Making changes -- Procrastinating

Last night I had a bit of ADD. I started off preparing to pay medical bills for the months of January and February. But once I counted out 37 envelopes filled with bills which meant that Haven had gone to the hospital 39 times in 2 months and that just kinda left me mindless.

So I decided instead that I would look at the bills this weekend and do something more fun. So I have started to make a few changes to her site. I talked with Kris about building a web site for me. Hey, he's been making web sites for over 11 years so I might as well take advantage of it since it's free!!!

So probably throughout the weekend you will notice a few changes to her site. I want to organize everything on here because sometimes I have to dig through to find things I wrote about 6 months ago. Plus Haven has had over 15,000 visitors since I started this site in December 2007 when we were first diagnosed with the Neuroblastoma Cancer.

Haven's Visitor Map Since July 2007

Haven is going to help with some of the design changes so it should be a fun little project for us and it will give me a reason to get my mind off the many worries and concerns I've had recently.

A few changes but this is not the official site I want yet:

Haven's Story

ROHHAD Children


More to come

Home from the Hospital!!!

We arrived at the hospital around 8:30 am on Thursday to get ready for Haven's surgery. She was all happy. We hadn't really told her too much about it yet. She gets very anxious now so we waited until we got upstairs to explain it all to her.

When we got upstairs we met with the anesthesiologist, Dr. Elliott, and her eye doctor, Dr. Guyton. I really want to say how happy we were and are with both of these doctors. You are handing your child over to someone you don't know and have to really feel comfortable. Both of them made us and Haven happy. Dr. Elliott is one of the best anesthesiologist we've ever had at JH. I told Kris I wish we could request anesthesiologists each time, because I'd request him.

Haven got the "G"s on the side of each eye. This notes which eyes will be done and Haven needed both.

Heading off to surgery. Bluesy went with Haven. She said all her other babies were too scared to go to the eye doctor. Bluesy also had her eyes done, Haven said. Dad took her back - as I said before I only went one time and won't do it again. She did very well. She put her own mask on to fall asleep. The flavor of the mask this time - Popcorn. I was informed by Haven that popcorn flavor is very nasty!

She did extremely well through surgery. When she came out after round 1 we were able to visit with her. I only went back to see her for a short time - Dad stayed with her most of the time. I helped with holding her down to get eye drops in. Then they put her back under for round 2.

All in all - we arrived at 10:30 and were released from recovery room at the eye center around 4pm.

Haven was taken to the 4th floor of the pediatric building. She had a private room to recover in. About 2 hours after surgery they said she could have something to eat and she was so excited because I was going to get to feed her like a baby. We had lots of fun playing "Guess what food this is?". She was very happy to get carrots because as she says "they are good for the eyes".

Haven finally gets some sleep. She had a couple doses of Oxycodone and this really helped with the eyes. They were burning quite a bit. She kept asking for a mirror but we told her that hospitals don't have mirrors on the kids floor. We knew if she saw her eyes that she would freak out.

Dr. Paz-Priel came to visit us on the 4th floor on Thursday to see how she was doing. We were very happy he came by. Then her nurse from Oncology, Katie, called to check in on her. She was very happy but couldn't understand why Katie couldn't be her nurse over here.

As soon as Haven was conscious enough she wanted to make some phone calls. As soon as she got out of surgery she was begging to call Lauren. She had to call Lauren because she told Lauren she would. We called Lauren twice and left a message. Then when Lauren called back she got in trouble for not answering her phone and was told that she couldn't put her phone back in her purse incase Haven needed to call. It was very sweet. Sorry family - Lauren came first! :) But Lauren is her bestest friend she says.


Haven's port. She likes to check out her port every day to make sure it is looking ok. She also likes to look at it a million times when it's accessed. She said I had to show everyone how it looks and that it doesn't hurt when it's paper tape.

Finally awake on Friday. Haven was moved to the 8th floor for Inpatient Pediatric Oncology in the middle of the night. We had hoped to receive the Rituximab in the wee hours but were not able to get it until around 11am on Friday.

This is Miss Haven's right eye. They had to do both eyes. The doctor said the absolute way to check the eye roll is when they are asleep. We thought only one of Haven's eyes were bad but when she is asleep both eyes roll very far out and a lot of correction had to be done on both.

While receiving our chemo on Friday Haven woke up with a bit of energy - not normal for her. She wanted to walk around the floor and check out the play room.

We all played a couple of games and after about 30 minutes she got a bit tired. We headed back for a bit of a nap. Around 3pm we learned that Cyclosporin actually comes in two different types. There is the "N" type and the "S" type. Even though we specifically went over it with the nurses on the 4th floor and we showed them the box of her Cyclosporin we somehow received the wrong type.

We now know Haven gets the "S" type. However, this evening she received the "N" type. Haven's levels are checked twice a week and she should be at 200, anything over 250 puts her at a toxic level and we usually skip a dose. Around 3pm the doctors came in and told us that we received the "N" type and Haven's levels were at 389. We had to run tests on the liver and kidneys and they checked her over for blood pressure and watch for tremors.

Waiting on our tests to come back from the lab Haven fell asleep on the couch for a long time with Dad. Dr. Evelyn was very apologetic and very comforting in our wait for the results. Finally around 8pm the results came back and Haven was clear to leave the hospital. We got all excited and started to pack up when Haven reminded us that she still needed her port out!

Haven's nurse, Kim, came in and this was Haven's most dreaded time. She had been anxious about removing the tape from the port and removing the needle. She wanted to make sure this nurse knew how to do it like Nurse Katie does. She asked if they could make her sleep to remove the tape. But we made it through and Haven helped out.

This is a close up of Haven's eyes on Friday evening. Check out the difference. The doctor did an awesome job and we are very happy with everything.

Haven slept very happy last night. She slept all night and never woke up and I don't believe she even talked in her sleep. She woke up around 8am this morning and went back to sleep around 10am. We told her she could sleep all day and even at 2pm she is still asleep. :)

We are happy to have our baby back home. All of Haven's photos are uploaded in to her FLICKR account. You can click HERE to see all of them.

