Beautiful Baltimore

Baltimore in July is beautiful!  We have enjoyed the sights and tried to not let the impending stress take away from enjoying our time together.  It is hard not to let the worries and concerns from our day of doctor appointments today creep into my thoughts. My son reminded me of a song my brother sings "His eye is on the sparrow" so.. Each time those thoughts begin to creep in I am so grateful for the peace and strength that comes when I pray and remember to have faith. There are tender mercies all around if I pause and look. Another tender mercy was our taxi driver this morning singing along to the song on the radio. One of the lines was "The Lord won't let me down."  

We started the day with Ricki getting a CT scan then off to see Dr Belzberg.  He reviewed Ricki's pain issues and she was tough as usual. She hardly complained to him. However, when he made light of her pain - bless her heart she had a total melt down and he realized how much pain she has been in. It seemed to melt his heart. He reviewed her MRI's and for the first time we saw some pretty clear pictures of the tumors.  Next we met with Dr Blakeley and she did a very thorough check and over all- with the exception of the paraspinal tumors in the thoracic area the other tumors in her body are pretty stable. The culprit of her pain is the deeper one near her aortic arch and one sitting by her left lung. The one by her lung is beginning to cause some small issues with her lung capacity. Currently we are grateful the one by her arch is not causing problems but they don't want to wait until,it does and then it could become inoperable. Ricki is in pain so much she can't even sleep and they suggested sleeping in a chair to improve her quality of sleep because it would put less pressure on her chest. We realized some of Ricki's best sleep lately is when she is sitting in a chair leaning next to her mom and dad. We discussed a few more ways to help her be as comfortable as possible until surgery.

Unfortunately her pain isn't going to go away because the tumors are only going to continue to grow and all of her options for pain relief are not working.  Currently  there are no other options than surgery. We would prefer to be at home for the surgery but they have an amazing collaborative NF team here at Johns Hopkins that have already met and been discussing Ricki several times before we even came to Baltimore. There is no doubt she is in excellent care here at the NF clinic. Because the tumors are deep it would be a very invasive surgery it will likely require a 4 week stay in the hospital here in Baltimore and then 2 more weeks in the hospital when we get back to Utah. 

We hate to see Ricki have to go through such an invasive surgery and then in addition be so far from home, family and friends for such an extended period of time. We will be meeting with the thoracic surgeon and cardiac/vascular surgeon the next couple of days.. Our neurologist warned us it would be overwhelming hearing all they would be doing but to be reassured that they wouldn't even consider doing the surgery unless they felt there would be a positive outcome from the surgery. 

Ricki only lasts for short periods of time when we go out but we have enjoyed the beautiful sites here in Baltimore. Honestly though I had a hard time enjoying it because my heart was aching and longing for a time when surgery is not the only option... I would love to see the day when they are able to put and end to NF... 

Tumor Review Board Results

I am having mixed emotions as I contemplate the call from Dr. Viskochil. He called today to tell us the results from the tumor board this week. He confirmed that the tumors in her chest are growing. As they discussed Ricki's tumors they felt that surgery for the tumors was possible and really the only option we have right now to give her relief from the pain. Unfortunately the only clinical trials right now are connected to the vestibular schwanomas and she has great hearing in her one ear so she does not qualify and we can't try to see if Avastin would shrink those tumors. Dr Viskochil felt it was very important we get a second opinion and it is a good thing to be going to Johns Hopkins.  The surgeon we will see there at Johns Hopkins is one of the best and Dr Viskochil said we would have to determine what will be best for Ricki and if it is even feasible to have the surgery done there. We do have fine doctors here but we want only the best for our sweet Ricki especially when it comes to surgery close to those vital organs. It will be interesting to hear the opinions and solutions the doctors at Johns Hopkiins come up with. 

In the meantime he said he would get an appointment with a cardio thoracic  surgeon who would assist our neurosurgeon with the surgery if we were to do the surgery here. We are always learning new medical terms and I wasn't exactly sure what a cardio thoracic surgeon was .....

A cardiothoracic surgeon is a medical doctor who specializes in surgical procedures of the heart, lungs, esophagus, and other organs in the chest. This includes surgeons who can be called cardiac surgeons, cardiovascular surgeons, general thoracic surgeons, and congenital heart surgeons.

