Dermatology, post surgery update and The Cape

Dermatology, Post-Surgery Update and The Cape

Well, it's been a little crazy around here lately.  However, it has been a quiet month for Elizabeth, because we have only had one doctor's appointment this month.  I can't remember the last time we had a month with only one doctor's appointment.  

We saw Dr. Kress at CHP Dermatology on Tuesday, April 14th. This was a follow-up appointment from our visit in February about Elizabeth's rash on her cheeks and her sun sensitivity issues.  We have been treating the Keratosis Pilaris (KP), which is a form of eczema, with a type of cream recommended by Dr. Kress.  The KP on her cheeks is much improved, and it really only shows up when she is not feeling well.  So, the plan is to continue treating the rash and hope that it continues to improves so we don't have to up the course of treatment with steroids,etc.  

As for the sunlight sensitivity, Dr. Kress believes that her symptoms describe a series of disorders called "Porphyrias." Porphyria disorders are rare and they are caused by enzymes not working properly to produce porphyrins (these help to form many important pigments in our bodies) and heme (responsible for the red color in red blood cells) in our bodies.  They often present themselves as neurological or skin problems, especially a sensitivity to sunlight, but sometimes you see both symptoms.  Dr. Kress suspects that Elizabeth has a Porphyria disorder due to her sensitivity to sunlight and her various neurological problems. He also explained that she could have one of the Porphyria disorders in conjunction with other genetic disorders/ diseases/ etc. that they are testing her for through the other various departments at CHP.  Dr. Kress had to order additional blood work to test her for some of the markers and identifying factors for the Porphyrias, because surprisingly with all of the blood that she has had drawn, she has not had the necessary testing done to test for the Porphyrias. So, I plan to have the blood drawn when we are at CHP in May for one of our various appointments next month.  She'll just get to earn extra beads for her Beads of Courage. ;-)

In case you are wondering what I mean by Elizabeth's sensitivity to sunlight issues, let me explain.  Since Elizabeth was born, we have noticed that she cannot be in direct sunlight for very long periods.  In fact, she will have a reaction to sunlight sometimes with as little as 10-15 minutes of exposure.  So, I have always had to keep her body covered up, and wear a sun hat on her, and use sunscreen.  She gets big red rings around her eyes and blotches on her skin that look like a bad sunburn that is about to blister. The redness disappears anywhere from a few to several hours after she is removed from exposure to the sun.  The first few times that it happened, it was very scary.  However, I've asked the doctor's about it, and it has always been a "mystery," which is why we were sent to Dr. Kress for some insight.  It could be that she is simply sensitive to sunlight.  Her skin is pale and thin, so that plays a role, obviously.  It could also be that she does in fact have one of the Porphyrias. The blood work and outstanding genetic testing will tell us for sure. It is just one more answer that we will continue to wait for and will face once we get it.  In the meantime, we will just hope for the best. 

Elizabeth continues to do well from her surgery.  She has been sleeping a little bit better, until recently as she now has a cold.  The runny nose started on Thursday.  So far, we don't have a fever or a cough, but she isn't her happy self.  We recently had a nurse start coming to the house to help with Elizabeth's care.  She is wonderful, and her name is Ann. I'm really happy to have her help, and she is amazing with Elizabeth. Elizabeth has been showing much more strength and is definitely more at ease.  She has been crawling all over the place, starting to pull to stand, cruise around the couch, etc.  It is amazing to see her do these things.  I love being here to talk to and watch all of the therapy sessions. Not only do we have an amazing team, but it is great to see how she has made so much progress from their amazing work with her. Elizabeth also is finally slowly gaining weight.  She has gained a total of 12 ounces since her surgery on March 30th.  Progress is progress no matter how slow. The consistent nutrition and the fact that even if she refuses that she still gets her food has significantly impacted her strength. I like to think that her being more at ease has to do with me being home with her.  She loves to cuddle, walk towards me in her gait trainer, smile at me. Honestly, her smile, even it is 1 am, is one of the best things ever.  Celtan has an amazing smile too, and he is so good with Elizabeth, and she adores him.  I love being here to watch them grown and interact together.  It is truly amazing. 

