6.27.17 Spine News

“And so, Lord, where do I put my hope? My only hope is in you.”

Psalms 39:7 NLT

Yesterday, Aimee and I met with the Orthopedic surgeon, along with the head of the Pulmonary department, to evaluate her scoliosis progression in conjunction with her lung restriction. We last met with this combo in December, at which point, the conclusion was surgery in the next 2 years. One of the x-rays that they use for tracking progression is a spinal traction. The surgeon pulls on her upper body while another pulls on her pelvis to get her spine as straight as possible and then a series of pictures are taken. These are stitched together into one long x-ray of the spine showing the inflexible curve. They used to take a photo of her flexible curve too, but it is just too ridiculous and unhelpful to see a picture of her bones slumped over in a sitting up position. 

December's spinal traction showed a fixed curve in her upper back of 45 degrees and yesterday's showed 50 degrees. This is the cut off point, the line where the surgeon says, "enough is enough." Aimee will need to have surgery to place growth rods this Fall before a further curve causes too great of damage or impairs her breathing beyond what she can handle. 

We plan to have them place magnetic rods, which will allow them to be lengthened with a magnetic remote and ultrasound every 4 months for about 3 years. After 3 years we would re-evaluate if the next size would be needed or a spinal fusion, depending on Aimee's growth. 

This is scary. Ed and feel pretty numb and terrified. 2 major surgeries in the next few months. Trying to schedule all the preparation as quickly as possible so that she can get through the GI surgery, recover completely, and still have time for the spine surgery before sick season starts. Honestly, even though I was surprised last time to hear that spine surgery would be coming in the next couple years... I was still really not prepared to hear "now". 

My beautiful, peaceful girl doesn't know what the journey holds in front of her. She doesn't have anxiety about the process. She is peaceful about tomorrow or an hour from now. Although it cannot be ignored and there is much to do in preparation, I am breathing in that peace she carries, the peace that is on her skin and in her eyes. She seeps it out and shares it if you sit with her and accept the gift. 

In other news from yesterday's appointment, we finally have a doctor who listened about Aimee's allergies and sent us for bloodwork! Every time I have asked, the response has been that there is no point, just give her meds if they help. I am excited, simply to know what is causing her this big challenge. Plus, as the doctor said, we need to know if she is allergic to dogs or other animals, things to which we could actually control her exposure. So, while blood draws are not uncommon, the larger volume ones are for Aimee. It took 3 attempts and a second tech to finally have success getting a decent vein. Yuck. Soon though, we hope to have an answer to this one. 

We also got another new inhaler prescribed by the pulmonary doctor for use during distress, which means I have 4 different inhaler treatments to use. For our original resistance and pride at having no medications, it sure seems like we have been humbled by turning into a home pharmacy. All we can do is pray for the wisdom to know what is the right step forward with Aimee. We pray that we will know when to say "yes" to medical interventions, surgeries, medications, and doctors. We pray that we will know when to confidently say "no" or "not that way". God is our only true light, the only hope for tomorrow. 

6.23.17 Process of Elimination

Poop is what it is all about lately. At least, that is how it feels around here. Yesterday we met with the GI specialist and Nutritionist. The short of it is that elimination is not going well. It seems that there has been a decrease in Aimee's neurological function in this area. The amount of times that we are needing to use a catheter for urine output is dramatically increasing as the success rate of enimas is going down. This is causing more infections and making it really challenging to leave the house. The specialist is recommending that we attempt a Cecostomy. This is both for Aimee's quality of life and in an attempt to not make her daily care decisions completely reliant on my knowledge of Aimee. Basically, in case something happened to me, Aimee's care would be more straight forward in this area. This surgery will be creating an opening into the top of the bowel and placing a tube for administering enemies from above. Initially the doctor decided that we should move ahead with a surgery that would disconnect the bowel and use a colostomy bag. After some discussion, we decided that it would be wise to try the Cecostomy (c-tube) before that more drastic course of action. However, the doctor is not certain that this will be a success in Aimee's case and, if it is a success, how long it will work for her.

The GI specialist is hoping to have this surgery done as soon as we can. However, we have to coordinate some appts over the next several weeks to meet with the GI Surgeon, the Reconstructive Pelvic Medicine team, Aimee's Urologist, and the Anesthesia team. There is a possibility that her Urologist may want to add another procedure on to this surgery to allow us to drain urine through a catheter in her belly button. There is also a possibility that the surgery may be more complicated by the scoliosis status.