Update

A short update until tomorrow - as we were admitted to the hospital but luckily my phone has Internet!!

Haven did very well through surgery! She made a couple of phone calls tonight before her next dose of Oxycodone and Tylenol.

She went in to surgery around 10:45 am and we finally got her out around 4pm.

Her surgery was done in two parts. 1st part: major adjustments of both eyes. He found that when she fell asleep both eyes rolled off to the side really far and there was a lot to correct.

When she was coming out of the anesthesia from the 1st round we were able to visit with her. I only went back for a few minutes - Kris went back first and stayed with her for about an hour. I can't tell you how hard it is to see your child with blood-filled tears streaming down her face and what looked like fishing lines coming out of her eyes! It was very very hard to see her. The whites of her eyes are bruised, bloody, and just horrible!

The 2nd round of surgery they put her back under after checking the alignment from the 1st round and then they make final adjustments, remove all of the lines and strings hanging out of her eyes and wait for her to come back out again.

Haven was definitely admitted for the night. Kris and I have spent the past 6 hours wiping bloody tears from her eyes. She still refuses to open them so we have kept wet gauze on them all night. She keeps falling asleep and then waking with sharp pains through her forehead - she is staying on the Oxycodone for the night.

Oncology came in to check on her around 7:30pm - which was a nice surprise. And Nurse Katie called to check up on Haven to make sure she was doing well.

Haven will be transported to the 8th floor around 1am for her Rituximab chemo.

I will update more tomorrow. I have a few photos - although I was yelled at constantly about the flash of the camera! More info tomorrow - good night.

Thank you to everyone around the world that have sent Haven prayers and good thoughts! We truly appreciate everything. Love to all

Haven's Surgery

I was able to talk to a few of Haven's doctors and nurses today.

We need to be at the hospital by 8:30am tomorrow. Haven can not eat past 6:30am.....THIS IS THE HARDEST PART OF ANY PROCEDURE! Haven's surgery will begin around 10:30am. Do you even know how many times we will hear her ask for food during that time!!!!!????

I talked with Oncology and due to her at home chemo needing to be mixed with milk she will not take that until she wakes up but we will take the blood pressure meds - if Haven misses one dose of BP meds her BP rises significantly and we have to keep it down.

Once her surgery is over Haven will be admitted to the 8th floor instead of the normal recovery on the 4th floor. Haven needs to go to the 8th floor because this is the only floor that has Chemo Nurses (ha ha - that's how they're known). When Haven comes out of anesthesia she will begin her chemotherapy Rituximab - which should take about 6 hours to administer.

Summary: Surgery begins at 10:30am and about 6 hours later she will be in recovery receiving chemo for about 6 hours.

Haven will be monitored at the hospital for the night. We will update every as we can. We can't guarantee when we will be able to contact anyone - we will definitely be sleeping all day Friday if we're home.

Love to all.

Surgery Thursday

Anesthesia called me yesterday to discuss Haven. He had already spoken with all of her doctors and read all of her files. With complications she has had in the past with seizures, anesthesia, and breathing problems Johns Hopkins has decided that Haven will remain in the hospital after surgery. All of Haven's doctors agree. He said due to Haven's high risk and the rarity of her syndrome all of the doctors felt this would be the safest for Haven.

So we agreed. Really, I don't think we had an option. He had already done all of his homework on Haven and even read about ROHHAD. Usually we push for her to come home but I know Haven needs to be watched by the doctors just in case. I have to say I was extremely happy and probably helped me say yes because he took the time to find out about her instead of waiting for us to show up and ask about her.

Haven will still have her Cyclosporin levels checked and will still receive her Rituximab on Friday. They will bring it to her room and administer it as an inpatient.

Chemo Plan

Friday Kris and I met with Haven's Oncologist and discussed the Neuroblastoma Cancer and that they will keep a watch on the tumor and monitor that. We also discussed ROHHAD and Haven's current condition.

We have seen some positive results over the past 2 weeks with the Rituximab. She has been going potty by herself and she has been sleeping....or at least she did for a week. Since Friday's dose of Rituxan she has not slept. I really hope this evening is much better.

We also learned that she will be on her current chemo plan for about the next year. They will reevaluate in 6 months and continue on for another 6 months after that. So we are looking at hopefully going off the chemo this time next year.

Haven's Oncologist also gave me a bit of homework - which I haven't started yet. There are a few questions we need to ask some of the other moms to maybe come up with another theory and may require more testing for Haven.

As for now Haven remains under the care of Oncology (as she will for a long time), Pulmonary, Endocrine, and her Pediatrician.

Rituximab Friday

Friday started with a bang......accident on the freeway and it took us almost 2 hours to get to the hospital. Doesn't make for a great start when you know you have 6 hours of chemo to get through. Plus we have to drive two cars so I can go to work.

Haven knows what to do at the hospital. She goes in immediately and gets set up to have her vitals taken. She was wearing one of my shirts. She loves to wear my clothes. We were very happy with the results. Still staying at 72 pounds and now we are 41 inches tall. Growth is super important with ROHHAD. One of the issues with ROHHAD is growth deficiency and Haven stayed at 39 1/2 inches tall for over a year.

We had LOTS of problems with getting the needle in to the port. It was horrible but her nurse was trying to make it as easy as possible on Haven. She put the first needle in but when she tried to draw the blood nothing was coming out. She put the flush in to it and it squirted out Haven's chest. Kris couldn't even watch and I had to keep looking away. Her nurse took the needle out and pushed on Haven's chest and all the saline flush came pouring out of the hole in her chest.

After feeling around a bit her nurse realized the port had floated and was turned sideways. OUCH. Haven had been saying it was bothering her for the past couple of days. Somehow the port flipped on to it's side even though it's sewn down inside. So Katie (her nurse) flipped the port back and had to hold it down really tight to get the needle in. Haven definitely felt it but never cried or made any noise.

