Monday, December 31, 2007

12/31: Last day of 2007!!

YEAH! This is the last day of 2007. Just think, we won't see most of the people we know until next year!

Emily's having some great days. She's vocalizing a bit over her tube and she's pretty active. Click here to see a video of her having a good time even though she is intubated. She is all hooked up to tubes and stuff, just to warn you ahead of time.

So, today we may hear something from Aetna about the transfer. That is probably optimistic considering our history with them, but we can hope, can't we?! I will ask the docs here to maybe call Aetna rather than waiting for Aetna to call them. Either way we are ready to get a move on. Either go to WI or go home soon -- hanging around here is not healthy on the mind, body, or spirit.

Peace <><

Sunday, December 30, 2007

12/30: More thoughts about a tracheotomy

I wrote an email to a Dr. today and I wanted to share some of the contents about our thoughts on a Tracheotomy procedure for Emily.

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Early in the AM on 12/26 Emily was reintubated. She had been doing incredibly well on biPAP, but seemed to be experiencing some swelling in her lungs and her secretions were getting very thick and gummy. Her "end titles" were getting worse, so her blood was becoming acidic. So, she was reintubated and we think it was the right thing to do. They called in the attending Anesthesiologist and the head of ENT b/c of her difficult airway and the Anesthesiologist was able to place her tube. After the procedure, which was not bad b/c it wasn't as emergent as when we were admitted, the attending expressed his concern over Emily's airway and discussions about a tracheotomy. Later in the day on Thur, 12/26, the PICU attending and the Chief of Pediatric Pulmonology spoke with us at length about the concern and were encouraging a trach for three main reasons:
  • Emily's chords were swollen and her airway was inflamed
  • Emily's glottis was 50% occluded with thick mucus and she "had no cough response to it whatsoever"
  • B/c of Emily's swollen airway and the angle of her airway she is very difficult to intubate so they are worried about her getting sick and having a true emergency
I have been thinking A LOT about this b/c it is obviously concerning. However, these are my thoughts and reactions to the news.
  • I spent quite a bit of time talking with a couple very trusted physicians, Dr. Swoboda and Dr. Schroth, about the situation. They provided great food for thought for us as we considered the possibilities of an actual airway intervention by means of a trach.

  • Of course Emily's airway is traumatized and swollen. She has RSV and has been intubated and received frequent deep suctioning - swelling is to be expected. That doesn't mean that I don't think it is an issue, but I do think she needs time to recover and let RSV run its course while intubated.

  • Her lack of response to the plug in her airway of course is concerning. However, looking at Emily's condition holistically and the progression of SMA -- Emily's cough is good and it, along with the NIV protocol, kept her out of the hospital for over a year. It seems out of place for SMA to have caused a sudden inability to cough when she has been coughing fine all along. I do think that she could progress to that point as she gets older, but not in a week's time. So, extenuating circumstances brought her to the point of not responding to something in her airway - RSV, exhaustion, sedation for the intubation procedure - not quickly progressive weakness due to SMA. The Dr. that placed Emily's intubation tube also administered Lidocaine down Emily's airway before the intubation, so I have to wonder if that is what he saw. Either way, my feelings about sudden weakness still stand. Sudden weakness is not characteristic of SMA from what I can understand.

  • Difficult intubation is common in SMA, maybe even inevitable. So, specialist will need to be called for nearly every SMA child, but that doesn't mean that a trach is proper for daily management. A history of increased inability to react to secretions in the glottis, thus causing frequent desating and sickness, would warrant a trach (possibly), but this is not the case with Emily b/c she is generally very healthy and able to cough... with our help. A trach seems to be the easy answer, but not the best one for Emily. She is still too strong.
So, we have decide not to trach Emily and we have requested a transfer to Wisconsin to see Dr. Schroth so Emily's airway can be seen by someone who sees SMA airways on a regular basis. Emily's anatomy COULD warrant a trach, but my hunch is that she is typical, but I can't make a decision for OR against a trach unless I know from an SMA expert that Emily's airway is a disaster or not. First and foremost, we feel that Emily will have the best chance for a successful extubation and recovery at the University of Wisconsin Children's Hospital since they are intimately familiar with SMA needs, SMA airways, and the NIV protocol. I want to know, for sure, that Emily's airway is as I suspect, typical for an SMA child.

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Hopefully this documentation will help other families brought into the same discussion by medical professionals.

Peace <><

Saturday, December 29, 2007

12/29: PICTURES!!

Hey Ya'll -- here some pictures of our stay at Hopkins. It has been great to have visitors and we were able to get pictures for most of them. Emily is quite happy in all these pictures, even if you can't tell. Thanks for your prayers!!



Peace <><

12/29: Aetna Update

Ok. So, Aetna had a major national call-in number failure yesterday, they claim, so that is why we haven't been able to get through on the phone. To tell you the truth, the story doesn't actually make sense b/c we could call just fine - no one would pick up, but what do I know.

I actually talked to a human being at Aetna this evening and it was a good thing I did b/c they rep that notated the non-par request said we were in WI as of yesterday! After some explanation, the guy I was talking to made some critical changes to the request and told me that our doc could call in with more information this evening so the request could go out as an "Urgent Non-par Request." So, if we can get a hold of the doc that is helping us, she's gone home for the day, this saga will be on hold till we get the decision from Aetna.

So, that is the good news for the day. B/c Hopkins is so serious about Emily's airway being difficult we feel that it is necessary to get her somewhere that sees SMA kids airways all of the time before she is extubated. It is extremely common for hospitals to advise EVERY SMA family to consider a trach b/c of a difficult airway - all SMA kids have difficult airways from what I understand. If we are to make a proper decision for OR against a trach, we will have to have eyes-on from an SMA expert in airways -- the University of Wisconsin Children's Hospital.

I want to mention something too -- one of the positive side-effects of being in the hospital with Emily is that Diana and I do get to spend a lot of time together. Granted, I am making no money while we are here, we tend to get stinky, and we're always tired, but PICU stays allow us to spend time together that we do not get in the midst of the craziness at home. That part is nice -- sorta like time stands still -- then lala land ends and we're back into another discussion with docs, nurses, and RT's about optimizing Emily's care :)

Peace <><

12/29: Am Update - A calm weekend

We have made no ground with Aetna, so we're just praying for a calm weekend while Emily is intubated. We are making sure that the settings on her intubation vent are optimal for her situation. We don't want her breath rate to be too low b/c then she needs to exert too much energy to breath and will get worn out, but we also don't want her rate to be too high b/c it creates a risk that she will start relying on the vent to breath. We need her to keep breathing on her own, which she is doing very well. She is also on a very slow drip of sedation medicine which keeps her comfortable, but very awake and interactive. She's handling the tube very well and we do as much as we can do give her some excitement. :)

Anyway - I'm hoping to catch up on some school work today and tomorrow and we'll keep trying to get a hold of Aetna -- but we may be out of luck until Mon or so. We'll see.

Peace <><

Friday, December 28, 2007

12/28: Aetna woes

This is a discouraging post, but this a blog... so why not, right?!

I am SO ticked right now at Aetna's continued lunacy. They are NEVER helpful, NEVER give us all the information we need, they stop answering the phones before they are supposed to be closed, and deny nearly everything for Emily simply out of principle. Need I say more?!?!

We tried to start the request to transfer to WI today but the docs just got "no" for an answer. It took me prying and prying into policy to find that our PCP has to make a "non-par request" to be transferred to an out-of-network facility if it is an emergency -- that would have been nice to know UP FRONT -- well, I think it's clear that Aetna has put us in an emergent situation with their original ingeniousness to deny Emily Synagis shots, the vaccination for the horrible virus that has nearly killed her - but they decided to go home early today, so now we can't complete the multi-step process to make a non-par request... until Mon... if they decide to work.

We cannot go to WI now and we can't even get the process rolling till next week b/c of their.... argh... very frustrating!

Emily is doing fine, that's the good part. I would love to have her extubated somewhere where difficult SMA kids are the norm, but we can't go ANYWHERE with Aetna's finger up their noses finding ways to make our lives more difficult than they already are.

It is also my B'day today and we had some friends stop by to take me out to lunch and visit. My parents also came up with my a niece and nephew of mine to visit and have a little party. Thank you guys, you know who are you (point, wink, and click of my mouth) for coming up to visit us on my B'day and thanks for the other phones calls, emails, and other web-based communicated notes that others gave me today. :)

I'm tired now. Hopefully I'll have more positive things to type here tomorrow.

