Ok, I’m going to try and keep this short in the event this doesn’t post I don’t want to retype everything. LOL!
This past summer has been very enjoyable for us although Danielle has declined a bit. It was become increasingly difficult to keep her comfortable. Typical meds to help someone don’t seem to work the best for her. No one, and I mean no one, can tell us how to stop the abnormal posturing she exhibits daily. Repositioning her doesn’t always work, meds don’t always work nor does a combination of the two. Sometimes the posturing is so bad that if I were to lay her on the ground the only parts of her body that would be touching would be her shoulder blades and the backs of her ankles. She is not moaning or seem like she is in pain but it can’t possibly be comfortable. I’ve been told that this usually is one of the last things noted with this disease……she’s been doing this for 8 months now. So who knows how accurate that statement fits this disease. The retching/vomiting continue daily sometimes hourly. I have been able to bring some relief regarding this issue, however, it is a hard decision to make because it can be a contraversial one. I had to decrease her fluid intake in order to stop some of the retching. Her gut is shutting down and just doesn’t need what it once did. Little by little she will gradually get less as her body rejects it. We’ve had to increase some of her meds as she has become accustomed to the old doses. Almost daily she will have an episode of profuse sweating and have a high heart rate in the upper 160′s. No one really knows why this is happening…only speculation, again because MLD effects each person differently.
Even through all of that she will occassionally give a big smile. Make A Wish granted her/our wish and installed a hot tub for her. The first day we got in it was on my birthday and she gave me 5 huge smiles. It was wonderful. I wish I could have bottled those moments up in a jar to open on a rainy day/night. She doesn’t always get relief in there either but I do believe she enjoys the time spent in there.
DIAPERS! Ugh, let me tell you about diapers. So back in Feb she had a picc line placed and with that she gained 10lbs. Before that she fit fine in babies diapers. Well one of our nurses took pity on us as she was gaining the weight and brought us diapers bigger diapers that she was able to get a hold of. Before that supply ran out I decided it would be great if our insurance company would cover these. Figuring they cover everything else…why not?? Right. So after 4 months of going back and forth w/the insurance company, researching a supplier, contacting and setting everything up the insurance company says “sure”. Oh how life couldn’t be any better…..so I thought. Ya see if I had to pay for these special diapers out of pocket it would cost us $180.00 a month. I get the first shipment of diapers and everything is going great until the insurance company called and decided to change their minds. The diapers aren’t medically necessary. WTF!!!!!!!!!!!!! They cover nursing care and everything else she needs but they won’t cover diapers. So now I have a month supply of disposable diapers left with the only option of going to cloth diapers. One of my other nurses works on a case who actually uses cloth diapers and she says they work very well. So I go online and find these things only to realize that Danielle will need XXL. Who knows how long it will take to special order XXL. Well divine intervention happened once again and I was able to order these things and have them delievered in a week. $500.00 later. I only got 24 of them. They are really cute and colorful. But what a pain when she has a bm. Needless to say there is a lot of soaking and laundry being done. One night I was rocking with her and she urinated through the darn diaper and onto the bed pad I had underneath her (I’ve learned my lesson a long time ago w/that), I asked her why now all of a sudden since we got these new diapers does it seem like she’s urinating more. Is it because I now have a lot more work to do…..LOL! She smiled and gave me the loudest vocal sound I’ve heard in a long long time. My beautiful princess is still very much aware of what is going on. Hahaha
Anthony is doing well. He just loves school and is doing so well in it. His teachers are always complimenting me on how well he’s doing. Wish I could take the credit for that but it’s all him. He always has been a very quick learner. We’ve had a bit of a virus floating around here. Anthony has been home all week w/a slight fever and productive cough. Danielle has been battling a slight fever too but hard to tell if it’s mld related or virus. People keep asking me if we’re going to get any flu shots or swine flu shots. Nope. Just not for us. I hope I don’t regret that decision but at this time I feel no need for it.