There are some other things that we are seeing in her that are of some concern.  We have been noticing a change in her muscle tone lately. Elizabeth is becoming more and more spastic in her motor movements, and this is causing her to have some difficulty in her moving her limbs, keeping them still, etc.  This is one of the reasons why the Cerebral Palsy clinic doctors ordered her leg braces to help counteract the spastic movements in her legs to create greater stability while she is standing. She also sometimes has difficulty putting her hands together even to clap due to the spastic movements in her arms.  These changes are hard for us to watch. While we are glad to see her increase her strength, grow and do more and more things, it is hard to see her inability to control her movements. There are treatments for spastic movements, but they would negatively impact the low tone that she has periodically and in the other areas of her body where she consistently has low muscle tone.  So, right now, treatment for the spastic movements is not really an option.  Why we are starting to see this is unclear and could be due to a variety of reasons. It could be changes in her brain, it could be changes in her nervous system, it could be a lot of things.  We may be able to treat it.  We may not. So, as always, we will watch, wait and monitor and hope for the best.  

On an awesome and exciting note, my friend and colleague, Jen Kramm, nominated Elizabeth to receive a cape from Capes for Heroes.  I am very pleased, excited and honored to announce that Elizabeth has been officially named a superhero, and Capes for Heroes has sent her a handmade cape especially for Elizabeth. I put the cape on her, and she smiled.  She just loves it!  What an awesome program.  

Also, a huge thank you to our secret angels who continue to send us cards, gifts and little things to brighten our day and to ease the burden of my unpaid time off.  You will never know how much we appreciate all of it. It means so much to us.  Also, thank you to those of you who are continuing to send contributions towards Elizabeth's travel fund, the benefit, etc.  It is truly incredible, and I am working on sending out thank you cards for the benefit, but I'll never be able to adequately thank all of you for your love and support. I would like to thank Suzan Graham for the totally awesome set of Thirty-One Gear that she sent to us for our trips this summer!  I'm so excited to use all of these beautiful items. Lastly, I would like to thank my amazing friends and family near and far who have been getting me out of the house, calling, and texting to see how things are going.  It means so much to me.  I'm especially looking forward to a girls weekend near the Ohio Pyle area next weekend and to seeing the original Elizabeth (Lug) as she is home from NYC to help her amazing sister Laura care for their mother, Monica, as she just had surgery.  Their family is near and dear to my heart, and as you pray for Elizabeth, I'm sure they would appreciate some extra prayers for a speedy recovery!

Speaking of needing prayers, there are two other families near and dear to my heart whose daughters are fighting some very serious battles.  These beautiful girls, and their amazing families, have been through hell and have come back time and time again fighting like warriors.  I admire their strength, honesty, and love for each other more than they know.  We pray for healing for Sydney, Bella and their families every night, and if you have some extra prayers to send I'm sure they would appreciate them.  As a parent of a special needs child, you walk a path that is different and often lonely. I am appreciative of the bonds that I have formed with the amazing parents of other special needs children.  No matter the battle, we understand and are there to support each other.  So, please continue to think of them, if you don't mind saying an extra prayer or two. 

I have mentioned more than a few times in this post and in previous posts that we will wait and hope for the best.  People have often remarked to me about how I'm so strong, how they admire how we are handling this, and I often don't know what to say in response. There are times when I cry, when I'm up late at night unable to sleep because I'm full of worry, times when I need a drink by 9 am, some days I'm so overwhelmed to even be around normally developing kids.  I'm not always strong.  I cry. There are days when I want to scream or throw things, to be completely honest, even though I don't do that because it doesn't solve anything.  However, I always try every day to find some kind of silver lining, to find one thing that gives me hope or happiness or reminds me of the wonderful blessings that surround me.  Whether it is a smile from Elizabeth, Celtan's laugh, the fact that we are about to celebrate a second birthday with a baby girl that I wasn't sure would ever even make it here alive, or the fact that I'm really surrounded by some really incredible people, I make it my mission every day to find something to be grateful, and that is how I stay strong and feeling blessed and hopeful.  It is not always as bad as it seems, and it could always be worse.  I count my blessings, and all of you who read and follow Elizabeth's story are among those blessings.  