Thursday, December 27, 2007

12/27: Evening Update - serious thoughts

Emily had a good day. She is still intubated, but relatively happy despite the discomfort of having plastic in her airway.

Today launched long, gut wrenching discussions about giving Emily a Tracheotomy (a tracheotomy is the actual procedure while a tracheostomy is the result.. the whole or "mouth" in the neck -- just to clarify). All the doctors and specialists here at Hopkins are very concerned about Emily's ability to protect her airway. We share their concern b/c Emily has a very difficult airway (she is very hard to intubate), but we don't necessarily share their approach to rectifying the situation with a Trach. We obviously have to consider the idea b/c of the complications associated with SMA and respiratory illnesses, so we have been struggling with that today... alot. A trach represents a lot of things to me personally. There are the less legitimate concerns of aesthetics - they don't look that great and create speech difficulties as well as mobility difficulties. But the more complicated issues it presents is knowing when to say enough is enough for Emily. SMA kills kids. That's a fact. My impression, be it right or wrong, is that a trach and mechanical vent for Emily would inhibit our ability to assess Emily's happiness and ultimately her desire to live. I never want to be in that situation. I never want to be sitting here suddenly realizing that I've been so focused on Emily living that I'm incapable noticing her suffering. Never ever. That is not where we are now, but it is where we could find ourselves.

At this point we are not going to pursue a trach and I feel that tomorrow we will pursue transferring Emily to the University of Wisconsin Children's Hospital in Madison, WI, where there is an SMA Pulmonary specialist, Dr. Schroth. If not only to get a second opinion about this, but also b/c we are more comfortable with her knowledge about SMA kids difficult airway problems specific to kids like Emily and we know she shares our feelings about the trach. Could we find ourselves in the same situation there? Certainly. But I believe so completely in their ability that I am willing to take that risk. I am praying that these decision we be clear and that our insurance company will respond the way we need them to.

So - that is the situation. The up and up part of this is we are no longer fighting for an SMA protocol in the Hopkins PICU. As of today, any SMA or neuromuscular family that comes to Hopkins should be able to request Emily's protocol for themselves, which is based on Dr. Swoboda and Dr. Schroth's own PICU protocols -- evidence that the system can change. A doctor was given the responsibility of clearly writing how Emily is to be cared for from a respiratory standpoint based on our recommendations. I do not believe this has been at risk to Emily. She has RSV, it's horrible, and we wouldn't be home yet even if we were in Wisconsin. We'd be in nearly the same situation we are in now, minus the trach discussion though. There are still differences in opinion about fasting here, but that can be circumvented by pushing the timely request for PPN or TPN for extubation day and they are willing to negotiate the fasting time a bit.

Peace <><

Wednesday, December 26, 2007

12/26: Intubated and doing well

Emily's had a good day today despite having been reintubated. She seems relaxed, for the most part, and has started to bounce back. We do not have an expected extubation date, but I'd guess a couple of days. This time we will make sure that TPN has been ordered so when they want to fast her she will still have some nutrition. Best case scenario would be that they fast her for 2 hours before extubation and 2 hours after, MAX. I think the fasting issues are the only remaining beef we have with Hopkins - well, that and consistency of care with SMA kids. I hope they've been taking notes about the things we have been fighting for so future SMA families do not have to recreate the wheel, per se.

At this point we are keeping the option to transfer to CHOP open, but we feel like things are under control. They wouldn't let us mix Emily's food here for a day, but that stupidness ended quickly. DON'T MESS WITH AN SMA KIDS DIET if the parents say so, period. It is a serious thing to mess with an SMA child's diet b/c many parents like us have spent countless hours dialing in the nutrition per our child's particular needs. For some kids changing things around is deadly! No kidding.

So, the fact that we are pretty much in control of Emily's routine here says a lot. Change is happening, but there is still a lot work to do. Hopkins needs a protocol for SMA kids that is followed by every PICU doc so all of this teaching/showing the doctors and RT's isn't needed.

Thanks for your thoughts, prayers, and encouraging notes! We are blessed by you!!

Peace <><

12/26: reintubated (back on life support)

Emily is reintubated. She had a sudden respiratory issue yesterday and it was the only option. So, what we understand about RSV is that it really restricts the lungs and can get worse after seeming to get better. This situation is a combo of restricted (swollen) lungs and a thick, thick mucus plug. They don't seem to think that she'll need to be intubated that long and that this can be expected with RSV when it is bad. She was just doing so incredibly well for the last two days - it is very sad. The good thing is they starting hr right back up on feeds following a chest xray and they will be coughing her as well.

The Peds anesthesiologist just talked with us b/c they are concerned about Emily's difficult airway. Every time Emily is intubated it is VERY difficult b/c she has a "short chin." This was an issue w/ Linnea as well, although she was only intubated once for her gtube and her chin wasn't that bad at that point. Anyway, the anesthesiologist talked to us about a trach tube since her airway is so difficult he could foresee Emily passing prematurely b/c of a virus like his down the road causing extremely difficult intubation and increased airway problems. Not sure what to think about that, but her chin issue does not seem to be getting any better.

That's it for now. We are going to discuss transferring care to another hospital, mainly so we can sleep in her room, but we have to figure out where b/c we can't just keep leaving Annabelle with people, even though they are family. She needs her mommy and daddy too. We're going to contact KKI in the morning to see if they can accommodate us. Otherwise it's CHOP or all the way to Wisconsin I think.

Peace <><

Tuesday, December 25, 2007

Merry Christmas!!

- Nathaniel, Diana, Annabelle, Emily, & Aunt Cindy

Monday, December 24, 2007

12/24: Emily's doing great

This evening Emily is just doing great! I held her for a bit after I bathed her and it was nice. She just looked up into my eyes and sorta made little noises. Ok, so I don't know why she was making the noises, but I think it's b/c it felt nice to be held. :) I can't wait to get her home again!

If things keep going the way they are we'll be home before we know it. :)

I wanted to point out that, despite our disagreements with the docs and protocols here at Hopkins (there are MANY disagreeable points), the nurses and respiratory therapists have been great. They've worked with us through the most frustrating of times and always respected our expertise while looking after Emily with their own personal touch. Thanks! In my opinion there is still a lot of work to do if Hopkins is going to be a safe place for Type I SMA kids, but I think the right people are here to make that happen. I plan to do what I can to encourage those changes!

Peace <><

We're up - Emily's doing well

Good - the site's back up and Emily is doing pretty well. She is on 30% oxygen and her BiPAP setting are 20/4 with a backup breathing rate of 24 breaths/min. This is good. We had to go up on her pressures a bit in order to decrease her oxygen, but she is looking good. She has some atalectisis in her right upper lungs, to be expected with RSV, so we are working it out with Q2 treatments, PRN cough assist, and The Vest every 4 hours. For the non-medical -- that's fancy talk for "we're very busy." Emily is getting aggressive respiratory treatments 12 times a day vs. three standard treatments/day when she is home and well.

We're not home yet, so there is still a lot of work to do here at the hospital to make sure plans aren't changed and folks are staying on top of Emily's care every second.

Thanks so much for your words of encouragement and prayers. Hopefully we will be home soon!

Peace <><

Server Down

If you are reading this our website just came back online. There was a MAJOR problem with the server our site is hosted on. Sorry for the inconvenience.

Sunday, December 23, 2007

12/23: Feeds started

It is nearly 5 and Emily's feeds have been started. 13 hours without food when the recommended maximum fast time for SMA kids is 4-6 hours. :(

However, Emily seems to be doing well. The affects of the longer than needed fasting time will add difficulty to our home life as we help Emily recover at home. She is happy to have her tube out and she is getting used to the BiPAP again. It is good to have her off the vent b/c weak kids like Emily can become dependent on the vent, so we are so happy that she has been extubated.

Thank you very much, again, for all of your prayers. Blogging is good for me, but my prayer is that my posts are good for you too. I pray that I am providing a window into a real-life situation that is not "doom and gloom" even though the outcome of SMA is not life. I pray that you can find encouragement in the midst of our discouraging moments b/c we do not "blame God" for any of this, but rely on Him for strength and purpose as we deal with a parents worst nightmare, a suffering child.

Thanks for visiting. None of this is over yet, so please visit frequently if you desire. You can even subscribe to the updates by clicking here.