The holidays are approaching fast. It’s always bitter sweet around here during that time. I know the other mld families that read this completely understand what I’m talking about. The thought never seems to get easier thinking about what to buy Danielle for her birthday or x-mas. I try to do the logic thing but that doesn’t work well on the emotional side. I’ve learned that it’s impossible to think logically and be emotional at the same time. Ugh!
Well it’s Danielle and med time. After that a cat nap. I have no nurse tonight. I’ve had a rather diappointing situation happen and they can’t find a replacement for this evening so who knows you just might get another update at 2am. LOL!
Thanks for checking in on Danielle!!
Lots of love,
Megan
Happy Easter!!! Hope everyone had a great day. We had a lovely day here. Anthony couldn’t sleep in because he was so excitied about the Easter Bunny coming. After eating about 2 lbs of candy for breakfast we skipped outside for a bit for an egg hunt. Then I made a traditional lunch and we laid around and watched the White Sox game. They won of coarse!!!!!! Danielle loved all the new hair gadgets and lip gloss the Easter bunnie(s) left for her plus all the cute clothes too!
Danielle has been having better days lately. We started w/a scope patch earlier in the week which we thought was the reason for her having a fantastic day on Wednesday but that theory was shot down on Thursday and Friday. Overall I do think she is more comfortable on this patch than any other med she has been on for vomiting/retching. As you’ll see below we’ve gotten more smiles and smurks out of her lately than in the past year combined. I’m hoping to hear back from the np at Children’s tomorrow about removing the PICC line. Can’t wait for that thing to be gone and to be done w/Children’s doctor’s.
Were getting closer to having Danielle’s wish from Make A Wish completed. We decided we are going to ask for a hottub for her. It’s about the only thing now that she can truly enjoy plus therapy water does wonders for everyone’s emotional status. She hasn’t been back to school on a regular basis for a while now so I’m hoping tomorrow is a start to a good week for her. Last week she decided it would be fun to once again trick everyone and spiked a 102.6 temp out of nowhere. So going w/the school policy that meant she had to be temp free for 24 hrs before she could go back. That ruled out school for the rest of the week.
Both Danielle and Anthony are in my brother’s wedding next month so I’m keeping my fingers crossed that she’ll have a fantastic day that day!!!!!
Thanks for checking in on Danielle today!
With love,
Megan
After reading my last post I realized that I have a lot of misspellings. Sorry about that everyone. I really do know how to spell. I was in a hurry because Danielle was in the middle of a very happy moment and I kept losing track of what I was trying to say. Anyway, we are on poop patrol at the moment. One of the GI docs wants to run a test on her stool so as soon as she decides to cleanse herself out I’ll be running it to the lab. I think I’ll keep the windows down in the car considering it’s at least a 45 minute ride. LOL! We may have to skip school tomorrow if she doesn’t go today before a reasonable time. This just seems to be one of those hilarious situations where there can be go real good outcome. When she goes……..she really goes. A month or so back one of our nurses went home w/my underwear and jeans on because Danielle just bombed everything while sitting on her. LOL! So we sit and wait!!!!!!
I can not believe that Danielle now weighs 45 lbs!!!!! It’s hard to believe that at one point during this disease was was 20 lbs lighter and surviving. Things have been really good the past 3 days. We have seen more smiles when we’ve seen in a year. Almost everytime I talk to her or Anthony talks to her she is smiling. It’s so great to see!! Just puts a permanent smile all over my face. Here is the best picture I have gotten in a long long time. She is still retching/vomiting though. That will never go away it seems. She was vocal for the first time in over a year though the other night. She was acknowledging Anthony jumping from couch to cough. Never really thought I”d encourage him to keep jumping but it kept her engaged. It was awesome!!!!!!
She is still struggling with the same ol’ issues but at least if she’s smiling or sleeping we know she’s comfortable for the moment.
Thanks for checking on Danielle today!
