and to all a good night!
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.
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!
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!
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.
Lucy’s new wheelchair is here! She has outgrown her old chair both in size and medical need. Her new chair is bigger, heavier, more comfortable for her (not quite as comfortable for me to push), can support the weight of all of her medical equipment, has an IV pole, is pink, and has her name embroidered in pink letters on her seat to match her chair. This is just a little something special that our wheelchair clinic likes to do for their kiddos The first thing Lucy said when she saw her new wheelchair, “It my name!”, referring to the embroidery on the seat. She just about feel out of her chair the first time she sat in it because she was so excited to show anyone and everyone her name.
Her new chair has both tilt and recline, which means nothing to those of you who have never ordered a wheelchair before, but to those who have this is the reason that Lucy is more comfortable in her new chair, and also why her chair is so heavy(over 100 lbs.). These two features combined mean that Lucy can be positioned in such a way that we are able to reduce the pressure on her belly, thus allowing her to be more comfortable.
Baby now needs a brand new ride for her new set of wheels. We are nearing the end of the process of purchasing a wheelchair-accessible-van. If you thought buying a car was a process, try buying a car with a wheelchair ramp! Having a ramp van is going to make our life a whole lot easier when it comes to transporting our crew, especially Lucy! We had some specific needs for our new wheelchair-accessible-van, mainly adding a fourth seat to the backseat as opposed to just having the third row back seats. Because of this our options for what type of van and where we could purchase our wheelchair-accessible-van was limited and expensive, the ramp conversion costs almost as much as the van!
Our family has been blessed by the kindness, caring, love, support, and generosity of others in so many ways we feel humbled and honored! There are several fundraisers occurring to “Help Us Lift Lucy into Her Van!”. On Friday night The Hockessin Music School, a school that our family has been involved with for the last 10 years, hosted a family music night in honor of our family in hopes of raising some money for the cost of our van. We never expected the outpouring of support that we received, it’s simply… amazing! Additionally, Musikgarten, the music curriculum that our children have learned from, just released a new lullaby CD. Jill, our friend and children’s music teacher, is selling the new CD for $12.00 as an additional fundraiser. She and her husband are donating $5 for every CD sold, her sister who also teaches music classes offered to donate an additional $3 for every CD sold, and two very loving and supportive music school families each agreed to donating another $1! This now means that for every CD sold $10 is going towards the cost of our wheelchair-accessible-van! I was told that at last count 90 CD’s have been sold! If you are interested in purchasing a CD please leave me a comment in the comments section and we will make this happen.
If you live locally, there is another fundraiser occurring this Thursday, December 8th at Wackadoodles Toy Shop. Betsy, the owner of Wackadoodles, heard of our story and very graciously offered to host a fundraiser for our family. I cannot tell you how amazing it is that our family, our life, our littlest one’s heroic struggle with mitochondrial disease has touched the lives of so many. It is every parents hope that their child make a difference in this world, we could never have imagined that ours would be doing this at such young age! We feel so blessed to have the love and support of so many, to be living among those who care so much, to have a community that wants to reach out to those in need…we could never do this alone! Thank you!
Here are the details for the upcoming Wackadoodle’s fundraiser, feel free to spread the word!
Help us lift Lucy into her Van!
Lucy Marlett is a beautiful three year old girl with Mitochondrial disease. Lucy recently moved to a wheel chair that does not fold and weighs over 100 pounds, which makes a wheelchair-accessible-van a necessity!
Here is where you can help. Wackadoodles Toy Shop has graciously offered to donate a percentage of their total sales for one day to the Marlett family to help with the cost of their new wheelchair-accessible-van.
When: You are invited to attend a fun day of shopping at Wackadoodles Toy Shop on Thursday, December 8, 2011.
Where: Wackadoodle’s Toy Shop 61 Jenner’s Village Center West Grove, PA 19390 (Next to Giant) Phone: 610-869-2404
The Details: Wackadoodles Toy Shop will give you 10% off of your total purchase and donate 10% of your total sale to the Marlett family, or you can opt to have Wackadoodles donate the entire 20% or your total sale to the Marlett family. All you need to do is present this coupon to show your support.
This coupon is good for 10% off of your total purchase at Wackadoodles Toy Shop on Thursday, December 8, 2011. Wackadoodles will donate an additional 10% of your total sale to the Marlett family or you can opt to have Wacakadoodles donate the entire 20% of your total sale to the Marlett family.
Total Sale*__________________________________________
*Offer not valid with any other coupons, promotions, or sale items. This offer is valid for in-stock items only. Total sale does not include tax.
We have so much to be thankful for…
From our house to yours, Happy Thanksgiving!
A few weeks ago we had an appointment with a cardiologist who specializes in pediatric cardiac arrhythmias, also know as a cardiac electrophysiologist for those of you medical junkies out there. Dr. R likes to harass quiz me on my medical knowledge anytime he can, I have to remind him that I specialize in Lucy, not medicine.
