What’s been going on…

This post is for all you who have been wondering what on earth has been going on with All the Marletts…thanks for caring.

If our main doc got paid for each and every e-mail he receives and sends about Lucy he just might have enough to retire, but then we would be without him, and that would be no good!  Last night I sent an e-mail to our good doc who just so happens to be in South America helping another family in need, yeah he is amazing like that!  He e-mailed me back saying that he is “concerned”, and then discussed a plan that he was going to relay back to the other doctors in the group.  Concern, concerning, concerned, those three tenses just about sum up Lucy’s past, present, and future health! 

I have mentioned on more than a few occasions how Lucy has been battling reoccurring yeast, she is coated with it from head to toe!  In particular she has been suffering with severe oral thrush that we just can’t get rid of!  After several e-mails, discussions, appointments, and cultures, we learned that Lucy is growing a very rare form of candida yeast that is resistant to the antifungal that we have been using to treat it.  Infectious disease was consulted, and it was decided that we need switch Lucy’s antifungal.  This type of med change typically requires a stay in the hospital; however, Dr. R was able to get permission from infectious disease to allow us to have her first infusion given in day med.  This  meant no overnight hospital admission, but if an admission was the only way she was going to be able to get the med, we were willing to do it.   That’s how badly she needed to get started on this med!  Poor girly, the corners of her lips and and mouth were covered in blisters.   

Since starting Lucy on the new anti-fungal on Monday, she has developed quite a few concerning symptoms: diarrhea, fever, tachycardia, erratic heart rates, hypotension, and vasodilation.  Why am I surprised(insert sarcasm, fatigue, and worry)?  Antifungals are difficult meds to be on, this particular med that Lucy is on came with an eight page packet of side effects.  What’s confusing is that our girl already experiences a lot to the side effects of the medication.  After talking with the on call doc this weekend, we have decided to do an on-off-on trial with Lucy to see if indeed her symptoms are related to the new med, if her symptoms improve while off of the med then we are going to have to decide whether to continue or change her to a different anti-fungal.  Of course our main doc is still out of the country which makes these decisions all the more challenging.

In other issues, because you know there’s always more, we recently had an appointment with pulmonology.  Lucy is not new to pulmonology, early on her life we had a need to definitively rule out cystic fibrosis as a possible diagnosis.  However we  have been referred to a new pulmonologist, one who specializes in neuromuscular diseases.  In the past few months we have struggled with seeing a decline in Lucy’s overall muscle strength and vascular health.  She is a shallow breather, she has been most of her life and her body has compensated for this up until now.  Her need for supplemental oxygen is not going away post transfusion, she visually looks blue at times, and her profusion in her hands and feet are little to none .  The thought is that everything that we are seeing in Lucy is as a result of disease progression, and that Lucy is in need of more support respiratory wise.   A sleep study was ordered to get a better picture of what is happening to her when she is sleeping, discussions about which meds we can use to help open up and clear out her lungs were had, and orders for chest PT, a.k.a vest therapy, were given.  We don’t disagree with any of these recommendations, we feel that they will help to keep Lucy at her best.  What is difficult is knowing that we are having discussions about disease progression and not being able to do anything to prevent it!  We can treat the symptoms but not the disease.

Happy New Year!

On your mark, get set, go go go…that is exactly how our year started, not unlike last year, or the year before that…

Our life is busy, of course it is we have four kids!  Our days are full of the typical everyday stuff and the the not so typical everyday stuff.  Last year at this time I was begging and pleading God for more time.  I specifically prayed not just for time, but for more memory making opportunities, and for time to process and reflect.  We have a peace about our life this year that we did not have at this time last year.  Prayers big and small get answered everyday, and we are blessed again to be beginning another year as a family of six!

We begin every new year off with the celebration of Jack’s birthday, he graced us with his presence eleven years ago on January 4th.  They(whoever “they” are) weren’t kidding when they said they grow up fast!  Why is it that I feel older on Jack’s birthday?   Jack is growing up to become one fine young man, he makes his mama and his daddy so proud.

So many have asked us about our holidays, thanks for asking.  We celebrated a wonderful Christmas at home, the six of us and my parents!  The week leading up to Christmas was stressful, of course, but not just because I had a million things to accomplish for the holiday, but because Miss Lucy had to go and make things a little exciting, you know with the whole serotonin syndrome business.   If I recall the week leading up to Christmas we could be seen at DuPont almost everyday for one reason or another.  Lucy got a new GJ-tube(tube #24, but who’s counting) for Christmas as she was in need of one that would drain! Jack happened to get a virus for Christmas this year that landed him in bed for the better part of Christmas and a few days following, not the best present but he has fully recovered back to the Jack we know and love, so all’s well that ends well.

