Monday, December 29, 2014

The end of the year

It's the end of 2014....good riddance! While I can't complain about everything that happened this year. It certainly was another year we lost more than we gained. Let's recap some high points....

Audrey had only 4 hospital stays all year!!! This is huge! Her stay in February was the whole month, and was the scariest time we've ever had with her, but she has come a long way since then.

Audrey started ballet. This is a dream of hers, and we are doing everything in our power to make it happen for her. It is so hard to get her there. I have to give her extra fluid before hand (think about doing exercise for an hour but unable to drink anything) and then fluid after. She has to get extra steroids during class or she can't physically make it through class. She usually has very long nights after class with low blood pressure and low oxygen, however, she's never happier than when she is dancing. So because of that, I will continue taking her. You may ask why? It seems to take so much from her to go, why put her body through that? Because everyday, little by little, cell by cell she is losing her battle with mitochondrial disease. I hope and pray that we can slow it down, that someone will find something to stop the damage being done. But her reality as of today is that there will come a day when all of the interventions I can possibly do will not be enough for her to dance. Until that day comes, I will do everything in my power to encourage her dreams. She twirls around the house telling me, "I have to make my body stronger so I can dance on my toes!" This is not her acknowledging her limitations, this is her repeating what Angelina ballerina said when she wanted to dance on pointe, but Audrey has no idea how right she is.

Audrey does not recognize her limitations which is a blessing and a curse. She doesn't let mito hold her back. She charges ahead with all the determination of any other 5 year old with a goal in mind. She does not stop when she's tired. She does not recognize her muscles starting to ache or her tummy starting to hurt...until the pain is more than she can ignore. And then she crashes. We have nights like tonight, when she had so much fun playing with her brother who was gone all weekend, she didn't notice how tired she was getting. SO when I said it's time for a tub, and she had to stop for a minute, she literally lost control of her body. She screamed for over an hour, she was in horrible pain, her blood pressure was low, her glucose was low, the sound of running water was deafening to her, she kicked and she screamed and she could not stop herself. While some might think it was just an epic tantrum (and I don't doubt it started that way) it certainly did not end that way. It was simply the price she paid for having too much fun today. Tonight I will pay the price as I work to keep her vital signs stable because she played with her brother today.

However, back to our recap. Audrey had a great summer! She did ballet, she swam in the pool, her Nana came out to visit, she did not go to the hospital. In fact she had very few doctors appointments. This summer was the best we've had since she got sick. Thankfully since Audrey started using her insulin pump filled with hydrocortisone to manage her adrenal insufficiency in July, things have gone so much smoother for her. We are so grateful to her endocrinologist who was willing to take a chance and try something different. Audrey ended the summer celebrating her 5th birthday at Disneyland with her best friend. She did the royal princess makeover and easily had one of the best days of her life.

Behind the scenes, this year has been rough. While Audrey's medical needs have continued to increase, the ability to find anyone to help care for her decreased. I know I have mentioned before how her complexity prevented finding a home nurse to care for her while I went to work. This only continued this summer until finally in November we were forced to relocate to a more affordable location. We are now 2 hours away from our old home, learning a new way of life. It's a rural community and we have a lot of land. I hope that the kids and I get to learn new skills to make ourselves more self sufficient. I have already greatly expanded my power tool collection and have learned new tasks such as laying laminate floor, installing baseboards and using a jigsaw. There is so much more to learn it is a little overwhelming. As most homeowners find, there is an ever growing list of projects, however, add in Audrey's schedule, it is very difficult to get through projects which should be fairly quick. Audrey's life has not changed much, as so much of her day revolves around a complex schedule of care which is the same no matter where we live. Audrey's brother however, has had a harder time. He has left the school he spent the past 6 years at. He left his friends and is 2 hours from his father and grandparents. I hope and pray every night that this was the right move for him too. His needs are frequently behind Audrey's needs due to a matter of necessity, but I want more than anything for him to grow up happy and not feel like he was neglected for his little sister.

