Wednesday, April 29, 2015

Hello Sping and long awaited updates

Hello everyone. I am such a bad blogger! Life gets busy and this seems to be one of the last things I do. I should write more as it is so therapeutic. Life has been good in our home. Logan had a great winter. He stayed pretty healthy and avoided any major infections. We have worked on tweaking some things over the winter. We have adjusted his tube feedings a few times and have been trying to get his seizures under control.

Logan has generalized seizures up to 30 times a day and a couple grand mal seizures a day. We have done many medication adjustments and have recently added a third medication. Hoping this one is the trick. It is so distressing to see him experience seizures all throughout the day.

We have been patiently, at least as patient as I can be, to hear back from our genetics clinic. They sent a request to the National Institutes of Health, Undiagnosed Disease Clinic to look at Logan's case. They sent us a letter a couple of week ago stating they were not going to accept Logan into the program. This sounds harsh, but wait for it...they think a gene mutation that he and I share is the answer to his problems. They not only think that HUWE-1 is the answer for us but gave us the name of a doctor that is researching this gene. From my quick view of her research in medical databases, it looks like it is being studied from a cancer perspective. It will be interesting to see if our genetics team finds more and learns more from her. I am hoping she is able to reach back from their requests and help us out.

With genetic testing these days, there are more and more rare diseases being diagnosed every day. Maybe Logan can be part of the puzzle to a diagnosis for many other people. I know we cannot cure him, but we can help future generations in our family understand the possibility of this being carried through to other generations and at some point in the future, eradicate it. I love my son, but do not wish this upon any other child. Logan is my angel and teacher, but if he was to choose his path, would it have been this one? I don't know and we will never know.

In the meantime we plug on as we always have. We are having a little shortage of home care nursing staff. It seems as though nursing in general is in need across our area. I hear it from our home care company, nurses I know in long term care, and within the hospital community that I work in, that experienced nurses are hard to find. I can't definitively say what the true factors are attributing to the issues but I have my own conclusions. The economy is pretty decent, gas prices aren't too bad, baby boomers are aging, health care is offered to more and more people every day, we have less primary care providers, and our population is growing every day. I'm no expert, but I feel we are at a tipping point in health care. More people are using services and less people are available to provide services. Logan and our family are unfortunately affected by this as we deal with a shortage of nurses in our home. I do admit we are picky but my next few paragraphs may speak to why that is.

We just had an appointment with Logan"s pulmonologist. I truly adore, respect, and appreciate him. Our visits with him fill my cup! I say that as a nurse, health care professional, care provider, and parent. He spends the time ensuring Logan's needs are addressed and addresses the family unit as well. He is so incredibly knowledgeable but beyond that, he is honest and factual. Aside from the developmental pediatrician that told me three years ago "something is wrong with Logan and we are going to figure it out", our pulmonologist has been up front from the beginning.

I will never forget the first visit we had with him. I was on my own with Logan. That was back in the day when I was able to take him out and about in his baby carrier with not a care in the world. At the first visit, he walked in the room (Logan was in my arms) and sat in a chair just inside the door across from us. He sat, watched and listened as Logan breathed. I knew what he was doing and did not feel awkward or put off by it. As a nurse, I understand how easy it is for a doctor to lose the ability to gain a good assessment on a child the minute the child sees them in the room. From that moment forward, our doctor gained my trust.

During that visit, we had a very open and honest dialogue. He told me he was concerned about Logan's respiratory status and was worried that his future would include a tracheotomy. Remember, I was there by myself. It wasn't easy to hear, but I needed to know what the future may hold. As an ER nurse, worst case scenario is how I am trained. At that point, these conversations were becoming somewhat normal. I prepared myself to hear the worst case scenario at each visit with each new specialist.

So now we are back to the present and I am talking with him about advanced directives and code status. Logan has a palliative care team that helps us address this at least every six months. We have yet to make a decision to draft any paperwork to decide on how we would proceed in the event of an emergency or significant medical event. Joel and I have discussed it, but have not moved forward from there.

