Johns Hopkins December 2016

The Neurology team and staff are so warm and welcoming at Johns Hopkins. We are so blessed to be with some amazing experts in the field. 

Ricki was given a additional diagnosis of Postural Orthostatic Tachychardia Syndrome - which is of course another rare condition! Ricki is definitely one in a million in more ways than one. In some ways it is finally a relief however odd that seems.  We have been to several doctors and they have been telling us it is all in her head:( so it is a comfort having a cause for some of what she is experiencing. The next step is to experiment on some of the suggestions they gave us to lesson the symptoms and make life a little more comfortable for Ricki. We will be focusing on diet and some very specific excercises. They were so cute as they worked with Ricki. One of the challenges is any type of movement needs to be taken slowly.... No wonder she loves Flash the Sloth from the movie Zootopia! One suggestion was to sing "happy birthday" between each movement to let her body adjust to each new level. This is something she already had  started doing naturally but not all the time. 

The other issue that has been of concern was with some of the tumors on her c-spine. There has been talk of surgery but Dr. Blakely felt a better route for now would be an occipital nerve block that could relieve the pain without surgery. Her concern was that surgery could actually irritate all the nerves back there even more and make the pain worse so it is better to wait as long as possible to remove the tumors. 

Several other things we're discussed and we loved how thorough Dr. Blakely was. It was fun to watch how detailed she was and she knew exactly what to be looking for. She was vey optomistic and felt Ricki was really doing great with all she had going on! 

We feel so blessed and have some relief knowing we have someone with more experience keeping an eye on things with Ricki that we can continue to go to for the future care of Ricki.

                             

                                           

                             

Dad's Post fromJohns Hopkins

Johns Hopkins medical team was awesome. So good to be with specialists that understand what's happening with this awesome girl. They said Ricki is coping amazingly well with all she has going on. So true. She is the toughest, most positive person I know. Love you girl! 

    

                                

Big week!

   We have a big week ahead! We are so excited! We had the opportunity to meet Dr. Blakely at an NF conference and she was so sweet and kind to Ricki. Ever since, we have wanted to work with her in trying to figure out what is going on with Ricki. This week our dreams are coming true and we have our appointment with an NF2 specialist Dr. Blakely in Maryland. We have high hopes that Dr. Blakely can give us some answers or avenues to improve Ricki's health and ability to function. Our hearts have been breaking for Ricki. She has been in a lot of pain this week. Ricki has never been much to complain about her situation. She lets us know she is in pain but never complains or cries about her life.... Until this past week. She is tired of being in pain, not feeling well, and not having the energy to do much. Just getting ready for the day wears her right out and she  needs to rest. It breaks our heart to see her eyes tear up in frustration. We are feeling so blessed with this opportunity. We are so blessed by the love and support of many and recognize there have been many miracles that have transpired since we began this NF2 journey with Ricki. Thank you to everyone for the  love support and prayers - we couldn't do it alone.

            

                                               

Our Extraordinary girl!

This was a recent post from Ricki.  She has been so touched by everyone's love and support! 

Loving my ExtraOrdinary necklace! It's a daily reminder that being extraordinary doesn't mean being perfect or being more than just ordinary. It means that what you fight through everyday is worth something. You are amazing, fantastic and yes extraordinary. Every one has battles, in the end are we not all warriors?🎶we are the champions my friends da da and we'll keep on fighting to the end🎶 haha no but really I find it amazing how we all have different battles yet we all are connected through similarities we thought weren't there! I love you all so much. Keep fighting your battles like the Warriors you are. And keep being ExtraOrdinary💕 #ExtraOrdinary#JourneyGirl #KeepFightingWarriors

Blessings

It has been a week filled with blessings....