Peace <><

12/23: Extubated, but not right

Emily has been extubated. The breathing tube has been taken out and she is now on BiPAP, an external breathing assist device that we use regularly in Emily's care. The problem is that she is very irritated and very cold. If her body does not have nutrition and protein to break down, causing energy (HEAT), then she is going to be cold. If there is no nutrition to break down then there is only her own muscles and fat, which has has very little of - or none at all, depending on how you look at it. Emily has NO reserve for this situation and THAT is why we have advised the docs against it. I still cannot understand why we are even dealing with this when there is documentation clearly defending what we have been talking about regarding nutrition, and everything else SMA related for that matter.

We are praying that Emily will stay strong and fight through this and that the docs are learning something. I will say, with some confidence, that we are done with Hopkins after this. We are going to do everything we can to get Emily somewhere else if she is getting sick, avoiding these situations altogether.

12/23: noon

It's nearly noon and Emily is still sleeping away. The attending Doc agreed to extubate at 7 hours and try to start feeds 4 hours later, but she has to wake up. I would be glad for the compromise, but with Emily's extremely low weight the risk of her becoming catabolic from the lack of nutrition I am not pleased.

All of that aside, my heart (actually the Holy Spirit) reminds me that I am not in control, and neither is the Doctor that I do not favor this morning. THE physician, God, knows what is happening and I do believe He is watching over us. I have to relax - for my families sake as well as mine.

Imagine, though, what it is like to live at a constant and accepted level of stress with your child's life hanging in the balance with every breath, for years. We've live by tried and trued ways of managing Emily's health, so we have grounded opinions, especially since the Standard of Care for SMA document was published. It's just crazy that we are having this fight yet again. The alternative to fasting is TPN, which is essentially food though through an IV. They
should have ordered TPN yesterday morning so we'd have it for the extubation, but it wasn't ordered, so now Emily has to fast. Why, b/c we didn't bug them about it. Lesson learned: bug the docs about everything so nothing will be left undone.

Please Lord, give me faith to understand how to deal with these frustrations when my daughters well-being is in the hands of someone inexperienced with her condition and not willing to adhere to the recommendations agreed upon by the actual experts.

Peace

12/23: Two things

  1. I guess I'll start with the article that is running in Washington Times today. It's provokes a funny feeling when your family is on the front page of a national newspaper, but what a great thing for Spinal Muscular Atrophy (SMA)! Thanks, Julia and Joe, for speaking with us and sharing our story.


  2. I am pretty frustrated and disappointed this morning. Hopkins has decided that Emily can stay off feeds for up to 18 hours. Totally unacceptable and unnecessary. A recent publication authored by SMA experts (one of them the top neurologist @ Hopkins) clearly states that SMA kids are NOT to fast for more that 4-6 hours b/c of complications it will cause. There has been one doctor advising and discussing with us about Emily's care, he's not hear this morning - SO, these feeding and extubation decisions are being made by a doctor unfamiliar with Emily and indifferent to published writings of SMA experts about how this is supposed to be done. So, now we are stuck here with this doctor that wants to do something OTHER than what was planned and against the decided upon standard of care for SMA. This is VERY uncomfortable for me and I am not excited about what is happening right now. If there was a way to transfer even across the street to Kennedy Krieger, I'd do it. He just looks at me and shrugs, telling me, "we'll do everything we can do make this work." You'll do whatever you feel is best and then, no matter what happens, you'll say you did everything you could. We've been reasonable and respectful while we've been here. This is just asinine. Unless things change we are scheduled for a 12pm extubation, rather than the 10am we had discussed.

Saturday, December 22, 2007

12/22: Evening Update

Today was another good day for Emily. She did have a pluggin episode (thick mucus plug in her lung), but they were able to get it out and everything was quickly back to normal. Hard part is that we were gone for a short time when it happen. We hardly ever leave Emily alone and hardly anything has happened this whole stay, then she plugs while we are away.

Anyway, the plan is to extubate tomorrow morning (Sun) at 10AM. She has pretty much been weened from everything, so it all looks good at this point. I don't think that means we will be out for Christmas, which is the #1 question we get, but our time is not that important to us anyway, so whatever happens is fine with us so long as we have our Emily. :)

We were visited by some Church friends this week and it was nice to spend some time with them. If you ever have friends in the hosptial, pay them a visit if you can. Bring food, that's always a big hit! They may not know what to say, but it's always nice to have people to hang out with during these times. If you can't, shoot them an email or something b/c I am sure they will appreciate it. :)

Take care! Hugs your kids or someone else's -- preferrably someone you know ;)
Peace <><

12/22: Looking Good & Washington Times

Emily continues to look better. Today we're going to talk about
extubation (taking the breathing tube out). She is nearly on room air
rather than supplemental oxygen, so we're lookin good in that regard.

Annabelle is here for the weekend so we are going to take her to the
aquarium, providing everything is going well with Emily. The Ronald
McDonald House has free tickets to various places around town, so we
try to do something fun with Annabelle when we stay here.

Anyway - we're praying for a rapid recovery and a quick extubation.

ALSO - The Washington Times is running a story on us tomorrow, Sun.
12/23 - so, keep your eyes peeled for the article. :)

PEACE <><

Friday, December 21, 2007

12/21: A good day

All in all Emily had a great day today. Her heart rate has come down a bit and she has been more comfortable. We fell that the cough assist has really helped make her secretions accessible for suctioning, which is going to shorten her intubation period. She is on just a little O2 and we are hoping for an extubation before too long. She is still breathing heavily, but that has also slowed a bit too, so she is resting more now.

Anyway - that's the news and our spirits are high. Thank you very much for checking in. We are not out of the woods yet, but things are looking good and we are glad that we caught this early. Please continue to pray for Emily to stay strong and thank the Lord for his goodness in this. We are blessed to have you all praying for us and thinking about us.

We had a nice visit from a co-worker, Mike, who brought us lunch today and spent some time with Emily -- even participated in CPT (Chest Physiotherapy) that Emily gets on a routine basis. It was nice to have him here for a quick visit and the food was great! Thanks Mike.

Peace <><

12/21: AM update

Emily is looking so much better this morning. We've been warned that the worst days for RSV are days 4 or 5, so we're watching her very closely. Depending on when she actually caught RSV today is day 4 or 5, I believe - but she's doing much better than yesterday so far. RSV is horrible.

Thanks for the email notes and words of encouragement. Thank you for your prayers and well-wishes. Hopefully we'll be home soon, enjoying the holidays.

Peace <><

Thursday, December 20, 2007

12/20: HOT NEWS!!

At about 4pm today Hopkins mechanically coughed Emily for the first time via her intubation tube!! I believe Emily is the first a Spinal Muscular Atrophy patient to receive Cough Assist treatment while intubated at Hopkins. They had a negative experience a few years back trying this (not sure what disease it was associated with), but we've convinced them to do it with Emily and it went very well. We got some good thick stuff out which is what we we believed would happen. We had some help from other Docs around the country, so thanks to them. Hopkins WOULD NOT cough Emily via her tube last year when she was intubated, so this is great progress for Emily and for all SMA patients that visit Hopkins in the future!!

This changes the situation with going to CHOP. It is still a more comfortable hospital for an SMA family to be in, but we'll see how things go.

Thanks for your prayers and for checking in.

Peace <><

12/20 Update: transfer requested

Finally getting to a computer. Emily is having a tough day. Yesterday was good, today is rough. Her HR and respiratory rate has been high, her O2 sats have been low, and she has been very uncomfortable -- not the combination we're looking for. This is s difficult process b/c we believe that Emily's condition requires a more aggressive approach if things are going to get better. We continue to discuss this with the PICU docs and it seems like they are changing their minds a bit, but there is a great deal of scepticism about what we are asking for. Oh yeah - sorry, we are asking that the Cough Assist, a machine that helps Emily cough, be used via her intubation tube (ET tube). This is a tube that goes into Emily's airway to give direct access to her lungs. This enables mechanical breathing, or assisted breathing, a well as deep suctioning. Anyway, they don't want to cough her b/c of some reservations based on past negative experience. There are good arguments either way, but at least three other big hospitals are coughing intubated kids with GREAT success that they claim leads to shorter intubation periods and, therefor, quicker recoveries. No studies have been done, so Hopkins is sceptical.

BOTTOM LINE: Emily needs respiratory care that is tailored to her needs, not based on standard PICU protocol. We are asking for tailored care and that is not a typical request.

