mIBG is done!!!

Haven did a great job today at her mIBG. She is so loved by all her doctors. She met with the nurse you see in the first picture and they let her pick out her smell for the mask. They put chapstick in it so it has a good smell. She picked chocolate.





This is her getting her stickies on so they could monitor the heart rate.










They even let Haven gas herself. It was so cute. Her arms started getting weak and she could barely hold the mask on her face.





After a couple seconds the nurse took over and gave her a good hold on the mask. She was out quick. We gave her a bunch of kisses and left her to her testing.







Two hours later we were in recovery and she was doing great. All she ever wants to do is get the IVs out. They were able to put the IV in on the first try.



This test was needed before surgery. It will show the doctors exactly where all the cancer is in her body. Plus what we like is that the radiation starts working on the tumor before surgery. She was awake so easy out of this one and ready to go that they let her leave after only 40 minutes in recovery. She was an absolute angel.

Surgery Confirmed

Surgery is confirmed for Thursday, January 31st. Pre-Op will be at 10am. The guess right now is that she will be back home on Sunday sometime.

Today

We did the radiation injection today. There was a bit of confusion on her paperwork and they were concerned about her BP medicine with the radiation. But it was all approved by her Oncologist and the injection proceeded. Took a while to get a vein - the first arm was not a success. The second arm I practically laid on top of Haven and Kris and the other doctor had to hold on to her arm so tight she will probably have handprint bruising. They even warned us of that because of how hard they had to hold her.

So tomorrow we will get the scan - she must be there early to meet with the anesthesiologists before they give her the anesthesia. She will be gased again before they try to put an IV in.

Anyway - she did a great job and we were super proud of her!

When is surgery?

Well Kris has been on the phone with doctors most of today - I have only talked to one. Seems as tho there is a rumor that surgery will be next Thursday January 31st. There is still more testing that they want to do before surgery. Again her doctor reminded us that she has an extremely rare case of this type of cancer so they want a lot of before and after tests to observe her.

During surgery they will be performing the following:

Removal of tumor - as best they can
Hickman Line - tube inserted for chemo
Bone marrow extract
Spinal Tap

We are not confirmed on surgery nor do we have a time. Just a rumor right now.

Some good news!

Well we finally got to hear something good. Even tho I'm still having an extremely hard time believing it. But Haven is down to 53 pounds from 61 pounds. We're hoping this is a sign that maybe the tumor has slowed down in production of the weight hormone which could mean it's growth is slowing. I don't know. But we're pretty excited about it.

Our secret: Snack time is sugar free jello with only 10 calories! :)

Meet with Neurology - Kennedy Krieger Center

Well another day sitting in a doctors office for 5 wonderful white wall filled hours! This appointment was set up by Haven's Oncologist so it wasn't something we requested. Anyway, they were testing her for development skills and we discussed the seizures and sleep walking episodes. Really what they are checking her for was not on our priority list. A few things we want to check in to but we will not be pushing them at this time.

We did receive a prescription for some medication for seizures - in case she is to have another one. She did test high on most of the tests but a lot of them she refused to talk to the doctors but would answer when I asked her. Instead of repeating the same sentence back to the doctor she'd correct the doctor.

Doctor would say: "Dad went to the store"
Haven would say: "No, my mom went to the store"

or they'd say: Mom did something and she'd correct them and say Dad did it not Mom.

The Oncologist have taken Haven off her blood pressure medicine to do the radiation testing over the next two days. And in 2 days her BP has gone from 106/64 to 126/78. So Kris is at the doctor with Haven now to figure out what we can do. They will not administer anesthesia if her BP is too high and by Friday it may be too high. Plus she has surgery the week of Feb. 4th so we're worried if they don't put her back on she won't be able to have surgery.

Will update once I have more information. :)

Schedule for next week

Tuesday: Neurology appointment 12pm
Wednesday: Blood pressure check at pediatrician
Thursday: Radiation injection
Friday: mIBG scan and meet with Oncology

So we definitely have a full week ahead of us. We have to get her prescription for potassium iodine drops that she will take 2 times a day for 1 week - due to radiation scan. Meeting with Neurology and probably another MRI scheduled on February 4th. They want to discuss the behavior, weight gain, seizures, sleep episodes - so that will be a whole day.

Oncology says we have to get her off the blood pressure medicine before the mIBG scan. So we're cutting her back over the next 5 days down to nothing by the 23rd. So this is why the blood pressure check.

Haven has been a bit sick and achy the past few days. She's been sleeping a lot. We've been sleeping on the big heating pad - it helps her bones and aches.