Finally the port works and Haven begins to draw her blood. She always does her own blood and Katie just loves it. Haven helps out so much. She was definitely uncomfortable with the port today.

This is Haven's chemo set up. As you can see multiple nurses have to check off on the bottle to confirm the correct Chemotherapy is in there, the correct dose, and the correct name. I like to see all these little check marks and initials.

Kris and I met with Haven's Oncologist while Haven was getting hooked up and prepared. She fell asleep almost instantly.

When I left about an hour or so later she was barely awake but really out of it. She was just kinda sitting there staring off.

Around 2pm I received a text from Kris that they were getting unhooked and leaving the hospital. Then I received another text a while after - Haven wasn't doing well and she had pottied all over herself and her bed and was soaked. She was changed and it happens again. Then she realized her toes were hurting and her foot was swollen. She couldn't get her shoes on and was freezing. She hung out at the hospital for a bit longer and the nurses gave her a heated blanket to wrap up in. Kris finally left after a while with Haven in a wheelchair and a big blanket wrapped around her still unable to wear her shoes.

They made it home and Haven was sick and tired the rest of the night. She was asleep instantly after getting home and stayed asleep until 7:30 when she woke up to eat a very small amount and went back to bed. See her blue whale...that's Whaley! :)

Saturday she was much better but we kept her low key. She was upset because Stryder was here and she couldn't see him on Friday and she definitely had to see him on Saturday. She got to see him for a little bit and Sunday was much better for her.

She is still having quite a few issues with the port. Her toes are split pretty bad but she is feeling much better.

Saturday and Sunday Haven did not sleep at night. Sunday was Kris' shift and I believe he maybe got 2 hours total of sleep. She was awake about every 20 minutes. I hope tonight is a much better night...she has doctors at 8am tomorrow!

Hanging with Stryder and lots of thank yous

My brother came home from Iraq last Friday and Mr. Stryder came to visit us. These are all from Sunday - when Haven was feeling a lot better.


Stryder is in love with Grandpa B-Honey's back yard and all the ROCKS! He is a huge fan of throwing the rocks and especially the BIG ONE! He is just so fun to watch. He runs around crazy. Definitely not the way I'm used to seeing a child!


Stryder gave Haven a hug but she just doesn't know how to act around him. She's so still and doesn't know what to do around kids. She usually just sits back and watches him.

Haven received this beautiful big puppy (hanging in the background) from a MACS friend. He is absolutely AWESOME! I love him so much. He's like a big body pillow. He hangs out in Haven's bed....we'll he takes up most of her bed! There were a bunch of stuffed animals and gifts in the box. THANK YOU!!!!

This is a beautiful Easter basket put together by my friend at work, Aleah. There were a bunch of crafts inside it - we made a caterpillar out of the beads inside it!

This was one of the stuffed animals that came in the MACS box. The cute little knitted hat was made by and sent to Haven from her Aunt Martha in Utah. Isn't it just adorable! I couldn't believe it. Thank you Aunt Martha.

Haven also had a couple of knitted hats made for her by Aunt Martha! Check out the kool-aid moustache! Now that is what being a kid is all about!

Haven received the cutest duck and books in a package. She was screaming and freaking out. She loves him so much. His name is "Littlie".....he is currently in her bed sleeping with her right now. She has been with a blue whale that she received the other day. His name is "Whaley". I will put a picture of him up - he was another gift she received. Believe me - all these animals sleep with her...it's amazing that she can still fit in her bed.

Shirley Anderson sent Haven a package with a wallet inside. This surprised Haven - there were photos inside, Old Navy gift card, and play money. She loves it. Shirley put the package together, Heidi gave the wallet, and Kelly gave the money. Absolutely great! She was so excited about the photos and couldn't understand how Shirley had photos of Grandpa Beard Off and Grandma Sing! :)

Aunt Dottie and her friend Andrea did it again! They sent Haven a beautiful package of custom made clothes to fit her. She received lots of spring clothing. Even capris and pink velvet pants. Haven has chemo tomorrow so I wonder what she will pick to wear! :)

Thank you everyone - we love you.

We're tired

Sorry everyone - I had hoped to have a bit of energy this weekend to get some photos up and an update on Haven. But it has been a weekend of no sleep and I just didn't even care to turn the computer on. I didn't even have my phone on this weekend.

I will hopefully get some updates on here Monday evening.

Love to all

Quick Post

Hi to all......I'm extremely tired even though I was asleep by 9:30pm last night.

Haven had a pretty rough day on Friday with chemo. She did not handle it very well and had an even rougher time at home that evening. Haven usually doesn't feel well about 2 days after chemo so it was very odd for her to have such a bad day. Her nurses were concerned as they had never seen her like that either.

I will give a better update tomorrow. We tried to spend a bit of time with my brother today. He just arrived home from 15 months in Iraq. So we have been trying to enjoy the day. Aunt Katie and baby Stryder are visiting as well.

I will give more updates tomorrow and maybe even take a picture or two!

Love to all

Medical Updates

Haven has Rituxan tomorrow - her full day of chemo at the hospital - and it has been requested by her Oncologists that I join Kris and Haven at this meeting to review everything. I do not normally go to the chemo appointments but since her doctors are urging me to be there I will join them.

(PS: Hi Oncology Doctors - they told me they read Haven's blog so this will be a test!)


What I know:

Haven will remain on Cyclosporin twice a day for a minimum of 6 months
Haven will remain on Rituxan for a minimum of 6 months

They will not alter this schedule or make any changes. I have been and will continue to keep a daily log of Haven and monitor her for any changes - positive or negative. At the end of her 6 months her doctors will discuss her files with the medical board for further review.