At this point I am not interested in challenging policy anymore... Emily's life needs to take precedence RIGHT NOW. If that means going to CHOP, then so be it. Maybe I shouldn't press it here since they don't know what they are doing when it comes to coughing intubated kids, so maybe we HAVE to go to CHOP. That's what we're trying to figure out, but there are risks with transporting kids like Emily too. Oh the mess of it all.

So, this morning they changed out the ET tube for a bigger one b/c of a massive leak and we are trying to get Emily calmed down.

Please pray for Emily to remain resilient as we negotiate her care (seems like an insane thing to negotiate). Please pray for clarity as we discuss these things with medical experts. Please pray for a quick recovery. Please praise God for the things He provides for us during these difficult times.

Peace <><

Wednesday, December 19, 2007

Intubation - Pneumonia

Emily is intubated. I can't believe it came to this. Just Monday she was as happy as a clam. On the way to the ER last night she was satting the highest in two days, 98 on BiPAP, and giggling on the way. We get there and as we enter the ER she starts to drop. We started up CPT and coughing again and that was the end of it. Must have shifted gunk around to really make matters extremely worse. They had a very difficult time getting a tube.... ahh. I can't believe this. Her right lungs has very bad pneumonia and the docs kept commenting, "there is A LOT of stuff in there."

She is stable now, but on extra O2 and still under some of the anesthetics. I'm home getting some things. Poor Annabelle is sick too and was throwing up big time last night. She'll be home with her aunt while we are at the hospital. I have mid-term exams this week, this is just very frustrating (understatement).

We are tired. Please lift Emily up in your prayers. She has a lot of life left to live!!

To the ER

We're taking Emily to the ER. She's just not doing well. More updates later today... Tuesday.

Peace <><

Tuesday, December 18, 2007

2. Quickly Sickly 1. Aetna Approves!

After yesterdays great appointments a couple of things changed very fast.
  1. Aetna has approved the infusion therapy for Emily! This is great news. We will have to re-schedule since we had to make a lot of changes when it was denied - so I'll make a note here when we are going to be heading in for those treatments.

  2. While we were out Emily was quite happy, but her heart rate started to become elevated on the way home -- common for her if she's getting worn out and needs to take a good rest. However, after we got home it continued and was soon accompanied by heavy breathing, a fever, and a very unhappy and lethargic little Emily. By bed time we had antibiotics, but through the night her HR has been in the 160-170's which is high for Emily. If her HR is that high it means that her body is working very hard. She continues to have rapid breathing and flaring nostrils, dropping oxygen saturation --- but very little coughing. {shrug} Not sure about that. We've picked up on more frequent treatments. Please pray that Emily's little body will turn this around so she won't develop any serious illness. Ok - some of that request is selfish, so we won't have to spend Christmas in the PICU, but I'm allowed a little bit of selfishness, aren't I? :)
It is tough to see Emily's body working so hard -- it takes me back to Linnea's suffering and the road that Spinal Muscular Atrophy takes us all down at some point. That is where my mind is.

BUT, enough of that - we are not there with Emily. She is sick, but will pull through it b/c she is so strong both in body and mind -- she's our little Emily. :) Still, I look forward to the return of Jesus, when all of this sort of stuff will be over. Come soon, Lord Jesus, come soon!!

Monday, December 17, 2007

A good weekend and a Dr. visit

We had a good weekend. Emily was happy and well and we seemed to get a lot done -- and Diana and I even got to see a movie. :)

Sat morning we were visited by Julie Duin, a reporter for the Washington Times, and Joe Eddins, Chief Photographer for the Washington Times who are doing a story, of some sorts, of our lives and Spinal Muscular Atrophy. We talked for quite a while and Joe accompanied us to church the next day and then to our appointment this morning. Great people! I'll post a link to the article when it is all said and done.

We went to Hopkins this morning to meet with Dr. Crawford at the MDA clinic and get fitted for a TLSO and leg splints. Even though Emily has no scoliosis (HURRAY!! b/c almost all SMA kids do), we need a TLSO to best support her weight when we get her in a power chair. Dr. Crawford did not think that she needed a TLSO at first, but after some discussion we all agreed that it would provide good support while in that seated position. His main concern was that sometimes TLSO's can invite scoliosis b/c kids can get twisted in the clam-shell. I can totally see how that could happen, but we're talking about giving Emily better support while she is driving her chair, which is a negligible amount of time in the scheme of things.

Emily needs the leg splints to keep her feet/ankles from continuing to deform. Well, we didn't get all that we had expected out of the visit (measuring for braces), but we did meet the man that is going to make it happen. I don't recall his name right now, but we will be making another visit soon to get all measured up. :) More to come on that...

We also discussed the infusion therapy with Dr. Crawford and he confirmed my suspicion that nobody really knows what to do about bone density in SMA kids b/c no history has been gathered to compare against. Also, it doesn't seem that there is any definitive evidence showing that the therapy actually corrects the issues... at least not with SMA kids. Anyway, we also discussed the fact that there are very little risks associated with it, so what are the reasons for NOT going through with it if Aetna will pay?? We have heard from one family that has been advised against infusion therapy for bone density b/c, the opinion is, it can cause brittle bones, but I've found no supporting evidence for this as of yet. So, yet another area of SMA where there is very little experience. Dr. Swoboda, from Primary Children's in UT, has explained that these infusions are pretty standard for her SMA kids if their density is as bad as Emily's, so I think she has the most grounded opinion in the matter simply b/c she is seeing more SMA kids and density scans are a normal part of the studies going on at that hospital. :)

So anyway, we're not sure where the infusion stuff is going to go - but we are getting bracing soon (Lord Willing) and the visits today were very productive and good. It is great for us, a somewhat unique SMA I family who's child has beaten the odds, to "show off" Emily to Dr's that are top in their field -- makes us realize that we can have an affect on medicine if we are willing to put ourselves out there and stick up for our kids.

Peace <><

Saturday, December 15, 2007

Emily's Awesome - Aetna is NOT

I'll start this with something positive, b/c the last part is not so great.

On Monday we are going to Hopkins to have Emily seen and fitted by an Orthotist for some bracing (torso and legs). B/c of her inability to support any weight with the trunk of her body or legs, they are starting to grow in ways that can be damaging to her body and even painful. It is essential we get good bracing for her. Additionally, we feel that these braces will help her sustain a sitting position at a great enough angle for her to drive a power chair. Very exciting for us. We're praying that it will all work out and Emily will enjoy some of the "freedom" that a 2-year old wants and needs. Please pray that the visit will go well.

Also - Emily's been communicating pretty well with us. Since she spends her nights with nurse Udeme, she love talking with her too. It was so cute the other day when Udeme arrived and was talking with me about something or other. Emily was in her medical chair having some sit-up time and saw that Udeme wasn't paying attention to her. She started sorta yelling for Udeme. It was like "HI! HI!" (look over here... here I am). I told Udeme that Emily was calling her and when she turned Emily just smiled, looked her in the eyes and babbled on. It was precious and Udeme felt so blessed to be wanted so entirely by little miss Emily at that moment. She's great. I'm tearing up a bit typing b/c it was so neat to see Emily express herself like that to Udeme. :*)

-----------------------
I sat down to post to this bLog b/c have had a lot of things on my mind -- good things - things about what Jesus said, "when you refused to help the
least of these my brothers and sisters, you were refusing to help me." My heart has been heavy over thoughts of SMA kids around the world being discarded by their parents, the state (country), or relatives b/c of the difficulty it presents in life. People all over the world chose their own lives over the lives of "the least of these" - out of convenience, but they are also refusing Christ himself. I tell you the truth, we will care for the least of these until we breath our last breath, and Christ expects the same from everyone of us.

Along those lines (
doom and gloom part - read at your own risk b/c it may make you really mad), I am really ticked at our insurance right now. We received news today that Aetna has denied the request for Emily to get the infusion therapies this coming week for her sever osteopenia. Yet another decision by Aetna HMO to NOT pay for Emily's OBVIOUS needs. This is even worse than their original decision about nursing, based on the statement by their all-knowing medical director that "there is no skilled nursing need in the home" when we had more medical equipment than most nurses would know what to do with, no offense to the great nurses out there. :) In fact, if we take Emily to Johns Hopkins they won't even let her on the Critical Care floor b/c they say the nurses aren't trained enough -- honestly, that is what they told us one time at the ER.

