Tuesday, June 30, 2009

Hi CY - Day 2

Well it's been a bit crazy around here and we've met many doctors and nurses. But it's nice to know the ones that are assigned to Haven and that they are able to learn about her and this crazy disease.

Haven has been having wild sleep patterns - which we are told is due to the high levels of Cytoxan. I think she even scared her nurse Patel the other night. The nurse told me she came in the room in the middle of the night and Haven told her to be careful because there was a little girl outside her window that keeps staring at her. She told the nurse this over and over. Haven is on the 8th floor!! The nurse was joking and telling us that she wasn't going to come back in here.

Then during a diaper change Haven decided to become a ninja and beat everyone up with her superstrength. Kris said that was around 4am and by the time they were done changing her both had a big sigh of relief.

We finally got to give Haven a shower yesterday. She is required to have a shower each day to keep the germs away. This is a huge task. We have to get the nurses to unhook her cables and wrap up her central line so it does not get wet. Then we had to put a chair in the shower so she wouldn't slip - she fell the last time she was here and took a shower.

After all this we got settled down when the Cytoxan hit her bad. I spent the rest of the afternoon up to my elbows in diarhea! Yea, that was fun!

Around 6:30pm Haven said she was really tired and wanted to take a nap. She was asleep within 30 seconds and about 1 minute later I was calling the nurses and doctors in. She was snoring very loudly and very deep in her chest. The first nurse and I couldn't wake Haven up so she called the Oncologist in and by that time Haven was awake.

She fell back to sleep about 1 minute after they left and I watched her. I took 4 videos so I could prove I wasn't crazy. Her O2 was at 100 and her HR was at 130 resting. Everything looked good but she was extremely loud and having difficulty breathing. I opened the door and both nurses came out of the station and yelled down to me "is that her?". They grabbed the other Oncologist and headed in to our room. He listened to Haven for a few minutes before waking her up. He just wanted a base line to know if it changes over night.

The Hi CY is given to Haven at 10pm. We were going to give her Benadryl to help her relax during the treatment but about 10 minutes before it was to begin we canceled that. I was too worried that putting her in to a deeper sleep during chemo would be a bigger problem than we needed.

Sunday, June 28, 2009

Hi CY - Day 1

Well yesterday was very stressful so we were hoping for a less stressful day today.

Yesterday afternoon we received the news that Haven did not have Hepatitis B and was clear to start chemo. So the order was placed. By the time the pharmacy made it and cooked it she started around 10pm last night. Within the first 30 minutes she had a pretty strong headache.

They have kept her loaded up on Zofran today to help with the nausea and we have started her back on Colace as well.

She is on a constant drip of fluids which requires her pee to be tested every two hours.

Haven also had her EKG today which came back great and the doctors were all very happy with it.

We were also shown how we will clean her port. However, while she is in here the nurses will clean it. I think the removal of the tape will be the worst part of all of it.

This is the line of the new port. You can see the scar on the upper left which is where her other port is located. Her Central Line looks great. As usual, Dr. Price did an awesome job. The line gets pretty heavy with 3 lumens hanging from it with medication dripping in so we have rigged it up with a safety pin that is held on to her hospital gown.

We're very excited also because Nurse Clair has signed on as Haven's primary nurse. She will be Haven's main nurse throughout this entire treatment and Nurse Patel and Nurse Stephanie will be her night nurses. Haven really likes Nurse Clair.

I have not met Nurse Stephanie but I really like Nurse Patel as well. We had a few funny little incidents last night with Nurse Patel. When Haven went to sleep at 9pm Nurse Clair was still here and then around 9:30 Nurse Patel came in to do Haven's vitals. Well she freaked out and wouldn't let the nurse touch her because she didn't know who she was. She wanted Nurse Clair. It was funny. Kris and I had to go through the changing of the nurses with Haven 3 times before she let her do her blood pressure.

Then Kris told me around 4am they had to do a diaper change but Haven was sleep walking at the same time. There was quite a bit of fighting and the nurse told Clair all about it this morning. She said it was a huge sigh of relief when the diaper change was over!