As Drew and I surveyed the presents under our Christmas tree and the stockings all hung by our chimney with care, I was in awe of all that we had accomplished.  How and when we had time to communicate with the North Pole I just don’t remember, but I impressed myself this year!  All of our kids were happy, which is the best gift any parent can get for Christmas.

Merry Christmas to all…

and to all a good night!

A quick update…

So far cultures are still negative, we would be very surprised if that changes. Lucy’s urine culture did come back with bacteria that she has grown before. We believe that her bladder is most likely colonized with the bacteria, we are not really surprised since she has a neurogenic bladder and is cathed several times a day, everyday. We believe that Lucy experienced a very rare side effect, serotonin syndrome, from the medication that she started on Friday. We started Lucy on Lexapro, it is an SSRI-selective serotonin reuptake inhibitor. These types of meds are most commonly used to treat depression, this is not why we were using this med for Lucy. SSRI’s are also used as an effective pain management medicine for individuals with neuropathic pain, the type of pain that we believe Lucy experiences on a daily basis. In theory this would have been a good alternative to narcotic pain relief for Lucy…if it had worked. Serotonin syndrome can be fatal if it is not detected early, the treatment is discontinuation of the medicine and supportive medicine for the resulting symptoms. Lucy only had one dose of the medicine, thank God, we don’t even want to think about what could have happened if she had any more than that. Lucy’s autonomic system is storming due to her adverse reaction to the med; blood pressures, heart rates, body temperatures, are all over the place. Her neurological system took the brunt of the impact, affecting her balance, speech, movements, and causing seizures. As the medication metabolizes through her system we are seeing a decrease in her symptoms.

When discussing Lucy’s symptoms with our main doc he said that he was surprised, but then again not, it’s Lucy! I know I have mentioned this before, you know the fine print that reads less than 1% of people will experience this, that, or the other when taking this medication, that’s Lucy! Our palliative and hospice care team have officially been initiated into Lucy’s medical world.

No news…

I have not heard from the hospital or our main doc, which means that cultures have not grow anything as of yet, that’s good.  The cardiologist on call last night confirmed, based on the EKG they did in the ED ,that Lucy is experiencing sinus tachycardia.  We’re not at all surprised with this finding, but what we are wondering is what is causing it.  The fear is that it could become more serious, for now we are monitoring her heart rates very closely, and will be in contact with our main doc and cardiologist tomorrow…I see another holter monitor in Lucy’s near future. 

We started Lucy on a new med Friday night which we hoped would help her neuropathic pain.  Although very rare(for the general population that is, but we are talking about Lucy here so rare is really not that rare), there is a possibility that Lucy’s symptoms are as a result of an adverse reaction to the new medication.  Needless to say, we discontinued the med and will be in touch with our palliative and hospice care team to discuss other options for our girly.  Lucy’s urine came back with an impressive number of white blood cells in it, indicating infection but we won’t really know until we get the culture back.  Perhaps our biggest fear is that the yeast that has overtaken Lucy’s GI tract is circulating in her blood system.  Yeast and central lines are not good, not good at all.  Yeast is difficult to grow on a culture, it likes to hide and then surprise you with one really sick kiddo.  We are praying that this is not it!

Something is up, what it is we just don’t know yet…this is the hardest part of all of this, the not knowing! Once you know what it is, then you can do something! 

Twas a week before Christmas…

And all through our house sounds of Lucy’s heart rate monitor are alarming us all!  We are currently sitting in the ED getting labs and cultures drawn.  Lucy’s heart rates are high >170bpm, she is sitting right at the cutoff for what we consider a fever, and she looks like a little tomato.  On the plus side, she is not really acting “sick”…yet.  We spoke with our main doc, who just happens to be on this weekend, and he agreed to have us come in for labs, cultures, and a EKG and then we can go home and wait for him to call.  We both agree that this is out of Lucy’s norm, and with Christmas just a week away we are trying to be proactive.

Update:

Six hours later we are still here, in the ED.  We are waiting on lab results.  Cultures are cooking and Dr. R has convinced the ED docs to let us go, just as soon as we get a few more lab results back.  Ugh…time definitely does not fly when you are in the hospital!  We are looking forward to picking up our other three kiddos and going to bed.  Please pray for negative cultures, and that things calm down with Lucy’s body. 

Update:

We arrived home at around 12:30 am.  We are going to be keeping a close eye on our girly.  Here’s hoping for a good nights sleep, lord knows we could use one!

