Her eyes are fine..... rheumatologist appointment on Monday.

Hi everyone--

Just a quick update for this early Saturday morning. Grace had her opthamologist appointment this week, and her eyes are perfect. The concern was that given the proximity of the lesion to her eyebrow that somehow the muscles of the eye had been affected. Another concern is that such massive doses of steroids can cause cataracts and glaucoma. Fortunately, none of this has turned out to be the case.

For now, our attention has turned to Grace's First Communion tomorrow. My sister and her family and my parents will be here in a couple of hours, and I am really looking forward to it.

We have an appointment with the rheumatologist on Monday. I'll post that afternoon with any updates we may have.

Thanks for your cotinued well wishes!

Love,
Sabrina

We're just keepin' on keepin' on!

Thanks to all who have called or emailed asking how things are going. They are going fairly well, and we have settled into somewhat of a routine. Grace is going to the hospital less often right now. I've really discovered that the treatment of this type of condition can be a little bit of a moving target. She does not go to the hospital this week or next week. Instead, I am giving her injections at home. She still has many of the same side effects, but it is easier to not have her miss school, or spend those hours at the hospital! The injections at home are only the methotrexate--not the steroid, which is the more difficult drug on her body. Her weight gain is at about 10 pounds right now, but by far the biggest effect we're noticing is her emotional rollercoaster. As Rob so accurately put it, "it's like we have a 14 year old". If this is what puberty's going to be like, BEWARE!!!!

Thank you to those who have brought the Wednesday gift baskets. Please know that even though we're not going to the hospital as often right now, that the items are still coming in very useful because the day of the injection--even if I give it at home--she still feels a little run down, and often likes to play a little more quietly.

We have an opthamologist appointment on Tuesday to make sure that the eye has not been affected. It takes 3 months to get into see the opthamologist at Children's Hospital, so that is why we are just now going to see him about this. They have told me to expect a 2 hour appointment!!! Our next follow-up with the rheumatologist is the following week, and we should have a much clearer indication of the future course of her treatments. For right now, her next IV infusion isn't until June 12.

Though I don't think they read here, I'd like to publicly thank Grace's teacher and the secretaries at the school for being so great in working with her and her changing needs. It has been a huge help.

Right now, we are focusing our energy on something positive: Grace will receive her First Holy Communion on June 8. I am really looking forward to seeing all the family that will be coming for the event. I will be sure to post pictures!

I will also post an update after our upcoming doctors' appointments.

Thanks to everyone for your thoughts and prayers.

One more update and a couple more pictures

I would like to take a minute to acknowledge all of Grace's aunt's and uncles, Jon and Eileen, Brian and Molly, Tad and Ali, and Jeff and Adrianna for going in together and purchasing her a new MP3 player to take to the hospital with her. Due to some shipment problems, it just arrived this week, and we just gave it to her this morning. Following are a couple of pictures of her opening it. Thank you so much for thinking of her. We appreciate it more than you will ever know. She is lucky to have a family like all of you.

Some updated pictures

Good Saturday morning to all of you!


A relatively quiet, uneventful week here in our home--at least as far as Grace's condition goes. The rest of our life was it's standard, chaotic state. Grace did not have an infusion this week, which was nice. I gave her an injection of methotrexate here at home, which was traumatic for her, but we got it done. This Thursday will be her next infusion at Children's.


I also found a First Communion dress for her! (Actually, my mom did. She is THE BEST at hunting clearance racks and finding bargains.) I have bought/borrowed two dresses for Grace, but because of the weight gain caused by the steroids, neither of them fits anymore. Understandably, I didn't want to spend alot on yet another dress, but my mom found one that will work perfectly!



I thought you might enjoy seeing a couple of updated pictures. The first one shows the lesion--you can see that it is less inflamed and that the color has faded to a more greenish/yellowish color. The surface of it is also much more smooth and less "stretched" looking for lack of a better description. The second one shows that Grace has gained a bit of weight--you can see it in the fullness of the face. However, she still has a great smile--when she chooses to show it!



The dermatologist we met with last week did say that the hair loss is likely permanent, which is too bad, but not unexpected. The above picture does not really show the area, as Grace is pretty self concsious about it. She also mentioned that plastic surgery would be an option to consider when she is a little older. Obviously, that is not something we will consider while she is still growing.

