Day +202 Our Agenda

I hope everyone enjoyed their Memorial Day weekend (here in the US :) ). We did for the most part. Dani and I laid low and spent the weekend at home. She finally cut two molars and is working on a third. She had a fever overnight Sunday night into Monday. I was hoping it was just the teething and I think it was. You could tell she did not feel good though. Poor baby. She has created some new words, but we are not sure what they mean. But at least she is trying. I made her watch racing all day Sunday (instead of dancing to music). I am hoping to convert her early. hehehe

Jason and the boys went camping overnight Sunday into Monday. They fished and caught tabpoles and such. They love going and sitting around the campfire. Ethan was attacked by every bug known to man so he enjoyed himself immensely. We BBQed on Monday and just took it easy. It was fun. Jason and I have really been working on the house inside and out. I will post pictures of all the flowers we have planted and the bar downstairs that he has remodeled soon.

I did not mention it before but we went to "The Angelman Syndrome" walk-a-thon a couple of weeks ago. Website http://www.angelman.org/angel/ . Our really good friends Rick and Suzanne's oldest baby boy has this. It was an awesome event. It was held at Jefferson Barracks State Park and we all enjoyed ourselves. Us five went and then a friend from work, Jenny and her son Jack met us there. The boys and Jack had a blast following the trial and enjoying each other. We met up with a few friends we had not seen in awhile (Trevor and his new wife Kim) and then Ricky's parents. We will definitely be going from now on.


Well today we were going to go to the Zoo but it was rained out. Tomorrow Dani is scheduled for an MRI (under anesthesia) and then Friday we have the IVIG (Immune Boost) infusions and the eye doctor appointment. I will let you know how it goes.


Vibeke is getting ready to start infusions and it is very exciting that she finally got approval. Thanks for all the love and thoughts.


Erin ..... I will try the teddy grahams next but I am really working on veggies right now. I did buy baby goldfish but have not opened them yet. She is still more picky then I am used to. The boys use to just shovel everything in. She is more stubborn than Gabe which amazes me.

She is definitely a smart one and knows what we are talking about and the schedule for the day. She tells me what is next in the morning when getting ready. She will tell me when it is time to brush her teeth and when it is time to clean her ears. She now will not keep socks on her feet.

Please do not forget the Golf Tournament on Saturday June 23rd. It should be a lot of fun and there is always prizes to win.

Day +197

WBC 5.1
Hemoglobins 14
Platelets 382
ANC 3315
Retic 5.3

Well this morning Dani decided to worry us because she had a rash under her chin. This afternoon it is better so we will keep an eye on it. Her numbers are good. We are waiting for her Retic number to see about weaning her steriods so more.

She is now on a sippy cup and is tap dancing. She loves her dancing. I will try to take a video of it. She is still not the happiest about table food. She is starting to eat Honey Nut cheerios.

The boys are out of school and are very happy about it. Grandma Dunn is getting her other hand done on the 6th of June so more happy thoughts her way. PJ (my nephew that was in the accident at the end of last year) is back on a Naval Carrier and outside of Iran. Send positive thoughts his way also to come home safely. He is on the USS Nimitz (sp?).

I hope everyone enjoys their long holiday weekend (US bound anyway) and all have a nice weekend!!!!! Send a special prayer that Vibeke in Norway will be started on Enzyme Replacement Therapy soon. She has been fighting so hard to get it and she is getting some positive comments back now.

http://www.caringbridge.org/europe/vibeke/

Updating:

Yvonne, Dani's nurse practioner, called back and she is going to get IVIG (Immune System Booster) next Friday. It is a four hour infusion so I hope we make our 1:00 appointment with the eye doctor to talk about the lens implant. They lowered her steriods from 3.0 daily to 2.8 ml daily. The rash we will keep an eye on and see if it gets any worse.

Day +190

WBC 6.8
Hemoglobins 12.8
Platelets 289
ANC 5644
Weight 28 lbs 14 ozs
Retic - 5.4

It was a good visit. All the numbers are going in the right direction. We are back to going to clinc every two weeks ... very exciting!!! They lowered her steriod dose. We will go to a local Quest Lab next week just to get numbers. They were talking earlier about starting IVIG (which boost the immune system) but that is not definite and I think the numbers might be good enough this week not to have too.

I hope everyone had a wonderful mother's day. We laid low and relaxed the entire day. We are getting a lot done around the house which is nice and so ready for the boys to be out of school. The next clinic visit is the same day as the prep session for Dani's eye surgery. It will be an interesting day.

