Articles by Megan

It was brought to my attention (thanks mom) that there are two funeral homes in Spring Grove. We will be at Spring Grove Funeral and Chapel off of Wilmot Rd. Tues 4-8 and Weds. 10am service. Her obit. will be in the daily herald sun. & mon.

It is with a very heavy heart that we write that Danielle peacefully passed away this evening at 5:50pm. Tony and I were most fortunate to hold her in our arms as she took her most graceful, last breathe. She fought very hard to the bitter end……and she won. We won! She is no longer suffering or struggling. She is with God and many of her mld friends. For those who wish to know arrangements will be at Spring Grove Funeral home in Spring Grove, Il. Wake Tuesday 4-8 and funeral Wednesday 10am. In lieu of flowers we ask that you make donations to the MLD Foundation in memory of Princess Danielle. Address to come or see mldfoundation.org
Thank you all for your support during the most trying years of our lives. Danielle knew she was loved by each and every one of you. God Bless.

With love,
Megan and Tony

Hope this update finds everyone doing well. Wish I could say the same for Danielle. The past 3 days or so have been pretty rough for her. To this point she hasn’t stopped seizing for over 24 hours. This started 2 days ago when she had a couple of seizures in a few hours which is pretty abnormal for her. Usually she’d follow that up w/a high temp but that didn’t happen. Instead the seizures starting coming more frequently until they are where they are at now. She has slept maybe 4 hours in the past 24 because they keep waking her up. We’ve given her all the meds we have and nothing (again) is working. Hospice is coming out today to reaccess meds so hopefully they can give us something that will stop the pattern. I stopped counting seizures sometime yesterday at 22. The night nurse said she had a break between 2 and 5:30 but as of now she’s already had 4 since then. The weird thing is that the seizures aren’t getting worse in presentation or duration just a huge increase in frequency. The worst part is that she crys through them. That is so heartbreaking! It’s the only time I can hear her vocalize and it’s when she’s crying……just heartbreaking. I have an email out to try and get ahold of one of the docs out in Boston to see what his opinion might be. I stopped taking her to our local neuro because it was more of a battle than I was willing to put up with. I wasn’t getting much involved teamwork. So hopefully someone will come up w/and idea to bring her some relief. In the meantime I will just lay in bed with her to comfort her and pray that God can lighten the load a bit.

Thanks for checking in on my princess.

With love,
Megan

Brrrr………..it’s a bit cold and has been for some time. One could say we are living in the frozen tundra but we are nice and snuggy warm in the house. Glad the holidays are over. Life is back to “normal” or a distorted version of. Danielle has been pretty well. She’s been having funky temp issues again but that’s nothing different. It seems like once a week she’ll run a temp around 102.5 then it disappears into thin air but then will continue to run a low grade one. The nurse on staff today gave her a nice spa treatment included with the warm bubbles were a mani/pedi and a nice pretty scented lotion rub down. Her eyes were open for most the day today which is a bit unusual. I take her this coming Friday down to Children’s for her feeding tube to be changed out. This is one appointment I get so frustrated with. There is not another hospital closer to us that can do the placement yet it only takes 5 minutes at most. When she just had the g-tube I did the change outs at home myself. But since this is a g-j the IR dept needs to do it because they have the microscope to see that they are in the correct position of the intestine. A 2 hr drive down there for a 5 minute appointment with a 2 hr drive back. Oh well, there isn’t much I wouldn’t do to keep my princess comfortable. Besides maybe we can pop in on the one and only fabulous doc down there, we’ll see. Maybe we should stop in an see the cardiologist that predicted her death over Halloween. I’d really like to talk to him to get more understanding of some things. Chances are that won’t happen cause they are sooooooo busy even with appointments but it’d be nice. There is a new movie coming out, I’m sure most of you have seen the commercials for it. Extraordinary Measures. It’s about a father’s journey to finding a cure for Pompe disease which afflicts both his daughters. Pompe disease is a lysosomal storage disease just like MLD. I’m looking forward to seeing the movie although I KNOW I will be crying before the previews even start. LOL! Truthfully it reminds me of Dean and Teryn (founders of the MLD Foundation). What a story to be told. While Lorenzo’s Oil is a great movie and again very much like MLD, the times have changed so much and research has as well it’ll be interesting to see if this movie will reflect that. Not much else to report other than to ask for prayers for Emily. One of Danielle’s MLD sisters. She’s pretty sick and uncomfortable. Praying for comfort for her and her family.