Haven is doing as well as can be expected. The doctors are all very proud of her and how well she handles taking all her medications and working with the nurses. We met with 3 Oncologists today and all were happy with her numbers.

She will receive the 2nd round of Hi CY tonight around 1opm.

Saturday, June 27, 2009

Confirmed to go

Haven's usual Oncologist, Dr. Paz-Priel, came in to see her today. I had a feeling he might show up and then I told Kris that I thought I saw him in the hallway.

He came in to talk to us about the Hepatitis B and what we would do if it came back positive. We passed back and forth a few jokes about how he just likes to make sure we're worried enough. He said a few more grey hairs wouldn't hurt! :)

He looked Haven over and checked her breathing. Everything looks great. He told us that the lab is not usually open to run these tests on a Saturday - which we were worried about. But they were able to call in a favor and the lab person came in to make sure we knew asap.

About 45 minutes later he came back in to the room with our Inpatient Oncologist, Dr. Farah, and said ALL TESTS ARE NEGATIVE!!!! Yeah! So we are good to go with Hi CY.

Haven should begin Hi CY within the next 2 - 3 hours. Our other Oncologist, Dr. Chen, will be checking in on her tomorrow. Haven will be required to go potty exactly every 2 hours after the initial receipt of the Hi CY. Even at night - the nurse will come in to wake her up every 2 hours to make her go to the bathroom. We can not leave the Hi CY in her bladded any longer than that or we will have major complications.

Also with the Hi Cy we are required to put this paper in to her diaper to check the amount in the urine.

We are extremely excited she does not have Hepatitis B. I'm really nervous now about the future blood transfusions she will have while on this treatment. But I really have no choice - I can't deny the transfusions.

Haven is finally sleeping. She did not sleep at all last night. She had the fevers off and on and was just very anxious to not be in her bed. So I am very happy she is finally out of it.

Friday, June 26, 2009

24 Hour Delay.......

Around 4pm today our Inpatient Oncology team came in to talk to us and they were a bit to serious this time. They sat Kris and I down to inform us that Haven has tested positive for Hepatitis B.

I was in extreme shock I didn't even know what to say at that point. I just looked at her and said "I don't know what that means". Kris jumped to his feet and had a bit harsher language and more reaction than I could muster. He then calmed down and regrouped.

They don't know how she could test positive. We were told it could've been from a blood transfusion during surgery. She's only had 1 surgery that required a blood transfusion and that was February 2008. We couldn't believe that after 1 1/2 years she'd test positive now. We have blood taken every week and spent many days giving blood the last 2 weeks.

The doctor told us she called Haven's Oncologist to inform him. They believe it could be a false positive but to be sure they have had to delay the start of her chemo 24 hours. They did a lot of tests this evening and alerted the lab that they were on the way.

We will find out tomorrow what is going on.

Then tonight around 6pm or so we noticed Haven was looking really bad. She has had diarhea all day long, her eyes were now sunken in and dark circles, she has a rash under her left eye and red streaks on her face. Then she developed a fever. She has been closely monitored for the past couple of hours and her port site has been checked for sign of infection. We have been told they believe it is an allergic reaction to the anesthesia used today.

We will again find out more about this tomorrow. She will be closely monitored all night.

We are extremely exhausted. We are tired of worrying all the time. I believe today added another 5 years to us. It has been an extreme roller coaster but we are trying to stay calm.

Can't we just get one break here?????????

I have to say my baby is AWESOME and AMAZING. She is the most amazing girl I know. She has been awesome through all of this. The past almost 3 years of doctors and hospitals and she never complains. She just goes along and does all their tests. I love her to death. I only wish I was like her.


Around 9am we met with the surgeon and Haven was headed to recovery. The surgery went very well - no complications. He was able to get the line in to her left jugular and the other line is playing perfectly in her artery to her heart right next to the line for the other port.