For over a year we have observed changes in Lucy’s cardiac health. She was diagnosed with tachycardia, accelerated heart rhythm, last year around this time in addition to postural orthostatic tachycardia syndrome (POTS), in which Lucy’s blood pressures and heart rates would change over 20% with changes in position. We originally thought, more like hoped, that Lucy’s tachycardia was related to her chronic anemia. If we corrected her anemia then we hoped we would see an improvement in her heart rhythms. Sadly, we did not see much of an improvement in Lucy’s heart rates post transfusion. After wearing a 24 hour Holter monitor, Lucy was diagnosed with having sinus tachycardia, tachycardia resulting from the sinoatrial node due to autonomic dysfunction. Little did we know how chronic Lucy’s anemia was, her bone marrow is affected by her mitochondrial disease and as such does not have the ability to make an adequate amount of blood for Lucy’s body. Couple that with weekly lab draws and daily procedures in which blood is taken from her body, and she loses more than she can make up for on her own. Lucy becomes symptomatic of anemia(accelerated heart rates, neurological changes, and oxygen dependent just to name a few) anytime her hemoglobin falls below 10g/dL, her need for blood transfusions is now about every four to six weeks, sooner with illness.
Changes in Lucy’s blood pressures, dramatic edema, and heart rhythms, had us all scratching our heads trying to figure out what could be going on with our girl. Lucy was diagnosed with hypertension during her acute illness in April/May which has proven to be persistent. Clonidine is working to keep her pressures low. We had nephrology do a full work up on Lucy’s kidneys to rule out kidney disease as a cause for Lucy’s hypertension. Although Lucy’s kidneys are affected from her mitochondrial disease, they are not the cause for her hypertension. In July, Drew and I noticed prolonged periods in which Lucy’s heart rates were even more elevated than her normal(140’s/150’s). These prolonged periods appeared to be causing her some distress as she seemed agitated and was unable to sleep well during these times. After discussing these symptoms with Dr. R and Lucy’s cardiologist, we decided to do another Holter monitor test. The results from this test showed changes from her pervious Holter monitor done in December, it showed that Lucy had multiple episodes of premature ventricular contractions (PVC’s), in which her heart would skip multiple beats in a row. We look for trends in Lucy’s labs or tests that indicate change, since this was a dramatic change from December we decided to monitor Lucy again in eight weeks to compare the data. The results from this Holter monitor showed what we feared, Lucy has ventricular tachycardia, a type of tachycardia that can be fatal. It was at this time that our cardiologist referred us to the Dr. T, who specializes in these types of arrhythmias.
Before our appointment with Dr. T, it was decided that Lucy would need to wear a cardiac event monitor which recorded her heart rates over a thirty day period of time…oh yeah, we took that monitor with us to the beach. As if Lucy does not have enough stuff coming off of her torso, we had to find room to attach three cardiac leads and pager like device which recorded every beat of her heart for THIRTY days. At the end of each day, or whenever the monitor alarmed we had to call into the monitoring station to transmit the data.
On the day of our appointment with Dr, T, he ordered a repeat EKG and echocardiogram of Lucy’s heart to compare it with her previous tests. Our reason for meeting with Dr. T was to discuss with him what, if any, medical interventions could be done to lessen or improve Lucy’s cardiac issues. It is always difficult meeting new doctors at this stage of the game, granted if you are having to see a sub-specialist cardiologist you have to have some pretty significant medical issues occurring. Dr. T is not new to complex medical train wrecks like our daughter, but he is also not an expert in mitochondrial disease. During our appointment Dr. T discussed with us the reasons for Lucy’s tachycardia and other cardiac issues. First off, he confirmed her diagnosis of dysautonomia, adding to it that it is severe! Your autonomic nervous system is responsible for controlling things such as heart rate, body temperature, blood pressure, and digestion all areas that Lucy’s body clearly struggles with. Lucy’s dysautonomia is as a result of her primary mitochondrial myopathy. Secondly, Dr. T also discovered that Lucy’s heart is significantly undersized for her body, but is not atrophying! He told us that this is not an uncommon finding in kids with severe dysautonomia. Lucy’s heart has to pump faster to circulate blood throughout her body, one reason for the tachycardia. Finally, small heart size is not the reason for Lucy’s arrhythmias, these are caused by failures of Lucy’s autonomic nervous system to signal her brain to tell her heart to beat regularly. Lucy’s heart muscle is “normal”, albeit undersized, for lack of a better description. She does not have a cardiomyopathy(muscle disease) which is what most mitochondrial patients with cardiac disease have, instead Lucy’s cardiac issues are as a result of a cardiac neuropathy (nerve disease).
What if anything can be done about all of this? Dr. T told us that there is an experimental surgery which to his knowledge has never been preformed on a child as young as Lucy. This is where Drew and I stopped him, we are not looking for Lucy to become the first of anything in cardiac surgery, our goal for Lucy is to give her the best quality of life that she can have in her time here on this earth. Dr. T was relieved to hear this, he agreed that any experimental surgery would be just that, an experiment. The first creed in medicine is to do no harm, he believes that if we attempt to correct Lucy’s cardiac issues either by surgery or with medications that we could be doing more harm than good. All of the medications used to correct the cardiac issues that Lucy suffers with have serious side effects, and will interfere with many of Lucy's existing metabolic and cardiac issues. We must continue to monitor Lucy’s cardiac issues closely with regular Holter monitors, and bi-annual cardiac event monitoring. Due to the progressive and degenerative nature of mitochondrial disease, it is assumed that Lucy’s cardiac issues are not going to get better.
