Craniosacral therapy

We saw angel again today after about 3 weeks. We saw another therapist in between. I caught her up on the recent changes with her casts and hip ultrasound. She says to me " you don't like your doctor, do you?" I became tearful and she told me my body language spoke volumes when telling her about it. We talked about how I'm working on finding someone I feel more comfortable with. She told me she feels Dylann can gain her range of motion in her knees, hip, and ankles in just the work we are doing and that she's made incredible improvements in just the 5 weeks we've been working. 

We then had the most amazing session! In a half hour she worked her hip back into socket and taught me how to do that at home each day as well, worked out the tightness of her quad and hamstring in her left leg and was able to bend it about 60 degrees even though our orthopedist said it wouldn't bend at all without surgery, and worked her feet so both ankles moved 25% more than they did prior to getting there. She is simply amazing! We are moving back to weekly sessions for now!

We also received the most incredible act of kindness from some friends of ours. My friend and her family do a white elephant auction each Christmas for their exchange and with the money donate it to a charity or family experiencing some medical or other hardship. Her daughter nominated Dylann as the recipient when the family was discussing who to give to this year. The rest of the family agreed and Dylann will be able to use this money for 8 sessions with angel!!! We are touched with how thoughtful this was and excited to see her progress more and more with each session!

As I work to find a provider I like, I am so comforted with our work with angel. I had never even heard of craniosacral therapy 3 months ago and now can't imagine what we do without it!

Gilettes visit 12/23

Not a lot of positive news today. Dylann had a hip ultrasound to see if they were affected. Her right one was in socket but the left one was not. Our doctor said typically they treat hip dysplasia with a harness the child wears for a number if weeks until the socket is realigned and growing properly. However he said for kids with arthrogryposis and the placement of her legs that it wouldn't work for her anyways. The only option is for her to have surgery between ages 6 to 12 months to place it in the socket. He said this is successful 75% of the time. The other 25% of the time it becomes dislocated again and could negatively affect the blood supply in the joint causing some of the connective tissue to die out. This would result in permanent damage and needing to wear a body length brace from there on out. He said in a few months we will have to have a discussion about these risks and if it's worth it once she shows us more how she will try to move. 

She also is no longer showing progress in her ankles so we are no longer doing the casts. She has braces that she will wear full time for a few months to keep the progress she has made and then will need surgery. If we decide to do the hip surgery then he will do the ankles at the same time to avoid multiple major surgeries for her. Even if we do all this, our orthopedic doesn't seem to feel to strongly that she will be able to walk someday. We don't come back now for a month. I think I might get a second opinion in the meantime. I have a hard time following this doctor and just want to be sure of all our options.

Thought we would cross a few things off the to do list this week..

But not so much. Yesterday Dylann had a 2 hour genetic appointment at Children's.  This has been recommended to us by most of her providers since many genetic conditions can cause arthrogryposis so they wanted to make sure to assess that. 

The genetic doctor asked me a question that no one had asked before so I hadn't thought about it. She told me there are 100s of tests that can be done so " how important is it to me to know what caused her joint issues?"  Hmm.. I told her what's important to me more is to know it isn't the severe and progressive things and that we are doing the right treatments.  She told me that after meeting with us and looking her over they her arthrogryposis isn't typical so she has a harder time just saying it's that. She also has a hard time saying it was amniotic band syndrome since she can't see the banding with her casts on and that her wrists have some joint issues and wouldn't explain how symmetrical that looks. That being said,other than her joint issues she is normal in her development and function which you wouldn't see with most genetic conditions. She did test for one form of muscular dystrophy that it could be albeit a 1% chance or less. MD was the diagnosis I feared the most throughout this whole wait time. Not sure when we hear back.   Next steps are to see her again in a few months and we could test for other gene mutations that cause arthrogryposis.

This morning we had a hearing test for Dylann. She failed her left ear test in the hospital. She failed again today. We have to see her pediatrician to check if fluid is stil in there then go back for a brain stimulus hearing test in 3 weeks too asses if she can hear out of that left ear. 

She remains a trooper through all of this !

Cast update.

Had Dylann's second casting change. Her ankles and knees made enough progress to continue!! He is hopeful it will help enough to avoid surgery on her ankles. He did say that the most difficult part is probably going to be how tight her calves are that of isn't allowing her ankles to move to a 90 degree angle. That might need surgery in a month or two. Her hips are also still pretty tight so we have an ultrasound on 12/23 to see if there is more going on. Moving in the right direction!! 