Just reading this broke my heart. It is so difficult to see my daughter in daily pain and just want to be held. I wish  and long that there were better solutions for these tumors other than surgery. There are so many risks to surgeries and I hate to see her have to go through this. 

We will continue to keep you updated as we learn more....

More MRI's, hearing tests, and a spirometry

We have had a busy few weeks trying to get all the testing Dr. Blakeley has requested. 

After our appointment with Dr. V we had a Spirometry test done to get a baseline of her lung function. It came out within normal limits but did show a slight obstruction in her lung function. 

Next were hearing tests. Her hearing continues to remain stable in her good ear which we are so grateful for!

Following that last week she had a searies of MRI's. She has been in a great deal of pain and did not feel that she could hold still for the whole time so she had general anesthesia for her  MRI. When the anesthesiologist spoke with her before he asked of there was anything he could do to make it more comfortable so she requested something for the pain before they finished. When she woke up and I let her know her sister Angie was there she opened her eyes smiled and said "yo, what's up."  It was delightful to have her wake up in a cheerful mood. 

We received confirmation that they are planning on seeing us at Johns Hopkins at the end of the month. We are excited to get some opinions from NF2 specialists to know what route we should take to help Ricki with all the pain she has been experiencing lately. She normally is pretty optimistic but it has been wearing on her so we are hoping for some fresh perspectives and ideas on how to help her. 

Tomorrow Dr V meets with the tumor board to review Ricki's case. The tumors in her chest have a slow but steady growth pattern so it will be interesting to get their perspective as well. 

Appointment at the NF clinic and a full week

Ricki continues to have her good days and bad days. On her good days she still has tumor pains in her chest. But she gets out and pushes herself. For example, Monday she went to the Clarke planetarium. She came back excited because she loves science. She was exhausted and went right to bed. The next day she was miserable and slept all day and night. 

Wednesday was our visit to the NF clinic. Dr Viskochil was wonderful to take time with us. He shared some things he had learned from the recent NF conference in Washington DC. He explained how a Dr in the UK was researching pain on NF2 patients and discovered that those with Mosaic mutations experienced more pain. So basically what I gathered is that because they still have some good cells that can react to the pain causing them more pain than those with the inherited NF2.  This Dr on the UK is hoping to start a pain trial.  Also he mentioned that hopefully there will be some NF2 trials on the horizon. They can't come soon enough for Ricki...

I am not sure why but the Drs just have never taken the time to show us some of her MRIs. They just give us a quick look at the one they are focused on and that is it... For many years we have just thought she had one tumor by her lung. Then last year we found out there were two and the other one at the level of her aortic arch.- not a great place for a tumor.  Dr Viskochil took some time to review the thoracic area and now we find out she actually had 3 tumors in that area. The one in the brachial plexus area which they removed but she still has another one on the top of her left lung and the one by the aortic arch.. It was disheartening to see that tumor sitting right by the aortic arch. I guess that is maybe why they hadn't shown us... Dr Viskochil basically said no one would operate on that tumor:( ugh! All the more reason there needs to be a trial come available soon to start shrinking that tumor.  

Next we got to meet Leah who also has NF2. She is so cute with red hair and reminds me so much of Ricki when she was that age. They both have strong spirits. It is always comforting to visit with other parents who understand what challenges you face as a parent. Leah has had  some pretty tough battles to fight and continues to be in our thoughts and prayers. 

Thursday Ricki pulled herself together and went on a small hike to antelope island. She enjoyed it as much as she could with the pain she was experiencing. She is such aamazing gal! I am so impressed with how she has learned to work through the pain. Bless her heart though she was so tired she fell asleep on the bus on the way home and then while she was still sleepy and not realizing they weren't home yet texted and ask me to come pick her up...

We are trying to get more information to Dr Blakeley  at Johns Hopkins for her to review. She has worked with far more NF2 patients and so we are hoping she can think of some other solutions to give Ricki some relief. 

All in all it's been a good week!

Botox #2

Ricki got her second set of Botox injections for her migraine headaches.  We are hoping she will respond well again to them.  Last time it was like we had our old Ricki back for about 3 weeks and they say with each injection it usually improves.  The only downside this time is that she has been having these terrible tumor aches in her chest. So ...she may not be as pain free but we are hoping she will have some needed relief.  