So with moving came a change in jobs. I was forced to leave my place of employment where I have lived, worked and grown for the past 10 years. This was such a difficult choice to make, though in the end, there was no choice at all. It really came down to the fact that I cannot work and take care of Audrey. I had a couple fellow nurse friends who pitched in to help when they could, but this was not steady or reliable enough to make it possible to continue my employment. So I have become yet another statistic to the world of special needs parenting. First it was my marriage, then it was my home, finally it was my job. All of it lost in order to care and provide for my child. Don't get me wrong, I wouldn't change a single thing. Looking back I would chose my daughter, both my children over everything. Clearly my husband wasn't who I thought he was. My home was replaced with another home where we hope to have even better memories. My job gave me the skills I need to care for my girl. It gave me the connections I needed to get her the best doctors I could to care for her. And while I was so sad to leave, and I miss the adult conversations which are absent now, it turns out I was more attached to my job than it was to me. The fact that after 10 years of employment, I had 3 people wish me well, showed me I had out stayed my welcome.

This is what happens when you enter this world of special needs parenting. You lose friends, jobs, opportunities. It starts slowly with people not wanting to call you because they are afraid they might bother you and you have so much going on. Then they stop inviting you out to things because you can't go and even if you did, your days are so much different than anyone elses, it's hard to find common ground to speak about. Next thing you know, it's been months since you've spoken to your "friends." You get the obligatory Christmas cards and Facebook responses, but you know not a single friend knows what is going on in your life everyday. The fact that your whole life revolves around one little person and their needs, precludes the possibility of maintaining anything but the strongest of friendships. And though your employer may be sympathetic to your absences and short comings, everyone has a business to run and you are not a valuable employee. You quickly learn what it's like to live just outside of the world that everyone else seems to be apart of.

These are the lessons I have learned the hard way. Live life to exhaustion. Follow your dreams until you physically cannot possibly move, and then keep dreaming you will do it someday. Love your children with all of your being. I cannot imagine my life without them, and though they may exasperate me, I will do anything in my power to make them happy. Take a chance, make a move. Hope and pray for the best when things are not certain. If friends are true friends, they will be there, you don't need the rest. Keep fighting. Never give up. These are the thoughts I have as we enter into 2015. Once again I am hoping and praying for a better year. A year of peace. A year of health. A year of life.

I wish everyone happy holidays and a happy New Year.

Sunday, November 2, 2014

It's November again

It is way past time to update the blog. Once again life has gotten in the way and time is passing before I can realize it. When I look back to where we were this time last year, I amazed at how much has changed. This time last year Audrey was being readmitted for the second time in a week for her motility testing. We didn't go home after that testing for 3 long weeks because she had test after test, procedure after procedure and finally surgery and recovery. She had so many procedures in radiology she had actually gone over her maximum radiation allotment by the time we went home. After that she had sepsis after sepsis until her big downfall in February. This year has definitely been an improvement in quality of life. Though from the outside looking in, it may seem that she is doing better health wise, it is really that we have become better at managing her at home. I have become a pro at giving multiple fluid boluses, dextrose infusions, IVIG, and an endless list of IV medications, all to keep her out of the hospital. This plan has worked, she has only had 2 hospital stays for the whole year (knock on a whole forest of wood!!) but it has definitely come with it's consequences.

Audrey is just too complicated for home health. This seems to be a concept that is completely foreign to most people. The general public assumes, the doctor orders nursing care, the nurse shows up, life goes on. This couldn't be farther from the truth for us. Audrey qualifies for 24 hour nursing care. She has all the waivers in place to fund it. But despite 18 months of recruitment and meetings and training 2 RN's myself, ultimately no one was qualified to take care of her. There is a huge discrepancy in RN's who work in the hospital and those who do home care. Not to say there are not a few little gems who have been scooped up like the treasures they are, but on the whole a home health RN does not go to work expecting to manage crashing BP's, hypoglycemia, steroid drips and IV med after IV med. This however, is exactly what Audrey needs. This is what we go through night after night, because of course, night time is the hardest time for her. So her nursing company gave up on her. They weren't willing to fund a RN who could actually take care of her so they discharged her from their service and said "Good luck!" I have tried hard to find someone else. I have called every single nursing agency who will service my area and not a single one is able to provide for her. So thanks to a couple of awesome nurses I work with, I have been able to work a few shifts here and there and take a much needed break from my 24 hour, 7 days a week nursing shift at home. I could debate the unfairness of it all, lobby for better paid nurses and more training for the home, after all with the rising costs of health care more and more, sicker patient swill be transferred to their home, but who has time for all of that?? I can barely manage hour to hour day to day.