We like this surgeon because he is "real". He tells it to us straight and will tell us when we're crazy. He has remained informed over the past 1 1/2 years on Haven and how she is doing. He told us this only marks an extremely rough road ahead for Haven. His sincerity and concern truly make us confident in doing any of Haven's surgeries.

He did warn us that he is now out of locations on her body to put in any more equipment. As he said - "we are out of room - no more ports!" Believe me, we don't want any more ports either.

He thanked us for allowing him to care for Haven and to be part of her life and he looks forward to the day when she is no longer at Hopkins.

Hopefully in about 4 months she will only need one more surgery to remove the Hickman line (Central Line) from her chest as the risk for infection is so high we don't want it in there.

Thank you Dr. Price and thank you to your dedication to medicine....without your extreme skills my baby wouldn't be in the best hands! We owe you a lot!

Here is Miss Haven and her latest smile. She has decided that this is the new smile. Really - it looks like she's just puckering her lips but this is the only way she would smile for the camera.

This was taken around 1pm. This is the first time ever that we were not able to remove the oxygen line from Haven. She was out of surgery at 9am. Every time the nurses would test her O2 levels by removing the line she would immediately drop in to the low 80's. So we had to keep this on until she remained stable at 92.

You can see by this picture where the incision is at her jugular on her left side and her newest port in her chest. She is extremely cautious of this new port and will not walk fast at all. I was able to sponge bathe her and get all the orange iodine off her body.

Around 3pm she was well enough to go to the playroom. Little did I know we needed to have the mask on if she leaves isolation and we had to have her room door shut tight! The nurse let me know immediately and she put the mask on Haven. So I won't forget that next time.

Haven loves to do puzzles so we worked on a Spongebob puzzle. During this time I also met with Haven's 3 new Oncologists. They work in the Inpatient Oncology wing. Dr. Jennifer, Dr. Farah, and Dr. Chen. We'll be working with them a lot during these next few weeks.

Haven in surgery

Here we were at the hospital at 6am. Only one other little girl in there getting ready for surgery.

Miss Haven ready to go. This is the pediatric surgery prep room. We have been in this room so many times all of the nurses know Haven. She is also good friends with the Child Life girl, Lauren, that works in this room.

Here she is with Lauren and the other girl that was getting ready for surgery. They were working on crafts. The best thing about this hospital is the Child Life team. They are awesome with the kids. Haven loves playing with them.

Ready for surgery. I was super excited about our anesthesia team. Kris and I always put a request in as to who we want to be Haven's anesthesiologist. Many times we get our wish and today was perfect.

Dr. Greenburg (on the left) was Haven's anesthesiologist during her first surgery done by our favorite surgeon, Dr. Price. It was a 14 hour surgery and they were awesome....so much we request them often.

Dr. Patel on the right was Haven's anesthesiologist last week during her MRI. He really learned Haven very fast and having her history made it much easier on us this morning. Usually we spend an hour with each doctor explaining what ROHHAD is. Today ALL 3 doctors knew Haven, knew everything about ROHHAD, and knew her complications.

I couldn't have picked a better team if I tried ---- sshhh I did beg a little for this team! :) Thank you everyone!

Thursday, June 25, 2009


That title is about how my mind feels right now.....just dotty and blank and numb and tired. I can't describe to anyone the gnawing feeling in my stomach at what is about to happen to our baby. Kris has been packing and preparing for his 5 month lockdown and I have been getting work in order to take as much as I can with me to the hospital next week.

It's now 10:15 and I'm washing clothes-typing on here because I haven't in a long time-and packing whatever I can remember to pack.

We will get up at 4am to get everything ready and in the car and then get Miss Haven up to head to the hospital by 5am. We must meet with doctors and anesthesia by 6am and surgery is scheduled for 7am.

After recovery from anesthesia Haven will be taken to her room on the 8th Floor Inpatient Pediatric Oncology. This is where she will remain. Her chemotherapy is set to start immediately after surgery and will continue for 4 days.

This is an extremely high dose of chemo called Hi CY. These 4 days of chemo will take all of Haven's counts to 0 and her stem cells will be forced to rebuild her immune system on it's own. These 4 days of chemo will keep my baby on lock down for many months. These 4 days will eliminate my baby's immune system for an entire year.

