Make A Child Smile - SOPHIA

This is our beautiful friend Sophia. We met Sophia shortly after Haven was diagnosed with ROHHAD and Ganglioneuroblastoma Cancer.

Sophia also has ROHHAD. She is supported by numerous medications each day and can barely make it in to school 2 - 3 days a week with all the doctor appointments she has.

Her mother, Maureen, has helped us out so much with Haven and my sanity that I want to help her daughter experience the joy Haven has had from Make A Child Smile. It's an awesome organization.

PLEASE SEND SOPHIA A NOTE TO LET HER KNOW YOU HEAR HER STORY AND YOU ARE THINKING AND PRAYING FOR HER.

She is truly an angel.

To get Sophia's story and address you can click her picture, click the Make A Child Smile link, or CLICK HERE!





We love you Sophia!!!

Tape allergies

Texting Grandpa Beard-off in Utah.


TAPE TAPE TAPE! We've learned over the years of chemo treatments and taping tubes to Haven that she is allergic to tape. We always have the special tape at sleep study and have never had this issue.....but she is swollen and covered in itchy bumps.

With ROHHAD she shouldn't have Benadryl before bed....so we got Benadryl lotion to put on that won't make her sleepy.

Comparing where the leads were and the tape we know this was the issue.

Hopefully with the lotion it will go away soon.

Sleep Study 11-26-10

The day after Thanksgiving Miss Haven was scheduled to go in to sleep study....which means a lot of driving for mom while dad stays up all night watching her. It's a 2 hour round trip drive and I do it twice each study - they have to be there at 8:30pm and have to be picked up at 5:30am.

To get ready for the study - if you've never had one - is about a 1 hour and 20 minute prep.

First they pretty much scrub her head, face, chest, and legs with this gritty stuff (see photo). It smells kind of like toothpaste but is very sandy. Then the chest and stomach straps are placed around her with the monitors hooked to that. Then she has both toes wired up for heart rate and oxygen levels. Once those are all in place and working they can start with the wires.

Here you can see Haven's chest strap and face wires. The wires are stuck to her with a glue and then taped on. Only problem - Haven is allergic to tape! Doesn't matter what tape it is...she is allergic to it.

After they wire up her face they wire up the head. The glue is horrible and I'm sure not at all comfortable to sleep with. I can't imagine if my child was not cooperative......say like some of my nieces and nephews!!! I can't imagine them sitting still for 1 1/2 hours while getting wires glued to them and then having to sleep with them on. Luckily my daughter is better than I would be at this.

As the night wears on you can see the happy child! This shows both straps and all the wires so far hooked up behind her. Haven did have extra wires this time due to the monitoring for seizure activity.

Finally the gluing has stopped and she is wrapped up and ready to lay down.

This is a sensor they put on her throat to monitor the snoring. Haven has had some pretty extreme snoring the past couple of months and even causing us to miss some sleep lately.

All of her colorful wires. They always let Haven pick which colors she wants to put on first.

And it's night night time


We are hoping for some good results from this study. This was the most Haven has ever slept at a study - she usually only sleeps 1 - 2 hours....this time she slept the entire time. She was also snoring with in 2 minutes of falling asleep and that stayed constant all night. Luckily they also video tape her during the sleep study as she was a crazy sleep walker (glad they have bars on the beds to keep her in). She was screaming so much and so loud the technician kept coming in asking if Haven was ok. Kris kept telling her yes that this is exactly what we've been waiting to show the doctors.

Saturday after we got home I gave Haven a long soaking shower to remove all the glue and tape residue but it didn't help. Still two days later she is blistered from the tape and has a bad rash on her face.

We won't have results for at least 2 weeks but am hopeful they can get to us sooner - we just have to wait for Neurology to look over her results first. Over this weekend she had us very concerned as each breath while sleeping also caused her chest to sink in pretty deep. We checked on her about every hour.

