Emily Evans Memorial Donation Drive

As it is

This new stage of our lives is difficult.  Trying to continue on with life, attempting to smile when all we want to do is cry.  Figuring out the answer when people ask "How are you" or the even more dreaded "How many children do you have?"  I've been asked that once so far, and my answer made the lady cry.  What a sucky thing to have to explain to someone.  What a sucky thing to have to live through.

We struggle on, daily, trying to continue with some semblance of normal.  Jacob is turning 12 in a week and a half.  He deserves parents who are able to continue living.  I am so thankful for Jacob, for all the normal reasons, and then even more so because he gives me a reason to have to keep going. 

Life is weird.  Life is hard.  Sometimes, life just plain sucks. 

There are good, positive things going on even through these times of crap.  Today was the last day of school.  Jacob, who will be 12 next week, is officially a 7th grader now.  Yay!  He is doing so well.  He continues to take tennis lessons, and will attend a tennis academy this summer.  He also continues to play alto saxophone, and will be taking private lessons from his school band director this summer.

We are trying to keep busy, and stay occupied.  It's not always easy.  Some days I want to do nothing more than go to the cemetery, lay on the grass at Emily's site, and sob the entire day away.  Other days I wake up, go to the gym for an hour or so, and feel better.

I just hope that at some point the good days will outweigh the bad days. 

Two months without our Princess ...

It feels like forever.  It feels like just yesterday.  It feels like a nightmare.  It feels like hell. 

We are having a HUGE garage sale tomorrow, to raise money for Emily's headstone.  We need to raise at least $1200.  We are ordering it on Sunday (worst Father's Day present ever).  We want it to arrive so that we can have it placed on her 9th birthday, which is on October 16th.  We will have a get-together to celebrate her birthday and remember our sweet girl.

We have been asked by many people who are not local how they can help.  I created a ChipIn account for anyone who would like to donate to our family.  The money will go directly towards the cost of Emily's headstone.  Thank you so much!

CLICK HERE FOR THE CHIP-IN ACCOUNT!

Emily, you are SO much and you are SO loved.  I hate this new reality.  I cannot believe the depth of this pain.  :(

How it happened

Many people have asked me about Emily's last week, days, hours, minutes ... and I haven't been able to tell almost anyone.  This weekend, a very close and dear friend of mine (Hi Carol!) asked me and I told her.  I went over the last week, the last day, the last hour, the last minutes.  And it didn't hurt as bad as I thought it would.  In fact, it feel good.  It felt good to talk about her last minutes and explain that they were peaceful.  That she was comfortable. 

(Insert some foul words and screaming here)

I just typed a HUGE entry.  I typed it all through tears and crying and then?  I hit some key and it disappeared.  I literally yelled "Oh no!!" and it's gone.  I will retype it soon, in word, and then cut and paste it here.  I can't believe it just deleted.  :(

Adjusting?

How are you supposed to get used to this new way of life?  Being Emily's mommy is what I am.  It's what I have been for the last 8.5 years.  Yes I am Alex's wife, and Jacob's mom.  But those things are easy and relaxing.  Being Emily's mommy meant being her nurse, caregiver, mom, tube-feeder, catheterizer, med-dispenser, suction-machiner, therapy-doer, chauffer.  So what do I do now?  I have no idea.  I get up, get Jacob off to school.  Come home and do laundry and dishes, and then what?  I watch a lot of bullshit TV that I never watched before and can't stand.  Tuesdays I go to Emily's site and get her solar lights and butterfly decoration, so that they don't get discarded Wednesday.  Wednesdays the cemetery removes all flowers and mows the lawns.  Thursday mornings I go back with new flowers and her butterfly. 

Other than that, no idea.  I don't know what to do.  I re-joined the gym, and started aqua aerobic classes again.  I am watching what I eat, and tracking my food on SparkPeople.com. 

I want to get a tattoo, of the Hello Kitty with angel wings that was on the back of Emily's funeral program. 

I have no motivation to do anything else.  I got all my scrapbooking stuff out and sorted, I need to organize it and put it away so that I can scrapbook.  But I am tired.  Just tired. 