Drew and I were not expecting miracles from this appointment, and we very much liked Dr. T. We along with Dr. R were “hoping” that something could be done to improve Lucy’s cardiac symptoms with little or no side effects. When discussing this appointment with Dr. R, he commented that it was “disappointing but not surprising”. The decisions for Lucy are increasing in difficulty. As a mama, it is heart breaking to know that this disease is overtaking our little one’s body, one organ system after another.
I am referring to germs, they found us and apparently are tough enough to stand up to the copious amounts of Lysol used in our house. Jack has been sick for the better part of a week, he has been struck with what we thought was strep throat and some sort of upper respiratory virus (para virus) that is lingering and according to our pediatrician has the reputation of causing secondary pneumonia. The poor kid has not been feeling well at all, but I sent him back to school on Wednesday after being on antibiotics for 36 hours, and no fever for 24 hours. He looked awful and had no appetite, but we worry about him getting too far behind. I know, I am a contender for “worst mother of the year” for sure. The kid is smart, really smart, but slow, really slow. Yesterday, he came home from school and it was just the two of us sitting at the kitchen table sharing a bowl of soup. He was so tired he could not hold his head up. He shared with me that he did not feel well, but he liked that it was just the two us at the table. We talked about some very intense stuff, he cried, I cried, and told him that I was happy that he talked with me. I am not sure if it was the fever or the emotions brewing inside, but he needed me and I was so glad that I could be there for him. His fever is better today, but his symptoms are not. I will bring him back to our pediatrician on Monday morning if he is not any better.
Miss Sophie woke us in the middle of the night on Tuesday stating, “I don’t feel good…”, which is code for I am going to throw-up all over you in less than ten seconds. Thankfully she was on Drew’s side of the bed because it is closer to our bathroom. I yelled at him to get her to the bathroom a.s.a.p, since he was in a deep slumber. Just as he got her there, he yelled back to me “good call babe!”. Poor Soph, she practically turned inside out that night. Drew had to leave for work at 4 am for a very early morning meeting which meant that neither he nor I got any sleep. I washed five loads of laundry between the hours of 2 am and 7 am all the while attending to Sophie, doing Lucy’s 5:30 med schedule, and washing and cleaning. Sophie’s GI bug lasted only 24 hours, but it had me doing a subterranean cleaning for 48 hours. She was back to her happy perky self by Thursday morning.
Germs are the enemy in our life! Illnesses surround us this time of year, and we have to try our best to keep them away as any illness can be detrimental to Lucy’s life! Lucy’s immune issues are severe not only because she has mito, but because of the fact that she has secondary hemophagocytic lymphohistiocytosis (HLH), or macrophage activation syndrome (MAS). What happened to her in April as a result of coming in contact with the flu B virus can and most likely will reoccur when her body comes in contact with another virus. It is a difficult balance of living life and living in a bubble. We isolate our kids when they are sick as a way of protecting Lucy, but as you can imagine it is difficult. Lucy wears a mask whenever she is out in public, it always amazes me that she never complains about it, it is her norm. I have tried wearing a mask for an hour or longer, it’s uncomfortable not to mention hot. Our kids are programed to wash their hands the minute they walk in the door, eat, or want to play with their sister. We use copious amounts of soap, hand sanitizer, and Lysol products. We are aware of the literature out there that states anti bacterial products are to blame for the resistance to antibiotics, I don’t disagree that there is probably some truth to those statements. However, in our situation it is our best defense at keeping Lucy “healthy”.
So far Megan has been spared of any illness. She and Sophie share a bedroom, and on Tuesday night Megan woke up and moved herself to our bed saying that she did not want to breath in whatever Sophie was throwing up. Lucy too has not shown us any signs that she has what Jack or Sophie has, but she is “off”. We were at DuPont on Friday for an appointment with the cardiologist who specializes in arrhythmias(I’ll update in another post about this appointment), while we were there Dr. R saw our girl and ordered labs and blood and urine cultures, he agreed she does not look good and is concerned.
We are praying for good health all the way around, that Jack starts feeling better, and that whatever is causing Lucy’s symptoms disappears without a trace.
Thanks as always for your thoughts and prayers.
A snow storm, an autonomic storm, a pumpkin shortage, a migraine headache, fever, vomiting, and strep throat tried to trick us into not celebrating Halloween his year, but we managed to find the treat in celebrating. No, Lucy is not the sick one (yet), it’s Jack.
Happy Halloween!