We start the 3rd round of Cushings/Paraneoplastic testing tonight. This will be 3 nights at 11pm and two mornings at 8am of chewing on the cotton thingy. She's used to that so she's a pro. I'm sure when she wakes up she'll be excited to know I was able to find the tubes again. They are very hard to find and take a few days to finally locate a place that has them. Kris picked them up this morning when our attempt to last night in the SNOW failed.

Those are my updates for now. I have been on the phone with doctors since about 3pm and it's now 5pm. Talk to everyone soon!
Love ya
M

Meeting with the Surgeon

We met with the surgeon that will be taking care of Haven in a couple of weeks today. It was a long process/meeting. First we waited for 2 1/2 hours to see him - after they placed us in the fluorescent lit WHITE room. After the first hour I believe we all started going slightly insane....especially all locked in a room together. I started wandering the halls looking at posters of The Hulk and Wonder Woman, Haven kept clicking a pen that drove me crazy and Kris kept telling us both to calm down.

I personally have to say one thing that is really irritating to me is that every nurse we talk to always asks us why were in. When they start the conversation off like that I instantly get upset. If I'm coming in to a cancer department or a surgical building I believe you should know why I'm there or get someone who has read my file. Don't make me go through the entire 2 year history each time.

So the surgeon finally came in to review the MRI and report and what the surgery will entail. We went through EVERTHING.....it was a long meeting. We did get to see the actual tumor and it was shocking to us and the surgeon could only say that it will be "interesting".

If you can picture it: The tumor is shaped like a kidney bean (which is extremely rare) and is actually "glued" on to the spine and cradling the Aorta. The Vena Cava is wrapped around the tumor on the left side and has actually grown with the tumor. These are the two major arteries that run to and from the heart.

Normally a Neuroblastoma tumor is a ball on one side and generally an easier surgery. Haven's is shaped like a kidney bean and actually wraps around the Aorta 1/2 way. With this the surgeon is unsure he will be able to remove all of the tumor and will not sacrifice anything vital to do so. He will not be able to confirm what he will do in surgery until he can get inside and see it. There are two ways: 1. move the arteries away from the tumor to take it out; or 2. take out as much of the tumor without moving the arteries - leaving a small section behind to be desolved by chemo.

The tumor is on the spine but is confirmed NOT in the spinal column. The nerves are slightly weaved in to the tumor so he will not be able to remove the tumor from those. He will remove as much as possible without touching the nerves.

She is also confirmed to show no signs of Metastasis - a 2nd form of cancer that is sometimes caused by Neuroblastoma.

Her tumor shape is extremely rare - the doctors have already met with two large board members that only work on tumors and none of them have had a Neuroblastoma like this. It will be a tricky surgery.

The incision is to be 5 inches long and cut vertically in the left side of her abdomen. They are unable to go horizontal at bikini line or through the belly button because they need a lot of room to get in there. This tumor produces a hormone that causes weight gain and because of that she has a lot of layers of fatty tissue around her. Thus increasing the difficulty of the surgery and healing process could be slightly longer. They will not have external sutures or staples. Everything will be internal and she will not need to come in to have anything removed. Due to fatty tissue we have a higher risk of infection so we will receive detailed instructions on cleaning and bandaging the wound as he assures us the fat will cause it to have a bit of puss.

As for the tube for her future treatments of chemo and IV's they are still trying to decide if she will have port or the tube. If she has the tube (due to fatty tissue) it will be about 7 inches long. They can either put it in her neck or in her chest - which hasn't been decided yet.

He is guessing that the entire surgery will be about 4 hours (they can't keep her under too long) but I have thoughts that it will actually be longer. mIBG is being done the 24th and 25th of January. Right now it looks like surgery will be right after that. There are a few more tests we must do before surgery and a few more meetings.

As for how Kris and I are doing - we are ok I guess. We're tired and a bit worn out. By the time we get through the doctor meetings we have to get back to work or finish out the day. We don't know if it's day or night sometimes. Haven has been sleeping much better - my parents bought her a new mattress that's really soft and Kris' mom and Simon got her some princess sheets and she's loving them.

Thank you everyone for calling or emailing or texting. We're here --- just sometimes a little busy. Grandma Virginia has been staying with us and will be here through Haven's birthday. She's been a great help to Kris and I and Miss Haven.

Sorry for the book it's been a big day. Love to you all. - M

What is Paraneoplastic Syndrome?