How do you begin a new life?  I want my girl.  I am her mom.  I hate the idea of going three more weeks without her.  Three more months without her?  The next three years without her?  I don't want to meet any new people, ever ... people who will know me and never know Emily. 

I am so lucky to have Alex and Jacob.  Jacob is an amazing, tennis playing, saxaphone learning, 11 year old.  Without him, I would have no desire to keep on keeping on.  Thank heavens for Jacob.

I just want my girl back.  I never wanted this.  I never wanted to tell people that I am the mom to one on Earth and one in Heaven.  I wanted everyone to meet and know my Princess, and see her amazing smile.  I wanted to be her mom and caregiver and snuggle buddy forever.  This is not what I had planned.  :(

Three weeks ....

I can't believe it's been three weeks since our sweet princess passed away.  It feels like a million years since I last snuggled with her or touched her little face.  If this is how horrible three weeks is, I can't imagine the rest of my life.  :( 

I miss you so much, sweet girl.  I have never been anything more incredible than your Mommy. 

October 16, 2003 to April 16, 2012

This is the post I have never, ever wanted to write. 

Thank you all for loving my Peanut.

Waiting, cuddling, crying, loving

That is what we are doing right now.  Emily's fluids have been turned off and she is getting a lot of medications to keep her comfortable.  All we can do now is wait, cuddle, cry, and love.  And we are doing all those things. 

Please pray for her to be peaceful, comfortable, not afraid, and for her to know how much we truly, truly love her.  Our Princess. 

Weekend pictures

(Me and my girl)

We went to my Grandma's house yesterday.  We took lunch and an Easter basket, and had a good time.  Emily slept most of the time, like she does daily now, but she did wake up a bit to smile at my Grandma and Aunt, before going back to sleep.  She was very uncomfortable in the car on the way home though.  The drive is about 90 minutes each way.  Needless to say, we will not be taking any more trips for a while.  The pictures with my Emily and her great-grandma and aunt are priceless. 

Last night I went out to dinner with three special mama friends, whose beautiful kids are similar to Emily medically.  We had a good time, although the feeling of guilt was heavy on me.  I know going out and trying to relax a bit is good for me, but it is hard not to feel guilty about giving up any time at home with Emily.

(Jacob, wearing the Easter Bunny mask from our cake)

This morning, our house filled with love.  My parents, my in-laws, my sister and her daughter, my sister-in-law and her son, and then some good friends.  We had a ton of food, and laughs, and relaxing.  Emily managed to be awake about an hour, and gave some great smiles during that time. 

(My parents and kiddos)

She is clearly uncomfortable most of the time she is awake, and gets a lot of medications around the clock.  I don't know how much she weighs now, but she is getting more bloated by the day.  She has some other weird symptoms that I am sure are to be expected at this point. 

Tomorrow morning will be the hardest so far.  While Jacob goes out to breakfast with my in-laws, the hospice nurse is coming to do our official intake.  We will be making the DNR formal and inquiring about what medical things we can stop.  I have already stopped some drugs and daily procedures that are not necessary.  Things she hates the most, I am not going to do anymore.  Then the day will get harder, as we have a 3:00 appointment at the funeral home and cemetery.

Please pray for Emily's comfort, and also for strength for us.  This is incredibly exhausting and heart-breaking.  We are soaking in every minute of Emily as we can, and trying to remember to eat/shower/sleep in between. 

It's tough to type this update ....

Emily is home from the hospital, and we are here to stay.  She has appointments with three of her doctors on Wednesday, and I have a phone call in to her hospice team.  We are making the switch formally to hospice at this time and will be doing whatever is necessary to keep her comfortable at home now.  She is on a lot of medications and mostly sleeps all day.  She wants to be held, and doesn't want to be alone.  This is not a hardship as we want to do nothing more than hold her and cuddle with her and sit in the recliner all day with her on our laps.

Today we are meeting with a therapist to talk about how to tell Jacob this news.  He is a very smart kid, and I am sure he already knows she is dying.  The therapist is going to help Alex and I through our grief, and give us advice and suggestions on how to talk to Jacob about it.  We will be bringing Jacob to her as well soon, as he already knows her and feels comfortable with her. 

