Sunday, December 31, 2006

A Song for Emily

This is pretty neat! Songs of Love is a non-profit org that created a personal song for Emily. We filled out some paperwork and sent it in -- Just click here to download Emily's song.

And here are the lyrics:
Emily watches VeggieTales,
Just like Linnea used to do,
She plays with her pacifier,
And watches Dora too.

She grabs at Mommy's glasses,
She grabs at Daddy's tongue,
Annabelle will play and dance,
It's a lot of fun.

And Annabelle will sing,
And she like to whistle too,
Emily likes to watch her,
It's a favorite thing she likes to do.

Emily Hope Lee,
She's one beautiful sister,
She's one of three and I'm sure you see,
No one can resist her.

When Linnea would go for walks,
She went with her red wagon,
Emily too will walk it's true,
And with Cindy Ah-yee she plays peek-a-boo.

Emily Hope Lee,
She's one beautiful sister,
She's one of three and I'm sure you see,
No on can resist her.

We call her Emily-bemily,
We call her Sugar-booger,
One thing's for sure, it's easy to see,
Everyone loves Emily.

Emily Hope Lee,
She's one beautiful sister,
She's one of three and I'm sure you see,
No on can resist her.

Emily Hope Lee,
She's one beautiful sister,
She's one of three and I'm sure you see,
No on can resist her.

Monday, December 25, 2006

Christmas Pics

We were adopted for Christmas. WOW! I don't even know what to say but THANK YOU!! Thank you to everyone who made this such a special Christmas for Annabelle, Emily, Diana, Cindy, and me. Here are some pics of the last couple of days from a Santa visit to presents on Christmas morning. Thanks! :)

Emily's Christmas Morning :)
Annabelle's cache :)
Yummy... videos
What "Santa" left
Helping Emily
Santa, Annabelle, Amanda, & EmilyLiu Family
Pwau-pwau & Emily
One happy Cindy!!
Beer mug, YEAH!!
Now that's a smile :)

Wednesday, December 20, 2006

Scenes from our life at Johns Hopkins Hospital

Mommy Make-over Time!!

Monday, December 18, 2006

High Numbers

Well, that title means a couple of things to me. Emily's O2 saturation has remind in the high 90's for a few days now and we are actually starting to get some rest. More accurately, Emily's incredible super-mom is getting more rest than even a few days ago. In case anyone was wondering, that is a good thing. The other thing that it means is that we have had over 9000 visits to this website! There are a lot of people checking up on Emily and our family, what an amazing thing to think about. As we care for Emily we sometimes think about all the people that care about her and the rest of us. For me it's kinda like when you are trying to think about how many stars there are in the sky and you end up getting side-tracked and start thinking about something else b/c you can't REALLY think about how many stars there are... well, I get side-tracked like that when I try to fathom the amount of people that are thinking about and praying for us. Thanks.

A couple things to note:
  1. Diana and I were able to leave Emily home with her incredible Ai-ee (Aunt in Mandarin), Cindy, to spend some time with my parents and see the Canadian Brass in concert on Sat, a surprise from them!! It was a little nerve-racking to leave, but not enough to keep us home. Thanks so much Cindy!
  2. We've been taking Emily on walks b/c it's been like 20 degrees warmer than it's supposed to be. I don't know why they call it "Winter" around here. They should have a different name for the seasons depending on where you are. This is Late Fall and after this we will have Spring. She just LOVES her walks. I gave her a leaf and a pine-cone to taste test the other day... we need to do more of that. When Emily is no longer with us I don't want to say, "remember that time when we were in Emily's room and..." I want to talk about when we were out and about tasting life. What's the point in preserving life if you then shelter it. If you safeguard you kids and keep everything from happening to them, then NOTHING will happen to them. Let 'em live. :) Easy for me to say, right? I mean, I know my kids are going to die. It happens when it is supposed to happen. No sooner, no later. I believe that.
We love you so much Annabelle and Emily! There is nothing greater than to see you grow and taste life.

Peace <><

Thursday, December 14, 2006

12/14 - Another Situation

First of all I am sorry, but I noticed today that I had 11 comments to "moderate" when I didn't even know that I set it up that way. So, the comments are now there. Please feel free to comment on the blog entries, I have relinquished my control-freakness.

Emily's been developing another infection and we've been battling it with the NIV protocol. Basically, we have been using the BiPAP, Cough Assist, Postural Drainage, CPT, and Suctioning to clear her out and get those thick secretions out of her lower lungs.

DISCLAIMER: They way we do things with Emily does not make other ways right or wrong. It is just the way we do it. Also, we are learning to tune her therapies a little here and a little there for optimal support. This is a "living" experience and is constantly changing. But, the bottom line is that with these devices we can improve Emily's respiratory condition, we believe, vastly. They are tools. We don't read much about the "bad times" that other families have, so I am recording all of this here, so others can learn from it... or critique it... we're open to anything.

I had just finished one of Emily's longer treatments (neb/cpt, cough assist, drain, cough assist, suction) and was setting Emily up to for a comfortable evening with Veggie Tales when she started coughing pretty bad. I switched on the cough assist again to, well, assist her coughs. In addition I started doing manual abdominal assists in sync with the cough assist. To give you a picture: I had the bottom side of my forearm below my elbow behind her head to give some resistance while I held the mask against her face. My arm ran down her side so I could place my hand below her Xiphoid Process, on her abdomen. It sort of looks upside down if you can imagine. During the exhale I would give her a thrust sort of in and upward. Try it, it makes you cough. :) Anyway, this is important b/c it is SUPER helpful in getting things up from the lungs and making them cough WITH the cough assist machine EVEN IF YOU ARE BY YOURSELF. After one set of 5 breaths I started suctioning when her sats started dropping into the 70's and then the 60's.

Emily's right nostril is very difficult to get down very far, so I was worried I wasn't going to get the good stuff out. I took the suction catheter and pulled on it with my fingers like you would scissors and a ribbon to make it curl. I wanted to get it to curve somehow to get down into her airway easier. Maybe b/c that warmed it up and stretched it a bit, or both, but it made it flimsy so it went right down where I wanted it to. The problem was that the secretions were so thick that they wouldn't suck out. I dripped some saline in and turned up the suction a bit, but I couldn't get the stuff out of her. Then she started gasping for breath.

I didn't want to pull the suction catheter out for fear of not being able to get it back in, so I gave her some cough assists with it still in (with manual abdominal assists) to give her the volume she needed to actually cough with the machine.

It is general considered dangerous to use the cough assist with the catheter down the nose b/c it could get pulled up and put down somewhere other than the larnyx where we need it to be. But that is what I chose to do at the time b/c I was more concerned about her getting some breaths than anything else.

I would have used the Ambu bag as well, but I wasn't EXACTLY sure where it was after our big move and I didn't want to leave Emily b/c she'd pull the catheter out. So, I used the cough assist to give her some breaths and coughs since every breath was really followed by a cough anyway. This didn't work right away, but after a few sets of this and continued suctioning down the nose and in the mouth (with a yankauer, NOT the suction catheter that I was using down her nose) her sats started coming up again.

Let me tell you, the abdominal assists really work. I wasn't getting anything up with the coughs until I started incorporating them. We've done it with two people, but I was alone this time. She started spitting out all this nasty green stuff that had come up from deep down, that is what you want to see. :) Ask your Pulmonologist and/or Respiratory Therapist about this if you don't know about it. It also brought up the plugs to where I could suction them out. I am sure everyone has slightly varying ideas of what a "plug" is... if we get a clump of junk that is either white or green (the kind that makes the tubing jump while it makes that loud *SCHRUNK* noise when you get it out) we call it a plug, it's easier. But, a plug is really anything that blocks
the alveoli from filling with air, whether it is an inhaled object or mucous of some sort. When the alveoli can't fill with air lung tissue will start to collapse, like a balloon with no air. This is called atalectasis. Every thick thing isn't a plug and all plugs aren't necessarily thick.

Anyway, with the suction catheter already down in her airway I was right where I needed to be to get the secretions up and out. The whole thing lasted about 10 mins before she was back at 98-99 all on her own. I put her back into a flat position to give her a break from draining and started with CPT again.