Thanksgiving

We had a very nice Thanksgiving day at Grandma and Grandpa's house. Grandma made ham and turkey and lots and lots of sides. Haven and I made green bean casserole. She loves to cook and it's been a while.

Adding the finishing touches.

Ready for dinner


Playing her game on Facebook and waiting for a text.

Holiday Photos

Well it seems we can't ever get everyone together for a photo but Haven loves to get her photos done. She is so awesome too....I really put her to the test on these. The woman that took the photos at sears had no idea what poses to do so I took over and did what I wanted. Haven did a few too.

These are some of what we didn't buy but wanted to share.

Miss Haven wanted to do this one....she loves the photos where she doesn't look at the camera...so dramatic!

Always need a good up close photo


I thought we'd like this background but I wasn't a fan of the glare on the ground so we chose not to get this one.

I always love these...black background and Haven looking up


We're excited for the holidays this year.

Oncology November 15th Visit

Miss Haven went in to Oncology for her 6 week visit on November 15th. We were told at her last visit on October 4th she would need IVIG. So we made a plan to be there the entire day - this is usually a 6 - 7 hour dose for Haven. However, Haven's nurse Katie (above photo) called and said that Haven's IgG level tested at 500 (normal is between 700 - 1400) and that her doctor wanted to skip IVIG this time and see how her body handles itself and see if it can boost up on it's own. So Miss Haven did her labs and was out within a few hours.

Her next Oncology appointment is in January. To explain a bit of IVIG -

Intravenous immunoglobulin

IVIG is: a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months.

IVIG is used mostly for the following diseases:

• Allogeneic bone marrow transplant
• Chronic lymphocytic leukemia
• Idiopathic thrombocytopenic purpura
• Pediatric HIV
• Primary immunodeficiencies
• Kawasaki disease
• Chronic inflammatory demyelinating polyneuropathy (CIDP). Only the "Gamunex" brand manufactured by Talecris is approved for CIDP (in 2008), under the U.S. Orphan Drug law provisions
• Kidney transplant with a high antibody recipient or with an ABO incompatible donor

However, it is in Phase 3 testing of Alzheimer's Disease but can be used in these and others as well:

• Autism
• C. Diff
• Guillain-Barré syndrome
• Muscular Dystrophy
• Stiff person syndrome
• Opsoclonus Myoclonus

Haven really has never had any side affects from IVIG except she does sleep the entire day it is received - but back to normal the next.

Pulmonary November 4 visit

Last Thursday was my turn to take Haven in to her doctor appt. These things always run about 2 - 2 1/2 hours....plus drive makes it a full day! We left at 9:30am and got back around 2pm!

We met with the student doctor first - they always do this at Hopkins. I understand the need to teach but I hate repeating every aspect of this horrible syndrome! However, this time was different - may be it's because I've walked out of doctor's offices before because they didn't even take a minute to look at her file...but this student knew about 75% of ROHHAD and all of Haven's symptoms. I was very impressed!

After getting a thorough check up we met with her regular Pulmonary doctor and discussed her sleep walking habits and a few new issues that have popped up. Well not that sleep walking for her is new (she's been doing it for almost 4 years) but it is no longer at 11pm start. It starts between 1 - 4 am now and sometimes last for over an hour at a time. Even as I write this - last night (november 8th) she was going ALL NIGHT! There was a break here and there but it was pretty much non-stop. Even when I left for work at 6:15 this morning she was still going.

This is where the doctor is concerned on the sleep walking. Normal sleep walking should happen around 3 or so hours after falling asleep. With Haven's starting 5 hours after or not even starting until 5 or 6 am it raises some concerns. His main concern is seizure activity. He is concerned that it is possible that there is some seizure activity happening in her sleep causing the sleep walking to occur at these times. He made sure we have her emergency seizure medicines and what we need to do. But Haven hasn't had a seizure since 2007.

