Adjusting to our new normal

Here we are, half way through summer (or as we are calling it in north west Ohio, Monson season) and half way through 2015. Time has flown by and has shown no mercy for my desire for a slower pace. The last several months have been a challenge and have shown us that Batten disease does what it wants, when it wants and doesn’t care how much we plead with it to leave our daughter alone.

First let me apologize for the length of this update and for how long it has been since we shared with all of you how Kate is doing. It has been busy few months and over the last several months we have had to adjust to Kate’s new normal, which requires more of our time and attention and at times was hard to accept.

June 22 marked the five year anniversary of our daughter’s diagnosis. I hate June 22! I woke-up on June 22, 2010 without the knowledge of Batten disease and with the belief that our daughter would soon regain all of the skills she was losing and her seizures would get under control as soon as we found the right medication. I had no idea that in a few short hours Kate’s neurologist would call and turn our world upside down. Life today looks nothing like it did 5 years ago. The world is different. We are different. As with all changes, some things are better and some things are devastating. Over the last five years we have learned to see and focus on the good. How miserable would we be if we didn’t look for the silver lining in each cloud?!

Since late December Kate’s vomiting episodes and congestion have increased in frequency and severity. It has become a challenge to keep her meds and food in her system and keep her airways cleared. After meeting with a GI doctor and a pulmonologist it became clear that the congestion and vomiting were due to a common factor. Kate’s stomach was no longer working as it should. Kate was having a lot of reflux, meaning the contents of her stomach were leaving her stomach and moving up her esophagus. This caused her to vomit and could cause damage to her esophagus if left untreated. The pulmonologist was concerned that the fluid was entering her lungs, thus causing her congestion and was the reason she had pneumonia two times within the last six months. The GI doctor ordered a test to see how severe it was and the results were alarming. Based on their advice and the test results we decided to have a GJ tube placed in her stomach. She had a G tube already. The G tube allowed us to feed Kate via her stomach. The GJ tube would allow us to feed her via the stomach or small intestine. We have adapted to many things since Kate’s diagnosis. We have learned to use all types of medical equipment, learned about different medical conditions that affect her, etc. We have done so willingly and have adapted quickly to her new needs. When we first realized that Kate needed a G tube we were nervous and sad but moved forward quickly. We saw the benefits and were ready to do it for everyone’s benefit. I can tell you I didn’t accept the news of needing a GJ tube with the same mind set. The thought of the GJ tube to this day angers me. We had it placed a few weeks ago and I still don’t have peace with it. I know it was and is necessary. I see some of the benefits. I am thankful we had an option to keep her safe and a live. However, the thought of her being hungry because her stomach is empty bothers me and I hate that this disease has taken another piece of my daughter. The doctor assures me that Kate isn’t hungry. When someone is hungry they can feel weak, shaky and disoriented because they need nutrition. Since Kate is receiving nutrition via the small intestine she doesn’t feel those things. That brings some comfort. She has handled this a lot better than I have and once again proves that she is stronger than the disease.

Our hope was that the GJ tube would stop the vomiting and congestion. It hasn’t. This saddens me. Her congestion is about 80% better and while she doesn’t have anything to vomit we have seen a few times each week episodes of her wanting to vomit. Had she had food in her stomach she would have. Instead, she dry heaves. The GJ tube was not the miracle or solution we prayed for but it has increased her quality of life. For this I am thankful.

Kate became ill on Father’s Day, June 21. After a long day of treating her at home we went to the ER. It was determined during that visit that Kate had pneumonia; this was the third time since October. The doctor was willing to release her and allow us to treat her at home since we had everything the hospital would use at home. I was pleased with his decision and felt equipped to do everything Kate needed. I under estimated how sick she was and the difference between the IV antibiotic given at the hospital vs. the antibiotics we use at home. The following day was horrific!!! Kate was miserable and required constant care. She worked for every breath. Her oxygen levels would decrease to 70-80 without the use of oxygen and we didn’t have enough oxygen on hand to last an entire day. The oxygen company couldn’t deliver to our home for another day, so I had to leave Kate and travel 1.5 hours (one way) to get more oxygen. When I returned home she was showing signs of pain; her face communicated she was in pain, she had a few tears, and she looked like she wanted to scream. We havent seen her in pain since her brain surgery four years ago. Her heart rate was 150 (her norm is 70-120), which indicated she was in distress. Between her pain, O2 levels, and constant need for suctioning we were in over our heads. Thankfully we had a nurse with us during the day and at night. Brock and I agreed that if things weren’t better by the morning we would take her back and ask for her to be admitted. You can imagine how scared we were for her if we were asking to be admitted to the hospital. Thankfully she made a huge improvement over night and appeared to be 75% better. She still required a lot of attention and constant treatments – breathing treatments, vest treatments, suctioning, etc. We were relieved to see how much better she was doing. The following 10+ days were long and stressful but with each day she showed small improvement. It wasn’t until this weekend we felt she was back to herself.

Since Kate was so sick we were able to obtain new medical equipment that we requested several months ago; a cough assist and pulse ox machine. It is sad but a relief to have these machines. We know the equipment will help keep her healthy and safe.

Since Brock and I both work full time one of the grandmothers has been caring for Kate after school and during the summer. Due to change in the grandmother’s schedule we had to find someone to take care of Kate during the summer. Fortunately, we were approved for additional nursing hours and were able to find a few nurses to take care of Kate during the day. Kate seems to enjoy the nurses and the activities they do with her each day. We feel blessed to have found nurses that are compassionate and loving. I think Kate misses school but the nurses do all they can to keep Kate busy.