Haven is still on schedule for surgery on March 26th. Haven is at a high risk on this surgery due to the ROHHAD and the chemo. The biggest concern for her doctors is her being in anesthesia. The risk of her stopping breathing is significantly higher due to the ROHHAD. We have already been called about Haven staying over for observation - but I have requested that this only be done on an as needed basis and not automatically.

I also spoke with Haven's nurse today - the ABG (Arterial Blood Gas) will also be performed during surgery. She has to be under anesthesia to have this done so we had to make sure it happened at the same time as the other surgery. Haven's ABG will be done through her wrist. I am told she will have more pain from this than the actual surgery.

On March 27th - Haven will be back at the hospital for her full day of Rituxan.

April 9th - We will meet with Haven's Pulmonary doctor - Dr. McGinley. He will begin to handle Haven's case from this point forward - re: respiratory functions. He will review her ABG results with us and begin to keep a constant monitored file for Haven.

Early May - We will meet with Haven's Endocrine doctor Dr. Cooke - for her 6 month check up. We will look at her growth and talk about her immediate future where he is concerned. He has been working close with Haven's Oncology team and the Orthopedic doctor.

Grandparent's Week

Haven had a busy week last week as her grandparents came in and out throughout the week. After April 1st we will be keeping the house locked down pretty tight as we get deeper in to Haven's chemo so everyone had to come see the Princess.

We were missing a couple of the grandmas though - Grandma Sing was hanging in Utah and Grandma B-Honey is in Korea. We have a few Great Grandma's out there that we want to say HI too...we don't see them much at all but we wish we could.

We had a blast the entire week and Haven kept everyone busy.

Grandma and Grandpa Virginia came out early. Grandma stayed with Haven for the entire week and they were constantly working on crafts when Haven wasn't at the hospital. Although they only had 2 full days the entire week together they stayed really busy. As you can see in the background they worked on the tree. Now I haven't had time to take it down and we joked about having a tree for every holiday. Haven is now working on a new tradition - we have the first Easter Tree. As you can see they made egg ornaments, bunny ornaments, and a big easter egg bow is on the top of the tree. It's absolutely adorable.

They also made a St. Patrick's Day set up with a leprechaun on the side table. He is still there and she has been reading her books with us each day. I'm sure all of this will stay where it is until Grandma comes to change the holiday look. :)



Later in the week, Grandpa Beard Off came to visit us from Utah. He brought with him a most amazing quilt made by Grandma Sing. There are sections on the quilt for all of her family members. There are also hidden messages sewn in to the quilt that say "kisses" "hugs" "Haven" and flowers and butterflies. The quilt is absolutely amazing. There are also little charms sewn on to match different family photos. Haven has been asking to sleep with it but mom has put it away in a zipped back to keep it safe. Haven will absolutely cherish this quilt as she grows older. THANK YOU SO MUCH GRANDMA SING (and grandpa for your computer skills)....WE LOVE IT. We hope you can visit next time.

Haven randomly likes to try out new smiles. She practices them in her floor length mirror and this is one of her new ones. She says she doesn't do big smiles anymore because she's a little bit bigger now. I know, I have no idea what that means but I don't really argue with her much. But Grandpa certainly doesn't have a problem with big smiles! :)

Haven and Grandpa stayed in this position for two days. Haven asked me on Saturday night if she could stay up and "party". Of course I rarely say no to her and her Grandpa hasn't seen her in over a year so she partied. She and Grandpa worked on this sticker book that was sent to her from a MACS friend. They started the book right after chemo at 8pm and we had to beg Haven to go to bed at midnight.

On Sunday, She and Grandpa started early. We couldn't tear these two apart. I thought we were going to have to pay Haven to let Grandpa take a break. Haven's friend Mason sent her a 1st grade workbook that is about 500 pages.......They worked on this book from 8am until 11:30pm that evening. I think Grandpa took two 15 minute breaks to help Kris with the trash (sorry to put you to work Gpa). I'm not even sure Grandpa ate the day.

PS: They almost finished the entire book!

Before Grandpa Simon left with Grandma on Sunday we had to have a Grandpa photo. These are Haven's Grandpa's and some of the men that love her like no other in this world. You have Grandpa B-Honey, Grandpa Simon, and Grandpa Beard Off.

Wow, doesn't my girl look older! She has no make up on but she looks like she does. She has changed so much over the past year. She is our world and our princess.

Thank you to everyone for visiting our girl. Everytime we see her smiling and laughing and having a great time we just take it all in. We stop whatever we are doing just to hear her laugh. She has never giggled and laughed as much as she did this week.

All our love to our families for the support and care you have shown Miss Haven.

PS: we have lots more photos on Haven's flickr site at the right side bar. Just click the box and you'll see them or click a photo above.

Roller Coaster Week

Well you would think we had some time to relax last week but we were completely busy the entire week. With little sleep and constantly on the go we have been beyond exhausted. When you are constantly meeting with doctors, doing new tests, constantly making sure medication is taken and ordered, plus normal life of work and 24/7 stress and worrying about your child you are easily worn down and get grumpy.

I have been working on the grumpy part and trying to keep smiling but the past few months have just worn us down. My dream someday is to only have to worry if my towels match or if I have curtains on my windows! :)

Last weekend I got Haven's chemo in my eye - we washed it out but it BURNED. Then the next day while trying to use hand sanitizer I got a big squirt of that in the same eye. Now my eye has been swollen for over a week and it hurts more and more everytime I blink!

Monday I left for California for two days - which was nice because for the first time in a very long time I was asleep by 9pm and in a bed. I woke up in a panic around 130am and sent Kris a text....forgetting it's 430am in MD! :) So I went back to sleep to catch my bus to the airport at 415 am.

While on a layover in Denver I received a phone call from Kris who was clearly upset. He was at Haven's Podiatrist appointment - and I have purposely stayed away from the computer to clear my head about this - but through a really painful conversation with the doctor we will NOT be doing her toe surgery next week. We are looking for a new Podiatrist though. Enough on that....