By this time Diana was back from the store and I had to, um... see a man about a horse, so she continued with another couple sets of cough assist to get what she could completely out. We did try going down her nose again, but couldn't get down so we got what we could out of her nose and the back of her throat. She is still coughing a bit off and on and we will do some more assists and suctioning before another official treatment time, but she is maintaining on her own and SHE IS HAPPY (the whole reason we keep doing this!). Diana was out getting Emily's antibiotics with Annabelle when this happened, so now we have some good ole' drug help too. :)

I have to explain two things.
  1. First of all I have a "flighty way of thinking" as one of the PICU Dr's put it, so let me know if this post is a bunch of pish-posh and hard to read.
  2. Second, Emily is our second SMA baby so these things have been a normal part of our life since 1/12/2004, when Linnea was first hospitalized in Denver... and that is why I am writing about it... because you need to know that you can do it too. Quick reaction to difficulty becomes sort of second nature during these unfortunate times and I think that we SMA parents need to feel empowered with the information that is available to us, especially because we live it day and and day out. If you think about it, very few of these Dr's know what SMA looks like outside of the hospital/PICU "acute" setting, so stand up to them and tell them what your child needs. There is plenty of medical experience backing up these protocols and we are the experts when it comes to the ongoing (chronic) care of our kids. Don't let anyone take that away from you. A good Dr. should tell you the same thing.
P.S. - Emily is getting her 5th tooth!! They're so cute, but they sure cause a lot of problems with all the secretions they create. :(

Tuesday, December 12, 2006

Christmas Cheer :)

I have said it before, we are a blessed family. Tonight a couple ladies from work, Christine and Joan, and their family brought us a Christmas tree, decorations, and stalkings!! They brought us our favorite, Chick-fil-A, and some other stuff to make us feel special... IT WORKED!! They hung out for a bit and it was a lot of fun chatting about normal stuff for a while. We love just hanging out, so, THANK YOU so much!!

Emily had a pretty good day. We caught wind of a nice cart that was designed for SMA Type I kids, so we are looking into it for Emily. We want to be able to take her out more comfortably, so maybe this will do the trick... as long as my insurance will pay for it. ;)

Monday, December 11, 2006

Back to work

So I started working again today. It had been 4 week since I was last at work and a lot of things change in 4 weeks! It was very weird and a bit depressing to be back at work knowing that Emily and my family was at home. Given Emily's fragility right now I didn't really like being gone all that much. Plus, Diana was up most of the night and I was sort of in that in between state, so we're learning to live zombie-like. I am sure other SMA families know exactly what I mean. :)

Since this stay we have become more active in the online SMA community and it has been fun getting to know many of the families, virtually. We have actually had the chance to meet a couple of them in person while we were taking Emily out to UT, so it has been neat to re-visit via the internet. There are a lot of incredible parents out there doing amazing things for their kids.

Emily continues to keep us busy. We are getting her diet dialed in, but yesterday she started this fever thing. I don't know who she thinks she is!! :) So, we are continuing with the frequent treatments b/c she is still weak from the hospital stay.

K - she letting us know she needs us. Signing off - Peace <><

Saturday, December 09, 2006

12/9 - Update - Doing Great!

Wow! Emily is just doing great. :) We have been working on getting her hydrated just right and I think we are getting it done. Too much hydration and these SMA kids have too many secretions. Not enough and they dry up which is bad for a number of reasons. Right now dry is not good b/c she still has some "junk" in that right lung. Dry junk is sticky and heavy and causes atalectasis, which is like you running out into traffic for an SMAer... not good. In case you didn't click on the link, atalectasis is basically some level of lung collapse. Severe atalectasis would be a complete lung collapse, which was the issue with Emily when we went to the PICU.

I'll just put a plug in here for one option SMA families have for pulmonary care. It is not hard, but intimidating -- but the results can be phenomenal. Proper use of the Cough Assist, BiPAP, CPT, Positioning, and suction can GREATLY increase your child's quality of life and their length of life. I'll be honest and tell you that we are not interested in putting Emily through Hell on Earth to keep her alive, but we are interested in treating her pulmonary condition just as we would our own. Heck, I have to use an inhaler at times and if I had sleep apnea or snoring problems I'm sure I'd use a BiPAP or CPAP as well. Anyway, Emily is our second SMA daughter and in comparison we have a better understanding of how the proper use of these machines can vastly improve your child's life. (We used them with Linnea as well, but not as aggressively for various reasons.) After a 3.5 week hospital stay and a completely collapsed right lung we are able to keep Emily's O2 sats in the upper 90's quite easily. She tolerates the Cough Assist treatments and all b/c it is a normal part of her life now and she even sleeps through the Cough Assist sometimes! The BiPAP is a welcome part of the day for an SMA child too, after some getting used to, b/c it allows them to really relax and rejuvenate. If you are an SMA parent considering the options for your child the NIV protocol is a viable option for your family and you can take it only as far as you want.

OK - so right now Emily has been at 99% or 100% O2 saturation for HOURS and she is as happy as can be playing with all her things. Since we've been recovering she has spent most of her time in bed, but we are making the most of this time and getting things situated. So, today we moved our entire third floor around to better accommodate Emily. We have moved the Toddler Medical Bed (looks pretty cool and is EXTREMELY helpful when doing her treatments) into the house and Emily has since taken over our Master Bedroom her stuff. We have subsequently moved into Annabelle's room (much smaller than the MB), and Annabelle has moved into Emily's old room (the smallest of all). I think it will work though. There was some disagreement about the practicality of the whole ordeal, but the point was to make Emily's care and comfort more appropriate for her needs and that is what we have accomplished. If we are fortunate enough to get a night nurse they will have space to work and there is plenty of space to hang out with Emily if she is not up to venturing around the house or elsewhere even if the nursing doesn't work out. That is the big news for the day, besides Emily doing incredibly well, of course.

Thanks to the families that have brought meals and for the families that have been spending time with Annabelle. You are a tremendous help and essential to our lives. I hope you know how much we appreciate what you do for us! :)

Thursday, December 07, 2006

12/7 - A Scary Day

Around 9AM this morning Emily's oxygen saturation started dropping... fast. We have dealt with these episodes before, so we felt prepared for another little event. They are always intense, but not like this morning. We skipped the Chest Physiotherapy (CPT) and went straight for the In-Exsufflator Cough Machine (aka Cofflator or Cough Assist) and cranked up the BiPAP machine for support between coughs, all the while suctioning any secretions that would come up. These episodes that come on so suddenly are caused by mucus plugs that shimmy their way into an area of the lung causing, in some cases, extremely fast desaturations. SMA kids often die this way. Emily's sats kept dropping while I was using the Cough Assist and suctioning in between sets. The last number we saw on the Pulse Oximeter was 11%. To give you a frame of reference -- if her sats drop below 92% we worry.

When I get nervous my voice shakes. When I am terrified my hands shake. My hands were shaking badly this morning.

At this point Emily was completely unresponsive. I had watched her eyes roll back into her head as I was calling her name, then she stopped breathing. We pulled out our Ambu bag (resuscitator ) and started giving her breathes with 6 Liters of oxygen attached to the other end. For about 5-10 mins we had her "bagged" like this, making her breath. During this time her oxygen saturation started to climb ever so slowly. After jumping up into the 80% range I started with the cough assist and suctioning again. This went on for maybe 30 mins. We were listening to her lungs with the stethoscope and there was very little, if any, air movement through her right lung. We feared another collapse.

During that time we switched from Cough Assist to BiPAP and gave her CPT. With Albuterol nebulizer treatments, CPT, Cough Assist, Positioning, and BiPAP we were able to recover her to a somewhat stable condition, but she was still requiring 6 Liters of Oxygen to keep her around 90%. At this point we could also hear more air movement in her right lung, but we knew we had to make a decision:
  1. Take her to the ER to be re-intubated
  2. "Let her go"
  3. Keep working on her the way we were and hope to get her stable
We had a few conversations with other SMA parents, but chose to keep working on her via a protocol that we are familiar with:
  1. CPT 10-15 mins
  2. Cough Assist 4 sets of 5 breaths
  3. Postural drainage
  4. Cough Assist 4 sets of 5 breaths
  5. Continue until able to sat above 92%
After about another hour of this Emily started sating well in the 90's and we turned her oxygen down little by little until we were at 0 Liters. She finally stabilized around 98% with NO oxygen and her lung was sounding much better. Amazing!