This is a hard topic as we have always looked at Logan's situation as being respiratory. He has a tracheotomy and requires so much assistance with suctioning and maintaining a clear airway. We have been programmed through training and symptom management to address this first. As a health care provider, airway is always the first area to address. Well, trust me, we have this one down to a tee! We are so good that his pulmonologist was beyond impressed that Logan stayed out of the hospital all winter long. In fact, he didn't even require a visit to the doctors office or urgent care. We were able to rely on our nurses to implement orders quickly and notify his pulmonologist team when further medications were needed to treat respiratory symptoms. Our nurses are truly top notch. They are the reason Logan has such an amazing quality of life and remains so healthy despite all the things going against him. Their team work, communication, and dedication to Logan far exceeds my expectations.

Back to the pulmonologist appointment.... we did discuss Logan's advanced directives and code status and had a frank conversation regarding concerns related to his seizures. Logan has a lot of seizures. He has two kinds of seizures as I stated earlier in the blog. They are short lived and don't seem to cause him too much anguish over a long period of time. Don't get me wrong, they don't look comfortable and I'm sure he is sore and upset afterward, but they last less than 30 seconds a piece which is encouraging.

The one area that I was thrown a curve ball during our pulmonologist appointment was the possibility of seizures being a life threatening event. I have for so long put in my head that the biggest concerns for Logan's long term health would be a respiratory virus/bacteria that would move toward a nasty and worsening case requiring us to make some big decisions. Well, after this visit, seizures have also been brought up as a great concern. Our pulmonologist does not only have experience in that line of work, he is also an intensive care doctor. He has seen the worst of the worst over the years and understands the full gamut of presentation and long term complications. Seizures are a great worry in our world for Logan but no one has truly spoken up and put heavy weight behind the concern that continued seizures would have on Logan. At this appointment, the concern was fully laid out and I certainly heard it.

It is never easy to hear someone tell you they are worried and for them to fully explain the amount of concern they have. I did listen and it was pretty brief but trust me it wasn't a quick comment on the fly, it was made with the intent to spark thought for Joel and I. I am not one that can jump right into a territory I am unfamiliar with and begin a strong line of questioning. I am one that needs some time to chew on concepts, do a little research if needed, and then come back with my line of questions. Since that appointment, I have a boat load of questions and concerns. Thankfully, I feel confident enough in the team around us and with Joel, that I am able to come to an understanding that no matter what, "it is what it is"!

We cannot control everything. We will do our best to provide Logan with a full and happy life. We will do our best to support one another in our marriage and ensure that Lauren is a healthy and well adjusted child among the chaos and confusion. When curve balls are thrown our way, we will duck, jump, plow ahead, or create an invisible shield to allow ourselves to manage the changes we encounter.

Logan is a true angel on this earth. Just holding him and looking in his eyes gets to the soul of who he is as a human being. And that interaction is not only one sided, he looks deep into my soul and touches me in ways no one else can. I feel he does this to everyone that comes into contact with him. His dark brown eyes, long stares, and calm touch transcend much farther then basic human needs. I sit with him and cuddle him into my chest with skin to skin contact and we connect in ways that words can never describe.

So yes, we have some difficult times ahead of us. Logan's brain is degenerating. No one can tell us what this may bring exactly but we are continuously reminded of what we must bring to the table to fulfill Logan and our lives with what is most important. With the warmer days, time outside has become important. Thanks to one of our nurses, we have another seating option for Logan. We have a split level home with an upstairs, two main levels between kitchen and family room, as well as a basement and with the addition of a secondary seating system, we are easily able to include Logan in meal time and time out on the back deck.

As always, thank to those that continue to support us on this journey. There are good and bad days, but truly mostly good days and any check ins's are welcome. I may break down on you when you ask, but please know that I am always willing to share our story. It may not be easy, but it is real!

Monday, March 2, 2015

Maple Maze

Today we had a day off from school. It was warm enough to venture out so Logan, Lauren, Cora, and I went to the Maple Maze. It's at the community center and is a big indoor jungle gym. Of course Lauren was in heaven from the start! We scoped out the place to see what Logan could do. At first it wasn't very promising but then we figured out how to make it work for him. 

He and I sat on a purple dinosaur for a while. He sat there and watched all the kids around him. Then he hung out with a crocodile and we found a fun spinning wheel. It was just enough for him. The other bonus, despite it being loud and busy, he was calm and had zero seizures. He also had a groupie named Charlotte! She was so interested in him and followed around Lauren and played with her. All in all it was a good day!

Wednesday, February 25, 2015

What's going on

I had a pit in my stomach feeling when I got home tonight. Something just isn't right with Logan. Nothing acute, it feels like the occasional decline in function or loss of skills. Logan has had a really tough time with seizures. They just aren't under control and seem to be worse. With all the med changes it is hard to decipher what is worrisome versus a medication side effect. 