Then there is a new development in her breathing while sleeping......the past 3 months she has begin to snore. It started off small...random snores. I tried making the house colder, hotter, sleeping sitting up and changing her pillow. Now she is snoring every night and practically all night long. Even when taking naps on the couch she is snoring.

So with both of these issues happening around the same time the doctor is pushing her sleep study up to get us in quicker. There is usually a 3 month waiting period. He will be involving Neurology on this sleep study and adding an extended EEG to the test. So it will take a bit longer to get the results back as Neurology will need to read their section. If she is showing seizure activity she will start seeing a Neurologist regularly....but let's just hope that's not the case.

Haven's last sleep study showed tremendous improvement in her CO2 and sleep habits. She had dropped from a CO2 level of 50 to a 45 and wasn't really going crazy in her sleep. So once this is scheduled and done I will feel much better.

Next up in regards to doctors for Haven is Oncology on November 15th - she is due for another round of IVIG and then hopefully a quiet holiday!

Halloween

I was very excited this year that Haven chose NOT to wear a princess costume! Hopefully this means we won't have a spongebob birthday cake either!

This year she decided to go as a Rag Doll.......It was such a comfortable costume and it didn't rain!

Haven had decided before we left that she was going to get more candy this year than she has ever had. At the end of 40 minutes of straight walking house to house she had a completely full bucket that she couldn't carry. She looked at me and her grandpa and said "I'm over it....let's go home!"

When we got home we found Deezy:

dressed up in her pumpkin PJs!


Haven separated the candy for the Great Pumpkin to visit and left him some cookies as well....in return the Great Pumpkin traded the candy for $25.00!!!!

Pennsylvania Renaissance Faire

Just throwing this in there...this house is only about 5 miles from us...I really want it!

More scenic driving on our way to Pennsylvania for the day.

1 1/2 hours later we arrive!

Miss Haven wanted to take these photos....she loved all the pumpkins and the colors of Fall.

I love my girl!

The PA Ren Faire was definitely different than Maryland but then it was the same as well. I loved actually going there when they are ready for Halloween - they even did trick or treating.

Haven was very excited to donate money to the Royal Hound.

Learning the crossbow! She actually did a very good job - 3 out of 5 cans were hit.

The blue eyes get me every time!

We really had fun visiting a new Ren Faire....however, all in all......I think we agreed that we liked the MD faire better (except for the bathrooms!)

Pumpkin Time

This year instead since Haven was finally home for October and able to get outside we decided to go to a local farm- Applewood Farm - for our pumpkin instead of me just going to Walmart really quick to get a pumpkin.

Yea west coast is nice for it's weather but it has nothing on the Fall season of the east coast!


Miss Haven at the petting zoo at the farm.

She led us through a huge corn maze on the farm. She is very good at directions!

Well.....except for this dead end! :)


Finally found the end of the maze!

Miss Haven scouted around to find the best pumpkin and ended up bringing home a huge 25 pounder!

We are also heading back to pick out our Christmas tree at this farm....this will be our first real tree and my first time cutting down our own tree!

Endocrine Visit - October 11th

Well...I'm a bit behind but we've been very busy I assure you!

Miss Haven went in for her Endocrine visit on October 11th. I had already emailed her doctor that I was concerned about her height but for the first time in almost 5 years that was my ONLY concern at this point.

He checked her over and went through all her records to compare height, weight, and BMI......

DOCTOR - VERY HAPPY

He is very happy with how Haven is doing and where she is right now. She is still growing just slowly and her BMI has gone down a lot since he saw her 7 months ago. He was very happy with how her personality has changed.

We will still continue to monitor all of her levels and see her doctor every six months but this was a great visit!!!!

WHEW!!!!!

YES...it's been an insane month! I have lots to write about and hopefully will be able to stay awake tonight to update about the entire month. Haven has been busy with school, we've had lots of doctor appts this past 33 days, it's been a month and our car still hasn't been fixed by wonderful Geico, my dad has been in and out of the hospital all month with no results! Just been crazy!

So I should be updating all week......