Ricki wasn't in as good of shape after this set of injections because they didn't give her some torodal for the pain and they weren't able to get  a hold of the Dr. We will be more vigilant in making sure she gets it next time. So iwe got her a Dr. Pepper and a couple of ibuprofens and she was doing a little better.

Since we were so close to our college students we took a quick drive to Utah county. It was fun to get hugs and updates.  All in all it was a good summer day!

Be Fearless

Be Fearless in the pursuit of what sets your soul on Fire

That has and will always be our Ricki... Life has handed her some tough cards. In fact in an interview she says it feels like life doesn't like her but it doesn't matter because she loves life. 

What a week it has been! So Ricki's specialists followed up with us after our trip to the ER. She continues to have some pretty severe chest pains. The neurologist increased one of her pain meds. That has helped take a little of the edge off. Her neurosurgeon's nurse called back and let us know that both Dr Bollo and Mahan had been discussing the situation to determine what to do.  The nurse also said they were going to present Ricki's case to the schwannoma tumor board... So as usual since it is complex it will just takes time to figure out some solutions... However that is so comforting knowing they are taking their time and really trying to figure out what is best for Ricki instead of just rushing into something. We are forever grateful they have taken such great care of Ricki over the past few years. 

On the positive side of the medical challenges for Ricki we finally got an occipital nerve block for Ricki and her headache has been much improved at a 4-5 for 3 days so for and she feels it has really helped. So she seems much improved as a whole because instead of dealing with both the head and chest pain it is just the chest pain. It is comforting to at least feel like we are making progress in helping Ricki find some relief. 

Graduation!

Ricki is graduating from High school! It has been exciting to see Ricki take each challenge she has been faced with and completed each challenging course with grace and determination. 

In her High school career Ricki has had 2 brain surgeries,  which came with Traumatic Brain Injury side affects.( Finding it difficult to be in large crowds, constant headaches, memory loss, difficulty in processing, and a miraid of other side effects..). Ricki's left vocal cord had also become paralyzed and it was difficult to hear Ricki speak.  She went through a surgery called a vocal cord re-intervention. They moved the vocal cords closer together and she sounds so great no one knows one of her vocal cords is paralyzed.  Two tumors were growing on her face one above her lip and one on her cheek. The one on her lip was growing so she opted to have surgery for their removal.  The next challenge she faced was a Spinal Cord surgery to remove yet another tumor. Prior to that surgery she was dealt with losing her balance and strength in her legs causing her to have frequent falls - but she was determined to dance in her ballroom dance team and  also be a part of the Miss Cache Valley Teen Pageant. During all these surgeries she developed chronic migraines that did not respond to normal treatment. In addition to all that her body had gone through she began having non-epileptic seizures. For almost a year she experienced 1-2 seizures an hour. The following year They went down to just several a day and now for the past 6 months she has been almost seizure free. As she was beginning to get the seizures under control the tumor in her brachial plexus tumor had grown to the size of a grapefruit and she began experience severe pain and to lose feeling and the use of her left hand and arm. So....she had yet another surgery.  With each surgery there is always a lot of effort to regain any lost ability and as usual Ricki was awesome in her efforts to regain the motion in her hand and can again play simple songs on the piano. It seems she just doesn't have much of a break and currently she has another tumor in her left chest at the level of her aortic arch which has begun to cause a great deal of pain. I am amazed at her ability to move on with her life. She has this amazing strength and ability to find reasons to smile  Some of her reasons to smile are her family, serving others, her faith and determination, and of course anything that sparkles or is Disney will always bring a smile to her face. 

Ricki has definitely successfully completed each challenging course she has had to face through out her High School career. We are humbled by the many who have helped her complete the challenging courses. Her family and extended family have always been right by her side. She has had loving and patient  teachers. Amazing friends that have been there for her. The youth organization in our church have been amazing and made sweet efforts when she was faced with each surgery. And we would be ungrateful if we did not mention the many doctors and nurses that have done an amazing job getting Ricki through the medically complex situations she has  experienced. 

So Ricki's graduation is truly a celebration of not only Ricki's accomplishments but the many who have helped her along the way.