I will not go in to the gory details right now as I can barely process them in my head. I have emailed immediate family members and close friends with most of everything.

We have been told the ulcers will begin around day 7. Hair will completely fall out and many other superficial items we need to deal with.

We're guaranteed that Haven will be extremely sick. Due to the high risk of heart failure she will have an EKG done each done to monitor her.

Again I just can't think about this right now and I can't even explain it all again. My heart starts racing at the thought and I want to throw up. I just want my baby to be healthy and lead a full life and at some point enjoy being a child. We have been seeing doctors since the age of 2 1/2 and we are now 5 1/2. Too long to be in and out of hospitals.

Haven's Wheel Chair.....finished!

Her chair is complete and ready for her trip to surgery tomorrow. Painted Metallic purple with lavendar accents. The fabric is butterflies and flowers for the cushion. She really likes it now and is excited to show it off to her nurses.

Thank you to everyone for all of the emails this week. I probably received over 60 emails in the past 2 days wishing prayers and thoughts for Miss Haven. I am truly sorry I have not responded to probably more than two of them but under the circumstances I'm sure you can understand. I have been very quiet. This is the most serious and scariest thing we have ever had to do. I can't even describe the feeling in words.

I have to apologize to one of my bestest friends - Julie - I just haven't even really talked to her in over two weeks since finding out about Haven's treatments. I've just kept quiet. I have to thank her and Maureen for telling Haven's story on their web sites when their children have the same rare syndrome as my baby. I love you both. Julie I will call you tomorrow after surgery!!! Promise!

I also have to thank too many people in this world. Dad and Jeannie for the family love quilt/bringing Lauren in to Haven's life/visiting Haven a couple of months ago/and helping us mentally get through this. My mom and dad for helping care for Haven/buying 23 packages of diapers and totally realizing the cost of these things/making sure she has clothes that fit her. Mom and Simon for coming out every couple of months and staying for weeks at a time to help care for her/play with her/do workbooks with her. All of my family for keeping up with her medically and sending everything they can to make her smile. Aunt Dottie for her friend that makes all the custom fit clothes for Haven. All our friends and people we don't know that constantly send Haven cards/workbooks/crafts. My company...they have been so understanding of all of Haven's medical issues and have helped where they can and have given me so much support over the past 2 years.

There are just so many more people that write/email/call about Haven than I could ever thank. I only ask now if you could take 30 seconds of your day tomorrow and think of Haven so that she can make it through her 5th surgery in a year/massive chemotherapy treatment/5 months of isolation/and a huge welcome home by Thanksgiving.

All our love
M & K

Tuesday, June 23, 2009

Out of MRI!!

Miss Haven is out of MRI and was not required to stay the night! We are
very happy to bring her home. She did very well.

Friday, June 19, 2009

Hi CY - ready to go

Yesterday Kris picked up Haven's wheel chair. Thank you to everyone that donated to her - we have been able to load up on her supplies and get her the wheel chair. So very much appreciated. This will be extremely helpful at the hospital with wheeling her back and forth. That hospital is a city and it's huge.

Haven wasn't happy that it wasn't pink and cool - so she and Kris are going out this weekend to get fabric and pink things to make it cool.

Today we spent the day at Johns Hopkins. We arrived at Oncology at 7am to ensure we were there in time to get the medicine and take her blood tests. We didn't know how much blood they were taking and Haven wasn't allowed to eat until after her CT Scan. This was the first of 3 rounds of blood withdrawl. LOTS and LOTS of tubes. She had to have her blood taken every 30 minutes.

This is Haven finishing up the 3rd round in the play room. By 9:15 we headed down to CT and sat until 11:30 before we got in. Keep in mind Haven had a lot of blood taken in the early morning and couldn't eat or drink anything. She was beginning to look very bad, pale and weak. Then they loaded her up with 6 syringes of contrast for the CT Scan of the head, neck, and abdomen.