Celebrating Elliot's 4th birthday

On Wednesday we met with Aimee's Sleep Pulmonologist. Aimee is doing well with the increase in sedative for sleeping. We did find out that the Neurodevelopmental doctor had written the prescription for "by mouth", which meant that we received really challenging little pills that have already broken 3 pill grinders (they usually last us 9 months or so). Thankfully the children's pharmacy will compound this medication for us to allow it to go through a g-tube and make that little headache go away. The doctor is still concerned with the condition of Aimee's nose and continues to look for other options that will work with her tiny nostrils and tiny head without touching the bridge of the nose. She also noted that we are well past due for a titration study to check that Aimee's current overnight oxygen and bipap pressure levels are sufficient. They were able to find a spot for Aimee really quickly, so we will have that done July 3-4.  It seems like most of Aimee's doctors don't like to wait around in her case for normal scheduling times.

Tuning out the PT at the previous appt.

Aimee had another PT appointment at Providence on Wednesday and did really well. In the previous appointment, I requested that no more positions/exercises be done with Aimee that do not allow her a resting spot for her head. She gets really freaked out and scared when, for example, she is over the edge of a wedge with no support. I can't blame her for that. Her head is a heavy weight for her. If you were to hand me something really too heavy for me and I had no clue how I would set it down or idea how long I would have to hold it...I'd freak out too. Aimee particularly enjoyed rolling down a long wedge during this week's session. Clearly she is a thrill seeker. :)

Last week, Ed and I met with a UW Genetic Counselor. We had an ultrasound and discussed what genetic testing is available. Unfortunately, it will be a few more years before they can test the maternal blood for just any genetic disorder, which means we would have to do an amnio to find out prior to birth. We are not willing to take this risk (1 in 400 chance of miscarriage with an amnio currently). Instead, the midwife will be able to pull blood from the umbilical cord after delivery and send that to the lab to look specifically for KCNQ2. We won't know as soon as we had hoped, but it will allow us to find out risk free as soon as possible.

Otherwise, the ultrasound showed a very healthy little baby BOY! I sure do like have brothers and sons around me throughout my life. :) Caleb is suggesting names as often as he can. Little Couscous (the current favorite on Caleb's name list) is very active and we are looking forward to knowing him better.

6.7.17 Update

The past 2 months of silence here do not indicate a period with no events, rather one where I was too challenged to give you updates. Now that I am feeling well again, here is the reason for my silence, we are anticipating a family addition in early November.

With our previous pregnancies, we did not yet have an underlying diagnosis for Aimee. This time around we know and actually have the ability to test for this particular gene. Although both of our blood was tested showing that we were not carriers of KCNQ2, there is still a possibility of a mosaicism in one of us (where some of our cells carry a different set/arrangement of chromosomes than the rest of our cells). We have decided to meet with a genetic counselor so that if this little one has KCNQ2, both us and the hospital can be prepared for major seizure response in the first couple days of life. If the baby does not have this gene alteration, we can relax our seizure watch. We will be meeting with this counselor early next week to discuss the testing. At this point we do not know if the test is a big blood panel or if it is an invasive test, which would cause us to reconsider our decision. As a part of this meeting, we will also have our 20 week anatomy ultrasound and see this sweetheart for the first time. 

In Aimee's life things have not been quiet either. Over this past couple of months the biggest difficulty has been with allergies. This year is challenging in our area, because of the delayed Spring, causing so many pollens and grasses to be an issue all at once. Every year Aimee seems to have a harder time managing as well. She is on high amounts of allergy meds and still having difficulties on the high count days with reflux, bleeding eyes, and oxygen levels.

During this time Aimee has met with multiple specialists, including a new physical therapist through Providence. This evaluation was so scary that Aimee sobbed for about 9 hours afterward.

Her brothers surrounded her with their favorite stuffed animals to help her feel better. The therapist said that currently Aimee's biggest fight is gravity. Every part of her body has grown heavier and her muscles have not been able to keep up with it. Lately it feels like her body is being rapidly twisted.  We are trying to do a better job of getting Aimee time everyday on her left side to help her breath and to attempt to slow this twisting shape.

Aimee also added these new arm braces to her daily routine. I rather hate bracing up both her feet and arms, but do want to avoid any surgeries that we can for the future. 

She has had two addendums added to her IEP. We were disappointed to learn that after evaluation for both vision and physical therapy at school that she will only receive consult services for each. However, we are grateful that she will at least have consult added. 

Aimee has also started a sedative medication to attempt to regulate her sleep schedule. While it initially caused disturbing effects, she is currently sleeping as well as she ever has. She has not woken up in the middle of the night (aside from desats) or been up all night in a month. We hear that this may not last, but it has a potential to give us all a couple of years of better sleep. We will take it! This consistent sleep has increased her awake time at school from 50% to 75% and overall improved her alertness.

Looking forward to a beautiful season ahead with Aimee smiles, silly sounds, and contagious laughter. I sure love this girl.