I spoke with another mother of a ROHHAD child for the first time while in CA. Her daughter was diagnosed and on a ventilator since October 2006 - she passed away this January. Hearing her story just broke my heart in to a million pieces and has torn through my head all week. I even called to talk to Haven's Oncologist about her levels and decided to break down crying to him, drive the wrong direction on the freeway, and get stuck in traffic all at the same time. He reassured me today that I am ok and he is completely understanding of my anxious, worried, and insane concerns about Haven.

So it's only Wednesday and I have hit every emotion possible in 3 days!

By the way, Haven is of course doing great. She is sad that everyone left her at the same time but Lauren came today and will be here tomorrow. Haven had a lot of fun with her and told me how much she loves when Lauren comes to be with her.

Anyway, it's 11pm and Miss Haven has decided it's time to wake up and hang out. :)

Happy St. Patrick's Day

I have not been able to update Haven's blog in a while. We've had many visitors this past week - Grandma Virginia, Grandpa Simon came out on the 7th of March and stayed for a week. Then Grandpa Beard-off came out on the 13th and stayed through til the 16th. Then I left for California on the 16th and am coming home today.

We had great visits and lots of pictures to share.

Haven is at Johns Hopkins today to have her Cyclosporin levels checked and Kris is taking her to meet with the Podiatrist about her surgery on her toes.

I will update everyone on the happenings as soon as I can get a little rest from the past week!

Time to sleep on the plane! Have a great St. Patty's Day!

Pre-Op appt

Haven and I met with her Pediatrician today. It was a great meeting - we talked with her doctor for about an hour about everything that has been going on and how I feel and how Kris is doing.

Haven's Pre-Op exam was good. Mainly it's to review all of her medications, anesthesia she is not allowed to have, any past issues we've had with surgeries or anesthesia.

I received some info from Haven's medical files. Which is always nice to have them in our files.

It's weird......the doctors tell us that Haven has a serious disease and all that. But it's not until I read it in her files from last week where her doctors states "serious illness which is life-threatening" that it actually hits you. ROHHAD is a disease my daughter could easily die from --- not her Cancer.

All of our children with ROHHAD are in a life-threatening position that we have no control of. Our children may look fine today and be in the hospital for months at a time tomorrow.


Miss Haven is in Chemo all day today. This will be her 2nd dose of the Rituxan. We have been closely monitoring the toes for Tuesday's appointment.

Hope everyone has a happy Friday the 13th!

Chemo and Infections

Haven started her first dose of this other chemo last Friday. By Saturday, there is an infection in her big toe on the left foot. By Sunday morning, there are three more toes on the right foot that are infected.

By infected - I mean green, gooey, swollen.

By Sunday afternoon her big toe on her right foot is cracked open and bleeding. The bleeding has continued and during chemo yesterday at the hospital it was bleeding again.

She had 3 doctors look at her feet yesterday and the decision was made that she needs surgery on the toes.

Haven has less than a month to do this surgery because at that point her immune system will be gone and the risk increases significantly.

Considering that we already have surgery scheduled for her eyes on March 26th this only leaves me with next week available. Haven has an appointment with the surgeon on Tuesday, March 17th and hopefully we can get the surgery done next week.

Making it two surgeries in two weeks. If we're hard to reach over the next few weeks you will know why.

ROHHAD Mom's

I want to thank all of the ROHHAD mom's I have talked to either by phone or email and who are always here to support each other. I couldn't do it without them by my side. I honestly don't know how the few parents made it before ROHHAD was officially noted. There was no one there to help them. So I am grateful and amazed at how everyone has come forward, introduced their family to us, and shared pictures and stories of their child.


I can't express how horrible this disease is. I go crazy trying to figure stuff out to a point of completely confusing myself and having to go back and do it all over again. I'm told by one doctor that my daughter is on a progressive schedule to stop breathing by age 7 and then I have Oncology working their butts off to find a way to stop it.


I used to fight the doctors - only because the first 2 years of this we had horrible doctors that didn't care and it was easier to blame us. Now my only hope is in the hands of the doctors. We absolutely love our doctors at Johns Hopkins. Who else would give a mom their email addresses knowing she will email about everything and anything.


We're tired, we're frustrated, and we're scared. We go to bed wondering if this is the night Haven stops breathing.....I go to work and sometimes I wonder will I get a call that Haven is in the ER. I know people think I'm just an angry woman but after almost 4 out of 5 years of your child having something different about them that no one can tell you what's wrong and they blame you as the problem wouldn't you be angry.


I have tried to relax and let the doctors do their thing. No one knows how to fix this and no one knows where it comes from or why it happens.


Out of 20 or so symptoms of ROHHAD Haven has 11 or so. There are more but 11 are the main ones.


My child has NEVER been sick a day in her entire life but she has Cancer and ROHHAD.


Haven has NEVER cried a day in her life --- she's 5. THAT'S NOT NORMAL.


Haven doesn't feel pain - AT ALL. Cuts herself, falls, hits her head and has a huge lump, has 3 infected toes that get stepped on ---- NOTHING. No pain, no crying, no screaming. THAT'S NOT NORMAL.


She can pass out in the middle of a sentence in the middle of the day BUT SHE CAN'T SLEEP AT NIGHT!


We are frustrated....We are tired....


What keeps me going? How do we keep up the fight?


1. Haven's beautiful smile and her ability to keep laughing

2. Kris' patience with me and how he is always taking care of Haven

3. The ROHHAD mom's and dad's

4. The children that we can possibly help by sharing our stories



I can honestly say without your encouragement and help with spreadsheets and articles and stories of your children I would be insane. THANK YOU!

One mom who is constantly making me laugh daily -- even when she can't sleep and has blood shot eyes --- she makes me feel awesome! THANK YOU JULIE!

Miss Haven

I am still waiting to talk with Haven's doctors so I don't have too much to update medically.

Haven's toe infection seems to be getting worse. She is at the hospital Tuesday for a full day of chemo. Hopefully she will be home around 3pm or so.