Over the last few weeks Batten disease has gained a lot of publicity due Charlotte and Gwyneth Gray, daughters of well-known Hollywood producer. Both of the girls have Batten disease and the family has set up a foundation to fund research. They have been interviewed, asked their famous friends to get involved in raising money and creating awareness, etc. The Batten community is torn on how to feel about this news. While we have prayed for awareness we never wanted another family to receive the devastating diagnosis. And we would never want a family to have both of their children diagnosed with Batten disease. We embrace this family and welcome them to the Batten community, pray for them as they watch their girls progress and pray that their efforts, fame and fortune can bring a treatment and cure!

This weekend we will travel to Chicago for the annual Batten conference. We are excited to see our Batten family, hug the kids and get updates from the doctors and scientist. It is always an emotional weekend but one that we look forward to each year.

We hope you are enjoying your summer and thank you for your support, prayers and kindness.

5K Results

1 week ago was the 5K for Kate and we are still amazed, humbled and overwhelmed by the turnout this year. Thank you to everyone who came out to run, walk, do PiYo or just hang out with us. This year’s 5K was a huge success. When I say it was a success, I dont only mean we were blessed by the funds that were raised for Kate and the BDSRA, but we were successful in raising awareness about this devastating disease. Thank you for being playing a part in the success of the 5K. God blessed us with a great day, beautiful weather and amazing friends and family. I hope you had a great time as well.
Thank you to all of the sponsors, volunteers, businesses/people that donated items for the raffle and silent auction. Your generosity and kindness are deeply appreciated and played a key role in the success of the 5K.
I dont even know how to thank the team that helped plan the 5K. We are grateful for the time you spent sitting around our table discussing ideas, seeking donations, creating games, passing out flyers, etc. If left on our own I know Brock and I couldnt pull off the 5K. Thank you for your dedication, time, and support. But more than those things, thank you for your friendship!!!!

Attached are the results of the 5K. If you didnt receive your medal, please email me at elliebenroth@yahoo.com and I will ensure you receive it.

We hope to see you at 4th annual Team Kate 5K next year :)

God Bless!

5K RESULTS

Happy Spring

Spring is around the corner and we are ready for warm weather and the cold and flu season to be over. Kate has had pneumonia twice and bronchitis once (at the same time she had pneumonia). The most recent time was the beginning of February. Keeping her healthy this year has been a challenge. She is constantly congested and requires a lot of suctioning. Keeping everything out of her lungs requires a lot of work from her aids at school and us at home. She hates being suctioned and sometimes she is so tired she can’t cough. Our efforts almost seem useless. As soon as we think we have cleared everything out, she is congested again.

On top of this, she is also having issues with her stomach. She randomly vomits. She typically vomits after we feed her but a few times it has been well after her feed. (She is fed a natural formula via her g-tube). We aren’t sure if her stomach is struggling to digested the food, so when we feed her it upsets her stomach, if her stomach is weakening and cant hold all of the food or if the congestion is the issue. Is she chocking on the mucus, thus causing her to gag and vomit, or if there is mucus in her stomach, upsetting it and when we feed her it makes it worse. We think she is gaging on mucus and vomiting but we aren’t 100% sure. We are suppose to see a GI doctor but havent received an appointment yet. For now we have taken each of her feeds and split them in two. Each container of formula holds 8.45 ounces of food, so we are feeding her 4 ounces at a time and give her 2 hours in between each feed. That does seem to help, but this morning Brock really struggled to keep the food in her. She was so congested and he was trying to suction her when she coughed, but it was obvious she was trying to not vomit. I hate this for her!! How miserable this must be for her.

Her seizures are not as well controlled either. Early on we were told that we would never have 100% seizure control and would have to decide how many she could handle each day, how many we could handle and how sedated we wanted her to be / how much medicine to give to her to control the seizures. Since her seizures don’t make her tired and she recovers from them easily, we have kept the medicine to a minimum (although you wouldn’t know that by looking at all the meds we give her each day, several times a day). Over the last few weeks her seizure activity has increased and they seem stronger. She cries out during most of them. I don’t think she is pain but the sound of her moaning is heartbreaking. We were praying that it was a short term issue but after a week and half of her having a dozen or more a day, and requiring rescue meds twice, we have elected to increase one of her seizure meds. We are waiting to hear from her doctor on which one to increase and the new dosing amount.

Other than that, she is doing well :)

We are still struggling to fill all of the nursing needs we have. Cheryl has been with us for almost 3.5 years and stays with Kate Sunday night – Thursday night/Friday morning. We couldn’t survive without her. Since Brock and I still work full time, we need to sleep, and having Cheryl with Kate allows us to get the rest we need and we know she is in great hands. We also have a nurse that works every other weekend but we aren’t able to fill the other weekend opening. It is frustrating but we feel so blessed to have most of the shifts covered.