Maybe some of you wonder why I do I do all this at home? Why don't I just take her in to the hospital and let them manage her? Last year she spent 72 days inpatient. This year, if I had taken her to the hospital everytime her BP was low or her glucose was wacky, she would not have been home at all. There's not a day that goes by that there isn't something wrong with her that she could be in the hospital for. But why would I do that to her? For some reason Audrey was given to me. After all my training and schooling and experience, I got this amazing, special girl, who I am completely qualified to care for at home. And because of my ability to keep her home she has experienced things this year we never thought possible. Audrey was able to dance. She was in a ballet recital! She has made a best friend. She has found a best friend in our puppy. She spent months this summer getting to know her grandma. She went to "the world" (Sea World) and she was dressed as a princess at Disneyland. She has had more QOL shoved into this year than she has in the past 4, that is why I keep her home. That is why I stay up late every night checking BP's, glucoses, and urine dips, emailing and texting doctors, because she would have missed this all if I was not able to do this for her.

Don't mistake me for some sort of angel though. One of the most upsetting sentences that I hear daily is, "I don't know how you do it." I don't either, but if it was your child would you chose not to do it? If you had this perfect healthy baby for 18 months who suddenly got ill and required so much care, would you decide not to give it to them? What would you do instead? Put them in an institution? Let them die? Those are the options I face with Audrey and I don't even see them as choices. So everyday I keep going, keep sacrificing. When I stop and think how much I, we have lost because of mito, I want to scream and cry. My marriage, my career, two homes, more friends then I would think was possible, my whole future. Everything has been erased and now boils down to taking care of the two people who depend on me. You never realize how casual relationships are, how fleeting friendships are, until you are tested with the ugly truth. Suddenly friends don't want to talk when they can only hear how sick you child is and how tired you are and husbands don't want to stick around when they have to hold your hand while hearing your child will be lucky to make it to 18. Even family members disappear. Then you are left as the only person responsible for more than you ever thought possible, completely alone in the world with only a few fellow moms who understand how hard you are trying everyday. The rest of the world moves on and passes you by with an occasional "I don't know how you do it" or "You're doing a good job." This is my reality every single day. So don't assign any virtues to me that don't exist. In the end I am just a mom doing the best I can to give my children the best life I can despite all that has been taken from us.

Sunday, September 14, 2014

Mitochondrial disease awareness week

Mitochondrial disease awareness week

It's that time of year again. While Audrey has had much fewer stays in the hospital, the stays she has had have been serious and life threatening. Overall though, the last few months have been better. While I was hoping it was that her mitochondrial disease had finally stabilized, the events of the past couple weeks without her feeding tube and medications, have shown me we have just gotten really good at managing her.

So today is 99 days out of the hospital. It is the start of mitochondrial disease week. With these two facts, we are going to celebrate and hope that the words "mitochondrial disease' will reach more people than ever, someone who will find a treatment for my little girl.

Tuesday, August 5, 2014

It's been awhile!

Once again crazy life has detained me so it's been awhile since my last update. Many new things and many things remain unchanged. The good news is, Audrey has only been inpatient once since my last blog! She went 90 days to the date! That's the longest she's been out of the hospital since January 2012. This is definitely a trend I hope will continue.

