Toes, Hip, & Spine

Goofin off in the doc's office - silly little girl

The day started early for me and the wee-bit today.  We had an hour and a half drive to Gillette Children's Specialty Clinic in St. Paul, MN with an appointment time at 8:15am - much better than a four hour drive to Milwaukee, that's for sure!  Height and weight was first: Caity weighs 48lbs and is 43inches tall.  Next was xrays of her legs (for leg length discrepancy - LLD - comparison) and right hip.

Once in our room a resident and medical student came to check in with us first.  They asked questions and examined Caitlyn.  I explained her new CLOVES diagnosis and how her hip seems to have becoming more bothersome over the past year.  She stumbles and falls constantly and after walking or playing hard she will complain that her hip hurts.  I told them that I found this surprising because I had always just assumed her left leg would cause her the most discomfort.

Left: Current  Right: 1 yr ago

Shows she is "knocked-kneed"
this may need surgical intervention

Dr. Sundberg came in to speak with us after the resident was through talking.  He explained that he is not too concerned about her LLD and does not want to do any medical intervening at this time.  Instead he wrote out a prescription to have a lift put on the outside of her right shoe.

We then went on to talk about her right hip.  It is still showing minimal change as far as positioning and condition of her femoral head/socket relationship.  There is still significant avascular necrosis to the femoral head - meaning there is "bone death" due to there being a lack of blood supply to that area.  So it was decided because an xray can only show minimal amounts of data of her hip and her increase in discomfort we will schedule another arthrogram procedure.  This is a general anestesia procedure performed in a surgical suite. Pain/discomfort is very minimal and the procedure only lasts 30 minutes.  Once the arthrogram is done we will discuss whether or not moving forward with a surgery sooner rather than later would be appropriate - I'm hoping for the later. 

Left: Current  Right: 1 yr ago

Current

Current

Dr. Sundberg sent us back to xray so we could get images of her spine and feet.  Her scoliosis has definitely worsened over the past two years.  He is concerned about the sharp curve in her spine.  Although it only affects a small number of vertebrea it is quite complicated due to the way the vertebrea appear to be sitting.  He consulted a spinal specialist doc and it was agreed to also schedule an MRI of her spine.  Either way, no matter how her MRI comes out, she will either need to have surgery to help correct her curve or she will need to be put into a back brace.  I am not excited about this at all.

Looking and palpating her spine

Her feet show definite overgrowth of tissue and bone, which of course we already knew.  But it was

nice to see which exact bones were the culprits of her problem.  He informed me that in some cases there is the option to damage the growth plates in her two overgrown toes on her left foot and if that didn't help then we could consider a partial amputation - or total amputation - of those toes.  The purpose of this would be for comfort measures as well as making buying correct sized shoes a possibility.  The same can be said for her right foot, even though it doesn't have the overgrowth of toes it does have major overgrowth of tissue/muscle.  A debulking of her foot would be very complicated due to the increased risk of loss of sensation/feeling in that foot and/or toes.  So instead, we could do a partial of total amputation of the long (metatarsal) and short (metacarpal) bones of a couple of her toes.  Needless to say, I'm not thrilled about these options one bit.  I wouldn't mind, eventually, doing something about the growth plates in her left toes.  However, right now, I just want to focus on her hip, spine, and chemo/immunosuppressant therapy.

At the end of the day, I am thankful to at least have her home with me; happy and overall healthy.  There are many parents that have lost their children to this disease or similar syndromes  or parents who sadly have yet to take their babies home.  I, at least, get to have her here with me.  So we will take this as it comes and I will continue to try and find the positive in every medical situation we come across.

A special thank you to sweet Baby Forrest for helping me realize that my daughter's situation could be far worse than it is and to be grateful for the many things that I CAN do with her instead of feeling sorry for the many things that I CAN'T do with her.  You are absolutely amazing; from the very bottom of my heart I truly thank YOU for helping me to be a more thankful mother!

And many, many thanks too all of you for keeping Caitlyn in your prayers and well wishes and for finding time in your life to keep track of her story.  Feel free to contact me or share her blog.  The more people that know the better we can raise awareness!

Back to Bloggin with a NEW Diagnosis!

After deciding to take a total break from blogging, not knowing whether or not I was going to start back up again, here I am!  So much has happened this pas year and a half that I don't even know where to start.