We finished CT about 11:50 and headed straight for lunch. Haven ate and drank like crazy. Then it was immediately back to Oncology to talk with Haven's doctor and Child Life.
We reviewed everything about the procedure which I will put up here once I again review all the paperwork and books we received.

Child Life is extremely helpful and it's great to have them there. Not only do the play with the kids in the playroom and their rooms but Kris and I have had a really hard time trying to figure out how to talk to Haven about this. Kim from Child Life sat down with Haven and us to discuss the surgery and putting in the Hickman line. It was so cute. She had a book on the Hickman line that is written for kids. She went through everything with Haven so she would understand and Haven was very happy to know all of it.

I received a book on Bone Marrow Transplants in Children.

Now it was 2:30 and Haven was looking horrible. She had huge dark circles around her eyes, her speach was slurring, and she was wobbly while walking. We were pretty worried and even had her eat some candy - which she never has. Hoping that would put some sugar back in to her system. We got her in the wheel chair and headed to the car. Not even 30 seconds in the car and she was asleep. She was very worn out at home and laid on the couch for a few hours drinking fluids and eating. She is back to normal now and partying.

This is our last weekend home for a very long time so tonight we are partying and watching Haven TV. Tomorrow she will fix up her wheel chair and possibly see a movie. Then Sunday is Father's Day so she and Kris will spend the day together (not like they won't have many of those days coming up).

Thank you to everyone that keeps up on Haven. Thank you to everyone sending her gifts. Workbooks and coloring books are her favorite and she has a few to take to the hospital with her.

Thursday, June 18, 2009

Surgery/Chemo update

Deledo at the Surgeon's waiting room

Yesterday was a hospital packed day. We got down to the surgeon's office around 9am and luckily I like him a lot because we didn't get in to see him until almost 11:30. Child Life came in to discuss with Haven what will be happening during her surgery and why we are doing this surgery. Child Life come to talk to children about their diagnosis and what is going to happen to them in a way that children can understand. Haven knows almost all of the Child Life people now.

After talking with the surgeon we understand a bit more what will be happening. Haven will keep her current port (under her skin) - which is good because she loves that thing. The Hickman line will be placed in her chest on the opposite side. So she will in fact have two ports.

The best route is to get the Hickman line to go up the jugular vein in her neck - as this is the most direct route and safest. The surgeon seemed a bit concerned as to whether or not he will be able to fit it there. If it is unable to go in this way they will have to put it up under her clavical. This runs a higher risk of puncturing the lung - which he said if that happens they will put a tube in her side to release the pressure until the lung heals.

Surgery is scheduled at 7am on June 26th - we are required to be there by 6am. Once surgery is finished she will go in to recovery and then will be admitted in to the Inpatient Pedatric Oncology building. She will then begin her chemotherapy (Hi Cy) that day - beginning her isolation and rebuilding of her body.

Tracker at the surgeon's waiting room

After meeting with the surgeon Kris took Haven over to Cardiology to have their workup done. She had an Echo and an EKG. Then over to Oncology for lab work. He said he was concerned with how much blood they took that he got Haven a wheelchair. She told me it was 10 tubes. She does so well with all of this it's amazing.

After that they met me at TGI Fridays (Haven's favorite place) to have dinner. Haven was allowed to have anything she wanted (within reason of course). She picked Macaroni and Cheese and Broccoli. She never has Mac-n-Cheese so she was super excited.

Tomorrow we will be in Oncology by 7:30am to do more labs and a Cortisol check from Endocrine. At 9am we have a CT scan of the head, chest, and abdomen. After the CT scan we will head back to Oncology to meet with her doctors and sign all the consent forms and review again what the procedure is and all of the risks involved.

Then this is Haven's last weekend at home. My mom flew back in from Korea 2 weeks early last Friday as a surprise. She is going to make Haven a huge Korean dinner this weekend - Bulgogi.....I can't wait!!!! My plan is to do absolutely nothing except maybe take her to a movie of her choice.

