Tapering, Relapses and Chemo, oh my!

We were on track to taper Max's steroids to get him to a manageable level before the first of the year.  However with each taper we have slowly seen some intermittent symptoms breaking through.  A slight tremor here and there, some extra clumsiness, extra fatigue, more sleep disturbances, a little more crankiness, and a little more leaning on things and people to try to support himself.  Obviously symptoms like this in a two year old are to be expected, they were just showing up a little more than we would like to see. We finally got to a place last Monday where we actually dropped Max's evening dose of steroids and then it hit.  By Thursday Josh and I both were able to see the opsoclonus present in his eyes (if you recall last year this was so subtle that it fooled many doctors and Josh was never able to see it), the sleep disturbances were undoubtly worse, he was very clingy and cranky and then by Saturday morning he had 4 hours where he was physically unable to walk no matter how much we tried.  Sunday night he was awakened every 5 minutes crying and by 5am was completely unconsolable and cried until he vomitted, luckily we had a hem/onc PT checkup at the hospital Monday morning and he again was unable to walk, he chose to crawl and scoot on his bottom to accomplish all of the tasks in between the crying. All of our favorite hospital staff could tell this was not their charming little buddy who would run through the halls flirting with everyone. We were around a lot of people this weekend and we ran lots of tests to see if by chance he might have picked something up that might be an indication that a virus may be brewing, but nothing showed up in his blood counts.

The oncology appointment was next and she could clearly see the change from where he was prior to our last taper and said it would make sense that he would relapse at this point in his schedule so we decided to treat him with a pulse dose of steroids (6x the dose he was currently on) and we were able to get him in for an emergency infusion of IVIG yesterday to try to flush out some of the antibodies that are obviously attacking his brain.  If you recall we had a bad reaction in our last infusion and so we worked hard to track down a different brand of the immunoglobulin and we pretreated him with different medications and ran the IV's really slow to try to keep him from having a reaction.  It's still too early to tell if he will have a reaction or not since it usually hits day 3-5 so hopefully we can get through Christmas without any problems and by doing the infusion and the pulse dose it will also buy us 1-2 weeks until we can come up with a new treatment plan.

What about Boston?  Good news is that we got word at 6pm last night that our insurance company's Medical Director approved our request. Bad news is that at 7pm last night we got word that Max may not be in a place to be travelling right now.  So the Harvard Neuro-Immunologist has been in consultation with our team to give recommendations based on the information, charts, and tests, shared with him from us and our providers. However, we are still in the process of working on this.

So what's next?  this relapse makes it clear to everyone that the steroids have been masking the active disease that is still present in Max's body. We are working diligently with many specialists to come up with a new treatment plan, currently the only thing that everyone can agree on is the fact that Max will need to start a new more agressive chemotherapy type of regimen the first of the year.

How can you help?  Pray - We have a lot of coordination, research, decisions, questions, and praying to do in the next 2 weeks.

More to come later.....

In Loving Memory of our Beloved Grandma - Jeanette Kent

 



We are rejoicing that our beloved Grandma Kent has joined her Lord and Saviour on Saturday, December 3, 2011 at the age of 99.  She was a beautiful, humble, and gracious woman.  She was such a talented artist and loving sister, mother, grandmother, great-grandmother and great-great grandmother to us all. She will be greatly missed but we are excited that she is in a better place.

A service celebrating her life will be held at Boulevard Park Church on Tuesday, December 13th, at 3:00pm.

5 Generations

At the age of 99, Jeanette went to be with her Lord on Dec. 3, 2011.  She was born in Spokane, WA on Sept. 25, 1912; the second of four daughters of Milton and Ida Ramsey. Her family moved from Spokane to Seattle where Jeanette and her two sisters, Blanche and Mildred, attended Jefferson Elementary School in West Seattle. She later graduated from West Seattle High School where she met her future husband, Joe Kent.  She attended Edison Technical School (S.C.C.C.) on Capitol Hill in Seattle learning the skills to become a beautician.  She worked at Mary Stone Beauty Salon in downtown Seattle. Eventually, Jeanette and Joe were married and began to raise a family.  Jerry came first, followed by Jean, and later Judy.  Their first home was in Highland Park on Ninth Ave. where they became lifetime friends with Ina, Jim, Carol (who later married Jerry) and Jimmy Sherman. In 1950, Jeanette and Joe built their home on Gatewood Hill in West Seattle.  It was a courageous labor of love for a young couple having no previous building experiences.  Jeanette not only designed the beautiful view home, she pounded and pulled nails, poured cement, and roofed the house, etc. To supplement the family income she worked at Fredrick & Nelson in downtown Seattle for a few years. She was a very loving devoted wife, mother and homemaker while raising her family. Their home was always shared with the extended family as they hosted the much anticipated and appreciated gathering on Christmas Day.