There will be a lot of updates in May, as we have a lot of appointments, etc.  So, be on the lookout for updates.  Oh, and Elizabeth got new glasses... they are lavender!!  (Thanks to Amanda at Dittman Eye care for ALL of your help!)

We Now Have a Tubie!

We Now Have a Tubie!!

So, how about I totally drafted and typed a blog entry up while I was in the hospital with Elizabeth AND I forgot to hit "Publish."  Oops, sorry!

Since this will be old news for many of you that follow me on Facebook, I'm just going to summarize our hospital stay.  I'll recap highlights, etc. and give you a bit of insight as to how our first (almost) two weeks at home have gone so far. If you don't follow me on Facebook, feel free to find me there. You will find me as "Tara Bly Hackwelder."

Anyway, Elizabeth had surgery on Monday, March 30th to have a G-Tube/ MIC KEY button placed in her stomach.  Dr. Austin was her surgeon at CHP, and she was amazing.  I was grateful that Holly, my mom and Pastor Joe were able to come down and spend the morning with Jason and I as we waited for our precious girl to get out of surgery.  The waiting is always the hardest part.  (Tom Petty hit the nail on the head on that one.)  I was especially beginning to become nervous as the time kept ticking by and we weren't getting any news.  I especially became nervous when it became longer than expected and still had not heard anything.  Eventually, Dr. Austin did come out and she said that Elizabeth did very well during the surgery.  We were all very glad to hear that.  Dr. Austin also mentioned that she had found a small hernia while operating on Elizabeth.  She said it was not enough of a concern for her to repair it at that time, but it would be something that we will need to monitor over time. We all thanked her, and then we sat and held hands as Pastor Joe led us in prayer. We prayed for healing, for peace, for guidance and strength.  All things that we needed then, need now and will continue to need in the future. 

On a complete side note, we all got a good laugh that day over Jason's name tag.  It was horrendously misspelled, and the woman who registered him even took his name off of his driver's license, which is correct.  Here is a picture for your enjoyment!

They allowed us to see Elizabeth in the recovery area, and then they eventually moved her to her room on the 6th floor.  Jason went home in the afternoon, and Holly and my mom stayed with me.  Holly left in the evening, and it was just mom and I. We watched Elizabeth as she slept most of the day.  We talked to her nurses and met with Sarah from Child Life.  Sarah came up to meet Elizabeth and enroll her in the Beads of Courage Program.  This is an amazing program that enrolls children with various medical and special needs and awards them different beads for different milestones, treatments, procedures, etc. that they have had done or that they experience. I have heard of Beads of Courage, and I was very excited that Elizabeth was going to be enrolled in the program.  Elizabeth is the bravest girl that I know, and she is certainly full of courage. Sarah gave me some paperwork to fill out, and she said that she would be back on Wednesday to give Elizabeth her beads.  

It was a rough first night.  Elizabeth did not want to be held.  She cried, which she almost never does, because she was in pain and uncomfortable.  She threw up in the middle of the night.  The nurses were doing everything possible to help keep her comfortable, but she had not eaten since Sunday night and was continuing to take medicines to ease her discomfort and pain.  Pain meds and an empty stomach don't mix well.  They were giving her IV fluids, and they were planning to start her first continuous feed of Pedialyte from Tuesday evening until Wednesday morning.  If she tolerated that well, then we would be able to start to gradually work up to a full diet.  