We always get a little nervous when its time to get Ricki's hearing checked and we came away feeling so blessed and grateful for the time she has been blessed with to have good hearing in her one good ear. Dr. Shelton felt like her other tumor on her facial nerve is stable as well. Such good news! Just hours after her hearing appointment we received a phone call from the NF clinic at Johns Hopkins. They had one appointment in December for Dr Blakely and wanted to know if we would like it. We are feeling so blessed with this opportunity....when we met Dr Blakely, she was so kind and genuinely interested in trying to figure things out for Ricki and she wasn't even her patient yet.. 

Ricki continues to have migraines every day, is nauseous,has pains in her chest and back,tires easily, has daily stomach issues, hot flashes, and seizures almost daily as well. We are hoping to at least get a better understanding of what is going on and hopefully some solutions to improve her quality of life. 

Now we just have to figure out how to get there and where to stay. It will be fun to try to figure out all the details. I was visiting with a dear friend at church today and she encouraged me not to worry and that things would work out. I appreciated her faith and assurance. It was just what I needed. It was just a few months ago when the president of our women's organization at church picked me up and took me for a ride to see how Ricki was doing.  As we visited she asked "Janine if you could do anything for Ricki what would it be?"  I told her I really wanted to get Ricki to a specialist who knew more about NF2.... Since then little miracles started happening and now we have an appointment to work towards! 

I know Heavenly Father hears and answers our prayers and that he will give Ricki the medical supports she needs as she looks to her future. We are so grateful for the many prayers in her behalf over the past few years. We are grateful for the blessings of added peace and strength we have received as we have traveled on this journey with Ricki and know we have not been alone. 

Miss Cache Valley

This summer when they were granting Ricki's wish through Make A Wish they have them go through questions that lead them to "If I could be anyone who would I be?" and " If I could go anywhere where would I go?" Her response to the who would you be was "I want to be Miss America" 

This fall Ricki approached us to be in the Miss Cache Valley pageant in November. Knowing her love of pageants and desire for opportunities to serve we agreed.  She had grown and matured over the years and had learned to push through her pain and we had confidence she could rise to the occasion. It brought us to tears several times watching her determination as she would practice her dance for her talent and then be so sick it put her right down in bed for several days but then she would be back practicing her dance again in pain, exhausted, but determined.  

The week of the pageant approached and her dad and I were concerned.  We prayed throughout the week that she would have the endurance she needed to reach her goal of being in the pageant. Her sweet grandparents and family jumped on the bandwagon to give Ricki all the support she needed.  Ashley, our sweet daughter-in-law came to the rescue as Ricki's hostess and was able to help Ricki get through the pageant smiling to the end. During the pageant many I am sure were critiquing any small imperfections in her walk,  or in her talent, but as we watched our sweet daughter, every step, and every smile was a triumph for Ricki. As I sat next to a lady during the pageant she was critiquing all the girls walks during the swimsuit part of the competition. Ricki was a little more off balance than usual and little did people know she had just had a seizure in a room downstairs minutes before and had pulled herself together to go out on stage in high heels and a swimsuit and walk with a smile across the stage. I think if people knew what she was going through they would know how really amazing she is - but that is not what beauty pageants are about and Ricki was not about to let anyone but her sweet sister know what she was going through. Even after the pageant Ricki asked me not to say anything about the seizures she had that night but as a mom I want others to understand why she is so amazing in our eyes. When Ricki has seizures her legs are the first thing to go and they become like jello. So, for her to cross that stage after a seizure was an amazing feat of strength and determination. 

We are all so proud of Ricki! What an amazing young women she is becoming! She has grown in grace and confidence as she has participated in the Miss Cache Valley pageant over the years. It has given her something to look forward to when she has had some challenging health issues with her NF2.  In our family she will always be our Miss Cache Valley and our Miss America, She represents what life is about- enduring gracefully through pain and hardships  and knowing that true joy comes from finding ways to serve and help others along your way. 

Day 7 Make A Wish

Day 7 Even  though Ricki's make a wish had to come to an end the sweet memories will last forever. What a sweet blessing it was to just enjoy one another without any worries or concerns. 