I'd like to take a moment to wish my mom a very happy Mother's Day--I hope it is a fabulous one. And to all the other mothers reading this post, I hope your day is a great one as well!

Thank you for your continued thoughts and prayers.

Sabrina

End of a long week . . .

Hi everyone--

Sorry for not updating before now. It has been a long week. I am having dinner with my friend Anne tonight, and I must say that I am looking forward to a quiet dinner and a strong drink!

Grace had her infusion on Wednesday this week. It was another tough one. For some reason, she was shaking so badly that the nurse "blew the vein" when trying to start the IV. This isn't as scary as it sounds, though Grace is sporting a nice bruise on her left hand. It simply meant that we had to try again on the other hand. Because of Grace's anxiety, they had a nurse who specializes in "hard pokes" come down to start the IV. I had hoped that once the line was in and the meds were hung that Grace would relax, but unfortunately that was not the case. Once again this time she was very nervous and very nauseated--two conditions which seem to make the other one worse. She spent much of the infusion with her head over a basin, though thankfully only vomited once. They sent a specialist from Child Life Services (or something like that) to come up and talk with Grace and help her calm down. She practiced deep breathing with Grace, and also gave her a cotton ball saturated with peppermint oil. Peppermint is supposed to help nausea, and inhaling it is usually helpful to many of these patients. Unfortunately, Grace wanted nothing to do with these ideas, and though she did participate, I could tell her heart wasn't really in it. In the end, we ended up giving another dose of Zofran, which once again worked wonders. I must say however--though this probably makes me sound like a bad mommy--that Grace had it in her head that only the Zofran would work, and wasn't really OPEN to trying anything else. We don't want to get into a situation where she is becoming dependent on the Zofran, hence the reason we tried other things first. Oh well, we'll try again next time.

Then on Thursday, Grace had an appointment with the pediatric dermatologist. By this point in her treatment, Grace has seen quite a few different doctors, and she had clearly had enough by yesterday's appointment. Let's just say that her attitude, demeanor and behavior were a little "challenging" to say the least. We'll just leave it at that, shall we?

Today, Grace leaves for two days on a Brownie camping trip. She is nervous about being homesick, but I think we can both use the break. I've got all her medicines sent along, and one of the leaders is a good friend of mine (hi Heather!), so I feel very confident that she will do just fine.

Grace is up to about 89 pounds now--which is a gain of just under 10 lbs. Medically speaking, she's doing great. Emotionally, this has been a hard week. I'm not sure if it's withdrawal effects from the methylpredinisolone (we didn't do an infusion last week), or just all of this starting to take it's toll, but this week has been the most challenging in terms of behavior--emotional outbursts, obstinance, temper, etc. As I said, both Mama and Daughter need a break this weekend!

That's about all. For those of you who don't see Grace on a regular basis, I will post a picture soon so you can see the improvement in the lesion!

(By the way, the appointment with the dermatologist was rather anti-climatic, as I expected it to be. She prescribed an ointment for the lesion that will further help with the inflammation, but other than that said she concurs completely with the rheumatologists course of treatment. She will continue to be another set of eyes to monitor Grace, but her main treatment will continue to be monitored by the rheumatologist at Children's Hospital.)

Until next time,
Sabrina

This week's update

Grace got to take the day off today from going to Children's Hospital. The timing was perfect, because her class had a field trip to the Woodland Park Zoo today, and she would not have been able to go if we'd gone for the infusion.

Tonight I administered the first methotrexate injection at home. (We've had MTX in the infusion center, but this is the first one I did at home.) Grace was a little bit scared, but I did great (if I do say so myself!) and the whole thing was over in less than a minute. The first one is behind us, and it is always the hardest.

Next week we go back to the infusion unit at Children's. However, we will be going on Wednesday intstead of Thursday so that Grace can go with her Brownie troop to the Encampment weekend. After that, we'll see what our schedule is.

Grace has an appointment with a top pediatric dermatologist in Bellevue next week. (She is impossible to get into, and is only doing this as a favor to the first dermatologist we saw in February. ) I'm not sure what--if anything--will come out of this appointment. She might give us some ideas of what we can do to minimize the appearance of the lesion. I will also talk to her about the area of hair loss, and if there is anything that can be done about that. I've read that the hair loss is permanent, but who knows?