With love,
Megan
Happy again! 3/25/09
So I’ll try to pick up where I left off last. The pneumatosis is gone! The iv flagyl was stopped but then restarted again because the same ol’ ugly symptoms appeared again, vomiting/retching. This time it seems they’ve come back with avengance. This time the flagyl did nothing to help. So the nurses and I decided it was best to discontinue the antibiotic. The day we stopped it Danielle spiked a fever…..go figure. However there is an up side. It seems when she has a high fever froma virus or such the vomiting/retching subside. I think it’s because she is too lethargic to retch. A couple of nights before the fever she had not slept much. In fact one of our nurses said it was good that she (herself) has restless leg syndrome because that was the only way Danielle got any rest was for her to rock Danielle’s bed. Ha! So fast forward past the initial temp and the next morning she’s at 104.0 ax and needing O2 because her stats were in the low 80′s. Within seconds of oxygen being on she was back to normal stats. She stayed on O2 for most of the day although she didn’t really need it, she seemed more comfortable being on it. The next day we went back down to that dreaded place in the city to do a follow up w/the wonderful (NOT) GI team. The doctor we saw was one of the nicer ones however, I still have many doubts about her. Anyway, we are suppose to be weaning Danielle off the TPN and back onto a new formula. That should start tomorrow. She is back to running 101.0 temp with a rough cough with phlegm. The nurses consensus is that her lungs are clear but she sounds really bad at times. A blood culture was done on her yesterday because of the temp issue lately and I haven’t heard anything. Considering this is Children’s I will not be leaving it up to them to call me, I’ll be calling them.
As we speak the best doctors in the country and world are all together meeting in Germany discussing the latest developments in research and findings. Let me just share with you all how much they really care about our kids and adults affected w/MLD. One of Danielle’s doctors was in the airport waiting to board to flight to Germany and had an extra 10 minutes so she decided to give me a call just to see how Danielle was doing. With all the things on their minds and stuff that needs to get done for these types of conferences she took the time to make the call. Even though these docs can’t “cure” Danielle they do make every attempt to show compassion and make sure we have the things we need to make life a little more easier. Not to mention most of Danielle’s care is handeld over the phone or through email since they are out of state. That is a lot of time they are spending that they are got getting paid for, at least not from our insurance company. The the new President’s lifted ban on stem cell research I can only hope that in the next 4-5 years parents with an affected MLD child or adult will have many more options, if not a CURE!
Thanks for checking in on Danielle today!
With love,
Megan
It’s been a bit of a long two weeks. The good news is that the pneumatosis is gone!!!! YIPPIE! However, the vomiting/retching continue and seem to be getting worse. There are times when Danielle seems to be getting weak as far as the retching is concerned. I do believe she is starting to lose the ability to get everything up. This can lead or turn into pneumonia if she aspirates. She has been experiencing pain and discomfort that don’t seem to subside easily and in these instances morphine doesn’t always do the trick. The nights have been rough the nurses tell me. Tonight she is running a bit of a fever but it doesn’t seem as if it’ll get tooo high. Or at least I’m praying it doesn’t. We did catch a glimpse of a smile a week ago though. After she got done vomiting she looked up at Tony and smiled. I often wonder what goes through her mind. I often wonder how much more she can take. We did have a meeting with hospice the other day. Just trying to get a plan of care put into place. That was a hard meeting to get through but it needed to be done. A great big thanks to Diane for switching hours and staying longer that day….not to mention one of our other favorite nurses Ann for taking on 4 nights a week while working a full time day job as well. The dedication we’ve seen out of our nurses is so appreciated, I can’t even begin to explain what they all mean to us.
Next week we do the follow-up w/the TPN GI from Children’s. My expectations aren’t to high just because of the affiliation with Children’s. I”m assuming at that point we’ll determine whether to stop the TPN and go back to formula or what. The other good news is that since being on TPN Danielle has gained 3 lbs. I can’t believe that she’s actually over 40 lbs now. It wasn’t all that long ago that she was scary thin weighing 26 lbs shortly after dx. I believe this puts her back on the growth chart now.