We are bound and determined to celebrate Halloween-at- home this year, and we were going to kick off our home Halloween weekend with a hay ride out to the pumpkin patch, but… it’s snowing, it feels more like we should be getting our Christmas tree. I stepped foot into a Target for the first time in months on Thursday looking for wings for a monarch butterfly costume that Sophie very firmly stated she wanted to be for Halloween. Although yesterday she very firmly stated that she wanted to be a “white kitty cat, the very furry kind”, seriously. Guess where I found the costumes… right next to the Christmas decorations, what!?! I already feel behind the eight ball so to speak all of the time, seeing Christmas decorations out so early just doesn’t seem right, anyone else want to rally around this with me? I have been feeling more than ever that I just want life to s-l-o-w down!
I woke up last Sunday morning fully prepared to go to church when I leaned over to Drew and said lets take the kids to Sesame Place instead today. It was a warm and beautiful fall day, perfect “Lucy weather”, he smiled and in an instant plans changed and a day full of memories were made. I am so grateful that we seized the day knowing what I know now.
Lucy is “off”, something is not right with our girl. She looks, for lack of a better word, “sick”. She is sleepier, grumpier, sneezier…her symptoms are similar to those of the seven dwarfs. Her coloring is, well, whiter than the snow covering our front yard. Her body temps and heart rates have all taken a dive down. Clearly her autonomic nervous system is not happy. We are praying that this is just a bad mito day and nothing worse, but my mama radar is saying something is up.
Hopefully the snow melts in time for trick or treating on Monday, if not our kids are all going trick or treating as ski bums.
Lots of memories were made during our beach vacation… it’s hard to believe that it was just a little over a week ago that we were basking in the sun, with the wind at our backs, and sand in between our toes. We thought long and hard about how a vacation like this would work for our family. As you can imagine bringing four children anywhere is challenging, but having one very “special” child, one who can not get wet because she has four IV lines hanging off of her body at any given time, a urinary catheter taped to her leg for most of her day, two drainage bags attached to her g and j tubes, for the next month a cardiac event monitor strapped to her chest, temperature regulation issues, and at times has sensory issues beyond comprehension….well, you can only imagine the challenges that we faced. Lucy’s first time at the actual beach lasted, at best, ten seconds. She was drawn to it however, I think watching “her kids” run in and out of the waves and play in the sand with such enthusiasm intrigued her, despite her nervous system signaling her brain that it was too much. By the end of the week we were ALL able to enjoy the beach for forty-five minutes as a family of six! We rented a house right on the beach so that our three water babies could enjoy the sun, sand, and surf whenever they desired and for as long as they desired, all the while keeping Lucy on her strict schedule. While Lucy napped, the mama read two novels and buried her feet in the sand, the daddy built sand castles and hunted for sea shells, and the kids were thrilled with just being at the beach!
When we left, more than a few tears were shed, and conversations about doing this again next year were had…all signs of a great vacation!
That is if we would let her. Our kiddos have been fund raising machines this year, coming up with all sorts of ways to raise money: pizza night, lemonade stands, fruit and vegetable stands, carnivals, bracelet sales, comedy shows… and in their own way raising awareness for mitochondrial disease all in an effort to “save Lucy’s life”. For our kids, this is something that they CAN do to make a difference, what we as parents need to do is to gently inform them that raising money for a cure does not mean that there will necessarily be a cure in Lucy’s lifetime, but our hope is that their will be. This is so hard!
We made it to the beach! We still can’t believe it, packing and planning for this beach get away was almost too much for this mama who prides herself on doing the impossible. Lucy’s needs are enormous! Planning, packing, coordinating shipping of her medical supplies and medical therapies with our infusion company was nearly impossible, refilling prescriptions, staying on top of her med schedule, changing TPN on the side of the road (I have changed TPN in the most unusual places from the Magic Kingdom to a remote parking lot on the side of the road), and packing just the basics for our family of six, was A LOT!
It took me almost all of the three days I was home from the hospital with Lucy to make this happen. Drew and I nearly got divorced (ok not really, but fatigue and stress are not good for anyone much less anyone's marriage) in the process of packing to get away…but we made it… still married I might add.
Even though we had to pack what seemed like our entire house and a hospital to leave town, we need this week away…I need this week away!
We finally made it home tonight a little before 7:00PM…can you say loooooong day!
We were discharged this morning at 11:00 AM, just in time to head downstairs for an outpatient therapy appointment with Lucy’s PT. Afterwards we went straight to the outpatient pharmacy to attempt to pick up a prescription that I normally order from another pharmacy, but did not because we have been in the hospital for the past twelve days. Lo and behold, the script that the resident wrote was incorrect, so we proceeded to march upstairs to track down someone who could call in the correct script. While upstairs we were getting ready to begin the marathon of med infusions that occupy a majority of our day when Lucy’s small lumen, the one that would not flush well yesterday, began alarming “down occlusion”. This occurred at the same time that Dr. K just so happened to be on the floor checking on his patients from yesterday, he was shocked that her line was not flushing as he said it flowed like a dream when he checked it in the OR yesterday. He ordered Lucy’s line to be TPAed (Is that even a verb?), which meant that we had to be readmitted!