Dani was tap dancing in clinic today and it was too cute. She is figuring out a lot around the house. Which means we have stepped up on child safety gadgets. She wants to go outside all the time now and we are still scared. But she has been out and not sure about her bare feet in the grass. Jason bought her a blow up pool so I will take pictures when she (and her brothers) get in.

Mom did well with her surgery and her next one is scheduled for June 6th. Keep the good thoughts rolling!!!

Do not forget about the golf tournament June 23rd. Jason is playing and I will be a beer girl. It should be a lot of fun. Shoot me an email for details rdhdtrue@yahoo.com

Day +183 Last Infusion ... We hope

WBC 13.8
Hemoglobins 11.5
Platelets 352
ANC 12,834
Weight 28 lbs 4 ozs
Retic 9.32

Dani started the day of in a mood. She was not happy with anything on the drive into the hospital. I warned them when we got there and they pushed things along. It could of be worst or better but we were very happy it is hopefully the last infusion forever. Her counts are pretty good. Her ANC is high because her immune systems has been suppressed from these infusions and the calculation they use to get this number is errored (sp?).

She has a clear runny nose that we are hoping does not get any worse. She is kind of back to ground zero with her immune system so we have to continue to be careful with cleanliness and such. We have not really relaxed in that department so no big deal.

She loves her music and her gerber graduate crackers. I have a video I will download her in a bit of her dancing. She will grab the remote for the TV or just stand in front of any TV and start bobbing up and down to dance. She loves watching videos.

http://s5.photobucket.com/albums/y199/rdhdtrue/?action=view&current=DaniDancing.flv

May 15th

This is MPS Awareness Day around the world. NASDAQ will ring the opening bell in acknowledgment of it. I have been trying to get work to do a casual day for it but I think they are tired of us. Here is an independpent movie about MPS II.

http://www.jonathanformica.com/portfolio/courage.php

This is an article on a wonderful group of girls and their Mom and Dad's daily struggle.

http://www.mycommunity.com/town/Teaneck/Details_story/Three_beautiful_girls%2C_one_deadly_disease/view.php?action=Detail&type=story&sub_id=18705&area=&category=1

Please consider being a bone marrow donor. It is free to apply online until May 21st. Go to http://www.marrow.org to find out more information.

Day +180 - 6 Months Post Transplant

WBC 8.6
Hgb 10.7
Platelets 403
ANC 7396
Retic 14.44 (Normal .50 - 1.50)
Weight 28lbs 14 ozs (But it was a new nurse and she let her keep her clothes and diaper on)


Sunday was the big day!!!! Dani is 6 months post transplant. We did go to clinic yesterday (Monday) to make up for missing Friday. As you can see the numbers slipped a little but are still ok. The Retic is showing, because it is high, that she is still chewing up Red Blood Cells. So we want to see that come down. She will get her last infusion this Friday and hopefully her body will balance out. She is now being weaned off of the steriods. She went from 7ml to 4ml in the last week. Hopefully she will lose some weight so she can wear some of her new outfits she just got. She is a buddha baby right now.

I received an email from another Mom of a Hurler's patient (which I need to call ;) ) and her daughter is 10 years post transplant. She has Cardiomyothopy and had this particular Anemia ... amazing how similar they are. She said that she is free of the Anemia now but it took years to wean her off meds. So that kind of puts this in perspective. She also said she is a normal and social 10 year old with no orthopedic issues. I can not wait to talk to her more.

Last but not least to mention is the "Thanks Mom" Marrow Donor Drive through the National Marrow Donor Program is going on now till May 21st. That means that it is free to become a donor right now (even online!!!! ). If you have not done this yet please consider it and I will post the link below. Save a life!!!!

http://www.marrow.org/NEWS/Events/Thanks_Mom/index.html

Day +176

WBC 9.6

Platelets 509

Hemoglobins 12.2

ANC 6144

We did not go to clinic today. I have been sick all week and did not want to spread my germs nor sit with Dani in a small room to chance getting her sick. Dr. Hayashi is not there this week so they talked to Dr. Shenoy and she said we could wait till next Friday for her next infusion. I asked Dani's nurse Carrie to follow up with Dr. Hayashi on Monday to make sure that was ok. We went to Quest this morning for a blood draw to make sure her hemoglobins were ok and as you can tell all her numbers are really good!!!!

She has been doing well. The pictures above were taken by Grandma Dunn when she was watching Dani on Thursday. It was labeled Dani's new trick. This was the first time she climbed up. Her hips do not seem to be holding her back at all. Grandma Dunn gets her surgery next Wednesday so everyone keep her in your thoughts that all goes well!!!

Day +173 - 2nd Opinion

2nd Opinion on her vision

I LOVED this doctor. He knew we were coming for a second opinion. He took a lot of time explaining things and gave us as much information as he had.

