that is very large and it’s function is also decreased. According to the doctor this doesn’t really fit any other myopathy scenario she can think of.  (We clearly like to keep life exciting.)
5. Usually patients with this diagnosis have elevated heart rates.  The heart’s squeeze is weak so usually it beats more times a minute trying to get everything out like it should.  Not our Little’s heart! Her heart is beating a steady and slow rhythm. Every time a doctor listens through their stethoscope they shake their heads and mumble something about how good her heart sounds. She wore a monitor that recorded her heart for 24 hours and it showed that her heart rate could get up to 100 bpm; the doctor was encouraged by that but continues to be confounded by the slow steady beating as the norm.
6. Phlebotomists lie.
7. Because Little’s symptoms just don’t “add up,” the doctor wants to pursue more tests. They hope they can get some clarity by doing a MRI…both neuro and cardio. The MRI will give a clearer view of what’s going on inside and by doing an updated neurological scan we can see if there is a connection between her special brain and special heart.

So, nothing so new that we are reeling or rejoicing.  It was helpful for the sake of clarity. I am grateful.

Things to pray as we continue along:

1. Pray that Little will get her appetite back. She is really only eating one meal each day.
2. Pray the MRI will allow the doctors to see what is causing this strange reaction in Little’s heart and that the next step will be clear.
3. Pray that I will live TODAY and let tomorrow take care of itself.
4. Pray that we can get the mask Little needs for her oxygen and that she will keep it on her face. I have not been sleeping because of the anxiousness that comes with knowing she keeps taking out her oxygen.
5. Pray that starting back to school next week will go smoothly – for all three children but especially Little.