The first round of TPA did not work, so we attempted to try again. Five hours later, Dr. K came upstairs with the IV nurse to try it for himself, it was then that it began to flush better than it had, and even better we were able to draw back blood. Discharge papers were signed again and we were on our way home!
Now that we are home we began the process of organizing all of the meds, supplies, and med schedule. It is going to be a long week as the only time during the day where Lucy is not being infused meds is between 2:00 and 5:30 AM and PM. I am going to need to take advantage of those three and a half hours twice a day to nap. The reward for a week like this is going to be a beach vacation!
We are all happy to be sleeping, if only for a few hours, under one roof tonight!
It has been a long day, week, admission…
We went down to the OR around 1:3o. They were ready for our girl. Dr. K explained his plan and told us that it was going to take him as much time as it would take him…words that were both comforting as well as worrisome.
(Dr. K made Lucy a tiny gauze heart to cover up her sutures:)
A little over an hour later he came out and told us that things went well. He had a surgery fellow assist with the procedure, Dr. K said that he was breathing down his neck the entire time. He explained to the fellow that this CVL surgery is life saving surgery for our girl, without this line she would not be here. Dr. K is very particular about how he places a line in a kiddo like ours…in other words he told the fellow not to screw up!
When Lucy came out of the OR that is when things became difficult. Dr. K left her femoral line in because she was still infusing her antibiotics through that line. The plan was to get her to the pediatric anesthesia care unit (PACU) and pull her femoral line before she woke up fully from anesthesia. Once they began snipping the stiches on Lucy’s leg she woke up, and might I add, not happy!
They called us back just as soon as her fem line was pulled. She was unhappy and made sure everyone knew about it. We were waiting for radiology to take an x-ray of her new line so that we could get the ok to use it. This is when I noticed that they were only running D5 through an IV in Lucy’s foot! Lucy is used to triple the amount of dextrose at quadruple the rate they were infusing it. Needless to say her glucose plummeted and the PACU went on a wild goose chase to find Lucy’s TPN, you can’t connect her old TPN line to a new clean line because of the risk of infection. Only here’s the problem, TPN was never ordered for Lucy tonight as we were planning on going home this evening on our home TPN bags. This caused quite a stir in the pharmacy, on our floor, and in the PACU… what fluid were they going to give Lucy? As we were waiting for a higher dextrose fluid to be ordered for our girl I instructed the PACU nurse to bolus Lucy 60cc’s of D10 straight into her IV to help bring up her glucose, this is when the nurse told me she needed to get orders to do so…really!?! Thankfully a nurse who knows Lucy from the ER was working in the PACU, she remembered Lucy’s hypoglycemia issues and agreed to blousing her, telling the other nurse that mama knows best. Oh the drama…it only continued when they were trying to figure our what rate to run her D10 at, nobody could seem to get an answer. Poor Lucy she was cranky and irritable and her parents were quickly feeling so as well.
We left the PACU just as quickly as the nurse could document all that needed to be documented and we were returned back to our room where they immediately doubled the rate of Lucy’s D10 infusion. When we arrived were still had not received word that we could use Lucy’s new line. People started panicking as we only had one point of access, in Lucy foot mind you, to infuse everything. The IV team nurse finally got approval to use the line, this is when we noticed that one of the lumens on her line would not flush…ugh! The hope was that it clotted off and we could fix it by locking heparin in it for an hour to break up what if anything was blocking the line. This is when we made the decision to stay another night instead of going home.
The line is working and we are able to run continuously through it, but it is sluggish. We are going to talk to surgery about the line in the morning. As for tonight, we are working on getting Lucy back on schedule with all of her meds. She is on an every six hour med schedule with her antibiotics that run for two and a half hours, thirty minutes prior to running her antibiotics we need to infuse IV Benadryl and IV Tylenol to help with the red mans that Lucy gets from the vancomycin. This is in addition to the nine other med infusions that we run daily…yeah, sleep is going to be an issue this week.
Even though we are anxious to go home, I am welcoming the nursing help tonight as we work at getting Lucy’s meds back on schedule. We have just “a few” details to work out in the morning, but we should be all set for discharge sometime tomorrow morning.
Our weekend actually went by faster than I thought it would. Medically, Lucy did just what Dr. R ordered…to behave! Her cultures have all been negative, which means that our fear of reinfection is no longer, and her new line can be placed. We are scheduled for surgery tomorrow afternoon with Dr. K again. Afterwards we are hoping to pack up and go home! I spoke with our home infusion company on Friday and gave them a heads up that Lucy has a few new meds that she will be coming home on, one of the more difficult meds to find will be her IV Tylenol. This is a relatively new med to become available in IV form, and I for one am thrilled that this med is available for our girl! Our options for pain and fever relief are minimal as all of Lucy’s meds need to come in either IV or transdermal from. Our infusion pharmacist has never had an order for IV Tylenol or even knew that Tylenol came in IV form, but told me if DuPont has it then she should be able to get it.