The Readers Digest version…

Sad but true, I don’t have enough time during the day to sit down and write a blog post from start to finish.  I very often begin posts only to find that days later they remain incomplete.  By the time I sit down to finish writing, I am often too tired to complete a thought much less write one down.  My lack of blogging is certainly not because of a lack of things to blog about…quite the contrary. Things have been busy in our house…I am hard pressed to think of a time when they have not been. In an effort to catch up, I am going to attempt to write the Readers Digest version of medical events.

Lucy an I are spending our afternoon at the hospital in day-med getting a blood transfusion, which is why I have time to write.  Nothing slows me down more than being on hospital time watching blood drip ever so slowly into our little one’s body.  Luc is in need of a boost that blood gives her, we are looking forward to rosier cheeks, lower heart rates, and hopefully a decreased need for oxygen.  Hematology-wise Lucy has developed some new issues, aside from her reoccurring need for blood, it appears that she has developed a clotting disorder that has us all wondering why.  A while back Lucy occluded all four of her IV lines while they were running, to quote our infusion nurse “That’s impressive, but then again it’s Lucy!”.  For the last month we have been running tests on Lucy’s blood to help us understand the cause of her clotting issues.  Dr. R is consulting with hematology in regards to all of this.  On the plus side, we got approval to use TPA ,the medication that is used to break up blood clots, at home which means that when Lucy’s line clots off we no longer have to sit in the emergency room or day med for hours on end waiting for blood to dissolve.

Lucy is battling yeast again still, she is covered in yeast from head to, umm... bottom. For the last month we added oral antifungals to Lucy’s g and j tubes as well as swabbing her mouth with the med in hopes of eradicating the yeast from her entire GI system. We’ve had Lucy on a daily prophylactic IV antifungal since July in hopes of keeping the yeast from entering her bloodstream thus preventing it from getting to her CVL, but it does not work in her stomach and intestines as it does not go through her GI system. Infectious disease has been consulted on this issue because in theory her IV antifungal should be preventing the thrush in Lucy’s mouth. It is thought that Lucy’s poor immune system is playing a role in this issue. We had recent discussions with Dr.R about adding IVIG therapy to Lucy’s list of therapies. In theory this could help to boost Lucy’s immune system, but as we have learned Lucy’s body has other theories.  

We have not had any sleepovers in the hospital as of late and for that we are very grateful!   That is not to say that you haven't seen us around the hospital, we are here at least once or twice a week sometimes more.  In an effort to keep us from sleeping over we continue to increase Lucy’s level of care at home, trying to be proactive.    When Lucy had her most recent central line(CVL) placed in October, our surgeon cautioned us that access is an issue for our girl, she is not easy to get a line into, as a result we need to do everything we can to preserve the integrity of  this line.  In an effort to keep the infections at bay, we began daily ethanol lock therapy on Lucy’s CVL.  This has added another step to our daily med schedule that makes things even more complex, our home nursing agency has expressed to us that they are in uncharted waters when it comes to staffing nursing at our house.  Lucy’s level of care is A LOT, but because we have all of the help that we do have we are able to continue to live our life at home!

When you have a child like Lucy you are always waiting for the other shoe to drop, so to speak…we know she is going to have another line infection,  get sick with another virus, and end up in the hospital again sooner or later.  Drew and I  have had some very serious discussions about life with Lucy’s team, we all hope to give Lucy “the best longest life”.  Mitochondrial disease is a very difficult disease to try and predict, nobody knows what the prognosis is for our girl.  Dr. R uses phrases like “she is not heading in the right direction”, or “she is my sickest patient at the moment”, or “Lucy is the one I am most worried about at the moment,”, but he will also be the one to tell you that you never know what can happen.  He is a glass half full kind of guy, we often refer to him as our eternal optimist, but even so he continues to tell us that he is worried about our girl.  She is very unpredictable, yeah she likes to mess with your mind and heart like that, after all she is a girl.  Knowing what we know about Lucy’s health, hoping for the best, and with the support  and encouragement of Lucy’s team Drew and I agreed to put Lucy on pediatric hospice.  I will write more about this in a later post, but know that this does not mean that we are giving up on treatments, or that we feel that Lucy’s time here on earth is coming to an end.  It is our hope that by adding hospice to our team we will be able to provide Lucy, our entire family for that matter, with supportive care in our home environment.  I would be lying if I said that this was an easy decision, nothing about Lucy’s life has been easy, but we have some peace and comfort in knowing that we have the resources available to us that hospice can provide.

Thanks as always for your thoughts and prayers, love and support, we could never do this alone.