Medically, she's not doing better. We have been able to do more at home to keep her here rather than in the hospital. It's a lot more work for me but so worth it to keep our little family together!Audrey's doctors are, as usual, pushing the envelope of treatment for her. Her amazing genetics doctor is challenging himself to find ways to help her and other patients with her genetic mutations. For the moment we have stalled because we have tried everything, even the "in theory this may help" but I know the science will come up with something soon.

Audrey's endocrinologist has also been hard working. She has thought "outside-the-box" for Audrey and she is now using a insulin pump to deliver continuous hydrocortisone to treat her adrenal insufficiency. We found out through labs that Audrey metabolizes cortisol about twice as fast as most people. This made her management very difficult to control. Since starting her pump though, she has been doing much better! We are so happy to be finding these small things which dramatically improve her quality of life.

And lastly, her GI doctor. He works tirelessly for Audrey (and his other patients) trying to manage her GI system. Unfortunately she has complete failure right now and while we hope to improve this, no one has yet seen any of the children with her mutation improve their GI function. This is a very hard pill for me to swallow. I am hopeful that they will find something to help her but I am realistic enough to know they likely will not. That being said, at least the surgeries she underwent last year have kept her out of the hospital. We had hoped she would be able to tolerate more formula after her ileostomy but that is not the case. What her ileostomy has done, is allow her to stay home and prevent the massive abdominal distention and pain she was living with last year. Last year she was inpatient at least once a month, usually for a bowel clean out and now we are able to avoid that. That alone makes the whole surgery worth it. Like her genetic treatment, we are just about at the end of the line for options for treatment of her go failure at her age. As she gets older additional medications may open up (ones that are approved for adult use only) or we may go the route to try some under compassionate use, though the risks and benefits will have to be carefully weighed. If there is one miracle she needs most, this is it.

Speaking of getting older...guess who is turning 5 soon?!?! I can't believe she is one month from being a 5 year old. Everyday that I see her grow and develop and learn new things is such a miracle because I know how precarious her life is. I have loved seeing her push her self the last few months to do what she really wants to do which is ballet. Last year when she started asking to do ballet, there was no way she would have been well enough or out of the hospital long enough to try. Now she takes a toddler class twice a week and while its hard on her body and takes a lot of medical prep work on my part to get her there, she's so amazingly happy I would move heaven and earth to be sure she gets to go. I can't wait to see all that she will continue to accomplish! Keep it up my girl!

Sunday, February 23, 2014

February: The month we lost

The title of this post covers many things. Audrey was admitted on the first of February and discharged on the 22nd. Just 6 days shy of the entire month of February. This admission was so different from any other admission she's had. First she was really sick. While she has been really sick before, this was park the crash cart at her door in the ICU sick. Audrey had never been in the ICU before. She had never required vasopressors or rapid infusions of liters of fluid and blood products just to stabilize her. This was the sickest I have ever seen my baby and I have never been so scared for her. Seeing your chronically ill child this sick makes you confront some difficult facts. First, odds are this is not the last time I will see her go through this same scenario. The nature of her illness prevents her from being able to handle stress and infection like others might. Second, one day, this may become too much for her body to handle. This is the hard part of chronic disease. The hard part of a progressive disease like mitochondrial disease. Things are not likely to get better. This reality made this admission much more difficult for me than others.

Back to the title, Audrey lost major ground in her battle against dysautonomia. While at first we thought her vital sign instability was temporary due to her infection, we soon realized her body was actually unable to control itself enough to come off her vasopressor drips. The problem with dysautonomia is that there is very little to treat it and there is only one doctor in California who will see a pediatric patient with dysautonomia. You can imagine how popular he is and what his wait list looks like. The intensivists and all of Audrey's doctors brainstormed and tried multiple ideas and finally had to consult with outside specialists to figure out how to treat her dysautonomia. So she is on a few different medications which have helped control her vitals signs but she is still very dizzy and needs around the clock medication to control her nausea.