Grace is doing well. As of this week, she has gained about 6 pounds, but it really doesn't show all that much, and she is thrilled about the new clothes she has gotten out of this! I am in search of a new First Communion dress for her, however, since the one I bought her no longer fits. Good thing I've got until June 8 to find one!

That's about all for tonight's update. Thank you so much for reading.

Another one down!!

Hi everyone,

Sorry this post is a day late. Thank you to those of you who called wanting to make sure that everything was OK.

Yesterday went much better than last week. Unfortunately, Grace did vomit on her way to the infusion. I think she was just really nervous given the events of last week.

When the meds were hung, I went to get her breakfast in the cafeteria. In weeks past, she usually becomes ravenously hungry about 20 minutes into the infusion. I became a little concerned when that did not happen this week, and the breakfast went untouched. She started to feel more and more nauseated. The nurse came, and suggested that because Grace had started the infusion with a totally empty stomach (due to the vomiting), that we had created a vicious cycle: Grace couldn't/didn't want to eat because of the nausea and fear of vomiting. The empty stomach, however, was make the nausea worse. If she had started with a little food on her stomach, she probably would have been OK, but we had just started out on the wrong foot.

The nurse called her doctor, who prescribed a dose of Zofran, which is a FABULOUS anti-nausea med used in patients receiving cancer treatments. Grace took the Zofran, and started to feel much better within just a couple of minutes. It was miraculous. She still didn't feel like eating much, but she felt better.

Selfishly, I was glad she felt better because we had plans to meet an old friend for a quick lunch yesterday. Typically, Grace is "amped up" after a treatment, and after she started to feel better yesterday, this proved to be true once again. So, we walked around U-Village for a few minutes and then met Lara for lunch. Grace managed to eat most of a hamburger and some fries. By then, Grace was starting to get very tired, which is also par for the course. So, we loaded her up in the car and brought her home. She spent most of the afternoon on the couch, and felt great this morning and was able to go to school.

We are taking next week off from the steroid (methylprednisolone) therapy. I will administer a methotrexate injection at home next Thursday. The following week, she goes for her infusion on Wednesday in order to be sure that she feels 100% healthy by Friday so she can go to Encampment with her Brownie troop that weekend.

Thank you to Christine for a wonderful dinner last night!

Thank you everyone for reading.

All my best,
Sabrina

A little while later . . . .

She's doing better now. We're going to try and go for it and head to the coast. If anyone needs to get ahold of us, cell phones don't work out there, and we won't have email. You can reach us at the condo using it's land line if you need to. If you don't know the number, my mom has it.

Love to you all,
Sabrina

Some minor bumps in the road

Hi everyone--just wanted to give you a brief update.

I just got off the phone with Rob, who is on his way back from the hospital with Grace after her weekly infusion. I was unable to go because I am actually quite sick myself, though on the road to recovery. We had some minor problems at the hospital today.

Grace's infusion typically takes two hours. However, if the patient tolerates the medications well, the staff gives the parent and/or patient the option to speed it up to 1 1/2 hours. Because Grace had tolerated the previous infusions well, and because we are trying to get on the road for our trip to the coast, Rob went ahead and told them to speed up the drip. Unfortunately, Grace did not tolerate this well at all. She felt quite sick and lightheaded, and vomited twice. I just spoke with her, and she's doing better, but still not feeling too great. Lesson learned--take the whole two hours!!!! Don't try to rush it!

We are waiting to see how Grace does before we hit the road. The medical facilities in Westport are practically non-existent, so I want to make sure she is well recovered before we head out. Today is Rob's birthday, and we were hoping to hit the new Cabela's on our way, but we'll see.

In related news, Grace had an appointment with her rheumatologist earlier in the week. The lesion is looking a little better, and has actually shrunk by 1mm, which is great news. Grace has also only gained 1 lb, which is further good news. Though she is retaining quite a bit of water (another side effect) which makes her appear like she has gained more. At this appointment, I also learned to give her the methotrexate shots in preparation for when we can cut back on the steroid treatments and give her only the methotrexate at home. The nurse let me practice on her (Grace wanted no part of it), and I am proud to say that I am a natural. The nurse told me I missed my calling, and should have been a nurse!

The timeline for the treatments is also under scrutiny. Grace's liver enzymes were slightly elevated this week. Still within the normal range for a child receiving her treatments, but a little on the high end. She is sceduled to receive a treatment next week, but then will likely be taking a week off. We'll let you all know more as we find out additional information.