Once she was stable we started trying to figure out what we wanted to do. We needed to know more about what happened and the only place to do that was at the ER where they could take an X-ray to see if that lung was collapsed or not. We were reluctant to go b/c we didn't want her back in the PICU, but we really needed to see a Pulmonologist. We consulted the Pediatric Pulmonology team at Johns Hopkins and Emily's Pediatrician (also a Johns Hopkins Dr.) and decided it would be best to take her in, with full expectations of being seen by the Pulmonologists and her Dr. before any other decisions were made about her care.

Emily was stable and happy - two things we did not go to the ER with nearly four weeks ago. This visit was good. We saw the Pulmonologists and told them the story. I could see the grin forming on their faces when they said, "you guys did everything perfectly! The only complaint we have is that it wasn't taped because it would have been a perfect teaching tool for every Dr. in this hospital!" They were very pleased to hear how everything was handled and, let me tell you, we needed that encouragement after the last few hours, especially after the last few weeks in the PICU.

We have since made it back home and Emily is as happy as ever. The Docs helped us tweak Emily's machines a little to suit her needs better and we all agreed that back home is where we needed to be. We left with an inhalant steroid for Emily's lungs and she was very dehydrated (not a good thing AT ALL for SMA kids) so we are giving her more fluids. Oh yeah, her chest X-ray looked better than the day we were discharged from the PICU so things are looking very good! She still had junk in her right lung, but we are working on that... around the clock. We are still not able to have nursing covered by my insurance, so alone we will be until we can figure out a way to get shift nursing.

So, that's the story. Things learned:
  1. Most every SMA family needs a GOOD Pulmonology team on their side and the Johns Hopkins Pediatric Pulmonary team is a good one. They even support more aggressive care with Type I kids and that can be very difficult to come by.
  2. We are grateful for our incredible Pediatrician, Dr. Dudas - SMA families need one of these too
  3. With these two things SMA families can feel comfortable taking their child to the ER
  4. Diana's two cents - MOST IMPORTANTLY -- We learned that Diana and I are a GREAT team for Emily. We fall into our roles and get things done pretty flawlessly. We are grateful for each other's unique abilities and we cling to them. (Thanks for trusting me Diana!)
Thanks for reading.

Wednesday, December 06, 2006

12/6 - esta mañana

One night down, thousands to go. Emily was so happy to leave the hospital last night. We put her in her car seat and she started whipping her head back and forth looking at everything. She was watching all the lights and looking at all the people on our way out. She loves going for drives so it was fun to look back from the drivers seat and see her little face. She just looked so relaxed and happy.

We had tons of stuff to get situated once we got home. We had become accustomed to having certain things in certain places so we could grab them without thinking about. Well, it took some time to get those things where they needed to be. We are going to rearrange our home to better accommodate Emily's things. We're giving up the Master bedroom so we can all have a bigger room to hang out in with her when she needs to be closer to her stuff. Also, this will help us provide a more comfortable workspace for shift nursing, when we get it. So that's our next big project.

We were told that we don't qualify for coverage for Emily's special formula called Vivonex so I am trying to get a straight answer from MAMSI about that. They have told us that b/c it is "over the counter" we do not qualify, but she should b/c she is tube fed and it is medically necessary b/c of issues brought on by SMA. Anyway, just a little window into the types of phone calls we have to make every day.

Gotta post this b/c it is taking all morning with the phone calls.

Emily is HAPPY!!

Monday, December 04, 2006

12/5 - Exodus

So today is the day! We are scheduled to begin our mass exodus from Johns Hopkins and the Ronald McDonald House this afternoon. :) I'm getting the equipment details taken care of as best I can so when we get home we'll have everything we need. MAMSI is still holding out on some things, like Emily's special formula, but we'll keep working on that. Also, shift-nursing is still not covered so we'll have to see how we fare. The HR folks at my company are working on getting that part of the policy changed, but there are NO guarantees.

Emily has not totally recovered and our schedule is going to be pretty tiresome when we get home. I will continue making updates about her condition here so please keep coming back to see how things are going. The good thing is that we are out of the hospital. The not as good thing is that we are still in the middle of respiratory season. There is some anxiety in thinking about Emily's health this winter, but all we can do is what we can do. :)

You all have been so generous with your lives over the past 3+ weeks and we are forever grateful for you. We are going to have meals provided for a while as we transition and that is just incredible. Thank you so much for everything you have done for us during this hospitalization. We are so happy to be taking Emily home b/c there were times where we were not sure if we'd be able to.

Thanks to all our nurses and other medical staff peoples for taking care of Emily. I hope we don't have to see you again, unless it is for social reasons of course. ;)

I'll keep writing things here as we being our retrospective thinking this evening. We learn so much about life each time we are in the hospital and I am sure I'll write about it as much as I can in this bLog. So, if you'd like, please visit often. :)

Peace <><

12/4 - Update - Tomorrow

Well I came in this morning and the Dr's were rounding and talking about Emily. They looked at me and said, "What do you think?" I told them that we're ready to go home. Not because we are tired of being here, necessarily, but because we can handle her current level of care at home... and probably better from a respiratory standpoint since we can be more consistent since there are only three of us doing her treatments. There is still some weaning to do but we can do that at home. So, the plan is that we will go home tomorrow, if that wasn't clear. I didn't want to sound negative and say "maybe, because anything can happen," but that is sorta what's in my head. We pray beyond prayer that we will be going home tomorrow and Emily will be prepared to do so.

So our new challenge is getting shift-nursing for night help, at least, and then some other DME stuff like supplies for the equipment that we own. My insurance company, MAMSI, is telling us that shift-nursing is not covered, so we're seeing what we can work out. If any SMA families are reading this and considering insurance companies DO NOT use MAMSI's Optimum Choice if u can avoid it. They are not helpful. I don't know about their other plans, but ours is nicht so gut! (not so good)

So, we have a great nurse today, Aubrey, and she makes our days much more relaxing. Thanks Aubrey!

One interesting part of hospital living is getting to know the parents of other kids that are here. There are only a few that have been here as long as us, but we all have our stories. Other families are dealing with some crazy stuff too! We are not alone in our emotions and every time we notice some emergency going on we worry about which family it is all for.

OK - gotta figure some stuff out now. Cheers! :)

12/3 - Update - Blah

Today = Blah

We are pretty tired. We took Annabelle to see Happy Feet today, it was ok but we have a lot on our mind and plate. Plus, they put a little too much emphasis on... umm... mating. I don't think PG-aged kids need humanized descriptions or visual representations of what penguins do when they mate. Show me two penguins mating and I'm fine to let my kids watch, but they don't thrust their hips and act sultry -- at least none of the Emperor Penguins I have ever seen.

OK -- next on the agenda -- we are tired. Oh yeah, I already said that. We are on the home stretch. We are like horses smelling the trough or whatever that saying is. We need to get home where Emily can get consistent respiratory treatments from her mommy and daddy. I am with Annabelle at the RMH b/c she had a little break-down and needs to be with us. Goodness, she's been away from us for nearly 3 weeks with some visits in between. Diana is at the PICU b/c we we felt more comfortable having one of us with Emily while we are trying to transition back to home care and get home soon. We are not allowed to sleep in the PICU b/c it is an open room. Fall asleep and you get a ticket home. This makes things very difficult for us. It's hard to rest when you are, well, awake all of the time. We need to go home now.

So - some thoughts. Being around a hospital makes me think -- Man, we sure do a lot to save lives in this world. What so important about life?? What makes it so special?? Why do we go to such lengths to preserve it?? Just some questions -- Cheers.

Peace <><

Saturday, December 02, 2006

12/2 - Update & Pics

Well we are feeling pretty good today. All the medical staff seems to appreciate our perspective and we are able to plan out Emily's day pretty well. She's pretty tired from getting a lot of cough assist sets and all, but it is good. We are clearing a lot of secretions out of her system. We are not sure when we are going home, but maybe sometime this week. I have taken these past few weeks on Unpaid Leave as well and that cannot last forever. I am thinking about going back to work on Mon, but there are still a lot of logistical things to be planned for the return home.

We have had so many incredible people come to sit with Emily and/or us, bring us meals, and take Annabelle for play dates. Thank you so much. Hopefully we will be going home soon and until then your time will continue to be VERY much appreciated. :)

Friday, December 01, 2006

12/1 - Update - Conversatin'

Wow -- I can't believe that I haven't posted anything since LAST MONTH!! Ha, ha, ha!