We are in the process of planning the 3rd Annual Team Kate 5K and 1 Mile Fun Walk. This year we are adding a small kids carnival and PiYo class. We will also have a silent auction, raffle, 50/50 and bake sale. We hope you can join us on May 9th for the event but if you cant, you can do a virtual 5K. All the money raised is split between Kate to help with the cost of her medical needs and research endeavors. If you wish to sponsor the event, donate an item for the raffle, auction or bake sale, or volunteer to help, please let me know. Registration forms can be found on Kate’s website or you can register on-line. (www.HoldingOntoKate.com, https://teamkate.webconnex.com/2015)

Kate has received physical, occupational and speech therapy since 2010. The team of therapists have worked hard to keep her abilities for as long as possible, to keep her body flexible and muscles loose and find alternative ways for us communicate with her. They are a great group of women and the work they do with Kate has kept her physically stronger and mentally aware. Recently we were told that insurance has denied her therapy, therefore they are running things through her secondary policy. The secondary policy only allows so may visits per year, so rather than seeing the therapist once a week, she goes every-other week. I hate that insurance companies get to dictate the care she is able to receive but we are working with Kate at home to keep her flexible and engaged. I wish the insurance company could see outside their policies and the benefits of therapies.

Recently I have met two new Batten families and Brock has been conversing with a family overseas as well. All just received the Batten diagnosis. It breaks my heart to know another family is dealing with this horrible disease but it is such an honor to help them with their questions and encourage them. It always brings me back to the first few weeks after our diagnosis; such a horrible, dark time. I am so thankful God took all that darkness away and helped us find joy again. Not that we don’t have dark days or cry, we just have more to smile about than we ever knew! Please pray for these families. It is a lot to take in and a lot to adjust to.

One of the people who has had a positive impact during this period of life is a lady named Sally. She was a nurse practitioner at Nationwide and we saw her every time we had a visit with Kate’s neurologist. She specialized in Batten disease. She comforted us when we cried, laughed with us and gave us great advice with each new symptom, problem, etc. We saw her on February 24, which was the last day she was seeing patients before she retired. We are going to miss her. I hope she knows what an impact she had on our lives. We wish her nothing but the best.

One of the greatest blessings through this journey are the people we meet. The Batten families are an amazing group of people. As with all groups, you bond with some people more than others and one family that we have grown to love dearly are the Vigils. They were our buddy family at the first conference we attended. They helped us understand the conference, introduced us to other families, etc. They dealt with the disease with grace and compassion. Their daughter, May Payton, was in good health, considering she had batten. They included her in everything they did and we enjoyed seeing photos of them on Facebook. Two weeks ago, very unexpectedly, Mary Payton passed away. Her passing shocked the Batten community. I think we are all still in shock that their beautiful daughter has earned her wings. Please keep them in prayer (mom-Nikki, dad-Joe and sister-Maison). While we all know the end result of this disease, they had no warning that her final day was near.

Several of the Batten kids are battling illness and progression, including Westley, Olivia and Kaitlin. They could use your prayers.

I know the update on Kate isn’t great and we are struggling right now to know what to do to keep her comfortable and well, but I don’t want to leave you with doom and gloom. We could use your prayers for healing, knowledge, and peace. But know that while we are praying for these things, we are also praising God for the blessings he continues to give us. Today is Lauren’s 12 birthday and what a gift she is to us! I am so thankful for her, her health, her heart and her humor. She is an amazing girl and I love watching her grow up and seeing who she is becoming. I recently applied to Moody Bible Institute out of Chicago. I am anxiously awaiting their response to my application to become an on-line student for their bachelors program for Biblical Studies. Brock has started working on cars that have been wrecked and fixing them. He seems to enjoy this type of work.

I hope everyone is doing well and has a great spring and Easter. Thank you for your prayers and support. Hope to see you all on May 9th 

Happy Fall!

Happy Fall/Autumn!! Its hard to believe this year is almost over and the holidays are approaching. We are enjoying the change in season and hope you all are doing well. Today we had a visit with the doctor that is overseeing the clinical trial for cannabis. These visits are easy and quick since we talk to him every Wednesday via the phone. We have had several people ask how things are going, etc. I apologize for not updating sooner. Life has been busy with Lauren playing volleyball, Christmas play practices, and various other activities. The cannabis has been successful in some aspects and in other areas it hasn’t lived up to our hopes. Kate’s daily seizures seem to be about the same. She has on average 5-8 seizures a day. Some days she will have less, some days she will have more. On the bright side, she hasn’t had the seizures that cause us to go to the emergency room or any grand-mal seizures. We are very thankful we have stayed out of the ER and she hasn’t had any grand-mal seizures!!! We are able to increase the cannabis two more times and if those increases don’t improve her daily seizures we are out of options with the cannabis. We are praying the increase will be effective! Please join us in praying for this. Regardless of whether or not her daily seizures change, we will keep her on cannabis because it is helping with the other types of seizures.

Since starting cannabis Kate’s myoclonic jerks have increased and disturb her sleep. Whether or not this is a result of the cannabis, we don’t know. Its likely NOT a side effect because cannabis should decrease her jerks. WE upped one med in the hopes of helping but it has not been effective. At today’s appointment the doctor suggested we increase one of the other meds, so we are starting that tonight. Please also pray for this.

Another benefit we have noticed since Kate started cannabis is she is more alert. She is more engaged in her environment and the things going on around her. She tries to talk and participate. We love this!! We also have been able to reduce the amount of MiraLAX she takes on a daily basis. (I will just leave it at that ).

Overall, the trial seems to be going well. Most of the kids are having good results. A few had to drop out because cannabis was causing other issues and those issues were worse than the seizures. The other batten child that is in the trial is doing well on the med. We are thankful that the results for most are favorable.