Thank you to everyone for constantly inquiring about our baby. She is doing well right now. I know she doesn't fully understand everything and we haven't told her that she's not coming home yet. That will be next week. Right now we are just enjoying every minute we can with her.

Love to all

Monday, June 15, 2009

Beating ROHHADNET....Can It Be Done?

That is what we're hoping for.

Last Thursday, yes it has taken me a few days to get the nerve to write it down, we received the phone call from Haven's Oncologist. We are approved and ready to begin.

WAIT....right....no....we're starting immediately.

This whole thing is extremely terrifying and has us mindless. I believe Kris started calling family on Sunday - I only sent out emails. I haven't been able to call about it yet.

Spoke with Gwenn, Bone Marrow Coordinator, as she coordinates all of the appointments for this therapy. Here is what we have so far:
  • Meet with surgeon on Wednesday to discuss the surgery of removing the Cathoport and putting the Hickman line in.
  • Oncology lab work on Wednesday
  • Cardiology for Echo and EKG on Wednesday

  • Oncology on Friday at 7:30am for lab work
  • CT Scan on Friday of the sinuses
  • Oncology on Friday at 1pm to sign all consent forms

Gwen will email me when the following are scheduled:

  • MRI of abdomen
  • Pulmonary Function Test

Endocrine has requested another Cortisol test be done before surgery. This will be done at 8am on Friday this week. It has to be at 8am for the correct results.

We will find out the date of the surgery on Wednesday. Once surgery is complete Haven will begin chemotherapy immediately.

Chemotherapy will begin before the end of this month. This gives us 2 weeks to figure everything out and have multiple doctor appointments and surgery again.

By July 1st Haven will be in isolation (if all appointments go as planned). She will remain in isolation for a few months.

Tuesday, June 9, 2009

Haven Pink Toes

Haven's surgery went very well on her toes. She made it through with no breathing issues and no crazy reactions to the anesthesia. We met with the anesthesiologist for a long time on Monday before the surgery to review everything about Haven. She had done some reading on Haven - said there was a pretty good novel of medical paperwork. We discussed the possibility of us going home and at the time she said it had been requested that Haven be admitted for 2 days after surgery to be monitored.

Talking with her was very easy and we asked since the surgery was not going to be that long if we could just do the "mask" instead of anesthesia through the port. She agreed but would have the port ready to be accessed if necessary. Haven did so well, they didn't have to access the port and no intubation. She came out of surgery with little effects from the anesthesia and was ready to go within 2 hours. So we were released! NO OVERNIGHT STAY!!! YEAH!!!

We have battled a few fevers over the past couple of days since the surgery but she keeps breaking them. Last night we unwrapped the toes - she was not happy but didn't freak out like I thought she would. The biggest thing is she has serious OCD of the feet. She cleans them constantly so to have them bloody and yucky drove her insane. We wanted to let them air out before putting peroxide on them. So for about 2 hours she babied them telling them not to worry that she would clean them soon.

We had her walk a bit last night and the toes didn't seem to bother her. If I had to pick a positive about ROHHAD - I would say the best thing we've gotten out of this disease is the decreased sensitivity to pain. What would probably make me cry like a baby doesn't even bother her.

Thank you to everyone for checking up on her and wishing her well. She is as usual awesome about her doctors and medications. We are enjoying the lack of at home medications right now for a few more weeks. We only have to take Amlodipine and Bactrim right now and it's very nice.

We'll be meeting up with Oncology after a 2 week recovery to review what we need to do next!

Love to all

Sunday, June 7, 2009

Princess Haven

We've had a great weekend. I wanted to get Haven out this weekend and the weather has been perfect. She has surgery tomorrow morning at 9am - we have to be there at 7am. Since everything is about to start I wanted to get some pictures taken of her. She picked her beautiful princess dress she got for her birthday to wear. Here are a few sneak peaks. We took quite a few but thought I'd share a some.

Don't worry family - you'll get your photos in the mail! :)

She had a lot of fun. The photo people knew we were coming in to take them before chemo starts up and they really took their time with her. They also gave her what ever photo she wanted in any size for free. So she picked her favorite.
We should have the photos on June 17th unless they come in sooner!