We continued to struggle with pain Tuesday and Wednesday.  It was miserable for her and miserable for mom and I to watch her cry.  She did not want to be held, but once they started her on the Roxycontin for pain it helped her to want to be held for small periods of time.  Cuddling is a miracle drug for mommies and babies, and I do feel that it helped her.  We started to gradually work her up to a diet of Pediasure and pureed foods.  She did not want to eat anything by spoon, and at most she had a few spoonfuls of strawberry and banana yogurt. Sometimes she tolerated her feeds well, and sometimes she did not.  We had a bloating episode, and we had to drain her stomach.  In hindsight, I'm glad that happened and I was able to see what to do, because I've had to drain her stomach one time since we have been home due to her being in pain because she was so bloated. Even if she didn't want to be held, she still wanted to hold my hand.  We have held hands since she was a 3 pound wonder in an incubator bed in the NICU.  Even then, she would instinctively wrap her tiny hands around my finger. I sang to her now like I did then.  She would crack a little smile and sleep.  Elizabeth rarely let her Minnie Mouse out of her sight.  She even took Minnie back to the OR with her.  We watched/ listed to Frozen about 25 times during our stay, and she also wanted to hold her Sophia doll quite often. 

We had some bright spots on Tuesday and Wednesday.  A long time friend, Kristen Sichler, brought a nice gift of food, snacks, drinks and a toy for Elizabeth.  It was so great to see her, and I was very touched by her kindness.  If you've ever spent time in a hospital, you know that the food (no matter how good) gets to you after a while.  It was delightful, to say the least, to have some fresh snacks and food delivered to us by Kristen.  It was great to visit with her, too.  Also, Sarah from Child Life brought us Elizabeth's beads that she has earned by her experiences thus far.  Sarah also brought me some real shampoo, which I had forgotten, and some other toiletries so that I could wash my hair and feel refreshed from being in the hospital for the third day.  I had washed up, showered, brushed my teeth, etc. on at least a daily basis while being there, but I was desperate to really wash my hair.  (It's the little things.)  Anyway, the Beads of Courage took my breath away.  Obviously, I knew that Elizabeth has been through a lot in her short life, as I have been by her side through it all.  However, the beads were a visually representation of that, and it was amazing.  I gained a newly found respect for her continued bravery, strength, and I was filled with pride at this little miracle that we have been blessed with in our lives.  She has been through so many medical procedures, appointments, etc. that she had earned many "catch-up" beads, and that means that each bead signifies 20 or 50 procedures of a certain category.  Elizabeth had also earned a variety of other beads, and mom and I sat on the couch while she slept and strung the beads together.  We ended up with four strings!! She will continue to earn beads on her journey.  When she woke up, we showed her the beads and she held on to them. It was as if she knew exactly what those beads represented, and I cried.  I was tired, overwhelmed, and those beads mean so much to me.  I could tell they also meant something to her.  Here are some pictures of her beads, and if you would like to read more about the Beads of Courage program you can visit: http://www.beadsofcourage.org/

On Thursday, we finally began to see some improvements.  Elizabeth was wanting to play, sit up and stand up in her bed, and she even allowed me to hold her quite often. We had found the perfect cocktail of pain medicines, and she was tolerating her feeds better.  I was there by myself with her.  I watched her sleep. I prayed over her.  I held her hand.  I sang to her.  I tried to sleep.  Her nurse brought other nurses in so that I could share Elizabeth's story with them. We really had amazing nurses while we were there.  Finally, by Thursday afternoon, Elizabeth was perking around.  I decided to take her to the Austin's Playroom (sponsored by the Lemieux Foundation) on her floor.  She played in there for almost an hour.  I had my training on Wednesday about how to give feeds, how to give meds, how to flush, etc.  I was beginning to feel more comfortable, and I was also feeling very grateful that Elizabeth was returning to her usual, happy, self.  Jason came down to stay with us, and we were set to be discharged on Friday.  We took a car wagon ride to the first floor to greet daddy, and then we watched the new Cinderella movie in the lobby that night.  Child Life was showing the new Cinderella for the patients and families on the 6th floor Atrium on the big movie screen. They had balloons, wands, pumpkins with mice in them, and it was magical to say the least.  I finally got to meet Alisha Cooper, the amazing mom of Avery Sue, at the movie.  We have become connected over the past few months, and I'm so grateful for all of the support and encouragement that she has given to me.  It was so great to finally get to meet her. Also, during the movie, Elizabeth set her sights on an enormous Cinderella balloon.  She actually grabbed the strings and would not let go!! I was so thrilled that she saw the balloon, especially out of her peripheral vision, and the Child Life staff thought it was so cute and allowed her to keep the balloon. 