Sorry 'bout that silliness -- So we are still here. Today was full of conversation. We are sort of in a difficult spot where everyone is trying to determine when we should go home. We feel comfortable with Emily's current level of care, but we don't want to leave too soon and end up right where we are in no time at all. Our experience with SMA is that each of these sicknesses fast forwards our time with our baby. We brought Emily to the hospital with an acute problem (collapsed lung) and now we appear to have moved back to Emily's typical chronic issues (rigorous respiratory care with periodic plugs and thick secretions) with some different dependancies. Since we are still in the PICU when Emily has any respiratory issues it looked as another acute issue when it may just be typical Emily. They MUST see things that way in the PICU though, and we understand that, but it may not be the best for helping Emily. She needs aggressive respiratory treatments when she has problems if she is going to get "better."

I'm not criticizing the PICU, they're great, but we've gotta have an "exit strategy" and we sorta have to help everyone understand when we think Emily is ready to go home.

Anyway, I'm writing this from my cell phone so I'm gonna stop here. My fingers are getting tired.

Thursday, November 30, 2006

11/30 - PM Update - "Palliative" care

Oh man - I am tired. I cannot think anymore.

Every SMA family MUST deal with the topic of "palliative" care. says: "serving to palliate" -- not very helpful. Oh wait -- futher down it says: "Relieving or soothing the symptoms of a disease or disorder without effecting a cure." But, had the best explanation, I think. "1) Medical or comfort care that reduces the severity of a disease or slows its progress rather than providing a cure. For incurable diseases, in cases where the cure is not recommended due to other health concerns, and when the patient does not wish to pursue a cure, palliative care becomes the focus of treatment. For example, if surgery cannot be performed to remove a tumor, radiation treatment might be tried to reduce its rate of growth, and pain management could help the patient manage physical symptoms."

We chose to put Emily through her G-tube/Nissan surgery a year ago b/c we saw saw an opportunity to give her a longer life of good quality in a way that is more manageable for us. I have said it many times, we are not trying to "beat" SMA or make her live longer that she wants to, but with the palliative definition in mind, we have chosen to use the Cough Assist, BiPAP, suctioning, etc. as respiratory aids, to lengthen her life in a palliative manner. However, as we become more familiar with the minds of healthcare providers, we are learning that the term Palliative does not mean the same thing to everyone. Even among the teams that we are dealing with here in the PICU there are obvious differences about palliative care. I think of it in reference to time. Some of us feel a sooner death is better, others believe the opposite. We believe that good care can extend Emily's life without degrading the quality of it. We are trying to address the acute issues (e.g. Pneumonia) in a way that sets us up for the chronic issues. If we don't wean Emily off the BiPAP correctly, everything else is going to suffer. However, if we can wean her in the most effective way we will all be more prepared to deal with the ongoing care.

I write these things b/c they are constantly on our mind. We are trying to make the best decisions for Emily and dealing with the different medical personalities makes this a challenge b/c of nuances in understanding like I mentioned above. That being said, we had a good conversation with Pulmonary today. They seemed to think as we do about Emily's respiratory condition and we are going to make some aggressive changes to her care b/c it will, hopefully, set her up for more manageable and healhty care once we get home.

Thanks for reading. Peace <><

Wednesday, November 29, 2006

11/29 - PM Update - Break from BiPAP

Emily had a great day today. We were able to give her some time off the BiPAP and her Oxygen saturation was better off the BiPAP than it was on the BiPAP!! We got to her hold for the first time in 2.5 weeks and she gave us lots of smiles. That's our Emily! :) Nurses were coming in to check her out and everybody was so excited. We held her so Annabelle could see her from the doorway to the PICU and that made her pretty happy too. We are hoping to be home as early as Fri, so let's pray that Emily will just do great.

Quote for the day -- We are where we are because that's where we are.

11/29 - Early morning - Validating You

Do not let ANYTHING validate your existence more than Christ does. I've been thinking about this and I've come to realize something, sort of -- Wealth is not something I use to define or validate who I am in society, nor do I use possessions or my job, but kids. I have come to realize that having kids, to me, defines who I am and where I stand as a man -- I feel that they validate me. I need nothing else but lots and lots of children b/c only then will I be "rich." Having children has become more important to me that my relationship with Christ, so imagine how that has gone over in my life for the past few years!!

We cannot look for fulfillment in life anywhere but in Christ. We can own nothing but our love for Christ -- and even THAT is something God gives us. (I'll have to get back to you on where that is in the Bible b/c I can't remember -- I promise there is something that says this though)

I don't have a lot more to say about this right now - but I needed to get it out. I am slowly learning how little sense it makes to put my value, my stock, my worth, in life into anything but a relationship with God. He has made me who I am and is continuing too mold me into who He wants me to be. I am not there yet, but to work against this is INSANE!

Peace <><

Tuesday, November 28, 2006

11/28 - AM Update - Recovering

So - it looks as if we are more noticeabley in the recovery phase of this 2.5 week journey. It has finally been more than 24 hours without any set-backs in the weaning process. Emily is off her pain killer, she is on full feeds, there are no signs of infection, and she is doing well on the BiPAP. We have to wean her down on the BiPAP settings, but that is not a real big deal. There is also some congestion in her right lung, a good reminder of how important her respiratory treatments are. The only thing we are really waiting on is for her to be completely off Valium as well. We have to do some planning before we go home, however, b/c she has different needs now. We are considering a different route for her diet, something that every SMA family has to deal with at some point, and there may be a greater O2 need on a regular basis once we get home. We'll have to see.

Last night some folks gathered to pray for us. Thank you so much. There have been nights of seeming emotional and spiritual destitution, but last night was not one of them. We slept and dreamt well. There is no doubt in my mind that the many, many prayers that have been given for us have been showering us with grace and comfort during these two weeks and more. Also, thank you all for the generous donations to Emily's Medical Fund. There are many unforeseen costs with these situations from medical to personal -- it is an incredible blessing to not have to worry that much about finances. Thanks again!

Sunday, November 26, 2006

11/26 - PM - Annabelle = FUN

I have had a lot on my mind over the past couple of days and most of it has been pretty painful. I wanted to write about it, but the things below came to mind instead. I hope I can get to that other "stuff" some time soon.

An exciting thing happened today - Annabelle was able to spend a couple of minutes with Emily. When she has been at the hospital she stands by the entrance to the PICU and waits for the door to open to she can catch glimpses of Emily's bed or a stuffed animal on her bed. She would report, "Daddy! I saw Emily's tiger's ear!!" Then, when the door started to close she'd hit the button on the wall that makes them open again. Such a sly kid! Then she'd have to stop b/c someone would come lookin' for the adult responsible, me. hehe -- I just let her do it until someone seems to mind b/c I think it's great that she just wants to get as close to Emily as possible - I can't justify telling her that she can't do that. So, she got to give Emily some kisses and tell her she loves and misses her. I can't emagine what it is like to be Annabelle. I'd be much weaker I think.

Emily had a good day. She was as active as we've seen her in two weeks and some friends, Lita and Vicki, were able to experience that with us. My mom had some good Grandma/Granddaughter time with her too -- that's always nice!! She did have a rough night b/c her respiratory rate was so high and she wasn't able to calm down much, but maybe she was just trying really hard to make sure we all had a fun day. :) We are still here for a bit though. We have to get her back on a regular feeding schedule and we need to get her weaned from the BiPAP, at least to what we do at home. So we are not packing up just yet.

Annabelle and I attended New Song Community Church this morning and that was a blast. Pastor Williams spoke about "Redistribution" and how we are to live Open-Handed and Open-Hearted towards the poor and needy in our community. He spoke from Deut. 15:7-11 and Acts 4:32-37 and it was good. I was reminded that nothing I have is mine -- I am only a steward of the things I have b/c they are God's, including my children and my sufferings. I could not help but think about our situation. Not only are my kids not mine, but my emotions and the experience is not mine either. I must be willing to be open-handed and open-hearted with our experiences just as readily as I might be with our finances and time. If I am unwilling to share these things with you, then I am not trusting God's purpose for them. I think we often do not think of it that way. We think of sharing as if it only has to do with physical possessions... and sometimes time, but what about our oh-so-private emotional possessions and experiences -- those things we hoard to our own detriment?