Thursday, June 4, 2009

The Plan

Well...I believe about 15% of this plan has finally sunk in to our heads. We started gutting out the house preparing for the next steps. Our meeting with Oncology was comforting and scary as hell, as usual! I do have to say this is the first time I have ever felt confident in Haven's doctors where I am willing to hand my child's life in to their medicines. I know all of these doctors care for Haven and have her best interest at heart. They are really the best doctors I could ever hope for in a unknown and uncharted territory.

It took a long time to believe our daughter has cancer....then it took even longer to believe she has a rare syndrome called ROHHAD - that ONLY 30 or so kids worldwide are diagnosed with. You can't imagine how lost you feel when your child is diagnosed with a disease that has no information and no treatment and most definitely no cure. And somehow through almost a year of reasearching and scouring the internet I have found other children who have ROHHAD and have become friends with their mothers. Such a rare disease and to actually find one is amazing. I know quite a few!

For months I fought with myself and argued with doctors about Haven's diagnosis....that there was no way she had this. I dug and dug through every forum on the internet, every article, compared notes with parents, and even broke down Haven's symptoms one by one in comparison only to find the awful truth that my daughter does in fact have ROHHAD Syndrome.

Over the past 1 1/2 years Haven has been on different chemotherapies testing how the ROHHAD reacts to the different types and different levels of immune suppression. Luckily, Haven has taken on all of these challenges with a smile (mostly) and handled all of the doctor appointments amazingly. She has probably been to over 150 doctor appointments in the past 1 1/2 years - and that might even be a low number. Not counting multiple hospital stays, MRIs, and surgeries.

What does all of this lead up to?

We have been testing to get a closer idea as to what ROHHAD could possibly be. Is it autoimmune or is it something else? We don't have a definite answer but with all the testing we're pretty pretty sure it's autoimmune. I believe Haven has an extremely high immune system that is very strong. My child has never been sick in her life - never had a cold - not even once. She is 5 1/2 years old. If it weren't for cancer and ROHHAD she'd be abnormally too healthy. Not even a runny nose!

Tomorrow Haven will receive a full dose of IVIG - which usually takes an entire day to administer.

Monday Haven will have surgery to remove her toe nails - they have been infected for over a year. I wouldn't have pushed this surgery and we would've continued soaking them and lotioning them if it weren't for the ultimate decision we had to make.

After the surgery on her toes Haven will have a couple of weeks to recover. Once recovered she will undergo a few more tests with Oncology and Pulmonary to prepare her for the next surgery. Removing her Portacath and inserting the Hickman Line.

Right now Haven has a Portacath (above) inserted in her chest that has a tube up her jugular vein and into the vein in her chest. She has a pretty large one due to the fatty tissue around her chest. She loves this thing. She thinks everyone should have one. She never has to get pokes or anything. I can't even tell her she is having this removed...I just can't.

Above picture shows how her Port is sewn in to her chest at the muscle. This is hidden under the skin - it can be felt but not seen (other than the 2 inch scar across her chest)

It involves two incisions, one at the jugular vein and one on the chest wall. The central line (the long white one) is inserted (tunnelled) under the skin of your chest into a vein. The tip of the tube sits in a large vein just above your heart.

The reason we need to change out her Port is because she will need multiple lines for multiple medications to be administered at the same time.

Once Haven recovers from this surgery she will undergo more testing from her doctors to ensure she is ready for the next step. Once again cleared from this surgery and testing Haven will begin her new chemo treatment.

This is the big stuff....this has been the part we've been scared of but knew may be coming. We didn't know it would come so soon but the doctors have enough information and are confident with their findings that we will now begin this year long treatment.

Haven will receive 4 hard doses of chemotherapy. She will be admitted in to the inpatient Oncology wing of the hospital. We have read and been told by our doctors all of the risks and we understand the main ones they want us to know about. The doctors are always truthful and tell us everything.