Elizabeth had a good night from Thursday into Friday, and we were discharged on Friday afternoon (Good Friday). Jason, Elizabeth and I headed home.  The visiting nurses and the medical supply company came shortly after we came home to drop off our pump, supplies and to provide me with some other important info. I finally was able to see Celtan after a week, and we hugged and hugged and he did not leave my side the rest of the night. It is hard to be with her all week in the hospital, but it as equally as hard to not be able to see him and tuck him in bed at night.  

A huge thank you to everyone who helped us out with Celtan during the week we were in the hospital:  Aunt Lori and Uncle Steve, Nuria and Jamie, Aunt Deb.  We could not have gotten through the week without you.  A huge thanks again to Kristen Sichler for the care package, and to all of those who sent cards to us through Children's Hopsital while we were in the hospital that week.  Also, thanks to my friend Lindsay Loheyde for the amazing picture frame.  It is beautiful, and I love it. We also had a "thank you" for the benefit and prayers put up on the sign at Gino's in Chicora.  We are still overwhelmed by the love and support from everyone at the benefit and through the Go Fund Me.  Your continued love, prayers and support are no doubt the reason why Elizabeth continues to heal and thrive. 

So, we are home and we officially have a "tubie."  We had a lovely Easter with our families. I am home with both of my blessings, and I love being home with them.  I know that the feeding tube is responsible for the improvements we have seen so far with Elizabeth, even though it has only been two weeks, but I also know that my being home with her makes a difference.  If she isn't crawling after Celtan through the house, then she wants to be on my lap imitating sounds and smiling and giving me puppy kisses.  I could not ask for more.  She is growing, improving, and getting stronger.  I'm no longer having to worry on days when she is refusing to eat or only wants to eat small amounts.  Meal time is no longer stressful.  We no longer have to fight her to eat.  We have a pump that can give feeds slowly and continuously over a period of time that can be attached to an IV pole or be carried in a backpack for feeds on the go.  I have also learned how to do gravity feeds using a giant syringe.  I'm getting the hang of this feeding thing, and we are finally getting to see Elizabeth grow and thrive.  It is nothing short of amazing.  The tube makes all of this possible.  Whatever she doesn't finish goes into the tube.  Whenever she refuses to eat, it's ok, because I can just feed her through her tube.  It has truly changed our lives for the better. 

Also, since I am now home, I am taking every advantage to spend some quality time with Celtan, too.  We go to the park on any day when it is not raining.  Sometimes we even go twice a day.  He started T-ball and really loves it.  I like to go to his practices and watch him.  Sometimes it is painful to watch when he is playing in the dirt, but he is so proud of himself to be playing on a team.  He has a smile and a laugh that are totally infectious.  I totally adore my little boy, and Elizabeth adores him as well.  She really watches him, and she often gets upset if he leaves the room or she can't see him.  He is always hugging her, and he is really sweet and patient with her.  We had a nice visit from my cousin's wife and friend,Darian, and their new baby girl, Avery Quinn.  They are both gorgeous ladies, and Celtan was very proud to hold Avery.  Elizabeth touched her head, but that was about it.  It has really been a wonderful two weeks. Did I mention that I LOVE being home?