We never took Make A Wish up on their offer so we are now asking for her wish to be granted. Hospice came up with a great idea to go to Key Lime Cove. Since she does really well in water we thought it’d be perfect. I don’t know that the water will be warm enough for her but I”m sure they have a whirlpool that would be. Plus it’d be great for Anthony too. It’s close enough to home that our nurses could still do their shifts there if they wanted to. So I can’t wait to hear back from them so we can plan a date.
Today was her first day back to school in a month. I think it was enjoyable for her to get out of the house plus it was nice for me to have a couple hours of quiet time with both kids at school. Got some cleaning done and had a nice visit from Lake County Animal Control. Apparently our dogs bark too much for someone’s liking. Oops!!!
Thanks for checking in on Danielle today!
With love,
Megan
It’s almost been a week since we’ve been home. Wow, that was a long week. To sum it all up. Children’s in Chicago has employed some of the worst doctors I have seen. Danielle started to get increasingly worse in the hospital. She started having more and more seizures, vomiting and total body spasms. The first night home was terrible. When she wasn’t having a full body spasm she was seizing and when she wasn’t doing either of those things she was throwing up. Thank God I had a RN (thanks ANN) that first night home because Danielle was so miserable. She didn’t sleep for at least 48 hours because of the pain and discomfort she was experiencing. The night I got home I emailed the doctor up in MN explaining what was happening. She called the next morning and immediately discontinued 2 of the 3 antibiotics that Children’s put her on. Apparently those two meds are known to excaberate the underlying neurological disease, which happened. She also increased her pain and seizure meds. The concept of the antibiotics is to help heal the pneumotosis while giving the gut time to rest. I think the docs went over kill on the antibiotics. The docs at Children’s even spoke w/MN and they were told that we weren’t all that concerned about Danielle’s health but more her comfort so not to expect to “cure her”. They didn’t take MN’s advice and as the result Danielle suffered needlessly for 4 days. The good news is that she rebounded very well once those 2 meds got out of her system. She is back to baseline. The nurse called the doctor that ordered all these antibiotics and told him what had taken place….his response “Oh”. LOL! The comedy of errors continues with Children’s. We are suppose to go back on the 25th for an x-ray to check her gut. Our nurse today called to make sure the appointment was scheduled and when she did so she found out that the doctor wrote the order for an x-ray of her pelvis and knee instead of her abdomin. LOL!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!! What in the world is going on there? Clearly he had the wrong patient in mind but if she, the nurse, didn’t check today it would have cost us hours in delay on the day of the x-ray because they would have needed to get ahold of the doctor to correct the script. We all know how that goes. I realize that doctors don’t know everything…however someone needs to tell them that because they almost killed Danielle. I feel one positive thing that can come of Danielle’s dx can be a learning experince for everyone involved. It would be nice if all doctors realize they are undereducated in this disease and just try to learn something from her rather than continue to think they know all. Geez!!!!
Yesterday was a blood draw day. It took the entire 8 hour shift to figure out where to take the blood, what color viles, get the correct adaptive syringes and drive it to the lab. Once I got there we had one wrong color vile so back home I went (another 45 minutes drive) to get 1 vile of blood and back to the lab. All of this by the way had to be done before late afternoon as the pharmacy that makes the TPN needed to have results for shipment by Weds.
The nights that I’ve had LPN’s I’ve been having to get up for 45 minutes to infuse the antibiotic (one left) then go back to sleep. Those nights feel as if I don’t really sleep. A flash back to a year ago. But my nurses have taken pity on me and one of them is willing to work a night shift for me then in the morning go to her regular job!!!!! (Thank you ANN!!!!) About 2 or 3 more nights of me getting up then the rest of the schedule for the antibiotic the RN’s will take care of.
This whole PICC line thing is pretty easy. It just seemed overwhelming the first couple of days but now things have calmed down. I have made new friends w/the supply company. Because I don’t know what in the world every little piece is called and my explaination on things is pretty vague at this point, they are all laughing at me. Nothing works the way it’s suppose to so I have to end up compensating and it’s all a comedy of errors.