Anyway, there is well documented information about Emily's
sever osteopenia and she is very high risk for broken bones. In fact, she is so high risk that the docs do not feel comfortable waiting to give her the infusion therapy we were scheduled for this coming Tue-Thur, but we can't go fwd with it b/c of cost. Thanks Aetna! We'll send you pictures and a sound track of Emily suffering if any of her bones break - I'm not joking.

Our only option is for Emily to receive this IV therapy to strengthen her bones so they are less likely to break if they were subjected to even small amounts of stress. So, b/c Aetna is more concerned about business than seeing the reality of our little girls LIFE, we have to now go through the appeal process, which can take many months to complete with no assurance of a positive outcome for Emily. What is wrong with these people?!

I am told that 70% of claims are denied b/c most people don't appeal the decisions by the insurance company. I don't know what they actual numbers are, but I really don't care either - my little girl should not have to be subjected to bullying from companies like Aetna that could seem to care less about her condition. It's maddening!

I am trying to respond, in my heart, in a way that is redemptive - but I just can't at this point. I can't respond they way I want to when someone makes an unfounded decision that makes our life more difficult than it already is -- like we are asking for more than we deserve... for a favor of some sort. We pay our inflated insurance costs, so just give us our care.

Thursday, December 06, 2007

Family Fun

Well, we finally found one of our lost cameras -- it was strategically located in Annabelle's doll house. :0 Anyway, so there are some fun pictures from the Fall and a few from our recent SNOW!!!

Emily has been so happy lately, it is great. She's been babbling on and on and really enjoying life. Best of all, she has been well! We are set for some big appointments in the near future, so please keep us in your prayers. Our first appointment will be to Hopkins to get some measuring done for some bracing. Emily's body cannot support it's own weight in any way, so in order for her to get good support she needs a few braces. We plan on getting her into a power chair soon and these braces will help in that regard.

The next appointment will be a 3-day in-patient stay at Kennedy Kreiger Institute (KKI) where Emily will start infusion therapy to help her bone density. These therapies can be tough on our SMA kids b/c they can cause flu like symptoms, hence the 3-day stay. KKI wants to move fwd with the therapy, but does not want to risk any negative side effects happening at home. We are a little anxious about this, but Emily's bone density is very poor and she is at such high risk for fractures that we have to do something about it.

So, on to the pictures:



Friday, November 23, 2007

The Sovereignty of God

I didn't want to overshadow the last news of tonight (story about us in WORLD), but this video was too good not to post. Please take a moment to hear John's testimony about his faith. I need to watch this video and listen to John about 100 times a day -- I have a lot to learn. Oh yeah, he has SMA!



Peace <><

WORLD: In the "news"

Hello family and friends,

Just wanted to let you know that there is an article [about us] in WORLD magazine today. :) A good friend of ours wrote it a while back and VOILA! it was published for today's volume. Thanks so much Laurel!! Click HERE to get a short snippet of the article.

I'll get a new picture up on the bLog soon.

Peace <><

Monday, November 12, 2007

A real story

Frustrating story of the week (this may make more sense to kids with special needs, especially pulmonary needs):

We were at the Pulmonologist's office and finally got called into the vitals room. We were called in too early and stood in the hallway. Emily was still sickly at that point and needed to be suctioned and would soon need to be coughed. Diana turned on the suction and the nurse came out and said, "Um, you can't do that in the hallway." I looked up so fast and said, "Excuse me?!" She ushered us into the vitals room (they're all too small) and we plugged in the cough assist and started getting out the tubing b/c Emily really needed some coughs... it was urgent in other words. She looked at us again and said, "Um, why don't you just hold off on that so we can weigh her and then you can do her 'oral care' when you get to the room."

Holy Freakin' Crap Lady!

I said, "Um, we will cough and suction her wherever we need to, so you can just wait." Diana looked at her at the same time and said, "She can't breath!" You'd think we were in a Department store or something, rather than a SPECIALTY PULMONARY CLINIC!!! Geez Louiz. So, we finished and she didn't say anything else - a wise decision on her part. Maybe she saw the steam coming from my ears and realized it wasn't a sign of my love for her. She then led us to the room -- the one she previously referenced in a way that suggested it was right around the corner -- it was like a city block away! My goodness. The rest of the folks there were very nice and the Pulmo Doc loves us for living the NIV protocol. The whole appt ended up being a flop b/c Emily was just too junky that day. :/

Saturday, October 27, 2007

Can you believe it?! A new update!!

Hey Folks,

Thanks for the emails and phone calls reminding me that I've been lax on my bLogging. :) It's been an incredibly busy month, to say the least. School, work, Dr. Appts with Emily, Dr. Appts for me, etc, etc. Not an excuse, but it's just been busy.

First things first -- Emily has been doing GREAT. She really has and it's been amazing. Now, that means different things to different people I have come to realize. Emily still gets 3 respiratory treatments a day which includes Chest Physiotherapy, assisted coughing, and suctioning. She is still suctioned and coughed throughout the day outside of treatments to help control her secretions, and she is still fed through her Gtube. She cannot sit up, roll over, swallow effectively, or do pretty much anything else that "normal" kids do. She requires manual stretching and physical therapy throughout each and every day to prevent malformation of her joints, and her bones are VERY fragile due to Osteopenia, or low bone density. She has many visits every weak from the public school system to work on various speech, physical, and other therapies - and she always sleeps with her BiPAP on.

So - the reason she has been doing so great is b/c she's been healthy for quite some time now -- very few sicknesses and none of them very serious. She's been working really hard to get some words out, but she's having a hard time getting the coordination between her little mouth moving and getting those sounds out. Regardless, she has been very "verbal" and just loves being with the rest of the fam. :) The weather's been very nice (cooler) so her walks have become a more frequent again. We've been working on sitting her upright more and she's been tolerating it very well -- so, all in all she's just been doing real well.

In our recent Doc visits with her we have been talking about diet, joint and bone bracing, and assistive technology for better communication. These discussions have been good, but I don't know if I feel the docs are being aggressive enough. Since very few docs have seen little kiddos like Emily, it's pretty much a guessing game, so we have to make sure (as usual) that we are weighing the options and getting good feedback from other parents in our situation. Sometimes we agree with the docs, other times we don't. Gets tiring after a while, but the show must go on. More to come on this...

Other than that sort of stuff we just been navigating through the whole family/disease/marriage/parenting/
religious/occupational/death/life/
meaning of life/bills/schooling type stuff -- you know, the usual.

This weekend we are in GA for my 10yr HS reunion... remembering the good and bad times. It's been fun to see some old faces! The trip has had a toll on Emily though. Didn't help that I came down with a cold the day before we left, so, inevitably, I am pretty sure that Emily is coming down with it too. It's hard to avoid each other when we're on the road. :( In fact, I am sitting her at 2:11AM typing this b/c I can't sleep with my nose emptying like a garden hose left in the yard. Just can't sleep. We'll be happy to get back home Sun night. My mom's along to help out, so that is really nice.

Ok - looks like I've accomplished some "catching up" there, so I'm gonna sign off now. Thanks for keeping up with the bLog and please keep praying for us. We truly appreciate your care for us and I hope, in some way, that I can reciprocate your love and care by sharing our life through this bLog. One thing I feel God continues to teach me through all of this is the necessity for all of us to share our experiences with others more often. We all tend to be TOO private.

Peace <><

Sunday, September 30, 2007

A week in our life...

Ok - this has been an "interesting" week and it ended perfectly. :) I am NOT looking for sympathy - just thought I'd share real life experiences and some pics. :)

Mon - I went to Urgent Care for bad abdominal cramping
Wed - I went to GI Doc to get tests done
Thur - Emily appts at GI doc all afternoon
Thur Night - I woke up with an intense migraine - threw up
Fri - Annabelle comes down with something and decorates the house with stomach content, and continues to do so through the night
Sat - Annabelle continues "decorating through the day and eats something with Cashews (she's allergic) and suffers even more
Sat PM - I nearly fall out of dad's new boat and face-plant into the side in the process, splitting my eyebrow open
Sat Evening - Me and Annabelle both head to Urgent Care: stitches for my eye and dehydration for Annabelle. Turned out that Annabelle has Strep Throat and is an antibiotics. When I entered the clinic I asked them if they could stitch up my eye and they said "yes." After two hours of waiting they came in and told me that they "don't do face sutures - go to the ER" !!! Anyway, 5 stitches and a tetanus shot later I am writing this bLog entry. The fall was actually quite comical and my dad can't tell the story without laughing through the whole thing.
Sun - Spend the day WITH Emily and my parents on their new boat - no incidents :) Except for my dad cutting his leg :*(

So - all of this ended with a BEAUTIFUL day on the water with my parents. Check out the slide-show below for the pics. This was a first-run with Emily, to see how things could work on the boat. She LOVED it! Everything worked out and we know what to change for the next day-cruise to make it even better. I took some shots of the setup - next time we will probably leave the EasyS stroller in the van... she didn't really need it.