Casting

We just had Dylann's first recasting appointment after having her casts on for a little over a week. She has improvements in her ankles, knees, and hips!! They recasted for another week and next week they will know if there was enough improvements to keep casting and hopefully avoid surgery on her ankles!

Never a dull moment

 Dylann and I planned to snuggle all day since we haven't been able to do that yet :).  At 11 I got a call from the nurse at Gilettes regarding some labs we did over a week ago. She said we needed to go downtown to children's right away to repeat the labs as her "sodium was a bit high". I tried to get more information on what this meant but all she would tell me is it needed to be done today in case she needed "immediate intervention".  She also told me to wait at children's fir the results. So we rushed down. True to form in how our providers have been communicating for the last 5 months.... No one seemed to know what they were supposed to test for when we got there. Dylann and I waited an hour and a half while the nurses at children's and gilettes went back and forth about the order. Wierd to me that this "urgency" was preceded by an 8 day wait from when the labs were taken until they even called us then a few hour wait to even know what was being tested for. Hmmm?? When they figured it out, it then took two techs, 3 pokes, and one very very upset Dylann to get the blood. After 2 more hours waiting for gilettes to call me, the children's tech sees I'm still here and tells me she faxed the results an hour ago. I call gilettes, they can't find them, the new nurse on is confused why anyone told me to wait in the first place, and that the other nurse who called me this morning is now gone for the day. After several back and forth calls happily all her labs are normal. I have to say though that this is my 4th encounter with gilettes and each one has been disorganized, rushed, and frustrating. I keep hearing how great they are; so far I'm not impressed.

First two weeks home

Since being home, we have had about 10 appointments. There have been some tests and consults to rule out of a few things. Her chromosomal tests came back normal. She had a neurological exam that was normal. She has reflexes in her arms and legs and startles appropriately. Since she was a week old when this was done, the neurologist wants us to come back at 4 or 5 months of age to do another consult just to make sure there arent delays. We havent done much with the genetic testing as that seems to be the area they are least concerned about, but we did run some labs to make sure her muscle enzymes are good and we are still waiting on those. Treatments: First, she will be having weekly craniosacral therapy. This was also such a great find. My sister in law got the name of a great therapist in the area, Angel Phillips. She was referred to as the best in MN. I did some research on her and it looks like she has trained many of the therapists in the twin cities. When I contacted them for a referral she was not taking new clients but I told her that I would be open to anyone at her practice as long as they were familiar with arthrogryposis. Angel called me back and told me she was going to work with Dylann herself!! She has had two sessions and the movements in her hips and knees are dramatically different. We are excited to have found her and to continue the work. Second, she started with Gillettes for orthopedic work. We had our first appointment this week and they already casted her feet. We will be keeping these on for about two weeks to see if the MD can get her ankles to start changing position. If they respond, she will be in those for 6 to 12 weeks and then in braces. If she doesnt respond, then she will discontinue the casting and will need surgery when she gets a little older. Her knees will need something although we didnt discuss this yet. Not sure if that will just be physical therapy or if surgery will also be needed. Also, she will have an ultrasound on her hips in the future to see if they are in the right place to decide next steps. Ideally with both of these interventions, we hope she can get the best range of motion in her legs possible and be able to walk someday. She has been such a tough girl through all this! She is the most calm baby and only cries when she is hungry. She has also been sleeping very well and giving all of us almost a full night sleep other than to eat. :) Next week she has her craniosacral therapy and then we will change her casts Friday and start to see progress.

What is Arthrogryposis?