On Saturday the kids came to the hospital to help celebrate a birthday for a Wyatt’s sister…unfortunately, we know all too well how being in the hospital can interfere with family/holiday plans. Our family, combined with Wyatt’s family, and the third mito musketeers family made for one big party. Three mito kids and all of their pumps, wheelchairs, tubes, and medical needs at one birthday party was a nurses nightmare, but all went well.
Today was a cold and gloomy day, Lucy and I took lots of walks around the hospital to pass the time. Our friend Cherie came to see us and gave Lucy a mani/pedi. Lucy chose to paint her toes purple…I wonder what the OR staff is going to think about that tomorrow.
Please say a prayer that tomorrow goes as smoothly as possible as we are looking forward to going home!
Really, I will be saying TGIM on Monday when Lucy can get her new line put in and we can go home. Today was another day of waiting for bacteria to grow, or rather not grow…so far no growth. Medically speaking today was an uneventful day, Lucy’s red mans was better than yesterday, but not gone. We are watching her carefully for any increased signs of reaction. Her blood pressures are on the rise and we are not really sure why, we are keeping a close eye on those too. She is on a pretty hefty dose of clonidine so her pressures are concerning, but for the moment we are monitoring them closely.
Today I had a meeting with the new palliative care doctor that DuPont recently hired. She came from CHOP and trained with the very established palliative care team there. She knew about Lucy and our family from our consult that we had with the palliative care team at CHOP way back in December, and was very excited to meet us. Due to the multiple hospitals stays that we had over the winter and spring I never really followed through with the CHOP palliative care team. I was very excited to learn that DuPont has started their palliative care program. It was difficult to discuss all that we needed to discuss without Drew here and with Lucy demanding this and that. We agreed that we should arrange to meet soon without distractions and with my husband next to me.
Drew and the kids came to the hospital tonight for our usual Friday night pizza night. We told them that we were not going to the beach tomorrow…but rather we will be going next week! Drew and I are rearranging everything to make this happen.
Tomorrow we have a few fun things planned here at the hospital with the kids. We are trying to make the best of a less than ideal situation.
I am at the point in this admission where I just want to go home…I have found, for me, that there is that point in every admission where you just want to go home, today I hit that point. I remember Dr. R telling me last year that you need to take a break from here in order to make it through a longer than a week admission, he has no scientific proof of his theory, just a lot of personal experience. My break has come in the form of friendship and support from some fellow mito moms and the wonderful nursing staff that take care of us while we are here…I just love it when God places the right people in your life at just the right time.
We learned today that Lucy’s allergic reaction to Vancomycin, the antibiotic we are using to treat Lucy’s infection, increased. She has become more sensitive to the med than she used to be, and developed a more severe case of red man’s syndrome. Her heart rates and blood pressures were elevated, she turned a blotchy bright red, and she began aspirating her saliva all in a matter of minutes of administering the med…here I have been thinking that she just looked rosie from her blood transfusion. In addition to IV Benadryl, we added IV Tylenol to treat the red mans as well as slow down the already slow infusion rate to run over two and a half hours. Nobody knows why exactly the Tylenol helps to counteract the red mans, but it is helping.
We are on “auto pilot” until Monday according to Dr. R. We are scheduled in surgery Monday afternoon, that is if Lucy’s cultures are negative…so far no growth. Dr. R signed out for the next week as he is off to Haiti for a medical missions trip…when he left he gave orders to Lucy to “behave”.
Drew and I are working on trying to rearrange our beach vacation…we really need to get away! Hopefully, we can figure something out, we have not told the kids yet that we are not going this weekend…I don’t want to tell them until we have plans B through Z in place.
Thank you for all of your thoughts and prayers, love and support. We could not do this alone!
We went down to the OR around 1:30 this afternoon. The surgeon who pulled Lucy’s infected line and placed Lucy’s temporary femoral line was the infamous Dr. K, who has a reputation for being an amazing surgeon with a great bedside manor. He also happened to be the one to tell me that there is no way he is going to place a more permanent central line in Lucy on Friday, but he would put her on his schedule for Monday afternoon. What!?! He thought Dr. R was going to tell me this, apparently something got lost in translation as I did not get this message yesterday when I asked Dr. R about a possible discharge on Friday. Dr. R told me that if I want to go to the beach I should go with him to Haiti this weekend…umm no thank you, I have a different beach in mind. Dr. K’s reasoning behind not placing a line in Lucy until Monday is that the risk of reinfection is too great, if we place a new line too soon we run the risk of the bacteria sticking to the new line and then we are right back where we started.
To add insult to injury while Lucy was in the OR there was an urgent need for an isolation room as the hospital is full and they need to find places to put patients. Long story short… Lucy and I lost our private room as she is technically not on isolation, but is severely immune compromised and can not be around other “sick” kids. They moved us out of our room while we were waiting for Lucy to come out of the OR. The nurses on our floor were mortified that the head nursing supervisor made this decision and have been very apologetic and sympathetic to us. They placed Lucy in a room with a child who is of no risk to Lucy’s health and are going to work at finding her a private room just as soon as one becomes available. We work so hard to protect our girl from germs because we have been told that we have to…oh the irony.