Thanks for checkin’ in on Danielle

Love, Megan

Merry Christmas Everyone! As this year comes to a close all of us do a bit of reflecting and hoping for the new year to come. There are many things to be thankful for and to appreciate. Many things have happened this year that has brought about a certain kind of humbleness. A certain kind of love. And a realization that although life can be very short it is very beautiful. As Danielle continues to amaze her doctors, the shining light around her grows stronger and brighter. I spend most of my evenings in the rocking chair with her every night soaking up her beautiful face, the longest eyelashes I’ve ever seen and her soft, curly hair. Even though I sit with her every night it still seems like “not enough”. Not enough time, not enough love, not enough smiles. I couldn’t possibly get enough of her. I know on a higher relam that she understands ALL of this and even has the reason “WHY”. I can’t help but think she has had conversations w/the angels and fairies up above and hopefully she is looking forward to getting there. Looking forward to running free from MLD. That reality almost came about a few months ago and I am so blessed to have Danielle here to spend this Christmas with. This is one of those moments I wish I could bottle up in a glass jar and open it on a rainy day. I am sooooo thankful that the wonder med is still working like magic. No increase in dose or frequency. Such a simple med yet extremley complicated disease.
As Anthony is all excitied about waking up tomorrow morning and seeing the presents Santa left for him and Danielle, it’s equally exciting to see him BELIEVE. Believe in God and believe that life can be grand.
Thank you all for being there for us this year. Your kind words, thoughts, prayers and emails have meant so much. As we go into 2010 I hope for great health and prosperity for all of us and most importantly the wonderful comfort that Danielle has been receiving.

With love,
Megan

We had a pretty loooong week last week. First it started off w/Anthony coming down w/a cold on Monday morning. He woke up early w/rosey cheeks and a slight fever. He was home from school for two days. Wednesday during the day myself and the nurse orientated another new nurse to work some nights. The new nurse was then suppose to come back and work Wednesday night…that didn’t happen because of a scheduling conflict so I had to stay up Weds night and of coarse she had a terrible night. She could hardly take in any feeding. Was wretching w/all the meds and flushes. Somewhere around 3:30 I ended up in the rocking chair w/her and she was moaning. I took her vitals and she was ok, not the best but ok. My day nurse for Thursday took pitty on me and walked in the door at 4am that morning. When I came down 3 hours later Danielle was packed in ice packs and towels. The nurse said 106.1 temp at 6am. Ugh! Again! The rest of the day revolved around trying to make her comfortable and keeping that fever down. Thursday night the new night nurse came, told her of the latest fever so she knew what she was in for. By morning Danielle broke the fever and was back to her normal self. The new nurse said to me “you don’t just bounce back from a 106.1 temp”. I said “welcome to Danielle 101″. LOL! We had a good laugh which was nice. Haven’t had one of those in a long time. Friday morning came and now she has loose congestion that she can’t clear. She is sounding terrible. We try to suction her to stimulate a cough in luck of her either swallowing or coughing up the phelm. Sometimes it worked and sometimes it didn’t. Friday afternoon I start to feel as if I’m coming down w/something, by nighttime whatever it was had hit not to mention the shingles I had developed two weeks earlier. Saturday comes and she’s still sounding junky so the nurse decides to call the ped and get an order to treat for comfort so she’s now back on an antibiotic in hopes of warding off pneumonia. I forgot to mention too that while her fever was going haywire the pulse ox was too. Her heart rate was coming back at 250!!! After she took it manually a few times it started to come down into the upper 160′s but even she was shocked. We thought it was a malfunction w/the pulse ox. Ha!
Sunday was her birthday. Something we didn’t think we’d be celebrating 3 weeks ago. She’s now 7. We celebrated quitely. Just us at home. I gave her some frosting from a cupcake and well that didn’t go so well. But at least she got a taste of it. We have had great success so far w/the new wonder drug. If Dean and or Teryn are reading this I’d be interested in knowing what the medical advisory board thinks of klonopine. I’m sure Danielle isn’t the only child that suffers from posturing so it would be extremely beneficial for someone else to know. We have not had to increase the dose yet so I’m praying we keep getting this result. It’s so nice to see her so comfortable. It’s really enjoyable to sit and rock her w/o her body fighting me. Much calmer. I’m hoping for a peaceful week and time to catch up on some rest.