In October Kate was hospitalized with pneumonia. Kate struggles a lot with congestion. Her congestion sits in her throat and is hard to reach with the suction machine. If she is tired she doesn’t have the energy to cough and clear her throat. Most days this isn’t a big deal. We position her and do various things to help her out. However, one night none of our efforts were effective. She went to bed fine and by 4 am she struggling to breath, had a fever and her heart rate was very high. The moment the doctor saw her, he said she would be admitted. We had the choice to be admitted into our local hospital (St Ritas/SRMC) or go to Nationwide in Columbus. We elected to stay local. Kate received great care at SRMC. The staff was amazing and made our stay pleasant. It was loney and boring but Kate did well with all the treatments and meds she was given.

We recently decreased Kate’s outpatient therapy. She was doing OT, PT and Speech. However, the speech and OT therapist do not believe the therapies are as beneficial for Kate as they once were. After much consideration we agreed to decrease each of them. She now sees them every-other week. It was a hard pill to swallow. No parent wants to hear that their child has lost too much and the therapies are no longer effective. Its one more defeat. Rather than focusing on the negative, we are trying to do things at home to keep her engaged and active. My mom, who was a special ed teacher, put together didn’t “shoe box” activities. Each box has different activities and the activities engage different senses, textures, etc. She seems to enjoy doing these things.

Other than those two things, life has been pretty typically. Kate is enjoying school and the busyness that comes with that, such as after school activities, equestrian therapy, sleep overs, etc. Unless something major happens, I probably wont update again until after Thanksgiving. So we would like to wish everyone a Happy Thanksgiving. We pray it is filled with family, great food and love. We have so much to be thankful for and will take a moment to pause and reflect on those blessings.

Please keep Elijah (Elijah’s journey), Seth (Seth’s fight for peach), Becca, Asher (Asher Bash), and Ashlynn (Ashlynn Noel – A Batten Journey) in your prayer. They are fighting hard! As well as Westley’s cousin, Jarrod. He is a young man and is having heart surgery this week. Also, please pray for those families that will be “celebrating” Thanksgiving and Christmas for the first time without their precious child.

Thank you!!

Some very exciting news!!

It has been awhile since I last updated everyone on Kate, which could mean a few things – 1. Kate has been doing well, so there isn’t much to report OR 2. Things have been very busy and I havent had time OR 3. I’ve been lazy. Truth is all are true.

The summer was busy, especially July, and when I had time to sit down and write I was too tired to put into words how Kate was doing. Honestly for the most part, she has been great. I was thinking this morning about Kate’s current status and thanking God for how stable she has been. We are still battling her seizures but other than that she has been good. We havent noticed a progression in the disease or had to deal with new symptoms. She remains alert, most days, and happy. We still see her funny, stubborn personality from time to time and that brings a lot of joy to our lives.

Kate’s seizure control, for a batten kid, is good. She has on average five seizures a day. Each month we deal with her seizures going back-to-back (several seizures w/in a small period of time, which do not stop without medical intervention) for a prolonged period of time. Prior to June we used diastat to treat the seizures with no success. We would then head to the ER where Kate would receive meds through an IV. Finally, after trying a few different rescue meds, we have two on hand that stop her seizures and keep us from visiting the ER. We have come to the resolve that Kate’s daily seizure drugs will not prevent these once-a-month occurrence. Well, we had come to that resolution, until we were invited to participate in a clinical trial at Nationwide Children’s Hospital for Cannabis.

Nationwide was given permission by the FDA to allow 20 patients with hard to control seizures a form of Cannabis. The form of cannabis Kate will be taking contains 98% Cannabidol and less than 2% THC, which means she will not get “high” from the drug. Over the last several months Brock and I have read news reports and studies on cannabis and watched documentaries. We agreed that if Kate was ever offered the opportunity to take it we would do so. People with hard to control seizures seem to respond positively to the drug and have a higher success rate with it than traditional seizure meds. Considering all of the drugs Kate takes and types of drugs she is on, we feel that this route is safer for her body and will have a higher success rate. The study is for two years and is heavily regulated. It will require several visit, phone conversations, etc. but we feel beyond blessed that Kate was selected to be a part of this group. Over time, if she responds well to cannabis, we can decrease or even stop the regular seizure drugs she is on. The doctor also said that the kids who started cannabis last month are showing some improvements with muscle tremors and jerks. We are hopeful Kate will have the same response. Please pray with us that this goes well for Kate. We are praying that she responds positively to cannabis, does not have any serious, negative side effects, and tolerates it well. We are also praying that some of the positive side effects will happen, such as less to no tremors, jerks, etc.

Kate had a birthday last week. Hard to believe she is 8 already. Typically we celebrate her birthday by inviting family and a few friends over, but this year we elected to celebrate differently. Kate’s birthday is two days after our nephew’s birthday. Even though they were 14 years a part in age, they had a special bond, therefore we always celebrated their birthdays together. Over the last few years we also celebrated my sister-in-law’s birthday at the same time. As some of you know, our nephew, Dylan, passed away last December after battling cancer for three weeks. Since this was his first birthday in heaven, we decided to not have our traditional party. We felt it would be too hard to celebrate without Dylan. Brock, Lauren, Kate and I went to the Fort Wayne zoo and spent the together enjoying the animals, the weather and being together. It was a very nice day and something we will always remember. I was worried about how we would handle her birthday since she is now 8 and the average life expectancy for Kate is 8-12 years. Since her 7th birthday I have been anxious about her turning 8 but thankfully the anxiety has gone away. Rather than focusing on how long they say she will live, I am focusing on how well she is doing today and praying for the miracle of healing we so desperately want!

