Where We Go From Here

We saw Dr. Guttman today at the Buffalo Pediatric Multiple Sclerosis Center of Excellence. We came away with a lot of information and confirmation of things I have known deep in my gut for quite a while. First off, I am so grateful that Dr. Guttman saw us today. We were her only appointment as she does not see patients on Friday's, but because of the nature of Sarah's disease and the peculiar nature of its brutality on her eyes she took us in with care. I liked her right off the bat. I liked her personality and I liked the wealth of knowledge she seemed to possess. She was thorough and good at explaining in layman's terms what Sarah does and does not have and why. 

Here is what she explained to us:

1.) Sarah does NOT have Multiple Sclerosis. The type of lesions on her brain are not consistent with MS. Another indicator that she does not have MS is that MS patients with optic neuritis only have paleness around the edges of the discs in their eyes. Sarah has paleness in the entire disc in both eyes. Also, Sarah did not have any Oligoclonal bands in her spinal fluid. 

2.) Sarah has NMO, but not a typical NMO. Most NMO patients have both optic neuritis and transverse myelitis. Very rarely do patients have just one or the other. Sarah is one of those rare cases because she has not had any lesions show up on her spine (which is what causes the transverse myelitis) and because she has not had any symptoms of new lesions forming such as loss of leg and arm movement, loss of bladder control, etc...Dr. Guttman diagnosed Sarah with a type of optic neuritis called: CRION (Chronic Relapsing Inflammatory Optic Neuropathy). There is very little information out there on this disease, and what I did find confused me a bit because the literature led me to believe that you couldn't have CRION and NMO  - that NMO had to be ruled out in order to be diagnosed with CRION. I double checked with Bob to see if he understood Dr. Guttman the same way that I did in terms of diagnosing Sarah as being on the "NMO spectrum" (her words) and having CRION and he did. So, I don't know if knew research has shown that a patient can have both or what, but this is what Dr. Guttman diagnosed her with, and this woman knows her stuff. 

3.) Sarah's vision loss, for the most part, is permanent. She had an eye test done today while at the center that shows the amount of nerve damage and atrophy on her optic nerves and the discs and it is pretty bad. Dr. Guttman told us that the damage that is showing on this test is permanent, and that our job now is to save what we have left. She may have slight improvement, but she will be considered legally blind from here on out. (Another test she had showed vision at 20/800 in each eye - which is a slight improvement in her right since she had no vision in that eye just a couple of days ago. This is the type of progress that we can expect, but long gone are the days of 20/100 or better vision. 

So, what does this mean for Sarah and for our family? For Sarah, this means an increase in her medicines and IVIG every 3 weeks. The point of doing IVIG so often is to keep the NMO at bay. Studies have shown that maintenance IVIG can help prevent relapses in NMO patients. For the time being, Sarah will be going to the infusion center every 3 weeks for a 4 day stint of IVIG. This will last at least 6 months. Her dose of vitamin D was increased to 50,000 iu's weekly. Her prednisone was upped to 10mg every other day. Her Imuran is increased to 150 mgs daily, and her vitamin B is going to have to be administered by injection once a week for 2 months and then if her vitamin B levels increase she can move to the sub-lingual form. 

As for her mental spirit - she is doing rather well. She can see shadows and some things that are in a close range, so she has been able to visit with a friend or two since her last relapse. She cannot ride her bike on her own anymore, and has to be accompanied everywhere, but that is a small price to pay to be able to ride to a friend's house. At least she can still ride her bike.

 She is such a trooper, and I admire her strength. Her attitude is that she will just get used to her eyes the way they are, and then she will move on. She will learn to adjust to them, and make her life her own even with the visual deficit she has. Kids are pretty cool.  My girl? She is REALLY cool. 

Dr. Guttman was very good at explaining that with NMO there is no one way to treat all patients. The disease is different in everyone and so all that doctors can do is go by what works for most patients, and then alter the treatment as needed for each individual. 

As for our family, we will learn Braille. I found a great on-line braille bookstore and have already found the first book I am going to purchase. We are also going to seek out counseling again - it has been difficult to acquire counseling either because the facility does not offer the type of counseling we need or the facility is not returning my calls, but either way I am going to step up the efforts on my part to make sure we get the help we need. Josh, in particular, has been a mess over this latest relapse. My heart breaks for him in a million pieces. He has volunteered himself to be his sister's keeper, and I while I am honored that I have a son who loves his sister so much he is willing to take on this huge responsibility I want to make sure that it is not too much of a burden for him to bare. 