We first learned that something was going on with Dylann during our 20 week ultrasound appointment on July 12th. Up to that point, things had been "normal" for my pregnancy other than some more intense sickness at the beginning that I didnt have with Micah. We decided to bring Micah to the appointment so that he could learn at the same time as we did, what the gender of his new sibling was going to be. He was bored with the whole thing so it was very distracting while we were there and we didnt spend much time looking at the ultrasound with the tech. Towards the end the tech got quiet and said that our baby had club feet. She also said that her right leg was tucked under her butt so she was not able to tell us the gender at that time. She then went to talk to our MD and told us we were going to meet with her after. I knew then that something else was going on that they werent telling us. We met with the MD a few minutes later and she didnt tell us much more other than that babies who have club feet oftentimes have other things going on so they wanted us to go have a level 2 ultrasound to see if that was the case. I left there feeling sick and confused but was happy when I found out they were able to get us in to have that second ultrasound that same day so we didnt have to wait the entire weekend to learn more. Jeremy and I went that afternoon to see a Maternal Fetal Medicine specialist for the ultrasound. When we arrived, we were told we are first going to meet with a genetic counselor. That counselor is the one who told us they had seen more then just the clubbed feet on the ultrasound. I felt frustrated that we had to hear this from someone we knew for like 3 minutes. She told us that in addition to the feet, the baby's arms werent moving as much as they would expect and that her leg looked fixed in the tucked position under her butt. At that point, we went through a genetic genogram to see what ran in our family but that was pretty much a blur. All we knew was that we were not aware of anything that ran in our family so this was pretty much a shock. After that meeting, we had our second ultrasound that showed the same things noted above but didnt show any issues with the baby's heart, brain, spine, or other structural concerns. The baby was also measuring exactly the right size for the gestational age. That had to be promising, right? They also told us that they were 90% certain it was a girl! After the ultrasound, we waited for what felt like hours in the room waiting for the MD to come back. When she did, she came back with the nurse and counselor so we knew things werent going to be good during that conversation. I know we talked for half an hour but all I remember was the MD saying they were very concerned for the baby's prognosis, that what they were seeing is a symptom and could be the result of over 150 different diagnoses (this "symptom" is called arthrogryposis, which means congenital joint contractures), and that many parents choose termination at this point. From there I stopped listening. Through my own research, I learned that the different causes of arthrogryposis include muscular issues, joint issues, chromosomal problems, genetic issues like muscular dystrophy, neuromuscular issues, or central nervous system function issues. From there it was a blur over the two weeks while we had an MRI and more ultrasounds with the hopes that the diagnoses that were more severe or fatal would be ruled out. Luckily the MRI showed no structural issues with her brain or central nervous system and the ultrasounds didnt show more regression in her limb movement. We were offered an amniocentesis to test for some chromosomal and genetic issues but there werent any ones in particular the MDs were thinking it might be so the procedure seemed more risky with the odds of miscarriage being higher than any of potential diagnoses they might have found. We decided against it. So, from there, we were to wait. It was a very long 4 months trying to work and take care of a 3 year old with this on your mind. We took comfort in a number of things that were seen in the biweekly ultrasounds.. she was growing right on schedule, her arm movements continued to improve through many of the appointments, she managed to turn head down which indicated some normal movements in her arms and torso, and they didnt find any other issues with other areas of her body. She also showed breathing motions early and maintained those. Since I had a C-section with Micah and due to the placement of her legs throughout the pregnancy, the right one stayed tucked and bent and her left one was stuck in a straight position, we had a planned C-section for 11/13. We had to deliver at Fairview Riverside since they wanted a level 4 NICU nearby since they didnt know what to expect. Our MD kept telling us, "everyone is just so confused on what is going on with your daughter." That is helpful to hear over and over again. Thankfully and with great relief, she was born and started crying immediately. There were 4 staff from the NICU at delivery and they decided she was doing fine and didnt need to go there! She was able to stay with us in the hospital and came home with us as soon as I was able to leave after 3 nights. Her arthrogryposis only affects her lower limbs. She does have the clubfeet and her knees are fixed in the positions we saw on ultrasound. She also has limited movement in her hips from side to side. At this point, they think it was caused by an amniotic band. There is a ring around her right calf that looks like a rubber band was wrapped around it. Our MD and the NICU MDs think that this band was keeping her right tucked in that position and subsequently kept the left leg straighted on top of it. So, the joints didnt get to move in uetero like they needed to and got fixed in those positions. Her calf muscles are also underdeveloped as a result. None the less, she has and already has had a long road ahead of her. I will talk about her treatments and tests so far in another post. She is perfect!!

Updates

I decided to start a blog to keep those interested updated on Dylann's Progress. It has been difficult to try to keep in touch with everyone and we really appreciate all the support we have been getting friends and family and would like to be able to answer questions in a more central place. I will write an initial post that has the background and then try to update as we have ongoing doctor appointments. We want to again thank everyone for all the support and we are so thankful she is doing so well!