The end of July we attended the Batten conference in Columbus. As always it was a great time. It was nice to see everyone, catch up and make new connections. Since it was in Columbus and the Ohio Chapter was hosting we were busy helping with different aspects of the conference. It was nice being involved and working with the BDSRA, who did most of the work. As soon as the conference was over we were looking forward to the next one. Hurry up July 2015 :)

School is back in session and Kate has done well adjusting to the new year. Kate has always been someone that likes to go, go, go. So summer is VERY boring for her. We cant keep her busy enough or offer her enough chaos. Since school has started, Kate has been in a better mood, smiling more, etc. We worry about the germs and illnesses she is exposed to but she loves going and seems to benefit from the activities and therapies, so we send her. She did come home with a cold after a few weeks, but has recovered from it well.

Recently we had to let one of our nurses go. She was working every weekend and I found her sleeping. After watching the videos of previous shifts, we elected to go our separate ways. I was so angry. She was sleeping during every shift, up to four hours. She was setting an alarm on her phone so she would be awake and alert before we came to relive her in the morning. We really liked the nurse and were so upset that she didn’t treat our daughter’s life with respect. The majority of the time Kate doesn’t need anyone’s attention during the middle of the night, but when she does, it is imperative that someone be there to help her. The night I found the nurse sleeping I had told her that Kate had a bad day and asked her to watch Kate’s oxygen levels and seizure activity. Within one hour of reporting to work, she was a sleep. The more I think about what could’ve happened, the angrier I get. In my opinion, it was a complete disregard for Kate’s welfare.

Within the last 24 hours two sweet batten girls have gained their wings. Sophia and Ali are free from the disease and are running, playing and enjoying being free from batten disease. Since January 30+ children have passed away from batten disease. Please pray for these families.

All is well.

Here we are, heading into the middle of July. Time use to feel like it stood still but now it feels like it passes so fast and I can’t keep up. Hope you had a great 4th of July!!

Thankfully I don’t have a lot to report on Kate. She has been doing pretty well. We met with her neurologist a few weeks ago. We were anxious about the appointment but overall it went well. We had a lot to discuss and prayed that we would get answers that would help with Kate’s quality of life. After talking with the doctor for an hour we decided to increase two of her medications and change her emergency drugs (emergency meds/drugs are medications we give Kate if she has too many seizures back-to-back or if a seizure last longer than five minutes). These changes seem effective, so far. Her daily seizures have decreased and the two times we have had to give her the emergency drugs they have worked, which means we have avoided the ER for over a month. Praying this continues. Praying the status seizures go away. Brock and I made the decision to take Kate off of the hormone pill. We saw some side effects we didn’t like and didn’t see any positive effects from it. Kate is on so many meds, therefore we don’t want her on anything that isn’t helpful.

We got a call from Nationwide Children’s hospital a few days ago and they have a new medication they are making available to kids with hard to control seizures. The FDA hasn’t approved the drug but has given the hospital permission to allow 20 patients to trial the drug. It’s not an official clinical trial but for all intensive purposes, it is a trial. We are excited and nervous to be a part of the group trying the drug. Praying it will be a great option for Kate and hopefully it will allow us to decrease some of the drugs she is on.

Kate is sleeping a lot lately. She sleeps most of the morning and takes a nap in the afternoon. We don’t know if this is because of the medications she is on or if it is the progression of the disease. I hate seeing her sleep so much but I know her body needs the rest.

We recently went to Washington DC for a few days. It was nice to get away and spend time together. We went to Arlington Cemetery, a few museums and other national monuments. It was fun and relaxing! Kate did well during the trip. She tolerated the heat, being on the go and change in schedule. I am so glad we took the time to go and create some memories!

In a few weeks we will be attending the annual BDSRA conference. I am so excited to see the other families and catch up with them. It is always a good time and we come away feeling so blessed. Lauren is excited to see some of the friends she made last year and hopefully will make some new friends this year as well.

Hope you enjoy the rest of your summer and find time to relax and enjoy the beautiful weather.

Staring, teaching and accepting.

This isnt a normal post. I will update you on Kate within the next few days.

Over the weekend we went to Washington DC. Our vacations usually consist of going to the Batten conference, but this year the conference is in Columbus, Ohio, so we decided to take a “real” family vacation. We each had one thing on our list that we really wanted to do but other than that we were excited about not being on a schedule, not waking up to an alarm, and just hanging out. On our last day in DC we decided to go to the Smithsonian Zoo. The outside temperature was nice so we expected to see a lot of the animals. However, there was something else more interesting at the zoo than the animals.

From the time we entered the zoo it was apparent that Kate was more interesting than the pandas, elephants and tigers. We couldn’t go more than two minutes without someone staring at her. We are use to young kids looking at Kate and saying things like “what’s wrong with her”, “why is she in that (wheelchair)”, etc. What I am not use to are adults staring at her. Brock and I both said we should put a note on Kate that read “I am a person, not an attraction at the zoo.” Within an hour of entering the zoo we left. We left frustrated, mad and hurt.