I gave her a quick shower (hoping she would sleep better) and a few minutes afterwards her big toe on the "hot" foot cracked open and started bleeding again. So we cleaned it up and put her lotion on it. Kris will have it checked out and probably start a round of antibiotics.

Haven will also have her levels check Tuesday. She was at a toxic level again on Friday and we had to cut her home dose down through the weekend.


This was Haven last Friday at chemo. They give her a good size dose of Benadryl to avoid any reactions or hives from Rituxan and we like it because it does help her to relax. We did not have any throwing up this weekend but we did have very little sleep. By very little - I mean about 3 -4 hours total in 48 hours!

Haven received some beautiful clothes from Aunt Dottie in Michigan. Aunt Dottie's friend helps us out by making beautiful jeans for Haven. She hasn't been able to wear jeans in a few years and this is just awesome. The jeans have little cherries on them and are extremely cute. Haven absolutely loves the entire outfit. THANK YOU

She also received a painting kits from the nurses at Johns Hopkins Riverside. I'm excited to do these. There are butterflies and dragonflies and letters for her name. We're going to paint them and hang them in her room.

Haven's friend Sophia also sent her a package of gifts. She packed this tote bag up for her trip to the hospital on Tuesday. Absolutely adorable - THANK YOU SOPHIA (and mom!)

The cutest Zebra carpet ever - Miss Sophia sent this to Haven as well. Fits perfect next to her bed!

This is the matching pillow. Haven has her little babies sleep on this at night. She loves it because now she doesn't have to share her pillow with her babies!

Thank you everyone for caring for our daughter. We love you all.

Hopefully we'll see a few of the JH nurses on Thursday when we go to Haven's Pre-op appt.

ROHHAD and CHEMO updates

I know we have a few updates to post. Haven had a crazy weekend with chemo. We believe it has kept her up (more than normal) over the past few days. Kris did the Saturday shift and I did the Sunday shift. I think between the two nights we had a total of 3 hours of sleep. I can't wait to sleep tonight.

Haven began Saturday with an infection in her left foot (cold foot) that was gooey. On Sunday she had three more toes that are infected on the right foot (hot foot) and they are gooey. We soaked them and cleaned them. She then took a 3 1/2 nap and by 12:30 pm on Sunday her right toe was cracked and bleeding. So we soaked them again.

Now this I felt extremely horrible about - but we were in the kitchen and she likes to help with the groceries. But I stepped on her 3rd toe (which was gooey) which my shoes. A normal child would've have screamed and cried like crazy - not to mention it's already infected. Haven just gave a big sigh and we looked at it.

AGAIN she does not feel pain but knows she is supposed to make some sort of noise.

So back to soaking to clean off any dirt and goo that I added to the toe.

She didn't have a fever so we didn't take her to the hospital. I have been told she took a huge nap this morning after I left for work! NOT FAIR! :( I want to sleep too!

Anyway, I am waiting for more conversation with Haven's doctors before I post any updates.

Neuroblastoma Tumor

MRI results came back today - Kris reviewed them with Haven's doctor and EVERYTHING IS GREAT!

The tumor that still remains has not grown since last May so we will continue to focus on ROHHAD and monitor the cancer.

Thank you to everyone thinking about Haven! She is currently in chemo at the hospital. They have been there since 8am and she should be coming home today around 5pm - as long as it all goes well.

Love to all

MRI

Haven arrived at the hospital today around 8am for her MRI. I told Kris before going in that they were going to be on him about intubating her and he knew it. There is no fighting it anymore. We used to be able to fight but we know her file tells a different story now.

We used to meet with one anesthesiologist for an MRI - then it became two because we would fight about the tube in her throat and overnight stays. Today they were ready for the fight - they brought 2 anesthesiologists and 1 doctor out to meet with Kris. So he knew there was no arguing.

Kris took Haven back to the room to get anesthesia and usually she has them go straight through the port. This time she decided she wanted the mask first and then they could do the port after she was asleep.

They didn't have the nitrous oxide but instead made a mixture special for her that would help not make her so grumpy afterwards. Well, two breaths in to the mask and Haven freaked out. She panicked, of course scaring Kris, it took 3 doctors to hold Haven down while they held the mask over her face.

She was out.

I can't explain how hard it is to watch your child go out of it. I did it one time. That's it. I don't watch anymore.

Haven woke up and they brought Kris back. She actually only took 2 hours to recover and get her oxygen up high enough to be able to leave. They got home around 3pm and Haven slept until 7:45 when I woke her up for her chemo.

She has been coughing all night and has a horrible sore throat from the tube but she is doing well. She had a bad bout of diarreah today from the medication but I'm hoping she goes back to sleep soon or this is going to be a long night.

Tomorrow: 1st dose of Rituxan. This will take approximately 7 hours.

Dates.......can you believe it?

So we're looking back at everything from last year.......I pulled our medical records. These are all of Haven's hospital visits last year.


1/3/2008
1/4/2008
1/5/2008
1/8/2008
1/10/2008
1/16/2008
1/21/2008
1/22/2008
1/23/2008
1/24/2008
1/25/2008
1/28/2008
1/30/2008
2/1/2008
2/2/2008
2/3/2008
2/4/2008
2/5/2008
2/6/2008
2/15/2008
2/22/2008
2/27/2008
3/3/2008
3/11/2008
3/14/2008
3/27/2008
4/4/2008
4/8/2008
4/9/2008
4/10/2008
4/11/2008
4/15/2008
4/16/2008
4/17/2008
4/22/2008
4/28/2008
4/29/2008
5/6/2008
5/7/2008
5/8/2008
5/13/2008
5/21/2008
5/30/2008
6/4/2008
6/5/2008
6/6/2008
6/16/2008
7/1/2008
7/2/2008
7/17/2008
7/28/2008
7/29/2008
7/30/2008
8/26/2008
8/27/2008
9/23/2008
9/29/2008
10/7/2008
10/10/2008
10/14/2008
10/21/2008
11/10/2008
11/17/2008
11/18/2008
11/26/2008
12/5/2008
12/6/2008
12/16/2008
12/17/2008

INSANE!!! And to think this year is going to be even more. Kris takes Haven to the hospital 3 to 4 days a week!!!!! Since the first of the year.