Her faith and church were very important to her.  Jeanette grew up at Seaview Methodist Church in West Seattle where she and her sisters were active members.  They became involved in Christian Endeavor (C.E.) as young adults.  After Joe and Jeanette were married, they worshipped at Westside Presbyterian Church and then moved to Boulevard Park Presbyterian to be with their family.  After Joe’s death, Jeanette became actively involved in weekly ladies bible study (WOW), the monthly circle meetings, and the Clipper Club, each playing an important role in her life where she had close, loving friends.

Jeanette had many creative talents.  She was a superior seamstress and would sew for her children, grandchildren, and church circle.  She could paint ceramics, use a potter’s wheel and draw intricate pen and ink sketches, but Jeanette’s oil paintings were especially enjoyed by all.  She was an avid gardener and loved to keep her garden full of beautiful plants and flowers. In later years, Jeanette and Joe enjoyed their two poodles, as well as, traveling to many countries, cruising to the Caribbean and the Panama Canal, and taking many trips to the Hawaiian Islands. She cared about the people in her life.  Jeanette nurtured her relationships with her extended family, neighbors and friends.   She was a devoted daughter, wife, mother and grandmother. Her love of family was reflected in how she cared for her three children, seven grandchildren, eighteen great-grandchildren, and six great-great grandchildren.  She loved celebrating and attending each and every one of their birthday parties.  Jeanette was a very strong, positive role model for all the family members.  She was a peacemaker and an exceptionally warm and loving person.  She will always be loved and remembered by all of us.

Test Results

We had a nice meeting with our oncologist on Monday to discuss all the results of our recent tests.  She was very pleased with everything she saw.  Max's disease can be very complicated and even after a year of research we are still very overwhelmed by it all.  I am going to try to simplify, but feel free to ask me if it gets too complicated and you have additional questions or the following doesn't make sense:

We have two issues being evaluated - Neuroblastoma (malignant cancer) and his OMS (neurologic autoimmune disease)

1. Neuroblastoma Cancer - From a cancer perspective there wasn't anything to be concerned about. No malignancies or abnormal cells were found in any areas. We will continue to do another scan in 3 months to watch a couple areas and then possibly extend it to 6 months if everything remains the same. Praise God! We are very excited for this news!!

2. Opsoclonus-Myoclonus-Ataxia Syndrome (OMS) - When Max was diagnosed a year ago he tested at a 7-8 on a scale of 10 for the severity of his disease as evaluated by the clinical trial criteria.  He has slowly and steadily received better scores at each checkpoint along the way.  Now that we are approaching the end of the study he is now a 4 on the same scale, which is excellent, we are also very excited that he has made so much progress, however this is still considered a partial response.  Max's doctors were all in agreement today that perhaps now is the time to pusue other treatment options to see if we can try to get a complete response from him.  The problem is now that we are at the end of the study and we don't have many doctors in Seattle who are experienced with coming up with treatment protocols for kids at this stage.  

What next?  So...  we will be traveling to Boston Children's Hospital (Nations's #1 Pediatric Hospital) in the next few weeks to see a Harvard Neuro-immunologist who is having some success in treating children like Max.   There seem to be three other treatment options to consider -  more agressive type of chemotherapy to kill the remaining antibodies (not for cancer),  different types and schedules of steroids, and additional infusion options.

Many have commented on how well he looks and why not sit and wait and see if he gets even better - the problem with this disease is that with even the slightest lingering symptoms may mean he may still have antibodies that may be silently attacking his brain which can cause irreversible brain damage.  The good news is that he isn't showing any deficits in his development at this point which is a really good sign.