I understand seeing a child in a wheelchair isn’t a daily occurrence, but when did we as a society forget that it is rude to stare? I understand why children gaze, but I don’t understand why adults are so fascinated by Kate and children like her. Honestly, I think anyone over the age of 8 should know better than to stare at someone, regardless of the reason why. By the age of 8 they have attended anti-bullying assemblies at school (which includes bullying children due to disabilities), and have seen TV shows that include disabled children, such as Sesame Street, Little Bill, etc.
You would think by now we would be use to people staring at Kate and some days we handle it well, but other days, it is all I can do to not scream at people when I see their eyes fixated on her. How can we teach people to be polite and not stare? In my opinion, it starts at home.

1. Parents need to teach their kids that staring at someone is rude. They need to teach their kids that people are different. Some people are tall, others are short; some are skinny, others are not; some walk with their legs, while others have prosthetics or use a wheelchair. You don’t need a special moment to teach this common courtesy. There are children books that show diverse characters, TV shows, etc. When you are walking the grocery store, at church or just out and about, you can take a moment to show how beautiful people are and how their differences make them special.

2. When a child says something like “what’s wrong with her”, don’t get embarrassed and try to shut them up. Rather, answer the question. Make it a teachable moment. While you are at it, show them how they are more alike than different. Say something like “look at her shirt, it is pretty?” I remember walking through a NYC hospital with Kate. We were staying for a few days so we had suitcases. A small child, probably not even 3, said to her mom, “what’s that?”. Rather than the mom answering the question honestly, she pulled her daughter closer to her and said “those are suitcases.” I don’t know who she was trying to fool, us or herself. We both knew the daughter was asking about Kate and her chair. She wasted a moment to teach her daughter. She didn’t do her daughter or us any favors by not answering the girl’s question. I am not offended when someone inquires about Kate. I would rather a child ask than stare. I am offended when a parent doesn’t answer the question and/or ignores the question.

3. Adults and older kids, I am offended every time you stare. You know better. I have a hard time believing Kate is the first person you have seen in a wheelchair. If you are curious about Kate, please ask. Come up to me and say “Your daughter reminds me of… Can I ask about your daughter?” or “your daughter is beautiful. What does your daughter have?” (BUT DONT ask me what is wrong with her. That’s offensive. Nothing is wrong with her. She is perfect.) I will gladly share with you what disease Kate has, direct you to websites, etc. Awareness is key!
I beg of you, stop staring and start educating your kids on people like Kate.

While am at, please stop using the word retarded to describe things and people that aren’t. I know I have posted on this before but people, including friends, still say things like “that is so retarded” or “they are such a ‘tard.” It hurts, its offensive and it makes me want to scream in your face. I am over asking people to politely to stop using the word in front of me. I will no longer be silent when you say it in front of me. I will be kind but I will ask you to not say it.

Learn as much as you can while you are young, since life becomes too busy later. ~Dana Stewart Scott

Concerns, Questions and Answers…

I started to write an update the other day and was interrupted when Kate had a seizure. The last few month have had a lot of ups and downs. The last two weeks have been the most challenging we have had in a long time. For some this may come as a surprise because when asked how Kate was doing I responded with “good”. Not because I was lying but because I know things could be worse and because 90% of the time “good” is true. We are not a family that focuses on the 10%. Thursday of this week was the breaking point. I have been calm, patient and optimistic that we would find an answer as to why Kate is having status seizures and why her two emergency drugs do not stop them. However, we were in the ER for the fifth time since March 22 because of status seizures. To make matters worse, on Tuesday Kate had two grand mal seizures. Those are the first two grand mal seizures Kate has ever had. Something isn’t right. We need answers and we need them ASAP. We have increased seizures meds several times since March, added a hormone pill because we thought maybe the seizures were due to her monthly cycle, and have added and increased her emergency meds. I am tired of having our efforts to give our daughter a life filled with quality taken from us. Monday we have an appointment with Kate’s neurologist and I am hoping we can get things figured out. Do we need to add a drug and remove a drug? Has her body become tolerant to one or more of her seizures drugs? Do we need to do an EEG?

Along with the seizures, we have had issues with Kate’s legs and pain. Not pain in her legs. Her legs and pain are two separate issues, I think. When Kate is sitting or lying down her legs will get very stiff and stick straight out. They shake and it is very hard to bend them. I believe it is spasticity. I also believe it is treatable, but we have to decide if the med they use to treat it is necessary. Meaning, will it cause her to be sedated; is it necessary to put more meds into her system, etc.? Kate has had two issues with pain since mid-May. I am not sure what is causing the pain. Tremors? Constipation? Nothing but her brain is sending signals so she thinks she is in pain? Something else? More questions and mysteries. More answers needed!!

Oh yeah, we are also having issues keeping Kate’s body temperature at a comfortable temp when she is outside. Even on a nice spring day, if she is outside for a period of time, her legs and face get very hot and the only way to cool her down is to bring her back inside. It takes her body a long time to cool down. We try to keep her in the shade but if the temperature is more than 70 degrees she over heats. It’s even an issue if we keep a blanket on her in the house. If she doesn’t have a blanket on, she is cold and has goose bumps, but if we put a blanket on her she gets very hot. We have been using a sheet when in the house. It seems to keep her at a comfortable temp. Some families use a cooling vest for their kids when they go outside. We haven’t needed anything to control Kate’s body temp until recently, so we may need to look into the vest for this summer.