We headed to the airport a little early thinking we would grab some lunch there at the airport. We got there and Ricki had a seizure so we got her a wheelchair for her as we traveled though the long halls of the airport. We have found it is easier to handle her seizures in public when she is in a wheelchair and not on the floor. When we were going through security they told us to go through the wheelchair line which was on the furthest end of security. We told Angie and Jordan we would see them in a minute thinking it would probably take us less tine to go through security than them...but, that was not to be the case. The line was not that long with less than 10 people in wheelchairs and a couple with strollers but the workers there were not helpful at all and made it very difficult for those going through the line. Several in wheelchairs had to carried/supported by their caregivers to go through the scanner - which just didn't seem right and was very time consuming. When we went through somehow Rick got delayed and I was by myself to gather the luggage the three of  us checked. I was standing there with Ricki in the wheel chair, my backpack, three pieces of luggage and Ricks belongings from his pocket in my hands when a security worker raised their voice telling me I needed to move. I said I am sorry but I can't as I pointed to everything and he walked away. Minutes later another security worker did the same thing but was even more rude in his approach. I again explained I couldn't again motioning to all my things...A third security man came up to me and was about to tell me I needed to move but as I looked at him a big smile came over his face and said may I help you move your things? I was so grateful for his kindness and perception. So we finally got everything moved. Rick made it to where we were and then I had to go back because they had pulled Ricki's bag to check. So..when they did the test on her backpack it set off the alarm and they wanted to pat down Ricki but right then Ricki was having another seizure and a passerby was concerned and wanted to call 911 and Rick was trying to convince the woman and now security that it wasn't necessary. I told the security guard it was my bag and to please pat me down so we could catch our flight because by now we were not early but our plane was loading. I finally got back to Rick and Ricki was slumped over kind of out of it but ok to head to our gate. We made it there just in time to board the plane. Just as we were getting ready to board someone from security showed up to help us load the plane....it was a nice gesture after we had had such a hard time getting through security. Wow, what a way to end a trip! It just helped us appreciate even more how smoothly things had gone and what an amazing week it had been

Day 6 Make A Wish

 Day 6

Today was the highlight of the week. Ricki had the opportunity to be a princess at the princess tea parties there at Give Kids the World. She loved the interactions with the little girls. One little girl came running in and gave Ricki a big hug.  She had seen Ricki going into the building and insisted her parents stop in the middle of the road so she could go see Belle.  Another sweet gal was so sweet and kept asking for pictures with Ricki. Unfortunately we were unable to keep the pictures with other families because of privacy policies but will remember the sweet memories. Another cute family of little girls each dressed up in a disney princess costume asked to sit with Ricki. They were so excited and thrilled it was a delight. Ricki wore out after two of the parties and we headed out so she could rest.  We came back a couple hours later to volunteer at the cafeteria there in the village. Ricki took charge of the drink counter making sure it was clean and stocked and she also helped the rest of us clean tables and floors. We wished we had signed up for more shifts earlier in the week. Later that evening we felt like getting out and went to the animal kingdoms to go on the safari. It was beautiful and the animals were amazing. 

Day 5 Make A Wish

Day 5

We left for Epcot after lunch around 3. Ricki was well rested and in good spirits. Today was her best day so far. We got in all the rides she wanted and it was about 8:00 and we realized we hadn't double checked the closing times and discovered at  Epcot they were earlier and everything closes down for the night at. 9:00 before the fireworks. So we made a quick walk around the world showcase and finished just in time to watch the fireworks show. It was another day where less was more. 