Peace <><

Saturday, November 25, 2006

11/25 - Update - Things Are Good

Emily had a great night and a comparably good day so far. The attending Dr. thought it was good to start her feeds again so we'll see how that goes. She is responding well to the Cough Assist and we are getting a lot of thick stuff out of her lungs. All in all her health seems to be returning much better now. Her baseline strength and respiratory wellness is different than it was, so we'll be adjusting to a new home life when we leave here.

I want to thank everyone who has sent us such encouraging emails. I hope you understand why I can't get back to everyone though. I have barely been able to respond at all, but we appreciate and survive daily on every one of them. :) My hope is that you may find some response to your personal concerns for us in my daily posts. Your emails make me think and that drives me to post. Thanks!

We rushed home last night b/c I had an excruciating migraine - something that has only happened to this degree once before in my life. I had to get some allergy-free pain medication from home. Anyway, maybe this is my body's response to the erratic couple of weeks.

Oh yeah - Diana and I had a semi quiet couple of hours to ourselves and watched a couple episodes of LOST for our anniversary. :) It was fun. A big THANK YOU to the folks that sat with Emily yesterday so we could have some "time off." :) :) :)

Friday, November 24, 2006

11/24 - PM Update

Not much to report today. Emily had a good day despite her white blood cell count and we started her cough assist. She really wants to have a break from her BiPAP, but she just isn't able to do it yet. The best thing about today was that we were not reintubated. The longer we can hold that off the more likely it is that we will able to go home.

We had quite a few people sitting with Emily today to give us a break and that was such a blessing. Diana got to sleep, I didn't have too many decision making responsibilities today, and were even able to spend some time with Annabelle. We miss her and she misses us. I hope we get to go home and be a family again some time soon.

My parents and Cindy have been visiting on a regular basis and that has been sooo nice. It is great to have them nearby for support.

Thank you all for the support you give us in so many ways. We really couldn't do this without the prayers for us and concern that you show us. Thank you for sharing in this with us!

11/24 - AM Update

Well, here's to another wedding anniversary stunted by SMA. It's not all that bad though. Diana and I have had PLENTY of time to the two of us over the last, nearly, two weeks... just not the quality time that one might expect out of life. Speaking of Diana - she was up with Emily all night last night at the hospital while I battled it out with God on my knees at the RMH. This was followed by deep dreams and waking up throughout the night. Too much to think about and such weighty decisions.

After Linnea died Diana and I went on a "silent retreat" with Klesis in Connecticut. It was a very important experience for both of us. One thing I learned there is when I am talking to God, get out a pad of paper and just write what you "hear" or are thinking with NO filter whatsoever. I did this last night, in the dark. Call it want you want, but I was reminded/told/comforted (pick your own word) that no matter what condition Emily is in God already "has" her. I was pleading for Emily's life and wanting her back and I felt that God was asking me "are you sure?" I said "no." What I want is to not feel the way I do -- helpless, hopeless, comfortless, and used. If I cannot have Linnea and Emily then I want Him to make me feel the way they make me feel. I want to be caressed with the kind of love you can only get from the undeserved, reciprocated love of a child of your own. If Jesus is enough, then He can do this and the Bible says He will -- and I am going to understand what that means and learn to desire what it is.

Emily's white blood cell count was over 51,000 this morning. That is astronomically bad. She is fighting an infection that we cannot see or measure b/c it is not in her lungs, as far as we can tell. In fact, we are not sure where it is at this point. In order to start her feeds she either has to be reintubated or we have to tell them that we would not like her to be reintubated regardless of the situation. We are leaning towards the later.

We are spent and the logistical nightmares of continued DME provisions that awaits us is making me feel sick. We have been discharged from Hospice b/c that is the policy when the patient has been admitted to the ER and all of Emily's DME was provided through them, so now we have some decisions to make, again.

So - another thing I wrote down last night was that I should read Psalms, Proverbs, and Ecclesiastes. So, I will read them today if I can. Peace.

Thursday, November 23, 2006

11/23 - PM -- Tough Day

We had a pretty tough day -- it was made a bit better by some GREAT food from the Worths and a visit from Chrystopher and the Gillette's, but it was tough. There is a multitude of things that we are concerned about and the main thing is Emily's nutrition. We have been fighting for her to get better nutrition (food in the stomach), but our sense of urgency for her "good nutrition" just isn't shared by her Dr's here. It is very frustrating (maddening). We ALL want her to be successfully extubated, but if she is going to waste away in the process b/c of a lack of substantial nutrition and respiratory care there was no point taking her off the tube b/c we are going to take home a completely deteriorated little girl that will not have much longer to live -- the main reason we avoid taking her to hospitals the the first place. The medical staff at JH's has been genuinely concerned for Emily and her care, we believe that, but when it comes to weighing the risks and benefits of her total care we are just not on the same page and we are at the mercy of their orders in the end. We get talked to very often like parents that are just concerned and don't see the whole picture -- a grave misunderstanding of our knowledge and ability. We are constantly treated like we we are not concerned about the same things as the medical staff which is just not true. It's an uphill battle though. I don't know how much longer we can fight like this b/c all they have to do when they get tired of us, like a Respiratory Therapist (RT) did tonight, is call someone else to deal with us. An RT was called to deal with us this evening and he said "you SMA parents are the worst b/c you know the most!" It was a compliment and he said that he commends us for the job that we do. He talked things through with us and said not to give up b/c only we know Emily inside and out. We're so tired.

11/23 - Trouble (changed)

Please pray for Emily. She has had a difficult time keeping her O2 saturation up. We were surprised this morning to hear that the attending Dr. that wanted to see how Emily was doing this morning ISN'T her attending Dr. today, so that adds some frustration! Her blood pressure was high this morning too. Anyway, not a great start to the day.

Wednesday, November 22, 2006

11/22 - Introducing Emily

Thank you for your prayers!! After much review the peculiarity of the "fracture" has resulted in it being a moot point right now. Maybe there was one at some point, but there is no indication that anything is wrong now. Maybe we'll re-visit that at a later date. We are not out of the rough yet, but things are looking good. Thanks!

11/22 - IT'S OUT!!

1. Emily has been extubated!! That means her air vent is out and she is breathing on her own, for the most part. We use a BiPAP at home and she was extubated straight to that. So, she is no longer on life support and that is what we have been waiting for. Everybody will be observing her closely to see if she is able to stay extubated, but everything is looking great. Praise God!!

2. Diana's such a great mom and she knows Emily so well. B/c of mom's good looks (there's a double meaning in that) she was able to spot a part of Emily's right arm that seemed out of the ordinary. It seemed swollen around her elbow area and we noticed that it was somewhat sensitive. Turns out it is a fracture! :( Emily has a fractured arm! Over a month ago she did fall out of her bed and we are wondering if it is from that, but we are not sure. It could have happened here at some point too, so there is a Pediatric Orthopedic Surgeon and a Radiologist looking at the X-rays to see if they can determine how old the fracture is. The GOOD thing is that now we know why Emily has been strangely fussy during her stay, besides all the crap that put us here. The bad thing is that we don't know what to do about it, necessarily. She moves it around freely so that is good, but it is obviously tender and that MAY be b/c she has been straining against the "No No Straps" -- who knows really?! More to follow...

Tuesday, November 21, 2006

11/21 - Nearly a PM Update

It's another day on the tube. Emily is doing exceptionally better, however, and we are thinking that they stopped her sedation meds too fast. She was stressed yesterday and developed a high fever, so we are working things out today in hopes of extubating tomorrow. Her white blood cell count is high which indicates the body IS fighting, or is preparing to fight an infection. It's like a bunch of soldiers all suited up for battle and their marching off to war. Sometimes, though, there is no battle once they get there. Other than that she is still retaining too much water and that complicates some things. We'll see how things go as the day wears on.

So, since masterpieces can never be duplicated I'll see what I can do about replicating yesterdays post, but it may take some time -- I am still crawling out of my hole of despair.

To suffer is to live. That's my new quote (maybe someone else thought if it too, perhaps, but I just thought of it so I'm saying it is mine. :)). If we are not able to suffer then we are not able to really live. If we try to avoid suffering, then we are avoiding life itself b/c it is full of suffering. Some of us may not suffer as much personally, though, and that is what I want to write about. I don't want to ask "Why is there suffering?" but "Who is suffering for?" I think that, generally, "we" are always trying to avoid suffering. We shield our kids from it, we whisper about it, we turn the lights down and play soft music --- many (if not all) addictions are rooted in an attempt to escape suffering... and all of this only leads to MORE suffering. Ha! The very thing we are trying to avoid we end up complicating even more in the end. Craziness. Suffering is something we must learn to accept and live through successfully. I believe that our lives can be enriched and deepened through the experience of suffering and I believe that our willingness to enter into someone else's suffering may even be more important.