I am doing better. My husband and I make a great team, and he has been amazing throughout this last couple of weeks. He carried me last week and this week when I needed him to, and he didn't try and fix anything. He just let me be, and offered a hug and a shoulder to cry on. I am sure I will return the favor for him when the time comes. 

We will be selling our TV/DVD player because we have made the decision that if Sarah cannot watch TV we will not be watching TV. We want to be a unified front, and this is one simple way we can support Sarah and show her that we are one unit. (So, if when my children come over to your house all they want to do it watch TV you will know why.) 

We, as a family, will make it through this. There will be some amazingly hard, difficult, gut-wrenching times ahead - I am no fool, but I KNOW that we will be okay in the end. In spite of all of this heart ache we will find a way to thrive, and to allow our daughter to thrive. This is just a speed bump. That's all. Everyone has their tragedies - my family is no different than anyone else. Having heartbreak is part of the human experience. It may not be a sick child that you are dealing with, but maybe it is a divorce, or an affair, or the death of a loved one, but we ALL have our tragedies to live through. It is what we make of those tragedies that define who we are as people. 

When things upset me I will allow myself to feel what I feel at that time, but I will not live in that deep dark place. Instead, I will stand up, brush the dirt off of myself, and be the example my children need me to be. 

Things are looking up. Even if the view is not what I imagined it to be. It is the view I was given, and I will make it the most beautiful view I have ever seen - for me and Bob, but most importantly, for my children. 

So This Is It

Saw two of Sarah's doctor's today. Was told it is doubtful  that she is going to regain any of the vision that she lost this past week.

Learning Braille was encouraged by her medical team. 

I am not ready for this new life. I have no idea what I am doing anymore. 

I am shell shocked. 

Life As She See's It Right Now

Back from our infusion this morning, and my girl is back in bed sleeping. She is so tired this time around, and I cannot help shake the feeling that this round of vision loss has broken her in a way that she will forever be changed.

It is time to start thinking about real life changes. Things like Braille and altering the life as we now know it so accommodate a child that can no longer see very well. Oh, how I hope in a couple of weeks I can write you a post about how well Sarah is doing and how much she can see, but I cannot even imagine that day right now.

Her Akron Children's neurologist stopped in while Sarah was getting her infusion and we talked about how steroids are no longer working. I told him that it seems pointless to keep on putting her through this treatment when it is futile. This go around is the 11th time she has been on a high dose of steroids. 11 times in 15 months. Something has to give.

Our doctor mentioned doing another spinal tap and an MRI. I agreed wholeheartedly. I know that her Cleveland Clinic neurologist wanted to wait for Sarah to come off of her steroids before doing either of those procedures for fear that the steroids would skew her results, but her Akron Children's neurologist seems to think that she may never come off of them, so why wait? I tend to agree with him.

There are so many things going on in my life that I had wanted to share with you. My garden, my weight loss, etc...but none of those things seem important right now. I hope there will come a day that I feel differently.

God, this life is so hard. I wish there was an opt out button sometimes. A button that will message up to God, and say, "Hey. You picked the wrong girl. I am not strong enough to do this anymore. I cannot carry the weight of this disease anymore. I cannot watch my daughter deteriorate anymore. I think you have me confused with someone else. Someone stronger, wiser, more able, more mentally capable. Someone with more fighting spirit." I am tired of fighting for a life that so many get to have every day. I am tired of trying to be normal and feel normal.

I wish I could go to the highest mountaintop and just scream my lungs out. Just scream until there is no sound left in my voice. I don't handle prolonged crisis very well, as you all are witnessing. I don't know how so many others with children that are sick without any hope for a cure can carry on. I have got to find a way to thrive even in these circumstances. I am just so tired of fighting....

This is what Sarah see's through her eyes:

(All images taken from google.com)

This is what Sarah is currently seeing out of her eyes. (This is 20/800 vision)

This is the same picture as above as you and I would see it 

This is 20/800 where there is better lighting. 

When her vision improves this is what it looks like to her. This is as good as it has been in the past 2 months out of her right eye. 

As She Sleeps

As I sneak in to check on her I see my little girl asleep in her bed clutching her blankets. My exhausted sweet little one rests her body in the comfort of her home in her own room in her comfortable bed. This hospital stay has worn her out, and I feel a sadness creeping into my heart that I have not felt in a long time.

She came home from the hospital a little over an hour ago, and went immediately to her room and snuggled up on her bed. I found her there curled up in a ball with her eyes closed, not yet asleep, but not fully awake either. I quietly told her to rest body, and promised her that when she woke up we would go to get that hair cut she has been asking for for a while. She did not fight her body, and allowed it to take her to a dreamy land filled with hope and peace and comfort.