We still have some instability and trouble with balance at times, lingering tremors, and sleep and behavior disturbances that causes us to be very cautious, we were referrred to a be apart of a sleep study done in the next month to rule out any other issues that might be causing his sleep disturbances. Many kids like Max use sleep medication and we just don't feel very comfortable medicating him at this point without ruling out everything else and we would hate to mask any symptoms that might clue us in to there being a problem with a build up of those attacking antibodies.

Thank you for all your thoughts and prayers over the last week, we have all had an amazing sense of peace anticipating God to bless us as he has done all year.   We would love your continued prayers for all the pieces to come together so that the planning for the trip and gathering all the records would go smoothly and that we would have a peace about Max's next treatment plan. 

We would also love prayer for all the pieces to come together for our new health insurance. New health insurance you ask?  Yes, we found out the day before Thanksgiving that our health insurance was changing effective December 1st . As you can imagine there is a lot to coordinate when we have such a multi-disciplinary approach to Max's care.  Since we don't have a lot of options at this point we are trying to get all of his current care and doctors covered which has proven to be pretty difficult we also have a few services being reviewed by the insurance company's medical director in order to gain coverage, again the problem is that we are up against an extremely rare condition that has no standard of care so there are no policies over what is and isn't covered for a kid like Max.  We are just trusting that the Lord will work it all out, because other than lots of arguing and a little yelling there isn't much we can do.  =)

Thanks for keeping up with us, hopefully we will have more news soon.

Test Day!

Max got to drink his contrast by bottle so that we could be precise in our measurements. 
Max thought it was fun to pretend to be a baby and drink out of a bottle.

Max made it through a long day of tests. We were a little worried when we started off the day with many tears because he wasn't allowed to eat or drink and then we had a hard time getting his magic cream and tickle tape on him.  He ran through the house grabbing his port and yelling "don't take my blood" which I am sure was just a little anxiety over going to the hospital today and not understanding what was going to happen, but then the rest of the day went very smoothly.

We managed to get an MRI, CT Scan, and a spinal tap all done successfully.  He was under anesthesia for a lot longer than he is used to but he did very well this time (so far).  He got some good pain killers for the lumbar puncture which have made him in a particularly good mood. Overall he had to fast for almost 24 hours from all food and liquids so I bet tomorrow will be spent catching up on lost time.  I have had two specialists working on calling around to different doctors around the nation to find a lab that can analyze Max's Cerebral Spinal Fluid (CSF) for the last two weeks, as of this morning they were both still working on it, unfortunatly Children's doesn't have the lab equipment needed to run certain tests and doesn't have the specialists who are trained to read the results.  We are hoping that we can find both of these things soon before the CSF goes bad so that we don't have to redo this test or even worse travel to another state to redo this test. They were able to retrieve two different samples one of which got sent directly to the clinical trial that Max is a part of.  Stay tuned for the results from the other tests, hopefully this week. 



This was the view from our hospital room today. 
Do you think God could be any clearer?



Make-A-Wish Care Package

Max got a really fun care package from Make-A-Wish this weekend.  As you can see he is loving it!

Sleeping with Lightsabers

Monsters be afraid...

10th Infusion - IVIG - Complications

We had a big day today.  We managed to squeeze 7 appointments with providers, a 90 minute speech test and a 5 hour infusion today!  Whew!

Good News is that we  might not need to do another infusion for awhile, bad news is that he developed a reaction from this round.  After two rough days at home we ended up back in the ER trying to figure out what kind of reaction he was having. He was very tired, and was in screaming pain which he could clearly tell us was affecting his head.  Turns out he contracted aseptic meningitis from this particular batch of IVIG. I guess this can happen in 17% of patients that receive this infusion. It sounds much scarier than it is and luckily after 5 days he was back to his normal self.  I guess the reaction can build up after receiving infusions for awhile and our doctors are doing more research and contacting other doctors around the nation to decide what our next step should be.  In reaching out to other parents in our support group it sounds like there are many other OMS kids who struggle with this reaction. 