Community: Many have asked us how they can help -- Companionship -- Community is who suffering is for. There is a passage in the Bible that we have held tightly to (2 Corinthians 1:3-7) that, in essence, says that if you are willing to share in our sufferings you will will also share in the comfort God is giving us. Often this comfort comes through you all, but it also comes from Him. An analogy -- I'm on a soccer field kicking the ball in the goal. I will only experience a limited amount of satisfaction as long as I am alone. It is when I am with people and I can share the moment of joy when that ball goes in the goal that I experience life the most. We are built for community. We are designed to want to share our experiences with others. If we are not sharing in suffering then we will not share in the blessings that come from it. We love it when people are with us, with Emily, and/or with Annabelle. Even if you are far away a short note, text, or call that you are thinking or praying about us brings together a community that we can share our experience with. If we don't know you care, we cannot share. (hehe, that rhymed) It allows us to share in our experience and allows for you to enter into it as well. I think that we will often leave people alone when they are suffering b/c we don't want to "bug" them, but I think we are sorely missing the point of the experience. It is not something to just "get through," but to build community. So, if you know someone that is suffering, let them know you are thinking and/or praying about them - even if you think you'd be bothering them. Also, suffering people may not know what it is they need until they have "it" or "it" is offered, so feel free to offer whatever you feel you can give. It is good for you and good for them.

OK - That was a far cry from yesterdays concoction, but it is along the same lines. I hope it makes some sense. Thank you for checking our updates and let us know who you are if you haven't yet. We'd love to share what we have learned, comfort we have, and even the pain -- for our benefit as well as yours. :)

Monday, November 20, 2006

11/20 - 4:30 PM Update

I just spent an hour writing a masterpiece about suffering and my browser reset -- it is all gone. (I am seriously on the verge of tears) Now the library is closing down and I have to go. :( (Wish this PC had Firefox b/c that wouldn't have happened.)

There will be no extubation today b/c Emily just isn't ready. We will look at things again tomorrow. That is all I have time to write now. Thanks for checking...

11/19 - PM Update

Not much to report today. We've had Annabelle for a couple of days now and I am realizing how tired we are and how much this disrupts the standard craziness of our life as a family. She misses Emily and is not allowed to see her b/c it is Flu season. Sucks! As you start to experience the fatigue of reading these daily updates be glad your not writing them or experiencing the story they are telling. I would never wish this experience on anyone.

We took up some offers to sit with Emily today and went to Port Discovery with Annabelle. That place is like speed, for kids!! It was fun and Bob and his son Shawn joined us for a fun-filled afternoon. Thanks Maureen and Eileen for sitting with Emily while we were out!!

Tomorrow the plan is to extubate, but we will see how things look then. No promises. We want to make sure everything is in order so we won't have to stay here another 2 weeks.

Thank you so much for the generous donations to Emily's Medical Fund. It creates a warmth to my heart that is simply divine. Thank you.

Sunday, November 19, 2006

11/19 - Short AM Update - Not today

Well, Emily will not be extubated today. She was on some diuretic to get fluid off her lungs and her body did not respond as intended. The Dr. does not feel that it would be a good idea to extubate with this issue unresolved and we are in agreement. So we will try again tomorrow and hopefully all will go well. She seems pretty content, however, and that is good. I think she will do fine for another day. She is breathing well over her vent, meaning she is doing most of the breathing herself, so it would seem that she will do just fine. Time will tell. Thanks for your prayers!! :)

Saturday, November 18, 2006


Today was a GREAT day! Emily was in a great mood and she did well on her vent settings. We are now ready to extubate (take the air vent out)! Her right lung is still not inflated as much as we'd like, but we are not sure it can totally inflate anyway given the concavity of her right side. It is possible that her right lung has been depressed for some time without our knowing for sure. Every day blood gases are drawn and other measurements are taken to determine how her body is handling the weaning process. Everything points to a successful transition from the air vent to the BiPaP which is something we use routinely at home. Anyway - the procedure will happen at about 8AM and all eyes will be on her as we determine whether or not she is handling it well. There is a chance that things will not go well and she will need to be re-intubated, but we are hopeful and confident, at this point, that things will go well. If you remember, and you are awake at 8AM on a Sunday, please remember Emily in your prayers. If this all goes well we may be home by Wed. :)

As always -- thank you so much for the meals that have been brought every night, for the phone calls and emails, and for the visits where we just get to spend time with you. Thanks for those of you that have sat with Emily so we could have a break and thank you so much for those of you that have been so generous monetarily. You are a incredible bunch and we feel forever blessed to have such great support during these days!!

Disclaimer - no nurses were being personally targeted in that last bLog. It was some pent up feelings from all of our encounters over the last few years with Linnea AND Emily. You guys do an amazing service and we love you for it!!!

11/17 - PM Update

Emily had a good day today. After her Bronchoscopy she did well and was happy for the majority of the day. She also slept a lot, maybe from the sedation wearing off from the procedure, so when we left to go to the RMH she was still awake. We need sleep though, so we are trusting that the good Lord will keep her at peace and give her rest this night.

It can be tough seeing new nurses take care of Emily b/c we are always being re-introduced to another medical professional where, most of the time, we feel that we have to prove ourselves as medical caregivers. If we do not exude a certain level of competency with the medical world we tend to get what I call "parent answers." Those things that the staff says to us so we will stop bugging them, even if it is not really true (yes, that has happened before -- many times). Now, I am not saying that that is all we get, but it does happen. So far the majority of our nurses have been great and talk through things with us so they understand what we are trying to accomplish. This is good since we take care of Emily 24/7, with respiratory and other therapies, by ourselves, at home... with no medical specialists helping us day in and day out. How does one really get that across effectively?? Sometimes you just can't I guess -- but seriously, unfortunately we are no strangers to this care. -- this gets tiring.

Ok - that is enough semi-venting for now. I have to get to bed for goodness sake!!

Friday, November 17, 2006

11/17 - Noon Update

Emily had her Bronchoscopy today and they were able to suction out a lot of plugs and thick stuff from her right lung. Tomorrow morning another X-ray will be taken and it will be more telling of how her lung really is. She is much happier today, since they have changed medication, so we are able to relax a little. We are also hoping to spend some time with Annabelle this weekend -- so thank you to the people that have volunteered to sit with Emily while we are out, and please pray that she will have a good weekend!! Cheers, Nate

Thursday, November 16, 2006

11/16 - PM Update

Today was a difficult day b/c Emily was in pain. This has been going on for the past two days, or so, and it was very difficult to watch. She would fall asleep and then wake up suddenly with a cringe on her face, gap-mouthed. We are used to solving her discomfort problems by understanding the tone of her cry, but with the vent tube in her airway she cannot make a sound... this makes it very tiring to try and figure out why she is silently screaming. I hope she is simply tired of being in the hospital.

Diana spent some time with Annabelle today - Annabelle was SOOOO happy to spend some alone time with her mommy!!

Tomorrow we are having a Bronch Scope done to see if there is anymore that can be cleared from Emily's right lung b/c it is not cooperating. The sooner we can get her going on her own, the sooner she will be happier. I did manage to procure a pacifier and extra cushion for her bedding today, so maybe that will make her feel more "at home."

We continue to be provided for in many ways. Every day we get to experience the best side of everyone we know as you show your care and concern for us. You know who you are, so THANK YOU THANK YOU THANK YOU!!! :) You are all such a blessing. Please allow our situation to be a blessing to you as well -- it's perspective that we all need to add richness to our lives, so let our "suffering" and struggle in its fullness add that richness to your life, and may it be a blessing to you in your own struggles -- after all, is suffering only for ourselves??


11/16 - Donation Requests

Dear everybody -- people have been asking, so I have reposted the "Make a Donation" button to this bLog and also at A year ago a great friend of mine started the "Emily Lee Medical Fund" at the Bank of America so people could make donations for Emily's care in a tax deductible way. There are a few ways to donate to Emily's fund.

1. Walk in to any BoA location and make a donation to the "Emily Lee Medical Fund" which is located in Georgia.
2. Visit or and use the "Make a Donation" link to donate via
3. Visit and make a donation to

All of these methods will direct donations to Emily's fund.