Oh - BTW - Diana was awesome through all of our issues -- she never gets hurt (except for her suspicious "broken arm" mishap last Winter) or sick. God has blessed her that way -- or blessed us, depending on how you see it.



Peace <>< Nate

Friday, September 21, 2007

Linnea: 8/15/03-9/21/04

We are thinking of Linnea today. She went home to our Creator 3 years ago at 0158 today. Feel free to visit her site here... Give your kids some extra hugs and thank God for them today. Enjoy them :)

Monday, September 17, 2007

Aycaramba!

I can't believe it has been so long since my last post. We've been very busy - some good, some bad - but busy nonetheless. A few things have changed since the last post, I guess. Emily is getting her 2-year molars (a tiring event to say the least), Annabelle is in full-day Kindergarten, Diana is taking a couple of college classes, our day nurse took extended leave, our night nurse cut down to only three days a week, and my job started getting busier. Goodness gracious. When it rains it pours, eh?!

Anyway - we're surviving, that's all I can say at this point.

So, my company rents out Six Flags once a year and we get free tickets. This year we took Annabelle... her FIRST theme park experience! She was POOPED by the end of the day, but had a blast while we were there.

Emily is doing really well. We're along for the ride, so we never know what to expect. She has had some ups and downs over the past few weeks, but she's a trooper! Today is a HAPPY day for her. Just yapping and trying to get some words out. I think when she gets it down it will never be silent in our house again -- between Emily and Annabelle. :)

Thanks for checking in. - Peace <><

Tuesday, September 04, 2007

9/3: NYC and Doing Better

Hey - we were gone all day, but for good reasons - we got out of the house!! Thanks for your emails and prayers. Emily's night turned out much better than expected. She was coughing and just full of mucus - high HR - Fever - Low Sats -- junky lungs -- the works.... until she pooped EVERYWHERE. Yes, she is still "sick," but I think the culmination of "internal events" looked just like a lung was about to collapse.

Anyway, she was an incredibly happy little girl today and we took a day trip to Central Park, NYC. 10 hrs in the car all together and lots of BiPAP time - but it was BiPAP at home, or BiPAP in the van and lying around Central Park, so we treated ourselves to the later. She loved it and there wasn't much traffic, so that was cool.

She's still got some getting-better to do, but it is a relief to know that it was mainly a tummy poo-poo war going on. :)

Some pics from the day...



Peace <><
Nate

Sunday, September 02, 2007

9/2: Emily's Sick

Hey folks,

Please pray for Emily. Not sure how this night is going to play out, but Emily's been getting gradually sicker as the weather cooled and she's not doing so hot - sats hovering around 92 and dropping into the mid 80's over and over again. HR going up to the 180's from her normal 120-130's. Wondering if we should head to the hospital - or drive to CHOP so we don't have to deal with JH's. Hopefully our nurse will be on top of things tonight, but I'm sure Diana will be up most of the night. :(

Friday, August 24, 2007

Emily + Sitting

I thought I'd share some pics of Emily. After realizing that she has some serious bone density issues I started thinking, "Man, we need to get her in to some seats and standers pronto!" So, what we realized is that she handles it quite well.

Here is a pic of her in her Tumble-form


Another in the Tumble-form in her therapy swing
.

Here is a pic of her in her EasyS

Does anyones else use the chair this way? If we put the chair all the way up in a sitting position, but tilt the base all the way back, she does well and is able to use the tray. We were actually quite amazed!

I also did a little video of Emily in the EasyS

I am sharing these b/c Emily was NOT able to do this a year ago. I can't quite explain that, but she could not tolerate these positions well at all. Now she can. :) We are continually amazed with her. When she was diagnosed at only 5 weeks old she was digressing fast, so we were never expecting to see her like this. Hopefully the therapy we are getting into with Kennedy Krieger will take her capabilities and make them even stronger.

Peace <><

Monday, August 20, 2007

2nd B'day Pics!!

Hey everyone - Pictures from Emily's 2nd B'day party are up!!! Please visit Emily's Albums (I just made some changes and the link is different now) to see the photos.


Peace <><

Moving on

I wanted to make another post to move us on from Linnea's B'day - so here is another post. :)

Soon I will be putting some photos of Emily's B'day bash, so please be on the lookout. If you have any pics from the party and wouldn't mind sharing them - please send them to us and we will include them.

Also - a bit of clarification on the book project for Eminnea Inc. I have already started receiving some writings about disabilities to be included in the book, so thank you very much for the time you have put into this project. I want to add some direction, just in case anyone is confused -->

We have all seen or known someone that is or was disabled. We have all felt and thought something when we saw that disabled person. Some of us have learned life lessons from the disabled people we have seen or known. Some of us have learned to love more. Some of us have learned to live more. Some of us have learned to be more accepting. And, some of us have learned to accept ourselves.

What have you learned from your own interaction with disabilities? What has that interaction taught you about your own life? How has it changed your life?

An example (quick and straight from thought to keyboard, so be forgiving) - I have known only a few people with CP (cerebral palsy). To be honest, most of my life I felt very uncomfortable and awkward around someone with CP. I didn't know why, but I felt weird. I didn't know what to do if I saw someone with CP! I didn't know if I should stare, look away, ask questions, cry, pray for them, avoid them, talk to them -- if I did, how would I talk to them? - would they understand me? -- these thoughts would go on and on... I didn't know what to do. As time went on I realized that it wasn't CP that was uncomfortable to me -- I was uncomfortable with ME. I began to understand how unaccepting I was of people that were unfamiliar to me. Not just someone with CP, but people in general. I was ashamed with myself for only feeling comfortable with people similar to me in size and shape, athletic ability, musical ability, religious beliefs, etc, etc. The presence of disabilities in society teaches me to love people for who God made them to be. Disabled people in society have taught me to work hard and not take for granted the things God HAS given me. Disabled people in society have taught me that there is NO way I can fathom what someone else can accomplish with the resources they have. But, most of all, the presence and accomplishments of disabled people in society have taught me to cherish, love and support my daughter, Emily, b/c there is no telling what she too can accomplish amidst the challenges of her own life.

That is what this book is about. :)

Peace <><

Wednesday, August 15, 2007

Linnea


Our second daughter, Linnea, would have turned four today. We're thinking about her...


Peace <><

Monday, August 13, 2007

Time

Sometimes it can be so hard to know what to write. Other times it is as if I can't stop writing. In this case I have needed some time to sort out things in my head. There has been a whirlwind of activity with Emily turning two, and a sense of urgency that seems to recoil my life in preparation for the next set of battles to endure.

Emily is two - what a relief! "2" doesn't mean we are out of any dark valley, or that Emily will "be ok" b/c the facts remain the same, no matter how much joy we feel - or sorrow we feel we have avoided, SMA is terminal - that's the reality. The upshot to that is the longer these kids live, the more likely it is that their immune systems will survive the inevitable respiratory infections that occur throughout the year. --> I don't mean to be depressing or negative - maybe it's because Linnea would have been a beautiful little 4 year old in only two days -- I don't know, but it has been a tough couple of weeks.

Maybe it's like Buyer's Remorse. I've invested so much effort into getting a van, having Emily's party, communicating with medical people, anticipating the changing weather which will cause respiratory issues - thinking of Linnea. Too much. Since many of these things, thankfully and miraculously, have come together I find myself sorta depressed -- or stressed maybe - at the lack of something to fight for. This can be dangerous b/c then it turns into finding things to fight ABOUT.

So - we are getting the non-profit setup very soon. Paperwork for incorporation is in - filling for 501(c)(3) status is next. I can explain what we hope to accomplish in another post, but keep eminnea.org in your bookmarks b/c there will be a site there soon. Our non-profit will be called "Eminnea Inc." which, as you may have guessed, is a cross between Linnea and Emily.