The main risks of this treatment are:
  • 2nd rate Leukemia - which is fatal
  • high risk infertility
  • extreme risk of infection (toe nail removal necessary)
  • death

There are a few things we are guaranteed to happen and the doctors wanted us to know so we wouldn't freak out when it does happen:

  • hair will fall out
  • ulcers ranging from her lips down through her stomach
  • very sick and weak

Once Haven begins this new chemo she will remain in isolation for a few months as her body tries to rebuild. They will be bringing all of her counts down to 0. Once she is stable and can move from the hospital we will be put in temporary housing for a few months to ensure she is in a clean and sanitized environment. She will have nursing care and availability to doctors to make sure nothing goes wrong and that she doesn't get an infection. Any infection could kill her in these first few months. During this time we will not be able to have visitors in to see Haven. I do not want anything complicating her recovery. I have been reassured by our doctors that they have not lost a patient due to the treatment and don't plan to start now. This does make me feel good and I know they're not just saying it to make me happy. I feel the sincerity from our doctors and that's nice.

This treatment takes about a full year before Haven's immune system is back up in working order. I will ask that at the end of June we will not be able to give Haven any stuffed animals. She receives and loves the gifts from around the world and stuffed animals are her main pleasure in life. Taking these from her is going to be devastating. But it is a necessity. This weekend we will pack up her "pink" room and put all stuffed animals in large bags and put them in the basement. She will not be able to sleep with them or play with them.

The ultimate goal: Her new immune system will forget that it was attacking her body and instead start helping her body. They do not expect to reverse anything that has already happened but we've seen it reverse before. They only hope to stop it from progressing.

We ask that you please think of Haven during this rough time. She has this weekend to enjoy before everything starts. I plan to get her photos done one more time before we start. We are nervous, scared, and emotionally drained. We have no idea how we're going to handle this or how we're going to handle seeing her like this. But the good chance that this could help her and give her a normal life will bring us strength.

thank you to everyone who has followed along with Haven over the past 1 1/2 years. Your dedication to learning about our daughter and these rare diseases is definitely keeping me going.

All our love to our family and friends for understanding the position we are in and supporting us in our decision to move forward with this treatment. We appreciate all you have done for us and Haven and what you continue to do. When she is ready we'll be planning a large party and hope that all family members can come out.

Take care - and wish miss Haven many blessings through these next few surgeries and few months of chemotherapy.

Tuesday, June 2, 2009

Next surgery


From our meeting with Oncology last week we have moved forward with Haven's foot surgery. I called the Podiatrist yesterday and discussed Haven's needs and the urgency of this surgery. They contacted Haven's doctors and we are set for surgery on Monday June 8th. We're actually on stand-by right now - I'm waiting for them to call back with a time. All of the pediatric beds were full so they were going to move people around.

Haven's toes were so infected this weekend that a couple of hours at the mall caused them to crack open and bleed for the past 3 days.

Haven will be in Oncology this Friday to receive IVIG. This takes a full day to administer. Her dad will be with her and I'm sure all will be quiet because I won't be there bugging anyone! :)

I will update her site with the next plan of treatment soon. We're still trying to let it sink in a bit.

We are hoping they are able to get our last anesthesiologist, Dr. Rich Elliott, that did her eye surgery. He was really good and he's the one that called and talked to me prior to the surgery and then he followed up with Oncology. He was very good.

Love to all

Monday, June 1, 2009

Prayers and Thoughts for Mason

Hi Everyone

I am asking if everyone could please send some thoughts and prayers towards Mason. He is still in the hospital in isolation. There is no indications of when he can return home. He is still very sick and is now unable to keep his oxygen levels up even with the extra oxygen.

His web site is: http://www.caringbridge.com/visit/masonbyrom or you can click HERE.

His father refuses to go home from the hospital until Mason is with him and his mom is at the hospital as much as she can while also caring for their other child Cameron - who can not visit Mason in isolation.

I appreciate all of our family who visits his site and Aunt Dottie for always leaving little notes on his guestbook.

Lots of love to the Byrom family from us. :)