Thanks for checking in on Danielle!!

With love,
Megan

Just to let you know this might be an emotionally hard read. Just want to give you the heads up. Had a hospice visit today. They suggested starting Danielle on Klonopine to see if it would help ease the posturing. If we see no relief in 48 hrs we are to increase the dose, etc. until we reach the max then another decision needs to be made if we get to that point. Our whole goal all along has been symptom relief and pain control knowing full well things would eventually get worse. The hardest question for us to answer right now is when is it time to free our princess from this horriffic disease? I know there are no right or wrong answers which almost makes it worse. Just when we think she’s past the point of ever having a good day again she’ll have a great day, like today, and smile and be alert.
The option hospice gave us today was the possibility of stopping all her meds and puttin her into a medically induced coma where w/in a week or two she’ll slip away peacefully. Which is all we ever wanted anyway just didn’t want to be the one to make the decision. It’s hard enough getting use to the fact that we’ll be burying our child but to be the ones who actually end her life is something completely different. At times it seems like it would be much easier if she were to develop pneumonia or go into cardic arrest because we have a plan for that. This……I’m not sure anyone can plan for. Who are we to play God? Who are we to say when? In asking myself that question I realize this is about human compassion, dignity, and ending suffering. We have been given the oppurtunity to control how and when she passes. Not everyone does. I’m sure there are mothers and fathers out there that only wish their child didn’t die the way they did and would give anything to have the choice we do.
This will be a long and very well thought out process that we’ll go through. I do have the confindence that we’ll make the best one we can.
Thanks for thinking and checking in on Danielle today!!:)

With love,
Megan

Our princess is still hanging in there and doing very well considering she’s not suppose to be with us at all. We are not hearing an irregular heartbeat like we were told she should have. Aside from the occassional low o2 stat and high hr and of coarse the posturing/vomiting she’s doing surprisingly well. The ped called Friday when he got back in town because he hadn’t heard anything from us. He was very surprised that Danielle was doing as well as she is. When I say she’s doing well what I mean is that she’s mostly comfortable and sleeping. That now defines a good day. Needless to say Danielle continues to surprise and make a liar out of everyone. LOL!!!! We are taking the advice of her doctors very seriously but there is a part of me that is laughing at how baffled they are by her and how they don’t understand why she’s still living. Ah, the lesson keep coming!!!!!
She was running a real fever today (102 ax) but since we started her on scheduled tylenol a couple of days ago (for myalgia’s from the posturing) the fever has been masked.
We took advantage of the beautiful weekend we had and spent most of it outside. We brought Danielle outside w/us as we put up x-mas lights. I know a bit early…..but we don’t want to be out there when it’s 20 degrees.
So for now it’s one day at a time and being thankful for every minute I can hold her, kiss her and physically connect with her. We picked out her final resting place this evening, underneath a tree that will hopefully bloom beautifully in the spring.
Well, that’s all for now. Thanks for checking in on Danielle today.