As for Lucy’s procedure, her old line was removed without any problems; however, her femoral line did not go in as easily. Dr. K came out of the OR telling us that Lucy is “a pain to try and put a line into”. Which confirmed to him even more that we need to try and preserve every line that we put in our girl. He apologized to us for the mess he made on her legs, two lines broke and multiple attempts were made before he was successfully able to place a line. Lucy woke from anesthesia easily and was less grumpy than usual, a bright side to our day. She even let me rock her for a few minutes before asking to lay down in her bed.
When we came back upstairs we learned that another mito buddy was admitted on the unit. Three of Dr. R’s mito kids are on the floor, all three of them are affectionately referred to as the three musketeers down in urology, they are calling them the three amigos up here on the floor. If we have to be here it is nice to have the company of others who know how it feels to be living with mito… even though I would never wish this disease on anyone.
On our agenda today was a bath and blood…not a blood bath, just to be clear. Lucy was originally scheduled to be transfused in day med today, but since we are currently in-patient we transfused her from the comforts of her hospital crib. Her skin now has a pinkish glow and her cheeks are rosie red…she looks so warm and beautiful, and she smells good too.
This morning we woke to negative cultures only to learn ten minutes later that her cultures were indeed positive, and so began the game of trying to get an OR time slot. We were unable to get an add on time slot today, but we are on the OR schedule (not an add on, we are actually on the schedule) for tomorrow… we just don’t have a time yet. I, of course, am counting down the days until our beach vacation…if we pull the line tomorrow, then we wait two days for negative cultures, then put in a new line on Friday, we could be discharged in time to leave for the beach on Saturday. Of course, all of this is contingent on Miss Lucy behaving, cultures not growing in the next two days, and surgery having an OR time slot on Friday to place her new line. For a mama who has her days planned down to the minute on most days, these are the moments that I have to trust in the powers that be that all of this is part of a much bigger plan.
My mom is here helping us with the kids this week. She came over and had lunch with us in the new cafeteria, which coincidentally is very much like the old cafeteria, just a different color. I then spent the better part of my day trying to convince Lucy to take a nap, she was tired since she had only slept three of the last twenty-four hours, but she could not fall asleep again. Eventual, she succumbed to the fatigue and fell asleep ten minutes before her daddy arrived. She slept the entire time he was here, thankfully we were able to Skype with the kids and daddy just before bedtime…we are missing everyone.
Today started like many days in the hospital…early! Lucy has to have labs, labs, and more labs drawn daily for cultures, a CBC, antibiotic levels, and to check her chemistries. All of this happens somewhere between 3:30 am and 5:50 am…which is early! I learned from morning rounds that Lucy’s cultures are still positive for the same bacteria, the lumen that was negative yesterday is now positive again and the other lumen continues to remain positive for the bacteria. Apparently it is a “sticky” bug that doesn’t like to leave. Dr. R discussed a plan with me today which made me happy…having a plan that is, and not necessarily the plan, just to be clear. If today’s cultures are still positive, then the plan is to head to the OR sometime tomorrow to have her line pulled and replaced with a temporary femoral line as Lucy’s life depends on reliable access. We then have to wait two days for negative cultures, if cultures are negative then on Friday she will head back to the OR to have a new permanent line placed just in time for us to go home, pack, and head for the beach! Darn this child, she always cuts things to the last minute…I wonder who she gets that from.
Our days here have been long. Sleep is always a challenge, it seems to take a few days to get back on a schedule. Entertaining Lucy is also a challenge. Her attention span has been short, not unlike most toddlers, but she is unlike most toddlers and is confined to a bed and connected to machines with a half dozen lines coming off her body. She and I are going a little stir crazy staring at the same four walls, we have plans to head downstairs tonight to the PACU waiting area and visit the fountains. During this admission we have some fellow mito friends who are staying in the room next to ours, it has been nice to have someone else to talk to who “gets it”. Please keep Wyatt in your prayers as he is battling yeast in his line making him one very sick kiddo.
I feel a little guilty saying this, but it has been a bit of a break for me having all of the nurses on the floor taking care of the numerous daily medical needs for Lucy. They keep telling me that it is a break for them having a mom who is so involved in their child’s care, I guess you could say it is a win win all the way around. Lucy has a pretty grueling med schedule, currently she has sixteen IV med infusions daily in addition to j-tube meds, and an every three to four hour cathing schedule, she is the definition of high maintenance!
I miss Drew and the kids, we were unable to see them today as our Monday schedule is pretty full without adding a visit to the hospital in the evening. Hopefully tomorrow we will be able to eat dinner together, the newly remodeled cafeteria reopens tomorrow for the first time in nearly two months. We are definitely going to have to check it out.
Thanks as always for your thoughts and prayers, love and support, it means so much!
By positive, I mean Lucy’s cultures. We are continuing with the current antibiotics because in theory they should be able to kill the bacteria identified in her line. Of course with Lucy, everyone knows that she likes to take the road less traveled. Despite this quality about our daughter, I too am going to remain positive that we are on the right track.