To give you an understanding of our needs:
I was going to start LWOP (Leave WithOut Pay) yesterday, but my company has advanced me leave, which means that I will be many hours in the hole, not necessarily a good thing but the only thing my work can offer. B/c of Emily's visits to Salt Lake City and other appointments in the area, my leave balance is typically around "0," so I was not able to have any saved up for this sort of emergency. We are not interested in "asking for money," but I was avoiding responding to people inquiries about that aspect of our situation. Again, we are incredibly blessed in so many ways and we know that, one way or another, we will have what we need. Thanks for reading this, hopefully I won't have to write about it again b/c it makes me uncomfortable. :)

11/15 - Update - HAPPY 16 MONTHS EMILY!!!

Today Emily is 16 months old!! That is a big deal for SMA type I kids. She has beaten many odds in surviving this long and we are so glad that we get to be her parents. :)

Today was a little different b/c Emily's right lung showed signs of re-collapsing so her weaning process came to a halt while they raised the settings back up on her vent. She was generally irritated all day long b/c she needed a lot of suctioning and we think her G-tube site is hurting her. It is hard to just sit there knowing that she wants to move around, but she can't. She can physically move, but they have to restrain her little arms so she won't pull her vent tube out. They call the straps "no-no straps." "No, no Emily, don't pull your tube out." She doesn't like them and for good reasons. We try to move her arms and legs around throughout the day, so if you come in to see her we can help you do this too. :) The lung issues for today sorta make us step back a little, so we are not sure exactly when we will be able to get her off the vent, but we are hopeful that it will be soon. I'm not sure exactly what I meant by that, but we really just hope that she gets over this and we can go home soon. Annabelle misses us and we miss being a complete (minus 1) family again.

NEWS FLASH!! We are now staying in the Ronald McDonald House (RMH). We are no strangers to the amazing accommodations provided by the Hamburger King and we are blessed to be served in this way. They make these places just the way families need them when they are dealing with situations like ours. We'd love to help a local RMH some day when we are able. Community members provide daily meals and stuff for the residents. It's pretty neat. We will have a room here for $15/night for as long as we need it and there is FREE parking and a FREE shuttle to/from the hospital nearly any time we need it. What a blessing.

Thank you again for the calls, visits, gifts, prayers, and meals. We have an incredible support group (again, you all) and we are a blessed family b/c of you.

Tuesday, November 14, 2006

11/14 PM - Update

Another day has come and gone and Emily is still with us. Her right lung has not gotten much better in the last 24 hours, but she is doing well as they ween her off the tube. She is still intubated and probably will be for another day or two if things go in the direction they are now. She was alert a lot more today, but is very tired of having a tube in her airway and modeling as a pin cushion for blood samples and medicine distribution. The right side of her chest has been concave for a while now and we think that it may have something to do with her lung not being able to inflate all the way, but that is somewhat speculative I must admit. Again, we need to get her off the tube as soon as possible b/c she is weakening while she is on it and she can't really afford to get weaker. I am home again tonight (I didn't sleep in my van last night b/c there is no place to pee in the middle of the night!) so I am going to attempt to get some rest. I must say, thought, that it is disheartening for me to not here Emily's BiPaP and sleepy-time music going in the other room. It is all to reminiscent of the night following Linnea's death. I just hope that I fall asleep fast so I don't lie there thinking about it. I don't like leaving the hospital each night. We are looking into a hotel or the Ronald McDonald house for the rest of the nights. We miss Annabelle too, and she misses us... again.

Thank you everyone for your emails, visits, and phone calls. God has answered a prayer of mine, that we will feel His personal interest and touch in our lives right now and I believe He is doing just that through His "hands and feet." (That'd be you all) Thank you!

Monday, November 13, 2006

11/13 - PM - Update

It is 1823 on Mon, 11/13 and we are still at John's Hopkins with Emily. Bear with of as I make this update from my phone. Emily is on life support bc her right lung had completely collapsed, causing her body to start shutting down. All efforts now are aimed at getting her off life support as soon as possible, which is the worry given her low muscle tone. However, it may be a few days before she is able to be taken off the support, if at all. If she is not able to extubate we will have to discuss end of life issues in a more immediate fashion. Emily seems very content given the fact that she has a tube down her airway and she falls in and out of sleep all day long. The goal is to get her right lung to open so she can breath normally again. Annabelle is spending time with various friends/playmates as she is not allowed to visit this area of the hospital. She is very thoughtful about the situation and understands the implications bc we have had discussions about when this day would come. She is such a neat girl. We are exhausted and I'll be spending the night in the back of our van in the parking lot while Diana will stay in the hospital and maybe in a room for mom's. Please pray for wisdom, comfort, peace, undertanding, and the like. We don't always feel like God is here, but I trust that He is... that is tough. Thanks for your calls and emails today.

Emily - ER - Intubation

At midnight we admitted Emily to John's Hopkins b/c her breathing was
slowing and she was fading. She had to be resuscitated and now she is
intubated. This puts us in a situation that we said we would not be
in, but nevertheless here we are. At this point we have to decide to
stave death or submit to it. Pray that God will prepare our hearts for
whatever is to come. I am not confident they (Our hearts) can sustain
another loss right now.

Saturday, October 21, 2006


Greetings! It's been some time since the last update and that is b/c a lot has been going on. Emily has been pretty sick and we are not quite sure why... and neither are the doctors that we discuss these things with. She has two teeth coming in, so that makes for a lot of secretions and pain, but she has also had blood in her stomach and she has thrown up again. She continues to have a lot of gas and we've tried various medicines to help with some of these things. B/c of her condition we are not making this next trip to Salt Lake City which means we probably will not be going out there at all anymore. That means that we are no longer a part of the study and Emily is no longer eligible for PBA. That means that we either have to get into a study closer to home that can provide that medicine or we can try another medicine, VPA, that has had success with SMA kids. We are still not sure what to do about the medicine, but the reality is that if Emily is not on something all of her voluntary muscles will likely weaken so fast that she would not live very long at all. It would be a tragic downfall I'd guess.
The thing is, I don't know how long we can keep doing this. Emily had Diana up almost nonstop last night and I was up many times as well. We get a few hours of sleep every night b/c she is coughing, needs her feeds, needs to be turned over, etc, etc. We have inquired about "skilled nursing," but our insurance will not cover hospice expenses AND skilled nursing at the same time. We get out equipment through hospice, so we can't cancel that, but we can't continue getting NO sleep either. Not sure what to do about that. Lots to think about.

SO -- It has been brought up, by someone that I love dearly, that these posts do not entirely capture what life is like, at least from my perspective. I have to agree. For two main reasons I have kept these posts on the lighter side:
1. I feel a responsibility to not depress people with my antics, b/c they can be heavy, depressing, and wild at times. :)
2. When I sit down to type it is nearly impossible to put down in words how I feel at times. Usually when I sit down I am just trying to report. Although everything I type is from my heart, it is not always the entire picture. I hope you can all understand that.

Therefore -- I will divulge a bit so you can understand the bigger picture.

MANY people want to encourage us in our situation and that is Biblical and right and everything else. We should all do our best to be an encouragement to others that are suffering in one way or another. Right now we are having meals brought to us every few days and IT IS INCREDIBLE! Our friends and church members (who are also friends) are showing incredible selflessness as they care for us, the wounded. I feel wounded.