Something to think about: I recently hear a discouraging story about a Pulmonologist in Knoxville that said nothing should be done for Type I SMA kids. In fact, he went as far as telling the Type I child's father that he doesn't think society benefits from their existence!! As you can imagine, and may be feeling yourself, I was INFURIATED at such a remark... and saddened. My heart just sinks down to my callused toes when I hear things like this. So, I started thinking (I know, scary) - rather than calling that guy and telling him to go to hell, there must be a more loving, convincing, and diplomatic approach. It started like this: Why don't I email all the people that keep up with us (up to maybe around 300) and know Linnea's and now Emily's story, people that have CHOSEN to hear updates on Emily's condition, and ask them to write a paragraph or so on how our story with SMA has impacted their lives in a positive way. Then I'd compile them and send them to Dr's like this one in TN.... hmmm..... now THAT is an idea!! Why don't I don't I do that?! Why don't I make this my first book project and a function of Eminnea Inc. to raise money for our projects and spread awareness about SMA in a positive way? In fact, why stop there? The entire world could use volumes of incredible and positive stories from people on the periphery of disabled families that show how their existence in society is not only beneficial, but pivotal to the growth and maturation of humanity. Sure, we parents can talk all day about why our kids should live, and we will continue to do so - but, the testimony of the onlookers - the passers-by that want to stare, but aren't sure if they should - the public person who's heart hurts at the sight of an invalid and wishes they knew a little more - are the compelling stories. These are real feelings from real people that aren't captured in time - they are simply fleeting thoughts that, for the most part, are never resolved. I want to hear these stories - even if they have nothing to do with Linnea or Emily. I want others to hear these stories, so their lives can be changed.

So - if you have a moment, I'd encourage you to put some of your own thoughts into writing and send them my way. I will be compiling them for a future book on the benefits of disabilities in society. You can send them to: bookproject@eminnea.org

Peace <><
Nate

Saturday, July 28, 2007

Preparation

I know, I know - I haven't posted anything in a week. :( Well, we've been planning a big B'day party for Emily tomorrow, 7/29. Over 100 people are planning to attend, so we've been busy preparing all week... to say the least. How neat is that?!?! Over 100 people!!!

This is such a tremendous blessing to be able to host some of the hundreds of people that have kept Emily and our family in their thoughts and prayers. This will be a great day to honor Emily and her life.

Emily has been doing great. The van is a continued blessing as we needed to make two trips to the Docs office and it went so smoothly. We actually WANT to go places now and we even took her to a local lake front where they have live music last Sun. She LOVED it and we felt comfortable and prepared to make the trip. WOW - what a change from just a few weeks ago!!

Anyway, until my next post, we hope that all is well with you.

Peace <+><

Sunday, July 15, 2007

7/15: Emily is 2!!!

We knew she could make it,
but it had to prove true,
Emily has turned 2!!

What an exciting day it is! Our little Emily Hope has reached an incredible milestone for a child with Spinal Muscular Atrophy (SMA). It is estimated that 50% of kids affected by SMA will die before their second birthday. In a small way, Emily's life is showing the world, and the medical community, that SMA kids CAN have a good quality life. With good care, and properly educated doctors and parents, our kids CAN live to 2 years and beyond. With proper nutrition and respiratory care our kids CAN return home from Emergency Room visits happy and healthy. With proper care at home our kids CAN prolong the need for hospital stays, intubation, and ultimately, an untimely death... at least for a time. This care is rigorous and tiring, but their beautiful lives are worth the effort in our opinion.

Thank you all for the support you have given us - it enables us to continue giving Emily the level of care we are compelled to uphold. It has given her the opportunity to experience more of the lovely things - and it has given others the opportunity to experience a different, but rich side of life.

Thank you!

Peace <+>< Nate

Friday, July 13, 2007

It has arrived!! The van of course...

We now have a wheelchair accessible van!!! I never pictured myself in one of these - but now I don't have to. :) What a blessing. As soon as Emily is feeling better we are OFF on some new adventures... WITH HER. We are overwhelmed at the generosity that has made Emily's van a reality. We hope to visit as many of you as is possible in the very near future so you too an experience Emily in the flesh. :)

I'll get some new pics up when I get a chance.

Peace <+>< Nate

Tuesday, July 10, 2007

Coming at the end of the week :)

Well, Lord willing, this is the week that we will get our van. We are incredibly blessed to have raised $18,544.27 towards Emily's van and it is so exciting to think that we will be able to take Emily with us when we go out. It has been a very long time since we've been out as a family.

I just wanted to get that update out - I am actually very, very tired - so I am going to go and relax now. As always, thanks for reading and please keep Emily in your prayers. She has spiked some high fevers and her O2 saturation has been dropping quite a bit.

Peace <+><

Thursday, July 05, 2007

The Van

Okey-dokey. We've come a long way in a very short amount of time! With contributions outside of what can be seen on this site we have raised $18,450 towards Emily's van! We still owe on the mini-van, even though it is totaled, but will also count in the total loss money if worst comes to worst. That would bring the total to $25,150!! Thank you so much for your care and concern for Emily and our family. Most of you will no doubt be seeing MUCH more of her from now on. :)

The other news is that we've decided on a van!! We are purchasing a used wheelchair accessible Ford E-150 Conversion Van from Rollx Vans. I have been really impressed with this company b/c they have been MORE than helpful. Marty has spent hours on the phone with me talking through all our needs and helping me decide what will work. I am confident that this van will suite our needs well. We are not quite to the point where the total cost of the van and other modifications for Emily are covered, but we are well on our way and will not have to pay until next week. That is about as long as we can wait for a vehicle though. As always, we are well cared for and feel that moving forward with this purchase is the right thing to do. :) Thank you sooo much for your part in making this a reality for Emily!!!

Here are some pics of the van we are purchasing --



Peace <+>< Nate

Friday, June 29, 2007

The need to write right

Oh man - I am so hoping to express something that is really tugging on my heart right now. I won't claim to say this in the best way, but I want to let you in on what's been giving me a hard time the past couple of days.

So many people have been so generous to us for so many years. It as been overwhelmingly amazing to the point of exhaustion at times. We are just so amazed at how caring people can be. Thank you so much and thank the Lord for His goodness!!

As recipients of such beautiful selflessness we also struggle. It can be very hard at times to always be on the receiving end when we so much want to be on the giving end as well. Annabelle prayed before dinner one night, "And Dear Lord, please help us to find someone that we can help too." It is on our minds all of the time. We understand our predicament, however, and do what we can to show the love of Christ to others through our situation.

So, on to the thorn in my side, as they say -- Given the gaping hole of despair that we were no doubt tempted to fall into b/c of the series of events with the van, but also b/c our lifestyle is as such - when we receive donations or care in ways that dramatically shoot us forward in the process to recovery, it can really do a number on our emotions. This does not mean that all other amounts of care, be it financial - emotional - substantial - or the like, are any less meaningful and essential to us. We believe that no true value can be put on other's generosity to us, and I, by no means, intend to forget or leave out the fact that there have been more donors than the family I mentioned in my previous post. The nature of the amount was such that I was overwhelmed with calm as I realized what it meant for Emily. I hope I can portray this accurately. We appreciate EVERY ounce of generosity that we are blessed with from the email or phone call to see how we are doing to a sum of money to help with expenses. We cherish them all the same.

Peace <>< - Nate

Tuesday, June 26, 2007

Shaking in awe

Have you ever seen video of girls shaking and crying from hearing Pavarotti or the Beatles? On this years American Idol there was even footage of a teenage girl crying when one of the guys was singing... I never understood that really. I mean, I get music and I am in awe at the sound of good music, but c'mon - get a hold of yourself ladies!

Today we had our own Pavarotti moment - I was shaking in awe and wonderment, unable to contain myself. Today we received an unimaginable donation from an amazingly generous family, for Emily's Van Fund, totaling $15,000! Thank you so much. We are completely humbled, on our knees, under your care and generosity. Thank you.

Sunday, June 24, 2007

FSMA Conference '07



We had a GREAT time at our first FSMA conference. Hopefully the first of many. We finally got the chance to meet many of the families that we only know online through emailing and some phone calls - and we had the chance to sit through some very informative sessions on SMA care, research, home modifications, tax deductible expenses, family work shops, etc, etc. Emily got to ride a pony with the Make a Wish Foundation and we just had a blast. Having a blast with Emily comes with a price and we are very worn out now from the trip, but it was so worth every last ounce of tiredness that we might feel right now. :*) We drove through the night and I took some pics of the trip and conference - I hope you enjoy them. I was also battling a little critter on the drive that I wrote a little poem about. I hope you like it.