4,380 total miles back and forth to Johns Hopkins!

Missing 2005

So the other day I realized I was missing 2006 and I updated it. The other day I realized I'm missing 2005.....so I'm working on it.



Click the photo to go to Haven's flickr site with all of her pictures through the years - you have to click around to find the link or you can click HERE.

click for the photostream or on a set of photos to view them all.

Cardiology

Spoke with Haven's Oncologist this morning - Cardiology report came back great. Miss Haven has a good heart and the chest looks perfect!

We're very happy for this news.


Haven will be in MRI tomorrow for the Ganglioneuroblastoma Tumor in her abdomen. This is our followup. Her last MRI was in November 2008.

The hospital called yesterday wanting to reserve a room for her for overnight recovery and to be monitored. I was able to convince them not to make her stay overnight even though they were really urging me to have her monitored. The more her file grows with ROHHAD and the breathing issues involved with ROHHAD the harder it will be to fight this request. I'm sure by our next MRI they will keep her.

Haven has had issues with 3 other MRIs and had to be intubated during and after the test - but it was not due to her ROHHAD syndrome.

Mostly likely Haven will be in recovery for about 1 1/2 hours on oxygen. Her oxygen is usually very low after an MRI so once it is up to at least 97% they will send her home. I'm guessing, from past MRIs, she will be at the hospital for about 5 to 6 hours.

Reviewing the journals

First before I get in to reviewing the journals I would like to thank everyone that sends cards and gifts to Haven. We truly appreciate the smile it brings to her.



This afternoon - after a day at the hospital - Haven received a beautiful bouquet from Mason. When I came we enjoyed it together and she wanted to call Mason. So she ran in to the bathroom with my phone and got to talk to him for a little bit. They discussed the important things like "What did you have for dinner?" --- it was adorable.

Haven talks to Mason (and pretty much everyone) in the bathroom. She hides in there so we can't hear what she's saying. I have to stand by the door incase there is a machine but when the person answers I have to leave.

Over the weekend Kris and I had to sit down and review Haven's journal. Initial journal reports from early 2008 to now. There is one thing I realized over the weekend that we had forgotten about one big thing - that may not seem like a big thing to anyone else but could be a positive sign. Hey, we'll take what we can get right now!

Haven used to be a heavy sweater. It started around age 2 years and 8 months. She would be sitting and doing nothing and be pouring sweat. She would sit on the couch and color in her books and her hair would be dripping with sweat. It was crazy. Well we did chemo for 6 months last year and so far about another 3 months this year. I realized - Haven has not sweat like that since last summer. It just stopped.

Did one of her chemos from last summer stop that portion of ROHHAD? I don't know but it's a good thing.

Also some happy news:

Since starting chemo in January Haven has lost 4 pounds and grown 1.25 inches. In December (as well as all year) she remained a constant 39 1/2 inches tall. She is now 40 3/4 inches tall.

We are very happy to realize all of this. And hope we'll see more positive signs develop in the future. Let's pray this chemo actually works a miracle for us!

Fighting ROHHAD

I received a phone call today from Haven's nurse confirming her schedule and list of medication.

Rituximab will be every Friday and we will continue with Cyclosporin at 2 times a day with levels being check every other day. With Rituximab she will get Benadryl in the port prior to administering the chemo. This helps her with any reactions to the chemo and I like it because it relaxes her.

Rituximab takes about 7 hours to get the entire bottle in her system each visit. This also usually makes her quite sick about the 2nd day after receiving it. She will probably throw up on the drive home - as this was the case last year on the lower dose. So the car will be packed with proper buckets and such. We have a lot of risks with this chemo and this is why they don't really like to use it and definitely don't like to use it for very long. I have been refreshing myself with this chemo. Usually this chemo is used to treat non-Hodgkin's lymphoma.

We have received the possible side effects of Rituxan (same as Rituximab).

This is one dose of Rituximab - which takes about 7 hours and goes in to the port in her chest

RITUXAN IV: Rituximab may infrequently cause serious (sometimes fatal) side effects including severe breathing problems (e.g., hypoxia, pulmonary infiltrates, acute respiratory distress syndrome) or heart problems (e.g., heart attack, irregular heartbeat, low blood pressure). These effects are more likely if you already have heart or lung problems.

Due to the high risk of infection - if you are sick or think you are sick please respect that we do not want our daughter in the hospital and cancel your visit to our house. Anyone entering our house and planning to touch or be near Haven will need to use the hand sanitizer that is placed in each room.

For those visiting our house - a complete list of common side effects that are most likely to occur with Rituxan please click HERE. Knowing the side effects helps in determining when to call the doctors.

A temperature of 100.4 requires Haven to be in the emergency room due to high risk of infection in the port.

Tomorrow she heads back to Cardiology and has her levels check at Oncology.

Thursday she will have the MRI for the Ganglioneuroblastoma Tumor

Friday she will begin Rituxan and have her Cyclosporin levels checked

thank you to everyone for thoughts, prayers, and letters of encouragement! We continue to fight this with Miss Haven and all the other children we have learned about and love.

First real snow

shoveling the snow
Originally uploaded by bamboozleroo

We were hit pretty hard last night. Our electricity actually threatened to go off 4 different times. Everything kept flashing and then coming back on. This morning it got a bit crazy outside. Haven refuses to go out in the snow as she will get her hair wet.

Our measuring tape actually shows the snow is between 8 and 9 inches up here. I called down to my friend in Baltimore and they only had a couple inches of snow.

The woods in the back yard this morning

Out the front window across the street.