I watch her sleep and I wonder if she is dreaming about a life with no vision issues. I wonder if she is dreaming about what it is like to be happy and carefree. To only have to worry about the average 9 year old trials. I hope that her sleep is restoring her body and mind and that when she wakes up she will feel refreshed and renewed. She seems to have lost her will to fight this time around, and I know in my heart that if she loses her will to continue this battle with her body that I cannot fight it alone.


Dr. Locastro made it in to the hospital today. God love that man. He didn't have to come by as we have an appointment with him on Tuesday, but being the wonderful man he is he took time away from his own family to make a special trip up to the hospital just to see Sarah. After talking with her and going over some of his routine testing with her it appears that there is more damage in her right eye than her left. She cannot see at all out of her right eye. No vision what-so-ever. Her left eye is not fairing much better with her vision coming in at 20/800. Each day her vision is getting progressively worse, and I fear that this is taking a huge toll on Sarah's mental health.


She is so sad right now, and I find myself unable to help her more so than any other time. I am fearful that our family - that Sarah - is going to have to constantly live on high alert with no sense of normalcy ever to be found. Sarah has relapsed every month since January, and as each month goes on I find myself struggling to put the pieces of our family back together even more than the month before.

Josh has taken this relapse especially hard. He always does, but this time there is a new element of panic that is settling into our psyche as we all wonder if she is ever going to recover her vision. Nothing is working anymore - maybe it never really did. Not a higher dose of Imuran. Not repeated steroid exposure. Not plasmapheresis. Not IVIG. Nothing.

I am so sad today. It is an aching sadness that only those who have children that are sick can understand. It is the kind of sadness that resonates in the deepest parts of oneself. I feel defeated. I feel betrayed by life.

I have a little girl - a sleeping beauty - downstairs waiting for Life to kiss her on her lips and give her the happy ending she deserves.  May she be granted this ending - as I can think of no better recipient.

Another Relapse

Sarah is currently in the hospital after having another relapse while we were in NY this past week. We left NY early yesterday morning so that we could get her admitted to the hospital yesterday afternoon. She is beginning (another) round of steroids - 5 days total. She will receive her first 3 days of treatment in the hospital and then her last 2 days on an outpatient basis.

I am grateful that we are headed to Buffalo on Thursday night for our Friday morning appointment. I am hoping that one of the doctors that we will be meeting with to discuss Sarah's case will have some new information for us in terms of nailing down an actual diagnosis. I am also hoping that someone will have some news that will allow Sarah to have some reprieve from these constant relapses.

To say that I am heartbroken right now would be a complete understatement. I am having a really bad day especially after having taken the kids to see her this afternoon and hearing her tell me that her eyes are getting worse and not better.

I knew this was going to happen. I just kept pushing the gut feeling away that a relapse was imminent by telling myself that I was just being paranoid. I started to see the signs as soon as we got to NY, but Sarah kept telling me that she was fine. As the days progressed and the signs became even more evident that something was amiss I kept pushing the signs out of my mind because Sarah kept insisting that she was okay. Well, it turns out that she was not okay. In fact she was far from okay, but she didn't want to have to leave NY early and she didn't want to miss a friends birthday party today so she lied and told me that everything was fine. She was angry with me on Wednesday for having her dad notify her neurologist at Akron Children's that something was amiss, but in the end listening to my gut was the right choice. Her vision is terrible right now. Worse than it has been in a long, long time.  She cannot read, she cannot see more than 5 feet in front of her, and she cannot see the details of things closer. She confused her 12 year old brother (who was sitting down on the ground) with her 1 year old cousin. She is legally blind in both eyes right now and her vision is getting worse.

I am at a loss right now. I am angry and sad and tired. I don't care today that there are some who have it worse than me and that I should be grateful for what I have. I am not grateful for what I have today. I am jealous of all that I do not have. I am jealous that there are so many people out there with children who are healthy. I am jealous that there are mothers and fathers out there who do not know what it is like to know a wing full of nurses by name and who know your daughter by name. I am jealous of the parents who have never had their child try and find them in a room where there was no one else but them, but their child could not find them. I am jealous of those who do not know what it is like to have doctors tell them that their child is an outlier among outliers. I am jealous of those who have a name for their disease and an effective treatment plan.

I am most jealous though, of those who do now know what it is like to wake up almost every day and to wish that their reality was just a bad dream. Life is crushingly hard right now. The light that I was thought I glimpsed at the end of the tunnel was nothing but a dream. Instead I am left grasping for the dream that some day - one day - life will not be so hard for my family.