Day 2 Make A Wish

Day 2

Universal Studios and Harry Potter World here we come! This day started off a little rough with Ricki not feeling well but still turned into a magical day for Ricki. We arrived around 2 in the afternoon and by the time we had walked through the shopping areas to get to the entrance Ricki was already worn out so we started at the first aide station getting an ice pack to try to cool her down and hydrate her a little. We we're touched by the kindness of everyone there. As soon as they saw Ricki's lanyard showing she was on her Make A Wish they bent over backwards to help. The timing was perfect when we arrived at the wand shop. There was no one else in line and so we started off the day with Ricki getting her own private wand show where her wand chose her. The wizard doing the presentation was amazing and she had Rick and I in tears and Ricki glowing with her beautiful smile by the end.  With the special passes we were able to get in all the important Harry Potter rides in a short period of time. One worker was so impressive.. He told us we needed to put our things in a locker before we could go on the ride. As we were walking out of the locker area he was there waiting for us to walk us to the front of the line. We finished off the day with dinner at Three Broomsticks. The family platter was beautifully presented and the frozen butter beer was deliciously creamy.  As we were heading out of the park around 7 'o'clock It was hard leaving the park ...there was still so much to see and do but our sweet Ricki had done all she could. That night as I was saying my prayers I was praying that Ricki would have the endurance to enjoy her trip..in my mind that meant she would be able to get up early and go all day into the evening :) an impression came to my mind and it was "Less is More" As I pondered on this I realized that this trip we needed to not focus on getting it "All" in but really enjoying our precious time together.

Day 1 Make A Wish Trip

Day 1

We arrived in Florida and were greeted at the airport by a kind hearted volunteer from Give Kids the world.  He was a retired Firefighter and he and his wife had moved to Florida to enjoy their retirement. He was so sweet to Ricki and all of his conversation was directed to Ricki making he feel special from the moment we got off the plane.  He took us to the car rentals and let Ricki chose what car we would be driving for the week. He gave us directions to the resort and we were off.  

Upon arriving to the resort at Give Kids the World we were showered with even more kindness. We were taken to our home for the week and given a tour. We had  a peaceful

evening as we had dinner, went fishing, and enjoyed exploring and finished the night with ice cream.

Wish Granting Party @ ZAGG

Life has been busy and I am just getting all the details from her fun summer posted. Obviously the biggest highlight was that her brother whom she is very close to came home from his mission. We purposefully had put Ricki's trip off so that he could come with her. 

Wish Granting Party @ZAGG

A month in advance Make A Wish contacted us and let us know that the ZAGG corporation was going to sponsor Ricki's wish granting party. They went above and beyond our wildest expectations of what began a magical journey for Ricki.  We had told Ricki that ZAGG had heard about her service to Primary Children's hospital and wanted to include that in a company party. They also wanted to know if she would dress up as a Disney princess and perform for their employees children.  Ricki was thrilled at the prospect! We determined that we would put together the costume of Belles farm dress since that would be pretty easy. Then shortly after they surprised Ricki with a beautiful Belle dress to perform in. When we went to their head quarters to try the dress on they informed her the dress was hers to keep! She couldn't believe it and was so excited! The night of the party we told her it was time to go and she walked out the door to discover a limo waiting outside! She screamed in delight! She started pitting the pieces together and realized this was part of her Make A Wish. It was a happy drive to Salt Lake. Her sister in law had brought some Disney Music that Ricki her friend McKell and her cousin sang to all the way. 

Upon arrival they took pictures like she was a celebrity making her feel so special. They had dinner their waiting and then after dinner there was the service project where we all assembled kits for Primary children's Ricki had her picture taken with everyone and then went to change for her performance. She first sang the disney song "Summer" and then had the audience dance with her to the song "Be Our Guest" after that dance we had made a Beast costume for her brother Nate and she danced with him. After the performance was over they had Gaston come and sing to her which she absolutely Loved! It was definitely the icing on the cake! What an amazing way to start a magical summer for Ricki. 