Well that was our question when Haven's endocrinologist called and stated that she may also have this condition. Quickly I googled it to check it out and have somewhat of a description below. We're starting testing on this as soon as I can locate the tubes to do the testing. It's a special set of tubes we need and most places don't carry them. Not to mention we need 5 different tubes for this test. Three will be done at 11pm at night and two will be done at 8am in the morning. This will also check her levels of Cortisol - which they need to know for the surgery. Kris and I do not feel that this is the case as Haven does not express all of the symptoms below - but at least JH is continually researching her symptoms.

Below is a description.

A paraneoplastic syndrome is a disease or symptom that is the consequence of the presence of cancer in the body. These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor.

Organizations

What are Paraneoplastic Syndromes? Paraneoplastic syndromes are a group of rare degenerative disorders that are triggered by a person's immune system response to a neoplasm, or cancerous tumor. Neurologic paraneoplastic syndromes are believed to occur when cancer-fighting antibodies or white blood cells known as T cells mistakenly attack normal cells in the nervous system. These disorders typically affect middle-aged to older persons and are most common in persons with lung, ovarian, lymphatic, or breast cancer. Neurologic symptoms generally develop over a period of days to weeks and usually occur prior to tumor detection, which can complicate diagnosis. These symptoms may include difficulty in walking and/or swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, sensory loss in the limbs, and vertigo. Neurologic paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome, stiff-person syndrome, encephalomyelitis (inflammation of the brain and spinal cord), myasthenia gravis, cerebellar degeneration, limbic and/or brainstem encephalitis, neuromyotonia, and opsoclonus (involving eye movement) and sensory neuropathy.

Is there any treatment?

The cancer is treated first, followed by efforts to decrease the autoimmune response - either through steroids such as cortisone or prednisone, high-dose intravenous immunoglobulin, or irradiation. Plasmapheresis, a process that cleanses antibodies from the blood, may ease symptoms in patients with paraneoplastic disorders that affect the peripheral nervous system. Speech and physical therapy may help patients regain some functions.

What is the prognosis?

There are no cures for paraneoplastic syndromes and resulting progressive neurological damage. Generally the presence of cancer determines the diagnosis.

What research is being done?

Research on neurologic paraneoplastic syndromes is aimed at enhancing scientific understanding of them and evaluating new therapeutic interventions. Researchers are seeking to learn what causes the autoimmune response in these disorders. Studies are directed toward developing assays that detect different types of antibodies. Scientists also hope to develop animal models for these diseases, which may be used to determine effective treatment strategies.

Flowers with Mom

Well my 3 wick candle finally burned out and I do not have any more. So while out at Ikea today I decided to go big and spend my $5 on some rocks and twigs. :) Yes, it has lots of curly cues and funky white things and some green fake leaves. Looks beautiful and Haven helped me do it.







Dad got a couple pictures of us working through the mess. We have a lot of fun doing this stuff.

Grandma and Grandpa Visit

This is my beautiful angel. :) We love her. This is actually after Grandma Virginia did her complete make over but I love it so much I put it first. She has make up on and her hair curled. She was having so much fun.

















Make up time














Now time to add the lip stick!




















Cooking with Grandma!















The Starface is born!















Haven helped to set the table this morning for breakfast.















Giving Hercules and Hector their treats. They really liked Haven doing the treats. They receive a LOT of treats.




















The Dancing Duo















Doggies everywhere!

















Family photo before Simon was off on his 6 hour drive back home with the boys. Nice day for a picture.

Building with Dad

That's right. She's bad and she's on the East side now! She got her new shirt and hat from Uncle Brian and Aunt Lisa. Had to immediately put it on. She's so cool!















Dad decided with everyone coming out over the next few months that it would be wise to get a table. Wha Lha! This table seems familiar! We loved it so much the first time we went back for a 2nd one. :)















Haven loves to help out when building with dad!















Almost done.....














She loves to build with her dad. Anything and everything.














What a happy smile!

MRI has been completed!

Picture of Haven after the MRI in the recovery room about to go home.

Well last night Haven was so comfy sleeping on the couch and sleeping so soundly that we decided to let her sleep. I slept on the floor on the huge heating pad from my parents and around 5am she was thumping her way to me and crawled up next to me on the floor. I think it was the best night sleep she's had in a long time. So tonight she has been relaxing on "her" couch again!

Well we have been at the hospital all day (from 10am - 530pm) for Haven's MRI and in the recovery room. She had to have the MRI scan of the entire body so it took longer than a normal MRI - about twice as long. She was checked out by the doctors and nurses before undergoing the anesthesia as we have had many issues with anesthesia before. She is not able to have Verset or Ketamine so they always have to find a third alternative for her.