In the midst of her ICU drama, we find out Audrey has a new problem. She can now add adrenal insufficiency to her list of diagnosis. While its possible it has been going on for awhile, she was tested last year so its likely that it was the result of her infection. Thats what happens with mito. Every serious infection or stressor can lead to new symptoms and problems. So, for those that don't know, adrenal glands make cortisol which is an essential fight or flight hormone. Without this your body cannot respond appropriately to any stress whether its a hot day or an ear infection or a car accident, your body needs to be able to respond quickly and Audrey can't do this. Sounds familiar to dysautonomia right? It's been very difficult to tease out which symptoms belong to which diagnosis. Adrenal insufficiency in a child like Audrey is, of course, difficult to treat an manage because her body already doesn't handle stress in the best of circumstances. This will be an ongoing ordeal requiring patience from me and her medical team as we learn how to support her in this process.

So Audrey is home now. She's sicker than she's ever been. Her new baseline is way below where her sickest baseline was. I can only hope she will be able to get back some of the quality of life she had before becoming ill but no one knows if this is a realistic goal. For now we will continue this impossible home schedule with medications every 1-2 hours from 6am to midnight. With vital signs and urine dips and check ins with her doctors.  Everyone is so happy for us to be home but this is definitely the saddest I have ever felt coming home. I feel like we just lost a major battle and I don't know how we will rebuild and continue on. Her health status is so fragile I'm afraid to take my suitcase out of the car because one wrong move will have us speeding back to the emergency room. All I can hope for is some stability to slow this ever progressing, life-stealing disease.

Saturday, January 4, 2014

New year, new hope

It's been nearly 7 weeks since Audrey's surgery. She's been in the hospital twice since her initial discharge on the 25th. We've been struggling with urine infections since early October. With her last admission we added Infectious Disease to her medical team to help us get a handle on her. While they initially thought they would be able to make routine recommendations, they quickly understood the complexity that is Audrey. Her main problem is her dysmotility. She has spent the last 2 years on first erythromycin then augmentin trying to make her intestines move. Thrown in there are times she's been on flagyl or neomycin for small intestinal bacteria overgrowth. Then in March when we started cathing her, we added keflex to the mix. We've spent the last 2 years wiping out all the normal good bacteria from her body so we are left with more serious bacteria which is why she gets ill so quickly. Audrey is unable to use probiotics since she has a central line and unfortunately she's just not tolerating enough feeds to get her GI tract to make normal bacteria. There are a couple ideas we will discuss with her GI and urologist this month to see if we can get a different plan. For now we are holding our breath hoping she does not get sick before then.

Energy wise, Audrey is doing amazing!! When she is out of the hospital and feeling good I feel like I have my little girl back. She is feeling so much better. Even though she's not tolerating much feeding wise, she is so much happier and feels so much better, her surgery was definitely a success!! It may seem drastic to some to put her through an ileostomy and still be completely TPN dependent, however, if you could have seen what she was going through everyday you would understand. She had severe abdominal distention as her baseline, she had an enema every single day just to pass gas, she couldn't tolerate a single medication through her j-tube without extreme pain. That was her life everyday. She was tired and in pain. Now she is happy. Her tummy doesn't hurt, her medication list is cut in half and her body finally has energy to play instead of using it all trying to make her intestines work. It truly has been life changing for her.

While we have a long way to go for her,  I am finally feeling hopeful. This journey is so hard. Everytime there is an improvement somewhere, either something else fails or the improvement is short lived. But I have hope for Audrey. We are living the motto "Carpe Diem" right now. We are taking full advantage of her good days and making them amazing days, knowing she will likely need a day or two to recover. We are rejoicing that she is even having good days since it has been months without them. We are hoping to spend less time in the hospital this year. Last year she spent 77 days in the hospital. Fingers crossed it is much lower this year!! Audrey has also been matched with a runner in the Irunformichael program so we are so excited to participate with that. Over all we are looking forward to this new year and all the new chances it will bring.

Best ostomy belt ever!

First time swinging in over a year!
She loves her princesses!

Best big brother ever!
She was deliriously happy (literally)

What keeps her going!