February 20th

Today Addie has a great day planned. Phoebe, a cousin and friend came to visit. Addie’s Aunt who is currently there with her went to go find a slushy maker and a new release movie that Addie has been wanting to watch – and Addie is hoping to get up and get moving, perhaps a walk.

Last week was tough. Addie had to have two biopsies taken as well as have a right chest tube put in – fortunately the right chest tube is no longer draining any fluid, and should be removed today or early tomorrow. When they took the biopsy from the mass in Addie’s left shoulder they were also able to drain 400ml’s of fluid from the shoulder – which has allowed Addie to be more comfortable.

Talking to Addie this morning was great. She was in good spirits and excited about the day. She is really excited for tomorrow because Carrie Underwood will be visiting the hospital, and Addie is determined to get out of her room and down to the hospital’s Seacrest Studio to meet her.

Ryan and I have been very grateful to the two Aunts that took our place out in Philadelphia the last three weeks. Ryan and I were able to have sometime with each other and our other 6 children – which was wonderful and rejuvenating. Ryan will be returning to Philadelphia on Monday the 22nd. He is anxious to get back to Addie.

We are hoping that these biopsies will provide us with some answers – answers that will let us know what medications will stop this mass and promote healing. Love to all, Michal

January 30th

It’s been another quiet week. Lots of conversations, a couple ideas, a treatment plan being discussed – and home an ultimate goal. Addie will begin vincristine this week. It is a medication that other patients with this disease have used with sirolimus to try to stop the “flare up”. The “flare up” being the cyst in Addie’s back that appears to be growing as well as what appears to be responsible for the excess lymphatic fluid that is penetrating her left shoulder, arm, and chest cavity. We must stop the “flare up” in order to decrease the fluid so that the skin can heal. Once the skin is healed enough the doctors will put in the plurx catheter.  Addie can travel home with a plurx catheter, and we should be able to manage her medications and care from home with the help of CMC and possible a home health care program.

The vincristine can show its effectiveness within a few weeks up to a few months. Most of Addie’s care will be through medications at this point – so being home is reasonable, however travel is not possible without the plurx catheter – so patience again is required.

Addie is holding strong. Yes she is definitely tired of this situation, but she continues to not be in pain, and seems to be able to keep herself positive for the most part. Over the next three weeks, I will be traveling home to be with the family. Addie has two very close friends who will be coming here to be with her. She is excited that they are coming, and ready for a bit of a change. I am excited to go spend time with the rest of the family and to get a break from the hospital.

I will continue to keep you all updated – and hope to see Addie’s situation moving in the right direction soon.  Michal

New Chest tube

IMG_4257

Good Morning Everyone,

Yesterday was tough – Addie’s pleural cavity did fill up with fluid as did her left arm and shoulder. Addie was having significant difficulty breathing and asked for another chest tube. They hesitated on the plurx catheter because of the incision they would have to make in her (very angry hot) skin and because of the amount of fluid that was still being produced. So we have another chest tube and it is not the take home kind. This process has revealed that we must take care of the shoulder and the lymphatic mass in her shoulder before we can take care of the chest tube. How do we get this lymphatic mass to decrease in size and stop producing lymphatic fluid? – hopefully the sirolimus – a medication that can take anywhere from a few days to a year to start working. Addie has been at a therapeutic level on sirolimus for about 10 weeks. Addie did start taking prednisone yesterday as well as an antibiotic to see if that will help with the inflammation. On Monday we may decide to try vincristine to see if that medication will be helpful, but again vincristine could take up to 3 months to be effective, and wether it will be effective is unknown. As I have been told over and over – and as I have come to know personally – this disease is extremely complicated and the treatments are all considered experimental, – a statement that all of you are becoming common with as well.

So, yes- yesterday was rough. Fortunately morning comes – and a good nights rest allows the fighting spirit to continue. Addie and I listen to the reports of the crazy storm, and watch the wind swirl outside her window. I did get to walk this morning – and saw about 6 cars stuck, and very few individuals out and about. I honestly welcomed the 8 inches of snow, and enjoy the quiet that it brought.

Addie is currently face timing with the craziness of our home life in Missoula. We are enjoying watching and listening to the chaos that seems to constantly be ensuing at our home. We miss it, love it and nothing sounds more appealing.