Hoping for Better Days Ahead

Just over a week ago Ricki started having laughing seizures. When she first started we found it humorous and had fun joking with Ricki about them. On Tuesday we started her on the seizure medicine Keppra. It seemed to start working right off the bat and she didn't have a seizure on Wednesday. She had a seizure on Thursday and on Friday but Saturday she started having both the laughing and non-epileptic seizures. She started complaining that her head was feeling funny and making her think of doing weird things like leaning out the upstsirs window which scared her and she didn't want to be alone. She progressively got worse as the day went on,  she was not herself and was singing, rattling nonsense things, and crying that she didn't feel good. These were combined with all her seizures . She was having the laughing seizures and frequent nonepileptic seizures. We called the neurologist on call and she had us stop giving Ricki the Keppra and increase her Topomax, 

Today was a better day. She complained all day of her head feeling weird and hurting and she has had multiple twitches and frequent non-epileptic seizures throughout the day but no laughing seizures.  She walked out to,the car several times to try to get me to take her to the hospital. It breaks our hearts seeing her so miserable and not being able to help her other than sit by her and hold her hand. 

As the day comes to a close it is more peaceful and  Ricki seems to be back with us and acting normal again. We are hoping for better days ahead.

Gelastic Seizures

Bless Ricki's heart. I am always amazed at what a trooper she is! Besides the hot flashes she continues to have she began having more frequent seizures. At first I thought they were just more of the non-epileptic seizures  but as I started watching her more closely some of them looked more like an epileptic seizure. About a week ago she started having some seizures that looked quite different from the others. She would pass out on the floor but then she would start laughing. It's crazy but every time I still think she is really just laughing and then after a minute you realize she isn't laughing on purpose and is totally unaware of what is going on. She is drooling and doesn't respond to anything. Our neurologist agreed to try her on a seizure medicine and also to get the lab work done that Dr. Blakely had suggested. It is a pituitary/hypothalamus work up. It was interesting as I was researching the laughing seizures one of the causes could be from a tumor by your pituitary gland. 

Ricki went in today for the lab work. It will be interesting to see the results. I am hoping we can find some answers for Ricki. When she went in to get the lab work done the person drawing her blood had a tough time finding her veins. She was such a sport when he was digging around in her arm with the needle he asked her if it hurt and she said "no" as soon as we were out the door she started complaining how much it hurt! When I ask her why she had told him no she said it was because if she had told him it hurt he would have been even more nervous and it would have taken even longer.  Her wisdom amazes me:) 

NF Forum 2016

What a blessing it was to again have the opportunity of attending the NF Forum in Texas. We could not have done it without the generous help from Angels Hands. 

At the Forum we had the opportunity to meet up with other families that had a child or they themselves had NF2. Ricki finds much encouragement and hope as she spends time with others that understand the challenges she faces. It is also an opportunity to meet drs that specialize in NF2. Although we have wonderful drs here in the salt lake area we really do not have neurologists that really specialize in NF2 and have a full understanding of the patient as a whole. This year they gave individuals opportunities to sit with the drs during lunch and ask questions. I found it so helpful and reassuring to have connections to specialists that we can go to for second opinions. 

Ricki did not feel well for most of this forum and had not been at Dr. Blakely's lecture. I had really wanted ricki to meet Dr Blakely but she had a seizure and wasn't up to it.  When I mentioned it to dr Blakely she asked if it would be ok if she came up to meet with Ricki in our hotel room. I was so touched by her kindness. Ricki has been struggling with hot flashes and increased seizures lately and Dr. Blakely gave us some guidance as to which direction we should go. She write down the name of some blood work to request for Ricki's hot flashes and then also felt that Ricki might have something called POTS. She said she had seen quite a few patients with NF2 that also suffered from PotS. She gave. Ricki a list of things that she felt could help. She gave us her email and information on how to send her MRIs and see if it would be worth a trip to John Hopkins.  I had been told that unless Ricki started having hearing loss in her good ear that she didn't qualify to go on a trial for a medication that they are finding will shrink tumors in some NF2 tumors. I was excited to find out if Ricki has had any growth whatsoever in her vestibular schwannomas she can qualify for the treatments. I also discovered they should be doing a volumetric study on those tumors as well to get a better picture of growth. 

We definitely came away feeling blessed and grateful for the opportunity.

Having fun at the photo both.