We will be talking with the doctors on Wednesday about putting a DNR in place.  We are going to make arrangements with the funeral home and cemetery soon so that everything is in place when we need it.

If you are a local friend, please let me know if you would like to come over.  I would like everyone who wants a chance to see Emily to have time to do so.  Also know that just because we switch to hospice and make arrangements, does not mean we have any idea how long we have.  She could have weeks or more left, but we need to be ready now.  As we raise medication doses and frequencies to keep up with her discomfort, we get closer to the point where her body gives in.  We have no time frame and no expectations.  All we want to do at this point is love on our girl and let everyone else who loves her have the same opportunity.

Day by day (and stupid blog problems)

Things here change daily, and even sometimes ultiple times a day. Keeping Emily calm and comfortable is proving hard to do. She ended up having a reaction to morphine, which we thought might happen. We have added meds, changed meds, increased meds. She is happy at the moment, which is awesome. My blog is all messed up. It has something to do with the person who I paid to create the design and layout. Being in the hospital, I can't really work on it much so it will have a very basic design for now. Thanks for checking in on us!

Rough days

Emily has had two very rough days in a row. Emily has had a ton of coughing/gagging episodes that go on and on for hours. We have tried all her PRN meds and nothing helps. The only thing that helps a little is giving her some heavy narcotics to sort of knock her out. Mthere was a big team meeting last night about Emily. GI tells me the episodes are pulmonology, pulmonology tells me they are GI. I don't care what they are, or whose field they are, but she is miserable. It's not okay. Thankfully the Comfort Care Palliative Team here is amazing and standing p for Emily's comfort. Me are starting her on morphine for these episodes today. She has had an adverse reaction to morphine before, but it was 6 years ago. We are going to try a tiny dose first and see how she does. They are asking us some heavy questions. How far do we want them to go with her. What do we want if she codes. If she stops breathing. If her heart stops. If she needs a ventilator. It is surreal. Parents shouldn't have to answer questions like this about their 8 year olds. :(. Please keep Emily, and our family, in your thoughts.

Waiting for more surgery

Emily is inpatient again. She had the UTI last week, and the antibiotic they had called in was not the right one. By the time the sensitivities came back, she had landed herself in the ER again. She had a fever of 101.2 and was trembling and lethargic. Her urine is still positive, so the antibiotic was changed. This time her blood cultures were positive as well.

Here's the thing. We go to "First Class Big Huge" University Medical Center. Last admit, they transferred Emily to the sister hospital of this one, in the next city over. Same University affiliation but NOT the same level of care. It was awful. Her central line had been placed on Feb. 28th, and by March 3rd it was out of place. I had been telling them that the line was sliding in and out of her skin again, this is what caused the line to come out on Feb. 27th. So I wanted a different type of line, or a different type of securement. This is the fourth time a Broviac has slipped out of her chest without being pulled. Obviously, her body doesn't like this type of line and the internal cuff does not develop scar tissue to hold it in place. The surgeon at the sister hospital said he would be placing a Hickman when he took her to the OR. He specifically reminded me that her current (4 or 5 day old!) line couldn't be fixed because it had slid out of the skin some. They never, ever, ever re-instert a central line that has come out of the body. It is almost guaranteed to become infected. Imagine my surprise when she came out of surgery with not only NOT a Hickman, but the surgeon told me that he had re-inserted the line she had in. My jaw dropped.

Prior to her going to the OR, I had asked the surgeon about a port in place of a broviac. I was told no. He said that "First Class Big Huge" University Medical Center does not place ports on kids. Never. Not ever. I said I didn't believe this was true, that I knew kids at our regular hospital (not this little sister hospital) that had ports. He said they must have been placed elsewhere. Oh. *shaking my head in disbelief*

This leaves us where we are now. Inpatient at our regular hospital, because I refused transfer back to the sister hospital. We will never go there again. Her central line is infected, and has to be pulled. They are now saying "It may have been caused by the surgeon's actions, but we have no way of knowing that". Keep in mind that even after the surgeon re-inserted her line, it still continued to slip in and out with each dressing change.