Thanks for your good thoughts. We truly appreciate them.

On a selfish note, I'd like to take a minute to wish Rob a happy 39th birthday. I always knew that he is a great husband and father, but he has really shown that over the last few weeks. He's the best.

We'll update again later.

Love,
Sabrina

The 2nd infusion is behind us . .

Today's 2nd infusion is history! Grace did very well today, simply averting her eyes when they started the IV line, and taking everything like a trooper. Because of my pretty bad cold, we had to be in isolation while in the infusion unit. The poor nurse had to put on a gown, mask and gloves everytime she came in. This was done to protect the other children in the unit who are severely immuno-compromised. We were out of there in about 3 hours today--not bad!

Today's side effects have been a little more problematic. Thankfully, no nausea, but Grace is pretty agitated and irritable. She is finding it difficult to sit still for any length of time, and is pretty emotional.

I spoke with our school district nurse this morning, and we now have a plan in place for Grace on the day she returns to school after treatment. Last week, she made it through a couple of hours, and then was in the health room complaining of a headache. With this new plan, she will be allowed to relax in the health room, drink some water, have a snack and take some Advil if needed. She can then return to class if she feels like it. She will also be given more leniency for snacking/drinking in the classroom if absolutely necessary. In addition, because her immune system is suppressed, the district will notify us in the case of an outbreak of any communicable diseases such as chicken pox or strep throat.

Over all, this is becoming more routine. We meet with her rheumatologist on Monday, so we should have some more information on the length of treatments, etc. at that time.

That's tonight's update. I am now off to drink some TheraFlu and go to bed. Rob is also pretty sick--in fact he's worse than me. We are so thankful for the delicious dinner brought to us tonight!

Thanks for all your positive thoughts,
Sabrina

We're off to infusion #2 . . .

It's cold and dark, and Grace and I are about to make our pilgrimage across the Snoqualmie Valley and across Lake Washington to Children's Hospital and Medical Center. She's doing fine this morning, looking forward to seeing her favorite nurse, Amanda. (Gosh, I hope she's there!) Someone from her Brownie troop delivered another basket last night, and a wonderful volunteer from PTA is bringing us dinner tonight. The support and kindness of our friends has been so overwhelming . . . and so very appreciated!

Stay tuned for another update later tonight. I am a little concerned because some of the meds tend to have cumulative side effects, meaning that the relatively mild side effects we experienced last time might be a little more pronounced this round.

Thinking positive thoughts,
Sabrina

1st Infusion under our belt . . .. .

Well, we made it! Grace and I left our house about 6:30 this morning, and got to Seattle about 7:30. We even had time for a short detour to Starbucks in U-Village!! We arrived at the hospital about 8:00 and we were both quite nervous. The nurses are fabulous, however, and we soon felt more at ease. We received the good news today that our infusions will actually take more like 2 hours (rather than the 4-6 hours we were previously told.) However, the whole process takes longer than I thought, so a two hour infusion can actually take about 4 hours by the time they get the meds from the pharmacy, apply the numbing cream, find a good vein, put in the line, hang the meds, etc. I'm sure the process will get faster once we're a little more familiar with the routine.

We're home now, and Grace is doing well. She's very irritable, and very hungry. Both of which are side effects of her meds. The appetite is something we will have to control--or at least control what foods she eats, but for today I'm being a little more lenient. The grouchiness, however, is already taking it's toll on me . . . .. ;)

I must say however, that all of this was really put into perspective for me today. As hard as it is, and as overwhelming as all this seems, it is literally NOTHING compared to what so many families are facing. We saw alot of very sick kids today, whose meds are many times stronger and have many more insidious side effects than Grace's, and whose families spend hours and hours every week at Children's Hospital. It was very humbling.

Following are a couple of pictures. One of them is of Grace's lesion to give you an idea of what it looks like, and the other is of her writing in her new journal during the infusion.



Our best to all of you,

Sabrina

Our Bags Are Packed and We're Ready to Go!

Tomorrow is the day. Grace and I spent some time tonight gathering a bag of things for her to do tomorrow. Her Brownie troop has organized a gift basket for her, which will be delivered every Wednesday night in anticipation of her Thursday treatments. Tonight's basket contained High School Musical stickers, markers, a blank journal and other goodies. Grace is thrilled, and Rob and I are truly touched at the support our community is showing us. We are so grateful for each of you!