For those of you who like to know more details you can read more about this reaction here: http://www.annals.org/content/121/4/259.full.pdf                        

Pinkalicious - Just Think Pink



Madison's favorite book is Pinkalicious, about a little girl who eats one too many pink cupcakes and wakes up pink and has to eat green food in order to reverse it.  We read this book at least once a day and when it was time to dress up for halloween our first pick was to be none other than Pinkalicious herself.  However on the day before Halloween Madison wokeup with a body full of hives.  She must have eaten or come in contact with something that she is allergic to however the only thing she ate the night before was chicken, broccoli, and pasta, and a pink cupcake at grandma's house (all staples around our house).  We woke up on Halloween and after 24 hours of Benedryl her whole body was even more covered with hives, she was indeed pink.  I took her to her pediatrician and he confirmed that she was allergic to something and gave her a little stronger medication to try to cure her "pinkititis".  Since she wasn't uncomfortable we decided to hold off day giving her medicine so that she could play the part of her favorite girl that night. 



 It just so happened that Pinkalicious the musical made its debut in Tacoma this weekend. We enjoyed a cute show in a theatre full of little girls dressed all in pink. 

Thank You Costco

Dear Costco,
Thank you for having carts with large seats for kids.  It makes our weekly costco trips so much easier....

Happy Halloween

Love, Pinkalicious, Depot Guy, and Tinkerbell

Flu Shots

The Burt kids are all officially armed with flu shots now.  No flu mist for them this year.  Molly and Max did just fine getting their shots, but Madison seems to have a severe fear of shots.  Luckily our cousin was able to bribe her with a special cupcake bandaid. As you can tell it wasn't a pleasant experience.  Max reminded Mom to not cry when I got my shot and he offered to hold his sisters hands as well. 

 Have you gotten your flu shot yet?

Wish Granters

We had a nice visit with Max's new friends and wish granters from Make-A-Wish.  They came over to our home and brought toys for Max and the girls and got to know Max.    We had a lot of paperwork to go through and then they tried to get to know Max a little better to find out what things he likes. We are going to put things on hold for a little bit to get us through the next month. We have a lot of tests coming up this month as we try to restage Max's cancer and then hopefully we will learn what our treatment schedule will be and will also be able to get clearance from doctors for travel. They have a lot of paperwork and details to get through and some magic to arrange.  Stay tuned for more exciting news....

Welcome Home Molly!!

Daddy and the kids made a trip to Legendary Donuts to welcome Molly home.  

Madison picked out the tinkerbell for her and Molly and Max enjoyed his dirt donut.  Daddy even got a special UW husky donut.

The kids were excited to have Molly home, she bounced back right away and was feeling much better after a couple days.  She still has a kinda nasty sounding cough and wheezing which is probably from all the treatments she received .  We had her in to her primary pediatrician on Friday to follow up because her steroids were wearing off and the wheezing was increasing at night.  We are going to try an albuterol cough syrup to see if that will help.  She is still too little to try to learn to use an inhaler properly, but the doctor is hopeful that this was just a random isolated case and shouldn't necessarily indicate future issues.  He reviewed all the reports from our week and said that the hospital said that it was the worse case of croup they had seen. As a bonus, we did find an ear tube laying in her right ear, which means she has already grown out of her 2nd set of tubes. We are praying that she has outgrown all the ear infections, but may need another surgery in the near future.

Hormone Check

We had a nice visit with Max's Endocrinologist (Max's Favorite Doctor).  Max has officially remained the same height and weight all year.  He lost 5 lbs last year between his cancer diagnosis and the surgery, but other than that he has remained the same.  We are hoping in the next couple months to get him weaned down to small enough dose of steroids to encourage his system to start growing,  The next six months will be critical in order to see if his own body will kick in to gear and start producing those hormones that will get him growing. Molly is now 7 pounds heavier than him and 2-3 inches taller. 

 

We are home!

We got home succesfully tonight. After 10 epinipherine treatments, 5 times the amount of steroids that should have been needed, Molly is doing so much better.  We still have  a really sore throat, no voice and nasty cough, but we are breathing just fine.  We were able to get an emergency dose of steroids to give her tonight in case we run into any problems and if she does get into any more trouble they want us to come back immediately.  Both Mom and Molly are looking forward to getting a good night sleep in their own beds.  Thanks for all the prayers!