I am so irritated. Had the surgeon done the right thing by either putting a new Broviac/Hickman in, or placing a port, we would probably not be inpatient right now. Yes, she has a UTI, but we could have continued to treat that at home. The bug growing in her blood is not the same as the bug growing in her urine, so it did not translocate internally. This is a different infection. So frustrating!

In better news, she is not too miserable. She is sleeping a lot, almost all day. She is not running a fever anymore, and the trembling has stopped. She is getting two different IV antibiotics for the next 14 days, so we are inpatient for a while. After that time, we will most likely be placing a port. Please continue to keep Emily in your thoughts and prayers. No surgery is easy on her, and her other issues continue to get worse. Her sleep study showed a lot of central apnea. Her neurological status is worsening. Her general instability is huge. This (undiagnosed specifically) mitochondrial disorder is kicking her butt. She still continues to smile at us, through it all!

Bad blogger .... bad blogger!

I just typed an entire long update and then my computer ate it. :( The short version is that Emily is home right now on IV antibiotics for a urinary infection. We should know later today whether we can continue to keep her home and treat the infection or if we will end up inpatient. It depends on some final labwork. Emily had a sleep study last week, to figure out the cause of her desaturating during sleep. I know she did it during the study because they had to keep calling the pulmonologist to ask what to do. We started out on room air, and then had to keep going up and up on the oxygen until she hit 2.5 liters. So hopefully the results will be in soon and we will know what to do next with our girl during sleep. Emily had a (second) muscle biopsy done a few months back, looking for a mitochondrial diagnosis. Unfortunately there was a lab error and the sample never got to Baylor University. We have come preliminary results which are pointing towards a COX10 Deficiency. This diagnosis fits Emily's symptoms, unfortunately there is no treatment or cure. Because the sample cannot be formally tested any further, we are drawing labs on Thursday for a whole exome study. They will draw blood from Emily as well as her Dad and I. Hopefully this will (FINALLY!) lead to a diagnosis for Emily. That's about all that's going on right now! We take this all day by day, and today's goal is to get good news from the lab and be able to stay home with our girl. We don't want another admit, again! We just had a week long admit for a central line replacement, because her line came out. We would love a long, long stay at home now. :)

Another week, another ER trip

Emily earned herself a trip to the ER yesterday, after a long 4 days of trying to avoid it. On Friday, she had a bronchospasm that lasted three hours. It was horrible, but she was managing to maintain her oxygen saturations, so I waited it out. It did worry me and I packed a bag for the hospital, but then of course the spasms stopped. It was definitely the scariest breathing episode she's ever had. The GOOD thing is that it proved that the trach was the best thing we did for her. She was able to breath and get nebulizer treatments and suctioning without a fight. What a relief the trach is! Saturday she had another bronchospasm that lasted an hour. During the spasm I gave her a dose of Ativan, thinking it might calm her down. Thirty minutes later she was still spasming so we gave her breathing treatments even though they weren't due for an hour. Then she got some other medications that she was due for, and finally it calmed down. This time when she was spasming, she was not maintaining her saturations. Again, thank heavens for the trach! I was able to hook her up to oxygen and manage her at home. I did page the pulmonologist when this episode finally ended though, because I needed help with managing these. She called in a prescription for Atrovent nebulizer treatments, and told me to try that along with the Ativan in the j-tube next time it happened. So of course, Sunday, it didn't happen at all. We thought that whatever it was, was over. Until Monday morning, when she woke up doing it at 8 AM. Her morning nurse banged on my door to wake me up (Don't judge! LOL Jacob had the day off from school so we were sleeping in). She said "She's not breathing good! Come see please". Ugh, nothing like that to get a Mama out of bed quickly. I went in, and she was spasming again. We gave Atrovent via the nebulizer, Ativan via the j-tube, and waited a little. It stopped after about 20 minutes. We thought we were good, and then it started again. It went on, off, on, off. I paged the pulmonologist again and said we needed to be seen. She told us to come to the ER. We went in and did a chest xray, trach sputum culture, and some swabs. Her xray was normal and her swabs for RSV and Influenza A & B were negative. They tested her CO2 end tidal volume, and it was good. The decision was that we could go home, and raise her oxygen a bit while we wait for a sleep study. We know she has sleep apnea, and it's most likely central apnea. The pulmonologist feels that she will end up on a vent when she's sleeping, and wants to do part of the sleep study with her awake. Seems like an oxymoron to me. ;) She wants to get a better idea of what the spasms are. So, here's to another week of another journey. Hopefully it was the last issue we will have for a while! We have been home from the hospital for 3.5 weeks, which is a pretty good run for us lately!