Caitlyn had a debulking surgery done of her mons and labial areas, (her, "little lady parts" for those of you who don't quite know medical terminology), in January 2013.  After her recovery I decided that we were done with doctor visits, surgeries, and hospitals for a while.  I just wanted her to live "normally" for a while.

We did go to Gillette Children's for a second opinion about her hip - I am just not fond of the doctor she had in Milwaukee.  He is an amazing surgeon, but his bed-side manner could use some work.  I got her into the head of the department for pediatric orthopedics, who specializes in lower extremities.  We did a sedated arthrogram of her right hip and it showed definite necrosis of her femoral head and hip joint - meaning "bone death." She is not getting adequate blood flow to that area and as a result part of her bone/joint is dying.  She will definitely need surgery to correct this problem but with all that she has been through I decided to wait.  If her hip stays stable then hopefully we can do surgery in a few years; if not, then I may have to agree to surgical intervention sooner.  Only time will tell.

On March 24th, 2013 Caitlyn welcomed and beautiful new baby brother - David Allen-Lee!!!  She is
such a proud big sister and absolutely LOVES her brother...sometimes a little too much!

Our summer was fantastic!  We were finally able to give her a real summer without drainage tubes, PICC lines, or casts!  We didn't have a single trip to Milwaukee planned for her either.  We had one hiccup in July with Caitlyn's health.  Towards mid-July, Caity went to bed telling me that her leg her.  I chalked it up to her running to much that day outside and she just needed to rest it for a while.  The

next morning she said her leg hurt again.  I asked her to come out for breakfast and she refused to walk.  I thought at first she was just being stubborn and trying to sucker Grandma Louise into carrying her.  I took a look at her leg and noticed it was warm, red, and hurt to the touch.  She was also running a fever so I called Milwaukee and was advised to call 911.  Once to the hospital and after ruling out a blood clot the doctors determined she had cellulitis, (an infection that people with Caitlyn's condition are susceptible to getting).  She spent a few days in the hospital on IV antibiotics.  She was sent home on oral medication and the rest of our summer was uneventful - medically speaking.

In August we took her to Mt. Olympus in Wisconsin Dells. It was an AMAZING day and she had a blast!  I don't know if I have ever seen her smile and laugh so much.  Caitlyn was also nominated to be the Children's Miracle Network Hero for our area, which we gladly accepted!  More to come on that later!  School started back up at the end of August.  I was finally accepted into the core course classes of the nursing program - woot woot!!! 

By September I decided to contact Marcia in Milwaukee at the Vascular Anomalies Clinic.  I told her that over the summer I had noticed that the left side of her abdomen (which has involvement of malformations), has been getting bigger and bigger.  She was now wearing size 6-7 shirts and she was only 3 years old.  Caity also had numerous new blebs popping up her back and a couple on her belly.  We had also been having problems with Caitlyn controlling her bladder during the night and day.  Without any notice she would jump up and run to the bathroom; sometimes she would make it and other times she wouldn't, and sometimes she would start to pee herself and not even know she had done it.  We scheduled an appointment to meet with a Urologist, the VAC team, and get an MRI done in November.  

The meeting with the Urologist went well.  We could definintly see on MRI and ultrasound that her bladder is severely displaced due to the amount of malformations within her abdominal cavity pushing it over.  This is likely the reason she is having a hard time controlling and knowing when her bladder is full enough to urinate.  He started her on a medication and a diet plan to follow.

That same evening, as we were leaving, we met up with Dr. Drolet (Caitlyn's Dermatologist) on the skywalk.  She informed me then that we got her genetic testing results back, which we had started the year before, and she positively, without a doubt, has CLOVES Syndrome, not Klippel Trenaunay.  This was HUGE news and I was so excited to finally have an official diagnosis.  The VAC meeting the next day was kind of disappointing.  We

My CLOVES Baby!

discussed that the only way to reduce the size of Caity's abdomen was to do a debulking, and probably more than one would be needed.  Her mons and labia will also unfortunately need more work done.  Then the discussion with Caitlyn's oncologist was whether or not to start her back on the chemo that she was on as a baby.

After take a few weeks to think about it I finally decided to move forward and start her back on the Sirolimus.  Her belly has just grown so much that I felt like I couldn't just sit here and watch it get bigger, we needed to do something.  So far she has handled it pretty well; no major side effects as of yet.  Tired, crabby, no appetite - the usual for when someone is on chemo or taking an immunosuppressant. 