To catch everyone up. Dani is VERY near-sighted. She also hates her glasses and patching. I mean she will keep her glasses on for an hour or so because she knows she will be in trouble if she does not but she still hates them. We thought that if the glasses where really helping her she would get use to them. That has not been the issue. She was diagnosised in July of last year -14 in one eye and -17 in the other. That doctor (Dr. Tychsen) was very abrupt and did not seem like a people person (I know when you get to a specialist that might be the case). He wants to do a lens implant at the same time as the muscle correction for her crossed eye. When I googled that or talked to our insurance I could not find out much information. So I wanted a second opinion to make sure the direction he wanted to go was the right one.

Dr. Goodrich actually said he has referred some patients to Dr. Tychsen. Dr. Tychsen and another doctor (hospital) in Texas are the only ones doing lens implants. So he did not have a lot of information on the lens implant on children himself. He did try to figure out her refraction while we were in the clinic and he came close enough to Dr. Tychsen's that he had no issues with it. He said a few items he would not be able to determine unless he put Dani under like Dr. Tychsen did.

He also told us that this is a step above an experimental procedure. He said maybe 10-100 children nationwide have had this done. He said if we asked the clinic we can get the info of success rate and complications. He was not sure insurance would cover it. But he said it was appropriate for Dr. Tychsen to want to do this.

He mentioned another procedure which would be put a corrective lens on top of her pupil and get her to an almost zero for now. The downfall on that is cataracts getting worse (she already has corneal clouding) and glaucoma. But that would be later on down the line. Also her vision could continue to get worse so she would still need glasses later on down the road.

It just seems like Jason and I have to keep making these HUGE decisions and it will impact her life to a huge degree. I am selfish enough to want things to get easier at some point. It just does not seem to be in the cards right now. We will talk to the main nurse the first friday in June and make a decision then.

Day +172

Hi All,

I had a few people have been questioning the patch on Dani's eye. Sorry I should of commented on it when I put them up. Dani has Strabismus (one eye crossed) and they have been wanting me to patch her good eye to see if the muscles would strengthen on their own. I did not do it too much during transplant because she was going through enough and she hated it being on. We started again the last couple of weeks and she seems to tolerate it better.

She goes to the eye doctor for her second opinion tomorrow on the lens implant surgery. I am hoping she will cooperate with the exam so they can get an accurate reading. Wish us luck!!!

Day +169 Sixth time is the charm

WBC 16.3

Hgb 10.6

Platelets 306

ANC 15,332

Weight and Height were not taken this time. Ummmm we were there at 8:45 and did not get started with the infusion until 1:30. The nurses in the clinic tried both arms and blew them. Then an hour or so later a outpatient lab tech came in and tried her feet (never again). She screamed blood murder and the lab tech stuck her three times on her feet. She was sooo upset she had the blown veins swollen and they looked scary. So then 2 hours later the paramedic crew came in and choose her hand to try. They took a few moving the needle around and Dani did not like that but got a return so we where all relieved.

They started the hydrocortison and then the flush. When they slow the flush it started beeping. We looked at her hand and it was soaked with fluid (blood and clear). So they opened up the bandages and looked. They flushed it and all and it seemed to be fine. They then regave her the hydrocortisone and it seem to work fine. So we completed the infusion and it was 3:15 when we got out of there. That is amazing because this is a two hour infusion.
Anyway we are a little concerned over her high WBC and hope that settles again. Overall Dani was in pretty good spirits and doing fine right now.

Day +167

WBC 7.8
Hemoglobins 10.9
Platlets 286
ANC 5694

The numbers are good!!!! Nothing really more to report. We will be going to clinic on Friday for her 2nd infusion.

Day +166 Rituximab Infusion

WBC 7.4
Hemoglobins 11
Weight 26 lbs 6 ozs
Height 32 3/4 inches

I forgot her paperwork at home so I do not have all her numbers. But they all looked good. We started Rituximab yesterday. We got there at 8:30 and the infusion did not start until 11:00. She had tylenol, benadryl (both oral), and then hydracortison through her IV prior to the infusion. They hooked her up to a heart monitor (3 leads), a post ox on her toe, and then her IV in her arm. So there was no putting her down to walk. They also did not have a room so we where back in the infusion center. By 11:00 Dani is very done with the whole situation especially after she realizes she can not get done and be free. So she screamed for a half hour or so until she passed out from exhaustion. Not a fun moment in time. She slept for an hour and a half which was almost the whole infusion time which was nice. I laid on a recliner and she laid on top of me.