Doing well at home!

Emily had trach surgery on January 19th. She was discharged directly from the PICU on January 27th, the day before my birthday! What a great birthday present. She did great during the trach surgery, and was smiling within 6 hours of surgery. She is amazing! She came off the vent in recovery and was off oxygen and on room air within 24 hours. :) She's a rock star!

Being home was a bit rocky at first, but we are settled in now. There is quite a bit more care involved now, and leaving home is quite a feat. The number of pumps/machines/bags that must go with us is a bit nuts. In the end though, she's doing fantastic so no complaints.

Tomorrow is a big day for us. We are meeting with the most involved in Emily's team of doctors - GI, TPN nurse, Neurologist, Palliative/Pain Dr, and social worker. We have been asked to sit down and detail what we want in the future for Emily. What our choices are about her medical care. For example - if she was to become septic and need to be put on a ventilator, I say do it. Give her a chance to fight the infection and recover. But if she is no longer alert and aware and isn't going to recover and come off the vent, I don't want her on it. I am not willing to keep her alive in a shell of herself. Her personality, her facial features, her love - those are Emily. If we lose those, we've lost her. This meeting is not going to be fun. I am happy that my husband has taken tomorrow off of work and will be going with us. Emily has some new nurses finally, and a nurse will be with us as well to keep Emily occupied and entertained. If you have a moment, please pray that we get through this meeting and can come to some decisions. Look at this girl - this is what we fight for!

We made the decision ...

Emily was discharged from the hospital last Thursday late at night, after a 16 day stay and antibiotic treatment for a line infection. She gets line infections because her motility is horrible, and causes a leaky gut. So we went home Thursday night, and I got her TPN prepared. When I lifted her shirt to connect the TPN to her Broviac, I realized that the nurse who discharged us must have pulled the line somehow. It was coming out of her skin a bit and had been bleeding. I took pictures, redressed it, and sent the pictures to the GI and TPN nurse. They called the next day, Friday, and said to bring her in for xrays. We had to be sure that her Broviac line was still in far enough to use for TPN. As I prepped us for a quick ER trip for an xray, Emily spiked a fever. Of course. So we headed to the ER, only 14 hours after being discharged. They did labs and blood cultures, but her fever had dropped and she was acting fine. They sent us home. Saturday night they called to say her cultures were positive and we had to come back inpatient. :( We went in late Saturday night, and got a room of the pediatric floor early Sunday morning. This coming Monday, Emily will go to the OR to have her Broviac pulled. The will place an IV so that she can continue some nutrition, although it won't be her regular TPN. She is scheduled for surgery Wednesday, to place a new Broviac. She is also going to be getting a tracheostomy. This was a hard decision. We have gone back and forth over whether she should have a trach or not. Her breathing is great, her lungs are clear. But her vocal chord spasms are out of control and there is nothing else we can do about them. When she has these spasms, she has horrible coughing episodes and gagging/choking spells. She desats, turns blue, but worst of all she really PANICS. It scares the crud out of her. We can't easily suction her, because of her oral aversions. I manage to suction her orally but it's not easy. Trying to deep suction her, through her nose, takes four adults to hold her down. It's not feasible. So in order to prevent aspiration and allow her to breathe without coughing and panicking, we are going ahead with the trach. It is an impossibly hard decision to make. I am trying to remain optimistic. I am hoping that she will be able to make noises and laugh audibly, even with the trach. This is big surgery she is having. She will be taken to the PICU after surgery. I will try and update here as soon as I can!