Thanks for checking in on Danielle today!!!!
With love,
Megan
Still here……..but will be going home tomorrow. We would have come home today but I insisted on cycling the tpn for 12 hours instead of 18 (the usual). A bit of an altercation happened this morning between me and the gentleman doing his fellowship. He couldn’t give me a good reason why we had to do 18 hours as opposed to 12. Another doctor and I had discussed the night before doing a 12 hour cycle because that accomadates us better at home and the “fellow” was not only unaware of that discussion but disagreed with it and again couldn’t give me a good reason why. All he kept coming up w/was concern for her blood sugar which has never been a problem so the nurse decided they should check her sugar anyway and everything came back fine. So the extra day is so that they can cycle the 12 hours for me so when we go home we’ll be somewhat on track.
Once that got all squared away I took her for a walk around the hospital and outside. The access we had wasn’t the best, the courtyard was a bit cool as the sun wasn’t shining down in that spot at the time. It was still nice to take in fresh air though. We came back to the room and our roommate was leaving. She had bought a cute stuffed animal for Danielle the other day. Such a sweet girl. Her mom and I have spent hours talking. It was nice to have a considerate roommate. Then our favorite doctor here stopped in again to say Hi. We love Dr. Morrow. He tried to bring Danielle a Valentine but the place that had them had run out. It’s the thought that counts….especially because Danielle wasn’t even his patient this admission. We need to somehow get him into MLD research. I know all of our mld families would love to have him on their team.
Really looking forward to going home tomorrow. Although that’s when the real work begins. Timing is everything now. I think this whole “sterile procedure” thing is really over rated…….LOL!!!!! It can make you a nervous nelly if you let it. I”ll be stressed out for a few days but thank goodness for Diane. She pasted her IV test at Maxim and is ready to go. Between the two of us we can handle it…..LOL!!!!! Plus, the docs decided to add 3 different antibiotics last minute. One of them is given once a day but the other two are 4x a day….all through the PICC line so that’s where the timing of everything comes into play. This will be for 2 weeks then it’ll just be the tpn/lipids for the next 6 weeks.
Here’s hoping for an uneventful evening and home tomorrow afternoon!!!!!
Thanks for checking in on Danielle today!
With love,
Megan
We are still here. Looks like we’ll be going home tomorrow. This morning has been rough…..actually the last few nights have been rough sleeping. Monitors are constantly going off (Danielle’s that is), our poor roommate is in a lot of pain and not much is working for her. The child in the next room is having adjustment issues w/this ng tube. Crying and screaming alot. Can’t wait to get home. Danielle is back to vomiting/retching again. Even though there is nothing going into her gut, she still has issues w/bile. We continue to drain the g-port so that it’s not sitting in her stomach but it doesn’t seem to be helping much the past few days.
I’m suppose to be getting trained today on care of the PICC line and hopefully the case manager will get going on supplies getting sent to the house. New pump, tpn/lipids, extentions, bandages, etc……all that has to be home before we get there.
A BIG thanks to everyone that has come to visit Danielle over the weekend! Lots of smiles out of her. A special thanks to Angelo and Lindsay for rearranging their schedules and fitting in time for us and especially for bringing Danielle and I matching jammies. The nursing staff here loves the look. Even a few doctors have commented on it. And Grandma Diane, thank you so much for wanting to take Anthony for us but I know with Al coming home soon from the hospital it would be too much for you.
Hopefully the next update we’ll be home.
Thanks for checking in,
Megan
The PICC line is in and she did just fine. The NP forgot where I was going to be waiting and sent Danielle back up to her room while I was in the waiting room. I waited an additional 30 minutes before asking what was going on. They were so apologetic that they gave me a gift certificate to the gift shop!
I don’t know much more at this point. She’s resting just fine. The TPN will start tonight and I have high expectations to be out of here maybe on Sunday??????? I really don’t know about that but it’d be nice. \
More later,
Megan