Lucy continues to remain stable. Her labs look “ok”, with the expectation of her usual awful lab values. She looks puffy, but then again that seems to be more her norm as of the last six months. Her albumin levels were critically low, so this afternoon an infusion of albumin was ordered. Here’s hoping that with the extra protein on board that we can pull off some of the excess fluid in our girl. The albumin combined with a very high does of lasix should help to make a difference…that is if Lucy’s body chooses to respond.
Drew and the kids were here this afternoon. I took the big kids out for lunch while Drew and Lucy spent some quality time together. After lunch we ran a few errands and then came back to to the hospital where our friend Alicia came and picked up the big kids and took them back to her house so that Drew and I could spend some time together..foreign concept these days. We were able to watch the Packer game together all the while talking about how we are going to schedule the next few days.
We still have our eyes set on sunny skies, ocean waves, and sand in between our toes…here’s hoping we are out of here soon so that we can make this a reality.
Thanks for all of your thoughts and prayers, love and support, we could not do this alone.
PS I found my camera cord and will post pictures again soon… in case you were wondering where our kids have gone .
I may not have made it very clear in my last post, positive cultures mean that we absolutely positively have to be admitted…ugh! I will be the first to admit that when I received word that Lucy’s cultures came back positive I acted like a big baby about having to come in on a Friday night. I know, not very, mature, but I was not prepared mentally for an admission last night…I was ready to crawl under the covers of my own bed and go to sleep after the loooonnnggg week that we had. I put on my big girl panties so to speak and quickly made arrangements for the other kids, dressed them in their pajamas, grabbed our suitcase which I had packed from our earlier trip to the ED, and we headed out. We arrived at the ED around 10, they were expecting us and even had all of Lucy’s bands ready for her including her allergy band which simply read, “see list exclamation mark”. By the time we were brought up to our usual floor and went through the usual protocols it was well past 3:00am. Drew stayed with Lucy and I until we came upstairs and then went home to catch some zzz’s so that he could be the designated driver for the girls first dance classes of the season this morning.
Medically speaking, it was an uneventful night which was just fine with me. Sure we had a lot of meding, cathing, labs, and a dressing change to do, but Lucy was stable for which we were all thankful. Sleep was not in the cards last night, but hopefully in the cards this afternoon. We learned shortly after arriving at the ED that both blood cultures from Lucy’s line grew gram positive bacteria. In our eyes, this means that the likelihood of it being a contaminate and not an actual infection is decreased.
Dr. R came in this morning afraid of the mood I was going to be in, it’s not his fault but he knows and understands the challenges that come along with ALL of this. We know the drill, we need two negative cultures in a row before we can even talk about going home. We also we need to identify the specific bug(s) that Lucy is growing so that we can get her on the correct antibiotics. All of this is trying on one’s patience… Of course we have plans in six days that may or may not include the ocean, sand, and some quality family time. As we know all too well, Lucy has a tendency to make plans of her own.
Recently we had an opportunity to take all of our kiddos to Hershey Park, a local amusement park, famous for the smell of chocolate in the air and roller coasters, lots of them. Drew, Jack, and occasionally Megan and Sophie took advantage of as many of the rides as possible, Lucy and I rode the handicap accessible train over and over with the occasional carousel ride thrown in for variety. Drew kept asking me if I wanted to trade places with him so that I could ride a roller coaster or two. No thank you, our life is enough of a roller coaster ride for me .
Last weekend we participated in the DelVal 2nd Annual Energy for Life Walkathon…a high. On Sunday we had issues with Lucy’s CVL (central line) which lets just say caused me to want to throw her pumps out the window…a low. One Monday, one of the lumens on her line stopped working all together causing us to spend most of Monday at the hospital doing dye studies, consulting with surgery, tracking down Dr. R, and eventually landing in day med clot busting her line…another low. On Tuesday morning Lucy started back with music. She did better than I expected as did I…a high. Therapy was a bit of struggle this week as it is becoming more and more obvious that our girl is just not able to tolerate as many of the activities that she once did…a low. One Thursday morning I received a call from Lucy’s cardiologist that her holter monitor from last week came back showing ventricular tachycardia. Lucy is going to need to wear an event monitor for the next month to get a better picture of what precisely is going on with her heart…major low. We had a productive appointment with Dr. R Thursday afternoon…I guess you could call it a high. He discussed some options for our girl that are not necessarily what a mama wants to think about…a low. Last night Lucy woke with a fever, which landed us in the ED for most of the day…definite low! We drew cultures and Dr. R gave her a round of antibiotics and sent us home, telling us that he would call if anything grew …a high cause we did not automatically get admitted. Tonight we had dinner and movie night with our kids while Lucy slept…a high. After dinner Dr. R sent me an e-mail that Lucy’s cultures are positive…a big time low.
We are currently on our way back to DuPont for the seventh time this week! I would like to get off this ride please… I will update more when we know more.