Michal

January 21st

IMG_4006

Today Addie is quiet. She waits to see just how her body is going to adjust to this new change. The chest tube had been in for more than 3 months and now is finally gone. The hole that the chest tube was in  remains open and continues to be the path of least resistance for the lymphatic fluid. They have created a way to catch and drain this fluid as the body is allowed time to heal and seal the small opening on its own. They are going to give her body the weekend to heal the opening – and hopefully send the fluid to other places to be reabsorbed. If the body is unable to do it on it’s own, then Addie will receive a few stitches on Monday to manually close the hole. We will remain in ICU during this time so Addie can be closely monitored.

Love to all, thanks for your continued support, Michal

Chest tube out

IMG_4254

Hello Everyone,

Addie did have her chest tube removed today, this morning 9 AM, eastern time. She is being closely monitored to seen if her body is able to reabsorb the fluid and stabilize. If not, they will be inserting a plurx catheter, a chest tube that we will be able to monitor from home. I will let you know how the day goes. Michal

January 16th, 2016

IMG_5259IMG_5289

Saturday’s in the university area of Philadelphia are much quieter than the rest of the week. Today our friend Phoebe took me outside of the University area on a nice walk/hike. It was great to see a bit more of Philadelphia.

It was a quiet week for the most part. Addie did have a Zometa infusion on Wednesday night. Zometa is used to reverse the bone breakdown in Addie’s body – and hopefully help the bones become more dense and not so brittle.

Addie’s output is still about 1500 ml’s a day – no chyle – just pink lymphatic fluid. The lymphatic team feels like it is time to take out the chest tube and see what happens. The idea is that the body will reabsorb some if not all the lymph fluid. It has also been known with other people with this disease that for some reason their bodies want some lymphatic fluid in their plural lining – and as long as we keep sucking it out – it will just keep producing. So on Tuesday evening the plan is to pull out the chest tube. Addie will be put in ICU so that she can be closely monitored. If it does not reabsorb and reach a happy level within her body – they will put in a different kind- of chest tube that attaches to a chamber that attaches to her body. This is a set up that they feel she will be able to have at home. Addie is ready for the next step. The idea is that we manage this chamber at home – and perhaps as the sirolimus becomes more effective – the output will continue to decrease and then the temporary chest tube can be pulled as well.
There is one other concern at this time. Her shoulder is also full of lymphatic fluid that we believe is coming from lymphatic masses that will not go anywhere, and it appears that the fluid is increasing and accumulating in her shoulder and her back. The current thought is that we really need to do a biopsy of her skin on her left shoulder. The biopsy would tell us if her cells would be receptive to either prednisone or vincristine. If the cells are receptive to either of these medications they think they may be necessary in order to help slow down or shut down the lymphatic masses and lymphatic fluid in Addie’s shoulder and back. They are concerned that Addie’s disease is still being aggressive and that coupling these medications with the sirolimus may be necessary.

Addie and I miss Montana, our home, our family, and our friends. However the support and love has not wavered. Today and tomorrow Brittany McLaughlin with Writing with Light Photography and Design is hosting the 5th Annual Pictures from the Heart Benefit for Addie.

The Lowder’s continue to love and bless us from afar.  They have asked that in lieu of donations to their family after the passing of their precious son and brother that they would like donations to Addie’s medical fund. So far $5,800 have been donated to Addie’s fund in honor of Abe. I only wish I could shower them with as much love and consideration during their difficult time.

We press on – and hope and pray for answers and relief to come Addie’s way soon. Michal

Still in Philly,

I will try to find some good pictures for the next post. Per Addie’s request, I am not allowed to include any recent ones of her.

Fortunately we have had a very uneventful weekend. The week was a bit tough. By Thursday, we had not seen any decrease in output and the mass on Addie’s shoulder appeared to be getting worse. Thursday, after Ryan left, Addie experienced extreme stomach pain followed by blood in her throw-up and stools. The day became more difficult as I received very sad news from our hometown; our community had to accept that an extremely special person was traveling to heaven. Friday, the GI team came in and did a scope of her GI track to find ulcers in her stomach. The ulcers were no longer bleeding at that time. They have added another medication to Addie’s regiment, one that will coat her stomach and esophagus in order to protect it from future ulcers. Friday and Saturday were days of rest and healing. Today Addie is quiet, her output does appear less, and the mass on her shoulder appears a bit smaller with less discoloration. Tomorrow will bring new discussions and ideas. The pleurodesis will be discussed – Is this something we want to try now, or should we wait. The mass on her back will be discussed as well – Do we take a biopsy or do we wait.