More recently Caitlyn was approved for a wish through the Make-A-Wish foundation!  Her wish granters visited a few days ago and Caitlyn wished to be a princess at Cinderella's Castle!  Hopefully we can plan our week long Walt Disney World wish trip around school and Caitlyn's upcoming surgeries this summer.  I will be sure to update and post pictures of my princess enjoying her wish!!!

That is it for now on Caitlyn.  As things happen and change for her I will keep updating.  I promise not to take another hiatus.  I finally came to realize that writing her story down is healing for me and hopefully brings hope, understanding, and awareness to others reading this.  Please feel free to share her story or contact me if you would like.  I am more than willing to answer questions and educate appropriately.

Thank you so much I will write more soon!!!
Sondra and Caity-Bug

Finally Home!

Walking for the first time post-op!

After my interview with Dave and Carol we started having a roller coaster ride of events right up until Caitlyn was discharged.  The day after the interview we were finally able to remove Caitlyn's catheter and right away she went to the bathroom on the potty!  Before heading into this surgery Caitlyn was completely potty trained so seeing her using the big potty after eight days of being bed-ridden was very nice to see.  She was even trying to bear some weight on her legs in order to get to the bathroom.  It wasn't much but it was definitely a start!

However, we also noticed that the bruise at the end of her knee starting to get bigger and grow darker.  Thankfully, she had surgery scheduled for that day and Dr. King was able to take a good look at her incision and her knee.  He thought her incision and her knee looked really good.  The areas of concern on her thigh were no longer concerning and I was excited that all she had was a bruise, nothing more.

On Saturday, my husband made the trip down to spend a few days with us.  It was a wonderful reunion and Caitlyn was so excited to have her daddy with her again!  She much have been excited because she didn't fall asleep until midnight and was up again at 5am, then back to sleep at 7am for only an hour.

Caitlyn was approved for a 6 hour pass from the hospital; so, on Sunday we took advantage and took her to the Milwaukee County Zoo.  It was 92 degrees but we didn't care and neither did Caitlyn.  We were just so happy to be getting out of the hospital for a while.  Caitlyn really enjoyed her day at the zoo and she just couldn't get over all the monkeys!  Monday was Memorial Day which we usually spend with family out at the cabin and on the pontoon.  Instead, we requested another pass and took Caitlyn to Mayfair Mall for some shopping, candy, and ice cream.

Tuesday came and Caitlyn had another trip back to the OR scheduled.  The plan was if everything looked good the we could go home on Wednesday, as long as she did well overnight and her fluid output into her two JP drains didn't put out a considerably high amount. During surgery, Dr. King removed the VAC machine and covered her leg in gauze and wrap.  When he came out to talk to me all I got was conversation full of disappointments.  He confirmed that the skin on her knee was indeed dying so we would not be coming home the following day and possibly not at all for the rest of that week.  He didn't know at that time whether this would end in a skin graft or not.  Also, there were a couple of areas on her incision on her thigh that did not look very healthy either.  The only good thing that came out of that discussion was the fact that her skin was indeed healing down to her muscle instead of just being a large skin flap covering her leg.  I left that conversation feeling incredibly defeated and so full of sorrow.  I just wanted so badly to bring my baby home where she belongs. 

Enjoying the zoo train ride with her new baby tiger

Dr. King came in for a visit Wednesday morning and we made up a plan:  Thursday he would stop by and remove all of her dressing at the bedside.  If she looked healthy enough then we could go home and come back on Wednesday for an appointment in the clinic with possible surgery on Thursday if she needed it, in order to correct the unhealthy skin and fix her incisions.  If she didn't look healthy enough to go home then we just stay at Children's until she is.  In order to add some fun to our day, since we weren't able to go home, Peter and I decided to take Caitlyn back to the zoo.  We weren't able to see all of it the other day so why not go back and see the rest?  Caitlyn enjoyed the train ride this time too!