Logan has had a slow heart rate intermittently while sleeping at night. This isn't a big deal as he does not drop his rate low enough to need intervention. What is worrisome is that he does it and drops his oxygen saturation. His nurses do everything they can to get his sats up without oxygen but end up needing it as stimulation and position changes do nothing. He is virtually unresponsive. I have tried my mean nurse tricks to try and get him to wake up with little success. 

I have listened to nurse after nurse voice their concerns and uneasiness with his status. We have a new seizure medication change but that does not relieve the teams worries. I have a call out to palliative care and they want to see Logan.

I hope to hear that he is being overmedicated, but am scared that that is not the case. I pray that we are not seeing a decline in function. I worry that his brain degeneration will at some point mess with his ability to perform daily protective functions. He has already lost the ability to protect his airway thus the trach. I don't want him to lose the drive to deep breathe and oxygenate.

I am a nurse so you would think I know what the next steps in our journey entail but I don't. I have no idea what to expect next. I go off of my gut instinct as a mother and wait to see if his testing and or doctors opinions validate this. It is rare that I completely miss the mark. I hope my mommy warrior gut is wrong right now. He has been doing so well at school and has been so healthy. I pray right now is just a side effect of med changes and seizures and nothing else.

Logan is such a warrior and amazing human being. He never seems phased by the changes. His mom on the other hand is a little worried! Thank you to those who continue to pray and support! It means so much especially during those times of worry and helplessness. 

Tuesday, January 6, 2015

MRI Results

The MRI results are back. Logan continues to have mild atrophy of the brain. That means his brain continues to shrink. We have been told he has this on previous MRI's. This time they noted that the atrophy has mildly progressed from where it was two years ago. Basically it gives us a reason for Logan to be less interactive, have less movements, and more seizures. 

I am not suprised by these results. I have to let out a little sigh of relief that we didn't hear some major news. This is more like what is expected of Logan's course but I always worry about worse case scenario. Thankfully we were given news that is easier to swallow. 

So we continue on as is. The changes in his seizure medication dose has not made a difference. We are to call next week with an update to his neurologist and may have to change medications at that point. I just want the seizures to be better controlled. It has got to be miserable for Logan. 

I have also tried to connect with the metabolic specialist that saw Logan many many months ago. He was sending off a letter to the NIH to see if they had any ideas about Logan. I called again today looking for an update. Hoping to hear back tomorrow. I am having difficulty getting this doctor to keep me updated. 

...I wrote this blog a couple days ago and hadn't had a chance to send it. Now I feel like saying, "what the hell?" Seriously, I read the post and feel like I am totally sugar coating something that is a big deal. It is a big deal that Logan's brain continues to shrink. That's not normal! I'm trying to play it down so it doesn't bother me so much. It does bother me but this time it doesn't feel as devastating, more frustrating than anything.

I am frustrated because we still do not have a defined diagnosis for Logan. Nothing to say, here is the name and this is what to expect in the future. Instead we are presented a black hole that we are sent down with no end in site. 

I did talk to the metabolic doctor today. He has not heard back from the National Institues of Health. He will try one more time but does not feel optimistic. This time he is going to send all of Logan's records with. During this conversation I finally heard that we may have a diagnosis but can't land on it definitively because there are so few cases. Logan and I (if I remember correctly) have a genetic mutuation that has been found in only a few cases. Because it is so few, the doctor's can't say this is what is causing his symptoms. Those that have this mutation have some similariities to Logan but not as severe and there are some big differences in the cases. 

Well at least the doctor is being open about this and not dancing around it. I finally feel like I am being given some sort of an answer. I have to admit though that it is hard to trust. Logan has been seen by so many people, all with different ideas. My hope would be that the NIH might find his case interesting enough to take a peek at. Maybe there is a doctor in the US that is studying this. I wish I had a magic wand to find out. Ugh!

Other than that, we are in a deep freeze here in Minnesota. Hello WINTER! Logan has been cooped up all week as it is too cold to go out. Poor guy. You should see his hair. It needs a cut desperately. He looks like he fell out of an airplane (as Joel's family would say)! Hopefully warmer weather comes our way and he can get back to school and maybe head to great clips for some boy-scaping!