If you could please join me in prayer for Kate’s appointment on Monday – answers for her seizures, pain, body temperature, etc. I would GREATLY appreciate it.

We are in full blown summer mode. We are preparing for VBS, Lauren and Kennah’s 3rd annual lemonade stand (all proceeds are split between Kate’s medical fund and the BDSRA – June 10), our mini-vacation to Washington DC and the Batten Conference. We recently bought an outdoor swing for Kate. When it arrived we thought it was so big and would last us a few years. We were shocked when we realized it fits Kate perfectly. Gulp… Hopefully she will get two years out of it because she loves to swing. She loves the breeze on her skin, the warmth of the sun and the motion of the swing. She falls asleep in the swing.

We have a new night nurse. She was working every-other-weekend, but we like her so much we asked her to work every weekend. She accepted! We are thrilled to have her as Kate’s weekend nurse. Our struggle is always with the agency. They have a lot to learn about how to treat their employees! When nurses leave, it’s not because of us, Kate’s care is too much, etc. It’s always because they hate the agency. I am praying this nurse has a better experience and stays with us for a long time!

This weekend is Batten Disease Awareness weekend. I hope to share pictures, websites, and information as the weekend progresses. Please feel free to share Kate’s Facebook page (Holding Onto Kate) and / or her website. The more we share, the more people learn about the kids and the disease, the more people care, the closer we are to a cure. Those that know, care and support the research efforts bring us one step closer to a cure! Please help us help the kids who are still fighting and those yet to be diagnosed.

The Batten Conference is the end of July! This year it is in Columbus, Ohio so we helping as much as we can. I am procrastinator so I am a being challenged to stay on task, especially when I am planning VBS, applying to college, etc. (I always over commit myself but I do better when I have a lot going on, or so I say.) The BDSRA has some great things planned and we are looking forward to the conference. Each year we look forward to seeing the families and reconnecting with those who walk the same walk as us (or have walked the walk). We are beyond blessed to be surrounded by a supportive family, friends and community, but the only people who can understand what we deal with on an emotional, physical, mental and spiritual level are those who live with the disease 24×7 like us. So to be surrounded by those families is an amazing feeling. They don’t flinch when we turn on the suction machine, stare when we tube feed or need to change Kate’s diaper, etc. It’s just comforting. It is also nice to get advice face-to-face from the other parents. And, of course, we love seeing the kids! It is great to see the kids and know they made it through another year, but it is sad to see those families that now have a batten angel and know their heartache is overwhelming, deep and beyond painful. Lauren looks forward to reconnecting with the siblings. I think she enjoys being around other kids who know what it feels like to have a batten sibling. They understand the pain, the concern and all the other hardships she faces on a regular basis. I think for a few short days she feels “normal”. I can’t say enough good things about the conference.

Thank you to those who text, message, call and reach out to us in various ways. We appreciate your concern, prayers and words of encouragement. Some days when the darkness of the disease is getting the better of me, I receive a message from a friend or follower, and realize we aren’t alone and we can keep going.

Deuteronomy 31:6 Be strong and courageous. Do not fear or be in dread of them, for it is the LORD your God who goes with you. He will not leave you or forsake you.”

5K Results and Update on Kate

Over the last few days I have thought about what I would say to everyone who supported the Team Kate 5K last weekend and I have struggled to come up with the words. Over the last few years there have been 3 benefits, two 5Ks and 3 lemonade stands and at or after each event we have expressed our gratitude, feelings, etc. So as I sit here looking at my computer screen I my struggle with what to say and how to say thank you in a way that doesn’t sound repetitive and hollow. I wish that the words had come to me over the last few days but I find myself speechless. Each time we start the process of scheduling an event we pray that people will have an interest in the activities that we plan. Whether we donate $100 to the BDSRA for research or more we advance the cause for a treatment and cure. So I say with a genuine heart, I say with every fiber of my soul, I say with love and humbleness, “THANK YOU”. Thank you to those who helped plan (Kaye Heilman and Crystal Dunlap), thank you to those who sponsored the event, thank you to those who volunteered at the 5K, thank you to those who donated raffle/silent auction/bake good items, thank you to those who couldn’t make it but donated money and thank you to those who ran, walked and/or biked at the 5K. Without each of you the events wouldn’t be a success. Without you researchers would have less funding to find a cure. I hope one day we can repay each and every one of you back in some way. I hope you had a good time at the 5K. If anyone has suggestions on how to improve the 5K, please let us know.

Attached to the bottom of this post are the results from the race.

Update on Kate

Since March 22 Kate has gone to the emergency room three times for seizure activity. Kate goes into status, meaning her seizures happen one after another with little to no break in between them. We administered two emergency drugs at home but her seizures continued. In the ambulance they administer valium via an IV and they stop within a few minutes. We have avoided the ER two times, which we are thankful for, but we are overwhelmed with trying to find the right combination of maintenance meds, emergency meds, etc. so we can always avoid the ER or better yet, avoid her going into status. Brock and I believe that her seizures are due to her changing hormones cause by puberty. We have asked her neurologist to put her on the pill. We are hoping the pill will regulate her hormones and either stop her from going into status or help us find a pattern on when she will have seizures so we can increase her meds during that period. Since we aren’t doctors we aren’t sure if this is a good plan or make sense medically, so we are relying on the doctors to help us determine whether or not this is the best course of action. We would appreciate your prayers for this entire situation.