Unfortunately, since we didn't know if we would even be going home Peter had to leave us after Caitlyn's music therapy session on Thursday.  Which by the way, she LOVED just as much the second time as she did the first!  The plan was for all of us to go home together, but sometimes things just don't work out as planned -- something I have learned during this journey with Caitlyn.  Dr. King didn't make it to our room until late afternoon.  He warned me that I wasn't going to like what I saw so I tried to brace myself for a grotesque sight. Caitlyn cried while he took off her wrappings and gauze and of course it just broke my mama's heart.  But, much to my surprise her leg didn't look near as bad as I thought it would.  The areas on her thigh looked like they were starting to heal and her knee, although obviously unhealthy, wasn't any worse than it was the other day in the OR.  So at least she wasn't getting any worse.  The gauze and wrap was put back on and Dr. King said he was comfortable enough to send Caitlyn home!! 

We agreed to keep her PICC line in and to take her home with both of her JP drains still in place.  The nurse let me do a dressing change on her PICC, just so I felt comfortable enough to do it once we were home.  I had everything packed in record time, talked with the nurse about getting supplies sent to our house for Caitlyn's PICC, and did the PICC dressing change, had discharge paper all done within about an hour to an hour and a half.  By 5pm we were on the road and headed home!

Dancing to music on the boat!

Caitlyn was wonderful the entire drive back and as soon as we got to the house Caitlyn went running from room to room checking everything out and even laid down in the living room and started rolling all over the floor.  It was so cute to watch!  Our next week consisted of visiting family and friends and having grill-outs with those that love and missed us most. 

On Wednesday, Caitlyn and I made the drive back down to Milwaukee see Dr. King in clinic.  I packed the vehicle up just in case she needed surgery on Thursday and didn't know how long this hospitalization would last.  Fortunately, her knee and incision looked absolutely great!  Her knee is starting to heal from the outside-in and the areas on the thigh looked like they had sealed close and were healing nicely.  Dr. King said he didn't think Caitlyn needed surgery the following day so we were free to go home.  He did have me schedule an appointment for Wednesday the following week just to recheck her again to see how she was doing. 

After our visit with Dr. King we went and saw a central line nurse to do a dressing change on Caitlyn's PICC and then up to Orthopedics to see Dr. Thometz about Caitlyn's scoliosis, her leg length discrepancy, and her sub-luxing right hip.  I found out that her scoliosis is mild and is something to watch but not to be concerned about right now.  Her hip on the other hand is not doing very well.  Although it is still in, it is sub-luxing out.  Her hip socket is quite shallow and needs some reconstruction.  Her LLD is also concerning but because of her age it isn't something that we can fix right now and he really thinks we should focus on correcting her hip.  I asked if this something that we can wait to do until she is old...much older, when she is between eight and ten years old.  He pointed out that, although this is going to be an incredibly difficult surgery for her, with immense amounts of pain, and a tough recovery he doesn't want to let her hip "completely go to pot" by waiting too long to do the surgery.  My view is, I have already put her through so much and she has already had such pain in her life that I just can't justify putting her through a surgery as severe as this when she is still so young.  At least when she is older, she can vocalize her discomfort better, I can explain to her what is going on and she will understand what I'm saying, and she can be entertained a lot more easily than she can right now.  I just don't think I can do it.

Dr. Thometz would like to recheck her hip in four months and then plan a surgery shortly after that.  I told him that I am more than willing to come back and see him in four months but there really isn't much they are going to be able to tell me in order to convince me to do this surgery right now.  If the worse thing that would happen is her hip comes back out of place, then fine so be it.  We dealt with that before and it isn't at all painful.  However, if they say that this is going to stunt the growth of her leg (because I do know that her proximal growth plate of her right femur isn't very healthy) and she will forever be damned then I would consider the surgery an option.  But, with the knowledge that I have right now, I just can't bring myself to put her through a surgery as painful as this when she has already been through so much, not now.

Dressing change on Sunday.  Knee is healing but
the part on her thigh has started to split open again

This all now leads us to today.  I had to cancel Caitlyn's appointment to see Dr. King tomorrow.  I did a dressing change on Sunday and emailed him some pictures.  He said she looked good and was fine with us not coming down.  Next week he is on vacation so surgery has been scheduled for the 26th.  He will be removing her stitches then and fixing her knee as well.  Hopefully, he can remove her drains and we will have a very short stay at the hospital...hopefully.

I know I have said this before but thank you all for your continued support!  This is not an easy road to travel but having support from others makes it a little less rocky for us.  Thank you!