Stridor breathing

Here is a brief video of Molly with her stridor breaths.  If your child has this type of breathing do not take him or her to the hospital call an ambulance.  You will hear the code in the background calling all available doctors to our room. You will see that Molly looks uncomfortable but isn't overly anxious or upset which is why I didn't find it necessary to call an ambulance.  However, lesson learned.  Turns out her vital signs were pretty bad..

"Stridor is an abnormal, high-pitched, musical breathing sound caused by a blockage in the throat or voice box (larynx). It is usually heard when taking in a breath. Children are at higher risk of airway blockage because they have narrower airways than adults. In young children, stridor is a sign of airway blockage and must be treated right away to prevent total airway obstruction."

Molly also had tracheal tugging you can kinda see her throat being drawn in with every breath which is a sign of struggle,  we definately got immediate attention at Valley Medical, Children's ER, Children's ICU and in the hospital. We also had lots of researchers, medical students and residents watching Molly closely, because while Croup is really common and is filling the hospitals right now,  these stridor breaths are not common, they said she was in respitory distress and had reactive airway disease which may be a sign for asthma in the future, still too young to diagnose right now.  Just an abnormally bad case of croup.  Leave it to the Burt family to have a rare case of something, 

Molly was so good through it all. The IV team came to get her IV put in and they put on our "magic" cream that we put on Max at home and she got very excited and whispered "port?". The whole room laughed as they all know that she is a twin to a cancer patient and how different her world is that she is asking for a port instead of an IV.

Molly enjoyed putting stickers on herself while we gave her breathing treatments.

We are still at Children's in the normal medical unit, hoping to go home soon if we can get enough fluids down her without the iv. We are also trying to decide whether or not to send us home with breathing treatments or steroids in case we get into another emergency situation. Will update more later...

Molly's Hospital Adventures....

 Molly was just fine until after nap on Wednesday afternoon.  She woke up and had a super raspy voice and really hard time breathing, but was playing and having fun, smiley and happy, but given the news from the previous night, I knew it was going to be a bad night, I got all my medical books out, called all my medical resources on what needed to happen when things got bad in the middle of the night.  We tried all the home remedies for croup and nothing would touch Molly’s breathing.  She was working so hard to get a little air, even after humidifiers, hot steamy showers, cold walks outside, we even had her head in both of our freezers to see if we could get her little lungs to open up.  Finally at 10pm it got a scary, I rushed her to Valley Medical ER thinking that if she could just get a breathing treatment she would feel better. I was concerned enough about her breathing that I didn’t feel comfortable driving so f1ar without medical treatment when dealing with a breathing issue (and selfishly after spending the last day at children’s I really didn’t want to go again for a breathing treatment) when we showed up at Valley we were quickly rushed into a room and the nurse took her vitals and then ran out of the room and hit the alarms and called all available doctors to our room.  Things got a little crazy and we got quick shots of epinephrine and steroids to try to get her throat and lungs to open up, that didn’t work so they kept giving her breathing treatments of epinephrine and albuterol to try to get her vitals stabilized.  They worked hard for 1 ½ hours and then called the critical care ambulance (along with an ER nurse)to transport us to Children’s ER .  We got five more doses of epinephrine until finally she was able to relax a little  around 5:30am and they felt better about admitting her.  We were watched very closely by the ICU team along with lots of other doctors.  Will post more later….

Oh Croup....

Sorry I haven’t had a chance to make a lot of calls in the last 24 hours so please accept my apologies for not contacting more people personally.  Leave it to the Burt family to bring a little excitement to what was supposed to be a normal week.  

Tired of waiting for test results

We started out Tuesday night with Max developing a fever and we have strict orders to call the oncall oncologist and come straight in to the ER, so we did. After performing lots of tests to try to figure out what was wrong I finally asked to go home so that we could rest a little bit while we were waiting for test results and were able to get home Tuesday afternoon.      Then we got several calls from the hospital and oncologists letting us know they isolated the parainfluenza virus which is the virus that causes croup.  Max didn’t seem to have any normal croup symptoms because of the steroids he is on.  So far no other symptoms, he is up playing and feeling better all ready.  We will be watching him closely and meeting with his team again on Monday unless anything changes.. 