Aside from the incredible things that people do for us, and aside from the VERY important friendships that have developed over the past couple of years here in MD.... almost every day someone will make this comment: "Things will get better." Bull Sh^*. I am sorry, but God does not promise that anything will get better. Gazillions of people die in pain. Linnea died in pain except for the Morphine that gave her. Linnea's life did not "get better." There are times that we may feel equipped to endure some sort of pain be it mental, spiritual, physical, etc... but should we expect things to get better? Is that really faithfulness? God's intentions may be for us to suffer some string of tragedies until the day we die b/c He deems that to be more useful in His plan then excusing us from tragedy and suffering. That is more probable to me b/c we live to serve Him, however He wants us to serve. God wants us to serve him by living through the death of our children. He expects us to stick to it in our marriage even though we are tired, hurt, grieved, and can't get a long a lot of the time. I do believe that we can gain strength from the Lord in this and he DOES promise to give us the strength to endure it... but, if when people say that they mean "God will give you the strength and it will seem as though things are better b/c your relationship with Him is better," then I could agree, but I don't think that is what people are saying. Also, "God must be preparing you for something big." Well maybe he isn't. Maybe we are suffering so Annabelle can do "something big" with her life and we are just the stepping stones for the great way God will use Annabelle and it will really suck for us. Seriously, why couldn't it be that? Maybe God wants our marriage to be as difficult as it is b/c our constant need to work through things is a better witness to the people God puts in our life than a marriage where things are great most of the time. BTW -- I had a great conversation with a friend about how we are "so strong in the midst of this." I encouraged him to understand that if he sees strength it is b/c he sees Jesus, not us. It is hard to capture my thoughts on this in writing, but..... when I feel weak and rung out, I see how God is using us to infect other peoples lives. I don't want to feel weak all the time, but I do want to see how God is using us for His glory and to "further the Kingdom." I just feel all a mess most of the time. That does NOT, however, mean that I doubt God's goodness, or I curse Him, or anything of the sort. It just means that I am still learning how to deal with the responsibility that God has given me and us.

OKOK -- I don't know if I should keep on typing this. I want everyone to know that we DO feel encouraged by you and we DO appreciate your goodness to us. It gets fuzzy for me when I experience the meaning that others want to attach to our life. I want to serve the Lord b/c I want to serve Him, not b/c I think things will get better or b/c there is some big plan in our future that will make all of this seem worth it. We already think that this is "worth it," but that doesn't really make it less confusing. It's confusing b/c a lot of the time we just don't want it to be real anymore. We want it to all fade away like a bad dream... but it won't and we are learning to live with that every day. I think we will always be learning to live with it every day. The events of our lives are the ones that you want to forget, but you hope you never will. It's like what overcompulsiveness would be like spiritually I guess. We want it to stop, but it can't... but only b/c in the end, it is good.

Monday, October 02, 2006

Troubling Times

Hello faithful readers. This post may be a reality check to some, but a reminder to others. Emily has SMA and her body suffers the effects of it every day. However, the issues below are not normal. Here is an email I wrote to our Doctors about our recent weeks. Often we find ourselves just not knowing what to do.   

Good day!  We are hoping to get some thoughts about Emily and a recurring problem we are having with her... blood coming out of her stomach when we burp her via her G-tube.  

  We have brought this up before and surmised that there was a ulcer type issue in her G-tube track so we have been giving her Prevacid.  Over the last week we have had a VERY difficult time with Emily having loads of air in her stomach at various times, not necessarily associated with using her BiPap.  We have been giving her gas drops for a couple of days now and it seems to be a bit better, things are still not good and she is uncomfortable.  Last weekend I heard her crying in the middle of the night and found that her stomach was severely distended and hooked her up to burp her.  She was in obvious pain and was getting very "junky" in the process.  I burped her, got everything out, and she went back to sleep.  About 15 mins later she was crying real hard again so I hooked her to burp more... then she started throwing up... I'm talking out of her mouth.  Also, the 30cc syringe that I was using to burp her was overflowing b/c of the amount of air pushing the milk out.  I got her suctioned out and burped and she went back to sleep.  This gas problem has continued through this past week and this weekend.

So, this past Friday night she was crying again and we started to vent her again, but this time a lot of air, stomach acid, and blood clots came out.  Not just a few little spots of blood, but quite a few trails of thick coagulated blood. That was all that we saw of it though. Could this have been a result of her instense gas that caused her to throw up? Could she have ripped her Nissan or something?

She doesn't have a fever, although she has had a lot of secretions and is on Omniceph as of Fri for an apparent infection.

We can't figure out what this could be.  Do we need to get some sort of scan done to see if she has an ulcer problem or something else in her stomach?  Is the various medication she is on causing acidity problems in her stomach?  I have strange stomach issues, so maybe she does too.  ??  Her stomach gas/pain causes more secretions which means she is even more susceptible to respiratory issues during this cold season, so we are hoping to figure something out so she can go back to being her happy Emily self.

Thanks for your time! :)


Thursday, September 21, 2006

Linnea Grace Lee - 08/15/03 - Sept. 21, 2004

Two years ago today our family went from four to three when Linnea passed away from the effects of Spinal Muscular Atrophy (SMA). She was and is precious to us and we miss her very much. Hardly a day goes by that we do not think about her, and her life, and her death. Sometimes I want to forget about it, then I cry out of fear that I will. We look forward to seeing her once again when the Lord permits and we will push on until that time comes. For now, God has called us to the difficult task of once again caring for another of His precious little ones, Emily Hope Lee. Please remember to keep our family in your prayers. Blessings and Thank You! :)

Wednesday, September 13, 2006

Sept News

Good Day to all! I just wanted to give you a quick update on our situation, Emily.

Cindy has been living with us for about 2.5 months now and she has been an INCREDIBLE help. I just don't know how we could have survived as we have without her help and love for Emily and Annabelle. THANK YOU CINDY!!! :)

Cindy & Emily

Emily has been fighting a chest infection lately and we have had to up her medicine and treatment regiment. She is hanging in there despite some lethargy and overall feeling of irritation. She is still our little Emily, but.. don't touch her when she doesn't want to be touched!! We have been using the BiPaP on her when she is sleeping as much as possible to help her respiratory system regain its health, but she is the determining factor in whether or not we can use it. She hates it (rightfully), so she has to be pretty tired in order to tolerate it. It really does help her though, we believe. She needs good deep breaths to get the germs out of her system, just like we do regularly. She also needs some help getting deep breaths b/c she is weak. The BiPaP helps her do these things.

Other than that we are ordering a kid kart for Emily as I indicated in the last post. This will help us get her around easier, since she is simply too big for her car bed and stroller now. We manage to use them still, but sometimes it seems pretty ridiculous since she is so big now. With this kart we will have a ramp to get her in and out of the van, so we are wondering if that is still the best thing for us to have... not sure yet.

OK - well that is all for now. We hope all is well in the lives of you readers. Thank you for reading our updates. We hope that by sharing the goings on of our crisis, you can be encouraged in your own. We are plain ole' people with the same problems everyone else faces, we just have this addition as everyone has their own. We still fight, have fun, eat, sleep (sometimes), and work out every day problems. Please pray that we will do these things fairly b/c sometimes we just feel too tired. I am sure you can relate. ;)

I leave you with this thought --

We parents are not here to prevent our kids from experiencing all the bad in life, but to walk them through it when it shows its ugly face.

Wednesday, August 30, 2006


What a busy summer! We have had so much going on with trips here and there to camps and classes for Annabelle... it's been crazy. Well, Annabelle is starting school next week, Diana is planning her trip to the MOPS convention, I'm playing soccer and helping out with music at church... and Emily is mixed up in just about everything. Busy, busy.

A couple of weekends ago we had the opportunity to spend 5 days at The Master's Mission (TMM) to learn about what they do in the world. We had a GREAT weekend with the TMM folks. I'll let you read their site, but basically they are a mission organization that trains missionaries to live in the remote areas of the world, spreading the Gospel as they go. We felt very "at home" with everyone there and felt like we saw a direction that the Lord could be leading us in the very near future. More to follow on this topic...

Emily continues to surprise us by doing so incredibly well. In some respects she actually seems to be getting stronger, although SMA doesn't provide that sort of luxury, but still... she seems stronger. She is so deliberate with her hand and arms that it is hard to know what else to think. She loves playing and has a great personality. I think we'd have a busy little girl if her body would permit. :) ... it is really nice to see. As she grows we get to bond with her more and more. That is comforting and scary at the same time. We are now in fairly unknown point in our lives b/c Linnea did not live this long and she was never this strong. if you are reading these updates and you ever get a chance to come and see Emily, please do not hesitate to hop on over!!
Our next big project for Emily is getting a Kid Kart b/c she has outgrown her stroller and car bed/seat. This presents a problem with vehicle transportation. In order for her to ride in the van we either have to lay her down on a bench seat and strap her to the seat... we don't have any bench seats in the van. Or, we have to figure out a way to fasten the kid kart to the floor of the van in place of one of the seats. Anyway, lots to do and think about. Emily's positioning is the key to her comfort, so everything we do with her revolves around that. thinking... thinking...

Thank you for your prayers and support. Emily has been free of any respiratory infections for a few months now and that is INCREDIBLE. Please pray that God will continue to keep her healthy!!