Listen here, my flighty foe,
Mr. Mosquito.
I'm tryin' to drive!

My kid's asleep,
My wife's close behind,
and the LAST thing I need is a welt to find.

My arms are a flailin,'
Reared back for a slap,
My stolen blood all over the map.

Get AWAY from me,
go fly down and die.
Go to the mosquito place high in the sky.

What'er you do,
Don't touch my sweet baby,
For that would... SMACK!!!!
AHA! Gotcha you little sucker!

Tuesday, June 19, 2007

VanNotes

  • Van: Totaled (value $6700)
  • Cause: car battery acid spilled on the floor
  • Rental: Insurance will pay for our rental 7 more business days
  • Plan: work on good, solid, one-page fund raising letter to provide to church, friends, and charity orgs (maybe with a video montage of Emily's life with a script read during the video). Dilemma: Wondering - should I get a loan for a medical van and pay it back with funds rasied, or wait for funds before I get a van (likely to be $30k - $40k). Wondering what sort of van to get w/out knowing how much $ we will have in the end - wondering, wondering, wondering -- should we just get the van, b/c we need it, and leave the rest up to God? Yes AND No, I guess. How the heck am I supposed to really feel like I am being responsible when all of this is happening?
Aside from that I am waiting for the title to be cleared up between states and must present the correct title before I will see penny of that insurance check. Relying on the DMV depresses me. :( Oh well - gotta take this one in stride.

That's our current situation - oh yeah, that and the fact that I ordered a special vest so we can secure Emily in the van while we drive to Chicago with AMPLE time to get it before tomorrow - they failed to let us know that the darned thing was on BACK ORDER!! C'mon people!! Throw me a freakin' bone here!! So we had to find another source for the vest in a time-zone back from ours (Ohio I guess) so they could call the UPS guy back to get it to us by tomorrow (Wed). Another day in the life of SMA.

I'll leave you with good news - we got our friends van wired with the fancy shmancy inverter and we are ready and set to go - that's cool! Also, I still believe the van thing did not happen out of God's scope for our lives and we will end up being just fine in the end - still stressful though. One day these things won't even bother me any more. "House burned down - no biggie - just a house" (that didn't actually happen) -- you know, something like that. One day...

Peace <><

Saturday, June 16, 2007

VanNews and our FUNd Raiser

Greetings folks - we had a great day today! Emily was in a great mood and didn't have too much trouble with her secretions and we spent the gorgeous day having a yard/bake sale!! On Wed Diana decided that she wanted to have a back sale so we can bring some $$ to Chicago and participate in a silent auction to benefit Families of SMA (FSMA.ORG). We raised nearly $300!! Oh yeah -- Ummm, I guess I didn't mention this..... Diana, myself, and Emily are driving up to Chicago this coming Wed to attend the annual FSMA conference. If you don't already know, FSMA is an international organization focused on raising funds for SMA research, family care, etc. They are the largest organization of it's kind, serving SMA families. :) Anyway, we are blessed and have received scholarships to pay for our trip, so our main concern can be managing Emily. We have never taken a road trip like this with Emily and our van (I'll get the van in a minute) will not work... soooo, some of our awesome friends are loaning us there comfy van to make the trip in. Again, we are blessed! Please pray for us on this trip - that Emily would travel well and that our time with other SMA families and hearing the latest news about research and care would be incredible. We are excited.

The other news has to do with our "little" battery acid spill in our minivan. I have used baking soda, battery acid neutralizer, oder neutrilizer, MORE baking soda, vinegar, water, etc, etc and I CANNOT get that smell out of the van. In fact, the acid continues to corrode the metal and has started eating away at one of the bolts that holds the seatbelt to the floor. I have ripped out carpet, etc, etc. It is awful. My eyes and nose sting every time I get near and/or in the van. So, after trying everything I could I called our insurance company to see if this could be covered. Installing new carpet alone will cost upwards of $900, not to mention cleaning and detailing. Anyway, they directed me to a local auto body shop and that is where the van is now. The guy came out to make an assessment and immediately saw that they were not going to be able to adequately clean this van. :( To make a longs story as short as possible - the van may be totaled. Our insurance company is supposed to send out an adjuster to figure it all out, but I really don't know how it could possibly be saved with the corrosion that is already going on. Amazing!
So, we've been praying that God would work with us on this van thing - it wasn't working for us and Emily and we needed to sell it or trade it in for a medical van... I have no idea how this is going to turn out.

Ok - this is the crazy thing. I've been trying to figure out a plan to get us in the van we need for Emily. This seems to be a theme now - when I start planning something, these sorts of things start to happen. HONESTLY! I was making plans for my career (very un-me like) and then we end up moving where we are RATHER than farther West where I wanted to be. I decided that I could make a career out of managing specialty retail stores, and then I get the job I have now. Why plan?! I am not completely asking that question as if I really don't think we should plan, but should we? We must b/c we are human, I'll give you that much, but the Bible says, "a man makes his plans in his heart, but the Lord directs his steps," or something like that. Maybe it's not so much what we plan, but WHY we plan. I haven't figured that out yet, but I have my suspicions. But we're constantly forced to fly by the seat of our pants (aka - trust in God - which we do), so why the heck do I invest any of my heart and soul into some crazy plan of mine?!

That being said, I am trusting that God's plan, as always, is perfect. We will take what we can from this van experience, even if it getting the darned thing back and having to try and sell it or something. As I said before - if I only focus on how to NOT have that van then I will completely miss out on what there is to learn by having it. :) I love that van... NOT! But I love having it if that is what God wants for us right now.

Peace <><

Thursday, June 07, 2007

Lessons Learned

I bought a 12v liquid acid deep cycle battery to use as backup power for Emily's equipment, but decided that I'd rather get a closed gel-cell, so I put it in the back of our van to return to the store. The next day Diana drove the van somewhere and the battery flipped over and leaked all over one side of the van floor - DISINTEGRATING the carpet on that side and anything else in it's path as it spread. That is why we need a CLOSED gel-cell battery! :( Anyway, we'll have to replace the carpet in the van now - AHHHHH, but at least no one was hurt. That's the story for this week.

Emily has been doing SOO well and I hope you all enjoyed the video of her drawing. It's like her mind has just opened up to learning and communicating and it has been a blast watching it unfold a bit. Anyway, here's a recent pic of Annabelle and Emily.


Peace <><

Saturday, June 02, 2007

This is AWESOME!~!



I was playing with Emily today and propped up this magnetic drawing pad - she was goin' to town! Once before I could get the camera out she stopped and looked at Cindy like, "did you see how cool that was?!! I'm drawing!!" I was so excited for her. Watch her prop her own arm up, though. She figured that out all on her own and uses it anytime she needs to reach something. :) Smarty-pants!

Peace <><

Email Subscription

Dear Readers,
I've added a cool new feature to the bLog... Subscribe to Emily's Updates by Email. :) You can now subscribe to our bLog so when we make updates, you will know. Thanks so much for your loyal visits for Emily's Updates. I hope you have enjoyed the reading. Please take a moment to browse down the page past the archives where you can enter your email address and subscribe.

Peace <><

Just had to post this picture!

Friday, May 25, 2007

FRI thoughts

Again it is Friday. However, this time we have what we need - but, yet again, if we were to choose to take Emily to the ER, it would be on an inconvenient, long weekend. Typical. I'm not being negative, it's just typically how this works out. It's never a Monday or even during the day at all. It's at night and right at the weekend so there are no Dr's that we want to see at the ER or on the floor.

On a lighter side - Emily is having a relatively good morning. She is obviously sick, but managed by her team of doctors and nurses (us) here at home, she is doing ok.

Some thoughts that will seem unrelated, but they are - bear with me (these thoughts are easier said than typed):

Diana and I were watching the Season Finale of LOST the other night. That show always makes me think. It seems, to me, the entire show is about what happens when people can only focus on trying to change their circumstances -- their "Island," if you will. At the end of the show, those that were focused most on getting rescued were suffering the most once it finally happened. They were so discontent with their circumstances on the island that they learned the least about how to be happy once they were off.

If I focus too much, out of discontent, on changing our circumstances, when they do change I will have learned the least from them. Most importantly I will not have learned how to be content with what God has given me.

Peace <><