I am doing alright.

I have enjoyed walking around Philadelphia: the amazing beautiful old buildings, the diversity, the smells, the humidity.

I have been impressed with the hospital and the staff here.

We have had the fortune of having an apartment lent to us the past few weeks that has allowed for some very restful nights of sleep. And…

The highlight was yesterday when my brother Donald and his family came to visit me; I had not seen them in 7 years!!!!

I miss my home and my wonderful children there, as well as my job and the wonderful children there. It’s a good thing I know I have so much support in Missoula watching out for me and all the children I love.

Always hopeful, always praying – who knows what the week may bring. Michal

1/8/2016

IMG_4917

Today we are sad and our hearts hurt, not for us – but for one of our all time favorite doctors and his family. His smile and humor always brightened Addie’s day while she was in Missoula. I had the honor and pleasure of being Grace and Abe’s school counselor when they were in 8th grade – and Ann was the first person to bring me a meal when she heard that my husband and I had taken in a child who was needing some extra love and care in the hospital. Sometimes life really doesn’t seem fair – and doesn’t really make sense. I have been one to accept and cherish individual beliefs and ideas – but today I am so glad I believe in Angels and the next adventure. Love to the Lowder’s, Addie and I are holding you deep in our hearts, and wish we were there to give you all a hug. xoxoxo Michal

Philadelphia – January 5th, 2016

IMG_4010

Here is a picture of Addie in our camper this summer. Her faithful companion Sue close by. Addie is not interested in many pictures these days – and I know most of you would rather see pictures of her than of Ryan and myself.

Addie had her 4th procedure yesterday. It was another long procedure and by the time we were allowed to be with Addie the doctors were gone for the night. This morning the main surgeon came in to talk with Addie and me. He said that they did find a few more holes to patch. However, where the leakage is occurring is from  lymph “masses”, and the “masses” are growing, and he is not convinced they have been able to find them all. The next step will probably be to do a pleurodesis, a procedure where they would artificially obliterate the pleural space where some of the masses are located and leaking the fluid. This would hopefully clear up the chylothorax, but would not rid the body of the other lymph masses that also appear to be growing – one on her back and one in her shoulder. Dr. Dori and Dr. Itkin are working with Dr. Adams, an oncologist that has worked with Gorham’s patients to find out if there is another medication we should add to her sirolimus to stop or reduce the growth of the lymph masses. I have also turned to my Lymphangiomatosis & Gorhams Disease Alliance support group. From the support group I am hearing that others do have these masses, some of them have been able to control the growth of these masses with medication, and some have had their masses removed successfully.

We should here from Dr. Dori this afternoon or tomorrow when they will schedule the pluerodesis.

Ryan and I continue to enjoy being here together with Addie, but Ryan will return home to Montana this Thursday, and is very excited to see the crew. Addie and I will continue to blaze this trail. Although it has been one trial after another – I continue to feel the support, prayers, and love around us, Ryan and I’s spirit and strength remain strong. I continue to be hopeful that a solution will be found, yet aware that this disease is one we will and must be on top of for years to come. Addie remains   quiet about her thoughts – strong, stoic and quiet. I long to know her thoughts and ideas – but she often only gives information about her situation when Ryan and I are wrong – so every day we try to be wrong a few times 🙂

Love to all, Michal

January 1, 2016

IMG_5243

I made it to Philadelphia just in time for the New Year. I think Addie is glad I am here; I know Ryan is.

Addie is doing alright. She had another “procedure” on Tuesday. They felt that once again they were able to find some problem areas, however they felt that there are still significant problem areas to be discovered. Dr. Itkin will be returning this weekend, and a couple new doctors visited with us yesterday. Doctors that are part of a group that are studying and treating Gorham’s.

Ryan is amazing – keeping things as light and fun as possible. Addie is frustrated as any of us can imagine. This disease is complicated and Addie is tired of being patient. I keep reading stories about other warriors – they help me keep things in perspective. This disease and it’s complications are not simple and quick; the solutions take time and patience. Now that I am here I will try to give more updates – although I struggle with having to take all of you on this crazy roller coaster ride with us, but I know you are all here to support us and be involved. Thank you, Love to all, Happy New Year. Michal