Letter about ROHHAD

I'm sure many of you don't read the comments written by others to Haven's site. But I found this one from Vicki - Leigh Ann's mother - very important. I would like to share this with everyone.

Misty,

It is good that you have doctors at John Hopkins who are concerned for Haven. A doctor who listens and believes the parents about the symptoms of their ROHHAD child is very important.

The first pediatrician for Leigh Ann would not listen or believe me. I quit him after Leigh Ann returned home in Oct. 1992 from the children's hospital in KY. In Nov. 1992 I changed to an Evansville, IN pediatricain who was located 8 miles from our home. This pediatrician was open-minded and he would listen and believe me.The Evansville, IN pediatricain is the one who diagnosed Leigh Ann with LO-CHS/HD now called ROHHAD in Nov. 1993. He then sent her by air life-flight back to the children's hospital in IN. This children's hospital was 200 miles from our home.

Leigh Ann had been a patient in this hospital several times since Nov. 1992. Also we had made several trips for clinic visits at this children's hospital. But they had failed to diagnose LO-CHS/HD. The Evansville doctor had a very difficult time trying to convince the doctors at the children's hospital that Leigh Ann had LO-CHS/HD.

In Feb. 1994 the doctors wanted to transfer Leign Ann to a rehab hospital that used their doctors. But due to the problems we had experienced at this hospital, I had Leigh Ann transferred to a neurological rehab hospital in Michigan. There were good doctors at the rehab in Michigan who had a better understanding about LO-CHS/HD. Because of the problems with the children's hospital in IN, we started taking Leigh Ann to Vanderbilt in Nashville, TN. The local pediatrician in Evansville, IN asked the help of the Vanderbilt endocrinologist to explain LO-CHS/HD and to assist the other Vanderbilt doctors with understanding LO-CHS/HD.

The Vanderbilt endocrinologist had started Leigh Ann on growth hormone shots just prior to her death. She was treating Leigh Ann for her SIADH problem as well as other endocrine problems related to what is now known as ROHHAD.

All ROHHAD patients need caring doctors. An over-confident doctor who thinks ROHHAD symptoms is only a problem of too many calories can do more damage than good for the child.

There is still more knowledge that needs to be learned about ROHHAD and its treatment. However, it seems to me that at least Haven's doctors are trying and care about her.

Love & Prayers, Vicki

The Kuwait Medical Journal - CCHS or LO-CHS/HD or ROHHAD

YES I SENT AN EMAIL TO THE DOCTOR AND HOSPITAL - IF THEY READ IT, GREAT!
My expert diagnosis - ROHHAD not LO-CHS/HD....but that's my opinion. You can read the entire article HERE.

December 2007

THE KUWAIT MEDICAL JOURNAL
Case Report

Late Onset Central Hypoventilation Syndrome with Hypothalamic Dysfunction in a Kuwaiti Girl

Othman A Aboushanab, Saad A Alotaibi, Yasser A Shaalan
Department of Pediatrics, Farwaniya Hospital, Kuwait

Kuwait Medical Journal 2007, 39 (4): 376-378

ABSTRACT
Late onset central hypoventilation syndrome (LO-CHS) is now considered a well-established disease that develops in previously normal children after infancy and has been regarded as a distinct entity from the congenital central hypoventilation syndrome (CCHS). Both conditions are associated with neural crest tumours, but hypothalamic dysfunction (HD) is a feature of LO-CHS and not CCHS. We report a case of LO-CHS with HD (LO-CHS / HD) who presented in respiratory failure at the age of five years.

INTRODUCTION
Congenital central hypoventilation syndrome (CCHS) is characterized by abnormal autonomic control of breathing that results in severe hypoventilation occurring initially during sleep. It was first described in children by Mellins et al in 1970[1]. In most cases the onset of CCHS occurs in the neonatal period or during the early months of life[2]. A form of LO-CHS has been described in the literature. Katz et al[3] proposed that late onset central hypoventilation syndrome associated with hypothalamic dysfunction (LO-CHS/HD) is a distinct clinical syndrome. Both CCHS and LO-CHS have been associated with neural crest tumours and they generally occur in a histologically normal central nervous system (CNS). Both conditions have also been associated with PHOX2B gene mutation[4]. HD has been frequently associated with LO-CSH but not CCHS. We report on a case of LO-CSH/HD presenting in a five year-old Kuwaiti girl.

CASE REPORT
This Kuwaiti girl was five years old when she was first admitted to our pediatric department in coma, with marked hypoxemia and respiratory failure. She was successfully resuscitated and transferred to ICU for mechanical ventilation. Initial blood gas showed acute on top of chronic respiratory acidosis.

Preparing for Chemo

This weekend has been nutty --- not that we did anything but instead we've done absolutely nothing. With the news on Friday we were definitely down in the dumps. I was fine when I talked to the doctor on the phone but when I hung up and had to tell Kris it hit me. This is going to be rough. We still can't believe this is happening.

Friday night I couldn't sleep. I believe I slept a total of 3 hours broken in to half hour bursts. Saturday we had quite a bit to do to get ready for the week and I was exhausted.

Saturday night was a rough night as well. We just can't get it out of our head that Haven's health is declining right before our eyes. Haven had a major sleep walking spell around 2am - which hasn't happened in a long time. Kris was on duty and took care of her. So in the morning I rearranged her room and took a lot out so there is nothing that can fall on her if she is sleep walking.

Today I destroyed the house. This is how I clean. I throw everything out of a room and totally destroy the room I threw it all in and then I clean. Once everything is in the catch-all room I load up bags of trash or save bags. Haven's room has been completely remade.

We have been scrubing everything down and making sure all is sanitized in preparation for the chemo on Friday. Miss Haven has been very tired and sleeping all day. She did make time for some workbooks. She loves to do workbooks - and wants to be in school so bad.

We are getting ready to head over to Grandma B-honey's. She is headed to Korea tomorrow for a couple of months. So Haven wants to say goodbye and give her a big hug.

We hope everyone had a great weekend! :)