They originally said it would take 5 hours but only took half that amount which was nice. Next time it actually should be quicker than that. She had no reaction and was very stable through it all. I will take her into Quest by the house Wednesday morning for a finger prick to make sure her hemoglobins are staying up. Hopefully we will not have to go back until next week.

I feel like I am forgetting to include something so I might be back later.... I hope you all have a nice week.

Update*

I did find out they are only planning on giving Dani 4 infusions. I also asked them to change clinic days from Monday's to Friday's. Primarily because my mom (who watches Dani three days a week) is getting Carpal Tunnel Surgery on May 4th. Grandma Boni is available to watch her on Monday's and my niece Trish watches her on Tuesday and will help me with a couple of Wednesdays so I am trying to be able to get in as many hours as I can. I think my sister Vicki is going to work with Mom to help her take care of Dani too somewhat. Are you keeping up? Dani can not go to daycare until she is 2 years post transplant so that is not an option. Fun as always!!!

Day +161 Transfusion

WBC 12.4

Hemoglobin 7.6

Platelets 343

ANC 8928

Weight 26 lbs 9 ozs

Did not get a Bilirubin count

It was a long day but things went fine on Dani's end. Just in case there are newbies, if you are at the hospital and the say 15 minutes it really means an hour to an hour and a half. She was lower on her red blood cell count so received a transfusion. She will start the medicated infusions on Monday.

Miss Dani has started a new trend of rolling her R's when mad. With steriod she seems to be mad quite a bit. So when she is learning spanish in high school she should have a step ahead of everyone.

We arrived at the hospital around 8:30 and left at 2:30. Dani actually cut it short by pulling out the IV line from the blood warmer. Luckily we only had probably ten more minutes to go on the flush so she received everything she needed. With the warmer on there we are not given a very big leash so she actually tripped on the wheels of the IV pole and when she went down it pulled it out of the warmer. I guess they were not thinking of little ones when they invented that.

She is totally in love with the Gerber Graduate Veggie Crackers. I think we went through half a box just today. She did not sleep much so she wore both of us out. They actually did not pre-med her before the transfusion nor did they start low and ramp her up on her infusion rate. She did really good with neither being done.

It is still unclear where our journey will lead us and so far none (knocking on wood) of this is effecting Dani besides the steriods. They did say they will wean her off the steriods while doing this medicated infusion so hopefully all will work and we will be moving forward. Please keep her in your thoughts and prayers.

Day +158

WBC 10.0
Hemoglobins 8
Platelets 352
ANC 7500
Bilirubin - 2.1 (Normal .1 - .3)
Weight 26 lbs 1 ounce
Height 32 1/2

Ok ... Another interesting day. So as you can tell her Hemoglobins are going back down. The steriods are obvioulsy not helping. They took blood to start screening for a blood transfusion on Thursday. They will look at her counts then and decide the next course of action. Dr. Hayashi is talking about starting her on a drug called Rituximab. This would be a weekly infusion. It will help destroy the B Cells. The B Cells are what are attacking her red blood cells.

I put the Bilirubin count up there because it is high and we need to keep an eye on it. They said when Red Blood cells are broken down it lets out Bilirubins. We were concerned about her liver function because normally high bilirubin means her liver function is not good but they said that they can also monitor liver function by the AST and ALT level. I am not sure what they stand for but they are both fine.

She is still acting fine but of course we are on edge with all of this. She was a trooper when they were trying to stick her for blood today. She got poked three times. Her arms have track marks on them. Right now they are not considering putting the central line back in, but if her viens get too chewed up that might have to be considered.

Day +157

All is well but I do believe the steriods are starting to show. Missy Miss has been really cranky today. I will take that as opposed to the alternative. We are hoping for good news tomorrow at Clinic.

The pictures are from at least a week ago with Dani enjoying her brothers and her bath. The middle one is Jason and I going to a 70's party. It was a lot of fun dressing up.

Day +156

WBC 10.4
Hemo 9.4
Platelets 3920


Well I took Dani for a blood draw this morning. They said we would not have results until later this afternoon. I talked to her Cardiologist yesterday to see what he was thinking. He was not aware of her hospital stay so I filled him in. He said we would wait to see what her labs show on Monday and go from there. He said the bright side of this whole thing is with her hemoglobins down so low that if her Cardiomyothopy was bad she would of went into heart failure. Since she was non symptomatic that show her heart is pretty strong. So a bright side to the last week.


Very good results!!! We are relieved that the steriods seem to be helping. I will update after clinic on Monday. I hope you all have a nice weekend!!!