Just Me and the Kids

I am going on a vacation with the kids this weekend. Just me and my brood. No husband. We will be traveling about 8 hours by car to southeast New York to visit with my extended family. The kids are super excited as am I.

My family lives in the Hudson Valley area, so there is plenty to see and do. We plan on taking several trips while there in order to explore an area that is rich in history. I will be sure to take lots of pictures - although Bob is the real photographer in our family - so I am not sure of the quality of pictures you will get from me. Nonetheless, I will be showing you our week when I get back.

I would be lying to you if I said that I wasn't nervous about being so far from our medical community (and Bob) given Sarah's medical history. Because we are going on 5 weeks now with no issues I am on edge a bit more than normal waiting for the proverbial shoe to drop. She did wake up yesterday morning and told me that her eyes were not doing well, but then her issues appeared to have cleared up by the afternoon. I am not sure if this was a fluke or if that issue was a preview of a relapse to come. Either way, I am constantly looking for signs of visual deterioration. I wonder if I will ever be able to get to a point where I can just breathe and relax when it comes to Sarah's health.

I am looking forward to being able to have my kids all to myself this trip. No need to share them with Bob or with their friends. As they get older and older, I am not sure how many more opportunities I will have to steal them away from their lives so willingly. Soon it will be jobs or sports or any other number of things that will not allow us the luxury of just being able to pick up and leave on a whim.

I really love hanging out with my kids. They are growing into fun people who I truly enjoy being around.  I love doing anything with them that allows us to grow our bonds as a family. It will be a great time. I hope they end up cherishing this trip as much I plan on doing so.

As the great Billy Joel says, "I am in a New York state of mind..."

Just Checking In

All is well in our home. Been busy doing this and that which hasn't left much time for this space. I am okay with that though as there is a season for everything. Right now, I need to spend some time doing other things.

Here is the low down on everything in our home:

I am doing fabulous with my healthier eating. I have held true to my word that I would not weigh myself for 3 full weeks. It darn near killed me this week because I was doing so well, but I also knew that if the scale did not show the number of lbs that I felt like I had lost that I would have been more prone to giving up. I just keep telling myself to trust in the process, and it will not fail me. I started to meet with Bob again (Bob Harper that is - my exercise guru) and I have found myself both love and loathing him. I love him when I am done working out, but loath him all the while I am sweating like a pig asking my body to do things it hasn't done in years. Once again, I keep telling myself to trust in the process - eat well and exercise my body and it will not fail me in losing this excess weight. I think that 6-8 lbs in 3 weeks is completely realistic. Time will tell...

The boys are doing well. Enjoying their summer immensely. They are having a good time watching the World Cup and hanging out with their friends. 

The girls, too, are enjoying their summer albeit with a bit less freedom than the boys - although that is to be expected as they are younger than their older brothers. 

Miss Sarah is holding steady. Her eyes have not made any major improvements, and I worry that she has been on a bit of decline the last few days. She has told me that she is comfortable with where her vision is, so I am trusting her to know herself well enough to know when she needs to seek help. We have successfully made it a full month without any eye emergencies, and I am very grateful for that. I am hoping to make it the whole month of July without being admitted into the hospital. It would be the first month since last December when she has not had to be hospitalized/used the infusion center at least once during the month. 

We have an appointment with at the Neurological Institute in Buffalo on July, 25. (This is one of the Pediatric MS Centers of Excellence.) We are going to be seeing a neurologist who specializes in pediatric autoimmune diseases, specifically MS.. We are hoping to get some type of confirmation as to which disease Sarah has and also to see what can be done to help stop the deterioration of Sarah's vision. (She is currently still taking 5mg of steroids every other day, and I truly believe that this is what is keeping her vision loss at bay. She cannot continue to be on steroids forever, so we are looking for an answer as to why she keeps relapsing what we can do to prevent it.)  Sarah also submitted a saliva sample for genetic testing to see if she carries a specific gene for a disease called Leber's hereditary optic neuropathy (LOHN). We should get the results back in a couple of months. 

I am planning on taking the kids to NY this weekend to visit with my family and to take some pretty cool day trips. I am planning on taking them in NYC for the day since our overnight trip got cancelled with Sarah's May hospitalization. We will be staying in NY just shy of 1 week. 

Hope you are having a wonderful summer!

In The Garden

This is what my garden looks like now.....

Squash - both summer and zucchini

Tomatoes

My first pepper

My first tomato

Cucumber

Zucchini

Pumpkin

By the house. 

A crazy young lady that I found in the yard....

Wednesday's Inspiration

Quote taken from: www.heartworkandsoul.com