A few weeks ago we received machine that helps keep Kate’s lungs clear by doing percussions on her chest. It seems to be helping a lot. She still has congestion and requires suctioning and at times you can hear the congestion settling into her chest, but the machine has lessened how often that happens. The machine, for whatever reason, is VERY expensive and we were told that insurance may not approve it. We were under the impression that it was approved prior to delivery, but that wasn’t the case. We were very concerned with we would pay for it. Yes we have money in Kates medical account but this would take a large chunk of the money and we want to ensure we spend the money wisely. We came home from work on Monday and waiting for us was a letter from our insurance company. Anxiously I opened it and read it. It was approved!!! Thanking God for this amazing blessing!

Overall Kate has been doing well and seems more responsive over the last week. Seems weird that she is smiling more and is more alert since we increased on her meds again. Usually that makes her more tired and withdrawn. So thankful for her smiles and the occasional “talking” we have heard lately. Its amazing how much you can miss someone’s voice.

Over the last two weeks five children have passed away from the disease. It breaks my heart to know that five families are mourning and learning to live without their child. Please keep these families in prayer.

Again, thank you to everyone who supported the 5K. Thank you for helping make a difference not only in Kate’s life, but in the lives of many children. One day there will be a cure. One day this disease may not even exist. Thank you for your support each and every day. Your words, cards, prayers, and random acts of kindness are never taken for granted and are always appreciated.

5K Results

Happy Easter!

There is reason to celebrate today – Westley, after six weeks in the hospital, is home. Westley spent 4 weeks on life support and truly is a miracle. He fought so hard and tonight he is resting in his own bed. When I think about all he has been through, I am reminded at how amazing our God is. We were fortunate enough to visit Westley, Tracee and Willie a few times. I know they received a lot of visitors, and we were so thankful they allowed us to be one of them. Westley is proof that prayers are answered and God is the ultimate physician.

The last several weeks have been hard on several Batten kids. Many have fought illnesses and seizures. Some days it is overwhelming and heartbreaking to scroll Facebook and see all of the sadness, scariness and loneliness this disease causes.

Kate has been dealing with her own issues lately. She has had a lot of congestion in her chest and at times isn’t able to cough it up and out. Her cough is typically strong and productive, but by the end of the night her energy and strength is gone, so her ability to cough productively is diminished. There have been a few nights where her congestion has settled deep into her chest and into her left lung, causing her O2 levels to drop and her pulse to increase. It is hard to know what to do when this happens. We can go to the hospital but am not sure what they would do that we weren’t doing at home. We placed oxygen on her, positioned her so her mucus could drain, and did percussion. Eventually she was able to clear her lung and her levels improved. In an effort to help keep her chest clear we saw her respiratory doctor and requested the coughing vest. The vest does percussion on her chest and loosens the mucus so she can cough it up and out and keep her lungs clear. So far it is working well and she is tolerating it. Lauren tried it on for fun and said it wasn’t too bad – comfortable and didn’t cause any pain, upset stomach, etc.

Kate also has had issues with her seizures. This isn’t new information but the reason for them is unknown. We thought it had to do w/ her monthly cycle, but it has become clear that the two are not related. On March 22 she had several seizures within three minutes so we administered her new emergency drug, Versed, and it caused her to stop breathing and her face to twitch uncontrollably. We immediately called 911. Within 45 seconds she was breathing and her face was fine but she was still seizing. In the ambulance the EMTs gave her an IV of Valium and she stopped having seizures. Based on her response to Versed we were given a higher dose of Distat to use if she had another cluster of seizures. On April 9 she started having seizures at 6 am and at 630 Brock gave her Distat. At 8 she was still having seizures, so I took her to the ER. As soon as they called us back her seizures stopped, therefore they didn’t give her any medicine. We were released shortly after arriving. This past Monday, April 14, we saw her neurologist and she increased one of her daily seizures meds. We are hoping this will stop her from having clusters of seizures, thus keeping us out of the emergency room. We also discussed what drugs we can use if she needs an emergency drug. Once again we were given an increase in distat and told to use it and if it doesn’t work, call 911 and administer Versed. The reason we are to call 911 before giving it to her is in case she has another negative reaction to it. The doctor trained us on how to administer the drug and believes that the changes she recommended will make the drug safe for Kate.

Once again we have a new nurse. The agency we are working with seems to have some internal issues, therefore it has high turnover. It is frustrating because we really want a nurse that will connect with Kate, stay long term, learn what is normal for her, what are seizures vs. tremors, and become a valuable member of her primary care team. The last several nurses have been incredibly nice, so we were sad to see them go. Our new nurse is very nice and I am praying she is with Kate for a long time. We also had to let go of a nurse who has been with us for two years. She worked every other weekend, but over the last year her attendance hasn’t been acceptable and we caught her sleeping. We tried to work it out and be accommodating to her needs, but eventually we had to let her go because we need someone who will be here when scheduled. We rely on our nurses; therefore we need people who are reliable, knowledgeable, and compassionate.

We are in the final weeks of planning for the 5K. May 3rd will be here before we know it. We are excited and hope it is a great time. Its not too late to sign-up! Forms are found on this website. If you cant run the day of or aren’t local, you can be a virtual runner. We will also have a few raffle items, 50/50 drawing and bake sale. If you wish to help at the event, please let me know.

I hope you all have a happy Easter and find time to celebrate our Saviors resurrection.