Day +154

Dani slept over 12 hours from last night to this morning. She has been in a pretty good mood. I was astounded by the amount of steriod she is to receive everyday. I believe she is already getting her football shoulders. They also put her back on her anti-fungal which I am not sure why. I will ask on Monday. Above are some pictures from our hospital stay. You can see her IV arm on the first two days and she is lounging and watching Baby Einsten with Dad the last day while she was getting her second blood infusion.

I can not find my Kodak Camera's cable to download those so all I have is my cell phone pics. Hopefully the cable shows up soon. I have some cute bath time pictures.

We Are Home

WBC - 15.1
Hemoglobin - 9.3
Platelets - 338
ANC - 1102

We just got home an hour ago (6:30ish). They gave Dani the 2nd half of the blood and waited an hour and took her blood and let us go. We did not have the best experience today and I will not go into details but we are sooooooooooo happy to be home!!!!

We never did get to talk to a doctor today but the rumor was we would have Home Health Care come out Saturday to take blood for a count but that was not on the discharge papers so I am not sure. I know we have an appointment at clinic on Monday.

Dani is doing well. She is really tired as am I and we are hoping she will catch up on her sleep tonight. With her being back on steriods we assume her attitude will show and she will get her football shoulders back but whatever it takes to get her better.

I took some cute pictures with the cell phone at the hospital but do not have enough energy to download them right now. I will tomorrow. Thank you all for the thoughts and prayers and hopefully we are on an upswing.

Day +153 The Long Night

WBC 14.0
Hgb 8.3
Platelets 344
ANC 1134


First off everything is fine and Dani did really well last night.

They finally got the blood upstairs around 10:30 last night. They had to have a blood warmer on the IV pole because they had to keep the blood at Dani's internal temp. They hooked her up and ran it very slow for the first hour and took vitals all the time. Her blood pressure was showing slightly low but stable and no other appearance of a problem. After the hour they started cranking up the rate and Dani was sleeping so I snoozed for an hour or so. Dani woke up around 12:30 and then the IV started beeping that there was an occlusion. The two night nurses worked with her IV for a bit but we were running out of time because all the blood had to be given by 2:00 or it would have to be thrown away. They concluded that the IV originally given was so tiny it clotted with the blood.

They called in a Paramedic team to run a new IV in her other arm ... more fun!!!! Dani did really well and they had it in pretty quickly. Fortunately they could put in a larger tube and now we can get blood drawn out of it as well so no more poking. They finished a little bit after 1:00 and then we got the rest of her blood in by 2:10 am. I had to hold her for that hour because the lead from the warmer was not long enough for her to be very active. We finished the blood and then gave her her steriod and we were asleep around 2:45 to be waken a little after 4:00 am to get a new blood draw for counts. After that we slept until around 7:20am. When I asked about counts they said the labs had to be done a special way so they needed to redraw blood to get an accurate read. The last I heard (not officially) was her hemoglobins were at 8.3 (better).

The docs have not made rounds yet but the word on the street is they will give her the second half of the blood and she will be discharged today. Fingers Crossed!!!! I will update when I get more official word. Thanks for all the prayers!!!

Day +152

We have not gotten a full report yet from blood work but Hemoglobins are up to 6.0 ... normal is 10.5 to 13.5. She has gotten 3 doses of steriods so far. They still have not gotten any blood to give her. They are hoping they will get it this morning. She slept fairly well considering better than Jason and I. Her blood pressure is a little low ... bottom number 30 or 33 depending but that is also them taking it with the automatic machine that does not like Dani. I have not heard that they are concerned about that yet.

I will update again when I know more.

3:07 pm ... We are still waiting for blood. We found out she has both cold and warm antibodies. With this anemia it is normally one or the other. They had to take a fresh sample of blood and measure the temperature of it to get the transfusion to match. We will hopefully be getting things going soon.


5:37 pm ... Frustration setting in. Well a half hour ago they had two samples they matched (although not perfect ones) and they were going to let Dr. Wilson decided which one to try. He did not like how "reactive" they were and decided to confer with Dr. Hayashi and run another set of counts and decide if they would just let her bounce back on her own. Of course this means she got stuck again. Her poor arms looks like target practice. Although she is being a trooper and did not cry at all with this last one. So we are still waiting ....

7:43 pm ...

Hemoglobins 5.2
WBC 11.6
ANC 1730

Ok well something is not right but we are not clear on what it is. We just got the lab results back but have not talked to the doctors again. I will let you know when I find out. Obviously her Hemoglobins are even lower than yesterday and her WBC are much higher. Maybe this will end up being viral ... not sure.

9:46 pm ...

At some point we are going to do a blood transfusion but I am not sure when. We are apprehensize about reactions and such but they said if anything goes wrong we will head straight to ICU.