Grace is handling everything just fine--at least on the outside. I know she is nervous, but she seems to be doing OK. Tonight was her musical performance at school, which I'm sure helped to take her mind off of things. We'll all be glad to get through tomorrow. It will be easier when we have an idea of the routine of the place and how she will tolerate her medication and the IV in general.

I've packed my bag for the hospital, too. Though I'm thinking it's rather optimistic of me to think I'll get through 4 back issues of Gourmet and Bon Appetit! In a way, I'm looking forward to the time to spend alone with Grace.

I will update tomorrow night with how it went. Please keep up in your thoughts.

By the way, the above picture was taken in February at Grace's birthday party at Libby Lu's. Isn't her smile fantastic?

Just a couple of pictures

Thought you guys might enjoy seeing a couple of pictures. These were taken last week at Children's Hospital when Grace was having her MRI. The one where I'm holding her is before they put her to sleep. We were both a little nervous! The one with the popsicle is taken after she woke up. I really love both of them.

And So It Begins . . . .

I have created this blog to help keep our friends and family apprised of the situation we are facing with Grace. First, a little history . . . . .

About a year ago, Rob and I noticed a "bruise" on Grace's forehead. When we questioned her, she told us that she had hit herself on the forehead with her brush. Though the story seemed odd, we let it go. As time went by, we noticed that the "bruise" wasn't fading, but we really didn't think too much of it.

Last month, when we were in church, I happened to pull Grace's hair back from her forehead, and realized that the "bruise" had spread up into her hairline, and that her hair had fallen out in this area. About this time, we noticed that the surface of the skin was also starting to change, becoming stretched and shiny looking.

I took her to our pediatrician, who told us that he had no idea what it was. He referred us to a dermatologist. When the dermatologist saw her, she told us that it was a type of scleroderma called "en coup de sabre", a French phrase meaing "cut of the sword", so called because this is what the lesion resembles. The condition is very rare, possibly affecting as few as 3 out of every 500,000 children. This began a whirl of blood tests, and specialist visits while we struggled to get to bottom of what this disorder was, and how it would affect our daughter.

Scleroderma literally means "hardening of the skin", and this is what is happening to Grace. The skin and underlying tissues have atrophied, and the disorder has also caused a depression in the skull underneath the lesion. We underwent an MRI earlier this week, and found (Thank God!) that it has not affected the brain tissue underneath, as some of these lesions can. Her bloodwork so far has also been fine, indicating that there is no other organ involvement.

The next part of our journey is treatment. This will involve weekly trips to Children's Hospital for IV infusion therapy. The infusions will contain massive amounts of methylprednisone as well as smaller amounts of methotrexate, which is a chemotherapy drug. Because this is an automimmune disorder, it is treated like many other rheumatological conditions--the main idea being to suppress the body's immune system to stop it from attacking itself.

There is no cure. But we are optimistic that we caught it before any further damage can be done. When Grace is a little older, and depending on how things go, we will consider some kind of reconstructive surgery to fix the indentation, as well as the discoloration if she so chooses. For now, our goal is simply to get throught treatments.

Each infusion will take 4-6 hours, depending on how her body tolerates the medication. The main side effects will be nausea, fatigue (methotrexate) and weight gain (due to the steroids). Some patients also have trouble sleeping, become more irritable, and undergo some mild personality changes.

This journey for us truly begins on Thursday, March 27, with an 8:00 am appointment at the Ambulatory Infusion Center at Children's Hospital and Medical Center in Seattle-about 30 miles from us.

I would like to take a moment to publicly thank all our friends and family for their support as we struggle to learn as much as we can about this. Please feel free to pass the URL of this blog along to anyone you think might be interested and who I may have missed. It is a huge source of stress for Grace to hear me talking about this on the phone, and though I cherish my conversations with each one of you, I am going to try to limit my conversations to the school hours when she is away. In the meantime, I will keep this blog updated--I will have lots of time in the infusion center to do so.

On this Easter weekend, we ask you all to keep us in your prayers. Please pray for strength for our family as we get used to this new routine. Most importantly, please pray that the side effects are minimal, and that Grace tolerates the treatment well so we can get our healthy little girl back.

Rob and I send our love to each of you. You are always in our thoughts.

Praying for our daughter,
Sabrina