Due to her veins being so small and hard to find the 2 anesthesiologists decided to use the gas on her to knock her out first. This was a very hard decision and Kris and I did not agree on this at first. I didn't want it because the possibility of her stopping breathing due to the fatty tissue surrounding her neck. But this is the way the doctors chose to go as well. It was hard to see her. We held her hands until she went limp and they made us leave. We were allowed to give her a kiss good bye and told her we loved her and we'd see her soon.

The doctors ultimately had to use a breathing tube in her throat to keep her breathing and any collapse of the muscle could be bad. They did take 3 times to find a vein (which they ended up finding her the top of her hand). She is still bruised pretty bad from the last CT scan and blood work at the Oncology office.

She completed the entire scan without any issues and the doctors were very proud of her. Around 2:30 pm they brought her back out to us - of course we kept bugging them as it was supposed to be over at 1:30 pm. I get nervous when it's even 5 minutes past time. Then we went up to recovery and she had to stay there for a while. They wouldn't let her leave until she was completely conscious of everything and could stand. Her words were still a little slurred.

So finally around 4:15pm she was able to eat something. She hadn't eaten since 7pm the night before. So yes, she's been spoiled all night. As she says - she just wants to "B-lax" which means relax.

She has made plans to call her grandmas and aunts tomorrow when her voice is back. She can't talk right now as her throat is scratched up and hoarse from the tubes. But she is smiling and happy. Daddy rented her whatever movies she wanted and she got a whole box of Otter Pops for tomorrow.

Grandma and Grandpa Virginia come to visit tomorrow and will stay with her for the next few weeks. She is really looking forward to this. Plus the two fuzzy white doggies will be coming so I'm sure her days will be exciting and she won't bug dad about when I'm coming home from work.

Well this is a book - I've finally gotten rid of my migraine by sitting in the dark for 3 hours and having no one talk.

We love you all and thank you everyone for sending cards and text messages and gifts to Haven. She is definitely feeling the love from the world.

Update on Haven

I spoke with Haven's doctor tonight (Dr. Paz). They have finally been able to schedule her mIBG testing. This will be done the 23rd - 25th.

Now surgery will be scheduled the following week of January 28th. After we do the MRI on Thursday the surgeon and doctor will call. They want to make sure they will not need any other surgeon specialists. As the doctor said, and he's only saying it not confirming it, but if the tumor has made it's way to the brain they will need a neurosurgeon as well.

I will also be receiving a phone call from the Pulmonary doctors to set up Haven's sleep study. I informed them that I didn't want this to delay surgery - sleep is last on my list of priorities. But they believe it's very important to analyze her Hypothalamus functions.

Her doctor met with a lot of doctors (via phone/video conference) yesterday to review Haven's case. All of them have worked with Neuroblastoma but none of them have had a case like Haven's. Doctor again told me how rare her case is and he hasn't found someone who has worked with a case of this cancer causing all the other diseases. My guess is it's another way for Haven to be the only one doing or having something.....she likes to be in a class of her own! :)

Anyway, that's what I have so far or I'm forgetting something. I have to write everything down to remember it.

Love you all

Meet with Surgeon

I have been on the phone with doctors all morning. Our appointment with her surgeon is at 9:30am on Wednesday. We will be meeting to review the procedure and everything that needs to be done prior to the surgeries.

I also talked to her Oncologist and he has received a few of the tests back from last week. Thyroid test came back normal. Electrolytes in the blood came back normal. Prolactin came back high. Which they said they would expect as it is caused by Cushings and Hypothalamic Dysfunction. Prolactin becomes high when the Hypothalamus can not produce Dopamine - which causes your body to remain in a stressed state due to dopamine not being produced to calm you down.

More blood tests should be in today and the results of the latest Cushings test. All doctors will be meeting to review the tests and will get back to us with their findings.

Thursday we will be at JH all day. We need to arrive by 10am and the test will go through until around 3:30 pm. We will not have any news from these tests until later in the week or next Monday.

Haven is home with her dad and I'm sure driving him insane! :) It's her specialty. She is a non-stop talker and needs 100% attention at all times. You can only hope to be saved as she will pass out in the middle of a sentence....then you can take a breath! The more tired she gets the more she talks.

She woke up at 1:30 am last night and wanted to color. Both Kris and I were still up so I let her stay up until 2am. At 3:00 am she was still watching Incredibles in her room and then at 5:30 am I heard her slam her door shut. She doesn't like sleeping in a bed and was making her bed on the floor...her bed was too hard (not sure how the floor is softer). But I convinced her to come to our room.