With love,
Megan

This week has not ended like I anticapated it would. Danielle had an ok week. Nothing out of the ordinary except for some blood work that I asked to have. I have been having a feeling lately that something had changed. Wasn’t sure what but I felt the need to know. Hospice came out to do their weekly visit and I asked if they could do the draw. I was kinda thinking that her liver enzymes or kidney function was off more than we had known about in the past. It turns out that both of those are relatively ok considering, it’s her potassium and co2 again. Potassium is critically low 1.7 (normal 3.5-5) and her co2 is 48 (normal 20-32). She had this issue months back and at that time we decided to give apricot nectar a try. It did help to bring her potassium level up which was good. But since it has gone down again and much further than last time that prompted a lot of questions for me. Our ped (who is the best ped ever!!!!) couldn’t really answer any of my questions so he called down to Children’s and spoke w/a cardiologist who said he was very surprised that Danielle was still living. He never had a patient w/that low potassium that was still living. Most doctors don’t and will never see a patient w/that low of number because they treat it before it gets that low. In his mind, he doesn’t see Danielle living much beyond a week and I was told to begin getting things in order. In speaking with hospice it was agreed that in general they go off of how the patient is presenting not the numbers. That’s important to point out because we don’t know how long she has been sitting w/this low number. We haven’t done a blood draw in months so it’s likely that she’s been down at this number for some time which really negates the cardiologists opinion. Nonetheless, we all agree that no one can sustain life for very long at the numbers she got. She will probably start to exhibit an irregular heartbeat and go into cardic arrest. We do have a DNR in place and our nurses are aware of it.
Tony and I did take a trip to the funeral home today to start getting things in place. I realized how unprepared I was when we started talking about things. You’d think after 3 1/2 years of dealing w/this I’d know exactly what we wanted, etc. The one thing we knew for sure we wanted to do was to have her brain donated to science. All of the paperwork has been in for months now but we need to figure out the last minute details on that. From what I was told there aren’t many late infantile mld brains at the “brain bank”. I know that sounds funny but that’s the actual name. It’s important to us to do this in the slightest chance that her brain might be a “light bulb” moment for a researcher. We’d love to donate any other organs but they aren’t much good to anyone else.
In order to help keep her comfortable her meds were increased again this week. She sleeps all the time now. The only time she is awake is if she is in discomfort/posturing. She did take all of her feeding today though, so that was nice to see. Hopefully she’ll have a restful and good night. We are still moving ahead with plans that we have made for the weekend. Maybe she’ll open her eyes when she hears the chaos in the house tomorrow for Halloween. Lots of pics will be taken. That’s for sure.
Our little buddy Lucas is still in need of prayers. He’s doing slightly better but still has a long way to go.

Thanks for checking in on Danielle today. I’ll post more as needed.

With love,
Megan

What perfect timing my dryer has. Ha! It broke today while a pile of Danielle’s diapers lie there looking at me. At least I can still wash them. I suppose if Tony can’t fix it tonight I’ll be running to the laundrymat after dropping Anthony at school tomorrow.
The beautiful princess had a decent night last night, however not the best weekend. I”m hoping the upcoming weekend she has a better one. My in-laws and nephews are coming over since Halloween is on Saturday. We’re going to go Trick or Treating and let the kids have a ball. Danielle always seems to be so good when she sees/hears the laughter of good family and friends. I can see the twinkle in her eyes.
Off to the pharmacy then time to rock w/Danielle. Please say a couple of prayers for Danielle’s mld brother Lucas. He’s having an awlful time w/Graft versus Host. A complication from transplant. He’s in critical condition. John Evanosky as well. Sounds as if he’s doing pretty good so let’s pray he continues to do well. And of coarse all the other out there that we don’t know of.

Thanks for checking in on Danielle today!

Lots of Love,
Megan