No more tears!

 

We are back to sitting by ourself while getting our blood drawn, such a big boy doesn't need his mom any more. In the second picture Max is asking me to stop taking pictures because I am distracting him from the movie that is on in the background.

Make-A-Wish

www.wish.org

Max has officially received one wish from the make-a-wish foundation.  We will be meeting with wish granters shortly to interview Max and get to know what would make him happy.  The organization will try to "make every effort to provide hope, strength, and joy to each wish child through the granting of his or her most heartfelt wish".  They include parents and siblings in the wish and try to make all participants feel special, while making all the arrangements and covering all wish related expenses.  We are so excited for Max and can't wait to be a part of this magical experience.

What would you wish for if you were given one wish?

Monsters....

Max has struggled with night terrors over the last year.  He will have anywhere between 1-3 in any given night.  They do seem to change in intensity with the fluctuation of his medication, however up until recently we never knew how to really comfort him because he couldn't communicate with us in the midst of the screaming.  He will start screaming uncontrolably while still asleep, depending on the severity of terror it will take us some time to try to get him to snap out of it and wake up. We understand that this is a very common symptom that Max shares with other OMS kids, but our doctors are having us pursue other therapy to make sure he isn't also struggling with a post tramatic stress disorder as well.

The last couple months Max has been able to verbalize more and let us know that these dreams are very scary and include monsters.  Max does have Henry the good monster and Buzz lightyear and they try to protect him. Most mornings when Max is more awake we ask him to tell us about his dreams and most morning the only answer we get is "scary monsters".   However one day, we were asking Max about his dreams and Molly chimes in and shares a story about her monster experience. She claims that she has a monster named Pak who also has a sibling who shares the same name.  Pak and Pak come in her room at night and cuddle with her but most of the time they kick her and she doesn't like it.  She says the only way to get Pak and Pak to leave is to yell "Dance Party!" as loud as you can and then they will leave and then you have to dance.  No joke-from the mouth of a 2 year old.    Next time you see them feel free to ask them about their monsters.  I just love to hear the kids talking more, its so fun!

Doin the Puyallup

We were able to make it to the fair for a few hours.  Rode a couple rides, visited with a few animals, saw a few shows and ate a few treats.  The kids loved every minute of it.

Max was supposed to share his turkey leg with his sisters.  We couldn't pry it away from him

Molly got her first bee sting, must have been attracted to her yellow shirt

We managed to wear out the kiddos

Berry Pickers

The kids have all enjoyed picking berries this summer.  Since I have such handy helpers I told them that I would make goodies with all the berries they picked.  They picked blackberries, blueberries, strawberries, and raspberries this summer.  Then they helped me make jams, syrups, cobblers, and still had lots of fruit to freeze.

Corn Huskers

In honor of the dawgs the kids helped us shuck and cook 100 ears of corn to freeze for the winter.  Too bad it didn't help us win the game. =)

9th Infusion - IVIG

We had a good infusion day today, met with his oncologist and had good numbers in labs.  We had  a good discussion about what our schedule will look like this fall.  Our oncologist is off to start her teaching and conferences around the world for the next couple months so we won't have as easy access to her as we normally have, but we were able to discuss and plan out our CT scans, MRI Scans, Spinal Taps, and other tests to gear up for in the next couple months.    We have follow ups with all of the different disciplines that follow Max while our oncologist is gone.   We did have some minor issues with medications today and I am glad that I am an informed parent, there are so many things that could go wrong if you aren't paying close attention. Today was also Madison's first day of preschool so she was able to join us in the afternoon and tell us all about her first day.

Playing with Lego Mack our favorite toy while getting infused

Madison telling us all about her first day at preschool

Our visit with Puss in Boots from Shrek

Chelan

Twin Beach Bums

We had a nice labor day getaway in Chelan with my family.  We enjoyed great weather, lots of swimming, enjoying the sun, berry picking, bike riding, water slides, and fun hanging out with the family.