Update from the medical world

Thursday January 3rd we picked up more test tubes to continue the work on the Cushings disease as we have been told her Cortisol levels were a bit high. This is a 3 day saliva test that must be performed at a specific time each day and must have a certain amount of saliva.

Friday January 4th we arrived at Johns Hopkins to have a meeting with our new team of doctors at the Children's Cancer Ward. We are very happy with JH and couldn't ask for a better hospital or team of doctors and nurses. We were there from 1pm until 7:3o pm reviewing everything. Haven did two pee tests and had 8 viles of blood taken - to perform more testing on other types of possible diseases and to see what here levels were at. In a somewhat short version here is what we know for now...mind you there is a bigger list of things that will be done but the doctor didn't want to overwhelm us! (RIGHT)

1. Very rare form of Neuroblastoma (so rare we were pushed hard to sign a consent PACKET to have her tumor tested for research and to be published in medical journals). This includes the rare symptoms of high blood pressure and high heart rate.

2. She has 3 diseases currently caused by the cancer: Neuroblastoma tumor, Cushings Disease, Hypothalamic Dysfunction -- I have links to all of these at the side of the blog.

3. Tumor is confirmed located on the spine. It is behind the stomach - between the kidneys - on the spine. It is bigger than they originally told me on the phone - as he didn't want me freaking out at home. He wanted to review it with us in person and so we could see it.

4. She is confirmed that at her level of cancer she is at least in the medium risk category.

5. Surgery will take place in the next few weeks - she will have 3 surgeries at the same time. Main surgery is to remove the tumor for testing. 2nd will be to extract bone marrow from two different places (each side of the back of her pelvis). 3rd will be to implant a permanent tube in to the main artery of the heart - this will remain there for a long time. The tube will stick out from her chest and will be where they will take blood, insert IVs, and perform Chemo treatments.

6. She is confirmed to have Chemo after surgery and it will most likely be for about 6 months - with the way the doctor sounded. As the Chemo is need to kill the cancer and help in the reconstruction of the Hypothalamus.

Our schedule:

Although most of it is not set in stone it is general in:

Wednesday Jan. 9th meet with the surgeon to review the procedure of the surgeries and how long it should take if all goes to plan and her recovery and what she will need during that recovery period.

Thursday Jan. 10th we have the first MRI - this will be to scan all of her body with a focus on the spinal column. This is to confirm where the tumor is, how surgery should be done, and if it has branched in to the spinal column.

We will not know the exact date of the surgery until the mIBG is scheduled. This test is extremely important as it is a specific test for this type of cancer only. Therefore, it is not performed that often so scheduling it with an anesthesiologist is rather difficult. mIBG is a radiation treatment scan. They will inject radiation in to her veins that must go through her system for 24 hours. Then for 3 days straight she will be scanned by a specific machine for 2 hours at a time. This radiation will attach itself to anything cancerous in her body. Allowing the doctors to know how much cancer there is and where it's at. They will not remove the tumor before having this scan as it makes finding all the cancer extremely difficult.

Bone marrow will be sent off for testing and research as well. This will tell us how she got the cancer, what other cancers she is capable of having later in life, and where it has affected the body. We have already signed the consent form for this.

As we find out more I will keep everyone up to date. We should have the results by Monday on the latest round of Cushings. Blood tests should be back Monday from Friday's extraction. We will know more throughout the week and the next few weeks.

Kris' family will be flying in and driving in over the next few weeks to be with Haven and to help us out. We're very appreciative of them and look forward to the help. Haven is excited about all her grandparents visiting and has already designated where they will all sleep. She is in good spirits but wants to know everything we're talking about and everything that's going on. She's way too smart. We discuss pokes and doctors and she likes to know all of their names and will describe them by their clothing and hair.

My parents live near us (pretty close actually) and will be here for us and her. It's a nice comforting feeling. My grandma and Aunt Candy are due to come out after surgery to see her. My dad is going to go get them.

Love to you all - talk soon!

Christmas in Maryland!

Me with Haven and Baby Stryder


















Haven holding her baby cousin















Mommy (Katie) and Stryder







































Father and Daughter!


























Dusty and Mike















Our Angel









































































Uncle Kris and Baby Stryder



































































































Opening all the presents!












Grandpa B and Baby Stryder


















Kendall as excited as ever!













Dusty (sister) and Mike














































Grandma playing puzzles with Haven. She can do puzzles for hours!









Grandma and Grandpa B got Haven a bike for Christmas. She loves it. Had her first crash and got up laughing. Dad was laying in the grass trying to catch her. She got back on the bike immediately.